Phẫu Thuật Tạo Hình (Tập 5): Tuyến Vú – Neligan (2018)

488.e1
References
References
1. Smith BD, Smith GL, Haffty BG. Postmastectomy radiation and
mortality in women with T1–2 node-positive breast cancer. J Clin
Oncol. 2005;23:1409–1419.
2. Kane GM. Therapeutic radiation: principles effects, and
complications. In: Neligan PC, Gurtner GC, eds. Plastic Surgery. Vol.
1. 3rd ed. Principles. New York: Elsevier Saunders; 2013:654–675.
Comprehensive explanation of the physics and treatment plans for
radiation therapy as it applies to breast cancer patients.
3. Kronowitz SJ, Robb GL. Radiation therapy and breast
reconstruction: a critical review of the literature. Plast Reconstr Surg.
2009;124:395–408. A summary of the literature on radiation therapy and
its impact on breast reconstruction outcomes.
4. Moran MS, Rowe BP. Accelerated partial breast irradiation and
hypofractionated whole breast radiation. Oncol Hematol Rev.
2011;7:31.
5. Tong WM, Baumann DP, Villa MT, et al. Obese women experience
fewer complications after oncoplastic breast repair following partial
mastectomy than after immediate total breast reconstruction. Plast
Reconstr Surg. 2016;137:777–791.
6. Kronowitz SJ, Kuerer HM, Buchholz TA, et al. A management
algorithm and practical oncoplastic surgical techniques for
repairing partial mastectomy defects. Plast Reconstr Surg.
2008;122:1631–1647. Well-organized outline of how to approach
oncoplastic reduction with regards to patient selection and surgical
technique.
7. Lentz RB, Craig ES, Ross CC, et al. Does the left hand know what
the right hand is doing? What plastic surgeons need to know about
radiation therapy techniques. Plast Reconstr Surg. 2012;130:772e–
773e. Explanation of the common hurdles radiation oncologists face with
treating breast cancer patients and its impact on outcomes.
8. Seth AK, Hirsch EM, Fine NA, et al. Utility of acellular
dermis-assisted breast reconstruction in the setting of radiation: a
comparative analysis. Plast Reconstr Surg. 2012;130:750–758.
9. Kronowitz SJ. Delayed-immediate breast reconstruction: technical
and timing considerations. Plast Reconstr Surg. 2010;125:463–474.
Well-organized summary of the commonly used delayed-immediate
breast reconstruction approach to patients at risk for needing radiation
therapy.
10. Spear SL, Clemens MW, Dayan JH. Considerations of previous
augmentation in subsequent breast reconstruction. Aesthet Surg J.
2008;28:285–293.
11. Clemens MW, Kronowitz SJ. Current perspectives on radiation
therapy in autologous and prosthetic breast reconstruction. Gland
Surg. 2015;4:222–231.
12. Panettiere P, Marchetti L, Accorsi D. The serial free fat transfer in
irradiated prosthetic breast reconstructions. Aesthetic Plast Surg.
2009;33:695–700.
13. Crisera CA, Chang EI, Da Lio AL, et al. Immediate free flap
reconstruction for advanced-stage breast cancer: is it safe? Plast
Reconstr Surg. 2011;128:32–41.
14. Makmur L, Lim J, Lim TC. Radiation therapy in immediate breast
reconstruction with DIEP flap. Plast Reconstr Surg. 2003;112:920–921.
15. Clarke-Pearson EM, Chadha M, Dayan E, et al. Comparison of
irradiated versus nonirradiated DIEP flaps in patients undergoing
immediate bilateral DIEP reconstruction with unilateral
postmastectomy radiation therapy (PMRT). Ann Plast Surg.
2013;71:250–254.
16. Taghizadeh R, Moustaki M, Harris S, et al. Does post-mastectomy
radiotherapy affect the outcome and prevalence of complications in
immediate DIEP breast reconstruction? A prospective cohort study.
J Plast Reconstr Aesthet Surg. 2015;68:1379–1385.
17. Chatterjee JS, Lee A, Anderson W, et al. Effect of postoperative
radiotherapy on autologous deep inferior epigastric perforator flap
volume after immediate breast reconstruction. Br J Surg.
2009;96:1135–1140.
18. Baumann DP, Crosby MA, Selber JC, et al. Optimal timing of
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2015;22:361–369.

SECTION II • Reconstructive Breast Surgery
26 
Surgical management of breast cancer-
related lymphedema
Jaume Masià, Gemma Pons, and Elena Rodríguez-Bauzà
SYNOPSIS
■ Breast cancer-related lymphedema (BCRL) is the result of impaired
lymph drainage from the ipsilateral arm following surgical or
radiotherapy treatment.
■ BCRL develops in as many as 21% of women after breast cancer
treatment. This disabling chronic condition has a high impact on
quality of life and is a source of significant morbidity.
■ Advances in imaging techniques have allowed us to better understand
the anatomy and pathophysiology of the lymphatic system, and greater
mastery of supermicrosurgical techniques has given us the opportunity
to restore a dysfunctional lymphatic system in selected cases.
■ As yet, no optimal treatment has been found, but the use of
reconstructive approaches, and excisional or reductive techniques,
mainly liposuction, allow us to improve the quality of life of patients in
most cases.
Access the Historical Perspective section online at

Introduction
Breast cancer is the most frequent cause of lymphedema
in western countries.1 For many years, lymphedema
treatment has been based on conservative physical and
medical therapies. These treatments are often time-
consuming, difficult to perform on a regular basis, and
cumbersome, and such barriers lead to high rates of
non-compliance and patient dissatisfaction.2 Since the
early 20th century, several surgical techniques have
been described in an attempt to complement or provide
a more effective alternative to these conservative treat-
ments. Such surgical techniques are considered either
reductive or reconstructive. Reductive techniques, such
as Brorson’s liposuction,3 aim to reduce the hypertrophic
adipose and fibrotic tissue characterizing the intermedi-
ate and later lymphedema stages, while reconstructive
techniques, such as autologous vascularized lymph
node transfer (ALNT),4 derivative lymphovenular tech-
niques,5 and lympholymphatic bypass,6 aim to improve
or restore the functionality of an impaired lymphatic
system.
Nevertheless, no standardized protocol for lymph-
edema surgical treatment has yet been established. In
this chapter, the authors describe their approach in
surgical management of lymphedema, emphasizing the
importance of the preoperative assessment.
Key points
• Preoperative assessment identifies potential candidates for
reconstructive or reductive surgical techniques.
• ICG-lymphography is essential as the first step in the
assessment to appraise the viability of the lymphatic
system.
• Other imaging techniques help to determine the optimal
surgical technique for every patient:
• Lymphoscintigraphy is helpful to define the lymphedema
pattern and to know how many lymph nodes remain
active in the affected limb.
• MR-lymphography provides a 3D reconstruction of the
whole limb and shows both the superficial and the deep
lymphatic system.
• CT-angiography is crucial to minimize potential donor site
morbidity when an autologous lymph node transfer is
planned, especially when the flap is harvested from the
inferior epigastric system.
• Intraoperative ICG-lymphography improves precision
when harvesting the lymph nodes at the donor site.

489.e1
Historical perspective
Historical perspective
Over the 20th century, various surgical procedures, both
reductive and reconstructive, were developed in an
attempt to treat lymphedema of the limbs.2,7
Reductive methods
In 1912, Sir Charles published the first reported surgical
procedure for lymphedema.8 The approach consisted of
the massive resection of scrotal and skin and subcutane-
ous tissues, and the resulting wound was repaired using
a split-thickness skin graft. Some years later, Sistrunk
(1927) and Thompson (1967) applied similar techniques
to treat upper limb lymphedema arising from breast
cancer surgery.7 However, these procedures were highly
invasive, and post-surgical pain, healing complications,
infections, and lymph fistulas were common.
Liposuction was also first described many years ago
to treat lymphedema. Brorson3 reported results of pro-
spectively followed patients with overall favorable
results and proposed this as the surgical method of
choice. Liposuction effectively reduces volume in
therapy-resistant lymphedema of the limbs, but it carries
a potential risk of damage to residual lymphatic vessels.
Furthermore, it requires life-long management involv-
ing the use of compression garments and devices.
Reconstructive methods
Flap interposition
In 1950, Sir Harold Gilles described the first flap inter-
position for treatment of lower limb lymphedema by
transferring a flap of skin and subcutaneous tissue from
the arm to the affected groin in a two-stage operation.9
The concept of flap interposition was the precursor of
lymph-adipose tissue transfer; that is the transfer of
lymph nodes and surrounding adipose tissue in an
attempt to provide the nodes with essential nourish-
ment. In 1966, Goldsmith10 proposed the usefulness of
greater omentum flaps, but this approach was phased
out because of its high morbidity and unpredictable
outcomes.
Lymphaticolymphatic bypass
Baumeister and Siuda6 reported an approach to upper
limb lymphedema in which healthy lymphatic vessels
from the medial thigh area were used as a composite
graft. However, the morbidity of the donor site was not
negligible.
Some years later, Campisi et al.11 described a vein
interposition graft between the proximal and distal
lymphatic vessel bundles to bypass the obstructed area.
In this procedure, multiple lymphatic vessels are
inserted into the distal cut end of a vein graft and
secured by sutures, and lymphatic vessels in the supra-
clavicular area are anastomosed with the other end of
the graft.
Lymphovenous bypass
With the development of microsurgical procedures, a
new world of possibilities opened to surgeons. The
concept of a lymphovenous shunt operation was first
described by Laine in 1963 using a rat model. Yamada12
performed similar experiments in dogs and then applied
this technique to treat lower limb lymphedema in
patients. O’Brien et al.13 used the same approach and
reported on their clinical experience in treating lymph-
edema in the 1970s. Many others have refined the tech-
nique and since reported on their clinical experiences.7
In the last decades, technologic advances in micro-
surgical equipment allowed the development of
supermicrosurgery techniques. These techniques aim
to anastomose vessels at the capillary level with a
diameter of 0.8–0.3 mm. Koshima reported the first
cases of supermicrosurgical lymphovenous bypass.5
The difference between the work of O’Brien and
Koshima is marked by the technologic development of
ICG-lymphography and the development of supermi-
crosurgical instruments and sutures of 12-0 and 13-0.
Microvascular lymph node transfer
Becker et al.4 reported treating upper limb lymphedema
by transplanting composite soft tissue including ingui-
nal lymph nodes to the axilla and/or the elbow region
in the affected limb.
The concept of neo-lymphangiogenesis has a lot of
weak points because our knowledge about the repro-
ducibility and predictability of this technique is poor
and many factors play a role, such as the anatomic loca-
tion of lymph nodes in axilla, variation in suitable donor
sites, and factors that define the physiological intranodal
bypass. Further research is needed to understand the
mechanisms involved.

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SECTION II
CHAPTER 26 • Surgical management of breast cancer-related lymphedema
After an accurate clinical evaluation, an assessment
using diagnostic imaging techniques is performed to
study the functional and morphologic features of the
lymphatic system so as to provide the best therapeutic
option to each patient.
Clinical evaluation
A detailed anamnesis is essential to assess the cause of
the lymphedema and its long-term course. This step
differentiates between pitting and non-pitting edema, as
non-pitting edema argues for an advanced stage of the
disorder, characterized by severe fibrosis and hypertro-
phy of adipose tissue. It is also important to ask about
antecedents of recurrent acute inflammatory episodes
(cellulitis or erysipelas). Together with the anamnestic
data, clinical evaluation determines the lymphedema
stage according to the International Society of Lymph-
edema (Table 26.1).18
The clinical examination also involves measuring
limb circumferences at predetermined anatomic levels
and collecting photographic documentation. There are
several methods of measurement. Volumetry is the most
exact, but the circumference measurement is the easiest
and most fundamental method. Since the 2nd European
Conference on Supermicrosurgery in 2012, it was agreed
that measures would be taken every 4 cm from the ulnar
styloid process to the lateral malleolus in the elbow and
up to the shoulder (Fig. 26.1).
Diagnostic imaging techniques
Diagnostic imagining techniques are essential tools to
determine an appropriate therapeutic strategy for each
patient and to assess the improvement after surgery.
Our assessment protocol involves indocyanine green
(ICG) lymphography, lymphoscintigraphy (LS), mag-
netic resonance (MR)-lymphography and, in some cases,
computed tomography (CT)-angiography.
Basic science/disease process
Lymphedema can be caused by an intrinsic fault in the
lymph-conducting pathways (primary lymphedema)
or by damage from events occurring outside the lym-
phatic system, such as surgical removal of lymph nodes.
In BCRL, lymphedema occurs due to a mechanical
disruption in outflow resistance and a rise in lymphatic
pressure. The resulting lymphatic dilation is likely to
cause valve incompetence, and hence explains the
backflow of lymph, particularly towards the skin
(“dermal backflow”).14
The lymphatic walls undergo fibrosis, and fibrinoid
thrombi accumulate within the lumen, obliterating
many of the remaining lymph channels. Spontaneous
lymphovenous shunts may form. Histologically, in the
initial stage of lymphedema, both endothelial cells and
smooth muscles cells in the proximal level of the lym-
phatic trunks become damaged. Occlusions of the lym-
phatic trunks and degeneration of the smooth muscle
cells may start from the proximal regions of the limb.15
In recent years, work in BCRL has demonstrated that
obstruction to lymph drainage following removal of
axillary lymph nodes is too simple a mechanistic expla-
nation.16 It has become clear that other factors related to
lymphedema are involved, such as obesity, destruction
of pathways, and variability in the anatomy of the
lymphatic system.
Diagnosis/patient presentation
Classically, the diagnosis of lymphedema was based on
clinical evaluation supplemented by lymphoscintigra-
phy. Today, the current approach to diagnosis and
preoperative planning should be made from another
point of view, adding the results obtained by ICG-
lymphography to the clinical evaluation.17
The key to success with surgical treatment is to iden-
tify patients who still have a functional lymphatic
system and are potential candidates for reconstructive
surgical techniques.
Table 26.1  International Society of Lymphology (ISL) staging
ISL stage
Features
0/IA
Subclinical condition where swelling is not evident
despite impaired lymph transport
I
Accumulation of fluid, which subsides with limb
elevation. Pitting may occur
II
Limb elevation alone rarely reduces tissue swelling.
Pitting present, except in late stage II when more
fibrosis occurs
III
Lymphostatic elephantiasis where pitting is absent
and trophic skin changes (acanthosis, fat
deposits, warty overgrowths) develop
(Reproduced with permission from Masia J, Pons G, Nardulli ML. Combined
surgical treatment in breast cancer-related lymphedema. J Reconstr Microsurg.
2016;32:16–27.)
• Depending on the status of the axilla, in patients with
active lymphatic channels, lymphatic-venous
anastomosis (LVA) or LVA combined with autologous
lymph node transfer (ALNT) is performed.
• If in the preoperative assessment, there are no active
lymphatic channels and there is a non-pitting
lymphedema with an excess of adipose and fibrotic
tissue, the unique effective technique is the use of
reductive surgery, especially the vibro-liposuction
described by H. Brorson.3

491
Diagnosis/patient presentation
The preoperative planning assessment with ICG-
lymphography is done the day before the LVA surgery
or in the same day in the operating room. It provides a
map of the lymphatic system, and the lymphatic chan-
nels are marked on the patient’s skin surface. The pre-
operative mapping is completed with the information
provided by MR-lymphography, as explained in more
detail below (Fig. 26.3).
ICG-lymphography is also of paramount importance
during ALNT surgery. It helps the understanding of the
Indocyanine green lymphography
ICG-lymphography is an essential test image performed
as a diagnostic test in the authors’ out-patient clinic
during the first consultation. Depending on the results,
further imaging studies are planned.
This study consists of injecting 0.1–0.2 mL of indocya-
nine green dye subcutaneously at the II and IV inter-
digital webspace in both hands. The dye is captured and
transported by active lymphatic channels and can be
visualized on real-time as fluorescent channels on a
display. The entire exam is recorded (Fig. 26.2).
ICG-lymphography yields valuable information
in assessment, providing a double evaluation. First,
immediately after injection, it shows the velocity
of the contrast ascending to the axilla, and after
5 min it shows where the contrast is stored, allow-
ing evaluation of the dermal backflow. Second, it
provides valuable pre-surgical data about the degree
of impairment in the lymphatic system. It not only
gives information about the number of enhanced
lymphatic channels and their appearance but also
shows the exact location of the active lymphatic chan-
nels and their transport capacity. This information is
of crucial importance during the preoperative assess-
ment because only a patient with active lymphatic
channels can be considered a potential candidate for
LVA surgery.
The procedure is limited by the possibility to only
visualize lymphatics that are less than 2 cm deep with
respect to the skin surface. As a result, the authors now
combine ICG-lymphography with MR-lymphography,
especially when a patient is a candidate for reconstruc-
tive surgery.
Fig. 26.1  Circumference measurement in a 45-year-old woman with stage II
lymphedema. Measurements are taken every 4 cm from the ulnar styloid process to
the lateral malleolus in the elbow to the shoulder.
A
Fig. 26.2  IGC-lymphography. (A) ICG is captured by an infrared camera system,
and (B) functioning lymphatic channels can be seen on a display device as
fluorescent channels.
B

492
SECTION II
CHAPTER 26 • Surgical management of breast cancer-related lymphedema
Despite its advantages, lymphoscintigraphy does
not provide detailed morphologic and functional infor-
mation about the lymphatic system, and information
must be complemented with ICG-lymphography and
MR-lymphography.
MR-lymphography
To overcome the limitations of ICG-lymphography, we
use MR-lymphography to obtain a 3D reconstruction of
the whole limb. It shows both the superficial and deep
lymphatic system.
The authors perform MR-lymphography to obtain
more information when planning the surgical technique.
The data obtained not only assesses the lymphatic
system of the limb but also helps to select the most
suitable lymphatic channels for LVA. These can be distal
to a dermal backflow area; a sign of lymphatic obstruc-
tion. Two coordinates are chosen for each lymphatic
vessel and marked on the skin before LVA surgery.
Combining
the
information
provided
by
ICG-
lymphography and MR-lymphography, the contractile
vessels most likely to promote successful LVA can be
located (Fig. 26.4).
Computed tomography-angiography
CT-angiography is performed when planning ALNT. It
has become an essential tool to study the inferior epi-
gastric donor area and to minimize potential donor site
morbidity in the lower limb.
A
Fig. 26.3  (A) The lymphatic channels observed by IGC-lymphography and
(B) drawn on the patient’s skin thanks to the contrast medium (red color).
B
Fig. 26.4  Preoperative 3D coronal magnetic resonance lymphography showing
several tortuous lymphatic channels in a right arm with lymphedema (yellow arrows).
lymphatic pattern at the donor site limb. As the authors
normally use the superficial inguinal nodes as donor-
nodes for transplantation in the axilla, during ALNT
surgery, ICG-lymphography allows us to locate the
lymph nodes (LNs) draining the inferior limb and
exclude these from the flap. This approach helps to
reduce the risk of iatrogenic lower limb lymphedema.
Lymphoscintigraphy
Lymphoscintigraphy is a standardized imaging test
that gives a global evaluation of the functionality of
the lymphatic system. It also provides comparative
information for the postoperative assessment. Images
of the entire arm, the axilla, and the liver are taken
over 40 min. Further images are captured 60, 120, and
180 min after the injection and interpreted. The main
parameters evaluated are the tracer pathway, the time
the tracer appears at the axilla, the presence or absence
of major lymphatic collectors, visualization of nodes,
and the presence or absence of dermal backflow. If
tracer uptake in the axilla is absent, autologous lymph
node transplantation to the axilla level can be planned.
There is no reason for lymph node transfer in the axilla
in a patient with lymph node functionality at the axil-
lary region.

493
Patient selection
This technique is usually used to locate the abdominal
perforators when performing an abdominal flap for
breast reconstruction, and also to locate the lymph
nodes in view of the high anatomic variability.
The most cranial and lateral inguinal nodes are
selected. The first reason for this is that they are richly
vascularized by the superficial epigastric system or the
superficial circumflex iliac system, and the second
reason is that this approach reduces the risk of second-
ary iatrogenic lymphedema.
CT-angiography is used to identify the number and
location of the superficial inguinal nodes to be harvested
in the LN flap, and to assess the LN vascular pedicle
(superficial inferior epigastric system or superficial
iliac circumflex iliac vessels) to determine its size,
course, and exact location using exact coordinates, as
described previously for abdominal perforator flaps
(Fig. 26.5).19
Patient selection
The key point of BCRL treatment is patient selection. It
is crucial to individualize the treatment for every patient
independently of the clinical stage or the evolution time.
A complete preoperative study will determine the pos-
sible success of the technique and establish the poten-
tially effective technique.
Results from the preoperative clinical assessment
and ICG-lymphography, lymphoscintigraphy, and MR-
lymphography are of inestimable value in decision-
making. Based on our experience to date, we have
developed a treatment algorithm, the BLAST (Barce-
lona lymphedema algorithm for surgical treatment)
(Fig. 26.6):
Fig. 26.5  Axial view of computed tomography angiography showing the right
superficial inguinal lymph nodes (green arrows) and the nourishing superficial
circumflex iliac vessels (blue arrows).
Fig. 26.6  Barcelona lymphedema algorithm for surgical treatment (BLAST). (Masià
J, Pons G, Rodríguez-Bauzà E. Barcelona Lymphedema Algorithm for Surgical
Treatment in Breast Cancer-Related Lymphedema. J Reconstr Microsurg.
2016;32:329–335.)
Functional
lymphatic system
Clinical assessment
ICG-lymphography
Lymphoscintigraphy
MR-lymphography
Non-functional
lymphatic system
Reductive surgical
treatments
Power-assisted
liposuction
Non-impaired
axilla
Impaired
axilla
No amastia
LNT + LVA
Amastia
T-BAR
LVA
■ If there is no evidence of active lymphatic channels (ICG
assessment −/ lympho MRI −) and the lymphedema is
advanced and non-pitting, we opt for a reductive
technique such as liposuction according to the
Brorson technique.
■ If there is no evidence of active lymphatic channels (ICG
assessment −/ lympho MRI −) and pitting lymphedema
is present, intensive rehabilitation therapy is
performed to re-assess the possibility of a reductive
surgical technique.
■ If there is evidence of a functioning lymphatic system
(ICG assessment + and/or lympho MRI +), the
authors choose a reconstructive technique
according to the clinical characteristics of
the axilla:
• If the axillary area has good status, LVA is
performed in the affected limb.
• If the axillary area presents signs of impairment
(i.e., radiodermitis, abundant fibrotic tissue), an
ALNT approach is used in the armpit after
fibrotic tissue release, complemented by LVA in
the affected limb.
• If the patient has amastia and requests breast
reconstruction, ALNT with nodes included in
the abdominal perforator flap (DIEP or SIEA)
is opted for and LVA is performed distally on
the affected limb. The authors call this
procedure “T-BAR” (total breast anatomy
restoration).

494
SECTION II
CHAPTER 26 • Surgical management of breast cancer-related lymphedema
generally into the functional lymphatic channels, and
the transport of the lymph can be seen and the perme-
ability of the anastomosis can be verified (Figs. 26.8 &
26.9).
Autologous lymph node transfer
The procedure is based on the substitution of lymph
nodes that have been previously surgically resected or
damaged in the axilla, with a vascularized free tissue
transfer flap containing a few LNs (between 3 and 6
nodes) from non-risk donor sites.
The donor area that used most often is the superficial
inferior epigastric area with superficial circumflex iliac
vessels. Morbidity at this site is low, and the final cos-
metic result is satisfactory, as the scar is hidden. Other
donor sites are the submental, supraclavicular region or
the contralateral thoraco-dorsal axis. Before surgery,
these areas are studied using angio-CT to assess where
the superficial nodes and their vascular pedicle are. The
authors also check the number and distribution of the
deep lymph nodes, trying to make sure that the nodes
to be removed are not disturbing normal lymphatic
drainage of the lower limb.
ICG-lymphography allows us to locate the lymph
nodes (LNs) draining the inferior limb. Before surgery,
IGC is injected into the interdigital spaces of the lower
limb to locate the lymphatic system draining the leg.
This step reduces the risk of harvesting a flap containing
these LNs, which would induce an iatrogenic lower
limb lymphedema (Fig. 26.10).20
Before raising the flap (just before doing the skin
incision), the authors inject 0.1–0.2 mL of ICG and
0.2–0.4 mL of 2.5% patent blue V dye (Guerbet, Roissy-
Charles-de-Gaulle, France) intradermally in two or
three spots above and below the inguinal fold in the
potential drainage area to improve visibility of the
superficial lymphatic vessels during dissection.
To reduce the risk of seroma, there is precise dissec-
tion, clipping, and cauterizing of the tiny lymphatic
channels and side branches that are not included in the
skin-adipose-LN flap.
Within the lymph node flap, the authors include a
skin island of approximately 8 × 4 cm with some extra
vascularized fat from the superficial circumflex iliac
system. Its vascular pedicle is dissected with special
care up to the femoral vessels. The adipose tissue sur-
rounding the lymph nodes and the skin island included
in the flap are useful to replace the fibrotic tissue in the
axillary region and also to facilitate lymph absorption
through the physiologic lymph-venous shunts.14 The
skin island makes postoperative monitoring easier
(Fig. 26.11).
Fig. 26.7  Preoperative markings according to ICG-lymphography (green marks)
and magnetic resonance lymphography (red marks) data in a 44-year-old woman
with lymphedema in the right arm.
Treatment/surgical technique
Lymphatic-venular anastomosis (LVA)
The LVA procedure consists of performing skin incisions
about 2 cm length in the affected limb in order to locate
functional lymphatic vessels and to anastomose these to
subdermal veins. The objective of this procedure is to
redirect the lymph to the venous stream directly, without
going through the thoracic duct. Both lymphatic and
venule vessels have diameters of less than 0.8 mm, so
the technique used is called “supermicrosurgery”. The
intervention is performed under general anesthesia to
avoid patient discomfort in view of the lengthy proce-
dure using high magnification microscope and specific
supermicrosurgical instruments and sutures.
ICG-lymphography and MR-lymphography data
help to locate the most functional lymphatic channels,
which are likely to be anastomosed with superficial
venules. These lymphatic channels are marked on the
patient’s skin just before surgery (Fig. 26.7).
The first lymphatic channels to be explored are those
found at the coincident points obtained by these two
techniques and preferably those located in the proximal
region of the affected limb. In this way, if the anastomo-
sis is feasible, it is possible to recruit a greater amount
of lymph from the distal part of the limb. Most function-
ing lymphatic vessels, however, are found in the wrist
and at the distal two-thirds of the arm, as the proximal
vessels are normally damaged first.
At the selected cutaneous points and after injection
of a small amount of local anesthetic with epinephrine
to reduce bleeding, an approximately 2–3 cm skin inci-
sion is made. Lymphatic channels are carefully dissected
and subsequently anastomosed end-to-end or end-to-
side to subdermal venules of similar caliber, using 11-0
or 12-0 sutures. After performing the anastomosis,
between 0.1 and 0.2 mL of Patent V Blue dye is injected
about 2 cm distal to the incision. The dye is absorbed

495
Treatment/surgical technique
Fig. 26.8  A 55-year-old woman with right
upper limb lymphedema who underwent
lymphatico-venous anastomosis.
(A) Preoperative markings; (B) intraoperative
image; (C) postoperative markings.
A
B
C
Fig. 26.9  Intraoperative view of the termino-terminal anastomosis between the
proximal stump of the lymphatic vessel and the distal stump of the venule. The
lymph fluid can be seen flowing through the anastomosis along the vein.
Once the flap is isolated, the donor site is closed using
a quilting suture to avoid dead space, preferably a
continuous spiral barbed suture. This is used to co-opt
the walls of the donor site and prevent dead spaces,
which could favor the accumulation of seroma. Before
closing the area, a tissue sealant is applied to close the
small lymphatic dissected collectors (Fig. 26.12). A
suction drain is left in the donor area until the drainage
is less than 15 cc/day, and external compression with a
foam bandage is applied for 2 weeks.
As the site generally used for lymph node transfer is
the axilla, anastomosis is normally performed between
donor vessels of the circumflex scapular system, after
debridement of fibrotic tissue.
It is essential to place the lymph nodes on the apex
of the axilla in contact with the axillary tissue because
this is where the afferent dominant lymphatic channels
arrive from the arm.

496
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CHAPTER 26 • Surgical management of breast cancer-related lymphedema
Fig. 26.11  (A) Preoperative markings of the flap and its nourishing vessels in a
57-year-old woman who underwent total breast anatomy restoration surgery for left
breast amastia and upper limb lymphedema. (B) Shows the deep inferior epigastric
artery perforator flap with the adipocutaneous flap containing the nodes.
A
B
Fig. 26.12 Donor site closure using a quilting suture to avoid dead space.
A
B
Fig. 26.10  (A–C) Reverse mapping of the leg to prevent donor site lymphedema.
C

497
Treatment/surgical technique
If good lymphatic channels from the arm during dis-
section of the fibrotic tissue in the axilla are found, a
lymph-lymphatic anastomosis can be performed to the
afferent lymphatic channels from the transplanted skin-
adipose-LN flap. It is also important to place the extra-
vascularized adipose tissue making a bridge between
the healthy fat from the proximal armpit; this is the way
to use the physiologic L-V communications as an
absorbing system for the lymph. At the same time, the
contour of the anterior axilla line that normally is dis-
rupted by the axillary clearance and the effect of the
radiotherapy is improved.21
When the authors plan to approach lymphedema and
autologous breast reconstruction simultaneously using
an abdominal free flap, a compound abdominal (DIEAP/
A
B
Fig. 26.13  T-bar (total breast anatomy restoration). (A) A 49-year-old
woman who underwent immediate breast reconstruction with abdominal
perforator flap and (B) lymph-lymphatic anastomosis between the
upper limb lymphatic vessels and the afferent vessels from transplanted
lymph nodes from the inguinal area. (C) The immediate postoperative
result.
C
SIEA) flap containing the LNs with double vasculariza-
tion is raised, and in some cases, even with lymph-
lymphatic anastomosis. This is the concept called T-BAR
(total breast anatomy restoration), as noted above
(Fig. 26.13).
Liposuction
Liposuction permits effective volume reduction in
therapy-resistant lymphedema of the limbs.
Liposuction today is performed using power-assisted
liposuction because the vibrating cannula facilitates the
process. A tourniquet can be used to minimize blood
loss. Liposuction is carried out following the technique
described by Brorson.3 This type of liposuction is per-
formed circumferentially, step-by-step, from wrist to

498
SECTION II
CHAPTER 26 • Surgical management of breast cancer-related lymphedema
wearing the lifelong compression garments. Such gar-
ments consist of two sets of a sleeve and glove, custom-
made from a template based on the healthy arm.
Postoperative care
Lymph-venous anastomosis
In the immediate postoperative period (first 48 h), the
area where the LVA was performed is gently massaged
every 2 h to promote drainage. It is also highly recom-
mended to elevate the affected limb and decrease the
amount of activity. On the 3rd postoperative day,
patients start to activate muscles doing isometric exer-
cises with a rubber handball. On the 15th day, patients
can start a swimming or aquagym rehabilitation
program three times a week for 1 year. Depending on
the patient’s personal circumstances, manual lymphatic
drainage is recommended for at least 1 year, twice a
week during the first 6 months and once a week during
the last 6 months.
Autologous lymph node transfer
On the third postoperative day, muscle activation is
initiated by doing isometric exercises with a rubber
handball. On the 15th day, manual lymphatic drainage
is recommend for 1 year, twice a week during the first
6 months and once a week during the second 6 months.
Manual drainage should be directed towards the
transplanted lymph nodes in order to stimulate
neo-lymphogenesis. One month after surgery, the
patient can start a swimming or aquagym rehabilitation
program three times a week for 1 year. A light compres-
sion garment may be indicated for the first 6 months,
depending on the patient’s response.
Liposuction
In the immediate postoperative period, the arm is kept
at heart level on a large pillow with the compression
sleeve and glove in place. These garments are removed
3 days after surgery and replaced with a second set,
while the first is washed and dried. This routine is
repeated every 2 days for 2 weeks. After the 2-week
control, the garments are changed every day. Washing
is important because the shrinkage increases compres-
sion. The lifelong use of this compression is a prerequi-
site to maintaining the effect of liposuction.
Outcomes, prognosis, and complications
Outcomes and prognosis
Although various microsurgical procedures have been
described to help reduce the severity of lymphedema,
shoulder, and the hypertrophied fat is removed as
completely as possible. The authors use 15 and 25 cm
long cannulas with diameters of 3 and 4 mm.
When the arm distal to the tourniquet has been treated,
a sterilized made-to-measure compression sleeve is
applied to the arm to stem bleeding and reduce post­
operative edema. The tourniquet is removed, and the
proximal part of the compression sleeve is pulled up to
compress the proximal part of the upper arm. The inci-
sions are left open to drain through the sleeve (Fig. 26.14).
It is important to note that this therapy should be only
be used in compliant patients who are committed to
Fig. 26.14  A 55-year-old woman with advanced lymphedema and non-pitting who
underwent liposuction according to the H. Brorson technique, with 670 cc of fat
tissue extraction. (A) Preoperative images. (B) At 4 years postoperatively, with
stable results.
A
B

499
Surgical complications
occurs despite these precautions, regular percutaneous
aspiration must be performed and local compression
should be increased until complete regression is
achieved.
To minimize the risk of iatrogenic lymphedema, as
mentioned earlier, care must be taken to harvest only
the nodes located superficially and laterally to the
common femoral vessels. These nodes can be preopera-
tively located by CT scan and more easily identified
intraoperatively by performing ICG-lymphography of
the donor site to assess nodes which must be spared.
This assures us that we are not harvesting nodes drain-
ing the lower limb. Patent blue dye V is injected intra-
dermally immediately above and below the anterior
superior iliac spine, to assess those nodes which should
be harvested.
Secondary procedures
Clinical postoperative assessment is performed by
measuring limb circumferences the first month after
surgery, every 3 months in the first year, and then twice
per year, to verify the post-surgical improvement.
Limb lymphoscintigraphy (LS) is repeated 12 months
after surgery. The technique and data interpretation are
the same as those described for the preoperative exam.
LS can verify whether an improvement in the lymphatic
drainage has occurred respect to the preoperative exam.
It can demonstrate the effectiveness of ALNT, as the
viable transplanted nodes can be visualized in the
recipient area and new lymph drainage pathways can
appear. LS can also assess the patency of an LVA by
means of indirect findings. For example, it can show the
reduction of the dermal backflow or the disappearance
of the tracer in a site of an LVA due to tracer passage
into the blood circulation.
Postoperative ICG lymphography is repeated 1 year
after surgery. The technique is the same as that described
for the preoperative exam. Postoperatively, ICG-
lymphography evaluates whether the lymphatic trans-
port function has improved after lymphatic surgery
compared with the preoperative exam. In addition, it
helps to assess the patency of an LVA and to select new
functional lymphatic channels when a secondary LVA
surgery is planned. After ALNT, ICG-lymphography
can sometimes integrate information provided by
postoperative LS, as it can demonstrate the viability
of autotransplanted lymph nodes, when sufficiently
superficial (<2 cm of depth), because of the intrinsic
limit of the exam.
If remnants of hypertrophic fatty tissue, without
liquid, persist in the postoperative assessment, selective
liposuction in the thickened area can be performed.
no consensus has yet been reached regarding the
optimal treatment or benefits. Benefits described to date
include a reduction in a limb volume reduction and/or
improvements in subjective symptoms such as pain or
heaviness.8 Treatment, however, is not generally indi-
vidualized, and most groups have applied the same
technique in all stages of lymphedema.
In the authors’ experience, after more than 10 years
working with lymphedema, it is considered that
with individualized treatment, following the BLAST
algorithm, the degree of lymphedema and the subjec-
tive symptoms can be objectively improved. This
of course depends on the degree of damage to the
lymphatic system.
It has been found that microsurgical results are
best when surgery is performed in the early stages of
lymphedema following a rigorous preoperative study
of the patient, as both LVA and ALNT need to be
minimally functional to be successful. The preop-
erative study identifies patients who may benefit from
a reconstructive microsurgical technique; patients
with no severe fibrosis and a minimal residual func-
tionality of their lymphatic system in the affected
limb. On the contrary, those patients with dominant
fibrosis of the limb and without a minimal residual
functionality of their lymphatic system are more
likely to benefit from reductive procedures such as
liposuction.
Surgical complications
Although LVA is minimally invasive and an almost
complication-free procedure, some side effects or minor
complications have been described, such as the presence
of retrograde blood flow, which reverses spontaneously
within 48 h of surgery.
ALNT can be associated with certain complications
and morbidity of the lymph node donor site.
Complications at the recipient site, such as those
regarding arterial vascularization or venous drainage of
the LN-flap, must be approached as for other microsur-
gical free flaps.
Specific complications of the ALNT procedure mainly
affect the lymph node donor site. Seroma is a frequent
complication at the LN-flap donor site and is mainly
due to the dead space left in this area. Spraying a tissue
sealant can be useful. To avoid dead spaces at the donor
site after raising an LN-flap, a barbed monofilament
strand can be used to perform a continuous spiral suture
and close the donor site.
It is advisable to leave a suction drain in place until
the drainage is less than 15 cc/day and to use compres-
sive cycling garment for the first month. If seroma

500
SECTION II
CHAPTER 26 • Surgical management of breast cancer-related lymphedema
1. DiSipio T, Rye S, Newman B, et al. Incidence of unilateral arm
lymphoedema after breast cancer: a systematic review and
meta-analysis. Lancet Oncol. 2013;14:500–515.
2. Mehrara BJ, Zampell JC, Suami H, et al. Surgical management of
lymphedema: past, present, and future. Lymphat Res Biol.
2011;9:159–167.
3. Brorson H. Liposuction in lymphedema treatment. J Reconstr
Microsurg. 2016;32:56–65.
4. Becker C, Assouad J, Riquet M, et al. Postmastectomy lymphedema:
long-term results following microsurgical lymph node
transplantation. Ann Surg. 2006;243:313–331.
5. Koshima I, Inagawa K, Urushibara K, et al. Supermicrosurgical
lymphaticovenular -anastomosis for the treatment of lymphedema
in the upper extremities. J Reconstr Microsurg. 2000;16:
437–442.
6. Baumeister R, Siuda S. Treatment of lymphedemas by microsurgical
lymphatic grafting: what is proved? Plast Reconstr Surg. 1990;85:
64–74.
7. Suami H, Chang DW. Overview of surgical treatments for breast
cancer-related lymphedema. Plast Reconstr Surg. 2010;126:1853–1863.
14. Mortimer PS. The pathophysiology of lymphedema. Cancer.
1998;83:2798–2802.
15. Koshima I, Kawada S, Moriguchi T, et al. Ultrastructural
observations of lymphatic vessels in lymphedema in human
extremities. Plast Reconstr Surg. 1996;97:397–407.
17. Masia J, Pons G, Nardulli ML. Combined surgical treatment in breast
cancer-related lymphedema. J Reconstr Microsurg. 2016;32:16–27.
18. International Society of Lymphology. The diagnosis and treatment
of peripheral lymphedema: 2013 consensus document of the
International Society of Lymphology. Lymphology. 2013;46:1–11.
Access the complete reference list online at

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References
References
1. DiSipio T, Rye S, Newman B, et al. Incidence of unilateral arm
lymphoedema after breast cancer: a systematic review and
meta-analysis. Lancet Oncol. 2013;14:500–515.
2. Mehrara BJ, Zampell JC, Suami H, et al. Surgical management of
lymphedema: past, present, and future. Lymphat Res Biol.
2011;9:159–167.
3. Brorson H. Liposuction in lymphedema treatment. J Reconstr
Microsurg. 2016;32:56–65.
4. Becker C, Assouad J, Riquet M, et al. Postmastectomy lymphedema:
long-term results following microsurgical lymph node
transplantation. Ann Surg. 2006;243:313–331.
5. Koshima I, Inagawa K, Urushibara K, et al. Supermicrosurgical
lymphaticovenular -anastomosis for the treatment of lymphedema
in the upper extremities. J Reconstr Microsurg. 2000;16:437–442.
6. Baumeister R, Siuda S. Treatment of lymphedemas by microsurgical
lymphatic grafting: what is proved? Plast Reconstr Surg.
1990;85:64–74.
7. Suami H, Chang DW. Overview of surgical treatments for breast
cancer-related lymphedema. Plast Reconstr Surg. 2010;126:1853–1863.
8. Charles RH. Elephantiasis Scroti. London: Churchill; 1912.
9. Gilles H. The lymphatic wick. Proc R Soc Med. 1950;43:1054–1056.
10. Goldsmith HS. Long term evaluation of omental transposition for
chronic lymphedema. Ann Surg. 1974;180:847–849.
11. Campisi C, Boccardo F, Tacchella M. Reconstructive microsurgery of
lymph vessels: the personal method of lymphatic-venous-lymphatic
(LVL) interpositioned grafted shunt. Microsurgery. 1995;16:161–166.
12. Yamada Y. Studies on lymphatic venous anastomosis in
lymphedema. Nagoya J Med Sci. 1969;32:1–21.
13. O’Brien BM, Mellow CG, Khazanchi RK, et al. Long-term results
after microlymphaticovenous anastomoses for the treatment of
obstructive lymphedema. Plast Reconstr Surg. 1990;85:562–572.
14. Mortimer PS. The pathophysiology of lymphedema. Cancer.
1998;83:2798–2802.
15. Koshima I, Kawada S, Moriguchi T, et al. Ultrastructural
observations of lymphatic vessels in lymphedema in human
extremities. Plast Reconstr Surg. 1996;97:397–407.
16. Mortimer PS, Rockson SG. New developments in clinical aspects of
lymphatic disease. J Clin Invest. 2014;124:915–921.
17. Masia J, Pons G, Nardulli ML. Combined surgical treatment in
breast cancer-related lymphedema. J Reconstr Microsurg.
2016;32:16–27.
18. International Society of Lymphology. The diagnosis and treatment
of peripheral lymphedema: 2013 consensus document of the
International Society of Lymphology. Lymphology. 2013;46:1–11.
19. Masia J, Clavero JA, Larrañaga JR, et al. Multidetector-row
computed tomography in the planning of abdominal perforator
flaps. J Plast Reconstr Aesthet Surg. 2006;59:594–599.
20. Viitanen TP, Mäki MT, Seppänen MP, et al. Donor site lymphatic
function after microvascular lymph node transfer. Plast Reconstr
Surg. 2012;130:1246–1253.
21. Stamp GF, Peters AM. Peripheral lymphovenous communication in
lymphoedema. Nucl Med Commun. 2012;33:701–707.

SECTION II • Reconstructive Breast Surgery
27 
Reconstruction of the nipple
areolar complex
Edward H. Davidson, Francesco M. Egro, and Kenneth C. Shestak
SYNOPSIS
■ Nipple areolar complex (NAC) reconstruction completes breast
reconstruction for many patients through a variety of approaches
including, local “pull-out” flaps, skin/tissue composite grafts, tattooing
in combination or isolation.
■ The essential elements in successful post-mastectomy nipple
reconstruction involve appropriate position, projection, and
pigmentation of the reconstructed nipple and areola.
■ Tattooing techniques have evolved to produce a remarkably natural
appearance of the areola with an essentially non-invasive office-based
procedure, albeit with the shortcoming of lack of nipple projection.
With the introduction of “three-dimensional” (3D) tattooing, a
remarkable facsimile of nipple projection can be created.
■ To create a projected nipple, contralateral nipple sharing, tissue grafts
from other sites, and local “pull-out” flaps can all be performed, with
the latter constituting the current “state of the art”.
■ Local flaps usually require initial over-projection due to loss of
projection over time, they do not recreate natural intermittent erectility
and may be less desirable in reconstructed breasts with thin tissue
overlying a prosthetic implant, or following radiation.
■ Currently, no technique exists that restores erogenous or nursing
function of the NAC.
Introduction
Reconstruction of the nipple areolar complex (NAC) for
many women is perceived as the final battle in their war
against breast cancer, completing the restoration of form
that most closely resembles a native breast. Some breast
reconstruction patients are content with a normal
appearance in clothing following breast reconstruction
alone and may choose not to pursue NAC reconstruc-
tion. In those who do pursue NAC reconstruction, there
are the options of local flaps, skin grafts, tattooing, or
some combination of the above. Though immediate
NAC reconstruction, at the same time as the primary
breast reconstruction procedure, has been described, it
is more common to delay NAC reconstruction until the
final shape of the reconstructed breast has stabilized to
ensure correct and symmetrical positioning. Preserva-
tion of the NAC with NAC-sparing mastectomy, if
oncologically sound, usually and more reliably achieves
superior aesthetic results. Patients must be counseled
that all current techniques of NAC preservation and
reconstruction fail to confer native erogenous or nursing
function. This chapter is focused on post-mastectomy
NAC reconstruction in the female, but the consider-
ations and approaches outlined herein may also be
applied and indicated for NAC reconstruction in other
scenarios, including following burns, trauma, congenital
athelia (absence of nipple development), nipple loss
after surgery, and in male as well as female patients.
Diagnosis/patient presentation
General considerations
The anatomy of the female nipple areolar complex can
be as varied in shape, size, orientation, and color as the
breasts on which they sit. Nonetheless, natural anatomic
proportions between the breast and the nipple areolar
complex have been suggested to be areola–breast and
nipple–areola proportions of 1 : 3.4 and 1 : 3, respectively.1
Classically, the aesthetic NAC position is described as
at the point of maximal projection of the breast at some
point above the inframammary fold.2 Anatomic param-
eters for nipple position and areola diameter have also
been described in males: nipple position 20 cm from the

502
SECTION II
CHAPTER 27 • Reconstruction of the nipple areolar complex
At present, the ultimate in nipple “reconstruction” is
preservation of the patient’s native nipple in the form
of a nipple-sparing mastectomy. Since its introduction,
the inherent superior aesthetic result of NAC preserva-
tion compared with reconstructive techniques has con-
tributed to the trend towards the increased popularity
of this approach.
Etiology of athelia, absent nipple, may also influence
the approach to reconstruction. Careful patient and
tissue assessment is critical combined with good surgi-
cal judgment. For example, in reconstruction following
burn injury or after post-mastectomy radiation, results
with tissue grafts may be more predictable than local
flaps.8,9 Similarly, if thin mastectomy skin flaps are
overlying a prosthetic breast implant then, again, surgi-
cal manipulation of compromised tissue to create a bed
for a graft or to raise a flap may prove non-viable or
even violate an implant capsule and result in recon-
structive failure. In all these scenarios, tattooing and
especially 3D tattooing may be a more prudent option.
Treatment/surgical technique
Tattooing
Intradermal tattooing of the nipple and/or areola has
the advantage of being an essentially non-invasive
office-based procedure. Results can vary, but in skilled
hands this can produce a remarkably natural appear-
ance of both nipple and areola.10–12 The procedure has
evolved from using emulsions that produced a painted
experience to pigment gel suspensions which more
closely resemble natural skin pigments and can provide
the optical illusion of texture.6,13 Optimal results are
achieved by not using a darker pigment than the oppo-
site areola where applicable, as well as use of alcohol
skin prep, a short course of per oral antibiotics, and
topical antibiotic ointment. Due to pigment fading, it
has been our experience that tattooing will often need
to be revised at 1 year.
This method of NAC reconstruction is an especially
good idea if there is a paucity of tissue on pinch test and
especially if there is an underlying implant, multiple
scars, or previous radiation. The shortcoming of tattoo-
ing is the lack of nipple projection, and therefore, tradi-
tionally, this has served as a method for recreating the
appearance of the areola and pigmentation of a recon-
structed nipple. Tattooing can be performed before or
after any nipple reconstruction, as determined by prac-
titioner preference.
Newer, 3D tattooing techniques apply the artistic
principles of light and shadow to create depth on a 2D
surface. The 3D technique is essentially the inverse of
sternal notch and 18 cm from the midclavicular line;
nipple to nipple distance of 21 cm and areolar diameter
of 2.8 cm.3 Alternatively, the male NAC position can be
located using a technique based on the height of the
patient and the chest circumference.4 Adjusting the posi-
tion of the NAC is difficult, and so aesthetic NAC
reconstruction must therefore focus on position as well
as projection and pigmentation.
When considering unilateral versus bilateral NAC
reconstruction, the surgeon and patient can study the
contralateral nipple position, base dimension, and pro-
jection to aid planning and selection of reconstructive
technique. In planning for bilateral reconstruction, the
surgeon, with patient assistance, must establish the
position of the NAC without this luxury. Ignoring mas-
tectomy scars and, for example, with the aid of an EKG
lead pad, position is determined by the patient’s “eye”
with surgeon-led adjustment and explanation, as deter-
mined by the parameters outlined above. A unilateral
reconstruction also affords the possibility of contra-
lateral composite nipple–areola grafting, or “nipple
sharing”, especially if the native NAC is relatively
large.5
NAC reconstruction is usually postponed until the
final and stable setting of the reconstructed breast,
optimally 3–4 months following breast reconstruction.6
Some authors have proposed primary NAC reconstruc-
tion at the time of breast reconstruction in a single stage
procedure.7 Notably, this approach is limited to immedi-
ate autologous flap-based reconstruction, as opposed to
implant-based reconstruction when the mastectomy
flap blood supply is less robust. Furthermore, it is
believed that NAC reconstruction at the time of
expander/implant exchange should be avoided, as this
may be associated with a higher rate of implant loss due
to possible wound healing problems with the nipple
reconstruction portion of the procedure, which may
result in implant loss.
A plethora of methods of NAC reconstruction have
been introduced, and while some have been consigned
to history, others continue to evolve into the modern
surgeon’s armamentarium. Currently, reconstruction of
the nipple is most often performed with local “pull-out”
flaps, with tissue grafting (nipple sharing) having a
lesser role. These flaps produce a projecting nipple, but
a greater dimension of realism requires the introduction
of pigment for both the nipple and more importantly
for creation of the surrounding areola. For this reason,
the areola is most commonly reconstructed with the use
of an intradermal tattoo. This is especially true in nipple
reconstruction techniques, where there is direct closure
of the nipple flap donor area. A well-done tattoo pro-
vides shape, size, and color to the reconstructed areola.

503
Planning of the reconstruction
the traditional NAC tattoo. Instead of using a darker
inner circle to create the appearance of a nipple, a lighter
inner circle is created with a dark border. This border is
thickened inferiorly to create a shadow effect. The areola
is created according to the patient’s preferred diameter
and color and is typically performed 3 months after
breast reconstruction and takes approximately 45 min
for a unilateral reconstruction. The introduction of
professional tattoo artists to the reconstructive arena,
adopting professional fundamentals of machine speed,
needle type, and color mixing, as opposed to medical
grade tattooing techniques, has also heralded improved
pigment retention (Fig. 27.1).14
Tissue grafts for nipple and
areola reconstruction
Although currently not the first-line treatment for most
nipple reconstructions, composite grafts still have a role.
The most popular method is that of contralateral nipple
sharing, which affords the possibility of reconstruction
by replacing “like with like”. Many patients and sur-
geons are reluctant to risk the form and function of a
healthy nipple, though others have shown erectile func-
tion and sensation of the donor nipple are unaffected
by nipple sharing procedures.15 However, as recently
described by Zenn and Garofalo, this may be a particu-
larly appealing option in the case of hypertrophied or
large native nipples, and especially in irradiated or thin
tissues in which local flaps may be precluded.5
Numerous donor sites for skin grafting to reconstruct
the areola have been described including contralateral
areola, thigh, groin, labia, eyelid, retroauricular skin,
and oral mucous membrane, as well as testicular skin
Fig. 27.1  3D tattooing can create a remarkably natural appearance of the areola
and illusion of nipple projection.
in male NAC reconstruction. Thigh and groin skin have
been suggested as a good color match for darker areolas
with retroauricular skin, eyelid skin, and oral mucosa
more suited to lighter areolas. Nonetheless, skin graft-
ing from any of these sites may result in hyperpigmen-
tation or more commonly fading of pigment in the skin
that is hypopigmentation over time. Moreover and very
importantly, skin grafting for areola reconstruction is of
limited use at present and has fallen into disfavor by
inherent donor site morbidity.16–23 It has been supplanted
by the use of the intradermal tattoo method of areola
creation.
Local flaps
The concept of creation of a projected nipple through
local dermal-fat flaps was introduced by Hartrampf in
1984, which in a real sense was the original skate flap
(Fig. 27.2).24 There are now many techniques, though
most are variants or derivatives of the skate flap,25
including the star flap (Fig. 27.3),26 fishtail flap (Fig.
27.4),27 H-flap,28 bell flap,29 C-V flap,30 and arrow flap.31
These flaps all obtain their vascular supply through the
subdermal plexus, all share the challenge of maintain-
ing long-term nipple projection and necessitate initial
“over-correction”, and for all donor site primary closure
limits flap size or skin grafting is required.
Planning of the reconstruction
For unilateral nipple reconstruction, planning begins
with a study of the opposite nipple including position
and projection. Projection is best identified with the
patient standing in front of a full-length mirror using an
EKG lead pad with the central metal tab being nipple
position. After placement, measurements from fixed
landmarks such as the sternal midline and the supra-
sternal notch are made. It is important to realize that the
ideal position of the nipple may be slightly different
than it is on the patient’s natural breast, depending on
the size, projection, and position on the chest wall of the
reconstructed breast. If the position selected by the
patient is not aesthetically advisable, this is pointed out
to the patient and corrections are made. The same exer-
cise is undertaken for bilateral reconstruction.
Whenever possible, the design of the flap(s) in a
unilateral nipple reconstruction should include dimen-
sions (the width of the long limbs) which enable a
reconstruction that is 40% larger than the patient’s
natural nipple to allow for loss of projection as a result
of the contraction that occurs with healing. Projection of
the reconstructed nipple is dependent on the design and
the thickness and quality of the dermis of the skin

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SECTION II
CHAPTER 27 • Reconstruction of the nipple areolar complex
B
C
D
E
Fig. 27.2  The skate flap (Hartrampf design). All pull-out flaps (see Figs. 27.3–27.5) are derivative of the design. (A) The
design of the flap. The base is superior to the inscribed circle; the wings are the lateral extensions. (B) The wings are
elevated toward the central core at the deepest part of the dermis leaving a small thickness of the dermis. (C) The central
core is elevated at a depth of 4–7 mm (depending on the size of the nipple required). (D) The lateral wings and central core
resemble the fish called a “skate”. The central fat donor area is closed. (E) The lateral wings are wrapped around the central
core of elevated adipose tissue. (F) Flap closure routinely requires a skin graft over the dermis seen at the inferior aspect of
the drawing.
A
F
utilized. For this reason, the latissimus dorsi flap is most
ideal for creating nipples with excellent projection.
Planning and technique selection are also influenced
by previous incisions on the reconstructed breast and
their possible impact on the blood supply derived from
the subdermal plexus.
Technique selection
Advantages and disadvantages
Selection of technique should consider blood supply
and aesthetics in each clinical scenario. The skate flap
(see Fig. 27.2) can create large nipples but often requires
skin grafting for donor site closure. The star flap (see
Fig. 27.3) more easily allows primary closure of the
donor site. However, the disadvantage of this technique
is that is results in lesser nipple projection and slight
flattening of the breast contour that occurs when the
local donor site is closed. Creation of significant nipple
projection dictates lengthy lateral limbs that may extend
beyond the ideal areola size. Nonetheless, the technique
is extremely versatile for primary nipple reconstruction
and its reliability also renders it the senior author’s
(Ken Shestak) technique of choice for re-do nipple
reconstruction.
The similar fishtail flap (see Fig. 27.4) also allows for
primary closure of the donor site. The angle between the
flaps can be varied, and so this has become the authors’
technique of choice when the ideal nipple position is in
or immediately adjacent to a mastectomy scar, as blood
supply across the scar is not reliable. For this reason, the
fishtail flap does not contain a “cap”, and often the most
anterior aspect of the reconstruct is left open to heal by
secondary intention, since excessive folding of the flaps
can impair their blood supply. Others have described
successful incorporation of a pre-existing scar in the star
flap technique; if the intended site of nipple reconstruc-
tion bisects through a vertical mastectomy scar, the star
flap is designed as medially or laterally based, with its
two lateral limbs lying adjacent to the scar and the
transverse central component incorporating the vertical
scar. On the other hand, if the selected site of nipple
reconstruction passes into a horizontal scar, the flap is
positioned inferiorly- or superiorly-based along the
scar with its two lateral limbs adjacent to the scar

505
Technique selection
Fig. 27.3  Modified star flap or C-V flap. A derivative of the skate flap with primary
closure of the donor area. (A) Flap planning. The base of the lateral flaps and
central flap dimensions are illustrated. (B) The lateral and central flaps are elevated
to a depth of 1 mm into the adipose tissue layer. (C) A central core of fat 5 mm
thick is elevated at the base of the flap. (D) The lateral wings are wrapped around
the central core and inset into the skin. (E) The cap of the flap is brought over the
top of the overlapped lateral wings. The lateral and inferior skin flap donor areas are
directly approximated.
A
Lateral
flap
Central
flap
Base width
of opposite
nipple
a
1.5cm
1.5cm
2cm
B
4mm adipose tissue
C
D
note: Distal tip of flap is
past the midline to permit
‘stacking’ increasing projection
E
Fig. 27.4  Fishtail flap. Also a derivative of the skate flap. There is a central
pedicle, but unlike the modified star flap, there is no “cap” to cover the top of the
nipple column. (A) A circle, which is the base of the flap. The length of the wings
are variable, but most often, they are 2.5 cm in length. (B) The flaps are raised with
1–2 mm of adipose tissue on their deep surface. (C) The flaps are elevated towards
their central core, with 3–5 mm of adipose tissue being raised around the central
core. (D) The flaps are then wrapped around each other by stacking one upon the
other. (E) The donor areas are closed by direct approximation. At times, the flaps
may not allow closure of the most anterior part of the reconstructed nipple, and
topical ointment applied daily will allow healing in 7–10 days. This is preferable to
excessive folding or over-tightening of the delicate flaps.
A
a
b
2.5cm
1 to
1.5cm
B
a
a
b
b
C
a
a
b
b
a
a
D
E
and vertical central limb including the scar.32 When
designing the flap for reconstruction, whenever possible,
superiorly-based flaps are preferred, as these appear
more aesthetically pleasing to the patient; when she
looks down, she does not see incisions on the nipple.
The authors have reported a comparative assessment
of nipple and areola projection after reconstruction
using a Bell flap, a modified star flap, or a skate flap
with a full-thickness skin graft for areola reconstruction.
Patients with ≤5 mm of nipple projection on the opposite
breast were treated with either the Bell flap or the modi-
fied star flap. In patients where the areola complex
exhibited significant projection, a bell flap was chosen

506
SECTION II
CHAPTER 27 • Reconstruction of the nipple areolar complex
colleagues).44 The dissection is straightforward, and
the technique is rapid. After closure of the central flap
donor area, a small de-epithelialization at the site of
planned nipple position provides an excellent platform
to inset the nipple upon, and this likely contributes to
sustained projection. The resulting peripheral donor
area is closed with a purse-string suture (Fig. 27.5G).
Of particular advantage, all of the scars are contained
within the peripheral periareolar incision and thus can
be completely camouflaged by an intradermal tattoo.
Nipple projection has been consistently maintained
and appears similar to that of a skate flap (see Fig.
27.2). This is easiest to perform in the setting of an
autologous tissue reconstruction but may be used fol-
lowing implant reconstruction after careful assessment
of the skin flaps. In the case of a suboptimal mastec-
tomy flap due to thin flap tissue, often encountered
following tissue expansion, a simpler C-V or fishtail
flap may be safer and preferable.
Caution must be adopted when considering the use
of any of these flaps in thin irradiated tissues and/or if
NAC reconstruction is being performed over an implant-
based breast reconstruction rather than autologous
tissue. In these circumstances, there is a risk of jeopar-
dizing the implant, and therefore at the very least, the
surgeon should be prepared to repair the capsule if
violated and have a replacement implant available.
Results of local flaps for nipple reconstruction can also
be disappointing in radiated tissue.
A caveat to projected nipple creation with these
techniques is that patients should be counseled regard-
ing their constant projection rather than intermittent
erectility. Nor can these flaps provide erogenous func-
tion. Indeed, even in more adventurous regional flaps
that have been described such as the neurocutaneous
island flap based on the medial antebrachial cutaneous
nerve, though reportedly restoring sensibility, does not
recapitulate erogenous sensation.45
Nipple areolar complex preservation
As prevention may be preferable to cure, so in the case
of the NAC may preservation be preferable to recon-
struction. Nipple preservation, either through tissue
banking with delayed grafting or nipple–areola sparing
mastectomy, if oncologically sound, may be an option
to confer the best aesthetics to a reconstructed breast.
Millard first described NAC preservation by removal
of the NAC at the time of mastectomy and saving or
banking at the groin, buttocks, or abdomen with subse-
quent transfer to the breast after reconstruction.46
However, even when oncologically safe, this technique
has lost popularity owing to the aesthetic inadequacies
over the modified star flap. In those patients with >5 mm
of nipple projection, reconstruction with a skate flap
and full-thickness skin graft was performed. The best
long-term nipple projection was obtained and main-
tained by the skate and star techniques. The major
decrease in projection of the reconstructed nipple
occurred during the first 3 months. After 6 months, the
projection was stable. The loss of both nipple projections
when using the Bell flap was so remarkable that the
authors discourage the use of this procedure in virtually
all patients.33
Several adjunctive strategies have been touted to
improve maintenance of nipple projection with local
flap reconstruction. Use of auricular cartilage, rib carti-
lage, acellular dermal matrix, calcium hydroxylapatite,
and autologous fat grafting have all been described to
augment flaps with varying success.34–39 In the authors’
opinion, all of these are salvage efforts that suffer from
the effects of scarring of the bed and the inevitable
consequence of attrition over time.
Though the impact on long-term projection, if any, is
difficult to ascertain, most surgeons would advocate the
use of a fenestrated/“donut” or stent-like dressing that
protects/supports the newly reconstructed nipple as
part of the postoperative regimen.40–42
The authors’ preferred technique for local flap-based
NAC reconstruction is the double opposing periareolar
flap (Fig. 27.5).43 A pull-out flap is also a derivative of
the skate design. It is similar to the periareolar skate
flap described by Hammond et al.44 The nipple is
derived as the lead edge of one of two opposing skin
flaps contained in a circular design approximating the
areola complex of the opposite breast. The larger flap
gives rise to the nipple construct. Whenever possible,
it is oriented with the larger flap having a superior
location. The central nipple and the lateral and medial
extensions are elevated just below the dermis with
1–2 mm of subdermal fat included with the skin flap
elevation. As the dissection proceeds to the central area
of the actual nipple, a core of additional subcutaneous
fat (4–5 mm) is preserved deep to the dermis in the
nipple (Fig. 27.5A,B) The central donor area is closed
as the parent flap and opposing flap are incised at their
peripheral margins (which correspond to the outline of
the areola) with the incision made with a scalpel just
through the dermis sparing the underlying subdermal
plexus (Fig. 27.5C,D). The flaps are then easily
advanced towards each other by sliding them on their
subdermal plexus blood supply. The only undermined
area is the nipple flap itself (Fig. 27.5E,F). There is no
undermining of either the parent flap or the opposing
flap or peripheral breast skin (and this is a key differ-
ence from the technique described by Hammond and

507
Nipple areolar complex preservation
A
10-12mm
10-12mm
Nipple base
width
20-22mm
B
54mm
45mm
C
D
E
F
Fig. 27.5  Double-opposing periareolar fishtail flap is the authors’ preferred
technique for local flap-based nipple areolar complex reconstruction. It is designed
to have all of the scars encompassed by the circular closure of the peripheral donor
site when the parent flap and the opposing flap “slide” towards each other on their
subcutaneous flap pedicles. (A,B) Flap design. The planning is similar to the skate
design with lateral wings and a central core. These arise from a parent flap, shown
here superiorly-based. The projection of the nipple is determined by the width of the
transverse flaps adjacent to the center of the nipple construct. (C,D) Nipple flap
elevation on the parent flap (here superiorly-based). The lateral wings are elevated
with 1–2 mm of adipose tissue deep to the dermis from the periphery towards the
central core. A central core of fat is elevated and the lateral wings and the nipple is
reconstructed. (E,F) Closure of the central donor area. The parent flap of the nipple
is advanced to the opposing flap to close the central donor area. The resulting
central flap donor site is closed but releasing the parent flap and the “opposing” flap
by incision through the dermis with preservation of the subdermal plexus. This
allows closure of the central flap donor area.  
Continued

508
SECTION II
CHAPTER 27 • Reconstruction of the nipple areolar complex
of pigment and projection loss associated with repeated
harvest and grafting. More recent attempts at NAC
banking with cryopreservation remain unproven.47
Nipple-sparing mastectomy, as the latest evolution of
mastectomy technique, has proven to result in improved
reconstructive and aesthetic outcomes in breast cancer
care.48 In an analysis of the way women expressed their
reasons for choosing nipple-sparing mastectomy or
NAC reconstruction, a clear psychological advantage of
nipple-sparing mastectomy also emerged.49
The next paradigm shift in this arena is likely tissue
engineering of the NAC, though at present, this approach
is in its infancy.50
Conclusion
Nipple–areolar reconstruction requires an amalgama-
tion of considerations of position, pigmentation, and
projection. Many options for NAC reconstruction are
available to the patient and surgeon, with the choice of
technique dictated by aesthetic and tissue demands.
Tattooing confers a higher dimension of realism to
reconstruction and, with newer 3D techniques, these
may ultimately replace tissue grafts and flaps in many
situations, as well as being advantageous in thin and/
or irradiated tissue as described herein. Tissue grafts
and local flaps, however, in the absence of a clinical
reality for tissue engineered or preserved NACs remain
the mainstay for reconstructing a nipple with actual and
sustained projection. More novel approaches are still
required to restore erogenous and nursing functions of
the reconstructed NAC.
G
(G) The resulting peripheral flap donor area is closed by a
purse-string suture, and the skin is closed with a single layer of monofilament
suture. A subsequent intradermal tattoo will disguise all of the incisions.
Fig. 27.5, cont’d
1. Hauben DJ, Adler N, Silfen R, et al. Breast–areola–nipple
proportion. Ann Plast Surg. 2003;50:510–513. This anatomic study
elegantly describes the anatomic geometry of the nipple areola complex.
This may serve as a guide to reconstruction.
2. Regnault P. Breast ptosis. Definition and treatment. Clin Plast Surg.
1976;3:193–203. This is a landmark article classifying ptosis based on
nipple position. Understanding of this is fundamental to NAC
reconstruction in the context of its relation to the underlying breast.
5. Zenn MR, Garofalo JA. Unilateral nipple reconstruction with nipple
sharing: time for a second look. Plast Reconstr Surg. 2009;123:1648–
1653. This case series of over 50 patients is an excellent guide to the
indications and techniques for nipple sharing.
6. Farhadi J, Maksvytyte GK, Schaefer DJ, et al. Reconstruction of the
nipple-areola complex: an update. J Plast Reconstr Aesthet Surg.
2006;59:40–53. This is a comprehensive review that illustrates well the
commonly adopted local tissue flaps in NAC reconstruction.
14. Halvorson EG, Cormican M, West ME, et al. Three-dimensional
nipple-areola tattooing: a new technique with superior results. Plast
Reconstr Surg. 2014;133:1073–1075. This is the definitive description of
modern 3D tattooing of the NAC.
24. Hartrampf CR Jr, Culbertson JH. A dermal-fat flap for
nipple reconstruction. Plast Reconstr Surg. 1984;73:982–986. A
classic article from which all other pull-out flap designs have  
been derived.
33. Shestak KC, Gabriel A, Landecker A, et al. Assessment of long-term
nipple projection: a comparison of three techniques. Plast Reconstr
Surg. 2002;110:780–786. A retrospective review of 68 patients and their
nipple projection following reconstruction by either skate, modified star, or
Bell flaps.
43. Shestak KC, Nguyen TD. The double opposing periareola flap: a
novel concept for nipple areola reconstruction. Plast Reconstr Surg.
2007;119:473–480. This is the authors’ preferred technique for local
pull-out flap reconstruction of the NAC.
48. Endara M, Chen D, Verma K, et al. Breast reconstruction following
nipple-sparing mastectomy: a systematic review of the literature with
pooled analysis. Plast Reconstr Surg. 2013;132:1043–1054. A systemic
review outlining the success and safety of nipple-sparing mastectomy. This
surgical breakthrough can avoid the need for NSM altogether.
49. Didier F, Arnaboldi P, Gandini S, et al. Why do women accept to
undergo a nipple sparing mastectomy or to reconstruct the nipple
areola complex when nipple sparing mastectomy is not possible?
Breast Cancer Res Treat. 2012;132:1177–1184. This article explores the
relative benefits of NAC reconstruction and nipple-sparing mastectomy
and identifies a clear benefit of the latter.
Access the complete reference list online at

508.e1
References
References
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proportion. Ann Plast Surg. 2003;50:510–513. This anatomic study
elegantly describes the anatomic geometry of the nipple areola complex.
This may serve as a guide to reconstruction.
2. Regnault P. Breast ptosis. Definition and treatment. Clin Plast Surg.
1976;3:193–203. This is a landmark article classifying ptosis based on
nipple position. Understanding of this is fundamental to NAC
reconstruction in the context of its relation to the underlying breast.
3. Beckenstein MS, Windle BH, Stroup RT Jr, et al. Anatomical
parameters for nipple position and areolar diameter in males. Ann
Plast Surg. 1996;36:33–36.
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348–351.
5. Zenn MR, Garofalo JA. Unilateral nipple reconstruction with nipple
sharing: time for a second look. Plast Reconstr Surg. 2009;123:1648–
1653. This case series of over 50 patients is an excellent guide to the
indications and techniques for nipple sharing.
6. Farhadi J, Maksvytyte GK, Schaefer DJ, et al. Reconstruction of the
nipple-areola complex: an update. J Plast Reconstr Aesthet Surg.
2006;59:40–53. This is a comprehensive review that illustrates well the
commonly adopted local tissue flaps in NAC reconstruction.
7. Hudson DA, Skoll PJ. Single-stage, autologous breast restoration.
Plast Reconstr Surg. 2001;108:1163–1171.
8. Neale HW, Smith GL, Gregory RO, et al. Breast reconstruction in
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complex. Burns. 2005;31:1020–1024.
10. Spear SL, Convit R, Little JW 3rd. Intradermal tattoo as an
adjunct to nipple-areola reconstruction. Plast Reconstr Surg.
1989;83:907–911.
11. Spear SL, Arias J. Long-term experience with nipple-areola
tattooing. Ann Plast Surg. 1995;35:232–236.
12. Wong RK, Banducci DR, Feldman S, et al. Pre-reconstruction
tattooing eliminates the need for skin grafting in nipple areolar
reconstruction. Plast Reconstr Surg. 1993;92:547–549.
13. Masser MR, Di Meo L, Hobby JA. Tattooing in reconstruction of the
nipple and areola: a new method. Plast Reconstr Surg. 1989;84:
677–681.
14. Halvorson EG, Cormican M, West ME, et al. Three-dimensional
nipple-areola tattooing: a new technique with superior results.
Plast Reconstr Surg. 2014;133:1073–1075. This is the definitive
description of modern 3D tattooing of the NAC.
15. Edsander-Nord Å, Wickman M, Hansson P. Threshold of tactile
perception after nipple-sharing: a prospective study. Scand J Plast
Reconstr Surg Hand Surg. 2002;36:216–220.
16. Gruber RP. Nipple-areola reconstruction: a review of techniques.
Clin Plast Surg. 1979;6:71–83.
17. Adams WM. Labial transplant for correction of loss of the nipple.
Plast Reconstr Surg. 1949;4:295.
18. Cronin TD, Upton J, McDonough JM. Reconstruction of the breast
after mastectomy. Plast Reconstr Surg. 1977;59:1–14.
19. Schwartz AW. Reconstruction of the nipple and areola. Br J Plast
Surg. 1976;29:230–233.
20. Broadbent TR, Woolf RM, Metz PS. Restoring the mammary areola
by a skin graft from the upper inner thigh. Br J Plast Surg.
1977;30:220–222.
21. Brent B, Bostwick J. Nipple-areola reconstruction with auricular
tissues. Plast Reconstr Surg. 1977;60:353–361.
22. Gruber RP. Method to produce better areolae and nipples on
reconstructed breasts. Plast Reconstr Surg. 1977;60:505–513.
23. Adams WM. Labial transplant for correction of loss of the nipple.
Plast Reconstr Surg. 1949;4:295.
24. Hartrampf CR Jr, Culbertson JH. A dermal-fat flap for nipple
reconstruction. Plast Reconstr Surg. 1984;73:982–986. A classic article
from which all other pull-out flap designs have been derived.
25. Little JW 3rd. Nipple-areola reconstruction. Clin Plast Surg.
1984;11:351–364.
26. Anton MA, Eskenazi LB, Hartrampf CR Jr. Nipple reconstruction
with local flaps: star and wrap flaps. Semin Plast Surg.
1991;5(1):67–78.
27. McCraw JB Fish-tailed flap for nipple reconstruction. Presented at the
Aesthetic and Reconstructive Surgery of the Breast Symposium,
sponsored by Manhattan Eye, Ear and Throat Hospital, New York
City, May 30, 1992.
28. Hallock GG, Altobelli JA. Cylindrical nipple reconstruction using an
H flap. Ann Plast Surg. 1993;30:23–26.
29. Eng JS. Bell flap nipple reconstruction – a new wrinkle. Ann Plast
Surg. 1996;36:485–488.
30. Losken A, Mackay GJ, Bostwick J 3rd. Nipple reconstruction using
the C-V flap technique: a long-term evaluation. Plast Reconstr Surg.
2001;108:361–369.
31. Guerra AB, Khoobehi K, Metzinger SE, et al. New technique for
nipple areola reconstruction: arrow flap and rib cartilage graft for
long-lasting nipple projection. Ann Plast Surg. 2003;50:31–37.
32. Gurunluoglu R, Shafighi M, Williams SA, et al. Incorporation of a
preexisting scar in the star-flap technique for nipple reconstruction.
Ann Plast Surg. 2012;68:17–21.
33. Shestak KC, Gabriel A, Landecker A, et al. Assessment of long-term
nipple projection: a comparison of three techniques. Plast Reconstr
Surg. 2002;110:780–786. A retrospective review of 68 patients and their
nipple projection following reconstruction by either skate, modified star, or
Bell flaps.
34. Tanabe HY, Tai Y, Kiyokawa K, et al. Nipple-areola reconstruction
with a dermal-fat flap and rolled auricular cartilage. Plast Reconstr
Surg. 1997;100:431–438.
35. Heitland A, Markowicz M, Koellensperger E, et al. Long-term
nipple shrinkage following augmentation by an autologous rib
cartilage transplant in free DIEP-flaps. J Plast Reconstr Aesthet Surg.
2006;59:1063–1067.
36. Garramone CE, Lam B. Use of AlloDerm in primary nipple
reconstruction to improve long-term nipple projection. Plast
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37. Nahabedian MY. Secondary nipple reconstruction using local flaps
and AlloDerm. Plast Reconstr Surg. 2005;115:2056–2061.
38. Evans KK, Rasko Y, Lenert J, et al. The use of calcium
hydroxylapatite for nipple projection after failed nipple-areolar
reconstruction: early results. Ann Plast Surg. 2005;55:25–29.
39. Bernard RW, Beran SJ. Autologous fat graft in nipple reconstruction.
Plast Reconstr Surg. 2003;112:964–968.
40. Papay FA, Lucas A, Hutton D. A simple postoperative stent
dressing after nipple-areola reconstruction. Plast Reconstr Surg.
1997;99:1787–1788.
41. Hyman JB, Newman MI, Gayle LB. Composite syringe dressing
after nipple-areola reconstruction. Plast Reconstr Surg.
2005;116:340–341.
42. Liew S, Joseph D, Cordeiro PG. Nipple-areolar reconstruction: a
different approach to skin graft fixation and dressing. Ann Plast
Surg. 2001;47:608–611.
43. Shestak KC, Nguyen TD. The double opposing periareola flap: a
novel concept for nipple areola reconstruction. Plast Reconstr Surg.
2007;119:473–480. This is the authors’ preferred technique for local
pull-out flap reconstruction of the NAC.
44. Hammond DC, Khuthaila D, Kim J. The skate flap purse-string
technique for nipple areola complex reconstruction. Plast Reconstr
Surg. 2007;120:399–406.
45. Bertelli JA, Pereira Filho OJ, Ely JB. Sensitive areolar reconstruction
using a neurocutaneous island flap based on the medial
antebrachial cutaneous nerve. Plast Reconstr Surg. 1992;104:
1748–1750.
46. Millard DR Jr, Devine J Jr, Warren WD. Breast reconstruction: a
plea for saving the uninvolved nipple. Am J Surg. 1971;122:
763–764.
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508.e2
SECTION II
CHAPTER 27 • Reconstruction of the nipple areolar complex
48. Endara M, Chen D, Verma K, et al. Breast reconstruction following
nipple-sparing mastectomy: a systematic review of the literature
with pooled analysis. Plast Reconstr Surg. 2013;132:1043–1054. A
systemic review outlining the success and safety of nipple-sparing
mastectomy. This surgical breakthrough can avoid the need for NSM
altogether.
49. Didier F, Arnaboldi P, Gandini S, et al. Why do women accept to
undergo a nipple sparing mastectomy or to reconstruct the nipple
areola complex when nipple sparing mastectomy is not possible?
Breast Cancer Res Treat. 2012;132:1177–1184. This article explores the
relative benefits of NAC reconstruction and nipple-sparing mastectomy
and identifies a clear benefit of the latter.
50. Cao YL, Lach E, Kim TH, et al. Tissue-engineered nipple
reconstruction. Plast Reconstr Surg. 1998;102:2293–2298.

SECTION II • Reconstructive Breast Surgery
28 
Congenital breast deformities
Francesco M. Egro, Edward H. Davidson, James D. Namnoum, and Kenneth C. Shestak
SYNOPOSIS
■ Congenital breast deformities are a relatively common problem and
place an emotional burden on both the patient and their parents during
puberty and adolescence.
■ Breast deformities present unilaterally or bilaterally, as isolated
anomalies, or more rarely, with other abnormalities affecting other
organs. They can be broadly categorized as “hypoplastic” and
“hyperplastic” disorders.
■ Precise diagnosis and carefully timed surgical management is required
to optimize outcomes and the social and psychosexual impact on
patients and parents.
Introduction
Congenital breast deformities are a relatively common
problem, which place a major emotional burden on both
the patient and the parents, especially during puberty
and adolescence. These deformities have the potential
of causing anxiety, depression, peer rejection, and psy-
chosexual dysfunction. This has a major impact on the
patient’s normal psychosocial development, participa-
tion in school activities, and social life. Breast deformi-
ties present unilaterally or bilaterally, as isolated
anomalies, or more rarely with other abnormalities
affecting other organs. They can be broadly categorized
as “hypoplastic” and “hyperplastic disorders” (Table
28.1).1 Each condition represents a unique challenge,
which warrants consideration on an individual case
basis. Surgical correction is dependent on the type of
anomaly and involves decisions about timing and tech-
nique selection, with the goal to optimize the functional
and aesthetic outcomes and best satisfy the psychologi-
cal needs of the patient. This chapter discusses the most
common congenital breast deformities and their surgi-
cal management.
Embryology
The breast is a modified apocrine sweat gland, which
begins to develop at the 5th week of fetal development.
Linear ectodermal ridges (also called “mammary ridges”
or “milk lines”) develop from 15–20 buds found bilater-
ally on the ventral surface of the embryo, extending
from the axillary to the inguinal regions (Fig. 28.1).
During the 7th week, some of these buds undergo
apoptosis, leaving behind only a single pair of solid
buds (primary mammary buds) at the level of the fourth
or fifth intercostal space. Incomplete regression of the
ectodermal ridge leads to the development of ectopic
supernumerary nipples (polythelia) or breast tissue
(polymastia) along the mammary ridge. The ectodermal
primary mammary buds invade the underlying meso-
derm, and by the 12th week, secondary mammary buds
are formed, which will eventually form the mammary
lobules. By the 5th month, the mammary ridge penetrates
the underlying mesoderm, sending 15–20 epithelial
branches into the developing breast. The areola also
develops around the 5th month. During the 3rd trimes-
ter, these epithelial branches develop into primitive
ducts under the influence of placental sex hormones,
forming the lactiferous ducts and their branches. The
lactiferous ducts converge to open into a mammary pit,
which then becomes the nipple and starts protruding
through the areola during infancy.2 The breast paren-
chyma is contained within the superficial fascia, which
is continuous with the superficial abdominal Camper’s

510
SECTION II
CHAPTER 28 • Congenital breast deformities
ongoing enlargement of the breasts and areolae. Stage
IV describes elevation of the areola and papilla above
the breast mound. Lastly, stage V describes the appear-
ance of a mature breast, with elevation of the papilla
and regression of the areola. Most females achieve stage
V by 16–18 years of age.2
Hypoplastic disorders
Breast hypoplasia and amazia
The mammary gland can either be underdeveloped
(hypoplastic) or absent (amazia), and this may occur
unilaterally or bilaterally, in isolation or in association
with pectoral muscles defects. Breast asymmetry can
be managed with various surgical options, including
augmentation of the affected breast using implants
(most commonly in the submuscular plane5) or myocu-
taneous flaps (e.g., latissimus dorsi myocutaneous flap);
or most recently, by the use of autologous fat transfer
(fat grafting), breast reduction mammaplasty, or masto-
pexy of the larger contralateral breast, or a combination
of the above. Occasionally, the skin envelope needs to
be expanded using a tissue expander prior to the inser-
tion of a permanent implant. Minor corrections can
safely be achieved by means of autologous adipose
tissue transfer. The optimal timing for surgery depends
on the degree of the deformity and its physical and
psychological impact. It is probably optimal to tempo-
rize until the breast is fully developed whenever pos-
sible (usually by 18 years of age).1,5,6 The simplest and
most consistently successful method, when applicable,
is mammaplasty – breast reduction and mastopexy as
illustrated in Figs. 28.2 & 28.3.
Athelia and amastia
Athelia is defined as the absence of the nipple areolar
complex (NAC) and usually occurs with amastia, which
is defined as the absence of the whole breast unit
including the NAC and the mammary gland. Athelia
and amastia can be familial (autosomal dominant) and
may occur unilaterally or bilaterally, in isolation or in
association with amastia and other rare syndromes (e.g.,
scalp-ear-nipple syndrome, Al-Awadi/Raas-Rothschild
syndrome, and Poland syndrome).7 For this reason,
patients need to be investigated to rule out other ecto-
dermal abnormalities. The exact etiology is unknown,
but a deficiency in parathyroid-related hormone might
be the trigger to abnormal involution of the mammary
ridge.8 In 1965, Trier produced a classification of athelia,
based on its association with amastia: Group 1 describes
bilateral amastia with associated ectodermal congenital
defects; Group 2 defines unilateral defects; Group 3
fascia. The superficial fascia consists of a superficial
layer (outer layer covering the breast parenchyma) and
a deep layer (posterior boundary of the breast paren-
chyma) that lies on the deep fascia of the pectoralis
major and serratus anterior muscles. The deep layer is
penetrated by the suspensory ligaments of Cooper,
which extend from the breast dermis to the deep pecto-
ral fascia, joining the two layers of the superficial fascia.3
The formation of sweat glands, sebaceous glands, and
apocrine glands occurs during the 2nd trimester. Breast
tissue grows at the same rate as the rest of the body
during childhood, as long as the estrogen levels are low.
With the increase in estrogen levels during puberty, a
series of morphologic stages occur, which are described
by the Tanner classification.4 Stage I (preadolescent)
describes papilla elevation above the level of the chest
wall. Stage II describes breast and papilla elevation,
along with increased areola diameter. Stage III describes
Table 28.1  Summary of congenital hypoplastic and hyperplastic
breast deformities
Hypoplastic
Hyperplastic
Breast hypoplasia and amazia
Gynecomastia
Athelia and amastia
Virginal mammary hypertrophy
Tuberous breast deformity
Giant fibroadenoma
Poland syndrome
Polythelia
Anterior thoracic hypoplasia
Polymastia
Fig. 28.1  Mammary ridges or milk lines. (Reproduced with permission from
Standring S. (ed.) Gray’s Anatomy. 40th edn. London: Churchill Livingstone,
Elsevier; 2008.)

511
Hypoplastic disorders
characterized by a constricted base with a “high tight
inframammary fold”; contracted skin envelope in both
the vertical and horizontal dimensions; relative breast
hypoplasia and asymmetry; enlarged diameter of the
NAC and herniation of breast parenchyma through the
NAC; and most often breast asymmetry. The exact
incidence among the general population is uncertain,
but most surgeons agree that it is not an uncommon
condition.9 DeLuca-Pytell et al. showed in their study
that tuberous breast deformity was present in 88.8% of
the women demonstrating asymmetry, and only 7% of
those with symmetric breasts.9 The exact etiology is
controversial, but two hypotheses have been brought
forward. In 2003, Mandrekas et al. described the con-
stricted ring theory: a constricting ring is formed as a
defines bilateral amastia, with no other associated
defects.7 The surgical treatment depends on the presence
of athelia, amastia, or both. The breast reconstruction
can be performed using similar techniques to the ones
described for hypoplastic or aplastic breasts. In cases
where both NAC and mammary gland are absent, recon-
struction of the nipple is delayed until breast reconstruc-
tion is completed. Nipple and areola reconstruction can
be carried out using a combination of techniques includ-
ing small local flaps, skin grafts, cartilage grafts, acellular
dermal matrices, and/or NAC tattooing.1,6
Tuberous breast deformity
Tuberous breast deformity is a spectrum of aberrant
breast shape, usually presenting in adolescence, and is
Fig. 28.2  (A,C) Preoperative and (B,D) 5-year
postoperative images of a patient suffering from
breast asymmetry caused by right unilateral breast
hypertrophy who underwent right reduction
mammaplasty.
A
B
C
D

512
SECTION II
CHAPTER 28 • Congenital breast deformities
second hypothesis was brought forward by Costagliola
et al. in 2013, who believe that the deformity is the result
of a combination of factors: first, a congenital structural
weakness and lack of elasticity of the NAC, and second,
a hormonal imbalance between estrogen (horizontal
thrust) and progesterone (vertical thrust) occurring
during the breast growth period.10 Multiple classifica-
tions have been brought forward to describe the extent
of the deformity.10–13 The most commonly used is a
simplified version of the von Heimburg classification,
introduced by Grolleau et al. in 1999 (Fig. 28.4): Type I
describes hypoplasia of the lower medial quadrant;
result of thickening of the superficial fascia at the level
of the periphery of the NAC; this thickening prevents
the expansion of the breast parenchyma inferiorly,
which is instead rerouted towards the NAC, thus
causing herniation through the missing fascia.3 The
A
Fig. 28.3  (A) Preoperative and (B) at 4 months postoperative follow-up patient
suffering from breast asymmetry caused by right breast hypoplasia and left breast
hypertrophy, who underwent right partial subpectoral augmentation with a 360 cc
smooth saline implant and left superior dermoglandular vertical mastopexy with
5 cm nipple transposition.
B
Fig. 28.4  Grolleau classification of tuberous breast deformity. Type I breasts have
hypoplasia of the lower medial quadrant; Type II breasts have hypoplasia of both
lower quadrants; Type III breasts have hypoplasia of all four quadrants. (Redrawn
after Grolleau JL et al. Breast base anomalies: treatment strategy for tuberous
breasts, minor deformities, and asymmetry. Plast Reconstr Surg.
1999;104(7):2040–2048.)
Type III
Type II
Type I

513
Hypoplastic disorders
implant-related complications, with capsular contrac-
ture and implant malposition being the most frequent
of these.22 Mandrekas et al. (n = 11)3 and Pacifico and
Kang (n = 11)19 reported no complications. A more recent
study by Kolker and Collins (n = 26)20 reported compli-
cation rates of 7.8%, with capsular contracture in 3.9%
of the breasts and malposition in 3.9% of the breasts.
Patient satisfaction and aesthetic outcomes were found
to range from “good” to “excellent”. Mandrekas et al.
reported 100% patient satisfaction and 100% surgeon
satisfaction.3 Other studies confirmed patient satisfac-
tion and identified aesthetic outcomes as “excellent” in
75% and “good” in 25% of the cases/patients.19
Poland syndrome
Poland syndrome is a rare congenital malformation
with an incidence of 1 in 7000 to 1 in 100 000.8 It was
named after the anatomist Alfred Poland, a pathologist
from Guy’s Hospital in London, who described it in
1841. However, the condition was originally described
by Lallemand in 1826.
Poland syndrome is characterized by various degrees
of thoracic and ipsilateral upper extremity anomalies
including some or all of the following: absence or
hypoplasia of the breast; absence of the nipple; absence
of the pectoralis major or minor; absence of adjacent
muscles and sometimes costal cartilage; rib abnormali-
ties; and upper extremity deformities (e.g., syndactyly,
brachydactyly, or micromelia). The most obvious defor-
mities are usually limb deformities in both females and
males, and hypomastia in females. Breast deformities
in women are variable, ranging from mild hypoplasia
to aplasia. The typical breast deformity is characterized
by loss of breast parenchyma, high inframammary fold,
and a superiorly malpositioned and underdeveloped
NAC.6,8
The etiology of Poland syndrome is unknown but is
thought to be caused by hypoplasia of the subclavian
artery and its branches, around the 4th and 6th week of
gestation. This leads to vascular compromise of the
internal thoracic artery affecting the development of the
pectoralis major, and/or compromise of the branches of
the brachial artery, potentially causing upper extremity
deformities.8 Poland syndrome has been associated
with various pathologies and syndromes including
breast cancer and other malignancies;23,24 renal anoma-
lies; and Mobius and Klippel–Feil syndromes.25 Of note,
familial cases have also been reported.6
Foucras et al.26 introduced a classification to define
the extent of Poland syndrome:
■ Grade 1: Minor deformity consisting of pectoralis
major hypoplasia. Women present with moderate
Type II describes hypoplasia of both lower quadrants;
Type III describes hypoplasia of all four quadrants.12
One of the first procedures to correct tuberous
breast deformity was described by Rees and Aston in
1976, who advocated widening of the constricted base
by radial scoring.14 Teimourian and Adham in 1983
described a combination of subpectoral augmentation,
followed by donut-shaped periareolar de-epithelializa-
tion, excision of four wedges of breast tissue from below
the new areola, and closure of the gaps to reduce both
the herniated breast tissue and areola size.15 Dinner and
Dowden in 1987 advocated for a full-thickness incision
through the skin with transposition of an inframam-
mary skin flap to reconstruct the central breast, subcu-
taneous tissue, and breast parenchyma to release the
cutaneous band causing the deformity.16 Scheepers and
Quaba in 1992 overinflated tissue expanders before
exchange with a permanent implant.17 Ribeiro et al. in
1998 transected the breast parenchyma horizontally and
rearranged the inferior pole by unfolding the flap
inwards or outwards.18 Mandrekas et al. in 2003
described a similar approach to Ribeiro and colleagues,
except that the constrictive band is divided vertically
and the volume is obtained either by rearranging the
two pillars or by placing an implant in the subglandular
plane.3 Pacifico and Kang in 2007 focused on areola
reduction, subdermal undermining, and implant place-
ment in the subglandular plane, without transecting the
constrictive band.19 Most recently, Kolker and Collins
described their approach involving periareolar access;
dissection to the new inframammary fold; glandular
scoring; subpectoral implant placement; and mastopexy
techniques.20 Lastly, Coleman and Saboeiro reported the
potential of lipoaugmentation by injecting autologous
adipose tissue (370–380 cc per breast) into the subcuta-
neous tissue and pectoral muscle to enhance the contour
aesthetics of the breast.21
Despite the variation in techniques, the general con-
sensus advocates the use of a periareolar donut-type
mastopexy pattern to adjust the size of the areola, fol-
lowed by undermining, and division of the constriction
bands by scoring or transection. The remaining flaps are
rearranged to reconstruct the lower pole, and an implant
can be inserted to add volume to the breast. A two-stage
procedure, which entails initial tissue expander place-
ment followed by implant insertion, is usually reserved
for the most severe cases. The patient illustrated in Fig.
28.5 underwent a type III dual-plane release breast
surgery with placement of synthetic implants beneath
the pectoralis major muscle.
Complications reported in the literature derive mainly
from small case series and include recurrence, asym-
metry, delayed healing, scarring, loss of sensation, and

514
SECTION II
CHAPTER 28 • Congenital breast deformities
A
B
C
Fig. 28.5  (A–C) Preoperative and (D–F) 2-year postoperative follow-up images of an 18-year-old
woman suffering from a bilateral breast tuberosity defect, who underwent type III dual-plane augmentation
mammaplasty with subpectoral implant.
breast hypoplasia and asymmetry. Men present with
minor chest wall deformities. NAC is often small
and elevated. No skeletal abnormalities are found.
■ Grade 2: Moderate deformity consisting of pectoralis
major aplasia. Women present with significant
breast asymmetry, hypoplasia, or aplasia. Patients
present with moderate chest wall deformity. The
NAC is hypoplastic or absent.
■ Grade 3: Severe deformities consisting of complete
muscular and breast aplasia, as well as aplasia of
other chest wall muscles, and major chest wall
deformity. This results in severe chest wall
asymmetry. The NAC is also hypoplastic or absent.
The main goal of surgical intervention is to obtain breast
symmetry, which can be achieved by rectifying the bone
deformity, addressing soft-tissue deficiency, and recon-
structing the breast. Usually reconstruction is considered
a single-stage procedure in adults and a two-stage
procedure in children, especially if chest wall recon-
struction is needed. The best time for surgery is in the
late teens, but operations can be performed from 11–12
years of age and onwards to help with self-esteem and

515
Hypoplastic disorders
gluteal artery perforator.6,8,22 Pedicled latissimus dorsi
myocutaneous flap reconstruction, with or without
implant, has been considered a mainstay of the surgical
management of moderate to severe chest wall deficiency
and breast hypoplasia or aplasia. The benefits include
ease of harvesting the flap, replacement of pectoralis
major with similar tissue, and relative ease compared
with microsurgical procedures. In the past in men, a
customized silicone implant is preferred rather than an
autologous flap. However, this technique has been
associated with a high rate of complications, most
social and psychosexual growth. Reconstructive options
usually include a combination of thoracic cage remodel-
ing (e.g., bone grafts or prosthetic mesh), tissue expan-
sion (subsequently replaced by permanent implants) or
insertion of implant (shaped, round, or customized) in
a dual-plane (if the pectoral muscle is normal), autolo-
gous fat transfer, contralateral mastopexy or reduction
mammaplasty, and autologous free or pedicled muscle
flaps. Commonly used flaps include latissimus dorsi,
transverse rectus abdominis myocutaneous, deep
inferior epigastric perforators, and superior or inferior
D
E
F
Fig. 28.5, cont’d

516
SECTION II
CHAPTER 28 • Congenital breast deformities
In many instances, this is physiologic and commonly
occurs during three periods of normal development:
neonatal, caused by circulating maternal estrogens via
the placenta; pubertal, caused by a relative excess of
plasma estradiol versus testosterone; and elderly, caused
by a decrease in circulating testosterone and peripheral
aromatization of testosterone to estrogen. However,
while gynecomastia is not itself a pathologic process, it
can reflect an hormonal imbalance induced by underly-
ing disease such as tumors (testicular, pituitary, adrenal);
paraneoplastic tumors (colon, lung, and prostate);
hypogonadism; Klinefelter’s syndrome; idiopathic, iat-
rogenic secondary to medications (e.g., spironolactone,
digoxin, ketoconazole); thyroid disease; granulomatous
disease; renal failure; adrenal disease (Cushings, con-
genital adrenal hyperplasia); myotonic dystrophy;
medications; HIV; marijuana use; liver disease; and
alcoholism. The most common etiology in adolescents
is idiopathic, while in patients over 40 years of age, it is
due to medications. Given the varied etiology, surgery
can be considered only if there is certainty that gyneco-
mastia is not the presentation of an underlying pathol-
ogy. For this reason, selected patients may require
endocrinology and radiologic work-up (Fig. 28.6).
The severity of gynecomastia has been described
using various classifications. Webster31 based his clas-
sification on tissue type: type I describes the presence of
glandular tissue; type II describes the presence of fatty
and glandular mix; type III describes the presence of
simple fatty tissue. Simon et al.32 based their classifica-
tion on the degree of tissue and skin excess: type I
describes minor breast enlargement without skin excess;
type II describes moderate breast enlargement without
skin excess (IIa) or with minor skin redundancy (IIb);
type III describes gross breast enlargement with skin
excess creating a pendulous breast. Perhaps the most
useful classification is that proposed by Rohrich et al.30
who based their classification on glandular versus
fibrous hypertrophy and degree of breast ptosis (skin
excess): grade I describes minimal hypertrophy (<250 g)
with no ptosis, primarily glandular (Ia) or fibrous (Ib);
grade II describes moderate hypertrophy (250–500 g)
with no ptosis, primarily glandular (IIa) or fibrous (IIb);
grade III describes severe hypertrophy (>500 g) with
grade I ptosis; grade IV describes severe hypertrophy
(>250 g) with grade II or III ptosis.
Surgical management for gynecomastia is considered
in those patients with absence of an underlying pathol-
ogy, and persistence of breast enlargement for longer
than 1 year (preferably more than 2 years). Surgical
management targets removal of excess breast tissue and
minimization of scars. Various methods have been
used ranging from direct excision to suction-assisted
notably seroma and implant malposition or migration,
and most recently, it has been supplanted by autologous
fat transfer (usually requiring two stages). In women, a
two-stage reconstruction is preferred, starting with a
tissue expander and followed by either an autologous
flap (e.g., latissimus dorsi) with or without an implant,
or a prosthetic device without flap coverage.6,8,22
Complications reported in the literature are primarily
related to the reconstructive procedure itself. Implant
insertion may be compromised because of capsular
contracture, implant distortion, implant rupture,
implant migration, and seroma formation.22 Rocha et al.
described the rare phenomena of costal reabsorption,
parietal pericardium exposure, and mediastinal shift
following prosthetic reconstruction.27 Flap reconstruc-
tion may result in partial or total flap failure. Breast
asymmetry is very common following unilateral recon-
struction, which can be improved at a later stage by a
secondary corrective procedure, either on the ipsilateral
or contralateral side.22
Anterior thoracic hypoplasia
Anterior thoracic hypoplasia is a rare disorder, and
separate from Poland syndrome. It presents with poste-
rior displacement of ribs, anteriorly unilateral sunken
chest wall, hypoplasia of the ipsilateral breast, and a
superiorly displaced NAC. However, the sternum and
pectoralis major are normal. Surgical management
consists of augmentation mammaplasty using anatomic
implants. A latissimus dorsi myocutaneous flap recon-
struction is not usually considered because the pectoralis
major is present, which provides coverage for the
implant.28,29
Hyperplastic disorders
Gynecomastia
Gynecomastia is the most common form of pediatric
hyperplastic disorder affecting up to 65% of pubescent
boys.30 Gynecomastia is defined as a benign prolifera-
tion of glandular tissue in the male breast. It is initially
characterized by increased budding ducts and cellular
stroma and usually resolves spontaneously without the
need for surgery. Gynecomastia present for more than 1
year demonstrates extensive fibrosis and hyalinization
with regression of the epithelial proliferation, thus
increasing the need for surgical intervention. In normal
male development, androgens produced by male
gonadal tissue antagonize the effects of estrogen, which
is responsible for the proliferation of breast tissue.
Gynecomastia is typically a reflection of hormonal
fluctuations with a relative predominance of estrogen.

517
Hyperplastic disorders
pull-through technique.35 Other authors advocate the
use of subcutaneous mastectomy or breast disk excision,
with or without skin reduction, by means of a semicir­
cular periareolar (Simmons grade I), submammary
(Simmons grade I), circumareolar (Simmons grade II), or
inverted T (Simmons grade III) incision pattern.36 Lastly,
severe cases of gynecomastia have been managed with
transposition of the NAC on a dermoglandular pedicle,
or rarely with reposition of the NAC as a full-thickness
skin graft.37 When there is uncertainty about the need to
excise skin (grade III and IV), it is best to perform aggres-
sive liposuction as the primary procedure while employ-
ing envelope modification if needed at a second stage.
Complications commonly reported in the literature
include under-resection, over-resection, complex scars,
and hematoma. Other complications that are not as
frequent include wound dehiscence, excess skin, seroma,
lipectomy, depending on the severity of gynecomastia. A
major breakthrough was introduced by Courtiss, with
the introduction of liposuction as the primary modality
of treatment with surgical excision playing a secondary
role. This dramatically decreased the incidence of
post-surgical contour abnormalities and asymmetries.33
Rohrich et al. advocate the use of ultrasound-assisted
liposuction as a first-line technique in all grades of gyne-
comastia. If excess of skin or breast tissue persists, the
authors advocate for a staged excision after 6–9 months,
particularly in patients with Rohrich grade III and IV
gynecomastia.30 Lista and Ahmad reported the use of
power-assisted liposuction in conjunction with a pull-
through technique to sever the subdermal attachments
of the fibroglandular breast tissue.34 Prado and Castillo
described the use of a power-assisted arthroscopic-
endoscopic cartilage shaver as an alternative to the
Fig. 28.6  A preoperative algorithm for gynecomastia is illustrated based
on the possible etiologies. (Redrawn from Rohrich RJ, Ha RY, Kenkel JM,
Adams W. Classification and management of gynecomastia: defining the
role of ultrasound-assisted liposuction. Plast Reconstr Surg.
2003;111:909–923.)
Negative history and
normal physical exam
Gynecomastia classification and management
Positive history
>12 months
<12 months
Observation
Abnormal physical exam
Normal after work-up
Persistent gynecomastia
Detailed patient history and physical exam
(risk factors and medications)
Drug history: discontinue drug
Systemic disease:
correct underlying disease
Klinefelter syndrome:
Karyotype, bilateral mastectomy
Prepubertal, bilateral disease:
testicular ultrasound
Testicular mass: testicular US; serum
testosterone, LH, estradiol, and
DHEAS; urology and endocrine
consultation
Thyroid mass: thyroid function tests;
endocrine consultation
Breast mass: mammography,
ultrasound, biopsy; surgical oncology
consultation
Hypogonadism: serum LH/FSH,
estradiol, testosterone, and DHEAS;
+ Karyotype; +/- adrenal CT scan;
endocrine consultation
Abdominal masses/Hepatomegaly:
liver function tests; serum LH/FSH,
estradiol, testosterone, and DHEAS;
+/- abdominal CT scan; endocrine
Consultation

518
SECTION II
CHAPTER 28 • Congenital breast deformities
imbalance of endogenous hormone production occur-
ring during puberty; type 2b describes excessive breast
growth related to an imbalance of endogenous hormone
production occurring during pregnancy; and type 3
describes excessive breast growth induced by a pharma-
cologic agent.41
The standard treatment of virginal mammary hyper-
trophy is surgical resection. Early recognition of this
condition is key to minimize the extent of the surgery and
its associated morbidity. Reduction mammaplasty is
most commonly performed either as a pedicle-based
procedure or with a free nipple-graft. This technique is
usually preferred because of the ability to preserve lacta-
tion, and has better aesthetic and psychological outcomes.
Pharmacologic management has also been employed,
but potential side effects have limited their use. The
medications reported in the literature include tamoxifen,
dydrogesterone, medroxyprogesterone acetate, danazol,
and bromocriptine.40
Giant fibroadenomas
Giant fibroadenomas are benign, unilateral, fast growing
breast masses that usually present during puberty. As
with virginal mammary hypertrophy, giant fibroadeno-
mas result from breast tissue hypersensitivity to normal
estrogen levels. The diagnosis is confirmed by breast
tissue biopsy. The standard treatment of giant fibroad-
enomas is reduction mammaplasty, and the timing is
dictated by the onset of the rapid growth phase. The
pattern is designed in such a way as to incorporate the
fibroadenoma in the excision segment, and the pedicle
in the location of the greatest amount of normal breast
tissue to preserve breast fullness. A secondary surgery can
be performed to achieve symmetry including a match-
ing contralateral reduction mammaplasty or a delayed
mastopexy and/or augmentation mammaplasty.
Polythelia
Polythelia (also known as accessory, supernumerary, or
third nipples) is a benign condition that presents with
additional nipples or NACs and is a result of failure of
the mammary ridge to regress in utero. Thus they are
located along the milk line (see Fig. 28.1), extending
from the axilla to the pubic region. Polythelia has an
incidence up to 5.6%, and although normally presents
sporadically, familial cases have also been reported. In
rare cases, polythelia can be associated to renal anoma-
lies, which require further investigation (urinalysis and
ultrasound). Surgical excision is a treatment of choice.
Before puberty, pigmented lesions along the milk lines
should be excised; after puberty, a wider tissue excision
may be required, especially in women, because of the
infection, partial nipple necrosis, pain, and loss of nipple
sensation.36 Rohrich et al. demonstrated that 86.9% of
men that had undergone ultrasound-assisted liposuc-
tion for gynecomastia did not require additional proce-
dures. The remaining 13.1% of patients (all grade III and
IV) underwent a staged excision of skin and breast.30
The main benefit of liposuction is the ability of increased
breast volume reduction without the added scar burden.
Although studies have shown high aesthetic outcomes,
a recent study by Ridha et al. demonstrated that only
62.5% of patients within a cohort of 74 patients were
“satisfied” or “very satisfied” with their result, and the
postoperative satisfaction scores increased only moder-
ately in all groups (liposuction alone, surgical excision
alone, or a combination).38 Surgical management for
gynecomastia is therefore a decision to be taken after
careful patient assessment and discussion of realistic
expectations. Various approaches have been described,
but no technique has yet gained universal acceptance
by the surgical community.
Virginal mammary hypertrophy
Virginal mammary hypertrophy (also known as juvenile
hypertrophy, juvenile macromastia, or gigantomastia) is
a rare condition that results in a rapid, excessive, uni-
lateral, or bilateral enlargement of breasts that usually
presents in adolescents. Patients usually present with
the typical symptoms of macromastia (bra-strap groov-
ing, rashes, shoulder and neck pain) along with breast
tenderness, thinned skin, dilated veins, and striae. Vir-
ginal mammary hypertrophy has been reported in both
sporadic and familial forms; however, the etiology is
unclear. The most popular theory is end-organ hyper-
sensitivity to normal circulating levels of estrogen.
Estrogen levels have been measured in patients with
virginal mammary hypertrophy and have not been
significantly elevated compared with controls.39 Thus,
receptor hypersensitivity might occur due to increased
receptor density, receptor signaling, or another uniden-
tified compound that binds to estrogen receptors.40
Virginal mammary hypertrophy is a diagnosis of exclu-
sion, and other conditions such as phyllodes tumor,
virginal fibroadenoma, fibrocystic disease, and endo-
crine conditions must be ruled out.
Dancey et al. proposed a classification of virginal
mammary hypertrophy based on body mass index
(BMI), pubescent status, and the presence of an inciting
pharmacologic agent: type 1a describes idiopathic,
spontaneous, excessive breast growth in a patient with
a BMI >30; type 1b describes idiopathic, spontaneous,
excessive breast growth in a patient with a BMI <30;
type 2a describes excessive breast growth related to an

519
Conclusion
growth of associated glandular breast tissue. The NAC
can easily be removed using an elliptical excision.8,28
Polymastia
Polymastia (also known as accessory or supernumerary
breasts, multiple breast syndrome, or mammae erraticae)
is a benign condition identified in 1–2% of live births and
presenting with additional breasts and NACs located
along the milk line (most commonly in the axilla). Poly-
mastia is usually sporadic, but familial cases have also
been reported. In rare cases, it can be associated with
renal anomalies, which require further investigation.
Unlike polythelia, which is usually identified at birth,
polymastia is often not noted until puberty or pregnancy,
when hormonal influences enlarge the supernumerary
breast tissue. The treatment of choice is surgical excision
of the accessory gland and primary closure.8,28
Conclusion
This chapter has outlined the most common congenital
breast deformities and their surgical management.
Precise diagnosis and carefully timed surgical manage-
ment is required to optimize outcomes and the social
and psychosexual impact on patients. It is a field where
the plastic surgeon must treat the patient by selecting
both the most appropriate procedure and timing for the
surgical intervention, and manage the family. The
variety and heterogeneity of these conditions tests
the surgeon’s knowledge, experience, and skill in the
arena of breast surgery challenges. The surgery offers
the surgeon the rewarding opportunity to work with a
highly motivated group of young people and follow
them for many years during an important time in their
lives.
1. Sadove AM, van Aalst JA. Congenital and acquired pediatric breast
anomalies: a review of 20 years’ experience. Plast Reconstr Surg.
2005;115:1039–1050.
3. Mandrekas AD, Zambacos GJ, Anastasopoulos A, et al. Aesthetic
reconstruction of the tuberous breast deformity. Plast Reconstr Surg.
2003;112:1099–1109.
8. Latham K, Fernandez S, Iteld L, et al. Pediatric breast deformity.
J Craniofac Surg. 2006;17:454–467. This article provides a solid review of
the most common congenital breast disorders describing the diagnosis,
work-up, and management. It also provides key references related to
congenital breast disorders.
10. Costagliola M, Atiyeh B, Rampillon F. Tuberous breast: revised
classification and a new hypothesis for its development. Aesthetic
Plast Surg. 2013;37:896–903.
12. Grolleau JL, Lanfrey E, Lavigne B, et al. Breast base anomalies:
treatment strategy for tuberous breasts, minor deformities, and
asymmetry. Plast Reconstr Surg. 1999;104:2040–2048. This article
presents the most commonly used classification of tuberous breast deformity.
21. Coleman SR, Saboeiro AP. Fat grafting to the breast revisited: safety
and efficacy. Plast Reconstr Surg. 2007;119:775–787.
22. Nahabedian MY. Breast deformities and mastopexy. Plast Reconstr
Surg. 2011;127:91e–102e.
30. Rohrich RJ, Ha RY, Kenkel JM, et al. Classification and management
of gynecomastia: defining the role of ultrasound-assisted
liposuction. Plast Reconstr Surg. 2003;111:909–925. This article provides
a strong review of the etiology, pathophysiology, diagnosis, and treatment
of gynecomastia. Furthermore, it offers a clear algorithm of the evaluation
and treatment of gynecomastia based on a classification developed by the
authors.
40. Hoppe IC, Patel PP, Singer-Granick CJ, et al. Virginal mammary
hypertrophy: a meta-analysis and treatment algorithm. Plast
Reconstr Surg. 2011;127:2224–2231. This article offers a thorough
meta-analysis examining published case reports and presents a cumulative
algorithm for the diagnosis and treatment of virginal mammary
hypertrophy.
41. Dancey A, Khan M, Dawson J, et al. Gigantomastia – a classification
and review of the literature. J Plast Reconstr Aesthet Surg.
2008;61:493–502. This article presents a classification of virginal
mammary hypertrophy based on the cause, management, and prognosis of
the disease.
Access the complete reference list online at

519.e1
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meta-analysis examining published case reports and presents a cumulative
algorithm for the diagnosis and treatment of virginal mammary
hypertrophy.
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and review of the literature. J Plast Reconstr Aesthet Surg. 2008;61:
493–502. This article presents a classification of virginal mammary
hypertrophy based on the cause, management, and prognosis of the
disease.