Phẫu Thuật Tạo Hình Grabb & Smith’s (Bản Cập Nhật – Kevin C. Chung)

Nipple Projection
One of the greatest challenges in nipple reconstruction is achieving sustained projection. 22 Both graft and
flap techniques retract over time, with long-term loss of nipple projection reported to be between 40%
and 70%. 15 Studies suggest that the reconstructed nipple loses projection in the first 2 years
postoperatively, after which time the height remains relatively stable. A good flap design can help
minimize contracture of the nipple by providing well-vascularized tissue and reducing the number of
incisions and subsequent scars that are subject to contractile forces. Although there have been few studies
that directly compare flap techniques, it is though designs that include double wide-based flaps that
preserve rich subdermal vascularity may limit postoperative contracture.
16 Incorporation of
subcutaneous fat or de-epithelialized dermis into the lateral flaps may increase the bulk of the
reconstructed nipple. 20,23 Centrally based flaps such as the quadrapod flap are subject to the highest
degree of postoperative retraction. 24 Flap design must anticipate long-term loss of projection, and
surgeons should aim to create a nipple that is at least 50% larger than required. Protective dressings or
padding is frequently used to prevent local trauma and compression of the nipple in the early
postoperative period.
Augmentation techniques have been described in an effort to improve nipple projection. The addition of
autologous auricular cartilage was advocated in the 1970s. 25 With the advent of microvascular breast
reconstruction, costal cartilage was identified as a more accessible autologous material because it could be
harvested and banked at the time of the initial breast reconstruction and eliminated the need for
additional donor sites. 26 Although cartilage can help maintain projection, its use is associated with
relatively high complication rates. Pressure necrosis can lead to ischemia of the flaps with exposure and
loss of the cartilage graft. In more recent years, there has been increasing interest in the use of fat as an
alternative autologous material. The soft consistency of fat grafts may reduce the risk of flap necrosis, but
the impact on long-term nipple projection has yet to be established. 27 Acellular dermal matrix and
prefabricated collagen tubes can also be used to support local flap reconstruction, but studies still report
significant loss of projection over time. 28,29 Several alloplastic augmentation techniques have also been
described including calcium hydroxylapatite, silicone, hyaluronic acid, and polytetrafluoroethylene. 30-32
Alloplastic materials carry the risk of infection and extrusion, making them less attractive than
autologous options.
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Areola Reconstruction
Skin-Grafting Techniques
Skin grafts are commonly used for reconstruction of the areola, as they recreate natural variation in
pigmentation and texture that can mimic Montgomery tubercles. The contralateral areola is the ideal
source of donor skin but is rarely used unless the patient is undergoing a concomitant balancing breast
reduction. Skin is more commonly harvested from areas of increased pigmentation such as the inner
thigh, groin, or axilla. Grafting of the areola usually occurs at the same time as nipple reconstruction. An
area of deepithelialization can be incorporated into the nipple flap design to accommodate the skin graft.
Fading of pigmented skin grafts commonly occurs over time.
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Tattooing
Intradermal tattooing has become a popular method of areola reconstruction. 33 It eliminates the need for
additional donor sites and allows customization of areolar pigmentation, which optimizes aesthetic
outcomes. Tattoo pigments, consisting of iron and titanium oxides, are deposited into the dermis using a
dermabrasion technique. One of the commonest problems is fading of the pigment over time and the
need for touch-up procedures. 34 The initial tattoo should be darker than required in anticipation of this.
Deposition of pigment at the correct dermal level is also important because if it is too superficial, it will
slough off in the early postoperative period, but if it is too deep, it will be degraded by macrophage
activity. Tattooing is generally performed 6 to 8 weeks after reconstruction of the nipple. It can be
challenging to achieve homogenous deposition of pigment in scarred tissues. In an effort to avoid this,
some surgeons advocate tattooing of the areola prior to nipple reconstruction. 35
Recent years have seen significant advances in tattoo techniques. Using color shading, a very convincing
NAC can be recreated without the need for flaps or grafts. The tattoo simulates the nipple projection
through color contrasts and also mimics the texture of a natural areola. 36 Three-dimensional tattooing is
generally performed by specialized medical tattoo artists and requires several sessions to achieve the end
result.
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Prosthetics
Nipple prostheses are an attractive option for patients who wish to avoid further surgery on the
reconstructed breast. They may also be recommended in cases where NAC reconstruction might be
considered high risk. The prostheses are made of soft silicone and are attached to the breasts using
adhesives. They can be customized for color, shape, and texture to provide an exact match to the
contralateral NAC.
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Pitfalls and Complications
Although NAC reconstruction is relatively simple, it can be associated with significant complications
including infection and flap or graft failure. Overall complication rates are lower with flap reconstruction
of the nipple and tattooing of the areola, but selection of the optimal reconstruction technique in each
individual patient is critical. 15 Particular caution is necessary in patients who have had alloplastic
reconstruction with thin skin flaps and those who have had prior radiation. Local flap failure may be
more common in these patients, resulting in dehiscence and, in severe cases, exposure of the underlying
implant.
Malposition of the NAC can compromise the overall aesthetic outcome of breast reconstruction and can
be very difficult to correct. Repositioning of the reconstructed NAC usually requires local transposition
flaps, which result in additional unfavorable scarring on the breast. Careful initial planning of NAC
position and appropriate timing of reconstruction is essential.
The commonest complications are loss of projection and fading of pigmentation over time. As these are
almost inevitable with all current techniques, they must be anticipated and adjustments should be made
to the reconstructive design to accommodate them.
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Questions
1. A 59-year-old woman is planning to undergo a delayed right breast reconstruction with a free
deep inferior epigastric perforator (DIEP) flap. She will require a left-sided balancing mastopexy for
symmetry. When is the optimal time to perform NAC reconstruction?
a. At the time of DIEP breast reconstruction
b. 3 months after DIEP breast reconstruction
c. At the time of balancing mastopexy
d. 3 months after balancing mastopexy
2. All the following materials have been used to augment nipple projection in a reconstructed NAC
except:
a. Silicone
b. Calcium hydroxylapatite
c. Hyaluronic acid
d. Polyurethane
e. Acellular dermal matrix
3. Possible complications following nipple-areolar reconstruction include the following except:
a. Nipple malposition
b. Loss of projection
c. Increase in pigmentation
d. Decrease in pigmentation
e. Partial or complete nipple necrosis
4. What is the safest method of NAC reconstruction in a patient with a radiated implant-
reconstructed breast?
a. 3D NAC tattooing
b. Labial graft
c. Nipple share
d. C-V flap
e. Skate flap with skin graft
5. The following landmarks should be taken into consideration for placement of the NAC
reconstruction on the reconstructed breast mound except:
a. Position of the opposite native NAC
b. IMF
c. Midhumeral point
d. Breast meridian
e. Midclavicular point
1. Answer: d. NAC reconstruction should be the final step in the reconstructive process. Although it
is possible to reconstruct the nipple at the time of DIEP, it is difficult to predict the final position
especially in delayed breast reconstruction when the flap would be expected to develop increased
ptosis over time. Correct positioning of the reconstructed NAC is essential, and both the position of
the NAC on the reconstructed breast and its relationship to the contralateral natural NAC must be
considered. It is therefore important to perform the mastopexy prior to NAC reconstruction and to
allow some time to determine the final position of the natural NAC following the balancing
procedure. Nipple malposition can have a significant adverse effect on the final outcome of breast
reconstruction. Correction of nipple malposition is difficult and results in additional scarring on the
reconstructed breast.
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2. Answer: d. Several alloplastic augmentation techniques have also been described including
calcium hydroxylapatite, silicone, hyaluronic acid, and polytetrafluoroethylene. Acellular dermal
matrix has been inserted as well to increase nipple projection. Polyurethanes are in the class of
compounds called reaction polymers, which include epoxies, unsaturated polyesters, and phenolics.
They are not safe for placing into human tissues.
3. Answer: c. A common complication after nipple reconstruction is fading of nipple-areolar
pigmentation over time, rather than increase in pigmentation. The decrease in NAC tattoo pigment
can occur months to years after the procedure, and this is more commonly observed in patients with
fair skin.
4. Answer: a. The safest method of NAC reconstruction in a patient with radiated breast implant
reconstruction is 3D tattooing. In all the other methods of NAC reconstruction, incisions have to be
placed, a flap has to be recreated, or a recipient bed has to be made to accept the flap or skin graft. In
radiated breast tissue, vascularity is compromised, and all the techniques of grafts or flaps that rely
on blood supply may be complicated by wound healing problems. Therefore, noninvasive
procedures such as 3D tattooing may be the safest choice.
5. Answer: e. In a patient undergoing unilateral NAC reconstruction, the opposite or native position
of the NAC must be taken into consideration when designing the placement of the NAC. In a patient
undergoing bilateral NAC reconstruction, the caudal-cephalic position of the NAC needs to take into
account the mid-humeral point, the IMF, and the sternal notch to reconstructed NAC distance. The
medial to lateral position of the NAC needs to be placed along the meridian of the breast mound.
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References
Adams, W. Free transplantation of the nipples and areola. Surgery. 1944;15:186-189.
Farhadi, J, Maksvytyte, GK, Schaefer, DJ, et al. Reconstruction of the nipple-areola complex: an update. J Plast Reconstr Aesthet Surg.
2006;59(1):40-53.
Peled, AW, Wang, F, Foster, RD, et al. Expanding the indications for total skin-sparing mastectomy: is it safe for patients with locally
advanced disease? Ann Surg Oncol. 2016;23(1):87-91.
Momoh, AO, Colakoglu, S, de Blacam, C, et al. The impact of nipple reconstruction on patient satisfaction in breast reconstruction. Ann Plast
Surg. 2012;69(4):389-393.This article demonstrates the effect of nipple-areolar complex reconstruction on overall satisfaction and aesthetic scores in a
large series of breast reconstruction patients.
Wellisch, DK, Schain, WS, Noone, RB, Little, JW III. The psychological contribution of nipple addition in breast reconstruction. Plast Reconstr
Surg. 1987;80(5):699-704.
Delay, E, Mojallal, A, Vasseur, C, Delaporte, T. Immediate nipple reconstruction during immediate autologous latissimus breast
reconstruction. Plast Reconstr Surg. 2006;118(6):1303-1312.
Losken, A, Duggal, CS, Desai, KA, et al. Time to completion of nipple reconstruction: what factors are involved? Ann Plast Surg.
2013;70(5):530-532.
El Amm, CA, Sung, JS, Sawan, KT, et al. Immediate nipple reconstruction using the everted umbilicus. Plast Reconstr Surg. 2011;128(2):91e-
92e.
Millard, DR Jr. Nipple and areola reconstruction by split-skin graft from the normal side. Plast Reconstr Surg. 1972;50(4):350-353.
Zenn, MR, Garofalo, JA. Unilateral nipple reconstruction with nipple sharing: time for a second look. Plast Reconstr Surg. 2009;123(6):1648-
1653.This article discusses the benefits of the nipple share technique in selected patients and demonstrates that this is an excellent option particularly
following alloplastic reconstruction or post radiation where local flaps may have high complication rates.
Nakagawa, T, Yano, K, Hosokawa, K. Cryopreserved autologous nipple-areola complex transfer to the reconstructed breast. Plast Reconstr
Surg. 2003;111(1):141-147; discussion 8-9.
Adams, WM. Labial transplant for correction of loss of the nipple. Plast Reconstr Surg. 1949;4(3):295-298.
Klatsky, SA, Manson, PN. Toe pulp free grafts in nipple reconstruction. Plast Reconstr Surg. 1981;68(2):245-248.
Berson, MI. Construction of pseudoareola. Surgery. 1946;20(6):808.
Sisti, A, Grimaldi, L, Tassinari, J, et al. Nipple-areola complex reconstruction techniques: a literature review. Eur J Surg Oncol. 2016;42(4):441-
465.The authors provide a detailed review of the many nipple-areolar reconstruction techniques that have been described and the associated risks and
benefits. This is the most comprehensive review in the current literature.
Nimboriboonporn, A, Chuthapisith, S. Nipple-areola complex reconstruction. Gland Surg. 2014;3(1):35-42.This article provides an excellent
review of nipple-areolar reconstruction and presents a useful classification system for flap techniques. It also provides important insights into the
mechanism of retraction in the reconstructed nipple.
Little, JW, Spear, SL. The finishing touches in nipple-areolar reconstruction. Perspect Plast Surg. 1988;2:1-22.
Anton, LE, Hartrampf, CR. Nipple reconstruction with local flaps: star and wrap flaps. Perspect Plast Surg. 1991;5(1):67-78.
Losken, A, Mackay, GJ, Bostwick, J III. Nipple reconstruction using the C-V flap technique: a long-term evaluation. Plast Reconstr Surg.
2001;108(2):361-369.
Temiz, G, Yesiloglu, N, Sirinoglu, H, Sarici, M. A new modification of C-V flap technique in nipple reconstruction: rolled triangular dermal-
fat flaps. Aesthet Plast Surg. 2015;39(1):173-175.
Witt, P, Dujon, DG. The V-V flap—a simple modification of the C-V flap for nipple reconstruction. J Plast Reconstr Aesthet Surg.
2013;66(7):1009-1010.
Zhong, T, Antony, A, Cordeiro, P. Surgical outcomes and nipple projection using the modified skate flap for nipple-areolar reconstruction in
a series of 422 implant reconstructions. Ann Plast Surg. 2009;62(5):591-595.This article provides a detailed long-term evaluation of outcomes
following nipple-areolar reconstruction using a single technique in a large series of patients.
Jamnadas-Khoda, B, Thomas, R, Heppell, S. The ‘cigar roll’ flap for nipple areola complex reconstruction: a novel technique. J Plast Reconstr
Aesthet Surg. 2011;64(8):e218-e220.
Little, JW 3rd, Munasifi, T, McCulloch, DT. One-stage reconstruction of a projecting nipple: the quadrapod flap. Plast Reconstr Surg.
1983;71(1):126-133.
Brent, B, Bostwick, J. Nipple-areola reconstruction with auricular tissues. Plast Reconstr Surg. 1977;60(3):353-361.
Mori, H, Uemura, N, Okazaki, M. Nipple reconstruction with banked costal cartilage after vertical-type skin-sparing mastectomy and deep
inferior epigastric artery perforator flap. Breast Cancer. 2015;22(1):95-97.
Bernard, RW, Beran, SJ. Autologous fat graft in nipple reconstruction. Plast Reconstr Surg. 2003;112(4):964-968.
Garramone, CE, Lam, B. Use of AlloDerm in primary nipple reconstruction to improve long-term nipple projection. Plast Reconstr Surg.
2007;119(6):1663-1668.
Tierney, BP, Hodde, JP, Changkuon, DI. Biologic collagen cylinder with skate flap technique for nipple reconstruction. Plast Surg Int.
2014;2014:194087.
Evans, KK, Rasko, Y, Lenert, J, Olding, M. The use of calcium hydroxylapatite for nipple projection after failed nipple-areolar reconstruction:
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early results. Ann Plast Surg. 2005;55(1):25-29.
Lennox, K, Beer, KR. Nipple contouring with hyaluronics postmastectomy. J Drugs Dermatol. 2007;6(10):1030-1033.
Wong, RK, Wichterman, L, Parson, SD. Skin sparing nipple reconstruction with polytetrafluoroethylene implant. Ann Plast Surg.
2008;61(3):256-258.
Spear, SL, Convit, R, Little, JW III. Intradermal tattoo as an adjunct to nipple-areola reconstruction. Plast Reconstr Surg. 1989;83(5):907-911.
Spear, SL, Arias, J. Long-term experience with nipple-areola tattooing. Ann Plast Surg. 1995;35(3):232-236.
White, CP, Gdalevitch, P, Strazar, R, et al. Surgical tips: areolar tattoo prior to nipple reconstruction. J Plast Reconstr Aesthet Surg.
2011;64(12):1724-1726.
Halvorson, EG, Cormican, M, West, ME, Myers, V. Three-dimensional nipple-areola tattooing: a new technique with superior results. Plast
Reconstr Surg. 2014;133(5):1073-1075.
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C H A P T E R 6 2
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Congenital Anomalies of the Breast: Tuberous
Breasts, Poland Syndrome, and Asymmetry
Kenneth L. Fan and Maurice Y. Nahabedian
Key Points
Broadly speaking, pediatric breast disorders may be categorized into hypoplastic, hyperplastic, and
deformational.
Key moves in correction of tuberous breast deformity are periareolar approach to the breast, with
radial scoring of constriction bands to the deep dermal plane as necessary, as well as partial
subpectoral implant placement and circumareolar reduction of areola and herniated breast tissue.
Classically, Poland syndrome has been treated with a pedicled latissimus flap and implants. In such
a scenario, tissue expansion has been undertaken in the first stage to expand deficient breast tissue. A
pedicled latissimus flap with implant placement is performed in a second stage. Increasing implant
volume can provide an adjunct to camouflage chest wall deformities. Some authors have had success
with multiple stages of lipofilling and free flap reconstruction.
Gynecomastia requires complete medical workup for concomitant abnormalities in conjunction with
medical physicians. At onset during adolescence, 1 year of observation is required as spontaneous
regression may occur. Treatment involves liposuction with or without subglandular resection.
Congenital breast deformities represent a source of significant anxiety and distress to the pediatric patient
and their families. Broadly categorized, pediatric breast disorders can be thought of as hypoplastic,
hyperplastic, or deformational as a framework for discussion among plastic surgeons. The first two are
discussed in this chapter. The timing of surgical correction is critical as the operative plans must balance
growth-related factors in light of the psychological trauma that may result from a persistent deformity.
As a result, treatment goals rely on accurate diagnosis, timing, and technique selection in order to
provide the best aesthetic result while addressing psychosocial needs.
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Embryology
The breast is a modified apocrine sweat gland that commences development at around 6 weeks of life.
Paired, proliferating epidermal cells migrate into mesenchymal tissue and form primitive mammary
ridges, or milk line that extends ventrally along the embryo, from the axilla to the groin roughly lateral to
the midclavicular line (Figure 62.1). Failure of development within this time results in complete absence
of the breast. 1 Portions atrophy, except at the fourth intercostal space, which set the foundation for the
primary mammary bud. Incomplete regression gives rise to ectopic breast tissue (polymastia) or
supernumerary nipples (polythelia). Breast growth consists of ectodermal plaques branching into
underlying mesenchyme, which proliferate and canalize to form lactiferous tissue by 8 weeks. 1 The
lactiferous ducts open into the surrounding ectoderm, which develops into the areola at 5 months. By
6 months of gestational age, the basic framework and tubular architecture of the breast can be seen. With
circulating estrogen and progesterone from the placenta, the lactiferous tissue continues branching into
2 years of life. The normal gland remains quiescent from 2 years until puberty.
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FIGURE 62.1 Embryonic mammary ridges.
(Reproduced with permission from Weber, JR, Kelley, JH. Assessing breasts and lymphatic system. In: Weber, JR, Kelley, JH, eds. Health Assessment in
Nursing. 5th ed. Philadelphia, PA: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2014:396-415. Figure 19-3.)
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At birth, the neonatal mammary tissue is functional. Seventy percent may secrete colostrum because of
rise in prolactin. The nipples evert soon after birth because of proliferation of the underlying mesoderm.
However, inverted nipples that remain until puberty is not uncommon. By 2 years of age, breast tissue
grows at the same rate in the body, so long as estrogen levels are low. Sex hormones heavily influence the
development of the breast in morphological stages as described by Tanner. 2 During Tanner stage 1, the
breast is prepubertal, without appreciable breast parenchyma and slight nipple elevation. Tanner stage 2
begins with thelarche, around 9.7 years, as the nipple-areolar complex (NAC) widens and the breast and
nipple become a small mound. Stage 3 is heralded with a further enlargement, as the breast extends
beyond the borders of the areola. The NAC elevates above the breast contour as a secondary mound in
stage 4. In stage 5, the breast achieves the final mature size and form.
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Hypoplastic Disorders
Breast hypoplasia presents in a wide spectrum of phenotypes reflecting their underlying disease (Figure
62.2). Amazia, the absence of the breast without nipple absence, may occur unilaterally or bilaterally, in
isolation or in conjunction with pectoralis hypoplasia. Athelia describes the absence of the NAC. Amastia
is the complete absence of the breast unit, including the gland and nipple. Although these terminologies
suggest otherwise, these abnormalities often occur together: athelia often occurs in conjunction with
amazia.
FIGURE 62.2 Typical appearance of breast hypoplasia.
Accordingly, Trier et al. broadly categorized observed patterns of amastia and its suspected mode of
inheritance into three groups: bilateral absence of the breast associated with congenital ectodermal
defects, unilateral absence of the breast, and bilateral absence of the breast. 3 In congenital ectodermal
defect, a sex-linked recessive disorder, additional abnormalities with the skin and its appendages, the
teeth, and nails are present. Unilateral absence of the breast, when combined with pectoralis aplasia or
hypoplasia, is considered a variant of Poland syndrome. Genetically inherited bilateral absence of the
breast has been described. 1
Management of the hypoplastic/aplastic breasts requires consideration of the patient’s growth and
contralateral breast size. Optimal timing is contingent on the deformity and the psychological and
physical manifestations. Tissue expanders may be placed in the breast should the deformity cause
profound psychosocial effects while the child is growing or if the skin envelope requires expansion.
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Implants, myocutaneous flaps, and autologous fat transfer are viable methods of reconstruction. Careful
consideration of familial breast cancer history is mandatory when employing fat transfer techniques.
Usually, nipple-areolar reconstruction is undertaken after a breast mound is created. Techniques include
local flaps, skin grafts, cartilage grafts, acellular dermal matrices (ADMs), and/or NAC tattooing. 4
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Tuberous Breast
First described by Rees and Aston in 1976, the tuberous breast (e.g., tubular breast, herniated areola
complex, snoopy breast, constricted breast, lower pole hypoplastic breast, narrow-based breast, domed
nipple, and nipple breast) abnormality represents a spectrum of abnormal breast shape (Figure 62.3). 5-7
The term tuberous refers to the similarity in shape to root tubers. 6 Rearing its phenotype only during
puberty, the tuberous breast is characterized by 6 :
Constricted skin envelope in the vertical and horizontal dimensions
Deficiency in the base diameter (breast footprint)
Elevated inframammary fold (IMF)
Short nipple-to-IMF distance (high, tight fold)
Herniation of the breast parenchyma through the areola resulting in enlarged diameter of the NAC.
NAC involvement is present in about 50% of cases 8
Parenchymal hypoplasia
Asymmetry
FIGURE 62.3 Bilateral type III tuberous breast. Both sides suffer from constricted envelope, deficiency of the base diameter,
elevation of the inframammary fold with short nipple to inframammary fold distance, parenchymal hypoplasia, and asymmetry.
The left side has more areolar herniation than the right.
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Grolleau proposed the most widely used classification scheme based on the initial work by Von
Heimburg 7 (Figure 62.4):
Type I Deformity: Only the medial quadrant is absent, the lower medial edge is shaped like an italic
S, and the lateral breast is larger in comparison.
Type II Deformity: Both lower quadrants are deficient, the areola points down, and the lower pole is
constricted.
Type III Deformity: All quadrants are deficient, the breast base is constricted both horizontally and
vertically, and the breast is shaped like a tubercle.
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FIGURE 62.4 Grolleau classification of tuberous breast deformity. Blue on the right represents missing tissue. A. Type I:
Medial quadrant absent. Lateral breast is large in comparison. B. Type II: Both lower quadrants are absent. The areola points
down, and the lower pole is constricted. C. Type III: All quadrants are absent. The breast is constricted both in the horizontal
and vertical dimension.
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Kolker and Collins further described the three tiers with additional pathologic findings (Table 62.1). 9
TABLE 62.1
Kolker and Collins’ Additions to the Modified Grolleau Classification
Type
Base
Inframammary Fold
Skin
Envelope
Breast Volume
Ptosis
Areola
I
Minor
constriction
Normal laterally, minor
elevation medially
Sufficient
Minimal deficiency, no
deficiency, or hypertrophy
Mild,
moderate, or
severe
Enlargement
II
Moderate
constriction
Medial and lateral
elevation
Inferior
insufficiency
Moderate deficiency
None or mild
Normal, mild, or
moderate herniation
III
Severe
constriction
Elevation of the entire
fold, or fold absence
Global
insufficiency
Severe deficiency
Mild
ormoderate
Severe herniation
(From Kolker, AR, Collins, MS. Tuberous breast deformity: classification and treatment strategy for
improving consistency in aesthetic correction. Plast Reconstr Surg. 2015;135(1):73-86.)
Several etiologies have been proposed. Grolleau et al. postulated that anomalies in the superficial fascia in
the lower pole of the breast lead to strong adherence between the dermis and the muscular plane. 7
During breast development, the growth of the breast cannot overcome this adherence. The peripheral
expansion of the breast is restricted, resulting in preferential development forward. When the connective
and muscular structure of the areola is weak, as seen in the severe variants, the gland herniates through
the NAC.
Mandrekas expanded on this with a ring theory by histologically demonstrating a dense constricting ring
spanning from the periphery of the NAC to the lower part of the breast representing a thickening of the
superficial fascia. 10 He postulated that this condensation of fascia may be caused by the joining of the
deep and outer layers of the superficial fascia, which collectively envelope the breast, at a higher level, or
a thickening of penetrating suspensory ligaments in the area. The constricting ring prevents normal
breast expansion and development, and the breast tissue is forced through the outer layer of the
superficial fascia resulting in vertical breast development herniating through the areola.
The true incidence of tuberous breast deformity is variable and somewhat unknown. DeLuca-Pytell et al.
reported 73% of females presenting for breast augmentation were found to have tuberous breast
deformity, with the 60% of breasts having the most severe phenotype (Type III). 8 Ninety-eight percent of
the patients with tuberous breasts were asymmetrical in size as well. However, such high incidence is
debated, particularly by Zambacos and Mandrekas who report a 7% incidence among those who present
for mammoplasty. 11 This discrepancy represents a diagnostic dilemma in tuberous deformity, as milder
forms are commonly underappreciated and underdiagnosed. 9
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Surgical Correction of Tuberous Breast Deformity
Surgical management of tuberous deformity requires close counseling and management of patient
expectations. Although patients have semblance of breast deformity, few fully understand the
anatomic deficits present and the challenge of restoration to a near normal appearance. Without
proper education, many patients expect outcomes seen with primary breast augmentation. These
principles of surgical treatment in tuberous breast deformity are adapted from Brown and Somogyi 12
:
Release of the constricted base in both the vertical and horizontal plane from the tethering
fibrous attachments and/or bands between the breast parenchyma and deep fascia and pectoralis
muscle 6
Restoration of a normal nipple-to-IMF distance through expansion and/or mastopexy and
lowering of the IMF
Obliteration of the old IMF to avoid a double-bubble deformity
Reduction of herniated tissue
Correction of areola hypertrophy
Restoration of breast volume
Correction of breast asymmetry
Critical analysis of deficiencies helps formulate a surgical plan. Simply placing an implant will fall short
in correcting the tuberous breast (Figure 62.5). Kolker and Collins recently published an algorithm that
should be helpful in approaching the tuberous breast deformity (Figure 62.6). 9 In most cases, when the
soft tissues are distensible and the size goals are attainable, correction can be performed in a single stage.
Two-stage procedures with expanders are reserved for the most severe cases when the lower pole skin is
severely deficient, the base/IMF is severely constricted, and/or the patient desires a fuller size. In Kolker
and Collins’ series, 8% of total or 30% of Groleau type III tuberous breast required expansion. 9
FIGURE 62.5 A standard augmentation mammoplasty will fall short in correcting the tuberous breast deformity.
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FIGURE 62.6 Treatment algorithm for tuberous breast deformity.
(From Kolker, AR, Collins, MS. Tuberous breast deformity: classification and treatment strategy for improving consistency in aesthetic correction. Plast
Reconstr Surg. 2015;135(1):73-86.)
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Approach
The periareolar incision has become the most widely used as it provides access for the reduction of the
areola, to the lower pole subglandular plane, and for radial division of the constriction ring. 4,9,13 It is
important to avoid reduction of the areola until the implant is placed or tissue rearrangement is
performed because the skin envelope is deficient: what may seem to be excess initially may ultimately not
be. The alternative approach to the breast tissue is through an inframammary incision.
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Radial Scoring and Internal Tissue Rearrangement
Release of the glandular and breast base constriction with radial scoring is an essential component of
surgical correction. Rees and Aston first described radial scoring on the undersurface of the breast for
expansion. 5 However, their description did not call for scoring to the deep dermis. For adequate release,
bands at the base of the breast parenchyma and along the IMF are radially dissected from deep to the
superficial until the deep dermis to the point visual and palpable release of the lower pole (Figure 62.7). 9
FIGURE 62.7 Radial scoring for release of the glandular and native inframammary fold constriction.
(From Kolker, AR, Collins, MS. Tuberous breast deformity: classification and treatment strategy for improving consistency in aesthetic correction. Plast
Reconstr Surg. 2015;135(1):73-86.)
Several methods of internal tissue rearrangements have been described. Dinner and Dowden advocated
full-thickness skin and glandular incisions with transposition flaps, as they considered the skin itself to be
constricting. 14 The addition of internal gland reshaping allowed for release of the constricting base and
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redistribution of tissue without external scars or contour abnormalities. 12 Puckett and Concannon 15 as
well as Riberio et al. 16 described horizontal transection of the gland with folded-down internal flaps
based on the subareolar tissue or posterior chest wall, respectively, to reconstitute the lower pole.
Mandrekas and Zambacos utilized a periareolar incision to exteriorize the lower half of the breast. 10 A
radial six o’clock incision was made to release the constricting ring, creating pillars to redrape and
reconstitute the lower pole.
Abbate, Fan, and Nahabedian described a new technique for correction of a tuberous breast with
sufficient volume using a central mound mastopexy via an inverted-T incision. 17 The undersurface of the
breast was scored to correct the base constriction without violating the central mound perfusion. The
parenchymal herniation through the NAC was managed by de-epithelizing the central mound followed
by imbrication sutures to create a tight dermal base and minimize the risk for future herniation. The
success of these internal rearrangement techniques is contingent on the presence of enough tissue to
augment the hypoplastic lower pole. In severe cases, these techniques may be less successful because of
the paucity of breast tissue, thus necessitating the use of an implant.
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Implants
Volume correction is often required because of the prevalence of asymmetries. Selection of the
appropriate implant requires biodimensional planning that includes breast height, width, and tissue
compliance. 12 Devices of various projections can be utilized to augment deficiency in the lower pole and
maintain expansion of the released constricted base. The use of anatomical implants for tuberous breast
can be considered to increase the volume in the lower pole and for tailoring of height, width, and
projection. 12
Careful attention needs to be given to the IMF. Extending beyond the intended footprint of the breast
during dissection may be difficult to correct. Some surgeons avoid lowering the IMF whenever possible
owing to the possibility of implant malposition. 12,18 In patients with less severe tuberous deformities, the
IMF is well defined, and further lowering the IMF may be counterproductive. In patients with severe
tuberous breasts, correction is mandated because of a short nipple-to-IMF distance. In such situations,
effacement of the existing IMF is necessary to avoid the double-bubble deformity by lysing fascial
connections between the dermis and superficial fascia. 19 Extending radial scoring to the deep dermis may
assist in this endeavor. Algorithms, such as ones borrowed from the breast augmentation literature by
Tebbett based on implant volume, 20 Mallucci and Brandford ICE principle, 21 and Per Heden’s
Akademikliniken method, 22 can assist in planning the final IMF location. The surgeon may also use cues
from the contralateral IMF or the ridges made by the patient’s ideal bra to roughly determine where the
IMF will sit. Working backwards from the aforementioned IMF algorithms can help in selecting an
implant preoperatively. Correction of asymmetry may be adjusted by varying implant sizes, although
some authors remove excess gland from the deepest aspect of the subareolar gland to keep the implant
size similar and prevent future herniation. 9
The ideal plane for implant placement is somewhat controversial. The initial descriptions for correction of
tuberous breast demonstrated that implant placement should be in the subglandular plane for the
prosthesis to shape and expand the constricted breast without tethering of the pectoralis muscle. 9,12
However, subglandular implants have been associated with increased rates of capsular contracture with
visible implant margins and rippling in patients because of a paucity of parenchyma. The use of anatomic
implants can mitigate these effects, especially when placed in a dual-plane position. In patients with a
tuberous breast deformity, a dual-plane III, which is defined as prepectoral undermining to the upper
edge of the NAC, is usually performed to redrape the gland over the implant, and expand the
parenchymal malposition and the short lower pole skin.
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Mastopexy
Following placement of the permanent implant, final determination on the extent of areolar correction is
judged. The periareolar incision may be converted to circumareolar mastopexy with an interlocking
purse-string suture technique to reduce areolar dimensions. 9,23 When a patient presents with severe
ptosis and a less severe tuberous deformity, a circumvertical mastopexy can be performed as needed
using a tailor-tack approach. Complications seen in tuberous breast deformity are similar to that observed
in mastopexy. Implant insertion with the additional complications from implant malposition and
persistence of the fold produces a double-bubble contour. Management of these complications is further
discussed in the cited articles. 12,19,24
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Poland Syndrome
Poland syndrome, described by Alfred Poland in 1851, is a rare congenital malformation with an
incidence of 1 in 7000 to 100,000. 4 Generally cases are unilateral, but bilateral anomalies have been
described. Sporadic cases are hallmarked by 2 to 3:1 higher incidence in males, with 60% to 75% affecting
the right side (Figure 62.8). 25 Familial cases have equal incidence in sexes and laterality. Classically,
Poland syndrome was defined as the absences of the pectoralis major and ipsilateral hand abnormality
(Figure 62.9). Hypoplasia or absence of the sternocostal head of the pectoralis major results in the absence
of the anterior axillary fold, subclavicular hollowing, and a pathognomonic groove at the junction of the
superior anterior axillary line and chest wall. 13 However, current thought is that Poland syndrome
comprises hypoplasia/absence of the pectoralis major with at least two of these minor criteria 4,25,26 :
Hypoplasia or absence of the breast
Absence of the nipple
Absence of axillary hair
Absence of adjacent muscles (e.g., latissimus dorsi, serratus anterior, external oblique, deltoid,
infraspinatus, supraspinatus, and pectoralis minor)
Absence of costal cartilage and anterior ribs
Absent subcutaneous tissue
Axillary webbing
Ipsilateral brachydactyly, brachysyndactyly, or amelia
FIGURE 62.8 A. Moderate to severe Poland syndrome in a male patient. Note the lack of anterior axillary fold and sunken
chest. B. Moderate Poland deformity in the female patient. Note the lack of anterior axillary fold and subclavicular hollowing.
The breast is characterized by hypoplasia, elevated inframammary fold, and superiorly displaced and underdeveloped nipple-
areolar complex.
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FIGURE 62.9 Anatomic representations of the classic Poland syndrome.
Comprehensive clinical classification is difficult because of the diversity in presentation. The general
classification used was introduced by Foucras et al. and categorized the severity of disease to mild,
moderate, and severe, as summarized in Table 62.2. 4,6,27
TABLE 62.2
Expanded Classification of Poland Syndrome 27,29
Type
Breast
Nipple Areola
Pectoralis Major
Thoracic
Skeleton
Mild
Hypoplasia, asymmetry
Small, elevated
Hypoplasia
None
Moderate
Significant hypoplasia, aplasia,
asymmetry
Hypoplastic,
absent
Absent sternocostal head
Moderate
Severe
Complete aplasia
Hypoplastic,
Complete absence (including surrounding
Severe
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absent
musculature)
Breast anomalies among females are variable and range from mild hypoplasia to amastia. 25 Poland
syndrome is involved in 14% of breast aplasia. 6 In the mild form, structural abnormalities may only be
appreciated radiographically. 28 These patients may be referred to the plastic surgeon for mild
asymmetry, without a formal diagnosis. 29 The mild variant of Poland syndrome is more common than
the classic full presentation with an incidence of 1 in 16,500 live births. 25 The moderate variant of Poland
syndrome represents the classic form and is characterized by hypoplasia of breast parenchyma, high IMF,
and an underdeveloped and superiorly displaced NAC, with the absence of the anterior axillary fold. The
severe variant of Poland syndrome represents the most challenging to reconstruct and is characterized
with a marked deformity of the chest wall with tight chest skin and axillary webbing. 28 Ribiero and
colleagues suggested obtaining CT scans to evaluate the structural abnormalities. 29 Cases have been
reported in which a prominent posterior fold was evident on clinical examination with compensatory
hypertrophy of the teres major that simulated the normal contraction of the latissimus dorsi muscle. 30
The frequency of hand abnormalities with Poland syndrome is 13.5% to 56%; in turn, 10% of syndactyly is
a result of Poland syndrome. 25 The spectrum may range from webbing of the fingers, fusion of the carpal
bones, absence of phalanges, and syndactyly to shortened forearm and/or upper arms. Aplasia of the ribs
and cartilage involving rib segments two to five often leads to severe chest depression in 11% to 25% of
patients. Eight percent of patients may have lung herniation. Dextrocardia is present in 5.6% of patients,
which rises to 9.6% when the deformity was on the left side.
Poland syndrome can be associated with Mobius and Klippel-Feil syndrome. 25 Termed acro-pectoral-
renal defect, an association with aplasia of the pectoralis major exists with renal anomalies (e.g., unilateral
renal agenesis or duplication of the urinary collecting system). 5 Renal ultrasounds are recommended for
all patients with aplasia of the pectoralis. Certain cancers are known to exist with Poland syndrome
owing to suppression of homeobox and tumor suppression genes: leukemia, non-Hodgkin lymphoma,
cervical cancer, leiomyosarcoma, and lung cancer. Despite being hypoplastic, breast cancer has been
reported to occur in patients with Poland syndrome and therefore standard monitoring is required.
The etiology of Poland syndrome remains unclear; however, the leading theory suggests by the sixth
week of gestation, when the limb bud is adjacent to the chest wall, interruption of the embryonic blood
supply results in hypoplasia of the subclavian artery or its branches. 25 The severity and location of the
flow impairment dictates the structures compromised (i.e., internal thoracic artery leading to absence of
the sternocostal head of the pectoralis major; brachial artery leading to hand anomalies; and
suprascapular artery leading to elevation or winging of the scapula, Sprengel deformity).
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Surgical Correction of Poland Syndrome
Skeletal deformities should be addressed before breast reconstruction. 13 Significant chest wall depression,
inadequate protection of the mediastinum, and paradoxical movement of the chest wall are indications
for chest wall reconstruction. 6 When residual chest wall defects persist, custom silicone implants can be
considered. Owing to the lack of a pectoralis major as an interposing muscle, placement of a chest wall
prosthesis will result in implant-to-implant contact that may increase the likelihood of reconstructive
failure. 31 Seyfer et al. reported a 100% complication rate using this technique and eventually abandoned
it because of local complications that included migration, contour irregularities, and discomfort. 32 An
alternative for the simple chest deformity included using the latissimus dorsi muscle to contour the chest
wall followed by placement of a thin mammary prosthesis to correct the subclavicular hollow and to
provide a more tolerable feel and appearance.
The timing of breast reconstruction for patients with Poland syndrome is another important
consideration. It is commonly delayed until late adolescence or adulthood when breast development is
complete, with the pedicle latissimus dorsi flap most commonly used. Patients who have completed
breast reconstruction during their later years have been shown to have body image disorders that are
unalleviated by reconstructive efforts. 33 Therefore, there is a trend toward less invasive modalities such
as fat grafting and tissue expander placement at earlier ages to relieve psychosocial morbidity. The breast
of the Poland syndrome patient presents several challenges: deficient subcutaneous tissue, contracted
skin envelope, deficient subcutaneous tissue, NAC malposition or absence, high IMF, and caliber of
recipient vessels if free flap reconstruction is necessary. 6 Combination of modalities as listed below is
often necessary for optimal reconstruction in severe cases.
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Fat Grafting
The use of lipofilling to correct contour abnormalities has become a cornerstone in treatment of mild
Poland deformity. Lipofilling can be used primarily or secondarily with other procedures. Fat grafting
has made the correction of mild to moderate contour abnormalities, such as improvement of the
subclavicular hollow and absence of the anterior axillary fold, possible in short procedures. 34 Lipofilling
can also be used to camouflage chest abnormalities and may obviate the need for custom silicone devices.
The low morbidity of this procedure has permitted its safe use in adolescent females at the onset of
puberty. Transfers may be staged at 4- to 6-month intervals to simulate natural breast growth. 35 Fat
grafting techniques have also been used in patients with a history of breast cancer with long-term follow-
up demonstrating oncologic safety. 36 Although radiographic distortions are possible with fat grafting,
modern imaging modalities are able to differentiate between microcalcification due to fat grafting and
malignancy.
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Expander and Implant Reconstruction
Patients with less severe phenotypes (grade I or mild II) may be managed with prosthetic reconstruction
alone. A prerequisite to direct to implant reconstruction is adequate soft-tissue coverage. When there is a
soft-tissue deficiency and the IMF is high and tight, placement of tissue expanders can be beneficial. The
expansion of the breast lower pole may bring down a superiorly malpositioned NAC. A contralateral
symmetry procedure, such as a mastopexy or augmentation, may be required.
The primary complication arising from the placement of an implant alone is capsular contracture and is
most likely increased because ofsubcutaneous placement. Seyfer’s series treated 15 patients with implants
alone, and 6 (40%) required open capsulotomy. 32 In the event of prosthetic failure, autologous tissue may
be added to improve outcomes. In a series by Gautam et al., there were 12 cases in which a perforator flap
was used in patients with Poland syndrome who had prior implant failure. 37 Although our
understanding of prepectoral implant placement using ADMs has improved in patients following
mastectomy, there have been no descriptions of use of ADM to secure the implant and to reduce the
incidence of capsular contracture in Poland syndrome.
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Autologous Tissue
The pedicled latissimus dorsi flap in combination with a prosthetic device has been the most common
form of reconstruction in women with Poland syndrome. This can be performed simultaneously or in
stages. The staged approach often begins with tissue expansion followed by a latissimus dorsi flap. 13
Seyfer et al. reported a 0% revision rate of patients treated with this technique. The transfer of the
latissimus dorsi flap facilitated the creation of an anterior axillary fold and also camouflaged the
subclavicular hollow. The latissimus muscle may be reinserted anteriorly on the humerus, simulating the
pectoralis major insertion. Although 66% of patients required a contralateral symmetry procedure, the
latissimus transfers were well tolerated without upper extremity weakness.
The correction of Poland deformity with free tissue transfer is another option when pedicled options are
not available. Longaker et al. described their experience using free flaps (eight TRAMs, two SGAPs, one
IGAP, and one contralateral TRAM) in nine patients with Poland syndrome. 38 Complications included
one flap loss because of venous outflow anomalies in the subscapular system. Gautam et al. described a
series of 12 perforator flaps to reduce donor site morbidity. 37 The recipient vessels in all patients were the
internal mammary vessels. Success was excellent with no take-backs or flap failures.
Dos Santos Costa et al. described using a laparoscopically harvested pedicled omentum flap based
completely on the right gastroepiploic artery and tunneled subcutaneously through the IMF. 39 Four to six
months are required for volume equalization of the omentum. Eighty percent of patients required
additional implants. Huemer utilized myocutaneous gracilis flaps to approach reconstructions. 40 When
correction of the infraclavicular territory was necessary, an ipsilateral free gracilis flap was anastomosed
to the thoracodorsal vessels. When correcting for breast volume, the free gracilis flap was placed in a
retroglandular position and anastomosed to the internal mammary vessels.
Because of the incidence of vascular anomalies in the recipient vessels, preoperative angiography should
be strongly considered when proceeding with free tissue transfer options. 30 A secondary implant or
lipofilling can be considered as needed for additional volume.
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Anterior Thoracic Hypoplasia
Anterior thoracic hypoplasia was described by Spear et al. and represents a distinct entity separate from
Poland syndrome. 31 Although Poland syndrome has a hallmark of hypoplasia or aplasia of the pectoralis
major, anterior thoracic hypoplasia presents with hypoplasia of the ipsilateral breast, superior
displacement of the NAC, and posterior displacement of the ribs with sunken chest wall but a normal
pectoralis major and sternum position. All patients in his series were treated with a partial submuscular
anatomical implant.
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Hyperplastic Disorders
Virginal Mammary Hypertrophy
Virginal hypertrophy (e.g., juvenile hypertrophy, gigantomastia) of the breast is a rare condition
characterized by rapid, excessive, and unyielding proliferation of one or both breasts in the adolescent
years for at least 6 months (Figure 62.10). Reports have demonstrated that breasts may grow as large as 13
to 23 kg. 41 Virginal hypertrophy, which occurs after a few months after puberty, is differentiated from
prepubertal hypertrophy, which is before puberty and usually bilateral. Associated symptoms include
shoulder and neck pain, bra strap grooving, and rashes. The parenchyma is often tender, with thin skin
and dilated veins. Although there is no consensus on exact etiology, the leading theories include excess
local estrogen production and estrogen end-organ sensitivity. 6 The differential diagnosis includes but is
not limited to fibroadenoma, phyllodes tumor, lymphedema, endocrine conditions, rheumatologic
diseases, and lymphoma. 42 Optimal management usually requires a team approach with pediatric and
medical disciplines involved in making the diagnosis.
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FIGURE 62.10 Bilateral virginal hypertrophy in a 16-year-old girl.
There has been a myriad of medical options for management that have included dydrogesterone,
bromocriptine, and medroxyprogesterone; however, tamoxifen administration for 4 months has been
shown to be the most effective in retarding or arresting breast growth. Its use is limited by the side effects
in the pediatric population, which includes hot flashes, venous thrombosis, osteoporosis, and endometrial
hyperplasia. The ultimate treatment, however, remains surgical and most commonly includes reduction
mammaplasty and in some cases, mastectomy. Hoppe et al., in the largest systematic review of virginal
hypertrophy, demonstrated that recurrence of breast growth is sevenfold higher following a reduction
mammaplasty compared with mastectomy. 42 To preserve lactation and aesthetic outcome, most
physicians recommend reduction mammaplasty with preservation of the NAC on a vascularized pedicle
to avoid the psychologic sequela of a mastectomy in the pediatric patient. It is best to defer reduction
mammaplasty until the later years of adolescence to reduce the risk of recurrence and revision surgery.
41,43 In severe cases, early identification is important to minimize the extent of surgery and associated
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morbidity. 4,42
It is important to have in-depth discussions with patients preoperatively to set expectations and review
sequela, especially relating to breast feeding. With careful preservation of the subareolar gland, most
studies have indicated the rates of breast feeding in patients with breast hypertrophy with and without
reduction mammaplasty to be equivalent (∼60%). 44 In both control and operated groups, 34% to 39% of
these patients required supplementation of breast milk. Although this was not studied directly in virginal
hypertrophy, these statistics are helpful in counseling.
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Giant Fibroadenoma
During adolescence, 75% to 95% of breast lesions are fibroadenomas. 41 Generally, they are well-
circumscribed, painless, rubbery, and mobile masses of benign connective tissue and epithelial
proliferation. Giant fibroadenoma refers to when the tumor is larger than 5 cm in diameter and/or weighs
more than 500 g (Figure 62.11). At this point, skin changes, venous dilation, and in severe instances, skin
ulceration of overlying tissue may occur. Ultrasound may reveal a well-circumscribed avascular mass.
Although the diagnosis is clinical, confirmation may be obtained with a fine-needle aspiration. 41 Timing
is dictated by rate of growth. However, earlier intervention may be necessary to prevent distortion of
existing tissue. Treatment involves removing the lesion in the well-demarcated plane. To fill in large
cavities, tissue rearrangement techniques are required with consideration to the pedicle and nipple
perfusion. Pathologically, fibroadenoma is difficult to distinguish from phyllodes tumor, which has an
incidence of less than 1.3%. 6,41 Should a diagnosis of malignant phyllodes tumor be made, consultation
with surgical oncology or pediatric surgery is appropriate.
FIGURE 62.11 Giant fibroadenoma.
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Polythelia and Polymastia
Polythelia is relatively common and occurs in up to 5.6% of male and female patients along the milk lines.
45 These cases are generally sporadic; however, cases of familial inheritance patterns have been reported.
They may be associated with nephrourologic abnormalities and therefore a urinalysis and renal
ultrasound is necessary. Excision is typically performed before puberty begins with an elliptical excision.
Delay of treatment, especially in females, may require a wider incisional pattern as glandular tissue may
become involved.
Polymastia, with or without the NAC, may also occur along the embryonic mammary ridges, most
commonly in the axilla with a prevalence of 1% to 2%. 46 Although sporadic, familial cases and associated
renal abnormalities have been identified. Polymastia differs from ectopic breast tissue, as ectopic breast
tissue is outside the mammary ridges. Unlike polythelia, polymastia is usually not identified until after
puberty, pregnancy or lactation, and when hormonal influences enlarge the breast tissue. These lesions
generally present with discomfort during certain points of the menstrual cycle. This accessory breast
tissue may be excised and closed primarily over a drain as needed. If excision is forgone, then screening
for malignancy is necessary because breast cancer can occur with the accessory tissue at an equal rate to
the natural breast.
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Gynecomastia
Gynecomastia is the most common form of breast hypertrophy in men with an overall incidence of 32%
to 36% and up to 65% in adolescent males (Figure 62.12). 47 Bilateral disease occurs in 25% to 75% of
individuals. Although most cases of gynecomastia during adolescents are idiopathic, the most common
cause in those >40 years old is most often drug induced. Idiopathic or physiologic gynecomastia is
typically a reflection of the hormonal milieu, with a relative predominance of estrogen. Such times occur
with neonates because of circulating maternal estrogen, puberty when there is an excess amount of
estradiol versus testosterone, and in the elderly (>65) when both a decline in androgen and aromatization
peripherally of testosterone to estrogen occur.
FIGURE 62.12 Bilateral gynecomastia.
Workup of gynecomastia begins with a detailed history and physical examination as outlined by Rohrich.
47 It is necessary to identify pathologic causes that include testicular cancer, pituitary tumors, adrenal
tumors, liver disease, paraneoplastic syndrome, Klinefelter syndrome, thyroid disease, renal failure,
myotonic dystrophy, human immunodeficiency virus, marijuana use, alcohol, anabolic steroids, and
medications (Figure 62.13). 47 Physical examination will help distinguish pseudogynecomastia, for which
enlargement is due to fat deposition in light of obesity without glandular hypertrophy.
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FIGURE 62.13 Preoperative algorithm for gynecomastia.
(From Rohrich, RJ, Ha, RY, Kenkel, JM, Adams, WPJr. Classification and management of gynecomastia: defining the role of ultrasound-assisted liposuction.
Plast Reconstr Surg. 2003;111(2):909-923.)
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Various staging classifications have been described. Simon and Kahn described a classification system
based on skin and tissue excess: Grade 1: slight breast enlargement without redundancy, Grade 2A:
moderate breast enlargement without skin redundancy, Grade 2B: moderate breast enlargement with
marked skin redundancy, and Grade 3: marked breast enlargement and skin redundancy. 48 Webster
described a classification based on tissue type 49 :
Type I: Presence of glandular tissue
Type II: Presence of fatty and glandular mix
Type III: Presence of simple fatty tissue
Rohrich described the most utilitarian method for classifying gynecomastia, based on the amount and
character of breast hypertrophy and degree of ptosis (Table 62.3). 47
TABLE 62.3
Classification and Management of Gynecomastia
Classification
Description
Management
Grade I
Minimal hypertrophy (<250 g of breast tissue) without ptosis
 IA
Primarily glandular
Ultrasound-assisted liposuction or suction-assisted
lipectomy
 IB
Primarily fibrous
Ultrasound-assisted liposuction
Grade II
Moderate hypertrophy (250–500 g of breast tissue) without
ptosis
 IIA
Primarily glandular
Ultrasound-assisted liposuction or suction-assisted
lipectomy
 IIB
Primarily fibrous
Ultrasound-assisted liposuction
Grade III
Severe hypertrophy (>500 g of breast tissue with grade I
ptosis
Glandular or fibrous
Ultrasound-assisted liposuction with or without staged
excision
Grade IV
Severe hypertrophy with grade II or III ptosis
Glandular or fibrous
Ultrasound-assisted liposuction with or without staged
excision
(From Rohrich, RJ, Ha, RY, Kenkel, JM, Adams, WPJr. Classification and management of gynecomastia:
defining the role of ultrasound-assisted liposuction. Plast Reconstr Surg. 2003;111(2):909-923.)
The onset of gynecomastia is characterized by fibroblastic stromal and ductal proliferation, which may
resolve spontaneously. However, when persisting beyond a year, these tissues rarely regress, as stromal
fibrosis and hyalinization occur with minimal ductal proliferation. Should a pathologic cause be present,
treatment of such agents or disease is required in conjunction with a medical doctor. The risk of breast
cancer in patients with gynecomastia remains equivalent when compared with the normal male
population. The incidence of breast cancer is 60 times higher in patients with gynecomastia associated
with Klinefelter syndrome when compared with the general population and thus necessitates
mastectomy. 47 Tamoxifen has been successful in the treatment of glandular gynecomastia in which there
is a palpable nodule under the areola. 50 Other medications such as testosterone, danazol, and clomiphene
have had limited success.
Surgical management is considered for persistent breast enlargement after 1 to 2 years. Techniques have
evolved from the classic open approach to the minimally invasive approaches. Liposuction presented a
breakthrough when it was introduced as a primary modality over surgical excision, reducing the
incidence of postsurgical contour abnormalities and asymmetry. Rohrich advocates use of ultrasound-
assisted liposuction as a first-line treatment, arguing that the emulsification of the fat, connective tissue,
and skin retraction is advantageous when compared with traditional liposuction. 47 In addition to
suctioning in the intermediate fat layer, the subdermal and subareolar regions are suctioned for maximal
skin retraction and disruption of the fibroconnective tissues (Figure 62.14). In this study, 87% of men were
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treated with traditional or ultrasound-assisted liposuction alone. In 13%, 6 to 9 months were allowed to
elapse for maximal skin retraction before secondary excision was performed.
FIGURE 62.14 Pattern of ultrasound-assisted liposuction. Liposuction ports are made in the lateral inframammary fold crease.
Adherent zones in the upper outer quadrants (red), in proximity to the axillary tissue, should be suctioned with caution.
(From Stranix, JT, Hazen, A. Gynecomastia. In: Chung, KC , ed. Operative Techniques in Plastic Surgery. Philadelphia, PA: Wolters Kluwer Health; 2019.)
Most practitioners perform a single-stage correction, with liposuction and the excision of the
subglandular tissue through a partial periareolar approach. Overzealous subareolar resection may lead to
a saucer-type deformity under the areola. It is therefore advisable to leave a layer of fibrous tissue under
the areola for a smooth contour to avoid this complication. An alternative to excision is to use an
arthroscopic shaver to treat the excess subareolar tissue, obviating the need for an incision. 51 In patients
with severe gynecomastia, there are several methods by which the excess skin and gland can be managed.
These include circumareolar de-epithelialization and gland resection through an inferior transdermic
approach, circumvertical reduction pattern, or free nipple graft. 52,53 Postoperatively, compression
garments are worn for at least 6 weeks, and heavy exercise should be avoided for 1 month.
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Conclusion
Congenital anomalies of the breast represent a rare and diverse set of disorders requiring application of
various principles of breast reconstruction with consideration of growth and psychosocial impact. Careful
determination of all the deformities present is crucial, and surgeons need to have a frank discussion of
goals and the potential impact correction has to the aesthetic and functional (e.g., breast feeding)
outcomes. It has become commonplace for early identification of hyperplastic disorders and intervention
to limit the extent of surgery and body image disorders, as well as to preserve lactation and optimize
aesthetic outcomes. 42 Although correction of hypoplastic breast deformity has traditionally been
postponed until late adolescence after growth completed, studies now demonstrate improved
psychosocial benefit with the early minimally invasive techniques. 33
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Questions
1. What is the key pathological feature of Poland syndrome?
a. Hand abnormalities
b. Chest depression
c. Hypoplasia or absence of the pectoralis major
d. Hypoplastic or absent breast
e. Absence of the latissimus dorsi
2. Where is the most common site for polymastia to occur?
a. Axillary region
b. Underneath the breast
c. Near the groin
d. On the abdomen
3. An 18-year-old female with a history of tuberous breast deformity comes into your clinic
requesting evaluation for unsatisfactory aesthetic appearance. She has had a previous partial
submuscular breast augmentation performed by another surgeon. On examination, she appears to
have a double-bubble deformity. What key maneuver was missed in the initial operation?
a. Complete release of the pectoralis major
b. Ablation of the old IMF
c. Placement of the implant’s partial submuscular plane
d. Radial scoring of the undersurface of the breast
4. A 13-year-old boy with a BMI of 22 presents with bilateral fibrofatty tissue underneath his nipples
recalcitrant to exercise regimens for the past 3 months. The lesions have caused him significant
distress at the gym and when swimming. A full workup has elicited no pathologic cause. What is the
most appropriate next step in management?
a. Surgical excision
b. Selective estrogen receptor modulators
c. Continued weight loss
d. Observation and follow-up for 6 to 8 months
5. What tumor is most clinically difficult to distinguish from fibroadenoma?
a. Phyllodes tumor
b. Intraductal carcinoma
c. Fibrocystic lesion
d. Intraductal papilloma
1. Answer: c. Poland syndrome represents a highly variable constellation of syndromes revolving
around structures supplied by the subclavian vessels. Hypoplasia or absence of the pectoralis major
represents the central pathologic finding of Poland syndrome. Additional abnormalities that may be
seen include hypoplasia or absence of the breast, absence of the nipple, absence of the axillary hair,
absence of adjacent muscles (e.g., latissimus dorsi, serratus anterior, external oblique, deltoid,
infraspinatus, supraspinatus, and pectoralis minor), absence of costal cartilage and anterior ribs
leading to chest wall abnormality, and upper limb malformations.
2. Answer: a. The most common location of the polymastia is the axillary region. 46 These lesions
typically present with pain that may coincide with menstrual cycles or lactation separate from the
breast tissue itself. Excision requires preservation of the intercostobrachial and associated nerves. The
rate of malignancy is equal, and pathologic examination of the sampling is necessary.
3. Answer: b. Ablation of the old IMF is the key maneuver to eliminate double-bubble deformity. In
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an excellent review of the topic by Handel, he demonstrates the double-bubble deformity is not
caused by disruption of an IMF ligament but by failure to fully detach the superficial fascial system
from the dermis. 19 The IMF is created by attachment from the superficial fascial system to the
dermis. In routine breast augmentations without manipulation of the fold, the implant lies beneath
the pectoralis fascia, and the superficial fascia relationships are undisturbed. In the more severe cases
of tuberous deformity, owing to the short nipple-to-IMF distance, inferior dissection and displacing
the fold to a caudal location is typically necessary. Dissection is continued in a plane underneath the
superficial fascia. The superficial fascia and dermis attachments from the old IMF persist with
inadequate ablation, resulting in double-bubble deformity.
4. Answer: d. The patient likely suffers from benign pubertal gynecomastia, which may respond
spontaneously. Therefore, observation is prudent. Beyond 1 year, the tissue rarely regresses as
stromal fibrosis and hyalinization set in. At this point, surgical therapy is indicated. While weight
loss is encouraged, suggesting continued weight loss would resolve gynecomastia is inappropriate.
5. Answer: a. Fibroadenoma can be clinically difficult to distinguish from phyllodes tumor. Making
such diagnosis may require pathological examination. 41 Histologically, phyllodes tumor shows more
stromal proliferation. However, malignant phyllodes tumor has the ability to metastasize and recur
locally. These lesions are hallmarked by obvious sarcomatous elements. In adults, surgical treatment
requires a 1 cm surgical margin to reduce recurrence rates. There are some who believe the
adolescent phyllodes tumor is less aggressive and therefore can be excised with a small rim for a
surgical margin.
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