Operative Technique in Plastic Surgery – Kevin C.Chung

■ Late
■ Adverse scarring
■ Shape change
■ Loss of nipple-areolar complex function (sensation or lactation)
■ Fat necrosis
REFERENCES
1. Wolfswinkel EM, Lemaine V, Weathers WM, et al. Hyperplastic breast
anomalies in the female adolescent breast. Semin Plast Surg. 2013;27(1):49-55.
2. Hoppe IC, Patel PP, Singer-Granick CJ, Granick MS. Virginal mammary
hypertrophy: a meta-analysis and treatment algorithm. Plast Reconstr Surg.
2011;127(6):2224-2231.
3. Würinger E, Mader N, Posch E, Holle J. Nerve and vessel supplying
ligamentous suspension of the mammary gland. Plast Reconstr Surg.
1998;101(6):1486-1493.
4. Muntan CD, Sundine MJ, Rink RD, Acland RD. Inframammary fold: a
histologic reappraisal. Plast Reconstr Surg. 2000;105(2):549-556.
5. Chang DS, McGrath MH. Management of benign tumors of the adolescent
breast. Plast Reconstr Surg. 2007;120(1):13e-19e.
6. Pruthi S, Jones KN. Nonsurgical management of fibroadenoma and virginal
breast hypertrophy. Semin Plast Surg. 2013;27(1):62-66.
7. Xue AS, Wolfswinkel EM, Weathers WM, et al. Breast reduction in
adolescents: indication, timing, and a review of the literature. J Pediatr
Adolesc Gynecol. 2013;26(4):228-233.
8. Hammond DC. The short scar periareolar inferior pedicle reduction (SPAIR)
mammaplasty. Semin Plast Surg. 2004;18(3):231-243.
9. Hammond DC. Short scar periareolar inferior pedicle reduction (SPAIR)
mammaplasty. Plast Reconstr Surg. 1999;103(3):890-901.
10. Hammond DC, Khuthaila DK, Kim J. The interlocking Gore-Tex suture for
control of areolar diameter and shape. Plast Reconstr Surg. 2007;119(3):804-
809.
11. Cruz, NI, Korchin L. Lactational performance after breast reduction with
different pedicles. Plast Reconstr Surg. 2007;120(1):35-40.

60
CHAPTER
Pectus Excavatum and
Pectus Carinatum
Jamie C. Harris and Fizan Abdullah
DEFINITION
■ Pectus excavatum is the most common chest wall deformity that involves a
concave deflection of the sternum posteriorly, with a wide range of severity
from slight depression to almost reaching the level of the vertebrae. This
abnormality can be present at birth but more frequently becomes apparent
during puberty when rapid growth occurs.
■ It occurs more frequently in males than females (3:1 ratio).
■ Indications for repair include symptomatic relief, improved appearance, or to
alleviate psychosocial anxiety due to the defect.
■ Pectus carinatum is a congenital chest wall anomaly sometimes referred to as
“pigeon chest,” less common than excavatum, which is associated with a
convex deformity of the sternum, leading to protrusion of the chest wall.
■ Two general types are described: chondrogladiolar, which is symmetric
protrusion of the body of the sternum and cartilage, and chondromanubrial,
which is protrusion of the manubrium sternum with inward depression of the
inferior portion. This generally presents during adolescence and has a male
predominance.1
DIFFERENTIAL DIAGNOSIS
■ Pectus carinatum vs excavatum
■ Poland syndrome: absence of pectoris major and minor muscles, missing ribs,
and chest wall depression
■ Scoliosis: abnormal curvature of the spine that can make the chest wall appear
deformed

PATIENT HISTORY AND PHYSICAL FINDINGS
■ As with any patient, the first approach is a thorough history. The time course
of presentation is important to identify, as it can vary from infancy to
adolescence.
■ The progression of the deformity is important to note. For example, during
puberty the deformity can rapidly become much more prominent.
■ Symptoms associated with pectus excavatum (PE) are rare but can include
dyspnea or exercise intolerance. However, the majority of patients will not
report symptoms.
■ Often, the presenting complaint is poor appearance due to the enlarging
deformity at puberty.
■ Clear documentation of dyspnea, exercise intolerance, or worsening dyspnea
is important to facilitate insurance coverage.
■ Family history regarding chest wall deformities should be obtained; there is a
25% rate of positive family history.
■ Cardiopulmonary sequela has long been investigated; however, definitive
results are still lacking. It has been shown that patients have normal
spirometry preoperatively but can report subjective improvement in breathing
postoperatively.
■ On physical exam, symmetry of the chest should be examined. Often,
asymmetry can occur, most frequently favoring the right side. Calipers can be
used to determine the depth and width of the deformity; however, computed
tomography (CT) scan has become a more standardized way to categorize
defects.
■ Chest wall inspection includes observing chest wall symmetry or asymmetry,
as well as the type of deformity present.
■ Auscultation can reveal displaced cardiac sounds. Additionally, a systolic
ejection murmur can sometime be appreciated due to the proximity of the chest
wall to the pulmonary artery. Mitral regurgitation can also be present.
■ Examination of the rest of the body, looking for stigmata of Marfan syndrome
including long digits or history of bruising. If present, a full evaluation for
Marfan syndrome should be done.
IMAGING AND OTHER DIAGNOSIS STUDIES
■ Computed tomography of the chest has now become important for diagnosing
severity of the PE. The Haller index is calculated from the axial images using
the ratio of the transverse diameter from the inner ribs to the length of the

greatest point on defect between the posterior sternum and anterior vertebrae. A
ratio greater than 3.2 is considered a severe defect.2
■ Plain chest radiograph, posteroanterior (PA) and lateral views, has been
described to calculate the Haller index as well and in some studies has been
found to be equivalent for predicting severity.3 However, most patients will
generally still have CT scan for evaluation.
■ Pulmonary function tests in patients with pectus excavatum have been shown
to have 10% to 20% less of the expected lung volume on spirometry.
Additionally, the forced expiratory volume has been shown to be less than 80%
of the predicted value for patients of similar age without pectus abnormalities.4
■ Echocardiogram for cardiac evaluation is very important in this group of
patients. There is a higher prevalence of cardiac abnormalities including right
ventricular compression, mitral valve prolapse and arrhythmias. An
echocardiogram is important to evaluate if cardiac structure is abnormal to
allow for proper operative planning if identified.4
SURGICAL MANAGEMENT
Preoperative Planning
Pectus Excavatum
■ In general, it is preferred to time repair during the pubertal growth phase,
usually from ages 10 to 15.5 This is because the rapid growth phase can worsen
pectus and therefore cause a previously repaired defect to recur. However, for
children with severe exercise intolerance, earlier repair can be considered.
■ Indications for operative correction include Haller index greater than 3.2,
progression of severity of disease, pulmonary function tests (PFTs)
demonstrating obstructive or restrictive lung patterns, or cardiac abnormalities
such as displacement, arrhythmias, or murmurs.6
■ There are two different types of repair: open (or Ravitch procedure) and the
minimally invasive procedure (Nuss). There are a few important considerations
to make when deciding which approach is appropriate. In patients with severe
asymmetry, the open approach is often favored. Additionally, in patients
presenting with a failed Nuss procedure, the open procedure should be
considered for the second repair. However, overall, the Nuss procedure tends to
be the procedure of choice for consideration in lower postoperative pain as
well as improved appearance from its minimally invasive nature.

■ If pursuing noninvasive repair with a Nuss bar, it is crucial to determine if the
patient has allergies to metal, or a family history of metal allergy. If a family
history is present, subsequent skin testing for metal allergy is necessary before
proceeding. If an allergy is present, a titanium bar should be used instead of the
standard stainless steel.
■ The bar length for the Nuss procedure should be 2.5 cm shorter than the
distance from the right to left midaxillary line.7
■ General anesthesia should be achieved prior to beginning the operation, and a
secure airway must be obtained at the beginning of the case.
Pectus Carinatum
■ In general, most children with pectus carinatum will not require surgery. For
children that are younger and have not yet completed puberty, orthotic bracing
regimens are attempted to mold the chest into the correct position. Up to 75%
of patients will achieve significant or complete correction of the pectus
carinatum deformity using bracing alone.8
■ The most common reason for incomplete or failed correction is patient
noncompliance with bracing regimen. Noncompliance often results from
failure to wear the brace often enough or in time periods long enough to correct
the deformity.
■ If the defect fails to improve, surgical correction can then be pursued.
T E C H N I Q U E S
■ Nuss Procedure: Minimally Invasive Approach for
Pectus Excavatum
Positioning and Incision
■ Patient is supine with arms extended.
■ The operating surgeon is on the right and assistant surgeon on the left.
■ The initial incisions should be at the transverse midaxillary line at the level
of the deepest depression of the sternum, 4 cm in length on each side.
Endoscopic Bar Placement
■ Once the incisions are made, carry the right-sided incision down to the

pleura, entering under direct vision.
■ A 5-mm thoracoscope is inserted in two rib spaces caudal to the right-sided
incision to allow for direct vision of passage of the introducer.
■ Deep subcutaneous tunnels are made on both the left and right sides, staying
superficial to the muscle layer.
■ Next, the introducer is inserted on the right, at the level just lateral to the
beginning of the indentation. The introducer is passed through the intercostal
muscles.
■ Under direct visualization, the introducer is passed just posterior to the
sternum, using extreme caution to avoid injury to the heart (TECH FIG 1A).
■ Upon crossing the midline, the introducer is then passed through the left-
sided intercostal muscles just lateral to the edge of the depression and taken
out the skin incision.
■ Next, umbilical tape is tied to the introducer on the left side of the chest,
pulled through the tract, and removed on the right side.
■ The preformed bar is then attached to the umbilical tape, and it is then pulled
through the track, with the apex of the bar angled away from the sternum
(TECH FIG 1B).
■ The bar is then rotated 180 degrees, such that the convex portion of the bar is
against the sternum (TECH FIG 1C).
■ This is secured in place using bar stabilizers on both sides. Either wire of
heavy PDS suture is passed around the stabilizers and the rib to avoid bar
displacement.
■ For patients that have severe deformity, a second bar can be used. This is
placed in the same manner, slightly inferior to the original bar.
Closure
■ A 16-French red rubber catheter is placed in the tract of the camera port. A
positive end expiratory pressure of 5 mm Hg is given, and a Valsalva is done
by anesthesia to evacuate any residual air within the thoracic cavity.
Alternatively, Valsalva without a red rubber catheter can also be used to
evacuate residual air.
■ The lateral incisions are then closed in layers using an absorbable suture
■ The skin is closed with a running subcuticular suture
■ Rarely, a chest tube is required for this procedure.

TECH FIG 1 • A. The minimally invasive approach to
pectus excavatum, the Nuss procedure, inserts the
bar at the level of the deepest depression. B. The bar
is inserted with the bend away from the chest wall. C.
The bar is then rotated 180 degrees, correcting the
deformity.
■ Open Ravitch Procedure for Pectus Excavatum
Positioning and Incisions
■ The patient should be in the supine position with their arms out to the side
on the operating room table.
■ Both the chest and abdomen should be prepped and draped into the sterile

field.
■ The operating surgeon will be positioned on the patient’s right, the assisting
surgeon on the patient’s left, and the anesthesiologist at the head of the bed.
■ Two incisions can be used depending on the cosmetic outcomes. A sternal
incision or transverse incision at the fourth intercostal space is used in males.
■ In females, better cosmetic outcomes can be achieved using a transverse
incision instead of a sternal incision. Care should be taken to make this
incision within the projected inframammary crease to prevent injury to the
breast bud (TECH FIG 2).
Exposure and Resection of the Abnormal Ribs
■ Continue the incision creating skin flaps that are centered at the sternum.
The skin flap should extend cephalad to the angle of Louis, the
manubriosternal junction, and caudally to the xiphoid, allowing for adequate
visualization of sternum and costal cartilage.
■ Next, the pectoralis major muscle flaps should be elevated; passing an empty
knife anterior to the costal cartilage insures the correct plane. This is
continued cephalad until the highest abnormal cartilage is seen.
■ Next, the rectus abdominis is mobilized off of the inferior border of the
sternum.
■ The perichondrium is excised anteriorly to expose the bony rib up to the
sternum, exposing the cartilage at its insertion site on the sternum
■ Using a Haight elevator to protect the mediastinum, the deformed cartilage is
cut from the sternum using a knife. The deformed cartilage is then removed
and cut at the costal cartilage junction, leaving a 5-mm margin.

TECH FIG 2 • Open repair, Ravitch procedure, is used
for both pectus excavatum and carinatum. Incision is
made either midline for males or inframammary in
females.
TECH FIG 3 • A. Skin flaps are made, and the
periosteum is cleared from both sides of the sternum.
The deformed cartilage is cut from the sternum using
a knife and removed. B. The sternum is then either
flattened with a wedge osteotomy (for carinatum) or
elevated with a transverse osteotomy (for excavatum).
■ This is continued until all deformed cartilage has been resected. Then, a
sternal wedge osteotomy is made just cranial to the final resected cartilage
(TECH FIG 3).
Sternal Closure
■ At the level of the osteotomy, the sternum is fractured and lifted into a flat
orientation.
■ Although not necessary in all cases, a sternal bar can be inserted posterior to

the sternum at this point. This is secured to the adjacent ribs for greater
stabilization.
■ If a bar is not used, heavy silk suture is used to close the sternal ostomy,
fixing the sternum at the corrected position.
■ Perichondrial sheaths are then reappoximated if necessary.
Closure
■ A Hemovac drain is inserted through the inferior skin flap and placed along
the sternal edge of the cut cartilage.
■ The pectoralis muscle is then closed in the midline and secured to the
sternum.
■ The rectus muscles are then closed in the midline using an absorbable suture
and reattached to the sternum.
■ Layered closure of the subcutaneous tissues is done, and the skin is closed
using a subcuticular stitch.
■ Open Ravitch Procedure for Pectus Carinatum
■ The initial steps of the carinatum repair mirror that of the Ravitch procedure
for pectus excavatum.
■ The difference in technique from excavatum begins when the sternal
osteotomy is made. Unlike in pectus excavatum in which a wedge osteotomy
is made, a transverse linear osteotomy is made for correction of pectus
carinatum at the point where the superficial deflection begins.
■ The xiphoid process is detached from the sternum to allow for better sternal
mobilization. The sternum is then flattened posteriorly to create a flat surface.
The xiphoid process is then reattached once the sternum is in correct
position.9
■ Closure is the same as for Ravitch procedure for pectus excavatum.
PEARLS AND PITFALLS
Pectus
excavatum
■ Most patients with symmetric pectus excavatum are well suited for a
Nuss procedure.

■ Most patients who require redo surgery for pectus excavatum may
benefit from a Ravitch procedure or a combined Ravitch and Nuss
procedure.
Pectus
carinatum
■ Nonoperative treatment is the mainstay of treatment for carinatum;
however, asymmetric lesions tend to require surgery more often. For
symmetric deformities, bracing is first-line treatment.
■ In some hands, the combination of postoperative antibiotics with
sterile operative techniques has resulted in almost no wound infections
in patients with pectus carinatum10
POSTOPERATIVE CARE
Pectus Excavatum
■ Chest radiograph should be performed in the recovery room. This should
confirm appropriate placement of the bar and also evaluate for
pneumothoraces. It is not uncommon to have very small bilateral
pneumothoraces that can be monitored; these usually do not require tube
thoracostomy.
■ Pain can be significant in the postoperative period for both procedures.
Additionally, for some patients, pain can continue during the entire time the
Nuss bar is in place. This pain can be managed generally with physical therapy
and medications. Rarely, the bar is removed for severe pain.
■ For the initial 6 weeks postoperatively, patients must refrain from any sports.
■ Patients are forbidden from participating in any heavy contact sports, such as
wrestling, football, and hockey for the entire duration that the Nuss bar is in
place. Aerobic activities, such as running or soccer, are allowed after the initial
6 weeks.
■ The Nuss bar must remain in place for at least 2 years. Timing of removal is
based upon patient growth; for older patients in their rapid growth phase,
removal is usually closer to 2 years. For younger patients who have not yet
reached puberty, the bar can remain in place for up to 4 years.
Pectus Carinatum
■ Postoperative pain is initially managed with intravenous analgesia and then
transitioned to oral pain medication.
■ Generally, patients are discharged within 2 to 3 days postoperatively.
■ Intravenous antibiotics can be considered for 48 hours postoperatively, and

oral antibiotics are continued for another 4 days.
OUTCOMES
■ No difference in length of stay between open and Nuss procedures has been
demonstrated. Procedure length was slightly increased with open operation.
■ The Nuss procedure is associated with higher rates of reoperation,
pneumothorax, and hemothorax.11
■ Postoperative seroma formation is the most common complication in the open
procedure. Due to this, a drain is left in the potential space within the skin
flaps.
■ Patient satisfaction scores have not been found to differ significantly between
Nuss and open procedures, with 93.3% and 92% reported overall improved
cosmetic outcomes, respectively. Symptomatic relief was also reported by both
groups of patients.12
■ Overall recurrence rates vary among studies but have been quoted between 2%
and 10% in both open and Nuss procedures.13
COMPLICATIONS
Pectus Excavatum
■ Cardiac perforation: this is an exceedingly rare intraoperative complication;
however, if it does occur, it has an extremely high rate of mortality. Immediate
sternotomy with cardiac repair should be done. This complication is more often
associated with the Nuss procedure.
■ Pneumothorax: in general, asymptomatic pneumothorax can be observed.
Rarely, this may be symptomatic and require insertion of a chest tube.
■ Bar dislodgement in the Nuss procedure can occur in up to 5% of patients; this
can happen through the immediate or late postoperative period. If early, it can
lead to immediate recurrence, and reoperation is required.
■ Allergic reaction to the metal bar, ranging from rash to anaphylaxis.
■ Prolonged pain, requiring bar removal in some cases.
■ Recurrence has been documented after bar removal.
Pectus Carinatum
■ Fortunately, complications are rare following repair of pectus carinatum.10
■ Pneumothorax has been quoted at rate of 2% after repair10,14 but were less
than 10% of total lung volume.

■ Wound infection is a risk with any surgery and occurs at a very low rate.
■ Recurrence/continued exercise intolerance: almost all patients report an
improvement in exercise tolerance 3 to 4 months post procedure, and only
2.8% did not report an excellent result.
REFERENCES
1. Desmarais TJ, Keller MS. Pectus carinatum. Curr Opin Pediatr.
2013;25(3):375-381.
2. Haller JA Jr, Kramer SS, Lietman SA. Use of CT scans in selection of patients
for pectus excavatum surgery: a preliminary report. J Pediatr Surg.
1987;22(10):904-906.
3. Khanna G, Jaju A, Don S, Keys T, Hildebolt CF. Comparison of Haller index
values calculated with chest radiographs versus CT for pectus excavatum
evaluation. Pediatr Radiol. 2010;40(11):1763-1767.
4. Nuss D, Kelly RE Jr. Indications and technique of Nuss procedure for pectus
excavatum. Thorac Surg Clin. 2010;20(4):583-597.
5. Sacco-Casamassima MG, Goldstein SD, Gause CD, et al. Minimally invasive
repair of pectus excavatum: analyzing contemporary practice in 50 ACS
NSQIP-pediatric institutions. Pediatr Surg Int. 2015;31(5):493-499.
6. Frantz FW. Indications and guidelines for pectus excavatum repair. Curr Opin
Pediatr. 2011;23(4):486-491.
7. Kelly RE, Goretsky MJ, Obermeyer R, et al. Twenty-one years of experience
with minimally invasive repair of pectus excavatum by the Nuss procedure in
1215 patients. Ann Surg. 2010;252(6):1072-1081.
8. Banever GT, Konefal SH, Gettens K, Moriarty KP. Nonoperative correction of
pectus carinatum with orthotic bracing. J Laparoendosc Adv Surg Tech A.
2006;16(2):164-167.
9. Robicsek F, Watts LT, Fokin AA. Surgical repair of pectus excavatum and
carinatum. Semin Thorac Cardiovasc Surg. 2009;21(1):64-75.
10. Fonkalsrud EW. Surgical correction of pectus carinatum: lessons learned
from 260 patients. J Pediatr Surg. 2008;43(7):1235-1243.
11. Nasr A, Fecteau A, Wales PW. Comparison of the Nuss and the Ravitch
procedure for pectus excavatum repair: a meta-analysis. J Pediatr Surg.
2010;45(5):880-886.
12. Jo WM, Choi YH, Sohn YS, et al. Surgical treatment for pectus excavatum. J
Korean Med Sci. 2003;18(3):360-364.
13. Antonoff MB, Erickson AE, Hess DJ, et al. When patients choose:

comparison of Nuss, Ravitch, and Leonard procedures for primary repair of
pectus excavatum. J Pediatr Surg. 2009;44(6):1113-1118.
14. Fonkalsrud EW, DeUgarte D, Choi E. Repair of pectus excavatum and
carinatum deformities in 116 adults. Ann Surg. 2002;236(3):304-312.

61
CHAPTER
Section XVI: Thorax and
Back
Myelomeningocele:
Postnatal Repair
Gregory G. Heuer and Jesse A. Taylor
DEFINITION
■ Myelomeningocele (MMC) is a congenital malformation of the spinal cord in
the family of neural tube defects and spina bifida.1
■ MMC develops from a failure of neurulation of the spinal cord during the
fourth week of gestation. The exposure of the spinal cord results in dysfunction
of neural tissue as a result of the malformation itself and damage to the
exposed neural tissue.
■ MMCs occur in roughly 1 in 1000 live births and lead to significant morbidity
including bowel and bladder dysfunction, gait disturbances, and even paralysis.
Importantly, lack of sensation caudal to the MMC may result in significant
decubital wound issues.
ANATOMY
■ The MMC results from failure of neurulation, which results in an “open book”
of distinct elements that are normally closed over the spinal cord2 (FIG 1).
■ The placode, the exposed flat spinal cord tissue, is present in the center, with
nerve roots running ventrally and exiting through the spine foramen. Atrophic
nerve roots can often be seen exiting the placode and running to the skin and
soft tissue. These are nonfunctional elements.
■ The zona epitheliosa surrounds the placode and contains cerebrospinal fluid
(CSF). This is part of the sac or “bubble” seen on imaging studies.

■ The dura of the meninges is open and reflected and covers the fascia and
muscle.
■ The skin is located laterally to the lesion. Some of this tissue is extremely
thin and atrophic. The quality of the skin needs to be taken into account, as it
impacts surgical closure.
FIG 1 • Appearance of an MMC defect. The neural
placode (pl) is seen at the center. This is surrounded by
the zona epitheliosa (ze) and the skin (sk). The
myofascial layer can often be seen through the
translucent portion of the arachnoid surrounding the ze.
■ Elements of the posterior vertebral body—the spinous process and lamina—
are absent, and the spinal column is open dorsally. The open spinal column
can be seen laterally as the bifida bone elements.
■ In some cases, a sac is not present, and the placode is flat and in the spinal
elements. This condition is known as myeloschisis and is managed in the same
way as a MMC.
PATHOGENESIS
■ During neurulation, normally the neural tube undergoes an ordered process of
formation and closure. During this process, the neural folds meet and fuse,
followed by closure of the layers around the spinal cord—including the dura,
bone, muscle, and skin.1
■ MMC results from a failure of the neural tube to close at the caudal end,
resulting in the exposure of the spinal cord—the placode—to the external
environment.

■ Most cases are idiopathic, though risk factors include genetic susceptibility
and environmental risks such as toxins or medication exposure.
■ Risk factors include genetic susceptibility and environmental risks such as
toxins or medication exposure. In most cases, there is not an identifiable cause
and is thus idiopathic.
■ In some populations, the use of high-dose folic acid supplementation prior to
conception can reduce the risk of neural tube defects, as folate is a known
cofactor in many of the folding processes involved in neurulation.
NATURAL HISTORY
■ MMC leads to irreversible loss of nerve function at the level of the exposed
spinal cord.
■ This loss of function occurs from3:
■ Abnormal development of the spinal cord
■ Direct damage to the exposed spinal cord from trauma and toxic injury
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Patients with MMC are typically identified prior to birth.
■ Ultrasound examination is performed for three reasons:
■ To define the MMC defect and its spinal level
■ To look at associated abnormalities of the central nervous system
■ To analyze the fetus for other structural abnormalities
■ Patients with known MMC lesions are typically delivered via cesarean section.
■ Newborn MMC patients are cared for in a systematic fashion
(
neonatal-clinical-pathway).
■ General neonatal care must be the first priority, assuring that the newborn is
stable and healthy and avoiding the temptation to focus solely on the MMC
lesion.
■ In the presence of a MMC, the patient is generally positioned prone to avoid
undue pressure on the exposed spinal elements. A sterile gauze is placed on
the MMC lesion, and this is kept moist with a constant drip of sterile saline
from a bag suspended above the patient.
■ The patient is placed on antibiotics (ampicillin and amoxicillin) as
prophylaxis against bacterial seeding of the spinal elements.
■ Consults are obtained including the physical therapy, neurosurgery,
orthopedics, plastic surgery, urology, and spina bifida team.

■ An ultrasound of the head is obtained to establish a baseline of the
ventricular system.
■ A physical exam is performed on the infant.
■ The infant is examined for signs of hydrocephalus such as a full fontanelle,
large head circumference, or splayed sutures.
• In most instances, newborn MMC patients do not show clinical signs of
hydrocephalus until after the MMC closure is performed.
• The anatomy of the MMC defect is similar (see FIG 1) even if the overall
appearance of the lesion may be grossly variable (FIG 2).
■ The size of the MMC lesion is noted as well as the quality of the skin. These
factors are important to anticipate the need for specialized closure techniques
such as rotational flaps, allograft patches, or skin grafts.
■ A neurologic exam is performed to assess the clinical level of function. The
clinical exam is very difficult to interrupt in a newborn and should be done by
a trained clinician and followed over time for any changes. Movement
secondary to direct stimulation that is not clinically significant can be present
below the level of the lesion and should not be confused with volitional
movement.4
■ The infant should be examined for orthopedic deformities, such as talipes or
hip contractures. These are important to note prior to the MMC closure as
they may affect patient positioning both before and after surgery.
■ Surgical closure of the MMC is typically performed within the first 48 hours
after birth.
■ However, this can be delayed if the patient is not clinically stable as long as
the positioning, wound care, and prophylactic antibiotics are administered.
The risks of delaying surgery must be carefully weighed against the risks of
infection and further neurological deterioration. If the surgery is delayed for
weeks to months, the granulation tissue that develops can increase the
technical difficulty of the closure.
■ MMC closure is performed in a stepwise fashion to close the layers over the
spinal cord that should be present.
SURGICAL MANAGEMENT
■ The overall goal of the surgical closure is to obtain a multilayer closure of all
three layers of the back—dura, muscle, skin—that is watertight.
Positioning

■ The infant is placed prone with the abdomen suspended to promote venous
return and reduce bleeding during surgery (FIG 3).
■ The back is widely prepped on the table with Betadine. The back must be
prepped in a wide fashion to allow for skin rotational procedures.
FIG 2 • Various MMC lesions. Note the variability in the
size and appearance of the lesions.
FIG 3 • Intraoperative positioning of an MMC patient.
T E C H N I Q U E S
■ Release of the Placode

■ The tissue just lateral to the placode is sharply incised, releasing the placode
to float ventrally into the spinal column (TECH FIG 1A).
■ It is often necessary to make this incision further from the placode and the
residual tissue should be dissected from the placode.
■ The placode can be rolled back into a tubular structure by placing sutures in
the pial layer. This is often accomplished with 6-0 Prolene interrupted sutures
(TECH FIG 1B).
TECH FIG 1 • A. Dissection of the placode from the
surrounding tissue. B. The released placode is
brought into a tubular shape with fine sutures.
■ Skin Release and Mobilization
■ The skin is then released from the underlying fascia and muscle (TECH FIG
2).
■ The skin is undermined widely, resulting in greater exposure of the
underlying fascia and freeing up soft tissues that can be more easily rotated
over the fascia during the skin closure.
■ Care needs to be taken to stay in the proper plane and avoid tearing the skin
and soft tissues during this undermining process.
■ The edges of the skin are then trimmed back to the tissue that will support
dermal and skin sutures.
TECH FIG 2 • The skin is extensively undermined.
■ Dura, Myofascial, and Skin Closure
■ The next step is closure of the lateral tissue over the placode. The goal of the
closure is a watertight closure over the placode.
■ The skin is held with a retractor, exposing the lateral dura and fascia.
■ An incision is made in the lateral tissue. This tissue is normally the dura that

is present over the top of the muscle and fascia.
■ This is carefully mobilized over the underlying tissue and rotated over the
placode (TECH FIG 3A).
■ The dura may be thin or absent in the sacral portion of the MMC.
■ The next layer is the myofascial layer (TECH FIG 3B).
■ An incision is made in the fascial layer. This needs to be several
centimeters away from the opening to allow it to be rotated over the dural
closure without tension. Mobilization of the layer can be assisted by
releasing this layer from the opening spina bifida elements.
■ This layer is rotated to the midline and closed (TECH FIG 3C).
■ After closure of the myofascial flaps, the skin is further undermined.
Additionally, extension of the superior and inferior skin edges can increase
the mobilization of the skin flap.
■ The skin flaps are then rotated to the midline and closed in two layers
(TECH FIG 3D).
Additional Maneuvers
■ If the skin cannot be mobilized, additional maneuvers are sometimes needed.
■ This can include placing an AlloDerm patch (LifeCell, Branchburg, NJ)
(TECH FIG 4), relaxing incisions in the posterior axillary line, or Z-plasty
extensions.
TECH FIG 3 • A. The dural flaps are rotated over the
placode and closed in the midline. B. Exposure of the
lateral myofascia. C. Rotation and closure of the
myofascial flaps. D. Appearance of the skin after
closure.

TECH FIG 4 • Appearance of a large MMC
preoperatively (A), which resulted in a skin wound (B)
that required an AlloDerm patch (C).
PEARLS AND PITFALLS
Closure
■ A multilayer watertight closure is essential to prevent wound
breakdown and protect the placode from further damage or
infection.
■ If fluid collections form that are consistent with
pseudomeningoceles, this needs to be treated, normally with CSF
diversion.
■ It is important to be aware of the vascular supply to skin flaps to
prevent necrosis of the skin when performing Z-plasty or relaxing
incisions.
Intraoperative
care
■ The patient needs to be positioned to take pressure off the
abdomen and other pressure points; this will promote venous
return, reduce bleeding, and prevent pressure sores.
■ The patient should be prepped and draped widely to allow
multiple options for skin closure.
POSTOPERATIVE CARE
■ The patient is cared for in the neonatal ICU. Close observation for bladder
dysfunction, pressure points, and orthopedic issues is paramount.
■ Special attention should be paid to the examination of the wound. Wound care
is very important as the wound is in the lumbosacral area and is prone to

contamination with stool or urine. A drape separating the anal region from the
MMC closure should be placed to divert stool, but local wound care must be
maximal to prevent infection.
■ The child needs to be monitored for the development of hydrocephalus. In
patients with a full fontanelle, enlarging pseudomeningoceles, or other clinical
signs of hydrocephalus, they should be treated with CSF diversion, either with
a ventriculoperitoneal shunt or an external ventricular catheter.
REFERENCES
1. Copp AJ, Adzick NS, Chitty LS, et al. Spina bifida. Nat Rev Dis Primers.
2015;1:15007.
2. Heuer GG, Adzick NS, Sutton LN. Fetal myelomeningocele closure: technical
considerations. Fetal Diagn Ther. 2015;37(3):166-171.
3. Walsh DS, Adzick NS, Sutton LN, Johnson MP. The rationale for in utero
repair of myelomeningocele. Fetal Diagn Ther. 2001;16:312-322.
4. Rintoul NE, Sutton LN, Hubbard AM. A new look at myelomeningoceles:
functional level, vertebral level, shunting, and the implications for fetal
intervention. Pediatrics. 2002;109:409-413.

62
CHAPTER
Myelomeningocele, Prenatal
(Fetal) Repair
Gregory G. Heuer and Scott N. Adzick
DEFINITION
■ Myelomeningocele (MMC) is a congenital malformation of the spinal cord in
the family of neural tube defects and spina bifida as described in the previous
chapter (Myelomeningocele, Postnatal Closure).
■ Prenatal surgery was first attempted in the 1990s. A randomized controlled
trial, the Management of Myelomeningocele Study (MOMS), demonstrated
that closure during the prenatal period could be performed relatively safely and
can result in significant benefit to the child.1
ANATOMY
■ The MMC results from failure of neurulation resulting in an “open book” of
distinct elements that are normally closed over the spinal cord (FIG 1) as
described in the Myelomeningocele, Postnatal Closure chapter.
PATHOGENESIS
■ During neurulation, normally the neural tube undergoes an ordered process of
formation and closure as described in the Myelomeningocele, Postnatal
Closure chapter.
NATURAL HISTORY
■ MMC leads to irreversible loss of nerve function at the level of the exposed
spinal cord as described in the previous chapter (Myelomeningocele, Postnatal
Closure).
■ In addition, a fetus with MMC will progressively develop two associated
conditions as the result of the continued cerebrospinal fluid (CSF) leak, a
Chiari malformation with hindbrain herniation and hydrocephalus.2

FIG 1 • Appearance of an exposed MMC defect during
a fetal repair. The neural placode (pl) is seen at the
center. This is surrounded by the zona epitheliosa (ze)
and the skin (sk). The myofascial layer (mf) covered by
dura can be seen through the translucent arachnoid.
PRENATAL EVALUATION
■ Expectant mothers receive extensive prenatal testing to determine if they are
candidates for prenatal surgery. The inclusion and exclusion criteria in the
MOMS trial are still used by most fetal therapy centers (Table 1).3
■ Ultrasound (US) examination is performed for three reasons: to define the
MMC defect and its spinal level, look at associated CNS abnormalities, and
evaluate the fetus for other structural abnormalities.
■ With regard to the MMC defect and CNS-associated conditions, US is used
specifically:
■ To define the level of bone and skin opening
■ To analyze the posterior fossa, specifically to look for the presence of
hindbrain herniation (must be present as it confirms an open neural tube
defect)
■ To determine the size of the lateral ventricles and degree of ventriculomegaly
■ To look for any associated abnormalities such as a spinal cord syrinx, a split
cord malformation, or a significant kyphosis that may affect the eligibility of
the patient for fetal MMC repair.
■ Patients also receive a fetal magnetic resonance imaging (MRI) scan. These
scans provide a more detailed anatomic evaluation of the MMC defect and
associated abnormalities, particularly the presence of hindbrain herniation.

■ In addition to imaging, the fetus undergoes an analysis for genetic or
chromosomal abnormalities. Also, amniotic fluid analysis is performed to
confirm an open neural tube defect.
■ A detailed medical history for the mother is obtained, specifically looking for
significant maternal health issues and risk factors associated with premature
delivery.
SURGICAL MANAGEMENT
■ The overall goal of the surgical repair is to obtain a multilayer closure that is
watertight. To provide the greatest possible benefit to the fetus, the closure
needs to protect the neural structures from direct trauma, prevent the leakage of
CSF, and prevent the exposure of the neural structures to amniotic fluid.
Additionally, the closure needs to be done in a manner that is safe for the fetus
and the mother, as extreme premature birth can erase all potential gains of the
surgical procedure. There are some modifications of the general technique
outlined below, but the principles above should not be compromised.
Preoperative Planning3
■ A preoperative US is obtained to confirm the location of the fetus and the
placenta.
Table 1 MOMS Trial Inclusion and Exclusion Criteria
Inclusion
Singleton pregnancy
Lesion level T1-S1
Evidence of hindbrain herniation
Gestational age of 19.0–25.9 weeks
Normal karyotype
U.S. residency
Maternal age of 18 years or older
Exclusion
Fetal anomaly unrelated to myelomeningocele

Severe kyphosis greater than or equal to 30 degrees

Multiple gestation

Previous spontaneous preterm birth less than 37 weeks

Short cervix less than 20 mm

Placental abruption or abnormal placentation (placenta
previa)

Obesity defined as BMI > 35

Maternal medical condition that would place an additional
risk to maternal health/surgical risk or the pregnancy
(insulin-dependent diabetes, poorly controlled
hypertension)

Documented history of incompetent cervix or
planned/current cerclage

Maternal-fetal Rh isoimmunization, Kell sensitization, or a
history of neonatal alloimmune thrombocytopenia

Maternal HIV, hepatitis B or hepatitis C positivity

Uterine anomaly such as multiple fibroids or müllerian duct
abnormality; previous hysterotomy in the active segment of
the uterus

Patient does not have a support person

Inability to comply with the travel and follow-up
requirements

Patient does not meet other psychosocial criteria to handle
the implications of the trial

Participation in another study that influences maternal and
fetal morbidity and mortality or participation in this trial in a
previous pregnancy
■ The mothers receive an epidural catheter for delivery for intraoperative and
postoperative pain control.
■ Antibiotics and indomethacin are given.
Positioning and Anesthetic Concerns3
■ The mother is placed supine on the operating room table with a bump placed
under the right side to avoid inferior vena cava compression by the gravid
uterus and compromise of venous return.
■ The surgery is performed under deep general anesthesia, with the goal to
achieve maximum uterine relaxation. In addition to maternal anesthetic, the
fetus receives a direct dose of intramuscular anesthesia (vecuronium and
fentanyl).
■ The cardiac function of the fetus is continually monitored by fetal
echocardiography. If a decrease in cardiac function is seen, the anesthetic to the
mother is adjusted or the fetus is examined for signs of umbilical cord or

placental compression.4
■ An arterial line is placed in the mother.
Approach
■ The uterus is approached via a low transverse maternal laparotomy. Of note,
the uterus is opened in the upper muscular portion and not the lower segment
used for typical cesarean sections (TECH FIG 1A).
■ The site of the placenta is mapped out on the surface of the uterus. If the
placenta is anterior, the uterus is rotated out of the abdomen to gain access to
the fundus and posterior uterine wall. The goal is to open the uterus at least 6
cm away from the placental edge. In addition to the placenta, the location of
the fetus is noted so that the hysterotomy is made directly over the MMC
defect to minimize the need for manipulation of the fetus. In some cases, the
fetus is moved prior to opening the uterus to aid in this exposure.
■ The uterus is opened under continuous sonographic visualization.
■ Two monofilament traction sutures are placed through the full thickness of
the uterus.
■ A Bovie cautery is then used between the sutures and the uterus opened.
■ An atraumatic clamp is placed through this opening to compress the uterine
tissue and to facilitate the firing of a specialized uterine stapling device that is
only partially inserted. This device places a row of absorbable polyglycolic
acid staples across the uterine wall and fetal membranes. This device cuts the
uterine wall at the same time it places the staples, holding the layers together
in a normal anatomic position and occluding crossing blood vessels. This is
then repeated in the opposite direction to create the 6-cm hysterotomy.
■ An infusion of body temperature lactated Ringers with a level I infusion
device is used to maintain the uterine volume and keep the fetus warm and
buoyant.
■ The fetus is then held in the hysterotomy incision so that the back and MMC
defect are in the opening.
■ After the MMC repair the uterus and then the maternal laparotomy are closed
in a layered fashion.
■ Full-thickness stay suture is placed first, followed by a running full-thickness
layer that incorporates the absorbable staples. During this closure the uterus is
filled with the lactated Ringers solution to reconstitute the amniotic fluid
volume, and intra-amniotic antibiotics are added.
■ The running layer suture is tied followed by tying of the individual stay

sutures.
■ The uterine closure is then covered with an omental patch to help seal it and
provide blood supply.
■ The laparotomy wound and skin are then closed.
T E C H N I Q U E S
■ Fetal MMC repair is performed in the same manner as postnatal repair.
There are three important differences to the prenatal repair compared to the
postnatal repair.5
■ First, one advantage of fetal MMC closure is the chance to maintain spinal
function, and therefore, even greater care is needed in the fetal patient to
minimize any trauma to the placode and neural tissue.
■ Second, the fetal tissue is more delicate than in a newborn, and therefore it
may tear with even minor manipulation.
■ Lastly, maneuvers, such as pulling the skin tightly, that may be tolerated in
a newborn may lead to cardiac depression in the fetus and are therefore
avoided.
■ Release of the Placode
■ The tissue just lateral to the placode is sharply incised, releasing the placode
to float ventrally into the spinal column (TECH FIG 1B).

TECH FIG 1 • The laparotomy incision is shown in A.
The incision in the uterus is performed in the upper
segment. B. The exposed MMC defect. The fetus is
positioned to allow access to the MMC defect and
monitor the fetal cardiac function. C. The exposed
MMC defect is then repaired.

■ It is often necessary to make this incision further from the placode than
desired to avoid damaging the spinal cord or exiting nerve roots. In these
cases, the residual tissue should be dissected from the placode to reduce the
risk of subsequent inclusion cyst formation.
■ Skin Release and Mobilization
■ The skin is then released from the underlying fascia and muscle.
■ The skin is undermined using scissors in an elliptical fashion for several
centimeters.
■ Undermining of the skin allows the skin to be mobilized, resulting in greater
exposure of the underlying fascia and skin that can be more easily rotated
over the fascia during the skin closure.
■ Care needs to be taken to stay in the proper plane and avoid tearing the skin
during this undermining process.
■ Dura and Myofascial Closure
■ The next step is closure of the lateral tissue over the placode.
■ The goal of the closure is a watertight closure over the placode.
■ The skin is held with a retractor, exposing the lateral dura and fascia. The
skin opening can be extended superiorly and inferiorly to aid in the
exposure of the fascia.
■ An incision is made in the lateral tissue and this tissue is then rotated over
the released placode. Some authors close only the dural layer, but we prefer
a thick myofascial flap with the dural layer on the mesial surface to ensure
watertight closure and direct contact between the delicate dural layer and
the neural placode.
■ The myofascial flaps are then closed with a running suture such as a 4-0
polydioxanone (PDS). When this thick layer is closed in the midline, care
is taken to approximate the dural surfaces and to protect the placode. This
protection can be assisted with placement of a freer retractor over the
placode during the suturing (TECH FIG 1C).
Skin Closure
■ After closure of the myofascial flaps, the skin is further undermined.
■ The skin flaps are then rotated to the midline; an assessment is then made if

flaps can be rotated to the midline without tearing the tissue significantly or
leading to tension-related cardiac depression.
■ A running monofilament, such as 4-0 PDS, is placed. The suture should be
placed loosely; once the suture has been placed across the opening, the suture
is pulled tight after each stitch in a stepwise fashion until the skin edges meet.
This “lacing” technique allows the tension to be spread across the incision
and reduces tearing. Additionally, to reduce the chance for tearing the suture
through the skin, the suture should be placed away from the skin edge and in
the thicker portion of the skin flaps.
■ If the skin cannot be closed, a graft is then sized to the opening. This graft is
normally constructed with AlloDerm (LifeCell, Branchburg, NJ) and is
elliptical in shape. It is sutured with two running monofilament sutures.
■ Other groups have described the use of relaxing incisions in the flank to aid
in mobilization. However, these are generally not used because they are at
risk to fail from devascularization after skin mobilization.
PEARLS AND PITFALLS
Selection
■ A detailed study of the mother and fetus is necessary for proper
patient selection, confirming the presence of an open neural tube
defect and no other associated fetal or maternal abnormalities that
will prevent a good outcome.
Closure
■ A multilayer watertight closure is essential for maximal benefit to
the fetus.
■ The goal of the surgery is to release the neural tissue and then
rotate and close the layers over the placode, consisting of the dura,
muscle, and skin.
Intraoperative
Care
■ A dedicated fetal team is essential for a good outcome. This team
should include fetal surgery, experienced anesthesia, obstetrics,
and fetal cardiology.
Outcome
■ Fetal MMC repair can result in significant benefits for motor
function, reversal of hindbrain herniation, and reduction in
hydrocephalus.
POSTOPERATIVE CARE

■ Postoperative care is focused on maintaining the pregnancy.
■ During the operative procedure, the mother is given a bolus of magnesium
sulfate and started on an infusion. This reduces uterine contractions and the
risk for premature delivery. This infusion is continued for the first 18 to 24
hours.
■ The mother is started on oral nifedipine after the magnesium sulfate infusion.
■ The mother is given indomethacin prior to surgery, and this is continued for 48
hours postoperatively.
■ The mother is observed as an inpatient for an average of 4 days and then is
discharged to modified bed rest. The mother is followed closely for signs of
premature labor or amniotic fluid leakage.
■ Delivery is performed via an elective cesarean section prior to the
development of labor and contractions, as this could lead to uterine rupture.
The goal is to deliver at 37 weeks, but often the delivery is performed earlier
than this time.
REFERENCES
1. Adzick NS, Thom EA, Spong CY; for the MOMS Investigators. A
randomized trial of prenatal versus postnatal repair of myelomeningocele. N
Engl J Med. 2011;364:993-1004.
2. Sutton LN, Adzick NS, Bilaniuk LT, et al. Improvement in hindbrain
herniation demonstrated by serial fetal magnetic resonance imaging following
fetal surgery for myelomeningocele. JAMA.1999;282:
1826-1831.
3. Danzer E, Adzick NS. Fetal surgery for myelomeningocele: patient selection,
perioperative management and outcomes. Fetal Diagn Ther. 2011;30:163-173.
4. Rychik J, Cohen D, Tran KM, et al. The role of echocardiography in the
intraoperative management of the fetus undergoing myelomeningocele repair.
Fetal Diagn Ther. 2015;37:172-178.
5. Heuer GG, Adzick NS, Sutton LN. Fetal myelomeningocele closure: technical
considerations. Fetal Diagn Ther. 2015;37:166-171.

63
CHAPTERSacrococcygeal Teratoma
Nicholas J. Ahn and William H. Peranteau
DEFINITION
■ Usually benign extragonadal germ cell tumor that develops prenatally,
involving the coccyx with various degrees of pelvic and intra-abdominal
involvement.
■ It is the most common congenital neoplasm found in fetuses and neonates.
■ Between 11% and 35% of the tumors may contain malignant elements such as
yolk sac tumor or embryonal carcinoma.1
■ Large or solid tumors may lead to poor clinical outcomes due to high-output
cardiac failure, hydrops fetalis, tumor rupture, or internal hemorrhage.2
■ Surgical resection is the mainstay of treatment but has associated morbidities
such as problems with micturition, defecation, or cosmetic dissatisfaction up to
40% to 50%.3 Large tumors with significant pelvic/intra-abdominal
involvement have the most significant risk for urologic and anorectal
complications.4
ANATOMY
■ All sacrococcygeal teratomas (SCTs) involve the coccyx but are classified into
four categories according to the location and extent of the tumor—the Altman
Classification Scheme.5
■ Type I: primarily external with a small presacral component
■ Type II: external with intrapelvic extension
■ Type III: primarily pelvic and intra-abdominal with external component
■ Type IV: completely intrapelvic with no external component
PATHOGENESIS

■ SCTs are thought to originate from the primitive knot (also known as Hensen
node), where the three germ cell layers start to form.
■ SCTs may be cystic, solid, or a mix of both. Solid tumors tend to be more
vascular and are associated with an increased risk of high-output cardiac
failure, hydrops fetalis, tumor rupture, hemorrhage, and fetal demise.
PATIENT HISTORY AND PHYSICAL FINDINGS
■ The majority of the lesions are now diagnosed by the time of birth as early as
18 to 19 weeks of gestation due to advancement in antenatal imaging.
■ Type I to III lesions that are not diagnosed prenatally are identified at birth by
an obvious protruding mass originating from the sacral region.
■ A rectal exam must be performed to assess the degree of intrapelvic extension
of the tumor, especially for tumors that were diagnosed postnatally.
■ Rarely, some type IV lesions can go undiagnosed until the child is up to 4
years of age and present with compressive symptoms of the bladder or rectum,
such as urinary retention, obstruction, or constipation.
IMAGING
■ Most SCTs are diagnosed prenatally at the time of approximately 20-week
gestation fetal anatomy ultrasound. If diagnosed, the mother and fetuses should
be referred to a high-volume multidisciplinary fetal diagnosis and treatment
center where a high-resolution fetal ultrasound will be performed to assess the
anatomical extension of the tumor and the characteristics of the mass (solid,
cystic, vascularity) (FIG 1A).
■ Ultrafast fetal MRI is used as an adjunct to ultrasonography to better assess the
SCT, including the pelvic or abdominal extension of the tumor and its
involvement with surrounding structures such as the colon, urinary tract,
vagina, hip, and especially the spine (FIG 1B).
■ After birth, hemodynamically stable infants may undergo a CT scan or MRI to
assess the extent of the tumor for preoperative planning (FIG 2).
DIFFERENTIAL DIAGNOSIS
■ Meningocele
■ Lymphangioma
■ Enteric duplication cyst
■ Dermoid cyst
■ Retrorectal hamartoma

■ Meconium pseudocyst
■ Tail remnant
■ Lipoma
FIG 1 • Prenatal SCT imaging. A. High-resolution fetal
ultrasound demonstrating a predominantly cystic type I
SCT with blood supply originating from the hypogastric
vessel. B. Ultrafast fetal MRI demonstrating a large type
I SCT that has solid and cystic components.
FIG 2 • Postnatal SCT imaging. Postnatal CT scan
demonstrating a predominantly type II SCT.
SURGICAL MANAGEMENT
Preoperative Planning

■ Fetuses with external tumors that are larger than 5 cm, predominantly solid, or
have a significant vascular supply should be delivered by cesarean delivery to
decrease the risk of catastrophic bleeding or tumor rupture.
■ If the patient is stable, the tumor can be resected within the first week of life.
■ CBC, type and screen, and cross-match must be performed in anticipation of
surgery.
■ Alpha-fetoprotein (AFP) and β-HCG levels should be obtained prior to
surgery.
■ CT scan or MRI of the abdomen and pelvis with contrast should be performed
as discussed above in stable patients.
Positioning
■ Most type I and type II lesions can be resected with the patient in prone
jackknife position and completely resected from the posterior approach (FIG
3).
FIG 3 • Positioning. Prone position of newborn with
large type I SCT.
■ Lesions with significant intra-abdominal components often require an anterior
(laparotomy) as well as posterior approach for complete resection and thus
should be positioned supine for the anterior approach initially.
■ Towel rolls or gel pads should be used to support the patient’s shoulder and

pelvis from underneath when in the prone position.
■ If uncertain regarding possible laparotomy, the patient should be sterilely
prepped from the chest to the toes to allow for free intraoperative repositioning
without repreparation and draping.
Approach
■ Most type I and II lesions can be completely resected from the posterior
approach.
■ Lesions with large intra-abdominal components (some type III and type IV)
require a combined anterior approach via a laparotomy and a posterior
approach.
T E C H N I Q U E S
■ Posterior Approach (TECH FIG 1)
■ Landmarks: gluteal crease, sacrococcygeal junction, anal orifice
■ A Foley catheter is placed prior to placing the patient in the prone jackknife
position.
■ The incision is made over the external component of the tumor in an
orientation that allows for the development of large skin flaps. This often
involves a transverse incision in an inverted V shape that starts at the
sacrococcygeal junction.

TECH FIG 1 • Posterior approach for postnatal SCT
resection. A. Most type I and II SCTs can be
approached from the posterior approach. Care must
be taken to identify the anus/rectum to avoid injuring
it. The coccyx is identified and resected with the
specimen. The surgeon must also try to save the
attenuated muscle fibers to reconstruct the pelvic floor
following tumor resection. B. Resected specimen.
Ideally the tumor and coccyx are resected as a single
specimen. Often thin, extra skin is resected with the
tumor specimen as there is frequently redundant skin
available for appropriate skin closure. C. Skin closure
following SCT resection.
■ The incision is carried down to the tumor capsule, which, in many cases, is
very superficial.
■ Being cognizant to stay on the tumor capsule to preserve attenuated muscle
fibers that will later be used to reconstruct the pelvic floor, the tumor is
circumferentially dissected out as skin/muscle flaps are raised.
■ Identify and always be cognizant of the position of the anus and rectum,
which are often displaced and closely opposed by the SCT. Placement of a
Hegar dilator or large red rubber catheter in the anal opening helps the
surgeon identify and avoid injury to the anus/rectum.
■ For tumors with a large intrapelvic component, similar attention should be
paid to the ureters.
■ Dissection of the tumor along its posterior superior border will lead the
surgeon to the coccyx, which must be identified and resected.
■ The sacrococcygeal junction is identified and circumferentially dissected
out.
■ Care is taken to stay on the anterior surface of the sacrococcygeal junction
during dissection to avoid injury to the vascular supply to the tumor that
usually originates from the middle sacral artery or branches from the
hypogastric.
■ With an instrument on the anterior surface of the coccyx to protect
underlying vessels, the sacrococcygeal junction is transected with a Bovie.

■ Ligate the vascular supply to the tumor after the coccyx is transected at the
sacrococcygeal joint.
■ The tumor and coccyx are ideally resected as a single specimen (TECH FIG
1B).
■ The muscles of the pelvic floor and anorectal sphincter are reconstructed.
■ Excess skin is removed to allow for the best appearance.
■ The skin is often closed in a “Mercedes” sign but can be closed in a vertical
incision (often following complete resection after an initial debulking, see
below) if more cosmetically appropriate (TECH FIG 1C).
■ If large skin flaps are raised, the skin is closed over a small drain.
■ Anterior Approach
■ Tumors with large intra-abdominal/intrapelvic components often require a
laparotomy via an anterior approach in combination with the above posterior
approach.
■ The anterior approach is done initially with the goal of identifying and
controlling the vascular supply to the tumor prior to significant dissection of
the tumor.
■ Large intra-abdominal/intrapelvic tumors may be closely associated with the
colon, intestinal mesentery, and the ureters all of which should be identified
and preserved.
■ The laparotomy is performed via a transverse incision between the umbilicus
and pubic symphysis or, in cases with a very large intra-abdominal
component, may be approached via a midline incision.
■ After controlling the vascular supply to the tumor and dissecting out the
intra-abdominal component of the tumor, the patient is flipped to the prone
position and the remainder of the tumor and coccyx resected.
■ Special Considerations—Debulking (TECH FIG 2)
■ Large, predominantly solid lesions often have increased tumor vascularity
and may be associated with high-output cardiac failure prenatally or
immediately after birth. Fetuses with these lesions require close prenatal
follow-up with assessment by high-resolution ultrasound often twice a week
to monitor combined cardiac output and for signs of hydrops fetalis.

■ If the SCT has a significant solid external component and there are signs of
hydrops or early heart failure, the fetuses may be a candidate for debulking of
the external component of the SCT. Depending on the gestational age of the
fetus, this may occur as a cesarean delivery with immediate debulking after
birth or open fetal surgery.2,6
■ The techniques of open fetal surgery are beyond the scope of this chapter.
■ Surgical debulking includes the following:
■ A posterior approach to the tumor
■ The anus is identified and a Hegar dilator or red rubber catheter placed to
assess the tract of the rectum.
■ The skin at the base of the external component of the SCT is scored with a
Bovie.
■ A tourniquet consisting of an umbilical tie, and a cut large red rubber
catheter is placed at the base of the SCT where the skin had been scored.
■ The tourniquet is tightened, and a harmonic scalpel is used to transect the
tumor above the level of (distal to) the tourniquet.
■ The tourniquet is slowly released, and hemostasis is obtained by
oversewing bleeding vessels with a 5-0 Prolene.
■ The skin is reapproximated and sutured over the cut edge of the tumor.
■ The infant is then resuscitated and cared for in the neonatal intensive care
unit.
■ All patients undergoing surgical debulking will require a second operation
to complete the resection of the tumor, including the coccyx, once the
infant is stable and well recovered (usually greater than 6 weeks after
debulking).

TECH FIG 2 • SCT debulking. Large type I SCTs that
threaten hemodynamic stability may require debulking
prior to or immediately after birth. Some of the main
instruments required for debulking (A). The skin of the
tumor is scored with a Bovie near the base of the SCT
(B,C). A tourniquet constructed from an umbilical tie,
and a cut large red rubber catheter is placed around
the base where the skin was scored (D). A harmonic
scalpel is used to transect the tumor above the

tourniquet (E,F). The tourniquet is released and
bleeding vessels oversewn. Note, unlike formal
resection of an SCT, debulking does not remove the
coccyx or intrapelvic/intra-abdominal tumor
components (G). These components will be resected
after recovery and stabilization from debulking.
PEARLS AND PITFALLS
Positioning
and prep
■ If uncertain of need for laparotomy, prep the patient from lower chest
to toes to allow for repositioning during the case without the need for
new prep and drapes.
Technique
■ A Hegar dilator can be used to identify the rectum and avoid damage.
Complete
resection
■ The coccyx must be resected to lower the risk of recurrence.
POSTOPERATIVE CARE
■ Immediate postoperative care
■ The patient should be positioned to avoid pressure on the wound as much as
possible.
■ The wound should be monitored for bleeding, dehiscence, or infection.
■ If the tumor had a significant intrapelvic extension, urinary retention may
occur, requiring a Foley catheter or intermittent catheterization.
■ Follow-up
■ Final pathology should be followed up to rule out malignancy.
• Benign lesions may be managed with surgical resection and observation
alone.
• If malignant elements are noted on histology, a consultation with oncology
may be needed.
■ All patients should be followed long term with rectal examinations and AFP
levels every 3 to 6 months until the age of 3.

• The AFP level should return to normal by 9 months.
• If still elevated after 9 months or a mass is palpated on rectal exam, an MRI
or CT scan must be performed in suspicion of a recurrence.
■ Recurrence
■ Tumor spillage, incomplete resection, and malignant or immature histology
are risk factors for recurrence.
■ If recurrence is noted, a full metastatic workup including a CT chest,
abdomen, pelvis, and laboratory studies are required.
■ Management of recurrence requires re-excision with or without
chemotherapy.
OUTCOMES
■ The survival rate is greater than 90% if SCT is diagnosed on routine prenatal
ultrasound.
■ If the indication for the ultrasound examination was due to another
complication of pregnancy, the mortality rate increases to 60%.
■ If hydrops or placentomegaly is present, the mortality almost reaches 100%2,7
if no intervention performed.
■ Recurrence after resection occurs in about 10% to 15% of all patients with
SCT, as early as 1 month to as far out as 59 months post-op (median 10–16
months).
■ Survival rate is decreased with recurrence: 90% without recurrence and 65%
with recurrence.
COMPLICATIONS
■ Prevalence of anorectal morbidities including severe constipation and fecal
incontinence ranges from 6% to 60% of the patients.
■ Urologic bladder, vesicoureteral reflux, and urinary incontinence can manifest
in 5% to 45% of the patients.
■ Large intrapelvic extension of the tumor is associated with these morbidities.4
REFERENCES
1. Egler RA, Gosiengfiao Y, Russell H, et al. Is surgical resection and
observation sufficient for stage I and II sacrococcygeal germ cell tumors? A
case series and review. Pediatr Blood Cancer. 2016;64(5 suppl).
2. Hedrick HL, Flake AW, Crombleholme TM, et al. Sacrococcygeal teratoma:
prenatal assessment, fetal intervention, and outcome. J Pediatr Surg.

2004;39:430-438.
3. Kremer ME, Derikx JP, Peeters A, et al. Sexual function after treatment for
sacrococcygeal teratoma during childhood. J Pediatr Surg. 2016;51(4
suppl):534-540.
4. Partridge EA, Canning D, Long C, et al. Urologic and anorectal complications
of sacrococcygeal teratomas: prenatal and postnatal predictors. J Pediatr Surg.
2014;49:139-142.
5. Altman RP, Randolph JG, Lilly JR. Sacrococcygeal teratoma: American
Academy of Pediatrics Surgical Section Survey-1973. J Pediatr Surg. 1974;9(3
suppl):389-398.
6. Roybal JL, Moldenhauer JS, Khalek N, et al. Early delivery as an alternative
management strategy for selected high-risk fetal sacrococcygeal teratomas. J
Pediatr Surg. 2011;46:1325-1332.
7. Makin EC, Hyett J, Ade-Ajayi N, et al. Outcome of antenatally diagnosed
sacrococcygeal teratomas: single-center experience (1993-2004). J Pediatr
Surg. 2006;41:383-393.

64
CHAPTER
Section XVII: Abdomen
Prune Belly
Jamie C. Harris and Fizan Abdullah
DEFINITION1,2
■ Prune belly is an extremely rare disease, occurring in 1/35 000 live births. The
majority of patients are male (FIG 1).
■ It is characterized by three components: abdominal wall flaccidity, bilateral
undescended testes, and urologic anomalies.
■ Abdominal wall laxity is associated with increased rates of respiratory
infections, due to a poor cough mechanism, as well as increased incidence of
lordosis.
■ Urinary tract anomalies lead to increased rates of infectious complications as
well as ureteral obstruction; 81% of patients have megaureter.
• Urinary tract abnormalities can include renal dysplasia and ureteral
enlargement.
■ Additionally, both testes are intra-abdominal in the majority of patients.
■ Overall outcome of the children is variable, with some not surviving past the
neonatal period due to complications to some with minimal disease
complications.
DIFFERENTIAL DIAGNOSIS
■ Prune bellylike variant: abdominal flaccidity. However, unlike in prune belly,
the rectus muscles are not involved.
Patient History and Physical Findings3
■ Prenatal history is often unremarkable. However, oligohydramnios may be
present.
■ Physical examination

■ A wrinkled abdomen with more laxity noted in the medial and inferior
regions. This is a distinctive feature of prune belly. There will be redundant
skin over the abdomen.
FIG 1 • This demonstrates the laxity in the abdominal
wall of prune belly.
• As the children age, the abdomen will look more protuberant and less
wrinkled.
■ If portions of the urinary system are massively dilated, these can sometimes
be palpated on abdominal exam.4
■ Scrotal exam will reveal bilateral absence of testes in the majority of cases.
■ Cardiac anomalies can be associated with prune belly; a cardiopulmonary
exam should be included to listen for murmurs.
■ Close monitoring of urine output is important to identify any obstructive
uropathy that may be present. If present, nephrostomy tubes can be considered
for urinary decompression.
IMAGING AND OTHER DIAGNOSTIC STUDIES
■ Prenatal ultrasound
■ Hydroureteronephrosis, bladder distension, oligohydramnios, and
cryptorchidism can be seen on prenatal ultrasound.5
■ Postnatal urinary tract evaluation
■ Serum creatinine measurements

• This will identify any worsening renal function.
• Baseline measurements should be obtained at birth and trended to determine
changes in function, creatinine greater than 0.7 mg/dL has a higher rate of
developing renal failure.6
■ Kidney Ureter Bladder radiograph (KUB):
• Evaluation of the bowel for dilatation can be done using a KUB. Bowel will
extend over the lateral edge of the abdominal wall, which is diagnostic of
prune belly.
• Due to the lack of abdominal wall structure, intestinal malrotation can occur
due to poor fixation to the abdominal wall.4
■ Renal and bladder ultrasound4:
• An ultrasound will demonstrate atresia or absent kidney, hydronephrosis,
megaureter, tortuous ureters, or megabladder.7
■ Voiding cystourethrogram (VCUG):
• This is used for evaluation of the urethra. It can evaluate for vesicoureteral
reflux. If identified, prophylactic antibiotics are indicated.
■ Evaluation for associated anomalies:
■ Echocardiogram: should be considered if murmur is appreciated on physical
exam.
■ Chest radiograph: if signs of respiratory distress are present, a chest
radiograph can identify pneumothorax or pulmonary hypoplasia.
SURGICAL MANAGEMENT3,8
Preoperative Planning
■ Respiratory status should be optimized prior to abdominal reconstruction.
■ Urinalysis without evidence of infection should be done prior to surgery.
■ Nasogastric tube placed prior to operation to decompress intestinal contents.
Positioning
■ The patient is placed in the supine position.
■ The abdomen is prepped with antiseptic.
Approach
■ Two main open exposures have been described for umbilical preservation,
described by Ehrlich and Monfort.

■ The Ehrlich approach8:
■ A midline vertical incision is made from xiphoid to pubis; an inferior
transverse incision is made at the lower end of the incision. The umbilicus is
left on a vascular pedicle.
■ The midline incision is carried down to the peritoneum.
• If a urologic repair is to be performed in the same operation, this will now
take place.
■ The Monfort approach9 (FIG 2):
■ An elliptical incision is used to remove redundant abdominal wall skin from
xiphoid to pubis.
■ The umbilicus is preserved from the full-thickness skin excision with a
second incision to preserve its original location.
■ The skin and subcutaneous tissue is dissected away from the underlying
fascia, exposing the lateral abdominal wall.