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PEDIATRIC SURGERY ISBN: 978-0-323-07255-7Volume 1 9996085473Volume 2 9996085538

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Notices

Knowledge and best practice in this field are constantly changing. As new research and experiencebroaden our understanding, changes in research methods, professional practices, or medical treatmentmay become necessary.Practitioners and researchers must always rely on their own experience and knowledge in evaluating and

using any information, methods, compounds, or experiments described herein. In using such information ormethods they should be mindful of their own safety and the safety of others, including parties for whom theyhave a professional responsibility.With respect to any drug or pharmaceutical products identified, readers are advised to check the most

current information provided (i) on procedures featured or (ii) by the manufacturer of each product to beadministered, to verify the recommended dose or formula, the method and duration of administration,and contraindications. It is the responsibility of practitioners, relying on their own experience and knowledgeof their patients, to make diagnoses, to determine dosages and the best treatment for each individual patient,and to take all appropriate safety precautions.To the fullest extent of the law, neither the Publisher nor the authors, contributors, or editors, assume any

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Library of Congress Cataloging-in-Publication Data

Pediatric surgery. 7th ed. / editor in chief, Arnold G. Coran ; associate editors, N.Scott Adzick . . . [et al.].

p. ; cm.Includes bibliographical references and index.ISBN 978-0-323-07255-7 (2 vol. set : hardcover : alk. paper)I. Coran, Arnold G., 1938- II. Adzick, N. Scott.[DNLM: 1. Surgical Procedures, Operative. 2. Child. 3. Infant. WO 925]

617.98dc23

2011045740

Editor: Judith FletcherDevelopmental Editor: Lisa BarnesPublishing Services Manager: Patricia TannianSenior Project Manager: Claire KramerDesigner: Ellen Zanolle

Printed in the United States of America

Last digit is the print number: 9 8 7 6 5 4 3 2 1

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CHAPTER 75

Congenital Defectsof the AbdominalWallMichael D. Klein

History

Newborns with abdominal wall defects were reported in thefirst century AD by Aulus Cornelius Celsus, a Roman physi-cian, and then by Paulus Aegineta in the fifth century.1

Omphalocele is described in the sixteenth century printedworks of Ambrose Pare,2 and Lycosthenes may have beenthe first to describe gastroschisis at about the same time.3

Taruffi introduced the term gastroschisis in 1894.4 For manyyears gastroschisis was confused with an omphalocele thathad a torn sac.5 Moore and Stokes are credited with limitingit to the specific clinical entity as we understand it today,6

although they give credit to earlier authors who used the term,such as Bernstein,7 but not to the earliest collected series ofMassabuau and Guibal.8

The first successful repair of omphalocele was reportedby Hey in 1802.9 In 1873 Visick described the successfulrepair of gastroschisis.10 Ahlfeld11 in 1899 described paintingan omphalocele sac with alcohol to produce an escharand awaiting contraction and epithelialization. The use ofmercurochrome for painting the sac was popularized by

Max Grob.1214 Toxic effects of mercurochrome were descri-bed later.1517 A modern version was introduced by Ein andShandling, who used an adhesive semipermeable artificialmembrane.18 Olshausen in 1887 first reported skin flap cov-erage of defects after removal of the membrane,19 and Grossfurther demonstrated its effectiveness and popularized the tech-nique.20 Staged closure of the resultant hernia could be difficultbecause of failure of the abdominal cavity to grow without theimpetus of the intestines within it, and because of intestinaladhesions to the skin flaps. For this reason, the skin wassometimes closed over an intact omphalocele sac.21,22

In 1967 Schuster introduced staged reduction of largeomphaloceles with prosthetic material because he notedthat the abdominal cavity did not grow with skin closurealone.23 The fact that no operative technique has achieveduniversal success or acceptance is attested to by the manyingenious methods that continue to be devised including skingrafting,24,25 pneumoperitoneum and tissue expanders tostretch the abdominal wall in preparation for closure,2628

partial hepatectomy,29,30 lateral relaxing incisions in thefascia,31 and division of the rectus abdominis muscles.32

Spectrum of Clinical CongenitalAbdominal Wall Defects

The clinically important defects are all umbilical with intactrectus abdominis muscles (Table 75-1). Omphalocele is alarge defect (>4 cm) covered by amniotic membrane that con-tains midgut and other abdominal organs including the liverand often the spleen and gonad (Fig. 75-1). One unusual formof omphalocele is the cephalic fold defect, or pentalogy ofCantrell, in which the abdominal wall defect is supraumbilicaland the heart is in the sac through a defect in the pericardiumand the central tendon of the diaphragm.33 The other ele-ments of the pentalogy are an intracardiac defect and a sternalcleft. Ectopia cordis thoracis (when the heart is outside thechest with no pericardial covering as opposed to being insidethe omphalocele sac) might be considered a form of a cephalicfold defect (Fig. 75-2).

Another unusual omphalocele is the caudal fold defect,cloacal exstrophy, in which the defect is infraumbilical andaccompanied by exstrophy of the bladder, epispadias, diastasisof the pubic rami, and imperforate anus (see Chapter 120).The ileum prolapses between the two halves of the exstrophiedbladder.

Gastroschisis is less than 4 cm in diameter, has no coveringmembrane, and usually contains only themidgutwith the stom-ach and possibly a gonad. It is almost always to the right of theumbilical cord, although exceptions do occur (Fig. 75-3).34

Occasionally, a skin bridge may be present between the cordand the defect, but the abdominal wall and its muscles arenormal. At birth the bowel can appear perfectly normal, butmore than 20 minutes after birth, the extruded intestinemay be thickened and coveredwith a fibrinous exudatemattedtogether so that individual loops cannot be distinguished.There have been several reports of gastroschisis with a smallremnant of midgut appearing above a defect that has essen-tially closed, most likely caused by antenatal volvulus.35,36

Most authors recognize patients with gastroschisis and anassociated gastrointestinal condition such as atresia, perfora-tion, necrosis, or volvulus as a separate entity with a poorer

973

outcome.37 This is usually called complicated gastroschisis.Gastroschisis in the fetus is probably associated with intrauter-ine distress. Neonates with gastroschisis are more frequentlypremature and commonly have respiratory problems. Eventerm babies with gastroschisis are more likely to be small forgestational age3840 and to have younger mothers.41

Umbilical cord hernia is least common. It is also less than4 cm and contains only the midgut, but it is covered by amembrane (Fig. 75-4). It is often confused with omphalocele.The differences are that it contains only midgut, never liver,

and the abdominal wall above the defect is normal, with therectus muscles meeting in the midline at the xiphoid. Few as-sociated anomalies are reported with this defect. Like all ab-dominal defects in which the midgut has not returned tothe abdominal cavity before birth to allow for rotation and fix-ation, these patients have malrotation, although it is not usu-ally a cause of intestinal obstruction.

Umbilical hernia is distinguished from these anoma-lies by two features: (1) The defect is covered by normalskin, and (2) it is only rarely present at birth, instead usually

TABLE 75-1

Comparison of Congenital Abdominal Wall Defects

Defect Site Sac Contents Frequency Associated Anomalies Outcome

Omphalocelelateral fold Umbilicus Yes Liver, intestine,spleen, gonad

Common Chromosomal, cardiac Good (depending onassociated anomalies)

Omphalocelecephalicfold (pentalogy of Cantrell)

Superiorumbilicus

Yes Liver, intestine Rare Cardiac, sternal cleft, pericardialdefect, central tendon diaphragmdefect

Poor

Omphalocelecaudal fold(cloacal exstrophy)

Inferiorumbilicus

Yes Intestine Rare Bladder exstrophy, imperforate anus,epispadias

Fair

Umbilical cord hernia Umbilicus Yes Intestine Unusual Uncommon Good

Gastroschisis Rightumbilicus

No Intestine Common Intestinal atresia Good

Ectopia cordis thoracis Midlinesternum

No Heart Rare Cardiac Poor

FIGURE 75-1 Omphalocele.

FIGURE 75-2 Ectopia cordis.

FIGURE 75-3 Gastroschisis.

FIGURE 75-4 Umbilical cord hernia.

974 PART VII ABDOMEN

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becoming apparent in the first weeks or months of life. In theliterature before 1970 and in the literature outside pediatric sur-gery even today, these three forms are often confused, particularlyomphalocele/gastroschisis and omphalocele/umbilical cordhernia.

Infants with congenital abdominal wall muscular defi-ciency, or prune-belly, syndrome have all the normal layersof the abdominal wall but little muscle in the loose areolartissue. Much of the morbidity is related to similar musculardeficiency in the genitourinary and gastrointestinal tracts.

Other abdominal wall defects, often incompatible withlife,4244 have been described in humans45,46 including trueabsence of abdominal wall structures with evisceration ofbowel.47,48

Embryology

EMBRYOLOGY OF THE ABDOMINAL WALL

At 3 weeks gestation the flat disk of the embryo develops fourfolds that will enclose the body cavities (Fig. 75-5, A and B).Two lateral folds form the pleuroperitoneal canals once they

meet anteriorly in the midline. The cephalic fold brings downwith it the developing heart, which actually began distal to thebrain, but now takes its place within the anterior chest wall.It also carries the septum transversum, which continuesposteriorly and divides the pleuroperitoneal canals into thepleural and peritoneal cavities. The caudal fold brings withit the developing bladder or allantois, which started off distalto the anus. During this process the gut tube has formed alongthe length of the embryo with a communication at the umbi-licus to the yolk sac; the yolk sac will eventually disappear,sometimes leaving a vitelline duct remnant on the distal ileum.At about 5 weeks gestation, this gut tube elongates and de-velops within the umbilical coelom (Fig. 75-5, C), a cavity inthe body stalk on the anterior surface of the embryo. At about10 weeks gestation the gut returns from the space within theumbilical stalk to the peritoneal cavity and undergoes rotationand fixation.

EMBRYOGENESIS OF THE DEFECTS

Omphalocele represents a failure of the body folds to completetheir journey.49Most omphaloceles are lateral fold defects andare always at the umbilicus. The rectus muscles often insert farapart on the costal margins and for this reason cannot be

B

Amnion

Intestine

Mesentery Pleuroperitoneal space

Yolk sac MidgutA

Amnion

Neural tube

Yolk sac

Heart

D Umbilical arteries

Urachus

Umbilical vein

Coelom

Allantois

Vitelline duct

and vessels

umbilical vein

Resorbed rightUmbilical coelom

Umbilical artery

Cecum

Aorta

C

FIGURE 75-5 Embryology of the abdominal wall. A, Two-week embryo as a flat disk before folding to form the body cavities. B, At 4 weeks the folding iscomplete. The gut tube is about to be pinched off the yolk sac. C, At 6 weeks the elongating midgut enters the umbilical coelom. D, A view from inside theabdominal cavity showing the relatively unsupported right side of the umbilicus as a result of resorption of the right umbilical vein.

975CHAPTER 75 CONGENITAL DEFECTS OF THE ABDOMINAL WALL

brought entirely together in a repair. Whatever the insult maybe that causes it, this aberration occurs early in embryogenesisand is thus likely to affect other organ systems as well, so chil-dren with omphalocele frequently have associated anomalies.Cephalic fold defects result in ectopia cordis or the pentalogyof Cantrell, whereas caudal fold defects cause bladder andcloacal exstrophy. Gastroschisis, antenatal exposure of theviscera, is probably caused by failure of the umbilical coelomto develop.50 The elongating intestine then has no space inwhich to expand and ruptures out the body wall just to theright of the umbilicus, possibly because the right side of theumbilicus is relatively unsupported as a result of resorptionof the right umbilical vein at about 4 weeks gestation(Fig. 75-5, D).51 An alternate explanation that the yolk sacand associated vitelline structures fail to incorporate intothe umbilical cord, thus allowing the midgut to exit the abdo-men at the point they exit, is also reasonable.52 Thus gastro-schisis has no covering membrane. In gastroschisis the bowelis usually thickened, matted, edematous, and covered with afibrinous peel. Some have explained the latter appearance onthe basis of the change in amniotic fluid electrolyte composi-tion with the onset of fetal kidney function.5356 Evidencefrom studies in animals indicates that the peel forms inutero5759 and that it is a postnatal event.60 Some investigatorshave related the condition of the bowel to the presence ofmeconium from the fetus in the amniotic fluid.19,61 In a recentclinical study, neonates delivered with meconium staininghad a fibrinous peel, whereas those without staining hadnone.55 Our clinical observation of more than 50 deliveriesindicates that the appearance of the bowel is most often apostnatal event (Fig. 75-6). At the moment of birth, the bowelin gastroschisis is usually quite normal. Twenty minutes laterit begins to acquire the characteristic changes. These changesmay be due to exposure to air, but more likely they arerelated to mesenteric venous occlusion at the level of theabdominal wall defect with resultant edema and transudationof proteinaceous fluid.

Umbilical cord hernia is a simple failure of the midgut toreturn to the peritoneal cavity at 10 to 12 weeks. Thus thisdefect contains only midgut and is covered by a membrane.Such hernias are much smaller than omphaloceles and havea better outlook. Distinguishing umbilical cord hernia fromomphalocele both embryologically and clinically is important

inmanagingpatientsandreportingresults.Margulies61 first trans-lated the embryologicworkof Pernkopf62 andPolitzer andStern-berg63 into English, which permitted Benson, Penberthy, andHill64 to recognize it as a separate clinical entity.

Alternative explanations for these various defects, espe-cially gastroschisis, have been presented, but none have beengenerally accepted. Some have thought that gastroschisis mustrepresent failure of mesodermalization with actual absenceof abdominal wall components.65,66 Although such anomalieshave been reported, they are usually stillborn monstrosities.Nearly all live-born children with abdominal wall defectshave intact abdominal walls with normal muscle layers. Morerecently there has been speculation that decreased blood flowin the omphalomesenteric artery might be a cause.67 This hasresulted in many studies on the use of vasoconstrictive agentsin the first trimester. Because the omphalomesenteric arterysupplies intestine and not the abdominal wall, it is difficultto see how this has gained such credence.

There are many reports of abdominal wall defects inducedin animals by exogenous agents. In a comprehensive review ofthe literature gastroschisis was induced by 22 teratogens,omphalocele by 9, and umbilical cord hernia by 8.68 Suchstudies raise the issue of whether the apparent increase inincidence of gastroschisis since 1970 might be due to newenvironmental teratogens. Studies of abdominal wall defectsin animals might not necessarily be relevant. One recent exam-ple is the superb work of Brewer and colleagues on the mouseknockout model of congenital abdominal wall defects.69,70

These investigators demonstrate that the AP-2a transcrip-tion factor is important for the normal development of themouse abdominal wall. They provide beautiful illustrationsof the development of the normal abdominal wall and itsabnormal development in the knockout model. Yet theircareful description allows the clinician to conclude that thedefect presented does not represent any of the clinical defectspediatric surgeons see.

Possible causes of abdominal wall defects have also beeninvestigated in clinical material that has recently beenreviewed.71 For gastroschisis, demographic risk factors forwhich there is more than isolated evidence include youngmaternal age, low socioeconomic status, absence of the mater-nal father, poor maternal prenatal care, and primigravidastatus. Nutritional factors are low levels of glutathione anda-carotene, high levels of nitrosamines, absence of supple-mental vitamin intake during pregnancy, folic acid fortifi-cation, and general, poor nutrition. Maternal obesity wasactually found to be protective. Most, but not all, studies showno familial or genetic risk factors. There is little evidence thatliving near chemical plants, farms, landfills, or other specificsites is a risk factor. Vasoconstrictive agents are repeatedlyreported as related risk factors. Many studies confirmed thatuse of illegal drugs including cocaine, methamphetamine,and marijuana during pregnancy was a risk factor. Risk factorsfor omphalocele are different from those for gastroschisis.Demographic factors include both advanced and very youngmaternal age and maternal obesity. Nutritional factors arefailure to use multivitamins during pregnancy (especiallyVitamin B 12), lack of folic acid fortification, and alterationsin glycemic control. No strong evidence is found for medica-tion, illegal drug, or lifestyle factors, althoughmaternal historyof febrile illness and in vitro fertilization do appear to berisk factors.

FIGURE 75-6 Gastroschisis at delivery. The bowel does not appearmatted, edematous, and coated with a fibrinous peel.

976 PART VII ABDOMEN

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GENETICS AND FAMILIAL OCCURRENCE

Although rare, reports of abdominal wall defects, mainlyomphaloceles, occurring in families and even in twins haveoccurred.7274 No specific genes have been identified with gas-troschisis, but omphalocele is associated with chromosomalanomalies (especially Trisomy 18), and candidates have beenreported for specific genes includingPITX2,CDKN1C,MTHFR,and 677C-T (folate). OEIS is closely related to caudal foldomphalocele andmay be related to a specific gene.72Many syn-dromes include abdominal wall defects. Beckwith-Wiedemannsyndrome (congenital abdominal wall defect, macroglossia, andhypoglycemia with a propensity for the development of abdom-inal tumors later in life) is the most common.73,74 It is remark-able that the abdominal wall defect in Beckwith-Wiedemannsyndrome can be any of the three aforementioned entities,and yet we presume that they all have a different embryogenesis.This syndrome also appears to be associated with assistedreproductive technologies.75 Other syndromes that includeomphalocele are displayed in Table 75-2.

Antenatal Considerations

ULTRASOUND

Today, the diagnosis is usually made antenatally by ultrasound(US).76 Omphalocele can be distinguished from gastroschisisby the presence of a sac and from umbilical cord hernia bythe presence of the liver in the defect. The diagnosis allowsfor antenatal counseling, which given the generally goodprognosis, can be reassuring. In a report from 11 Europeanantenatal US registries in 2001, the sensitivity for detectingomphalocele was 75% (range, 25% to 100%), and that forgastroschisis was 83% (18% to 100%).77 The first age at whichomphalocele was detected was 18 6 weeks, and gastro-schisis, 20 7 weeks. Only 41% of fetuses with omphalocelesdetected antenatally were live-born. Twenty-two percent werefetal deaths, and in 37% the pregnancies were terminated.Fifty-nine percent of fetuses with gastroschisis were live-born,with 12% being fetal deaths and 29% terminations. Recentreports show no significant changes.78,79

TABLE 75-2

Omphalocele Syndromes

Name Description Inheritance OMIM #

Shprintzen omphalocele syndrome Malformation syndrome that includes mildlydysmorphic facies, omphalocele, scoliosis,learning disabilities, and pharyngeal andlaryngeal hypoplasia

Autosomal dominant 182210

Omphalocelecleft palate syndrome Lethal syndrome associated with uterusbicornis in one case, uvula duplex andhydrocephalus internus in another,omphalocele, and cleft palate

258320

Beckwith-Wiedemann syndrome(also known as exomphalos-macroglossia-gigantism syndrome[EMG syndrome] and Wiedemann-Beckwith syndrome [WBS])

Pediatric overgrowth disorder involvinga predisposition to tumor development.The clinical presentation is highly variable;some cases lack the hallmark features ofexomphalos, macroglossia, and gigantism.Abdominal wall defects common, as wellas visceromegaly including liver, spleen,pancreas, kidneys, and adrenals

Inheritance of BWS is complex. Possiblepatterns include autosomal dominantinheritance with variable expressivity,contiguous gene duplication at 11p15,and genomic imprinting resulting froma defective or absent copy of thematernally derived gene

130650

Gershoni-Baruch syndrome Large/giant omphalocele containingliver and intestines. Also associated withdiaphragmatic hernia and radial raydefects

Autosomal recessive hypothesis in onecase

609545

C syndrome (Opitz trigonocephalysyndrome, Trigonocephalysyndrome)

Unusual facies, polydactyly, cardiacabnormality, large omphalocele in afew cases

Autosomal recessive mostly, autosomaldominant in a few cases; disruption ofthe CD96 gene involved with encodinga member of the immunoglobulin family

211750

Donnai-Barrow syndrome(Faciooculoacousticorenalsyndrome)

Facial anomalies, ocular anomalies,sensorineural hearing loss, and proteinuria.Some cases include omphalocele as anassociated anomaly

Autosomal recessive; mutation in theLRP2 gene

222448

Thoracoabdominal syndrome (THAS) Diaphragmatic and ventral hernias, hypoplasticlung, cardiac anomalies, cleft palate,omphalocele, sporadic pentalogy of Cantrell

X-linked dominant 313850

Manitoba oculotrichoanal syndrome(MOTA) (Marles syndrome)

Hypertelorism, unilateral eye malformations,aberrant anterolateral scalp hairline, nasaland anal anomalies. Omphalocele notedin several cases

Autosomal recessive 248450

Craniosynostosismental retardationsyndrome of Lin and Gettig

Midline craniosynostosis, agenesis of thecorpus callosum, severe mental retardation,unusual face, contractures, camptodactyly,hypospadias, hypogonadism, smallomphalocele, and multiple small bowelatresias

218649

Continued

977CHAPTER 75 CONGENITAL DEFECTS OF THE ABDOMINAL WALL

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Antenatal US also detects associated anomalies.80 In gastro-schisis these anomalies are usually intestinal atresias.81,82

In omphalocele one study reports a 25% incidence of thedetection of major associated anomalies.83 The frequency ofassociated cardiac anomalies in omphalocele makes antenatalechocardiography helpful.84

Given the poor outcome of all forms of ectopia cordis,termination could be a reasonable alternative when US dem-onstrates the heart outside the chest. Some ultrasonographershave attempted to correlate the bowel problems of gastro-schisis with the antenatal appearance on US,85,86,90 butthis has not been successful in all centers including morerecent experience.82,91,92

Routine use of antenatal US has not been definitivelyshown to improve perinatal morbidity or maternal outcome,although there may be some survival benefit for a fetus witha life-threatening anomaly. There may, however, be a costsavings, if the mother chooses termination of pregnancy.Such has not been the case for either omphalocele or gastro-schisis,93 although termination rates for these treatableanomalies can be high (63% in one study).94 A recent reportdemonstrated that multidisciplinary prenatal care for motherscarrying pregnancies with gastroschisis produced infantswith higher birthweights and greater gestational age, althoughthere was no difference in the outcome for gastroschisis orthe likelihood for a successful vaginal delivery.95

AMNIOTIC FLUID AND SERUM TESTS

Elevated alpha fetoprotein (AFP) in both maternal serum andamniotic fluid and elevated amniotic fluid acetylcholinesterase(AChE) have been correlated with abdominal wall defectswhen there is no myelomeningocele.96 In a study of 23pregnancies with gastroschisis and 17 with omphalocele,second-trimester serum AFP was 9.42 times greater than nor-mal in gastroschisis and 4.18 times normal in omphalocele.97

Another study found elevated amniotic fluid AFP in 100% ofpregnancies with gastroschisis and in only 20% of those withomphalocele. AChE was elevated in 80% of pregnancieswith gastroschisis and 27% of those with omphaloceles.98

Obstetric Delivery

Intuitively, it may seem appropriate to deliver these patientsby cesarean section to avoid injury to the bowel or tearingof the omphalocele sac, and some reports claim a benefitfor cesarean section.99101 There is also, however, a reportof two patients with gastroschisis whose bowel was injuredduring cesarean section delivery.102 The more recent obstetricliterature finds no benefit of cesarean section.103110 Somereports even show no benefit with referral of the mother fordelivery in a pediatric surgery center.111,112 One must con-clude that the mode of delivery is a decision to be made by

TABLE 75-2

Omphalocele Syndromescontd

Name Description Inheritance OMIM #

Chromosome 9p deletion syndrome Trigonocephaly, flattened occiput, prominentforehead, broad flat nasal bridge, antevertednares, malformed external ears, hypertelorism,hypertonia. Omphalocele rare anomaly

158170

PAGOD syndrome (agonadism withmultiple internal malformations)

Agonadism, hypoplasia of the right pulmonaryartery, hypoplasia of the right lung, isolateddextrocardia with complex cardiacmalformations, and diaphragm hernia oromphalocele

202660

Acrocephalopolydactylous dysplasia(Elejalde syndrome)

Excessive birth weight, swollen globularbody with a thick neck, apparently shortlimbs, polydactyly, craniosynostosis withacrocephaly, omphalocele, andabnormal face

Most likely autosomal recessive 200995

Popliteal pterygium syndrome(lethal type) (Bartsocas-Papassyndrome)

Popliteal pterygium with a cord containingnerves and vessels, synostosis of hand and footbones with digital hypoplasia and syndactyly,facial clefts, ankyloblepharon and filiform bandsbetween the jaws, omphalocele, aplasia ofthe urethra

263650

Malpuech facial clefting syndrome(Facial clefting syndrome, gypsytype)

Mental and physical growth retardation,hypertelorism, facial clefting, urogenitalabnormalities, eye abnormalities, hearingloss, omphalocele, caudal appendage,umbilical hernia

Autosomal recessive (kindred highlyinbred)

248340

Cerebrocostomandibular syndrome Severe micrognathia, rib defects, mentalretardation,microcephaly, histologic anomalies,omphalocele

Both autosomal dominant and autosomalrecessive have been described

117650

Fryns syndrome Diaphragmatic hernia, abnormal face, distallimb anomalies*

Autosomal recessive 229850

OEIS complex Omphalocele, bladder exstrophy, imperforateanus, and spinal defects

258040

Data from references 194198.*Omphalocele is not reported in OMIM but is noted in several case reports. The first reference also points to other syndromes not noted by OMIM.

978 PART VII ABDOMEN

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the obstetrician on the basis of obstetric indications, not on thepresence of an abdominal wall defect.

The belief that the condition of the bowel in gastroschisis isdue to a relatively late (33 weeks) intrauterine event has ledsome to recommend preterm delivery.113 Because we thinkthat the condition of the bowel is a postnatal event, we donot believe that it is worth the risks of prematurity. It seemsmost likely that the good results observed with preterm deliv-ery by planned cesarean section are related to the fact that itallows for immediate repair and avoids the venous congestionof the mesentery and its effect on the intestine. Others havealso found no benefit of preterm delivery,99 and a recent studyshows increased morbidity with preterm delivery.114

Antenatal counseling and coordination with the obstetricteam are essential. Because the condition of the bowel in gas-troschisis and distention of the bowel and size of the liver inomphalocele are often related to the time between deliveryand repair, it is important to make arrangements for repairas soon as possible after delivery.72

Clinical Features

INCIDENCE AND ASSOCIATED CONDITIONS

Omphalocele

Before 1970, omphalocele was the most common of theabdominal wall defects; it is now the second after gastro-schisis. The overall incidence is 1 to 2.5 per 5000 live births8

with a male preponderance.115

Conditions associated with omphalocele are listed inTable 75-3. Up to 45% of patients with omphalocele havebeen reported to have a cardiac abnormality including ven-tricular septal defect, atrial septal defect, ectopia cordis, tricus-pid atresia, coarctation of the aorta, and persistent pulmonaryhypertension of the newborn.116 Chromosomal abnormalitiescan be found in up to 20%, and an association with Downsyndrome has also been reported.117 Patients with omphalo-cele are more likely to be large for gestational age (macrosomiaor > 4 kg in birth weight).118 Musculoskeletal and neuraltube defects are also reported in greater than expected inci-dence.119,120 Gastroesophageal reflux is more likely, with43% being affected in one study.121

Gastroschisis

Gastroschisis has become the most common of the abdominalwall defects over the past 30 years.122124 This may be relatedto the increased incidence of prematurity and the increased

survival of premature infants in general, or to the fact that itwas not until the 1970s that the distinction between gastro-schisis and omphalocele was regularly made.44 The incidenceis about 2 to 4.9 per 10,000 live births,122,125127 with a malepreponderance.28,115

The anomalies associated with gastroschisis are usuallyrelated to the midgut, with the most common being intestinalatresia (see Table 75-3).128 In the first year of life infants withgastroschisis are likely to have gastroesophageal reflux (16%)72

and undescended testicle (15%), although the latter oftencorrects spontaneously.129,130Many reports recognize congen-itally short or dysmotile bowel with gastroschisis.131 Althoughneither condition has been quantified in terms of severityor incidence, they are certainly lower than the incidence ofatresia (

effectively holds the intestine in place, and if skin flap closureshould be necessary, the secondary operation will be muchsafer. For primary closure, mattress sutures are placed throughall layers of the abdominal wall except the skin. It is importantto place these sutures through the rectus abdominis musclesand not just through the midline fascia, which may result ina postoperative hernia. It will not be possible to appose therectus muscles in the upper portion of the incision becausethey insert broadly on the costal margin. It is also not neces-sary because the liver fills this space. Turning anterior rectusfascial flaps to cover this defect has been suggested, but wehave not found this to be necessary. It is best to place theabdominal sutures without tying them and then pull alternatesutures to either side to see how the patient will tolerate fascialclosure. If the anesthesiologist can ventilate the patient withpeak inspiratory pressures of less than 25 cm H2O, closureis safe. The skin is closed with a running suture, and in mostpatients this makes a scar that looks like an umbilicus. Severalmethods have been described for performing a cosmeticumbilicoplasty at the initial operation.138141 It often sufficesto close the skin incision in a subcuticular purse-string fashionwith absorbable suture material while taking every second orthird bite to the fascia. However, frequently not enough skinis available for these procedures. In many cases the skinwill have a tenuous vascular supply and appear blanched.Adhesive tape should not be used for a wound dressing.

Other methods have been suggested to determine whetherthe fascial closure is too tight: a saphenous vein intravenousline that will not drip by gravity or intravesical or NG tubepressure greater than 20 cm H2O.

32,142 Splanchnic perfusionpressure (calculated as mean arterial pressure minus intra-abdominal pressure measured either intragastric or intravesi-cal) has been suggested as being an even better predictor.143

If the fascial closure is judged to be too tight, one can consideronly skin closure, but in our experience, if the closure is stilltoo tight after the maneuvers suggested, the skin closure willalso probably be too tight. In this case the viscera can be cov-ered with a prosthetic silo that will allow slow reduction of theabdominal viscera over a 1-week period. The simplest of thesedevices is the preformed Silastic chimney with a spring-loadedring at the bottom, which is placed though the defect beneaththe edges of the abdominal wall (Fig. 75-7).144 The surgeoncan suture this ring to the abdominal wall to prevent the ring

from extruding as pressure is applied to reduce the viscera.Antibiotic ointment is applied around the edges as a dressing.If this device is not available, one can use 0.007-inch-thickDacron-reinforced Silastic. We suture one piece to each sideof the abdominal wall so that the knots are on the outsideand the edges of both the fascia and Silastic face out. We thenseparately suture up the inferior and superior edges and overthe top (Fig. 75-8).

We do not perform a Ladd procedure or appendectomy. If atesticle is present in the sac, it can be placed in the abdominalcavity because in most cases it will be in the scrotum in a year.If an obvious atresia is present, we may bring out a singlestoma through normal abdominal wall or simply leave theatresia in the abdomen and wait at least 6 weeks beforereoperating.

Most pediatric surgeons agree that giant omphalocele is aseparate category of defect that is particularly difficult to treatand which has a much poorer outcome than the usual lateralfold defect.145 As eloquently described by Campos and col-leagues, there is no standard definition of this entity in termsof size or amount of herniated viscera.146 It simply consistsof a degree of visceroabdominal disproportion that defiestreatment with fascial or even skin closure whether appliedprimarily or after a staged closure. A recent report from theChildrens Hospital of Philadelphia147 defines both in uteroand newborn giant omphalocele as one with 75% or moreof the liver in the sac. Unfortunately they did not present atechnique for determining the volume of the liver, so thismay also be open to individual interpretation. It is oftenaccompanied by respiratory symptoms.148 The Philadelphiaseries is valuable in documenting 31 patients in a 6-yearperiod with 25 survivors. Seventy-one percent requiredintubation in the first hour of life, and 40% of survivorshad chronic lung disease. More than half of the survivorshad associated anomalies, more than half had neurodev-elopmental disability at 1 year of age, and three fourths hadfeeding problems.

Giant omphalocele requires some imagination and creati-vity to treat.149 Suggestions have included painting thesac with antiseptic,150 the use of skin flaps with grafting tothe open areas remaining,151 use of tissue expander,152 andsplit-thickness skin grafting.153 We have tried on severaloccasions to use Gore-Tex and then place skin flaps or graftsover the Gore-Tex, with failure on each occasion as a result of

FIGURE 75-7 Gastroschisis with bowel contained in a preformedSilastic silo.

FIGURE 75-8 Gastroschisis with bowel contained in a hand-sewnSilastic silo.

980 PART VII ABDOMEN

sepsis and sloughing. However, it has been possible to useflaps or grafts on the granulating surface that occurs afterremoving the Gore-Tex, and the underlying pseudocapsuleseems to stabilize the abdominal wall. Using an absorbablematerial covered by a VAC sponge dressing is an appealingalternative.154,155 The use of tissue expanders to either expandthe abdominal cavity or obtain more skin in older infants isalso an option.28,156

Postoperative Care With primary closure, most patientsrequire assisted ventilation initially, but over a period of severaldays the abdominal wall accommodates the abdominal con-tents. Intravenous fluids are administered at 150 mL/kg/hr ormore to maintain urine output at 1 mL/kg/hr. A central intrave-nous line is placed for parenteral nutrition. Short-term antibi-otics are administered if primary closure has been performed,but antibiotics are continued until completion of staged clo-sure with a prosthesis. The wound is managed with antibioticointment, and the sutures are removed at 3 weeks. We keepa 10-French sump NG tube on low intermittent suctionuntil there is evidence of bowel function including stooling,decreased distention, and reduced NG tube output. The tubeis then removed, and 12 hours later feedings are begun gradu-ally. It is not unusual for the appearance of bowel function tobe delayed. If intestinal function has not resumed in 3 weeks,a small bowel contrast study may be indicated.

If a prosthesis has been used, it is usually appropriate toinitiate reduction of the abdominal contents on postoperativeday 1. Reduction is accomplished simply by manual manipu-lation, which can then be secured in many ways: applyinglong clamps suspended by umbilical tape to the overheadwarmer, applying a TIA 90 stapler, running a suture backand forth, or using umbilical cord clamps (Fig. 75-9). A par-ticularly ingenious method is a ringer mechanism,157 but it isnot commercially available. Simply tying the prosthetic sacwith umbilical tape leaves a larger circular defect than closingit side to side does. One might consider adding several suturesto each side outside the prosthesis when it is applied. Thesesutures can then be tied once the prosthesis is flat to furtherappose the edges.

Despite Schusters original hypothesis, it is unlikely that theabdominal wall grows during reduction.23 It is more likelythat the edema of the bowel wall resolves and the intestinalcontents are emptied while the abdominal wall is simplystretched.

The several methods of measuring intra-abdominal pres-sure discussed earlier were originally used to monitor primaryclosure and then reduction of a prosthesis. The most impor-tant lesson learned from these studies is that we had beenreducing the abdominal contents too slowly, usually everyother day. It is important to be aggressive. Most often theprosthesis can be removed and the abdominal wall closedin 7 days. If it is not performed by 14 days, the prosthesismay begin to separate from the abdominal wall. Recently, Jonasuggested that active reduction may not be necessary.158 In hissmall series no pressure was applied to the silo, and theabdominal contents spontaneous reduced by day 8.

If skin flaps have been used, the definitive repair can bedone at any time, depending on the patients general condi-tion. If the intestine begins to grow into a large skin sac insteadof stretching the abdominal cavity, an abdominal binder canbe used to direct forces inward. When it is time to bringthe fascia together, the skin can be dissected off the liverwith little trauma other than some mild bleeding that willstop with pressure. If the edges of the muscular abdominalwall cannot be brought together, a prosthetic mesh patchcan be used as long as it can be entirely covered by healthyskin. If a part of it must be left exposed, it is unlikely to beincorporated and will become infected or extruded. If onecan predict that skin flap closure will not be possible initially,it is probably best to avoid dissecting flaps so that that tissuewill be healthier when it is time for the final repair.

If a postoperative hernia occurs, it can usually be repaired at1 year of age without the use of a prosthetic patch. In occasionalpatients the hernia resolves spontaneously.

Ectopia Cordis Thoracis and Cephalic Fold Defect

Operative repair of ectopia cordis thoracis and cephalic folddefects is especially challenging. It is difficult to replace theheart in the thoracic cavity without kinking the great vesselsor the pulmonary veins. Coverage with soft tissue to gain timecan result in cardiac tamponade, so wide intrathoracic dissec-tion around the heart and great vessels has been advocated toincrease the space within the chest. The fascia of the abdom-inal wall is closed once cardiorespiratory stability has beenachieved. In ectopia cordis thoracis, it is also important to pro-vide a rigid covering for the heart. This has been performedwith rib struts between the sternal halves and with prostheticmaterial. Reported repairs of these difficult clinical problemsemphasize creative use of available tissue and individualanatomic features.159162 In the cephalic fold defect (pental-ogy of Cantrell), the sternal cleft and pericardial defect needno special treatment. We have used immediate skin closureof the omphalocele and allowed the patient to grow and havethe intracardiac defect treated later. A Gore-Tex patch canthen be used to close the central tendon of the diaphragmwith no tension so that the heart can extend somewhat intothe abdomen. With growth the patch becomes taut andelevates the heart slowly into the chest, which can grow toreceive it. A valuable recent review of the literature has beenpublished.163

FIGURE 75-9 Reduction of intestine contained in a preformedSilastic silo.

981CHAPTER 75 CONGENITAL DEFECTS OF THE ABDOMINAL WALL

Caudal Fold Defect

This complex problem is more completely dealt with inChapter 120. Initial management might consist of closure ofthe omphalocele and creation of a colostomy. Preservationof intestinal length including salvage of any colon that mightbe attached to the bladder plate before creating a colostomyis especially important. Lund and Woo have reviewed thistopic well.164,165

Gastroschisis

Initial Care Two features of gastroschisis make initial caresomewhat different from that of omphalocele. The patient isfrequently premature, and closer attention must be paid toheat preservation, respiratory support, and the large surfacearea of exposed intestine. The latter is also responsible forincreased fluid needs and heat loss. Perhaps the best way tocontrol this problem is to place most of the infant immediatelyin a plastic drawstring bowel bag to control evaporative heatand fluid loss.166 Because in most cases the intestine will beperfectly normal immediately after delivery whether the deliv-ery is vaginal or cesarean, the faster the bowel can be reduced,the more likely primary closure can be achieved and the lessbowel wall edema and fibrinous coating will accumulate.

Previously we chose to deal with this issue by operatingimmediately after delivery167 because our obstetricians pre-ferred cesarean delivery for all children with abdominal walldefects. As more obstetricians become convinced by the liter-ature that the route of delivery does not affect outcome ingastroschisis, fewer cesarean sections are scheduled. A reviewof our more recent data indicates that rapid transfer from thedelivery room to the operating room (

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Complications

Complications may be related to prematurity (in gastroschisis),associated anomalies (in omphalocele), gastrointestinal tractanomalies (in gastroschisis), and a closure that is too tight.Mention has already been made of the increased fluid needsand delayed recovery of bowel function. It is important tobe prepared to treat the problems of prematurity includingheat loss, respiratory failure, hyperbilirubinemia, hypoglycemiaand hyperglycemia, and hypocalcemia. Inmanaging respiratorydistress it is important to obtain blood for capillary blood gasanalysis from the upper extremities because the lower extrem-ities are likely to be edematous and congested. Patients withgastroschisis and ruptured omphalocele are frequently hypovo-lemic. Philippart and colleagues studied fluid resuscitation inpatients with both gastroschisis and omphalocele by usingmuscle pH as an indication of adequate perfusion and resusci-tation.178 They found that all infants needed at least 25% ofestimated blood volume during surgery (17 to 80 mL/kg in45 to 120 minutes) and required 82 to 312 mL/kg in the first24 hours of life. Urine output is a good monitor of volume inneonates. Treatment of most of the gastrointestinal tract anom-alies can be delayed. In omphalocele, the bowel may be normalenough to sustain an anastomosis, but edema accumulates in alltissues and the liver expands with the length of the operation.

A closure that is too tight can lead to ventilatory compro-mise, decreased venous return and low cardiac output, andoliguria. The remedy is to return the patient to the operatingroom to remove the fascial sutures and perform skin closureonly. One may need to add a prosthesis. If a prosthesis hasbeen used initially, it may be possible to open it at the bedsideand reclose it more loosely. In omphalocele, postoperativemetabolic acidosis can develop as a result of kinking of the he-patic veins from reduction of the liver. In this case, removaland refashioning of the prosthesis may be necessary.

All thesepatients tend tohave a slowonset of bowel function,no matter how quickly the defect is reduced or how normal thebowel appears. Bowel function seems to return faster in patientswith omphalocele than in those with gastroschisis.179

Outcome

SHORT TERM

The survival rate for gastroschisis is 90% in most series.121,180

In a registry study in Texas, the survival rate of infants withgastroschisis was 93% in 1995-1997.181 In a study fromManchester, United Kingdom, a 94% gastroschisis survivalrate was obtained.182Of the seven patients who died, five diedof overwhelming sepsis. Primary fascial closure was achievedin 80%. The median time to feeding was 30 days (range, 5 to60 days), and the median length of stay was 42 days (range, 11to 183 days). Those who required a silo or had associated in-testinal atresia (8 of 91 patients) required more time untilfeeding and had a greater length of stay, but no increased mor-tality. In a 2010 report based on 2490 patients in the PediatricHealth Information System (PHIS) database created by theChild Health Corporation of America (Kansas City, Mo.) theoverall survival was 96.4%. Associated conditions includedcardiovascular 15%, intestinal resection 12.5%, intestinalatresia 11%, ostomy formation 8.3%, and pulmonary 5%.

Survival rates for omphalocele range from 70% to 95%,with most of the mortality being related to the associatedcardiac and chromosomal anomalies.180 In a report fromLos Angeles, there was no difference in mortality from ompha-locele for birth weight, size of the defect, or type of initial clo-sure.183 They also reported no significant change in mortalityfrom 1960-1970 (23%) to 1970-1980, when it was 19%.Mortality was mainly related to associated anomalies. Fortheir patients with gastroschisis, survival rates did increasein the more recent decade to 91%, and mortality was attrib-uted to prematurity, bowel complications, and Candida sepsisassociated with total parenteral nutrition.

Few patients survive any form of ectopia cordis,184,185

but Groner does report one normally active 12-year-oldwho wears a plastic shield to cover the as yet unreconstructedbony defect.186 OGorman and colleagues reported sevenpatients with pentalogy of Cantrell, of whom four survivedand three were free of a ventilator.187 Nearly all patients withcaudal fold defects survive, although bowel and urinary tractfunction vary.165

LONG TERM

In a report from The Netherlands in 2009 on 111 patientswith omphalocele treated between 1971 and 2004, 20% ofthe patients died, almost all related to associated congenitalanomalies.188 Readmission was required at some time in70%. The most frequent later operations were inguinal hernia,tonsillectomy, adenoidectomy, myringotomy tubes, fundopli-cation, bowel obstruction, malrotation, orchidopexy, and cos-metic revision of abdominal scars. Thirty percent of patientswere still taking medication of some sort. Only 3 patients everhad the feeling that omphalocele interfered with their choicesof activities or professions, and 10 reported that omphaloceledid affect some social relationships (teasing).

Davies and Stringer interviewed 25 of 35 patients whounderwent surgery for gastroschisis between 1972 and1984 and survived longer than 1 year.44 Their median agewas 16 years. Ninety-six percent were in good health andexperienced normal growth, and 35% required further sur-gery related to gastroschisis, two for small bowel obstructionand three for scar revision. Fifty-seven percent reportedthat absence of an umbilicus caused them some distressduring childhood. In 25 school-aged children with gastro-schisis reported from Oregon, 7 were held back a grade orenrolled in special classes, but all participated in normalphysical activities.180 Eighty-four percent of these patientsreported normal bowel movements. Those with abdominalcomplaints were usually evaluated as nonspecific or func-tional. Of 22 patients who required bowel resection at theinitial operation, 10 had bowel complications, whereas only2 of the 68 without a bowel resection had such complications.Ten percent of all patients underwent further surgery forabdominal wall hernia, scar revision, or undescended testis.In 2009 the Canadian Paediatric Surgical Network reporteda contemporary survival of 95%.109

In a combined series of omphalocele and gastroschisisreported from Oklahoma City, 94 patients had an 88% sur-vival rate.189 There was long-term follow-up in 61 patientsfor a mean of 14.2 years. Nineteen needed 31 reoperations,mainly for abdominal wall hernia and intestinal atresia. Eightypercent described their quality of life as good, but 40% were

983CHAPTER 75 CONGENITAL DEFECTS OF THE ABDOMINAL WALL

concerned about their height and felt inadequate in sports andsocial activities. Many also expressed concern about theabsence of an umbilicus. Another combined series with amean age at follow-up of 8.8 years was reported by Lindhamfrom Sweden.190 He noted several episodes of bowel obstruc-tion in the first year of life, recurrent abdominal pain withouta specific abnormality, and some concern in girls about thescar and absence of an umbilicus. Growth and development,however, were normal.

In a study from the United Kingdom, patients with gastro-schisis averaged the 32nd percentile for weight at 5 years ofage and the 52nd percentile after that.191 With complicatedgastroschisis (such as gastroschisis associated with intestinalatresia), they reached only the 25th percentile. The groupfrom Stanford192 found that most survivors of omphaloceleand gastroschisis had poor weight gain. None had gastrointes-tinal or metabolic problems at 3 years of age on the basis ofimaging studies, fecal fat excretion, and serum chemistry.One third had IQ lower than 90, and this was related to thelength of hospitalization and nongastrointestinal anomalies.

Koivusalo and colleagues from Finland sent detailed ques-tionnaires to 75 patients older than 17 years with congenitalabdominal wall defects (16 with omphalocele, 11 with gastro-schisis) and received a response from 76%.193 The only illnessfound more frequently than in the general population wasrheumatoid arthritis in 7%. Thirty-seven percent reportedsome morbidity related to the scar and absence of the umbi-licus, 51% had functional gastrointestinal disorders, and 12%had low self-esteem. Still, 88% reported that they were in goodhealth, and their quality of life and educational levels were nodifferent from that of the general population.

Adverse late cardiorespiratory and pulmonary effectsare seldom found in patients with either omphalocele or

gastroschisis.179 Many children do express concern later inlife about the absence of an umbilicus. Although both theshort-term and long-term outlook for patients with ectopiacordis and both cephalic fold and caudal fold omphalocelesis guarded, patients with lateral fold omphalocele, umbilicalcord hernia, and gastroschisis have an excellent survival rateand long-term prognosis. Most problems are related toassociated conditions, not to the abdominal wall defect orits repair.

The complete reference list is available online at www.expertconsult.com.

SUGGESTED READINGS

Boutros J, Regier M, Skarsgard ED. Is timing everything? The influence ofgestational age, birth weight, route, and intent of delivery on outcome ingastroschisis. J Pediatr Surg 2009;44:912.

Coughlin JP, Drucker DE, Jewell MR, et al. Delivery room repair of gastroschisis.Surgery 1993;114:822.

Jona JZ. The gentle touch technique in the treatment of gastroschisis. J PediatrSurg 2003;38:1036.

Lacey SR, Carris LA, Beyer 3rd AJ, Azizkhan RG. Bladder pressure monitoringsignificantly enhances care of infants with abdominal wall defects:A prospective clinical study. J Pediatr Surg 1993;28:1370.

Nichol PF, Hayman A, Pryde PG, et al. Meconium staining of amniotic fluidcorrelates with intestinal peel formation in gastroschisis. Pediatr Surg Int2004;20:211.

Riboh J, Abrajano CT, Garber K, et al. Outcomes of sutureless gastroschisisclosure. J Pediatr Surg 2009;44:1947.

Schlatter M, Norris K, Uitvlugt N, et al. Improved outcomes in the treatment ofgastroschisis using a preformed silo and delayed repair approach. J PediatrSurg 2003;38:459.

Shanske AL, Pande S, Aref K, et al. Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) in triplet pregnancy after IVF and CVS. Birth DefectsRes A Clin Mol Teratol 2003;67:467.

Shaw A. The myth of gastroschisis. J Pediatr Surg 1975;10:235244.

984 PART VII ABDOMEN