CONGENITAL

ABDOMINAL WALL
MALFORMATIONS
BY GROUP 3
Supervisor Dr. Odongo Charles Newton
Presenters
• ODER STEVEN
• KATO HUSSEIN
• KYESUULUTA BETTY
• ONGWEN EMMANUEL
• EGAU TONNY
Objectives
• Appreciate embryology of body cavity formation
• Describe the different congenital abdominal wall malformation
• Epidemiology and Prognosis
• Management of the different congenital abdominal wall conditions
Review of the embryology of
formation of body cavities
• At the end of the third week, intraembryonic mesoderm differentiates
into paraxial mesoderm, which forms somitomeres and somites that
play a major role in forming the skull and vertebrae
• intermediate mesoderm, which contributes to the urogenital system;
and lateral plate mesoderm, which is involved in forming the body
cavity .
• Clefts appear in the lateral plate mesoderm and coalesce to split the
solid layer into two;
• the parietal (somatic) layer adjacent to the surface ectoderm and
continuous with the extraembryonic parietal mesoderm layer over
the amnion.
• the visceral (splanchnic) layer adjacent to endoderm forming the gut
tube and continuous with the visceral layer of extraembryonic
mesoderm covering the yolk sac.
• The space formed between these two layers forms the primitive body
cavity
• During the fourth week, the sides of the embryo begin to grow
ventrally forming two lateral body wall folds .
• These folds consist of the parietal layer of lateral plate mesoderm,
overlying ectoderm, and cells from adjacent somites that migrate into
the mesoderm layer across the lateral somitic frontier.
• As these folds progress, the endoderm layer also folds ventrally and
closes to form the gut tube .
• By the end of the fourth week, the lateral body wall folds meet in the
midline ,fuse to close the ventral body wall and its aided by growth of
the head and tail regions causing the embryo to curve in fetal
position.
• Closure of the ventral body wall is complete except in the region of
connecting stalk( future umbilical cord).
• Similarly closure of the gut tube is complete except for the connection
from mid-gut region to the vitelline duct.
Omphalocele
• Congenital defect of the abdominal wall in which the bowel and solid
viscera are covered by peritoneum and amniotic membrane .
• varies from small defect or giant omphalocele in which the
abdominal wall defect measures 4 cm or more in diameter.
• Occurs due to failure of return of mid-gut portion that usually
herniates into the umbilical cord between 6th and 10 th week back
into the abdominal cavity.
• it includes a sac consisting of amnion, Wharton’s jelly, and
peritoneum and oftentimes contains the liver.
• associated with malrotation and Meckel’s diverticulum.
Epidemiology
• the prevalence at birth is between 1 in 4000–6000 live births.
• Its associated with Beckwith-Wiedemann syndrome, Lower midline
syndrome, Pentalogy of Cantrell, and trisomies 13–18, and 21.
• More prevalent in males.
pathophysiology
• Theory of failure of the lateral embryonic folds to migrate medially.
The lateral folds fail to meet in the midline resulting into a central
defect that is covered by peritoneum, amnion, and Wharton’s jelly. This
is evidenced by the findings in other conditions associated with
omphalocele.
• abnormal rotation of the midgut. In normal development the intestines
form outside of the fetus followed by rotation in a counter-clockwise
direction forming the duodenal C-loop and fixing the cecum in the right
lower quadrant.
• Failure of this reduction could result in a spectrum of disease including
omphalocele.
Diagnosis
• Ultrasound commonly the 18th week ultrasound. Omphalocele
diameter (OD) is measured compared to head circumference(HC),
abdominal circumference (AC) and femur length(FL).
OD/HC ratio >/= 0.21 and OD/AC >/= 0.26 may indicate increased
postnatal morbidity and mortality.
• Elevated maternal serum levels of alpha fetoprotein( not specific)
• Amniocentesis to diagnose chromosomal abnormalities.
• echocardiogram to rule out a structural cardiac defect.
• Vaginal or Cesarean delivery remains controversial for small
omphaloceles.
• In those with a giant omphalocele, birth dystocia, rupture of the
omphalocele, and trauma to the herniated liver are concerns.
• Cesarean section , if the omphalocele diameter exceeds 5cm or if a
significant portion of liver is within the omphalocele
management
initial resuscitation
• immediate placement of an orogastric or nasogastric tube to
decompress the intestinal tract and prevent intestinal distension.
• Normothermia should be maintained and the sac itself should be
covered with warm saline-soaked gauze immediately after birth to
minimize insensible fluid losses.
• IV access for fluid resuscitation.
• Monitor blood sugars to correct hypoglycemia that may be in neonates
with beck-with wiedemann syndrome.
• Intubation incase of associated pulmonary hypoplasia,
• Once stabilized, the neonate should be thoroughly evaluated for any
other anomalies.
• imaging with abdominal ultrasound and echocardiogram should be
obtained in all neonates with omphalocele.
• Perioperative antibiotics
Operative repair
Surgical options depend on:
 the size of the defect
 the presence and severity of other associated congenital anomalies
 gestational age
the status of the sac (ruptured or not).
 Can be immediate or delayed closure
Immediate closure
• Small omphaloceles in neonates who are clinically stable and acceptable surgical
candidate.
• the sac is typically dissected free from the abdominal wall to mobilize the fascial
edges for closure.
• .Care must be taken when dissecting the membrane adjacent to the liver because
damage to Glissen’s capsule can lead to uncontrollable hemorrhage in the neonate.
• autologous tissue repair or mesh repair is then performed.
Raptured omphalocele;
These are usually large omphaloceles and there fore a silo is placed to protect the
viscera. Later when the baby is physiologically stable, gradual reduction of the
prosthesis is performed.
Delayed closure
• neonates with large, liver-containing defects and neonates with
multiple other anomalies who are critically ill.
• the “paint and wait” method using povidone iodine application twice
daily, which results in formation of an eschar and eventual
epithelialization of the omphalocele sac.
• Silver sulfadiazine and dilute betadine ;most common agents used to
induce scarring and eventual epithelialization of the sac.
• Monitor thyroid hormone levels during early phase of treatment.
(Povidone iodine)
Complications of immediate closure
• uncontrollable hemorrhage(damage to Glissens capsule)
• hypotension( kinking of hepatic veins)
• abdominal compartment syndrome( tight closure)
• Rupture and infection of the sac
• Respiratory distress
Postoperative care
• monitor for signs of abdominal compartment syndrome, refractory
hypotension.
• Enteral feeds can be started when bowel function has returned.
• Parenteral nutrition is typically required while awaiting return of
bowel function and during advancement of feeds.
• Surgical site care .
 Gastroschisis….
• Defect in the anterior abdominal wall through which the intestinal
contents freely protrude, there is no overlying sac, and the size of the
defect is usually <4 cm.

• More common than omphalocele

• The intestines herniate into the umbilical cord region from 6th to 10th
week of gestation due to rapid enlongation and growth of the abdominal
viscera.
 Gastroschisis…..
• Failure of migration of lateral embryonic fold which is more common on the
right side, resulting in a defect to the right of umbilical cord.

• No peritoneal sac covering abdominal contents, sometimes a rind of

inflammatory tissue forms.

• Normal appearance of the intestines, rupture occurred relatively late during

the pregnancy.

• commonly intestine is thick, edematous, discolored, and covered with

exudate, more longstanding process
 Epidemiology
• Approximately 3.5/ 10000
• More common in children from thin women younger the 20 yrs and its
associated with intestinal atresia.
• Incidence in African American while higher in Hispanics

Risk factors
• Young maternal age <20 yr
• Low socioeconomic status
• Low BMI
• Race
• Tobacco use during pregnancy
 Diagnosis
• Prenatal- elevated maternal serum alpha fetoprotein
• Mid-second trimester US; bowel dilatation 10 mm prior to 30 WOG;
detection of free floating loops of intestine in amniotic fluid to the right of
umbilical cord
• Intestinal damage is seen in many cases

• Inflammatory peeling to serositis to intestinal necrosis to perforation

• Thought to result from exposure to the amniotic fluid and constriction

of bowel with lymphatic obstruction at abdominal wall ( volvulus)
Management
• Prenatal

• Perinatal:
Resuscitation and stabilization,
operative repair,
reduction and closure (Primary closure or silo and delayed closure)
Postoperative
 Management…. Prenatal
• Prenatal ultrasound scan after 20 weeks gestation
• The mother of a fetus with gastroschisis is referred for prenatal
consultation.
• Discuss with mother the anticipated prenatal, perinatal and postnatal
course, complications and surgical management.
• Discuss issues with the nurses and social workers: alleviate many concerns
and prepare them for postnatal care.
• Delivery performed at a center with all the resources needed for definitive
care.
• Labor may be spontaneous or induced as close to term as possible.
Cesarean section delivery should be reserved for the usual maternal and
fetal indications the postnatal care.
Management
Resuscitate and stabilize the newborn:
• ABCs, Endotracheal intubation (if indicated)
• IV access….20mls/kg bolus, IV antibiotics (Broad-spectrum antibiotic
coverage)
• Examine bowel for perfusion and perforation.
• Protect exposed bowel, prevent heat and fluid loses by placing a bowel bag
up to axillae and later placement of silastic silo.
• NPO and NGT for gastric decompression and associated ileus.
• IVF resuscitation: may require two to three times usual maintenance
• TPN for nutrition
• Antibiotics are administered while the silo is in place and continued for 24 h
after closure
 Operative management
• Patient and scenario dependent, but primary closure is often successful (50 %
to 80%).
• Goal should be an abdominal compartment pressure of less than 20 mmHg
after reduction of bowel.
• If this is not immediately possible the newborn will remain in the NICU with a
silo protecting the bowel

Reduction and closure:

• Enough intra-abdominal space to allow for complete reduction of intestine.
• The bowel should be inspected for associated atresia.
• If identified, atresia can be repaired at the time of primary closure or, more
likely, a diverting stoma can be created and takedown and anastomosis
undertaken at a later date (6-8 weeks later).
 Primary closure
• Immediate reduction of the herniated viscera to the abdominal cavity and
closure of the defect.

• The first step involves assessing the degree of inflammation and edema and
searching for an atresia.

• Empty the large intestine and rectum off meconium by performing irrigations
with a catheter placed through the anus if necessary.

• The contents are returned to the peritoneal cavity, the defect is closed in a
variety of ways. Fascial closure may be performed followed by skin closure
and umbilical reconstruction by preserving the base of the cord.
 Silo and delayed closure
• Top of the silo is suspended perpendicular to the supine infant and secured
to the top of the warmer.

• Gravity and gentle pressure allow the intestine to fill the abdomen over time
(usually within two weeks).

• The bowel should be examined frequently for perfusion and perforation.

• The bowel is then serially reduced over the next 2–5 days using clamps or
twisting and ligating the silo with an umbilical tie.
 Silo and delayed closure…..
Advantages Disadvantages
• Decreased effects of increased intra- • increased incidence of sepsis
abdominal pressure (Gentle • delay in closure
reduction)
• increased time to initiation of
• Reduced need: feeds.
for mechanical ventilation,
reoperation for intra-abdominal
hypertension,
incidence of necrotizing
enterocolitis
Post-operative
Expect delayed intestinal mobility/ileus.
• TPN will need to be maintained till infant can tolerate mouth.
• Maintain NGT until output decreases
• Feeds should begin once clamping of NGT is successful and does not
result in emesis.
complications
• Bowel obstruction – atresia, stenosis of anastomosis, adhesions.
Short gut syndrome.
• Abnormal intestinal motility – especially with associated atresias and
dilation of bowel.
• cholestasis due to prolonged TPN
• Pneumatosis intestinalis
• Patients with gastroschisis are at risk for NEC.
• Survival rate is 90%.
Differences between gastroschisis and omphalocele

FEATURES OMPHALOCELE GASTROSCHISIS

Size of defect 4cm or greater Less than 4cm

Location Centre Almost always to the right of the umbilicus

Structure Presence of peritoneum and No membrane covering

amniotic membrane

Association with other Frequently (60% to 70%) Rarely ( 10% intestinal atresia)
congenital anomalies

contents Intestine and liver( most cases), Intestine(100%), colon, bladder,

spleen ,colon and urinary gonads( occasionally)
bladder(occasionally)

Frequency/occurence Less common( 1 in 10000) More common(3.5 in 10,000)

Prognosis poor Good

PRUNE BELLY SYNDROME( Eagle-
Barrett syndrome)
• Congenitally rare acquired disorder primarily characterized by clinical
triad; deficient abdominal musculature, cryptorchidism, urinary tract
abnormalities.
• Presentation varies ranging from incompatibility with life to aging
normally and having children own children.
• The severity of renal dysplasia determines the survival and prognosis
• Perinatal mortality ranges btn 10-25% and directly correlates to
severity of pulmonary hypoplasia due to oligohydramnios from
reduced fetal urine production from renal hypoplasia
Etiology
Theory of urethral obstruction malformation complex:
• urethral obstruction during embryological dev’t produces bladder
distention that has secondary effect on dev’t of urinary tract, the
abdominal wall and testicular descent.
• Yolk sac defect
• Another theory points to a defect in lateral plate mesoderm which
embryologically gives rise to the ureters, bladder, prostate, urethra
and gubernaculum
epidemiology
• Incidence of 3.6 to 3.8 per 100,000 live male births.
• More predominant in males
• Less than 5% of those diagnosed are females.
• Female patients will have typical urinary findings and abdominal
appearance characteristic of prune belly syndrome
• Besides the clinical triad, about 75% of children with prune belly
syndrome have abnormalities involving other systems i.e
• Respiratory 58% e.g pulmonary hypoplasia, cardiac 25% e.g PDA,VSD,
ASD, Tetrallogy of Fallot, Git 24% e.g. malrotation of midgut, bowel
atresia, anorectal anomalies, MSK defects 22% e.g scoliosis, talipes
equinovarus and hip dsyplasia
Evaluation
Prenatal second trimester US- maternal oligohydramnios; distended
urinary bladder, dilated ureters, hydronephrosis.
• Deficient or absent abdominal muscle wall
ECHO- Screen for cardiac anomalies
CXR- small under-developed low volume lungs
RFT- To assess renal status
Renal and bladder US- to evaluate nature and severity of urinary tract
dysmorphism
CUG- to assess any presence of vesicoureteric reflux
Treatment/ management
Medical mgt
• Prophylactic antibiotics and elective circumcision to minimize risks of
UTI
Surgical mgt
• Orchiopexy –bilaterally at about 6months, or earlier but weigh against
higher anesthetic risks.
• Urinary tract reconstruction surgeries in patients with recurrent
febrile UTI or progressive renal deterioration.
• Abdominoplasty- to improve muscle tone, cosmetic reasons.
REFFERENCES
• PETER.M et al ,Fundamentals of pediatric surgery second edition
• T.W. Saddler langman’s medical embryology 12th edition
• Christopher P. Coppola et al, Pediatric surgery ,diagnosis and
treatment.
• AJ Pomajzi ,Sankararaman S .Prune Belly Syndrome.In:statPearls
Thank you