ANTERIOR ABDOMINAL
WALL DEFECTS
PRESENTERS; ARINDA NORMAN MChBV
KYAKUNZIRE FAITH MBChBV
MODERATOR; DR. PHYLLIS KISA
OUTLINE
Embryology of the anterior abdominal wall
Omphalocele
Gastrochisis
Other abdominal defects
Embryology
• Abdominal wall is formed from cephalic,
caudal, right and left lateral embryological
folds.
• They join to form the umbilical ring that
surrounds the two umbilical arteries, the
vein and the yolk sac.
• They are covered by an outer layer of
amnion and together form the umbilical cord
• Between the fifth and tenth weeks, there is
physiological herniation of the intestinal
tract due to rapid growth.
• The intestine gradually returns to
abdominal cavity as development is
completed.
• The process is completed with the
contraction of the umbilical rings.
• failure of closure of caudal fold leads to
exstrophy of the bladder and in extreme
cases cloacal exstrophy
• interruption of central migration of lateral
folds leads to omphalocele
OMPHALOCELE
• 1634 - Ambroise Paré (French barber
surgeon) first described Omphalocele.
• Derived from Latin word “Omphalos”
meaning prominence or navel.
OMPHALOCELE
• Congenital defect of the abdominal wall in
which the bowel and solid viscera are
covered by peritoneum and amniotic
membrane
• Due to failure of part or whole of the gut to
return to the coelomic cavity in early fetal
life
• General incidence is 1 in 4000 live births.
It may be associated with other anomalies
including:
•Cardiac abnormalities in 50%
•Neural tube defects in 40%
•Chromosomal abnormalities( trisomy 13,
18, 21 and 15) in 15%
•Exstrophy of the cloaca
•Beckwith-Wiedemann syndrome
Risk factors
• Increased maternal age
• Twins
• High gravida
• Consecutive children
Signs and symptoms
• Central defect of the abdominal wall
beneath the umbilical ring. Defect may be 2-
12 cm (Small-<5cm)(Large>8cm)
• Always covered by sac
Sac is made of amnion, Wharton’s jelly
andperitoneum
Signs and symptoms
• The umbilical cord inserts directly into the
sac in an apical or lateral position.
• Small contains intestinal loops only. Large
may involve liver, spleen and bladder,
testes/ovary
• >50% have associated anomalies
Omphalocele
Diagnosis
• Elevated maternal AFP – Elevated in
90%. 40% false positive rate.
• Fetal ultrasound after 14 weeks
gestation is the confirmatory test.
Shows umbilical cord insertion typically mid-
line on the mass; centrally located
• Contents include intestinal lops, liver,
spleen and gonads.
Pre-operative management
• ABC
• Temperature control
• Fluid management
• Nutrition
• Abdominal distention
• Infection control
Conservative management
1. Large omphalocele (10-12cm) apply topi-
cal application -Betadine ointment or sil-
ver
sulfadiazine to the intact sac.”paint and wait”
2. Secondary eschar formation and
granulation.
3. Healing lasts for 12 months then repaired
as ventral hernia.
Definitive management
• o Primary Closure
• Small defects (<4cm)
• excision of the sac and closure
• of the fascia and skin over the
• abdominal contents
• o Mesh patch
• Medium defects (6-8cm)
Post-operative care
• NICU
• Ventilation
• Feeding: Minimal volume
• 48 hours Antibiotics
• Hernia dealt with at 1 year old
Long term outcomes
• Small omphaloceles - recover well
– Gastro-oesophageal reflux - 43%
With majority improving over time
– 20% pulmonary insufficiency
– Respiratory Infections and Asthma
Feeding difficulties; 60% with giant
omphalocele May need gastrostomy for
feeding
– Failure to thrive
GASTROSCHISIS
• Defect in the anterior abdominal wall
through which the intestinal contents freely
protrude and not covered by membrane.
• The defect is located at the junction of the
umbilicus and normal skin. Most com-
monly on the right side of the umbilicus.
Etiology
• Failure of migration and fusion of the
lateral folds of the embryonic disc on the
3rd-4th week of gestation.
• Disruption of right omphalomesenteric A
as mid gut returns to abdomen by the 10th
week causing ischemia of the abdominal
wall and weakness then herniation.
• Rupture of omphalocele
Risk factors
• Young maternal age
• Low gravida
• Prematurity
• Low birth-weight
• secondary to IUGR
Clinical features
• Defect to the right of intact umbilical cord
allowing extrusion of abdominal content
• Umbilical cord arises from normal place in
abdominal wall
• Opening <=5 cm
• No covering sac (never has a sac )
Clinical features
• Evisceration usually only contains
• intestinal loops
• Bowels often thickened, matted and ede-
matous
• 10-15% have associated anomalies
• 40% are premature/SGA
Diagnosis
Prenatal ultrasound
• Normal umbilical cord insertion site
• Small bowel loops in the amniotic cavity
with no membrane over them
• Can include stomach and large bowel
• Majority occur to the right of the umbilical
cord.
Alpha-fetal protein; Elevated in 10%
Management
Pre-operative
• ABC
• Temperature control
• Fluid management and Nutrition
• Abdominal distention
• Infection control
• Baby placed with right side down to
decrease tension on mesenteric vessels
Definitive managent
• Primary closure if bowel easily reduced
• Staged closure Staged silo reduction al-
lows for gradual reduction on a daily basis
in those with thickened/ edematous intest-
ine. Surgical closure then in 1-2 weeks.
Silo fashioning:
• Sac excised and Silo sewn to rectus fas-
cia/full thickness
Post-operative
• NICU
• Feeding delayed, patients require Total
Parenteral Nutrition
• Oral stimulation/suckling reflex
• Broad spectrum antibiotics.
Long term outcomes
• Generally excellent prognosis if no atresia
• NEC:18.5% of neonates more with formula
• Bowel loss - short gut syndrome
• Cryptorchidism: 15-30% due either being
outside/prematurity. Replacement and
orchidopexy by 1 yr
• 60% have psychosocial stress if
umbilicus sacrificed
PRUNE-BELLY SYNDROME
This is a congenital disorder characterized
by
•extremely lax lower abdominal musculature
•dilated urinary tract. Genitourinary mani-
festations include bladder dilation, ureteral
dilatation, vesico-ureteral reflux
•bilateral undescended testes
• More common in males.
• may be associated with comorbidities like
pulmonary hypoplasia and skeletal ab-
normalities( dislocation or dysplasia of the
hip and pectus excavatum)
management
• Orchiopexy(fertility unlikely)
• Abdominal wall
reconstruction(abdominoplasty) at 6-12
months.
BLADDER AND CLOACAL
EXSTROPHY
• Congenital abnormalities resulting from
rapture of the cloacal membrane allowing
externalization of the lower urinary tract
(bladder exstrophy) or both the lower urin-
ary tract and distal gastrointestinal tract
(cloacal exstrophy)
• Bladder exstrophy- when membrane
raptures after complete separation of genito-
urinary and gastrointestinal tracts.
• Cloacal exstrophy- when rapture is before
the descent of the urorectal septum.
work up
• Obtain patient's baseline renal function
prior to surgery.
• Abdominal ultrasound- focus on kidneys
especially for cloacal extsrophy.
management
• Supportive care according to condition
• Place clean plastic wrap over the bladder
plate.
• Prophylactic antibiotics from delivery till
early postoperative period.
• Definitive management is surgical recon-
struction.
surgical techniques used:
•staged functional closure for classic bladder
exstrophy( MSRE)
•complete primary repair for classic bldder
exstrophy(CPRE)
•urinary diversion for classic bladder
exstrophy
•closure for cloacal exstrophy
postoperative care
• Techniques for immobilization like Bryant
traction, external pelvic fixators and spica
casts are used in the hospital for 3 weeks
after bladder closure.
• Kidneys and bladder are drained fully with
multiple catheters in the first week
• Nutritional support for patients with cloacal
exstrophy
Ventral hernias
• A hernia is the bulging of part of the con-
tents of the abdominal cavity through a
weakness in the abdominal wall.
• Ventral hernia occurs along the vertical
center of the abdominal wall.
• Include Epigastric, Umbilical, parastomal,
Spigelian, Lumbar, Incisional hernias and
traumatic hernias.
References
• Medscape
• SRB’s Manual of Surgery
• Rudolph’s pediatrics
• Up to date
