This document discusses various abdominal wall defects in infants. It describes omphalocele, where abdominal organs protrude into a sac through a defect in the abdominal wall near the umbilicus. Gastroschisis is defined as a defect to the right of the umbilicus where intestines directly eviscerate. Incidence, pathophysiology, clinical findings, associated anomalies, and treatment approaches like primary closure or staged closure using a silo are reviewed for each condition. Other rare defects discussed include umbilical hernia, prune belly syndrome, bladder exstrophy, pentalogy of Cantrell, and Beckwith-Wiedemann syndrome. Key differences between omphalocele and gastroschis
9. Clinical Findings
• Covered clinical
defect of the umbilical
ring
• Defect may vary from
2-10 cm
• Sac is composed of
amnion, Wharton’s
jelly and peritoneum
10. • 50% have
accompanying liver,
spleen, testes/ovary
• >50% have associated
defects
• Location:
– Epigastric
– Central
– Hypogastric
• Cord attachment is on
the sac
11. • The sac may rupture in utero in 10-18%
or from the delivery process in4%.
• The incidence of associated major
congenital anomalies in up to 81%.
13. Defects
of caudad fold
• imperforate anus
• genitourinary malformations
• bladder or cloacal exstrophy
• colon atresia
• sacral and vertebral anomalies, and
• meningomyelocele.
14.
15. GASTROSCHISIS
• It is the defect in the abdominal wall was
displaced to the right of the umbilicus and
eviscerated bowel was not covered by a
membrane.
17. Incidence
• 1:20,000-30,000
• Sex ratio 1:1
• 10-15% have associated anomalies
• 40% are premature/SGA
18. Pathophysiology
• Abnormal involution of right umbilical vein
• Rupture of a small omphalocoele
• Failure of migration and fusion of the
lateral folds of the embryonic disc on the 3 rd-
4th week of gestation
19.
20. Clinical Findings
• Defect to the right of
an intact umbilical
cord allowing
extrusion of
abdominal content
• Opening ≅ 5 cm
• No covering sac
21. • Bowels often
thickened, matted and
edematous
• 10-15% with intestinal
atresia
22. • Evisceration of the bowel leads to
malrotation.
• Constriction of the base may cause
intestinal stenosis, atresia, and volvulus
• Undescended testicles
• preterm or small for gestational age (SGA)
32. Primary Closure
• In 1967, Schuster technique
• A circumferential incision along the skin-
omphalocele junction; the omphalocele
membrane is left intact
• Teflon sheets
• DualMesh patch (Gore-Tex)
• AlloDerm patch (acellular human dermis)
50. • Have coarse, rounded facial features
• hyperplasia of the pancreatic islet cells
with hypoglycemia; visceromegaly
• genitourinary abnormalities
51. Omphalocoele Gastroschisis
Incidence 1:6,000-10,000 1:20,000-30,000
Delivery Vaginal or CS CS
Covering Sac Present Absent
Size of Defect Small or large Small
Cord Location Onto the sac On abdominal wall
Bowel Normal Edematous, matted
52. Omphalocoele Gastroschisis
Other Organs Liver often out Rare
Prematurity 10-20% 50-60%
IUGR Less common Common
NEC If sac is ruptured 18%
Associated >50% 10-15%
Anomalies
Treatment Often primary Often staged
Prognosis 20%-70% 70-90%
65. Note the bifid genitalia in this baby with cloacal exstrophy.
66. In the repair of cloacal exstrophy, the ileum in the middle of the bifid
bladder is excised and used to create an ostomy, and the bladder halves
are approximated.