CONGENITAL ABDOMINAL WALL DEFECTS

Dr. Ali M AhmadMBBCh, MS, MD, MRCS-Ed, EBPS

Associate Consultant Pediatric Surgery; KAAUH_ PNU

EXOMPHALOSBad baby with good intestine

High incidence of coexisting abnormalities (up to 50%)

Wrap exposed viscera in fresh kitchen wrap (ensure no twist) Place infant in Humid crib Nil orally, NGT & IVF

Explain management plan to parents Get consent for surgery

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EXOMPHALOS

Congenital hernia into the base of the umbilical cord

Caused by incomplete folding of the embryonic disc and failure of the umbilical ring to form normally.

The hernia is covered by fused amniotic membrane and peritoneum.

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EXOMPHALOS

Occasionally, the membrane ruptures before birth and the eviscerated bowel becomes matted and indurated with dense adhesions, due to chemical irritation from feces and urine in the amniotic fluid.

Rupture also may occur during delivery, in which case the bowel appears normal

Intact sac is shiny and translucent but lacks a blood supply and begins to dry out after birth. Within 12 h becomes opaque & later, becomes black, inelastic and desiccated

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EXOMPHALOS

Due to excess insulin-like growth factor during gestation, which leads to organomegaly , exomphalos

A large baby (e.g. 4 kg) with exomphalos and macroglossia is suggestive of the diagnosis.

Postnatal hypoglycemia is transient but dangerous because of the risk of brain damage, which may occur if an immediate infusion of glucose is not provided.

Beckwith – Weidman Syndrome

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EXOMPHALOS RX11ry Surgical repair is the best

If the defect is less than 5 cm The infant is fit

1. Patient condition• Weight• Maturity• Presence of other anomalies

2. Sac condition• intact or ruptured• Defect size • liver herniated/not into the sac

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EXOMPHALOS RX2

• it may be possible to reduce the bowel back into the abdominal cavity (A), but the surgeon is concerned about worsening pulmonary function if the fascia and skin are closed.

• Therefore, one technique is to cut a circular piece of Silastic sheeting (B) and place it in the abdomen and on top of the reduced intestine (C).

• Four to 5 days later, after the neonate has become more stable and the pulmonary function has improved, the Silastic sheet is removed and the fascia and the skin are closed (D).

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EXOMPHALOS RX3

(A) it is not unusual for an omphalomesenteric duct remnant to be found

(B) The diverticulum was excised primarily and the fascia and skin closed.

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EXOMPHALOS RX4

• Gradually Reduce the volume of the exomphalos over 7–10 days then the defect repaired surgically

• Side effect: infection around the sutures that anchor the prosthesis to the edge of the defect.

Silo1. large defects2. Ruptured sac with massive

evisceration

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EXOMPHALOS RX5

Painting the Sac with an astringent solution to make it a tough, dry eschar which separates when new skin has covered the area beneath it, after 8–12 weeks.

Use non-toxic substances Once the eschar has formed, and normal

feeding and stools are established, the infant can be managed safely at home

Non-operative management• large defects > 8 cm• or the Patient is Unfit1

GASTROSCHISIS

May result from rupture of a physiological hernia in the cord at 6–10 wks.

Nearly always to the right of the umbilicus Bowel Which become densely matted and

adherent with amniotic (chemical) peritonitis and fibrin from defecation and micturition in utero, particularly during the last trimester.

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BLADDER EXSTROPHY {ECTOPIA VESICAE}

Failure of fusion of the lower abdominal wall

Pubic rami not fuse and widely separated.

Ureters protrude from exposed bladder and dribble urine.

Urethra is exposed as a flat strip and the bladder outlet sphincters are not functional.

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BLADDER EXSTROPHY {ECTOPIA VESICAE}

Incidence of 1:40,000 births

Reconstructive surgery to close the bladder and abdominal wall with restoration of bladder sphincters is one of the greatest challenges of pediatric urology

An even rarer variant of bladder exstrophy is known as cloacal exstrophy. In this condition, the shortened proximal colon is fused onto the bladder exstrophy, and there may be an imperforate anus

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CONGENITAL ABDOMINAL WALL DEFECTS

Dr. Ali M AhmadMBBCh, MS, MD, MRCS-Ed, EBPS

Associate Consultant Pediatric Surgery; KAAUH_ PNU

Thank You