[size=20px]A newborn with gastroschisis is noted at primary closure to have abnormal intestinal rotation with a duodenojejunal junction to the right of the midline.

 

Operative treatment for this apparent malrotation with gastroschisis should consist of

 

A closure only.

 

B closure only with upper gastrointestinal series at three-month follow-up.

 

C Ladd procedure and closure.

 

D Ladd procedure without appendectomy and closure.[/size]

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A closure only if the baby is not symptomatic of malrotation and will keep the baby under follow up.

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A

Correct Answer

A closure only.

 

The disruption of normal embryologic rotation and fixation of the intestine caused by an associated congenital abdominal wall defect explains the association between malrotation with gastroschisis and omphalocele. However, the overall rate of malrotation and the proportion of patients who develop midgut volvulus is difficult to ascertain. Those patients with a malpositioned duodenojejunal flexure and a narrow based small bowel mesentery, are at the greatest risk for volvulus.

 

The largest series of patients to address this question is a combined institutional series of 414 patients: 299 (72%) had gastroschisis. A total of eight (1.9%) patients developed a midgut volvulus. The rates of occurrence were higher for omphalocele patients (4.4%) compared to gastroschisis (1.0%). Within the omphalocele group, there was an equal distribution of volvulus occurrence amongst patients with small (40%) and giant (60%) omphaloceles. The median time to volvulus across the entire group was 1098 days (range 21 to 2285).

 

Gastroschisis patients with malrotation may be somewhat protected from the development of a volvulus due to the adhesions arising from bowel inflammation. This may at least partly explain the lower rates of volvulus in gastroschisis compared to omphalocele patients where intestinal adhesions would be expected to be minimal. Therefore it would be reasonable for patients with omphalocele amenable to primary closure who are found to have a malpositioned duodenojejunal flexure and narrow mesentery to undergo Ladd procedure at the time of omphalocele closure.