Regional cluster of vanishing gastroschisis: A comparative study of antenatal and post-natal outcomes.

AIM

Vanishing gastroschisis describes the in utero spontaneous closure of the periumbilical defect. It is usually associated with intestinal loss due to ischaemia, necrosis and atresia. This comparative study aims to investigate the spectrum of pathology, antenatal ultrasound characteristics and post-natal outcomes.

METHODS

Our tertiary centre provides antenatal and post-natal care of major congenital anomalies for a population of 1.6 million. Medical records were retrospectively evaluated for all cases of vanishing gastroschisis from May 2014 to May 2015. Cases of normal variant gastroschisis born during the same period were used for comparison. Maximum antenatael bowel diameter measurements were compared using the Mann-Whitney U-test.

RESULTS

Six infants with vanishing gastroschisis were born during the study period, representing 50% of all live-born gastroschisis. Antenatal ultrasound showed progressively increasing intra-abdominal bowel dilatation, with antenatal intra-abdominal bowel diameter significantly greater in vanishing, than normal, variant gastroschisis (23.2 vs. 4.1 mm, P < 0.01). The classification of vanishing gastroschisis severity comprised two type I, three type II and one type III cases. Complete midgut atresia affected three infants, leading to overall mortality of 50% for the vanishing gastroschisis group versus 0% in the normal variant group (P = 0.05).

CONCLUSION

Vanishing gastroschisis is a severe, often catastrophic variant of gastroschisis. Aetiological factors contributing to the recent high incidence of this rare complication in our population of newborns remain unknown, prompting secondary prevention strategies to salvage the midgut. We propose closer antenatal surveillance for fetuses with intra-abdominal bowel dilatation >10 mm to prompt consideration of earlier delivery to improve morbidity and mortality.