Inversion of ileum causing intestinal obstruction: rare complication in cloacal exstrophy.

Sir,

Cloacal exstrophy is a congenital developmental anomaly in which two hemibladders are separated by an area of intestine with mucosal surface, in the midline of the lower abdomen. We are reporting an unusual case of a newborn suffering from cloacal exstrophy with intestinal obstruction while awaiting surgical intervention.

A 32 week preterm neonate weighing 1200 g was admitted with a diagnosis of cloacal exstrophy in neonatal surgical intensive care unit (ICU) for work-up and single stage total reconstruction. On examination, there was an omphalocele with intact sac containing small bowel, immediately below was exstrophy of the cloaca with two hemibladder separated by everted cecum and a loop of everted ileum from the summit of the cecal patch. One week post-admission neonate developed abdominal distension with recurrent bilious vomiting. Abdominal examination showed tense, non-tender abdomen with shiny skin and visible intestinal peristalsis. Neonate had stopped passing stool. X-ray abdomen showed dilated small gut in the center of abdomen. The neonate was started on conservative management with intravenous (IV) fluids, IV antibiotics, nasogastric aspiration and other supportive treatment. No improvement seen in the first 24 h, incidentally it was noticed that the everted ileum, the only orifice for stool had inverted [Figure 1]. Bed side eversion of ileum tried but failed and finally a dried gauze piece was inserted into the visible orifice of the ileum on the cecal patch and carefully pulled which helped in the complete eversion of the ileum [Figure 2]. Thereafter the neonate passed a large quantity of stool and over the next 24 h his abdominal distension and vomiting subsided. Feeding started 6 h later.

Figure 1

Inverted ileal segment leading to obstruction

Figure 2

Everted ileal segment

Cloacal exstrophy is a major congenital birth defect and involves several important organ systems. The reported prevalence is 0.25-0.5:10,000.[12] It is commonly associated with other anomalies including cardiovascular, central nervous system, omphalocele (70-90%), vertebral anomalies (46%), upper urinary tract (42%), malrotation (30%), lower extremity anomalies (30%), double appendix (30%), absent appendix (21%), short small bowel (19%), small bowel atresia (5%), abdominal musculature deficiency (1%).[3] Usually, a foreshortened hindgut or cecum ends between the two exstrophied hemibladders. The orifice of the terminal ileum is located on the everted cecum. Neonates of cloacal exstrophy discharge fecal matter from the everted ileum on the patch of caecum.

Though this case report may seem trivial and unimportant, but the crux of the matter is that this neonate remained obstructed for 24 h and actually became very sick. The ileal inversion was serendipitously detected by the ICU resident and with a simple maneuver of eversion, the child passed stool and obstruction got relieved. Hence, it is important to keep this complication in mind while managing neonates with cloacal exstrophy.