A 1-day-old female weighing 2110 g at 35 weeks gestation was born via caeserean section to a 34-year-old mother (para 2). The patient was admitted to our department with abdominal distention, bilious emesis and failure to pass meconium. Plain abdominal radiograph was suggestive of neonatal intestinal obstruction. Initial management included restoration of adequate body temperature, hydration and electrolyte balance. The neonate underwent an urgent exploratory laparotomy. Mid-ileal volvulus was encountered with ischemic changes of the 15 cm of the involved ileum. There was no evidence of obvious pathologies responsible for volvulus like malrotation, intestinal atresia or congenital bands. The involved ileal segment was resected and end-to-end ileoileal anastomosis was performed. Histopathologic examination revealed an ischemic infarct of resected ileal segment. The postoperative course was uneventful. The baby is gaining weight and doing well and is under our follow-up.

Anomalies of the gut development including a narrow intestinal mesenteric root has long be considered as the major cause of volvulus with catastrophic end result like intestinal necrosis [1]. If whole intestine is involved, massive intesinal necrosis leading to short bowel disease may occur. Of the intestinal volvulus, 80% of cases present during the first year of life and of these 60% are diagnosed in the neonatal period [2,3]. PSIV is the torsion of a segment of small intestine without any other abnormalities. Volvulus without malrotation occurs in 19 to 26% of small bowel volvulus, and PSIV affecting ileum during the neonatal period is extremely rare and usually occurs in preterms [4].

The exact cause of PSIV is not clear. Some possible mechanisms include stasis of the bowel content, long, narrow, band-like mesentery, changes in the intraabdominal pressure and hyper-peristalsis, insufficient fixation of the intestines, immoderate initiation of feedings at an early stage of life and abdominal nursing including abdominal wall massage [5,6]. Our patient did not reveal any of these predisposing factors. The differential diagnosis of PSIV includes NEC, spontaneous intestinal perforation, meconium plug syndrome and ileal atresia.

Majority of the previous reports of children with PSIV include preterm neonates and the occurence of this entity in the neonatal period is extremely rare [5]. Although there no specific clinical findings revealing PSIV, clinical course of PSIV may involve catastrophic results including massive rectal bleeding causing intractable shock state. We did not observe rectal bleeding in our case but there was clinical evidence of sepsis. In a previous report, of the children with intestinal volvulus, ischemic changes of the affected bowel were seen in 90% of the cases without malrotation as compared to 18% incidence in the cases with malrotation [5]. Colon has the role of a cushion and a fixed cecum results in a tight volvulus while a mobile cecum results in a flexible volvulus. The end result is less severe ischemia and delayed necrosis. It should be kept in mind that extensive intestinal necrosis that may occur in volvulus is one of the three common causes of short bowel syndrome together with necrotizing enterocolitis and intestinal atresia. A limited segment of ileum was ischemic in our patient. Abdominal cavity was not contaminated and resection of volvulated ischemic intestinal segment with ileo-ileal anastomosis was required in our case.

In conclusion, diagnosis of PSIV is challenging due to the lack of specific clinical and radiologic findings and confirmation of this disease entity is only possible at laparotomy.

Funding: None; Competing Interest: None stated.

1. Kluth D, Hillen M, Lambrecht W. The principles of normal and abnormal hindgut development. J Pediatr Surg. 1995;30:1143-7.

2. Millar AJ, Rode H, Cywes S. Malrotation and volvulus in infancy and childhood. Semin Pediatr Surg. 2003;12:229-36.

3. Aguayo P, Fraser JD, Ilyas S, St. Peter SD, HolcombIII GW, Ostlie DJ. Laparoscopic management of small bowel obstruction in children. J Laparoendosc Adv Surg Tech. 2011;21:85-8.

4. Kitano Y, Hashizume K, Ohkura M. Segmental small-bowel volvulus not associated with malrotation in childhood. Pediatr Surg Int. 1995;10:335-8.

5. Kurashige T, Matsuyama S. Primary volvulus of the small intestine in infants. Jpn J Surg. 1978;8:228-35.