Neonatal bowel obstruction as a result of segmental dilatation of the gut is a very rare disorder with limited published cases [1–3]. Segmental dilatation of the ileum is a rare condition, with only 126 published cases [3]. The anomaly may present as an isolated entity (as it was in our case) or may be associated with other congenital malformations, gastrointestinal bleeding, anemia, abdominal pain, malabsorption, and growth failure in older children [4,5].

Congenital segmental intestinal dilatation was first described by Sjolin and Thoren from the Departments of Pediatrics and Surgery, University Hospital, Uppsala, Sweden, 1962 [7]. The condition is characterized by a sharply defined and markedly dilated segment of the gut flanked by normal caliber of the afferent and efferent bowel. During neonatal period the anomaly presents with acute intestinal obstruction or simulates Hirschsprung disease (vomiting, abdominal distention, constipation), in older infants may manifest with anemia, malabsorption, intestinal bleeding caused by ulceration of heterotopic gastric mucosa, chronic constipation, or features of intermittent intestinal obstruction.

Although the same anomaly may affect small bowel or colon, clinical sings and time of presentation may differ according to the site of involvement. Clinical picture of cases involving the colon is very similar to that of Hirschsprung disease, usually appearing in the infancy or later on.

Segmental intestinal dilatation is an exceptional pathology with an unknown etiology and a misleading clinical presentation. Several theories were proposed to explain this malformation; however, most authors are rather inclined to an embryological theory incriminating an extrinsic intrauterine intestinal compression. Pathological examination shows a bowel wall with all layers as well as a normal ganglion cells. Surgical resection of dilated intestinal segment is curative.

Segmental dilatation of the intestine should be considered as a cause of intestinal obstruction in a neonate with antenatal discovery of intraabdominal cystic lesion. Lack of specific of radiologic findings explains difficulties to postulate an exact preoperative diagnosis, however, favorable evolution after surgical resection of the dilated segment, points out to the diagnosis of the congenital segmental intestinal dilatation.

In our case the emergency surgical abdominal exploration leaded to final solution of the diagnostic dilemma. Routine prenatal sonographic examination at 34 weeks of gestation revealed a thin-walled dilatation of bowel loop which initially raised suspicion of ileal atresia. At birth the newborn passed copious amount of meconium and no clinical signs of intestinal obstruction were observed. Initial postnatal X-ray did not find any signs of intestinal obstruction either. Oral feeding was started and the neonate was discharged home. Poor feeding and abdominal distension had developed later and the neonate had to be admitted to the hospital nine days after delivery. Subsequent contrast X-ray found dilated segment of jejunum and surgical exploration was recommended. During the surgery a dilated, sharply demarcated, thin-walled segment of the small bowel was found and resected. Histological examination found normal mucous membrane and ganglion cells within the dilated segment.

The case represents a rare neonatal functional intestinal obstruction of unknown etiology. The anomaly often presents itself as an isolated dilated small bowel segment, without evidence of intrinsic or extrinsic cause of obstruction or abnormal neurologic innervations.

Mathé JC et al [8] in their study of the myenteric plexus with a silver staining preparation, explains the anomaly as a consequence of reduction in the number of argyrophil neurons and irregular and altered neuron cell bodies.

References

1. Saha S, Konar H, Chatterjee P, Basu KS, Chatterjee N, Thakur SB, Dasgupta A. Segmental ileal obstruction in neonates--a rare entity. J Pediatr Surg. 2009 Sep;44(9):1827-30.

2. Ojha S, Menon P, Rao KL. Meckel's diverticulum with segmental dilatation of the ileum: radiographic diagnosis in a neonate. Pediatr Radiol. 2004 Aug;34(8):649-51.

3. Waters KJ, Levine D, Lee EY, Buonomo C, Buchmiller TL. Segmental dilatation of the ileum: diagnostic clarification by prenatal and postnatal imaging. J Ultrasound Med. 2007 Sep;26(9):1251-6.

4. Shah AD, Kovanlikaya A, Beneck D, Spigland N, Brill PW. Segmental dilatation of the ileum in a healthy adolescent. Pediatr Radiol. 2009 Dec;39(12):1350-3.

5. Eradi B, Menon P, Rao KL, Thapa BR, Nagi B. Segmental dilatation of ileum: An unusual cause of severe malnutrition. Pediatr Surg Int. 2005;21:405–6.

6. Herlinger H, Maglinte DD. Philadelphia, PA: WB Saunders Co; 1989. Congenital and developmental anomalies in adolescents and adults.Clinical Radiology of the Small Intestine; pp. 249–73.

7. Sjolin S, Thoren L. Segmental dilatation of the small intestine. Arch Dis Child. 1962 Aug;37:422-4.

8. Mathé JC, Khairallah S, Phat Vuoung NP, Boccon-Gibod L, Rey A, Costil J. Segmental dilatation of the ileum in a neonate. Study of the myenteric plexus with a silver staining preparation. Nouv Presse Med. 1982 Jan 30;11(4):265-6. French.