Segmental dilatation of the intestine (SD) is a rare lesion defined as limited bowel dilatation with a three- to fourfold increase in size with an abrupt transition between the normal and dilated bowel and no intrinsic or extrinsic barrier distal to the dilatation. It was first described by Swenson and Rathauser in 1959, and over 100 cases have been reported since then. Several theories were proposed to explain this malformation; however, its cause remains unknown.
Most pediatric cases are discovered in neonatal periods, so the SD cases in neonates were frequently called congenital segmental dilatation (CSD). Neonates with CSD usually present with features of intestinal obstruction within days of birth. Older children present with anemia, hypoproteinemia, malabsorption, and gastrointestinal bleeding. Preoperative diagnosis is sometimes difficult because of the clinical polymorphism and the lack of specificity of radiological investigations. Patients with an unexplained obstructive intestinal pattern are occasionally found at surgical exploration. The usual finding on laparotomy is localized dilatation of an isolated, well-defined segment of bowel with apparently normal bowel proximal and distal to this segment.
The definitive treatment is resection of the dilated segment and anastomosis of the normal segments of intestine. Most patients have an uneventful course after surgical resection, and the prognosis is excellent. In most cases, histology of the resected segment is usually normal. However, some of the cases showed hypertrophied or very thin muscle layer in the involved segment in histopathological evaluation. The dislocation of the myenteric plexus and the ectopic pancreatic or gastric tissues are reported in dilated intestinal segment.
Chadha R, Gupta S, Tanwar US, et al. Congenital pouch colon associated with segmental dilatation of the colon. J Pediatr Surg. 2001;36(10):1593–5.CrossRefPubMedGoogle Scholar
Cheng W, Lui VC, Chen QM, et al. Enteric nervous system, interstitial cells of cajal, and smooth muscle vacuolization in segmental dilatation of jejunum. J Pediatr Surg. 2001;36(1):930–5.CrossRefPubMedGoogle Scholar
Harjai MM, Katiyar A, Negi V, et al. Congenital segmental dilatation of jejunoileal region in a newborn: unusual clinical and radiologic presentation. J Indian Assoc Pediatr Surg. 2010;15(3):96–7.CrossRefPubMedPubMedCentralGoogle Scholar
Helikson MA, Schapiro MB, Garfinkel DT, et al. Congenital segmental dilatation of the colon. J Pediatr Surg. 1982;17:201–2.CrossRefPubMedGoogle Scholar
Heller K, Waag LD, Beyersdorf F. Intestinal duplication-segmental dilatation of intestine: a common genetic complex. Pediatr Surg Int. 1989;4:249–53.CrossRefGoogle Scholar
Hosie S, Lorenz C, Schaible T, et al. Segmental dilatation of the jejunum resembling prenatal volvulus. J Pediatr Surg. 2001;36(6):927–9.CrossRefPubMedGoogle Scholar
Kaiser M, Castellani C, Singer G, et al. Huge congenital segmental dilatation of the sigmoid colon in a neonate: a “rarity to meet” and a “challenge to treat”. Case Rep Pediatr. 2016;2016:9685307. doi:10.1155/2016/9685307.PubMedPubMedCentralGoogle Scholar
Katsura S, Kudo T, Enoki T, et al. Congenital segmental dilatation of the duodenum. Surg Today. 2011;41(3):406–8.CrossRefPubMedGoogle Scholar
Morikawa N, Kuroda T, Honna T, et al. A novel association of duodenal atresia, malrotation, segmental dilatation of the colon, and anorectal malformation. Pediatr Surg Int. 2009;25(11):1003–5.CrossRefPubMedGoogle Scholar
Ojha S, Menon P, Rao KL. Meckel’s diverticulum with segmental dilatation of the ileum: radiographic diagnosis in a neonate. Pediatr Radiol. 2004;34(8):649–51.CrossRefPubMedGoogle Scholar
Okada T, Sasaki F, Honda S, et al. Disorders of interstitial cells of Cajal in a neonate with segmental dilatation of the intestine. J Pediatr Surg. 2010;45(6):11–4.CrossRefGoogle Scholar
Paradiso FV, Coletta R, Olivieri C, et al. Antenatal ultrasonographic features associated with segmental small bowel dilatation: an unusual neonatal condition mimicking congenital small bowel obstruction. Pediatr Neonatol. 2013;54(5):339–43.CrossRefPubMedGoogle Scholar
Park JS, Doh HJ, Park ES, et al. Segmental dilatation of the ileum presenting as a cystic lesion on prenatal ultrasonography in one twin. Pediatr Int. 2010;52(2):337–8.CrossRefPubMedGoogle Scholar
Porreca A, Capobianco A. Terracciano et al. segmental dilatation of the ileum presenting with acute intestinal bleeding. J Pediatr Surg. 2002;37(10):1506–8.CrossRefPubMedGoogle Scholar
Ragavan M, Arunkumar S, Balaji N. Segmental dilatation of near total colon managed by colon preserving surgery. APSP J Case Rep. 2012;3(3):18.PubMedPubMedCentralGoogle Scholar
Rai BK, Mirza B, Hashim I, et al. Varied presentation of congenital segmental dilatation of the intestine in neonates: report of three cases. J Neonatal Surg. 2016;5(4):55.CrossRefPubMedPubMedCentralGoogle Scholar
Rantan SK, Kulsreshtha R, Ratan J. Cystic duplication of the cecum with segmental dilatation of the ileum: report of a case. Surg Today. 2001;31(1):72–5.CrossRefGoogle Scholar
Rathod KJ, Mohd Z, Kanojia R, et al. Segmental ileal dilatation: an unsuspected cause of neonatal intestinal obstruction. Trop Gastroenterol. 2012;33(2):143–6.CrossRefPubMedGoogle Scholar
Taguchi T, Ieiri S, Miyoshi K, et al. The incidence and outcome of allied disorders of Hirschsprung’s disease in Japan: results from a nationwide survey. Asian J Surg. 2017;40(1):29–34.CrossRefPubMedGoogle Scholar
Thambidorai CR, Arief H, Noor Afidah MS. Ileal perforation in segmental intestinal dilatation associated with omphalocoele. Singap Med J. 2009;50(12):412–4.Google Scholar