The patient presented with history of CIP. On laparotomy narrowed distal ileum with proximal dilatation was present. Histopathology revealed intestinal neuronal dysplasia- type B of ileum. Similar findings were also present in the ileal segment found dilated and non-functional during second surgery.
The broad spectrum of pediatric enteric neuropathy with hyperganglionosis includes ganglioneuroma and IND-B. IND-B is considered to be a malformation probably resulting from the incomplete malformation of the submucosal plexus. The hallmark feature being increased “giant” submucosal ganglia (> 8 ganglion cells per ganglion) (1). Ganglioneuroma on the other hand are mucosal polypoid or diffuse types. The diffuse ganglioneuromatosis have hyperplasia of neural and glial elements in both the submucosal and myentric plexuses. They are often associated with multiple endocrine neoplasia type 2b (MEN2b) and neurofibromatosis type 1(NF-1). For pathologists it is important to classify the anatomical different forms of intestinal pseudo-obstruction for the appropriate management and follow-up.
Isolated IND-B presents as a chronic condition with signs and symptoms similar to HD. Findings range from chronic constipation and obstructive signs including abdominal distension and vomiting. Associated anomalies like anal stenosis, intestinal malrotation and malformations have been reported in 30% of cases (3, 4). The diagnostic workup must focus to rule out organic causes of intestinal constipation, particularly HD, the most prevalent intestinal dysganglionosis. However, anorectal manometry and barium enema, established tests for HD screening, are non-specific for IND-B (5). Thus, the diagnosis of IND-B essentially relays on histopathological analyses of rectal biopsies (6).
In recent years, IND-B in adults has shown rising trends. Some of them have experienced symptoms of severe constipation since childhood while others had onset of symptoms at adulthood (2, 7, 8). Our case was a child with symptoms since 4 years of age. Prognosis of IND-B is usually good, but symptoms may progress to serious complications like CIP, acute bowel obstruction or intestinal infarction. The intestinal resections should be the treatment for the refractory cases showing no improvement with at least 6 months of conservative management, or in patients having obstructive complications (1, 9, 10).
The present case showed classic features of IND-B with changes chiefly involving the submucosal plexi with increased density of ganglion cells. The IND-B involving only the ileum is a very rare occurrence (2).
Suspicion for IND-B involving ileum, although rare, should be kept in patients presenting with intestinal obstruction and showing minimal response to conventional treatment.