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Revista Española de Enfermedades Digestivas

versión impresa ISSN 1130-0108

Rev. esp. enferm. dig. vol.105 no.3 Madrid mar. 2013 



Sigmoid volvulus as a presentation of neuronal intestinal dysplasia type B in an adolescent

Vólvulo sigmoideo como presentación de una displasia neuronal intestinal tipo B en un adolescente



Key words: Neuronal intestinal dysplasia. Sigmoid volvulus. Hirschsprung's disease.

Palabras clave: Displasia neuronal intestinal. Vólvulo sigmoideo. Enfermedad de Hirschsprung.



Dear Editor,

We report a 17-year-old man admitted to emergency department due to abdominal pain and distension. He referred a history of 18 months with constipation. Computerized tomography showed dilatation of the colon and coprostasis. The patient improved after disimpaction with enemas. Colonoscopy and anorectal manometry were reported normal. A full thickness rectal biopsy revealed normal ganglion cells.

The patient was readmitted with a sigmoid volvulus. Sigmoidectomy and a termino-terminal anastomosis (descending colon-rectum) were performed (Figs. 1A and B). Histologically, neuronal intestinal dysplasia type B (NID-B) (NID) was reported (Fig. 1 C). Dehiscence and anastomatic leakage were found along with dilatation of the descending and transverse colon. Subtotal colectomy with a colostomy of the ascending colon and Hartmann's pouch were performed. The resected segments showed NID-B.



NID-B is a hyperplasia of the intestinal submucosal plexus and hyperganglionosis with increased acetylcholinesterase activity in the lamina propria of the mucosa, and the adventitia of the submucosal arteries (1-3). Incidence varies from 0.3 to 60 % depending on the population studied and the diagnostic criteria used (1,2). NID-B presents in children from 6 months to 6 years and rarely occurs in adolescents and adults (1-3). Clinically it is characterized by constipation, ileus, and symptoms that resemble Hirschsprung's disease (HD) (2,3).

The differential diagnosis includes HD, hypoganglionosis of the myenteric plexus and atrophic intestinal desmosis (4). The diagnosis is histological and quantitative (1,4). In more than half of the cases medical treatment is given with the use of laxatives, enemas, or colonic irrigation. Surgery is used in case of complications or in patients who do not respond to medical treatment (1,3,4). Initial surgical treatment failed because NID-B diffusely affects the intestine requiring a wider resection.


Joel Omar Jáquez-Quintana1, José Alberto González-González1, Ana Cecilia Arana-Guajardo2,
Ligia Larralde-Contreras3, Juan Pablo Flores-Gutiérrez3 and Héctor Jesús Maldonado-Garza1

1Service of Gastroenterology, Departments of 2Internal Medicine, and 3Anatomical Pathology and Cytopathology.
University Hospital "Jose E. González". Universidad Autónoma de Nuevo León. Monterrey, NL. Mexico



1. Skába R, Frantlová M, Horák J. Intestinal neuronal dysplasia. Eur J Gastroenterol Hepatol 2006;18:699-701.         [ Links ]

2. Koletzko S, Jesch I, Faus-Kebetaler T, Briner J, Meier-Ruge W, Müntefering H, et al. Rectal biopsy for diagnosis of intestinal neuronal dysplasia in children: a prospective multicentre study on interobserver variation and clinical outcome. Gut 1999;44:853-61.         [ Links ]

3. Simpser E, Kahn E, Kenigsberg K, Duffy L, Markowitz J, Daum F. Neuronal intestinal dysplasia: Quantitative diagnostic criteria and clinical management. J Pediatr Gastroenterol Nutr 1991;12:61-4.         [ Links ]

4. Meier-Ruge WA, Ammann K, Bruder E, Holschneider AM, Schärli AF, Schmittenbecher PP, et al. Updated results on intestinal neuronal dysplasia (IND B). Eur J Pediatr Surg 2004;14:384-91.         [ Links ]

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