J Korean Assoc Pediatr Surg.  1995 Jun;1(1):68-72. 10.13029/jkaps.1995.1.1.68.

Segmental Dilatation of the Sigmoid Colon: A Rare Cause of Chronic Constipation

• Park WH
• Choi SO
• Paik TW
• Lee HJ
• Suh SJ
• Kim SP

• KMID: 2422324
• DOI: http://doi.org/10.13029/jkaps.1995.1.1.68

Abstract

Segmental dilatation of the colon is a very rare disease entity of unknown etiology and may mimic Hirschsprung's disease. It is characterized by dilatation of a segment of the colon of variable length with obstruction due to lack of peristalsis in a normally innervated intestine. Recently authors experienced a case of segmental dilatation of the sigmoid colon in a 6 month-old male, who presented with severe constipation, abdominal distention, and abdominal mass since 2 months of age. Down's syndrome and congenital nystagmus were associated. Barium enema demonstrated focal dilatation of the sigmoid colon, but the rectum and descending colon proximal to the affected colon were of normal caliber. Rectal suction biopsy with acetylcholinesterase staining was normal and anorectal manometry showed normal rectosphincteric reflex. At operation, there was a massively dilated and hypertrophied sigmoid colon with increased tortuous serosal vessels, measuring 15 cm in length and 10 cm in width. Teniae coli were identifiable in the affected segment. Frozen section biopsies at the proximal, affected, and distal colon showed ganglion cells. Descending loop colostomy was constructed initially and segmental resection and end to end colocolostomy were carried out 3 months later. Final histologic examination showed 1) normal colonic mucosa with ganglion cells, 2) prominent submucosal fibrosis and marked muscular hypertrophy, 3) unremarkable acetylcholinesterase activity and immunohistochemical findings against S-100 protein. On 8 months follow-up, he has been doing well and moves bowels 1-2 times daily.

Keyword

Segmental dilatation; Colon; Constipation

MeSH Terms

Acetylcholinesterase
Barium
Biopsy
Colon
Colon, Descending
Colon, Sigmoid*
Colostomy
Constipation*
Dilatation*
Down Syndrome
Enema
Fibrosis
Follow-Up Studies
Frozen Sections
Ganglion Cysts
Hirschsprung Disease
Humans
Hypertrophy
Intestines
Male
Manometry
Mucous Membrane
Nystagmus, Congenital
Peristalsis
Rare Diseases
Rectum
Reflex
S100 Proteins
Suction
Acetylcholinesterase
Barium
S100 Proteins