Vasoactive intestinal polypeptide-producing ganglioneuromatosis involving the entire colon and rectum.
Vasoactive intestinal polypeptide (VIP) is known to cause the watery diarrhea, hypokalemia, and achlorhydria syndrome. A 14-year-old girl was admitted with a 4-year history of persistent uncontrollable diarrhea and hypokalemia. Computed tomographic evaluation of the neck, chest, and abdomen were normal. Numerous polyps covering the entire colon and rectum were noted on colonoscopy. The serum VIP level was 143 pg/mL. The patient underwent a total proctocolectomy with an ileal-J-pouch. The pathologic examination revealed ganglioneuromatosis. Postoperatively, the symptoms resolved, and the serum VIP level fell to lower than 5 pg/mL. This is an unusual case of the watery diarrhea, hypokalemia, and achlorhydria syndrome caused by ganglioneuromatosis of the entire colon and rectum.
Moon
Suk-Bae
SB
Department of Pediatric Surgery, Seoul National University Children's Hospital, Seoul 110-744, Korea.
Park
Kwi-Won
KW
Jung
Sung-Eun
SE
Lee
Seong-Cheol
SC
D000293:Adolescent; D003110:Colonic Neoplasms / Q000378:metabolism* / Q000601:surgery; D039021:Colonic Pouches; D005260:Female; D005729:Ganglioneuroma / Q000378:metabolism* / Q000601:surgery; D006801:Humans; D016737:Proctocolectomy, Restorative; D012004:Rectal Neoplasms / Q000378:metabolism*; D014660:Vasoactive Intestinal Peptide / Q000097:blood / Q000378:metabolism*
D014660:Vasoactive Intestinal Peptide