Neuronal intestinal pseudoobstruction
Overview
Intestinal pseudoobstruction is the decreased ability of the intestines to push food through, and often causes dilation of various parts of the bowel. It can be a primary condition (idiopathic or inherited from a parent) or caused by another disease (secondary). The clinical and radiological findings are often similar to true intestinal obstruction.
Causes
There is some evidence of a genetic association.[1] One form has been associated with DXYS154.[2] It can occur in conjunction with Kawasaki disease.[3]
Diagnosis
Attempts must be made to find the underlying cause of intestinal pseudoobstruction. Secondary intestinal pseudoobstruction may be caused by scleroderma (esophageal motility is also impaired), myxedema, amyloidosis, muscular dystrophy, multiple sclerosis, hypokalemia, chronic renal failure, diabetes mellitus, drugs (anticholinergics, opiates) Primary (idiopathic) intestinal pseudoobstruction diagnosed based on motility studies, x-rays, and gastric emptying studies. It may be caused by problems with the smooth muscle of the intestines (hollow visceral myopathy), or may be caused by problems with the nerves that supply the gut.
Treatment
Secondary pseudoobstruction is managed by treating the underlying condition. There is no cure for primary pseudoobstruction. It is important that nutrition and hydration is maintained, and pain relief is given. Drugs that increase the propulsive force of the intestines have been tried, as have different types of surgery. [edit] Medical treatment Metoclopramide, cisapride, and erythromycin may be used, but they have not been shown to have great efficacy. In such cases, treatment is aimed at managing the complications. Intestinal stasis, which may lead to bacterial overgrowth, and, subsequently, diarrhea or malabsorption is treated with antibiotics. Nutritional deficiencies can be treated with oral supplements, and, rarely, total parenteral nutrition. Use of octreotide has been described.[4][5] [edit] Surgical and other procedures Intestinal decompression by colonoscopy or tube placement in a small stoma can also be used to reduce distension and pressure within the gut. The stoma may a gastrostomy, enterostomy or cecostomy, and may also be used to feed or flush the intestines. Colostomy or ileostomy can bypass affected parts if they are distal to (come after) the stoma. For instance, if only the large colon that is affected, an ileostomy may be helpful. Resection of affected parts may be needed if part of the gut dies (for instance toxic megacolon), or if there is a localised area of dysmotility. Gastric and colonic pacemakers have been tried. These are strips placed along the colon which create an electric discharge intended to cause the muscle to contract in a controlled manner. There is also a transplant surgery that could be done. It includes taking out six organs which are the pancreas, stomach, small intestine, liver and duodenum. This surgery has only been performed once by Doctor Kareem Abu-Elmagd on Gretchen Miller, the subject of the Discovery Channel program Surgery Saved My Life.[6]