Intestinal pseudoobstruction caused by a new form of visceral neuropathy: Palliation by radical small bowel resection

Surgical treatment and prognosis of chronic intestinal pseudo-obstruction in children

“…These results are in close agreement with the previous study, reporting that some procedures (Ladd procedure in malrotation and volvulus and Duhamel pull-through procedure) were helpful, but that gastrojejunostomy and resection of the small bowel were not helpful [5,29]. Another author, however, suggested that intractable symptoms might necessitate radical resection of the small bowel [20,30].…”

Chronic Idiopathic Intestinal Pseudo-obstruction Treated by Near Total Small Bowel Resection: A 20-Year Experience

“…Single cases of successful segmental or subtotal small bowel resections were reported in 1961 by Paul,15 in 1985 by Schuffler,16 and in 2003 by Nayci et al 14 These three case reports had 3 ft (90 cm) or more of non-dilated ileum that was anastomosed to a short segment of jejunum so that all these patients could be fed orally and did not need HPN. Schuffler concluded that "radical small bowel resection is not recommended for patients with CIIPO, except in carefully defined, extreme circumstances".…”

“…Schuffler concluded that "radical small bowel resection is not recommended for patients with CIIPO, except in carefully defined, extreme circumstances". 16 In 1988, Mughal and Irving reported on subtotal enterectomy as treatment of end stage of chronic intestinal pseudo-obstruction from different etiologies. 17 In these three patients, a short segment of jejunum was anastomosed to the transverse colon and the longest survival was 2 years.…”

“…However, few authors have recommended more aggressive surgical interventions such as subtotal small bowel resection to relieve the symptoms in end-stage CIIPO. [15][16][17][18] In the mid-eighties, on an emergency basis for an acute hemorrhage from a duodeno-jejunostomy, we operated on a 10-year-old boy suffering from CIIPO and on HPN for 8 years. The bowel was so dilated that we had to resect all but 165 cm of small bowel and most of the colon.…”

Familial visceral neuropathy: A defined entity?

“…Histological investigation in one individual demonstrated neuronal eosinophilic intranuclear inclusions. Review of the English language literature demonstrated seven other families with a very similar phenotype, two of which also had neuronal intranuclear inclusions [Schuffler et al, 1978[Schuffler et al, , 1985Roy et al, 1980;Mayer et al, 1986;Faber et al, 1987;Arrindell et al, 1991;Camilleri et al, 1991;Barnett et al, 1992;Zannolli et al, 2002].…”