Volvulus as a complication of chronic intestinal pseudo-obstruction syndrome

Abstract

Chronic intestinal pseudo-obstruction syndrome (CIPS) is a severe motility disorder of the gastrointestinal tract that presents with continuous or recurrent symptoms and signs of intestinal obstruction without evidence of a structural lesion occluding the intestinal lumen. Mechanical obstruction might occur in these patients as well but is typically difficult to distinguish from an exacerbation of CIPS. We report two pediatric cases in which mechanical obstruction by volvulus mimicked an exacerbation of CIPS, requiring surgical intervention. Conclusion: Awareness of the possibility of true mechanical obstruction in CIPS patients during an exacerbation episode is needed, as this is a severe condition and usually requires surgical intervention.

Myotonic dystrophy type 1 and pseudo-obstruction in a child with smooth muscle α-actin deficiency and eosinophilic myenteric plexitis

“…These abnormalities may have caused volvulus, which is an infrequent complication of CIPO, even though eosinophilic plexitis, which occasionally occurs in Hirschsprung's disease, might have been a secondary phenomenon to the obstruction caused by volvulus (10,11).…”

Chronic intestinal pseudo‐obstruction in children and adults: diagnosis and therapeutic options

“…In children, there is a higher risk of colonic and small bowel volvulus secondary to severe dysmotility and gut dilatation, congenital adhesions or concurrent malrotation . Urological involvement is commonly identified in patients with familial and congenital forms of CIPO, particularly in the myopathic subgroup, ranging from 36% to 100% .…”

Guanylate Cyclase C Activation Shapes the Intestinal Microbiota in Patients with Familial Diarrhea and Increased Susceptibility for Crohnʼs Disease

“…Apart from a freely movable ileocoecum observed during surgery, the tendency to develop volvulus in FGDS is likely explained by GC-C related gut dysmotility, which may predispose for twisting and impair untwisting of the gut. 178,241 Intestinal torsion may eventually lead to compromised blood flow and intestinal ischemia and potentially to a so-called ischemia/reperfusion (I/R) injury. 242 Reperfusion of hypoxic tissues following surgical treatment releases reactive oxygen species and inflammatory mediators creating inflammation and scars locally, and sometimes a systemic inflammatory response with multi-organ failure.…”