Uncommon causes of postoperative chronic diarrhoea mimicking enterocolitis in Hirschsprung’s disease: is there a role for digestive endoscopy?

Abstract

Severe chronic diarrhoea secondary to enterocolitis is a severe complication of Hirschsprung's disease (HSCR). Persistent outlet obstruction, immunologic issues, and mucin/mucous imbalance can cooperate in the development of this complication. Furthermore, isolated reports described severe postoperative chronic diarrhoea mimicking enterocolitis in patients with sucrase-isomaltase deficiency, inflammatory bowel disease (IBD), or intestinal microvillus atrophy. This paper is aimed in describing three patients from our HSCR series who experienced severe chronic postoperative diarrhoea secondary to such uncommon associated anomalies: sucrase-isomaltase deficiency (one patient) and IBD (two patients). With an appropriate sucrose-free diet or immunosuppressive therapy these patients improved dramatically and their diarrhoea settled. These associated anomalies can be diagnosed with digestive endoscopies (both gastro-duodenoscopy and colonoscopy). Therefore, we developed a diagnostic and therapeutic algorithm for patients with chronic diarrhoea after a pull-through, which includes digestive endoscopy to be performed in selected cases.

Redo surgery in Hirschsprung disease: what did we learn? Unicentric experience on 70 patients

“…2. Alternatively, a dissection carried out not enough from above (classic open procedure) or started too high from Table 4 Algorithm for the diagnosis and treatment of patients with complicated HSCR, derived from what already suggested in patients with persistent enterocolitis [33] In case full-thickness biopsy results turn normal and conservative management fails, one should consider the possibility for a chronic intestinal pseudoobstruction and should be managed by a multidisciplinary team. Depending on symptoms, these patients can eventually require a definitive ileostomy.…”

White paper on guidelines concerning enteric nervous system stem cell therapy for enteric neuropathies

“…Short-segment HSCR, in which the rectosigmoid colon lacks ganglion cells, affects 80% of patients, while the remainder have more extensive aganglionosis proximal to the rectosigmoid. Current treatment involves surgical removal of the aganglionic segment, but functional outcome is variable and many patients suffer life-long complications (Conway et al, 2007; Laughlin et al, 2012; Ludman et al, 2002; Pini Prato et al, 2008; Tsuji et al, 1999). This may reflect dysfunction of the so-called “normo-ganglionic” segment (Di Lorenzo et al, 2000; Kohno et al, 2007), abnormal anal sphincter function, retention of aganglionic distal bowel, or the sequelae of proctectomy.…”