Abstract
Purpose: The symptoms of patients with allied disorders of Hirschsprung’s disease range from severe ileus symptoms to obstinate constipation. The extensive-type allied disorders of Hirschsprung’s disease has a poor prognosis, is indicated for small intestinal transplantation, and is characterized by continuous or repeated chronic ileus symptoms. Lesions often extend to the entire intestinal tract, and there is no obvious occlusion site. Thus, it is controversial where the intestinal fistula should be constructed for intestinal decompression. Here, we report on a retrospective examination of the enterostomy site, which can decompress the overstretched bowel in allied disorders of Hirschsprung’s disease patients.
Methods: We included 11 cases of the extensive-type allied disorders of Hirschsprung’s disease treated from 1980 to 2014. Twenty-two operations were performed in four cases of chronic idiopathic intestinal pseudo-obstruction (CIIP), four cases of congenital hypoganglionosis (HP), and three cases of megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS).
Results: The functional intestine-height ratios of the CIIP, HP, and MMIHS patients in the presence of postoperative intestinal decompression were 1.63 vs 3.65, 0.8 vs 2.2, and 0.63 vs 2.78, respectively. The recommended cutoff values obtained by ROC curve analysis for CIIP, HP, and MMIHS of nonintestinal decompression were set at 2.75, 0.8, and 0.7, respectively.
Conclusions: The extensive-type allied disorders of Hirschsprung’s disease is an orphan disease, and it was difficult to show statistically significant results. Performing enterostomy on the upper part of the jejunum of height × 0.8 cm from the duodenum in HP and height × 0.7 cm from the duodenum in MMIHS was effective.
