Koichiro Yoshimaru, Toshiharu Matsuura, Yasuyuki Uchida, Soichiro Sonoda, Shohei Maeda, Keisuke Kajihara, Yuki Kawano, Takeshi Shirai, Yukihiro Toriigahara, Alvin Santoso Kalim, Xiu-Ying Zhang, Yoshiaki Takahashi, Naonori Kawakubo, Kouji Nagata, Haruyoshi Yamaza, Takayoshi Yamaza, Tomoaki Taguchi, Tatsuro Tajiri
Hirschsprung disease (HSCR) and its associated disorders (AD-HSCR) often result in severe hypoperistalsis caused by enteric neuropathy, mesenchymopathy, and myopathy. Notably, HSCR involving the small intestine, isolated hypoganglionosis, chronic idiopathic intestinal pseudo-obstruction, and megacystis-microcolon-intestinal hypoperistalsis syndrome carry a poor prognosis. Ultimately, small-bowel transplantation (SBTx) is necessary for refractory cases, but it is highly invasive and outcomes are less than optimal, despite advances in surgical techniques and management...
September 5, 2023: Surgery Today