Skip segment Hirschsprung’s disease: diagnostic clues and surgical management

PEDIATRIC SURGERY INTERNATIONAL(2021)

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摘要
It is challenging to establish a definitive diagnosis and initiate timely management for skip segment Hirschsprung’s disease (SSHD). Herein, we report three cases of SSHD at our institution between December 2008 and March 2018. Patient #1 was misdiagnosed and underwent three successive operations within 2 years. Patient #2 and #3 were diagnosed timely based on previous experience. A segmental narrowing detected by barium enema may raise the possibility of SSHD. Laparoscopic-guided multipoint biopsy is regarded as a mini-invasive and purposeful way to take representative samples after locating the segmental narrowing intraoperatively and accurate the final diagnosis. The laparoscopic-assisted pull-through procedure with radical resection from the distal rectum to the most proximal margin of the diseased skip segment is safe and effective in treating patients with SSHD. Level of evidence : Level III.
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关键词
Skip segment&#160, Hirschsprung&#8217, s disease, Zonal aganglionosis, Segmental aganglionosis, Hirschsprung&#8217, s disease
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