Skip segment Hirschsprung’s disease: diagnostic clues and surgical management
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In brief
In brief
This case series describes diagnostic and surgical approaches for skip segment Hirschsprung's disease, a rare variant with discontinuous aganglionosis. Barium enema showing segmental narrowing combined with laparoscopic-guided multipoint biopsy enables accurate diagnosis, while laparoscopic-assisted pull-through with radical resection provides safe and effective treatment.
Written by the GCMD Library team from the article.
Abstract
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.