Jiang Meng, Li Chang-Li, Cao Guo-Qin, Tang Shao-Tao
1 Department of Emergency Medicine, Nanfang Hospital, Southern Medical University, Guangzhou, China.
2 Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
J Laparoendosc Adv Surg Tech A. 2019 Mar;29(3):424-429. doi: 10.1089/lap.2018.0551. Epub 2018 Nov 21.
Reoperations in Hirschsprung disease (HD) may be required due to pathological problems. We conducted this retrospective study to determine the incidence and outcomes of innervation disorders among HD patients following pull-through operation.
We retrospectively reviewed the data of patients who underwent pull-through procedure from 2005 to 2017. Only patients who underwent reoperation due to histological disorders were analyzed. Patients with mechanical obstruction that caused recurrent constipation were excluded.
There were 836 patients who got treated for HD in our department during the study period, and of these, 72 (8.6%) had redo operation. Thirty-one out of the 72 patients (43.1%) showed abnormal histological findings on full-thickness biopsies. Primary operations included totally transanal endorectal pull-through (18), laparoscopic-assisted Duhamel (5) and Soave (8) techniques. The full-thickness biopsies before the reoperation showed aganglionosis (n = 8, 1 was residual resulted from false-positive intraoperative frozen sections and 7 were acquired aganglionosis), transition-zone (3), intestinal neuronal dysplasia B (IND B, n = 15), and hypoganglionosis (5). The final diagnoses according to the resection specimens of the initial and second operations were HD (11), Hirschsprung-associated IND B (15), and Hirschsprung-associated hypoganglionosis (5). Reoperation consisted of laparoscopic-assisted Duhamel (14) and Soave procedures (17). In these patients, 77.4% had excellent/good bowel function, 16.1% were fair, and 6.5% were poor.
Innervation disorders are still the underlying causes of recurrent constipation in almost half of all HD patients requiring redo pull-through operation. Most patients have a satisfactory outcome after redoing laparoscopic-assisted Duhamel or Soave operation.
由于病理问题，可能需要对先天性巨结肠病（HD）患者进行再次手术。我们开展这项回顾性研究以确定HD患者经拖出式手术治疗后神经支配障碍的发生率及预后情况。
我们回顾性分析了2005年至2017年期间接受拖出式手术患者的数据。仅对因组织学异常而接受再次手术的患者进行分析。排除因机械性梗阻导致反复便秘的患者。
研究期间，我院有836例HD患者接受治疗，其中72例（8.6%）接受了再次手术。72例患者中有31例（43.1%）全层活检显示组织学异常。初次手术包括全经肛门直肠内拖出术（18例）、腹腔镜辅助Duhamel术（5例）和Soave术（8例）。再次手术前的全层活检显示无神经节细胞症（n = 8，1例为术中冰冻切片假阳性导致的残留无神经节细胞症，7例为获得性无神经节细胞症）、移行区病变（3例）、B型肠道神经元发育异常（IND B，n = 15）和神经节细胞减少症（5例）。根据初次手术和二次手术切除标本的最终诊断为HD（11例）、先天性巨结肠相关IND B（15例）和先天性巨结肠相关神经节细胞减少症（5例）。再次手术包括腹腔镜辅助Duhamel术（14例）和Soave术（17例）。这些患者中，77.4%的肠道功能为优/良，16.1%为中等，6.5%为差。
在几乎一半需要再次进行拖出式手术的HD患者中，神经支配障碍仍是反复便秘的潜在原因。大多数患者在再次进行腹腔镜辅助Duhamel术或Soave术后预后良好。
