Demir Mesut, Akin Melih, Kaba Meltem, Genc Nimetullah Mete, Sever Nihat, Karadag Cetin Ali, Dokucu Ali Ihsan
Department of Pediatrics, Sisli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey.
Sisli Etfal Hastan Tip Bul. 2020 Jun 15;54(2):218-221. doi: 10.14744/SEMB.2018.84565. eCollection 2020.
This study aims to evaluate patients who were diagnosed and treated due to Hirschsprung disease (HD) in our clinic.
We retrospectively evaluated the demographic and clinical findings of the patients with HD, who were operated in our clinic between January 2010 and December 2015.
During study period, 28 patients (19 male 9 female) were found to be operated due to HD in our clinic. Mean age was 16.8 months (1-168). "Transanal Endorectal Pull-through (TERPT)" was performed to 20 of them, Duhamell procedure to five and Soave procedure to three of them. TERPT was applied as laparoscopy assisted in four of them and biopsies were taken laparoscopically preoperatively from one of the patients from each group. Soave procedure was performed in three patients; one had anal stenosis and history of recurrent enterocolitis after TERPT procedure and pathologic analysis revealed neuronal intestinal dysplasia and the other one had total colonic HD and performed Soave procedure with colonic patch. Seven (25%) patients had enterocolitis. Frequencies of enterocolitis were three in two patients, two in two patients and one in three patients. Broad spectrum anibiotics and rectal washouts were supplied to these patients. Five of the seven patients with enterocolitis were operated with TERPT; two patients were operated with Duhamell procedure. Only one of them had total colonic HD. Three patients had total colonic HD diagnosis. Two of them were operated with Duhamell-Martin procedure and one was with Soave procedure with colonic patch according to Kimura technique. Anal stenosis developed in two patients after TERPT and treated with dilatations. Soiling rate was 3% (1/28) and this single patient was treated with laxatives and toilet training. Mean duration of hospitalization was 8.75 (2-14) days. Mean length of the removed intestinal segment was 23.6 (5-38) cm. Mean follow-up was for 35.5 (2-56) months. Neither of the patients was followed in the intensive care unit postoperatively nor died.
TERPT procedure win priority in HD, but other procedures keep importance. Recently, laparoscopy-assisted TERPT is preferred in our clinic in HD therapy due to easy biopsy, full exposure to the transitional zone, the advantage of meso preparation of colon and prevention of strained anastomosis.
本研究旨在评估在我院因先天性巨结肠（HD）而接受诊断和治疗的患者。
我们回顾性评估了2010年1月至2015年12月期间在我院接受手术的HD患者的人口统计学和临床资料。
在研究期间，我院共有28例患者（男19例，女9例）因HD接受手术。平均年龄为16.8个月（1 - 168个月）。其中20例行经肛门直肠拖出术（TERPT），5例行Duhamell手术，3例行Soave手术。4例行腹腔镜辅助TERPT，每组各有1例患者术前经腹腔镜取活检。3例行Soave手术；1例在TERPT术后出现肛门狭窄和复发性小肠结肠炎病史，病理分析显示为神经源性肠发育异常，另1例为全结肠型HD，行Soave手术并加用结肠补片。7例（25%）患者发生小肠结肠炎。小肠结肠炎的发生频率为：2例患者发生3次，2例患者发生2次，3例患者发生1次。给予这些患者广谱抗生素及直肠灌洗。7例小肠结肠炎患者中，5例行TERPT手术；2例行Duhamell手术。其中仅1例为全结肠型HD。3例诊断为全结肠型HD。2例行Duhamell - Martin手术，1例根据Kimura技术行Soave手术并加用结肠补片。2例患者TERPT术后出现肛门狭窄，经扩张治疗。污粪率为3%（1/28），该例患者经缓泻剂及排便训练治疗。平均住院时间为8.75天（2 - 14天）。切除肠段的平均长度为23.6 cm（5 - 38 cm）。平均随访时间为35.5个月（2 - 56个月）。术后患者均未入住重症监护病房，也无死亡病例。
TERPT手术在HD治疗中占优先地位，但其他手术也具有重要性。近来，我院在HD治疗中更倾向于采用腹腔镜辅助TERPT，因其便于活检、能充分暴露移行区、具有结肠系膜准备优势及可避免吻合口张力。
