Obata Satoshi, Ieiri Satoshi, Akiyama Takashi, Urushihara Naoto, Kawahara Hisayoshi, Kubota Masayuki, Kono Miyuki, Nirasawa Yuji, Honda Shohei, Nio Masaki, Taguchi Tomoaki
Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, Fukuoka, 812-8582, Japan.
Department of Pediatric Surgery, Field of Developmental Medicine, Graduate School of Medical and Dental Sciences, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890- 8520, Japan.
Pediatr Surg Int. 2019 May;35(5):547-550. doi: 10.1007/s00383-019-04462-9. Epub 2019 Mar 7.
Hirschsprung's disease-related short bowel syndrome (HDSBS) is characterized by aganglionosis that extends orally to 75 cm from Treitz's band. The condition is reported be associated with a high mortality rate of 50-80%. This retrospective study aimed to survey the current trends in HDSBS treatment in Japan.
Patient data were extracted from the results of a nationwide survey we conducted, resulting in the retrospective collection of the data of 1087 HD patients from 2008 to 2012 in Japan.
A total of 11 (0.9%) cases of HDSBS were noted. All patients underwent jejunostomy as neonates. Radical procedures performed in five patients (A-colon patch method in four, Duhamel's procedure in one). Ziegler's myotomy-myectomy and serial transverse enteroplasty (STEP) were performed in each patient as palliative procedures. No radical operations were performed in 4 of the 11 cases. The mortality rate was 36.4%. Four patients died, 1 patient who underwent STEP and 3 patients who received no radical procedures. The causes of death were sepsis due to enterocolitis or central intravenous catheter infection, and hepatic failure. All patients who underwent radical procedures survived and showed satisfactory outcomes.
HDSBS still showed a high mortality rate, although surgical approaches such as the A-colon patch method resulted in satisfactory outcomes.
先天性巨结肠相关短肠综合征（HDSBS）的特征是无神经节症向口腔方向延伸至距屈氏韧带75厘米处。据报道，该病症的死亡率高达50%-80%。这项回顾性研究旨在调查日本HDSBS治疗的当前趋势。
从我们进行的一项全国性调查结果中提取患者数据，从而回顾性收集了2008年至2012年日本1087例先天性巨结肠患者的数据。
共发现11例（0.9%）HDSBS病例。所有患者在新生儿期均接受了空肠造口术。5例患者接受了根治性手术（4例采用A结肠补片法，1例采用杜哈梅尔手术）。每位患者均接受了齐格勒肌切开-肌切除术和系列横断肠管成形术（STEP）作为姑息性手术。11例中有4例未进行根治性手术。死亡率为36.4%。4例患者死亡，1例接受STEP手术的患者和3例未接受根治性手术的患者。死亡原因是小肠结肠炎或中心静脉导管感染引起的败血症以及肝衰竭。所有接受根治性手术的患者均存活且预后良好。
尽管A结肠补片法等手术方法取得了令人满意的结果，但HDSBS的死亡率仍然很高。
