Yokokawa Hideyuki, Yoshida Mariko, Takazawa Shinya, Kutsukake Mai, Omata Kanako, Ogawa Shoko, Fujishiro Jun
Department of Pediatric Surgery, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
Surg Case Rep. 2023 Feb 15;9(1):25. doi: 10.1186/s40792-023-01602-1.
Segmental dilatation of the colon (SDC) is a rare disease that is characterized by an abrupt segment of dilated colon between regions of normal-sized colon. We herein report a case of SDC associated with Hirschsprung's disease (HD).
The patient developed abdominal distension soon after birth, and enema examination showed localized intestinal dilatation from the descending colon to the sigmoid colon with significant caliber changes on both the oral and anal sides of the dilated colon. The findings of the rectal mucosal biopsy were consistent with HD. We considered this case to be a combination of HD and SDC and performed laparoscopic-assisted Soave pull-through with resection of the dilated colon when the patient was 7 months old. Resected specimens showed steep caliber changes on the oral and anal sides of the dilated colon. In the pathological examination, no ganglion cells were found in the submucosa on the anal side of the dilated colon. Based on the above findings, we finally made the diagnosis of HD with SDC.
In HD with a characteristic dilated colon, the possibility of SDC should be considered.
结肠节段性扩张（SDC）是一种罕见疾病，其特征是在正常大小的结肠区域之间存在一段突然扩张的结肠。我们在此报告一例与先天性巨结肠（HD）相关的SDC病例。
该患者出生后不久即出现腹胀，灌肠检查显示从降结肠至乙状结肠局部肠管扩张，扩张结肠的口侧和肛侧管径均有明显变化。直肠黏膜活检结果与HD相符。我们认为该病例为HD合并SDC，并在患者7个月大时进行了腹腔镜辅助Soave拖出术并切除扩张结肠。切除标本显示扩张结肠的口侧和肛侧管径变化明显。病理检查发现，扩张结肠肛侧黏膜下层未发现神经节细胞。基于上述发现，我们最终诊断为HD合并SDC。
在具有特征性扩张结肠的HD中，应考虑SDC的可能性。
