Abstract：
BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) is a rare intestinal disorder characterized by impaired propulsion of the digestive tract and associated with symptoms of intestinal obstruction, despite the absence of obstructive lesions. CIPO includes several diseases. However, definitive diagnosis of its etiology is difficult only with symptoms or imaging findings.
METHODS: A 56-year-old man was referred to our hospital due to a 3-year history of continuous abdominal distention. Imaging, including computed tomography of the abdomen, and endoscopy revealed marked dilatation of the entire small intestine without any obstruction point. Therefore, he was diagnosed with CIPO. Since medical therapy didn\'t improve his symptoms, enterostomy and percutaneous endoscopic gastro-jejunostomy were performed. These procedures improved abdominal symptoms. However, he required home central venous nutrition due to dehydration. The pathological findings of full-thickness biopsies of the small intestine taken during surgery revealed decreased number and degeneration of ganglion cells in the normal plexus. These findings led to a final diagnosis of CIPO due to acquired isolated hypoganglionosis (AIHG).
CONCLUSIONS: Here, we report the case of a patient with CIPO secondary to adult-onset AIHG of the small intestine. Since AIHG cannot be solely diagnosed using clinical findings, biopsy is important for its diagnosis.
METHODS: A 56-year-old man was referred to our hospital due to a 3-year history of continuous abdominal distention. Imaging, including computed tomography of the abdomen, and endoscopy revealed marked dilatation of the entire small intestine without any obstruction point. Therefore, he was diagnosed with CIPO. Since medical therapy didn\'t improve his symptoms, enterostomy and percutaneous endoscopic gastro-jejunostomy were performed. These procedures improved abdominal symptoms. However, he required home central venous nutrition due to dehydration. The pathological findings of full-thickness biopsies of the small intestine taken during surgery revealed decreased number and degeneration of ganglion cells in the normal plexus. These findings led to a final diagnosis of CIPO due to acquired isolated hypoganglionosis (AIHG).
CONCLUSIONS: Here, we report the case of a patient with CIPO secondary to adult-onset AIHG of the small intestine. Since AIHG cannot be solely diagnosed using clinical findings, biopsy is important for its diagnosis.
摘要：
背景：慢性假性肠梗阻（CIPO）是一种罕见的肠道疾病，其特征是消化道推进受损并伴有肠梗阻症状，尽管没有阻塞性病变。CIPO包括几种疾病。然而,仅根据症状或影像学发现很难明确诊断其病因。
方法：一名56岁男子因连续腹胀3年病史被转诊至我院。成像,包括腹部的计算机断层扫描，内窥镜检查显示整个小肠明显扩张，没有任何阻塞点。因此,他被诊断为CIPO。因为药物治疗并没有改善他的症状,进行了肠造口术和经皮内镜胃空肠造口术。这些手术改善了腹部症状。然而,由于脱水，他需要家庭中心静脉营养。手术期间对小肠进行全层活检的病理发现显示，正常神经丛中神经节细胞的数量减少和变性。由于获得性孤立性神经节减少症（AIHG），这些发现导致了CIPO的最终诊断。
结论：这里，我们报道了1例继发于成人小肠AIHG的CIPO患者。由于AIHG不能单独使用临床发现进行诊断，活检对其诊断很重要。
方法：一名56岁男子因连续腹胀3年病史被转诊至我院。成像,包括腹部的计算机断层扫描，内窥镜检查显示整个小肠明显扩张，没有任何阻塞点。因此,他被诊断为CIPO。因为药物治疗并没有改善他的症状,进行了肠造口术和经皮内镜胃空肠造口术。这些手术改善了腹部症状。然而,由于脱水，他需要家庭中心静脉营养。手术期间对小肠进行全层活检的病理发现显示，正常神经丛中神经节细胞的数量减少和变性。由于获得性孤立性神经节减少症（AIHG），这些发现导致了CIPO的最终诊断。
结论：这里，我们报道了1例继发于成人小肠AIHG的CIPO患者。由于AIHG不能单独使用临床发现进行诊断，活检对其诊断很重要。
