- 期刊
An Adult Man with Refractory Constipation that is Diagnosed as ‘Adult-type Hypoganglionosis’
便祕的其他可能--成人型結腸神經節減少症
摘要
結腸神經節減少症是個類似Hirschsprung’s disease 為先天性巨結腸症的一種。九成的病人都在新生兒的時候診斷，平均診斷年齡為4.85歲。但我們要報告一個案在43歲時，因為嚴重便秘一年合併有巨結腸症，開刀切除腫脹的結腸後診斷為結腸神經節減少症。
關鍵字
無資料並列摘要
The Hypoganglinosis is a rare and resembles Hirschsprung’s disease in the male-to-female ratio, etiology and clinical presentation. Although chronic constipation is a common complaint in general population, the patients with constipation can accompany with fatal complication if the patient with megacolon which is related with hypoganglinosis. Up to 90.5% of patients with Hirschsprung’s disease are diagnosed in the newborn period and the median age at diagnosis of the patient with hypoganglinosis was 4.85 years old. We report a healthy 43 year-old male with severe constipation who is diagnosed as adult type hypoganglinosis. A 43-year-old man presented with chronic constipation, abdominal discomfort, and flatulence, and had been passing intestinal gas with unusual odors for 18 months. Computed tomography showed a severely dilated feces-filled proximal segment of the descending colon and transverse colon. The patient underwent subtotal colectomy with ileocolic anastomosis for a clinical impression of intractable megacolon. Histopathology confirmed a diagnosis of hypoganglionosis of the colon. The hypoganglinosis is rare disease in population, especially in adult group. However, the early and accurate diagnosis of adult hypoganglionosis is important for the physician to avoid fatal complication. We hope that adult intestinal innervation disorders will be considered readily in the differential diagnoses of chronic constipation.