小兒之先天性腸管旋轉異常

Malrotation of the Intestine as a Cause of Intestinal Obstruction in Infancy and Childhood

陳維昭(Wei-Jao Chen) ； 陳秋江(Chiou-Chiang Chen) ； 洪文宗(Wen-Tsung Hung)

《Acta Paediatrica Sinica》 18卷4期 (1977/12) Pp. 235-240

摘要

小兒先天性腸管旋轉異常，雖是小兒消化道異常中常見之疾患，但一般外科或小兒科醫師對本症之警覺仍較缺乏。臺大外科過去13年間所經驗的此種病例有47例，其中因腸管旋轉異常引起腸阻塞症狀接受手術的有33例，是為本文分析之對象。33例中男女之發生比率恰為2:1，與一般報告相符。接受手術時之年齡在一週以內的只有8例(24.2%)，與一般報告之50%以上在一週以內接受治療之情形相比，顯示多少有延誤之跡象。絞扼性腸扭結是致死的主因，33例中23例(70%)併發腸扭結，因而引起腸管壞死或穿孔的有7例(21%)，結果3例死亡。合併異常中較主要的為腸管閉鎖或狹窄、環形胰臟、橫隔膜疝氣、臍膨出等，在併有其他異常之18例中8例死亡，死亡率高達44.4%。

並列摘要

Congenital malrotation of the intestine is a relatively common cause of intestinal obstruction in infants and children, and is a malformation frequently associated with other congenital anomalies. During the last 13 year-period, 47 cases of malrotation of the intestine were experienced by authors in the Department of Surgery, National Taiwan University Hospital. Among them, 33 patients who were operated upon because of a manifestation of intestinal obstruction due to malrotation are the subjects of this report. For the remaining 14 patients, malrotation of the intestine was found incidentally during operation for other congenital malformations.
For the above mentioned 33 patients, there was a sex incidence of two to one, with a preponderance of male patient. More than half of these patients were operated upon at an age of less than one month. But only 8 of them (24.2%) were operated upon below one week of age.
Operative findings revealed that 24 out of 33 patients belonged to type I malrotation according to Grob's classification. Non-rotation was encountered in 4 patients. Volvulus was a common complication and was related to the mortality. Twenty-three out of 33 (70%) patients were found to have volvulus at surgery. Strangulated volvulus resulting in intestinal necrosis was experienced in 7 patients (21%), 4 of them developed peritonitis due to intestinal perforation. There were 5 deaths, of them two died of intestinal perforation: one of strangulated volvulus, and the remaining two of prematurity, infections and postoperative gastric perforation.
Coexistence with other congenital anomalies is common. Twenty anomalies were encountered in 18 patients including those 14 patients with malrotation of the intestine found incidentally at surgery. Intestinal atresia was the most common associated pathology. Less common were annular pancreas, diaphragmatic hernia and omphalocele. Mortality occurred in 8 cases (44.4%) of 18 patients who had an associated pathology. No death was encountered in patients who had neither a volvulus nor an associated malformation.
Although diagnosis of the malrotation of the intestine is usually not difficult, misleading is not uncommon. Differentiation from other causes of intestinal obstruction especially from those of duodenal atresia and annular pancreas is necessary. A possibility of the combination of two malformations, however, should always be kept in mind. A barium enema to identifying the position of the colon and cecum provides usually the best way to the establishment of the diagnosis.

並列關鍵字

malrotation of the intestine ； volvulus ； intestinal obstruction ； congenital ； malformations