Allarakia Jawad, Felemban Taher, Khayyat Waleed, Alawi Ahmed, Mirza Abdurrahaman, Alkhazal Batool, Yousef Yasmin
King Saud Bin Abdulaziz University for Health Sciences, College of Medicine, Jeddah, SAudi Arabia.
Department of Surgery, Pediatric Surgery Section, King Fahad Armed Forces Hospital,Jeddah, Saudi Arabia.
Int J Surg Case Rep. 2019;55:152-155. doi: 10.1016/j.ijscr.2019.01.028. Epub 2019 Jan 30.
Biliary atresia (BA) is a rare condition where the hepatic or common bile ducts get obliterated by fibrous tissue for unknown causes. It is diagnosed in 5-10 per 100,000 live births worldwide in whom liver injury and death are definite if untreated.
We report a case of BA with associated malrotation and situs ambiguous discovered incidentally during Kasai procedure. The small intestine was found to be malrotated with the duodenojejunal junction to the right of the vertebral column. The liver, stomach, and spleen were in the center, right side, and left side of the abdomen respectively. The malrotation was corrected by performing a Ladd's procedure in addition to the Kasai. She had an uneventful postoperative course. At follow-up on the second, sixth and 12th weeks postoperatively, she had normal stool color and liver function.
BA patients with associated structural anomalies might have a worse outcome after the Kasai procedure. Several studies demonstrated that the coexistence of malrotation with BA to be in the range of 0.3%-9.4% among BA patients. Also, the presence of situs abnormalities with BA has been reported in some studies to be present in 0.01-0.045% of BA cases. However, the presentation of BA with both situs inversus and intestinal malrotation has seldom been reported.
The diagnosis of BA should prompt further investigation for other anatomical abnormalities as the presence of which might affect the management plan.
胆道闭锁（BA）是一种罕见的疾病，肝内或肝外胆管因不明原因被纤维组织阻塞。全球每10万例活产婴儿中约有5至10例被诊断为此病，若不治疗，肝脏损伤和死亡在所难免。
我们报告一例在葛西手术过程中偶然发现合并肠旋转不良和内脏反位的BA病例。发现小肠旋转不良，十二指肠空肠交界位于脊柱右侧。肝脏、胃和脾脏分别位于腹部中央、右侧和左侧。除葛西手术外，还通过实施Ladd手术纠正了旋转不良。术后恢复顺利。术后第二周、第六周和第十二周随访时，大便颜色和肝功能均正常。
合并结构异常的BA患者在葛西手术后可能预后较差。多项研究表明，BA患者中肠旋转不良与BA并存的比例在0.3%至9.4%之间。此外，一些研究报告称，BA病例中内脏位置异常的发生率为0.01%至0.045%。然而，BA合并内脏反位和肠旋转不良的情况鲜有报道。
BA的诊断应促使进一步检查是否存在其他解剖异常，因为这些异常的存在可能会影响治疗方案。
