Swiss Pediatric Liver Center, Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland.
Division of Child and Adolescent Surgery, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, University of Geneva, 6, Rue Willy Donzé, Geneva, 1205, Switzerland.
BMC Pediatr. 2024 Sep 13;24(1):580. doi: 10.1186/s12887-024-05043-z.
Prenatally diagnosed hepatic hilar cysts are a challenging finding for the clinician. They can either be a sign of cystic biliary atresia (BA) or a choledochal cyst (CC), two diagnoses with different postnatal management and prognosis. Based on a case report of four patients, we aim to propose a management algorithm for prenatally diagnosed "hepatic hilar cysts".
A hepatic hilar cyst, ranging from 5 to 25 mm, was detected prenatally in all four girls confirmed postnatally along with the presence of a gallbladder. Stool color was normal until two weeks of life at which time the stool color became lighter, and the patients developed cholestasis. All were operated before seven weeks of life: Case 1 had a CC with patent but irregular intrahepatic bile ducts at intraoperative cholangiogram, and no communication with the duodenum. A Roux-en-Y bilioenteric anastomosis was performed. The cyst showed complete epithelial lining loss, and liver pathology showed BA features. Case 2 had the final diagnosis of cystic BA with patent but abnormal intrahepatic bile ducts. She underwent two operations: the first operation at four weeks as described for case 1, since intraoperative findings were similar, as was histology. As cholestasis increased postoperatively, she underwent a Kasai hepato-porto-enterostomy six weeks later, where distinct BA findings were found with complete scarring of the hilar plate. Case 3 had a cystic BA with the cyst located within the common bile duct and atretic bile ducts proximal to the porta hepatis. It exhibited no communication with the liver or duodenum. A Kasai operation was performed, with histology showing complete epithelial loss within the cyst wall and scarring of the hilar plate. Case 4 had a cystic BA presenting a completely obliterated hepatic duct with the cyst lying within the common bile duct. A Kasai procedure was performed. Histology showed a common bile duct with a residual lumen of 0.1 mm.
The spectrum of disease from CC to BA in the setting of a prenatally discovered hepatic hilar cyst is emphasized. Even if cholangiogram differentiates most patients with BA from those with CC, caution is advised for transitional types.
产前诊断的肝门部胆管囊肿对于临床医生来说是一个具有挑战性的发现。它们可能是囊性胆管闭锁 (BA) 的表现，也可能是胆总管囊肿 (CC) 的表现，这两种诊断的治疗和预后完全不同。基于 4 例患者的病例报告，我们旨在为产前诊断的“肝门部胆管囊肿”提出一种治疗方法。
4 名女孩均在产前发现肝门部胆管囊肿，大小为 5-25mm，产后均伴有胆囊存在。大便颜色正常，直到出生后两周，大便颜色变浅，出现胆汁淤积。所有患者均在 7 周龄前接受手术：病例 1 在术中胆管造影时显示 CC，肝内胆管通畅但不规则，与十二指肠无相通。行 Roux-en-Y 胆肠吻合术。囊肿完全失去上皮衬里，肝脏病理显示 BA 特征。病例 2 最终诊断为囊性 BA，肝内胆管通畅但异常。她接受了两次手术：第一次手术在 4 周时进行，手术方式与病例 1 相同，因为术中所见和组织学均相似。术后由于胆汁淤积增加，她在 6 周后接受了 Kasai 肝门-肠吻合术，发现明显的 BA 特征，肝门板完全瘢痕化。病例 3 为囊性 BA，囊肿位于胆总管内，肝门部胆管近端闭锁。囊肿与肝脏或十二指肠无相通。行 Kasai 手术，组织学显示囊肿壁完全上皮缺失和肝门板瘢痕化。病例 4 为囊性 BA，肝内胆管完全闭塞，囊肿位于胆总管内。行 Kasai 手术。组织学显示胆总管残留腔为 0.1mm。
强调了产前发现的肝门部胆管囊肿从 CC 到 BA 的疾病谱。即使胆管造影术将大多数 BA 患者与 CC 患者区分开来，但对于过渡类型仍需谨慎。
