Stringer Mark D, Jones Matthew O, Woodley Helen, Wyatt Judy
Children's Liver Unit, St. James's University Hospital, LS9 7TF Leeds, UK.
J Pediatr Surg. 2006 Jun;41(6):1180-3. doi: 10.1016/j.jpedsurg.2006.01.068.
A male infant was found to have a large congenital hepatic cyst, first noted in late gestation by prenatal ultrasound scan. The cyst communicated with the biliary tree and was eventually removed completely by an extended right hepatectomy. Histopathologic examination showed a thick-walled, unilocular cyst lined predominantly by ciliated, stratified squamous epithelium with an outer wall composed of smooth muscle cells and fibrous tissue. These features are diagnostic of a ciliated hepatic foregut cyst, a rare congenital malformation with histologic similarities to bronchogenic cysts. The young age of our patient, prenatal detection, large size of the cyst, and a clear communication with the biliary tree have not been previously described with ciliated hepatic foregut cysts.
一名男婴被发现患有一个巨大的先天性肝囊肿,产前超声扫描在妊娠晚期首次发现。该囊肿与胆管树相通,最终通过扩大右肝切除术被完全切除。组织病理学检查显示为一个厚壁单房囊肿,主要内衬纤毛、复层鳞状上皮,外壁由平滑肌细胞和纤维组织组成。这些特征可诊断为纤毛肝前肠囊肿,这是一种罕见的先天性畸形,在组织学上与支气管源性囊肿相似。我们患者的年轻年龄、产前检测、囊肿的大尺寸以及与胆管树的明确相通,此前在纤毛肝前肠囊肿中尚未有过描述。