Pawal Sambhaji, Arkar Rahul
Department of Interventional Radiology, Dr. D. Y. Patil Medical College, Hospital & Research Centre, Pune, IND.
Cureus. 2023 Jun 27;15(6):e41015. doi: 10.7759/cureus.41015. eCollection 2023 Jun.
The Abernethy malformation is an extremely rare congenital, extrahepatic, portosystemic shunt. There are many problems associated with this abnormal portovenous shunting and subsequent reduced hepatic portal venous flow. With the advances in non-invasive imaging technologies, these cases are diagnosed in more numbers; however, the presentation of patients is varied and the natural history is not completely known. The presenting symptom of the portosystemic shunt is mainly hyperammonemia, leading to encephalopathy. Management varies depending on the type of shunt and its clinical course; hence, the classification of the congenital portosystemic shunt is important in these patients.
阿伯内西畸形是一种极其罕见的先天性肝外门体分流。这种异常的门静脉分流以及随后肝门静脉血流减少会引发许多问题。随着非侵入性成像技术的进步,此类病例的诊断数量有所增加;然而,患者的表现各异,其自然病史也尚未完全明确。门体分流的主要表现症状是高氨血症,进而导致脑病。治疗方法因分流类型及其临床病程而异;因此,先天性门体分流的分类对这些患者而言至关重要。