Abdullah Lava, Hussein Lilav, Al Houri Hasan Nabil, Khouri Lina
Obstetrics and Gynecology Department, Police Hospital, Damascus, Syria.
Faculty of Medicine, Damascus University, Damascus, Syria.
Radiol Case Rep. 2022 Jul 12;17(9):3318-3320. doi: 10.1016/j.radcr.2022.06.045. eCollection 2022 Sep.
Abernethy malformation (Type 1B) presenting in a 6-year-old boy with hematochezia and hematuria: a case report Abernethy malformation is a rare congenital vascular abnormality defined by the diversion of portal blood flow to the inferior vena cava or its tributaries. Clinical presentations include neonatal cholestasis, liver tumors, and encephalopathy but variables in timing and symptomatology. Herein, we present a 6-year-old boy was referred to our hospital with complaints of hematochezia, hematuria, fecal, and urinary incontinence. A diagnosis of type 1b malformation was made depending on magnetic resonance angiography and cardiac catheterization findings, which demonstrated that the superior mesenteric vein and splenic vein joined to form a common trunk measuring 38 mm diameter and then drained into the dilated inferior vena cava with the absence of portal vein abnormalities in the liver. With further investigations, we indicated the presence of many arteriovenous malformations and urogenital abnormalities. The patient was managed conservatively.
一名6岁男孩出现便血和血尿的1B型阿伯内西畸形:病例报告 阿伯内西畸形是一种罕见的先天性血管异常,其定义为门静脉血流分流至下腔静脉或其分支。临床表现包括新生儿胆汁淤积、肝肿瘤和脑病,但时间和症状表现存在差异。在此,我们报告一名6岁男孩因便血、血尿、大便失禁和尿失禁前来我院就诊。根据磁共振血管造影和心导管检查结果诊断为1B型畸形,结果显示肠系膜上静脉和脾静脉汇合形成一条直径38毫米的共同主干,然后流入扩张的下腔静脉,肝脏门静脉无异常。进一步检查显示存在许多动静脉畸形和泌尿生殖系统异常。该患者接受了保守治疗。