Păcurar Daniela, Dijmărescu Irina, Dijmărescu Adrian Dumitru, Romaşcanu Mihai, Becheanu Cristina Adriana
"Grigore Alexandrescu" Emergency Children's Hospital.
"Carol Davila" University of Medicine and Pharmacy.
Medicine (Baltimore). 2019 Aug;98(31):e16679. doi: 10.1097/MD.0000000000016679.
Congenital portosystemic shunt (Abernethy malformation) is a rare entity causing the portal blood to drain directly into the systemic circulation, eluding the liver. These shunts arise through disturbances in the embryonic development.
A 9-year-old male patient was referred to our department for further evaluation of a vascular malformation which was diagnosed in another facility when the patient was 2 years old, following a routine abdominal ultrasound. The patient had no complaints and the physical examination was normal at all times.
Laboratory tests and esogastroduodenoscopy were normal. The abdominal ultrasound showed a side-to-side shunt between a short portal trunk and the inferior vena cava. A hepatic mass suggestive for focal noduar hyperplasia was seen in the left liver lobe. Abdominal angio-computed tomography (angio-CT) was performed and the ultrasonographic anomalies were confirmed. Multiple other vascular malformations were diagnosed-hepatic artery emerging from the superior mesenteric artery, with early division; hepatic veins forming a short common trunk before draining into the inferior vena cava; supranumerary right renal artery emerging from the aorta, tributary for the upper renal pole. Ecocardiography showed left superior vena cava persistence. The final diagnosis was Abernathy malformation type IB. In the meantime the patient was diagnosed with allergic asthma.
No surgical cure was pursued because the malformation was an incidental discovery.
The patient was followed-up closely from the final diagnosis (when he was 9 years old) to present (he is currently 10 years old) with no change in his status-he remained asymptomatic.
Angio-CT should be the performed whenever a vascular malformation is suspected in order to establish a correct diagnosis, because portosystemic shunts carry a high risk of severe complications. Knowing that patients with portosystemic shunts may have pulmonary hypertension, respiratory complaints should be carefully evaluated-in this particular case, even though the most probable cause for the respiratory symptoms was pulmonary hypertension, it was ruled out by cardiac ultrasonography and further investigations confirmed the diagnosis of allergic asthma.
先天性门体分流（阿伯内西畸形）是一种罕见的病症，可导致门静脉血直接流入体循环，避开肝脏。这些分流是由胚胎发育紊乱引起的。
一名9岁男性患者因血管畸形前来我院进一步评估。该患者2岁时在另一家机构进行常规腹部超声检查后被诊断出患有血管畸形。患者无任何不适，体格检查一直正常。
实验室检查和食管胃十二指肠镜检查均正常。腹部超声显示短门静脉干与下腔静脉之间存在侧侧分流。左肝叶可见一个提示局灶性结节性增生的肝脏肿块。进行了腹部血管计算机断层扫描（血管CT），超声检查异常得到证实。还诊断出其他多种血管畸形——肠系膜上动脉发出肝动脉并早期分支；肝静脉在汇入下腔静脉之前形成一条短的共同主干；腹主动脉发出额外的右肾动脉，为肾上极供血。超声心动图显示左上腔静脉持续存在。最终诊断为IB型阿伯内西畸形。同时，该患者被诊断出患有过敏性哮喘。
由于该畸形是偶然发现的，未采取手术治疗。
从最终诊断（9岁时）到目前（现10岁）对患者进行了密切随访，其状况无变化——仍无症状。
每当怀疑有血管畸形时，都应进行血管CT检查以确立正确诊断，因为门体分流有发生严重并发症的高风险。鉴于门体分流患者可能患有肺动脉高压，应仔细评估呼吸系统症状——在这个特殊病例中，尽管呼吸系统症状最可能的原因是肺动脉高压，但心脏超声检查排除了该病因，进一步检查确诊为过敏性哮喘。
