Abernethy syndrome is a rare congenital anomaly characterized by an intrahepatic or extrahepatic portosystemic shunt. Most patients are asymptomatic; however, due to the alteration in, or lack of, a portovenous flow, patients with Abernethy syndrome are at high risk of developing sequelae of liver failure. Once these complications develop, the only definitive treatment is transplantation. Patients with Abernethy syndrome are also at a higher risk of developing benign and malignant liver lesions, including hepatic adenomas. Here, we describe the first case of deceased donor liver transplantation as a treatment for a patient with type 1 Abernethy syndrome complicated by large, unresectable hepatic adenoma, found to have focal hepatocellular carcinoma on pathologic examination. Our male patient was found to have elevated liver enzymes at age 33, during a routine outpatient medical appointment. Despite being asymptomatic, his history of prior liver resection prompted CT imaging, which revealed two large liver lesions concerning for hepatic adenomas. When surveillance imaging showed a significant growth of the liver lesions, biopsy was pursued, which confirmed a diagnosis of hepatic adenomas. However, given the size of these lesions, resection was not a viable option for the patient. Instead, the patient underwent liver transplantation at age 41, which he tolerated well. Our case demonstrates the utility of deceased donor liver transplantation as a treatment for patients with Abernethy syndrome complicated by unresectable adenomas.

Congenital portosystemic shunts (CPSSs) are rare vascular anomalies characterized by abnormal connections between the portal/splanchnic veins and the systemic veins. CPSSs often occur as an isolated congenital anomaly, but they can also coexist with congenital heart disease (CHD). Owing to their myriad consequences on multiple organ systems, familiarity with CPSS is of tremendous importance to the care of patients with CHD. The rationale and timing for interventions to embolize CPSS in this scenario are discussed. Specific shunt embolization techniques are beyond the scope of this article.

UNASSIGNED: Acquired hepatocerebral degeneration (AHD) is a neurological condition associated with cerebral manganese (Mn) accumulation caused by portosystemic shunts (PSS), usually because of advanced liver disease. AHD is diagnosed by the identification of T1-weighted brain magnetic resonance imaging (MRI) hyperintensities coupled with the presence of PSS and neurological symptoms. Clinical presentations primarily involve motor dysfunction and cognitive impairment. As a result of the frequently concurrent hepatic encephalopathy, the psychiatric symptoms of AHD alone remain unclear. This report is the first documentation of unique psychiatric symptoms of AHD due to a congenital PSS (CPSS) and suggests the efficacy of shunt embolization in achieving sustained remission of psychiatric symptoms in such cases.
UNASSIGNED: A 57-year-old Japanese woman presented with recurrent severe depression, pain, and somatosensory hallucinations, along with fluctuating motor dysfunction, including parkinsonism, and cognitive impairments. Psychiatric interventions, including antidepressants, antipsychotics or electroconvulsive therapy, had limited efficacy or did not prevent relapse.
UNASSIGNED: T1-weighted MRI showed bilateral hyperintensity in the globus pallidus. No history of Mn exposure or metabolic abnormalities, including copper, was identified. Furthermore, no evidence of liver dysfunction or hyperammonemia was found. Eventually, a gastrorenal shunt was observed on contrast-enhanced abdominal computed tomography. The diagnosis of AHD due to CPSS was made based on the clinical manifestations and abnormal imaging findings. Shunt embolization was performed, which prevented the relapse of psychiatric symptoms and substantially reduced the T1-weighted MRI hyperintensities.
UNASSIGNED: This case highlights the potential involvement of AHD in adult-onset psychiatric symptoms, even in the absence of liver disease. Furthermore, this case underscores the efficacy of shunt embolization in treating the psychiatric symptoms of AHD due to CPSS.

先天性肝内门体分流是一种极为罕见的血管畸形，其异常分流道连接肝内门静脉与肝静脉或下腔静脉，导致部分血液绕过肝脏直接进入体循环从而引发高氨血症、高半乳糖血症等，严重者并发肝肺综合征、肺动脉高压、肝性脑病，治疗较为棘手。现报道1例以肝肺综合征为首发表现的先天性肝内门体分流V型患儿，应用Amplatzer血管塞Ⅱ型成功介入栓塞的案例，术后1d复查血氨降至正常，术后6个月患儿缺氧症状消失，临床指标改善。以期加深对该病的认识，提示介入治疗可作为部分患儿的首选治疗方案。.

Congenital portosystemic shunts are often associated with systemic complications, the most challenging of which are liver nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. In the present paper, we offer expert clinical guidance on the management of liver nodules, pulmonary hypertension, and endocrine abnormalities, and we make recommendations regarding shunt closure and follow-up.

UNASSIGNED: Down syndrome is due to trisomy 21 and is characterized by intellectual disability, dysmorphic facial features, congenital malformations, and gastrointestinal abnormalities. There is an increased appreciation of congenital portosystemic shunts in Down syndrome patients. Congenital portosystemic shunts have been associated with many defects in body systems, including cardiac, metabolic, and neurological.
UNASSIGNED: Herein, we describe a portosystemic shunt in a Down syndrome patient that resulted in hyperammonemia with altered mental status and choreiform movements. Computed tomography angiography of the abdomen and pelvis identified a connection between the right portal vein and inferior vena cava. An 18 mm Amplatzer PFO closure device was placed within the congenital shunt, significantly improving symptoms. The patient has no sequelae from the related shunt or the device at the 2-year follow-up. We extensively reviewed the literature and identified cases of portosystemic shunts in Down syndrome patients. Shunts can either be extrahepatic or intrahepatic and are classified by vasculature connections.
UNASSIGNED: From our literature review and case presentation, we identify other conditions in patients, including cardiac and gastrointestinal defects. We then review the available treatment options, whether observation or surgical, depending on the patient\'s clinical picture.

Congenital portosystemic shunts are rare vascular malformations in which portal venous blood from the intestines and spleen bypasses the liver and diverts directly into the systemic circulation through abnormal vessels. We report a case of a 4-year-old girl with heterotaxy syndrome, polysplenia, and situs inversus presenting with persistent hypoxemia who was found to have pulmonary arteriovenous malformations (PAVMs) and hypoxemia secondary to a congenital portosystemic shunt. Management of this patient\'s PAVMs involved endovascular occlusion of the portosystemic shunt with subsequent resolution of hypoxemia. PAVMs secondary to extrahepatic portosystemic shunt should be explored as a cause of progressive cyanosis in children with heterotaxy, polysplenia, and interrupted inferior vena cava with azygous continuation.

A 38-year-old woman was admitted to our university hospital with loss of muscle strength. She was diagnosed with dermatomyositis and underwent contrast-enhanced computed tomography of the entire body to check for malignant tumors. Computed tomography revealed multiple enhanced hepatic nodules and an extrahepatic portosystemic shunt. Although a needle biopsy of the nodule could not diagnose definitive hepatocellular carcinoma, some nodules increased in size after three months. Because of the inconclusive results of the second biopsy, we performed shunt embolization using a vascular plug. After another three months, the hepatic nodules shrank markedly, as expected.

Hyperammonemic encephalopathy is a neurological emergency that can lead to seizures and cerebral edema. Although early interventions have been suggested, no clear criteria have been established. Herein, we report a case of severe non-hepatic hyperammonemia resulting in refractory status epilepticus within a day. A 79-year-old woman presented with acute altered mental status. Initial evaluation revealed septic shock and hyperammonemia due to fecal bowel obstruction with congenital portosystemic shunt. The patient was unresponsive to medical treatment and developed refractory status epilepticus. After surgical drainage with colostomy and a decrease in ammonia level, the patient developed cerebral edema and did not recover from the coma. Severe hyperammonemia warrants early intervention, especially in critically ill patients, with treatment of the cause and augmented removal of ammonia with renal replacement therapy.

UNASSIGNED: Congenital extra hepatic portosystemic shunt (CEPS) is a rare vascular malformation in which splanchnic and portal blood is shunted into the systemic circulation eluding the liver. Type 1 CEPS is sometimes difficult to differentiate from pathologies such as chronic portal veinthrombosis as the portal vein may not be visualized in either entities.
METHODS: A 3-year-old male child with a week of abdominal pain was diagnosed with chronic portal vein thrombosis in an out-of-hospital setting. Repeat abdominal ultrasound was done at our institution and we were able to visualize termination of the portal vein to the suprarenal infra-hepatic inferior vena cava with an end to side pattern and a focal hypoechoic hepatic lesion at segment eight of the liver. There was no evidence of cavernous transformation or sign of portal hypertension. Subsequently, tri-phasic computed tomography revealed similar findings, with the portal vein terminating at the suprarenal inferior vena cava. The focal hepatic lesion showed peripheral contrast enhancement in the arterial phase and appeared as a central non-enhancing area with evidence of homogeneous enhancement on the subsequent sequences.
UNASSIGNED: Type 1 CEPS can be easily confused with chronic portal vein thrombosis as the portal vein may not be visible and the hepatic artery shows compensatory enlargement in both entities. However, portal vein thrombosis is usually associated with underlying predisposing factors and can result in the development of secondary signs of portal hypertension and cavernous transformation which are critical to distinguish it from CEPS.
CONCLUSIONS: Chronic portal vein thrombosis is a great mimicker that should be distinguished from CEPS on ultrasound. A meticulous scan with color flow is helpful to scrutinize vascular anatomy, identify findings associated with CEPS such as hepatic lesions, and exclude signs of chronic portal vein thrombosis.