A case of two shunts in the endovascular treatment of type II Abernethy syndrome

Background Abernethy malformation is a rare condition defined by a congenital extrahepatic portosystemic shunt, often leading to absence or hypoplasia of the intrahepatic portal venous system. Although there are no consensus treatment guidelines, interventional techniques now offer minimally invasive treatment options for Abernethy malformations. This case report describes a case of Abernethy Syndrome Type II where the patient had two separate extrahepatic portosystemic shunts treated with endovascular occlusion with two Amplatzer plugs and demonstrates the feasibility of this treatment for this rare condition. This case was in a young adult, adding to the scarce literature of treatment for Abernethy syndrome in the adult population. Case presentation We report a case of a 20-year-old female patient with neurocognitive behavioral difficulty, voracious appetite, and chronic encephalopathy secondary to type II Abernethy malformation with not one, but two extrahepatic portosystemic shunts. The patient had failed medical management and was not a liver transplant candidate. Therefore, she presented to us for an endovascular treatment option. The two shunts were treated with endovascular occlusion using Amplatzer vascular plugs. Following embolization, flow into the hypoplastic portal vein improved with near complete occlusion of flow into the portosystemic shunts, thus restoring blood flow into the native portal system. At 3 month follow up, a CT demonstrated complete occlusion of the two portosystemic shunts, and a portal vein diminutive in caliber. The portal vein measured 7 mm in diameter on both pre and post-procedure CT scans. The total volume of the liver was found to be 843 cm3 on pre-procedure CT & 1191 cm3 on post-procedure CT. Conclusions This report demonstrates the feasibility of using endovascular embolization to treat Abernethy II malformations. The management strategy of Type II Abernethy Syndrome should be to redirect blood flow into the hypoplastic native portal system, allowing for physiologic hepatic metabolism of splanchnic blood, hypertrophy of the portal system, and growth of the liver from the increased trophic flow.

Transformasi Teknologi Elektronik melalui Aplikasi Sistem e-Syariah dan i-Syariah dalam Sistem Pengurusan Mahkamah Syariah di Malaysia

Implimentasi teknologi maklumat dalam institusi kehakiman syariah disambut baik oleh semua mahkamah syariah negeri di Malaysia melalui aplikasi sistem e-Syariah. Inovasi dan transformasi teknologi elektronik ini diteruskan oleh sebahagian mahkamah syariah termasuk mahkamah syariah di Sarawak yang membangunkan sistem baharu yang dinamakan i-Syariah, iaitu Sistem Pengurusan Pintar Syariah. Artikel ini bertujuan menganalisis pembangunan sistem Pengurusan Mahkamah Syariah melalui sistem e-Syariah pada peringkat Persekutuan dan i-Syariah yang diaplikasikan di Sarawak. Penelitian juga dilakukan terhadap modul yang dibangunkan dalam sistem e-Syariah dan i-Syariah bagi dapatan maklumat penyelidikan yang menyeluruh. Metodologi penyelidikan dilakukan secara kualitatif terhadap perkembangan pembangunan sistem e-Syariah dan sistem i-Syariah serta melaksanakan metode komparatif dengan membandingkan sistem yang diguna pakai di mahkamah sivil dengan kandungan sistem yang diaplikasikan di institusi kehakiman syariah. Penyelidikan ini mendapati bahawa transformasi teknologi elektronik mahkamah syariah kurang menunjukkan perkembangan berbanding mahkamah sivil yang telah mengaplikasi e-Filing system (EFS), Case Management System (CMS), Queue Management System (QMS), Court Recording and Transcribing (CRT), Artificial Intelligence (AI) dan Community and Advocate Portal System (CAP). Walau bagaimanapun berdasarkan pelan pembangunan i-Syariah maka sistem-sistem yang diguna pakai pada peringkat mahkamah sivil ini akan diimplimentasikan di mahkamah syariah Sarawak untuk memastikan transformasi informasi teknologi maklumat seiring dengan perkembangan teknologi digital di Sarawak.

Multistage closure of a congenital extrahepatic portosystemic shunt

Background Congenital extrahepatic portosystemic shunts (CEPS) are rare shunts connecting the extrahepatic portal system with the inferior vena cava. Shunt dimensions and the risk of portal hypertension determines the closure strategy. Endovascular treatment is indicated for single stage occlusion of longer length shunts, whereas the remaining shunt types are preferentially surgically occluded. Herein we describe the technical details of a novel endovascular treatment for short length CEPS. Case presentation A 15-years-old male with a short length CEPS complicated with multinodular liver disease was submitted to a multistage closure, as indicated by the high portal pressure values during shunt balloon occlusion venography. Initially a transjugular intrahepatic portosystemic shunt (TIPS) was created and the CEPS occluded with an atrial septal defect occluder. In a second procedure the TIPS was embolized with a flow reductor stent and an amplatzer vascular plug II. At a 1 year follow up the liver nodules size reduced, the patient remains asymptomatic, and the shunt adequately closed. Conclusion This paper outlines the potential use of a TIPS and an atrial septal defect occluder combination in complex CEPS, supporting its usage as an alternative to the standard surgical treatment. Level of Evidence: Level 4, Case report.

Detection of gastric cancer metastases using splenoportography

The method of intravital contrasting of the vessels of the portal system with transparietal injection of a contrast agent into the spleen was first applied in 1951 by Leger and Souza Pereira on the basis of the experimental work of Abeatici and Campi.

THE EFFECTIVENESS OF SPLENORENAL SHUNT IN TREATMENT OF PREHEPATIC PORTAL HYPERTENSION IN CHILDREN

Objective. To evaluate the results of treatment of prehepatic portal hypertension (PPH) in children by method of side-to-side splenorenal bypass. Methods. A retrospective, monocentric analysis of 114 patients with prehepatic portal hypertension was carried out in the period from 2000 to 2018. The age of the patients was from 6 months up to 18 years, the average age 10,1±3,8 years (M±σ). All patients underwent side-to-side splenorenal shunt. In the postoperative period, on the 7^th day, ultrasound examination of the abdominal organs with Doppler sonography (US) was performed to assess the volume of the spleen, the rate of portohepatic perfusion, the rate of blood flow along the side-to-side splenorenal shunt, as well as to assess its diameter. Control US was performed after the 1^st, 3^rd, 6^th, 12^th months; to assess the regression of esophageal and gastric varicose veins, the control esophagogastroduodenoscopy (EYD) was performed after 3^rd , 6^th, 12^th months and then once a year. Subsequently, the control examination of the patients was carried out once a year. The follow-up was 2-18 years. Results. Out of 114 patients who underwent side - to - side splenorenal shunt, 5 (4,4%) patients had recurrent bleeding from the esophageal variceal vein at the follow-up period (2-18 years). One patient had intra-abdominal arrosive bleeding from side-to-side splenorenal shunt provoked by severe postoperative pancreatitis. The average diameter of the shunt was 12±1.9 mm. The spleen volume decreased by 39.7±10.3%. Portohepatic perfusion reduced by 39% in comparison with the indicators before surgery. The blood flow rate along the side -to-side splenorenal shunt was 2403±557.3 ml/min. in 95,4% of patients the regression of variceal vein was noted. Hypersplenism syndrome was arrested in 73.6% of children. Conclusion. Side -to- side splenorenal shunt is considered to be an effective method of portal decompression with the effectiveness inpreventingboth esophageal and gastricvariceal veins bleeding in 95,4% of patients. What this paper adds The results of treatment of the prehepatic portal hypertension by method of side-to-side splenorenal shunt have been firstly analyzed, and the effect of this variant of portosystemic shunt on portohepatic perfusion has been assessed. It has been established that this option of shunt is effective for the portal system decompression and occupies an intermediate position between selective and total variant of portosystemic shunt.

Portal Venous Thrombosis in a Special Operations Paratrooper: A Case Report

ABSTRACT Portal vein thrombosis is the thrombotic occlusion of the extrahepatic portal system, which can propagate towards the vena caval system. Although rare, it occurs primarily in those with cirrhosis, intra-abdominal infections, malignancy, or hypercoagulable disorders. This report describes the first reported case of a soldier within special operations without identifiable risk factors who was found to have a completely occlusive portal vein thrombosis after approximately 10 days of insidious abdominal pain. This case emphasizes the importance of considering this rare but dangerous pathology among this highly screened and capable special operations population.

Identification of the suprachiasmatic nucleus venous portal system in the mammalian brain

There is only one known portal system in the mammalian brain - that of the pituitary gland, first identified in 1933 by Popa and Fielding. Here we describe a second portal pathway in the mouse linking the capillary vessels of the brain’s clock suprachiasmatic nucleus (SCN) to those of the organum vasculosum of the lamina terminalis (OVLT), a circumventricular organ. The localized blood vessels of portal pathways enable small amounts of important secretions to reach their specialized targets in high concentrations without dilution in the general circulatory system. These brain clock portal vessels point to an entirely new route and targets for secreted SCN signals, and potentially restructures our understanding of brain communication pathways.