Life-threatening variceal bleeding after liver transplantation and extensive portal vein thrombosis: Desperate times call for desperate measures.

BACKGROUND: The management of complex, intra- and extrahepatic portal vein thrombosis (PVT) after liver transplantation (LT) is challenging. Although most of the patients remain asymptomatic or oligosymptomatic in the chronic setting, some of them may develop severe portal hypertension and related complications, notably gastrointestinal (GI) bleeding. In the emergency scenario, clinical and endoscopic treatments as well as intensive support constitute the bases of conservative management, while more definitive treatment options such as surgical shunting and retransplantation are related to high morbidity rates. Transjugular intrahepatic portosystemic shunt (TIPS) was largely considered of limited role due to technical difficulties arising from extensive PVT. Recently, however, new minimally invasive image-guided techniques emerged, allowing portal vein recanalization and TIPS creation simultaneously (TIPS-PVR), even in complex PVT pretransplant patients. METHODS: Herein, we describe a novel indication for TIPS-PVR in a post-LT adolescent presenting with life-threatening, refractory GI bleeding. RESULTS: The patient presented with complete resolution of the hemorrhagic condition after the procedure, with no deterioration of hepatic function or hepatic encephalopathy. Follow-up Doppler ultrasound after TIPS-PVR showed normal hepatopetal venous flow within the stents, and no evidence of complications, including intraperitoneal or peri splenic bleeding. CONCLUSIONS: This report describes the feasibility of TIPS-PVR in the post-LT scenario complicated by extensive PVT. In this case, a complete resolution of the life-threatening GI bleeding was achieved, with no major complications. Other patients with complex chronic PVT might benefit from the use of the described technique, but further studies are required to determine the correct timing and indications of the procedure, eventually before the occurrence of life-threatening complications.

Impact of hypoxemia on pediatric liver transplantation for hepatopulmonary syndrome.

BACKGROUND: The treatment of choice for patients with cirrhosis and HPS is LT. The clinical manifestations associated with hypoxemia result in limitations and a poor health-related quality of life of affected patients. The present report aims to study the differences in outcomes between patients with PaO2  < 50 mm Hg and those with PaO2  ≥ 50 mm Hg. METHODS: This was a retrospective study of 21 patients under 18 years of age conducted from 2001 to 2018; the patients were divided into 2 groups: G1-PaO2  ≥ 50 mm Hg, 11 patients, and G2-PaO2  < 50 mm Hg, 10 patients. Demographic, clinical, laboratory, and perioperative data; outcome variables; and post-transplant survival were compared between the groups. RESULTS: In total, 2/11 (18.2%) patients in G1 and 8/10 (80%) patients in G2 required supplemental oxygen therapy at home (P = .005). Patients in G2 required prolonged MV (median 8.5 days in G2 vs 1 day in G1, P = .015) and prolonged ICU and hospital stays (P = .002 and P = .001, respectively). Oxygen weaning time was longer in G2 (median 127.5 days) than in G1 (median 3 days; P = .004). One (9.1%) patient in G1 and three (30%) patients in G2 died (P = .22). The survival at 90 months was 90.9% in G1 and 70% in G2 (P = .22). CONCLUSION: The survival between groups was similar. Patients with very severe HPS required a longer MV time, longer ICU and hospital stays, and a longer O2 weaning time than those with mild, moderate, or severe HPS.

Schwannoma de reto associado à doença de Von Recklinghausen: relato de caso / Rectal Schwannoma associated to Von Recklinghausen disease: case story

Schwannomas são neoplasias de origem neurogênica benigna, com potencial de malignização, de crescimento lento, originárias das células de Schwann. São tumores raros que mais freqüentemente ocorrem intracranialmente no nervo acústico ou nos nervos espinhais. Podem ocorrer associados à Neurofibromatose tipo 1, que corresponde ao distúrbio clássico descrito por von Recklinghausen. Entre os pacientes com esta doença, 15-25por cento apresentam tumores neurais no trato gastrointestinal, envolvendo principalmente estômago e jejuno. É extremamente rara a ocorrência de Schwannoma em reto, principalmente na ausência de doença de von Recklinghausen. O presente trabalho é o relato de caso de um paciente do sexo masculino, com 42 anos de idade, portador de neurofibromatose de Von Recklinghausen e com queixas de hematoquesia e tenesmo retal, há três meses, quando procurou este serviço. Durante investigação diagnóstica, foi realizada colonoscopia que evidenciou tumoração retal, sendo indicada cirurgia. Realizou-se retossigmoidectomia anterior com anastomose colorretal primária com boa evolução. O exame anátomo-patológico da peça cirúrgica diagnosticou Schwannoma. Foram também biopsiadas as lesões cutâneas com diagnóstico anátomo patológico de neurofibroma. O paciente não apresenta metástases, tampouco recidiva, após 48 meses de acompanhamento.