Liver transplantation for hilar inflammatory myofibroblastic tumor: Case report and review of the literature.

BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) of the liver are rare neoplasms. These tumors are difficult to distinguish from other neoplasms by radiological examination, have uncertain evolution, and there is no consensus on the treatment of these lesions. Hilar tumors can involve the portal vein, hepatic artery, bile duct, and spread to the branches of the portal triad, causing obstructive symptoms, occlusive phlebitis, and portal hypertension. Thus, liver transplantation (LT) is an excellent alternative for locally advanced hilar tumors, since it ensures complete tumor resection with free margins. METHODS/RESULTS: We present a literature review and a case report showing a 3-year-old boy with liver IMT invading the hepatic hilum and inferior vena cava, who underwent a successful living donor liver transplantation that required portal vein and vena cava replacement. CONCLUSION: The incidence of hilar IMTs is low and there is no well-established standard treatment. Liver transplantation for a hilar tumor with vascular invasion was acceptable in this case because the radically of the surgery was the key for the cure, and because the tumor was benign in nature.

Anterior hepatic resection: A simple and safe technique for reducing the antero-posterior diameter of the liver graft for small pediatric recipients.

BACKGROUND: The techniques involved in neonatal and infantile transplantation require approaches that can sculpt a left lateral segment (LLS) to the right shape and size and avoid large-for-size syndrome. The aim of this article is to describe the anterior hepatic resection (AHR) of the LLS in pediatric LDLT. METHODS: A retrospective anatomical study of preoperative image studies, description of the technique for AHR, and short-term results. RESULTS: The AHR was performed in eight cases. All donors were male, with average age, BW, and BMI of 28.3 ± 5.9 years, 74.2 ± 9.3 kg, and 24.3 ± 2.6 kg/m2, respectively. Donors were discharged at an average of 3.6 ± 0.8 days. The median recipient age and BW at transplantation were 6.9 (2.7 to 11) months and 5.9 (3.9 to 8) kg, respectively, and the recipient-to-donor body weight ratio (RDBW) was <0.1 in all but one case. The mean percentage reduction in graft weight and in the antero-posterior diameter were 33.2% ± 5.5% and 38.3% ± 12.6%, respectively. The average (SD) GRWR was 4.8% ± 1.7% before all the resections and 3.5% ± 1.0% after the procedures. Seven patients were primarily closed. CONCLUSION: After LLS resection, a nonanatomical anterior resection of the LLS was accomplished without hilar vascular dissection to segments II/III. The final liver graft allowed primary abdominal wall closure in all but one patient, with meaningful adjustments in GRWR. AHR proved to be simple, safe, reproducible, and effective in the presented case series.

Intestinal complications are common in patients with acquired diaphragmatic hernia after pediatric living donor liver transplantation.

BACKGROUND: Acquired diaphragmatic hernia (DH) following liver transplantation (LT) is usually considered a surgical emergency. Interplay of contributing elements determines its occurrence but, in children, LT with partial liver grafts seems to be the most important causative factor. METHODS: This retrospective study describes the clinical scenario and outcomes of 11 patients with acquired DH following LDLT. RESULTS: During the study period, 1109 primary pediatric LDLT were performed (0.8% DH). The median age and BW of the recipients with DH at transplantation were 17 months and 11.1 kg, respectively; 63.7% of the cases had a weight/age Z-score of less than -2 at transplantation. The median interval between transplantation and diagnosis of DH was 114 days (32-538 days). A total of 6 (54.5%) of the patients had bowel obstruction due to bowel migration into the hemithorax. Ten defects were right-sided. Three patients required enterectomy and enterorrhaphy. Two patients required a new bilioenteric anastomosis, and one of them had complete necrosis of the Roux-in-Y limb. The patient with left-side DH presented gastroesophageal perforation. CONCLUSION: Most defects necessitate primary closure as the first treatment, and recurrence is rare. The associated problems encountered, especially related to intestinal complications, can determine increased morbidity following DH repair. Early diagnosis and intervention are required for achieving better outcomes.

Whole-body (99m)Tc-octreotide scintigraphy with SPECT/CT to detect occult tumor inducing paraneoplastic osteomalacia.

A 32-year-old woman presented with progressive myalgia, bone pain, fatigue, insufficiency hip fractures, high urine phosphate, and low serum phosphate and vitamin D levels. These findings were suggestive of oncogenic osteomalacia. A whole-body Tc-octreotide scintigraphy with SPECT/CT showed uptake on a sclerotic intramedullary lesion in the left medial tibia plateau. MRI depicted a solid lesion. The lesion was surgically removed; the patient became asymptomatic, and follow-up laboratory results normalized. Histopathologic examination revealed a vascular hemangiopericytoma-like tumor, positive for somatostatin receptor (SSR-2). Whole-body Tc-octreotide scintigraphy with SPECT/CT may detect occult oncogenic osteomalacia tumors.

Walled-off pancreatic necrosis and other current concepts in the radiological assessment of acute pancreatitis.

Acute pancreatitis is an inflammatory condition caused by intracellular activation and extravasation of inappropriate proteolytic enzymes determining destruction of pancreatic parenchyma and peripancreatic tissues. This is a fairly common clinical condition with two main presentations, namely, endematous pancreatitis - a less severe presentation -, and necrotizing pancreatitis - the most severe presentation that affects a significant part of patients. The radiological evaluation, particularly by computed tomography, plays a fundamental role in the definition of the management of severe cases, especially regarding the characterization of local complications with implications in the prognosis and in the definition of the therapeutic approach. New concepts include the subdivision of necrotizing pancreatitis into the following presentations: pancreatic parenchymal necrosis with concomitant peripancreatic tissue necrosis, and necrosis restricted to peripancreatic tissues. Moreover, there was a systematization of the terms acute peripancreatic fluid collection, pseudocyst, post-necrotic pancreatic/peripancreatic fluid collections and walled-off pancreatic necrosis. The knowledge about such terms is extremely relevant to standardize the terminology utilized by specialists involved in the diagnosis and treatment of these patients.

A pancreatite aguda é uma condição inflamatória causada por ativação intracelular e extravasamento inapropriado de enzimas proteolíticas que determinam destruição do parênquima pancreático e dos tecidos peripancreáticos. Consiste em uma condição clínica bastante frequente, identificando-se duas formas principais de apresentação: a forma edematosa, menos intensa, e a forma necrosante, a forma grave da doença que acomete uma proporção significativa dos pacientes. A avaliação radiológica, sobretudo por tomografia computadorizada, tem papel fundamental na definição da conduta nos casos graves, sobretudo no que diz respeito à caracterização das complicações locais, que têm implicação prognóstica, e na determinação do tipo de abordagem terapêutica. Novos conceitos incluem a subdivisão da pancreatite necrosante nas formas de necrose do parênquima pancreático concomitante com necrose dos tecidos peripancreáticos ou necrose restrita aos tecidos peripancreáticos. Além disso, houve sistematização dos termos: acúmulos líquidos agudos peripancreáticos, pseudocisto, alterações pós-necróticas pancreáticas/peripancreáticas e necrose pancreática delimitada. Tal conhecimento é de extrema relevância no sentido de uniformizar a linguagem entre os especialistas envolvidos no diagnóstico e tratamento desses pacientes.

Necrose pancreática delimitada e outros conceitos atuais na avaliação radiológica da pancreatite aguda / Walled-off pancreatic necrosis and other current concepts in the radiological assessment of acute pancreatitis

A pancreatite aguda é uma condição inflamatória causada por ativação intracelular e extravasamento inapropriado de enzimas proteolíticas que determinam destruição do parênquima pancreático e dos tecidos peripancreáticos. Consiste em uma condição clínica bastante frequente, identificando-se duas formas principais de apresentação: a forma edematosa, menos intensa, e a forma necrosante, a forma grave da doença que acomete uma proporção significativa dos pacientes. A avaliação radiológica, sobretudo por tomografia computadorizada, tem papel fundamental na definição da conduta nos casos graves, sobretudo no que diz respeito à caracterização das complicações locais, que têm implicação prognóstica, e na determinação do tipo de abordagem terapêutica. Novos conceitos incluem a subdivisão da pancreatite necrosante nas formas de necrose do parênquima pancreático concomitante com necrose dos tecidos peripancreáticos ou necrose restrita aos tecidos peripancreáticos. Além disso, houve sistematização dos termos: acúmulos líquidos agudos peripancreáticos, pseudocisto, alterações pós-necróticas pancreáticas/peripancreáticas e necrose pancreática delimitada. Tal conhecimento é de extrema relevância no sentido de uniformizar a linguagem entre os especialistas envolvidos no diagnóstico e tratamento desses pacientes.

Acute pancreatitis is an inflammatory condition caused by intracellular activation and extravasation of inappropriate proteolytic enzymes determining destruction of pancreatic parenchyma and peripancreatic tissues. This is a fairly common clinical condition with two main presentations, namely, endematous pancreatitis - a less severe presentation -, and necrotizing pancreatitis - the most severe presentation that affects a significant part of patients. The radiological evaluation, particularly by computed tomography, plays a fundamental role in the definition of the management of severe cases, especially regarding the characterization of local complications with implications in the prognosis and in the definition of the therapeutic approach. New concepts include the subdivision of necrotizing pancreatitis into the following presentations: pancreatic parenchymal necrosis with concomitant peripancreatic tissue necrosis, and necrosis restricted to peripancreatic tissues. Moreover, there was a systematization of the terms acute peripancreatic fluid collection, pseudocyst, post-necrotic pancreatic/peripancreatic fluid collections and walled-off pancreatic necrosis. The knowledge about such terms is extremely relevant to standardize the terminology utilized by specialists involved in the diagnosis and treatment of these patients.