Incidence, management and outcomes in hepatic artery complications after paediatric liver transplantation: protocol of the retrospective, international, multicentre HEPATIC Registry.

INTRODUCTION: Hepatic artery complications (HACs), such as a thrombosis or stenosis, are serious causes of morbidity and mortality after paediatric liver transplantation (LT). This study will investigate the incidence, current management practices and outcomes in paediatric patients with HAC after LT, including early and late complications. METHODS AND ANALYSIS: The HEPatic Artery stenosis and Thrombosis after liver transplantation In Children (HEPATIC) Registry is an international, retrospective, multicentre, observational study. Any paediatric patient diagnosed with HAC and treated for HAC (at age <18 years) after paediatric LT within a 20-year time period will be included. The primary outcomes are graft and patient survivals. The secondary outcomes are technical success of the intervention, primary and secondary patency after HAC intervention, intraprocedural and postprocedural complications, description of current management practices, and incidence of HAC. ETHICS AND DISSEMINATION: All participating sites will obtain local ethical approval and (waiver of) informed consent following the regulations on the conduct of observational clinical studies. The results will be disseminated through scientific presentations at conferences and through publication in peer-reviewed journals. TRIAL REGISTRATION NUMBER: The HEPATIC registry is registered at the ClinicalTrials.gov website; Registry Identifier: NCT05818644.

Hemoptisis secundaria a tuberculosis pulmonar complicada con pseudoaneurisma de Rasmussen. Reporte de un caso en pediatría. / Hemoptysis in Pulmonary Tuberculosis Complicated by Rasmussen's Pseudoaneurysm. A Pediatric Case Report.

Presentamos el caso de un niño de 12 años que consultó por hemoptisis, sin otros sín- tomas asociados. Se realizó radiografía de tórax (patológica), laboratorio con aumen- to moderado de reactantes de fase aguda, PPD (negativa), esputos x 3 con bacilosco- pias negativas y tomografía de tórax con contraste i.v. que mostró imágenes de árbol en brote en todos los lóbulos y una imagen de dilatación vascular de una rama de la ar- teria pulmonar en lóbulo superior izquierdo. Se plantearon diagnósticos diferenciales: malformación vascular primaria o lesión secundaria a infección. La angiografía digital permitió confirmar el pseudoaneurisma y embolizarlo. Luego de 17 días, 2/3 cultivos de esputo fueron positivos para Mycobacterium tuberculosis. El niño realizó tratamiento antituberculoso con drogas de primera línea con evolución clínica favorable. Este caso resalta la importancia de considerar el pseudoaneurisma de Rasmussen en- tre las posibles complicaciones de un paciente con tuberculosis y hemoptisis recurren- te o masiva.

We present the case of a 12-year-old boy admitted to the hospital due to hemoptysis without other symptoms. We performed a Thorax X-Ray (pathological), laboratory with elevated acute phase reactants, TST (negative), sputum x 3 with negative smear and computed tomography angiography showing a tree-in-bud pattern in all lobes, and di-latation of a brunch of the pulmonary artery in the upper left lobe. We considered pri-mary vascular anomaly or lesion due to infection as a differential diagnosis. The patient underwent digital angiography and therapeutic embolization of this pseudoaneurysm. After seventeen days, 2/3 of the sputum cultures were positive for Mycobacterium tu-berculosis. The patient received standard anti-TB therapy with favorable evolution. This case highlights the importance of considering complications such as Rasmussen's pseudoaneurysm in patients with pulmonary tuberculosis and recurrent or massive hemoptysis.

Prevalence, management and efficacy of treatment in portal vein obstruction after paediatric liver transplantation: protocol of the retrospective international multicentre PORTAL registry.

INTRODUCTION: Portal vein obstruction (PVO) consists of anastomotic stenosis and thrombosis, which occurs due to a progression of the former. The aim of this large-scale international study is to assess the prevalence, current management practices and efficacy of treatment in patients with PVO. METHODS AND ANALYSIS: The Portal vein Obstruction Revascularisation Therapy After Liver transplantation registry will facilitate an international, retrospective, multicentre, observational study, with 25 centres around the world already actively involved. Paediatric patients (aged <18 years) with a diagnosed PVO between 1 January 2001 and 1 January 2021 after liver transplantation will be eligible for inclusion. The primary endpoints are the prevalence of PVO, primary and secondary patency after PVO intervention and current management practices. Secondary endpoints are patient and graft survival, severe complications of PVO and technical success of revascularisation techniques. ETHICS AND DISSEMINATION: Medical Ethics Review Board of the University Medical Center Groningen has approved the study (METc 2021/072). The results of this study will be disseminated via peer-reviewed publications and scientific presentations at national and international conferences. TRIAL REGISTRATION NUMBER: Netherlands Trial Register (NL9261).

Actualización en anomalías vasculares de la órbita en pediatría: diagnóstico por imágenes y tratamiento / Update on orbital vascular anomalies in pediatrics: imaging studies and management

Las anomalías vasculares de la órbita (AVO) son un grupo heterogéneo de patologías que pueden presentarse con frecuencia en el cono orbitario, la región periorbitaria o dentro de la órbita misma. Las AVO se dividen en tumores y malformaciones. Su presentación clínica más frecuente es el exoftalmos, asociado o no a alteración del eje visual. Además, pueden presentar complicaciones agudas, como hemorragia intralesional o celulitis entre las más frecuentes, y complicaciones crónicas, como ambliopía y afectación de la agudeza visual a largo plazo. La evolución de las técnicas de imágenes, el uso de nuevos fármacos y la utilización de innovadores procedimientos en radiología intervencionista han posibilitado obtener una mejora significativa en los procesos diagnósticos y terapéuticos de estos pacientes, permitiendo un diagnóstico y tratamiento preciso.

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment. The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management.

Update on orbital vascular anomalies in pediatrics: imaging studies and management. / Actualización en anomalías vasculares de la órbita en pediatría: diagnóstico por imágenes y tratamiento.

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment. The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management.

Las anomalías vasculares de la órbita (AVO) son un grupo heterogéneo de patologías que pueden presentarse con frecuencia en el cono orbitario, la región periorbitaria o dentro de la órbita misma. Las AVO se dividen en tumores y malformaciones. Su presentación clínica más frecuente es el exoftalmos, asociado o no a alteración del eje visual. Además, pueden presentar complicaciones agudas, como hemorragia intralesional o celulitis entre las más frecuentes, y complicaciones crónicas, como ambliopía y afectación de la agudeza visual a largo plazo. La evolución de las técnicas de imágenes, el uso de nuevos fármacos y la utilización de innovadores procedimientos en radiología intervencionista han posibilitado obtener una mejora significativa en los procesos diagnósticos y terapéuticos de estos pacientes, permitiendo un diagnóstico y tratamiento preciso.

Mechanochemical Endovenous Ablation of Varicose Veins in Pediatric Patients with Klippel-Trénaunay Syndrome: Feasibility, Safety, and Initial Results.

PURPOSE: To evaluate feasibility, safety, and results of endovenous mechanochemical ablation (MOCA) for treatment of persistent embryonic and dysplastic veins in pediatric patients with Klippel-Trénaunay syndrome (KTS). MATERIALS AND METHODS: Thirteen MOCA procedures were performed in 11 patients (age range, 4-16 years) with KTS and symptomatic persistent embryonic or dysplastic veins during a 24-month period. All patients were evaluated with color Doppler (CD) ultrasound (US), contrast-enhanced MR imaging, and venography to assess the anatomy of the target vessels and patency of the deep venous system. All procedures were performed under general anesthesia with a ClariVein catheter and liquid sodium tetradecyl sulfate as the sclerosing agent. US and fluoroscopic guidance were used in all cases. Technical success rate, primary occlusion rate, adverse effects, and recanalization rates were evaluated. Clinical and radiological (CD US) controls were performed 1 day, 7 days, 1 month, and 6 months after the procedure and once a year thereafter, with a mean follow-up of 16 months (range, 6-25 months). RESULTS: Technical success and primary occlusion were achieved in all patients with no adverse events. During the follow-up period, CD US demonstrated partial recanalization and symptom recurrence in 2 patients (18%), 14 and 18 months after the initial procedure. These 2 patients had a second ablation procedure with no recanalization or symptom recurrence during the subsequent follow-up period. CONCLUSIONS: MOCA is feasible and appears to be a safe and effective technique for treatment of varicose veins in pediatric patients with KTS.

The Impact of Adjuvant Sirolimus Therapy in the Surgical Management of Scrotal Slow-Flow Vascular Malformations.

OBJECTIVE: To describe the effects of oral sirolimus administered before and after surgical resection of slow-flow vascular malformations of the scrotum in pediatric patients. METHODS: Retrospective review of 3 patients presenting with complex lymphatic-venous malformations of the scrotum who received adjuvant oral sirolimus 3 months before and 3 months after surgical resection. Demographic data, clinical course, imaging findings, and management strategies were reviewed for each patient. RESULTS: In each of the 3 patients, there was a significant volume reduction of the lesion within the 3 months after initial dose of sirolimus. Scarce lymphatic leakage during and after surgery was reported, associated with an adequate wound healing. Two years after the last postsurgical dose of sirolimus, all patients remain asymptomatic without any lymphatic leakage or lesion recurrence. CONCLUSION: Combined lymphatic-venous vascular malformations of the male genitalia are rare but associated with high morbidity and challenging treatment options. Pre- and postsurgical adjuvant treatment with oral sirolimus seems to be a promising therapeutic option that provides reduction of the lesion size before surgery and improvement of postsurgical recovery and wound healing.

Guía de práctica clínico-quirúrgica para hemorragia digestiva baja / Clinical practice guide in lower gastrointestinal bleeding

El objetivo de esta guía clínico-quirúrgica es homogeneizar conceptos y conductas para el manejo de la hemorragia digestiva baja (HDB), con el fin de protocolizar y unificar el tratamiento multidisciplinario de dicha patología. Durante el manejo inicial del paciente con HDB, resulta prioritario determinar la estabilidad hemodinámica. Cuando el paciente se presenta hemodinámicamente estable, la videocolonoscopia (VCC) es el método diagnóstico de elección, la cual debe realizarse con preparación colónica y dentro de las 48 horas, mientras que si se trata de un paciente inestable que no responde a la reanimación debe realizarse, de ser posible, angiotomografía para localizar el sitio de sangrado y posteriormente angiografía. Si las condiciones no lo permiten, se procede directamente a la cirugía de urgencia. Si se logra reanimar al paciente, el método diagnóstico de elección es la videoendoscopia digestiva alta (VEDA). Si la VEDA es negativa y el paciente permanece estable, se prosigue con VCC. Por el contrario, si continúa sangrando, el paso siguiente es la angio-TC. En caso de localizar el sitio de sangrado, se realiza angiografía terapéutica. Si falla o la angio-TC es negativa, tiene indicación de cirugía. Esta guía fue consensuada a partir de la bibliografía, guías internacionales y la experiencia de los Servicios de Cirugía General, Coloproctología, Gastroenterología, Diagnóstico por Imágenes y Hemodinamia.(AU)

Neurophysiological Monitoring in Radiofrequency Ablation of Spinal Osteoid Osteoma With a Progressive Time and Temperature Protocol in Children.

STUDY DESIGN: Retrospective. Level IV Evidence. OBJECTIVE: To assess the utility of intraoperative neurophysiological monitoring (IONM) to detect and eventually prevent impending neurovascular damage during computed tomography (CT)-guided radiofrequency ablation (RFA) of spinal osteoid osteoma (OO) in children. SUMMARY AND BACKGROUND DATA: To our knowledge, this is the first case series of spinal OO in pediatric patients treated at a single center employing IONM during RFA. METHODS: This is a retrospective study of seven consecutive patients (3 girls and 4 boys, mean age: 9 years 4 months) with imaging and clinical signs compatible with spinal OO who underwent CT-guided RFA, under general anesthesia, and IONM in a single center between 2011 and 2015. Before the RFA procedure, a CT-guided percutaneous biopsy of the nidus was performed in the same setting. RFA was divided into four cycles of increasing time and temperature and performed under IONM in every patient. RESULTS: Two patients had lesions located in the thoracic spine and five patients had lumbar involvement. The RFA technical and clinical success was 85.7%. Six patients presented with reversible neurophysiological changes either during biopsy needle positioning or RFA cycles. In the remaining case, as IONM changes did not improve after several minutes of neuroprotective hypertension, the procedure was interrupted. Neither neurologic nor vascular complications were observed after RFA treatment. In only one biopsy sample, OO was confirmed by histopathologic studies. CONCLUSION: CT-guided RFA is an accepted minimally invasive technique for the treatment of spinal OO in children. IONM may be a helpful tool that requires minimal additional time and provides feedback on the state of the spinal cord and nerves at risk during the procedure. We promote the use of IONM during these procedures to detect and possibly prevent impending neurologic damage. LEVEL OF EVIDENCE: Level IV.

Isolated plexiform neurofibroma mimicking a vascular lesion.

Plexiform neurofibromas are benign tumors originating from peripheral nerve sheaths, generally associated with Neurofibromatosis Type 1 (NF1). They are diffuse, painful and sometimes locally invasive, generating cosmetic problems. This report discusses an adolescent patient who presented with an isolated, giant plexiform neurofibroma on her leg that was confused with a vascular lesion due to its clinical aspects. Once the diagnosis was confirmed by surgical biopsy, excision of the lesion was performed with improvement of the symptoms.

Malformaciones vasculares: actualización en diagnóstico por imágenes y tratamiento / Vascular malformations: an update on imaging and treatment

Las malformaciones vasculares componen un amplio y heterogéneo espectro de lesiones, que frecuentemente se presentan como un desafío diagnóstico y terapéutico para el pediatra. El uso de una nomenclatura inadecuada durante mucho tiempo ha llevado a confusión. Dado que el tratamiento de esta patología depende de cada tipo de malformación vascular, su correcta clasificación e identificación es crucial. El objetivo es brindar la información necesaria sobre la clasificación y denominación actual de las malformaciones vasculares y los conceptos básicos sobre las herramientas disponibles para el diagnóstico y tratamiento de esta compleja patología.

Vascular malformations comprise a broad and heterogeneous range of lesions that often represent a diagnostic and therapeutic challenge for the pediatrician. For a long time, the use of an inaccurate nomenclature has led to confusion. Since management depends on the specific vascular malformation, a proper classification and identification is critical. The objective of this article is to provide the necessary information about the current classification and terminology of vascular anomalies, including basic concepts about available imaging diagnostic and therapeutic tools for the management of such complex condition.

Isolated plexiform neurofibroma mimicking a vascular lesion

Abstract Plexiform neurofibromas are benign tumors originating from peripheral nerve sheaths, generally associated with Neurofibromatosis Type 1 (NF1). They are diffuse, painful and sometimes locally invasive, generating cosmetic problems. This report discusses an adolescent patient who presented with an isolated, giant plexiform neurofibroma on her leg that was confused with a vascular lesion due to its clinical aspects. Once the diagnosis was confirmed by surgical biopsy, excision of the lesion was performed with improvement of the symptoms.

[Endovascular treatment of an hepatic artery pseudoaneurysm following pancreaticoduodenectomy]. / Tratamiento endovascular de un pseudoaneurisma de la arteria hepática posterior a duodenopancreatectomía.

Pseudoaneurysm formation is a serious complication in the context ofa pancreatic resection, reaching out a high mortality rate. Classically, surgery was the gold standard of treatment, but nowadays endovascular approach has been accepted as the first treatment option. The use of covered stents seems to be a safe and effective tool to treat this serious complication.

Ophthalmic artery microcatheterization for research purposes in pigs. A technical note.

PURPOSE: To describe a technique for micro catheterization of the external ophthalmic artery (EO) in pigs for investigational and training purposes. METHODS: Carotid angiography was performed in seven male domestic pigs. The external ophthalmic artery was reached with a microcatheter in order to administer a neoplastic drug in the eye. RESULTS: The external ophthalmic artery could be found arising from the infraorbital (IO) artery in the bend of the internal maxillary (IM) artery. It could be reached in every animal. CONCLUSION: Following anatomic landmarks of the external carotid (EC) artery the ophthalmic artery can be easily reached and catheterized for training and investigational purposes.

Granulocytic sarcoma of the urinary bladder in a pediatric patient.

Granulocytic sarcoma (GS) is a rare tumor consisting of immature cells of granulocytic lineage. It is also called chloroma, referring to the green color of the tumor caused by high levels of myeloperoxidase in the cells. GS is often associated with acute myeloblastic leukemia. We report the case of a 4-year-old boy with a diagnosis of acute myeloblastic leukemia. Abdominal ultrasonography demonstrated a solid, vascularized, heterogeneous, polypoid formation involving the posterior wall of the bladder. Further studies confirmed the etiology of the tumor. On control ultrasonography, a marked decrease in tumor size 15 days after treatment was revealed. We describe the imaging findings in this patient and review the literature about this infrequent entity. Although imaging findings are not specific and considering that extramedullary involvement in leukemia is very sensitive to treatment, inclusion of GS in the differential diagnosis and awareness of the possible sites of occurrence and imaging features is essential to avoid unnecessary therapeutic measures.

Hibernoma: tumor retroperitoneal de grasa parda: Informe de un caso pediátrico / Hibernoma: brown fat retroperitoneal tumor: Report of a pediatric case

El hibernoma es un raro tumor benigno de los tejidos blandos, compuesto por grasa parda, tejido que predomina en animales que hibernan y de allí su nombre. Debido a su infrecuencia en Pediatría y a su diagnóstico complejo, presentamos un paciente de 3 meses de edad, con diagnóstico de tumor abdominal. Los exámenes por ecografía y tomografía computada demostraron un tumor retroperitoneal de carácter infltrativo, vascularizado y con características lipomatosas. La exéresis del tumor permitió arribar al diagnóstico histopatológico de hibernoma o tumor de grasa parda. En esta presentación se describen las características del hibernoma, como tumor infrecuente en niños, y se revisan los tumores de tejido graso, según su frecuencia en Pediatría.

Hibernoma is a rare benign tumor of soft tissue, composed of brown fat. This tissue is predominant in hibernating animals and hence its name. Because of its rarity in Pediatrics and diffcult diagnosis, we report a 3 month-old patient with a diagnosis consistent with an abdominal tumor. Ultrasound and computed tomography exams showed an infltrative retroperitoneal tumor, with hypervascular and lipomatous features. After tumor excision, histopathological exam confrmed the diagnosis of hibernoma or brown fat tumor. This presentation describes the characteristics of this type of tumor, rare in children, and reviews the fatty tumors, according to their frequency in pediatric patients.

Transhepatic venous approach for balloon-assisted cervical collateral venous access.

Central venous catheter placement is indicated in many situations, and an increasing number of patients require temporary and long-term central catheters. Frequently, patients who have undergone multiple central veins catheterizations develop complete and diffuse venous occlusion, and this constitutes a difficult-to-manage clinical problem. We report a case of a 20-year-old patient who was referred to our department for central venous line placement who manifested bilateral femoral, jugular, and subclavian veins occlusion. A central venous catheter was implanted through a cervical collateral vein, targeting on and puncturing an angioplasty balloon, and advanced into the collateral vein through a transhepatic venous access.

Asymptomatic leukemic-cell infiltration of the pancreas: US findings.

Pancreatic infiltration of leukemic cells is a very rare manifestation at the onset of acute lymphoblastic leukemia (ALL) in childhood. Pancreatic enlargement in this situation is unusual and pancreatic involvement is often associated with biliary obstruction, cholestasis and pancreatitis. We report a 3-month-old girl who presented with asymptomatic leukemic infiltration of the pancreas, demonstrated by US with heterogeneous pancreatic enlargement associated with multiple hypoechogenic lesions, without cholestasis. Although these manifestations are rare, ALL should be considered a cause of pancreatic enlargement.