Pediatric Interventional Neuroradiology.

Pediatric neurovascular disorders can lead to substantial mortality and morbidity if not diagnosed early and adequately managed. Children with neurovascular diseases cannot be treated as small adults as the vascular and central nervous system anatomy, physiology, and pathologies in children differ greatly from those of adults. In addition, some neurovascular pathologies are seen exclusively in children such as aneurysmal malformation of the vein of Galen, pial fistulas, and dural fistulas in the context of dural sinus disease. In this review, we aim to present an overview of the common pediatric neurovascular diseases along with their endovascular management.

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.

Ultrasonography, laboratory, and cholangiography correlation of biliary complications in pediatric liver transplantation.

The aim of this study is to correlate the US, laboratory, and cholangiography findings in pediatric liver transplant patients with biliary complications, trying to identify reliable decision-making tools for the management of these complications. Retrospective review was carried out of US results in 39 consecutive patients, from 2011 to 2013, with biliary complications after LT, documented by PTC. According to US biliary dilation, patients were classified as: mild, moderate, and severe, and according to laboratory findings as: normal or abnormal serum bilirubin and level of serum GGT. Data were correlated with PTC findings, divided in three groups: mild, moderate, and severe/occlusive BDS. There was no statistically significant correlation between the US findings and the laboratory findings and between US findings with PTC. There was a statistically significant correlation between GGT and cholangiography. In our series, abnormal US could not predict the severity of BDS on PTC. Bilirubin results were not able to predict the US findings either. GGT results demonstrated a statistically significant correlation with the severity of BDS found on PTC. These findings emphasize the role of GGT in the evaluation and decision of biliary interventions in pediatric liver transplant recipients.

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.

Intracranial arteriovenous shunts in infants: A decade of experience from a quaternary pediatric center.

BACKGROUND AND PURPOSE: Intracranial arteriovenous shunts (IAVS) are rare vascular diseases in infants. They can be categorized into vein of Galen aneurysmal malformation (VGAM), pial arteriovenous fistula (PAVF), and dural arteriovenous fistula associated with dural sinus malformation (DAVF/DSM). We sought to review the clinical presentation, imaging characteristics, endovascular treatment (EVT), and outcomes of IAVS in infants presenting to a quaternary pediatric referral center over one decade. METHODS: A retrospective review of a prospectively maintained database was performed of all infants diagnosed with IAVS between January 2011 and January 2021 in a quaternary pediatric referral center. For each patient, demographic data, clinical presentation, imaging findings, management strategies, and outcomes were reviewed and discussed. RESULTS: Over the study period, 38 consecutive infants were diagnosed with IAVS. Patients with VGAM (23/38, 60.5%) presented with congenital heart failure (CHF) (14/23), hydrocephalus (4/23), and seizures (2/23), and three patients were asymptomatic. Eighteen patients with VGAM underwent EVT. Among those, 13 patients (72.2%) were successfully treated with an angiographic cure and three patients (3/18, 17%) died. Patients with PAVF (9/38, 23.7%) presented with CHF (5/9), intracranial hemorrhage (2/9), and seizures (2/9), and all of them were successfully treated endovascularly. Patients with Type I DAVF/DSM (4/6, 66.6%) presented with mass effect (2/4), cerebral venous hypertension (1/4), CHF (1/4), and cerebrofacial venous metameric syndrome (1/4). Patients with type II DAVF/DSM (2/6, 33.3%) presented with a thrill behind the ear. Patients with DAVF/DSM were treated endovascularly, five patients were cured, and one with type I DAVF/DSM died. CONCLUSION: Intracranial arteriovenous shunts are rare but potentially life-threatening neurovascular pathologies in infants. Endovascular treatment is challenging but feasible in carefully selected patients.

Clinical presentation of retinoblastoma in a middle-income country.

The presenting features of retinoblastoma in developing countries and their correlation with disease stage and patient survival are poorly known and they may be useful as background information for planning early diagnosis initiatives. Therefore, we undertook a retrospective review of 508 patients (467 evaluable, 296 unilateral) treated in Argentina from 1988 to 2008. Patients presented at an older age than reported from high-income countries [mean age 24 mo (range, 0 to 165 mo), 31 mo for unilateral (range, 0 to 165 mo), and 13.3 mo (range, 0 to 62 mo) for bilateral disease]. Leukocoria was the most common presenting sign (n=402, 86%). Strabismus was the only complaint in 25 (5.3%) patients. Forty-two patients (9%) presented with an enlarged eyeball and 37 (7.9%) with a red eye. Retinoblastoma was diagnosed in 22 (4.7%) asymptomatic children. These patients and those with strabismus alone were significantly younger and had a significantly better survival. Children presenting with enlarged eyeballs were significantly older and had significantly lower survival. In multivariable analysis older age and presentation with enlarged eyeballs were independently associated to advanced stage and mortality (P<0.001). Retinoblastoma is diagnosed in later stages in our setting and presentation with eye enlargement and increasing age at diagnosis correlate with worse outcome.

Congenital arterioportal fistulas: radiological treatment and color Doppler US follow-up.

BACKGROUND: Congenital intrahepatic arterioportal fistulas (APFs) are a rare cause of portal hypertension in children. Doppler US is a useful diagnostic imaging modality. Transarterial embolization is a minimally invasive and effective therapy allowing occlusion of the fistula and restoration of liver hemodynamics. OBJECTIVE: To describe the clinical and radiologic findings, percutaneous treatment and role of D-US in the postembolization follow-up of children with APF. MATERIALS AND METHODS: Between 2002 and 2011, four children with APF were treated. Initial diagnosis and follow-up was performed with D-US and confirmed by arteriography, followed by endovascular embolization in all patients. RESULTS: D-US demonstrated abnormal arterioportal communications in all patients. Six endovascular procedures were performed in these four children. In two children, no residual fistula was seen on D-US after the first procedure and symptoms resolved. In the other two children, D-US demonstrated residual flow through the fistula, with resolution of pathological D-US findings and symptoms after the second endovascular procedure. All four children were successfully treated and asymptomatic at the end of follow-up. The mean follow-up was 24 months. CONCLUSION: Interventional radiology has a key role in the treatment of congenital APF. D-US is a noninvasive and effective tool for the diagnosis and follow-up of these patients.

Direct carotid artery access for neurointerventional procedures in infants.

BACKGROUND: Femoral access is the primary route for neurointerventional procedures in children. However, endovascular treatment may not always be possible through a femoral approach, necessitating conversion to alternative access routes. OBJECTIVE: To review the feasibility and safety of direct carotid puncture (DCP) in infants undergoing neuroendovascular interventions. METHODS: We conducted a retrospective review of all infants who underwent DCP as the access route to treat neuroendovascular pathologies between January 2011 and January 2021. Patients' demographics, clinical presentation, imaging findings, and technical details were reviewed. RESULTS: Between January 2011 and January 2021, five infants aged between 28 and 150 days underwent DCP out of 1129 neuroendovascular interventions performed in our institution (0.4%). All five infants (100%) were diagnosed with intracranial fistulas and were found to have severe tortuosity of the cervical arteries. DCP was performed as the initial access route in 2/5 patients and as crossover after a failed femoral attempt in 3/5 patients. DCP was performed under ultrasound guidance in all patients. Closure was performed by manual compression, without complications. Ultrasound showed patent cervical vessels in all patients at 3 months' follow-up. CONCLUSION: Direct carotid access is a feasible and safe alternative route to treat neuroendovascular pathologies in infants and can be considered in cases of inaccessible or failed transfemoral access or in cases with severe arterial tortuosity in infants with intracranial fistulas.

Surgical treatment of thoracic outlet syndrome in pediatrics.

AIM: Thoracic outlet syndrome (TOS) is a condition that occurs when the cervical neuro-vascular bundle becomes compressed at one of the three narrow areas of the thoraco-cervico-axillary region. Conservative management is the first line of treatment. Patients who do not respond to conservative management should be treated surgically. The aim of this review is to present our experience with the surgical management of TOS in pediatric patients. METHODS: We retrospectively reviewed the outcomes of all patients with TOS operated at our Hospital between 2001 and 2020. We collected all demographic data, clinical features, imaging data, type of operation performed, intraoperative findings, complications and recurrence. RESULTS: We operated 9 patients within the study period. The median age at surgery was 14 (7 to 17) years. A transaxillary approach was used in 7 patients and a supraclavicular approach in 2. There was only one minor intraoperative complication (violation of the pleural space). There were no postoperative complications. The median length of stay was 3 (2 to 4) days. All patients were extubated in the operating room. Two patients developed symptoms on the contralateral side. One of these underwent a successful contralateral transaxillary Roos operation. The follow-up was 4 months to 20 years. All patients are asymptomatic. CONCLUSION: We believe that the Roos operation is a safe and effective treatment with excellent long-term outcomes for children with TOS that fail conservative management.

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.

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.

Vascular malformations: an update on imaging and management.

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.

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.

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.

[Influenza A H1N1 respiratory infection: radiologic findings and correlation with clinical outcome in pediatric inpatients. A pediatric hospital experience]. / Infección respiratoria por influenza A H1N1 en niños: Hallazgos radiológicos y correlación con el pronóstico clínico en pacientes internados en un hospital pediátrico.

INTRODUCTION: In the year 2009, the World Health Organization declared the Influenza A H1N1 virus as a pandemic. It has been reported that the radiographic pattern in chest x-ray may predict the clinical outcome in patients affected. OBJECTIVE: To describe the chest x-ray findings in pediatrics patients with confirmed diagnosis of Influenza H1N1 respiratory infection and its correlation with clinical evolution. POPULATION AND METHODS: A retrospective, analytic and descriptive study of 47 pediatric inpatients with confirmed diagnosis of H1N1 influenza who had available chest x-ray was performed. Medical records were reviewed for underlying medical conditions and patient's outcome. RESULTS: 94% of patients had abnormal x-ray. Consolidation and ground-glass opacities had significantly higher frequency in patients who needed more days of oxygen supplement. Initial chest x-ray with lobar consolidation was observed in patients with greater risk of Intensive care unit admission. CONCLUSION: The most frequent radiological patterns found were ground-glass opacities and peribronchial markings. Lobar consolidation and ground-glass opacities are associated with adverse outcome.

Massive hematuria due to portal hypertension in a child with bladder augmentation for vesical exstrophy.

We report a case of a 17-year-old patient with severe hematuria from an ileocystoplasty-augmented bladder for vesical exstrophy, with associated cirrhosis secondary to autoimmune hepatitis, diagnosed years later. Varix development and further bleeding from ectopic varices, related to cirrhosis and portal hypertension, are a rare cause of hematuria in patients with surgically augmented bladder volume. This child was successfully treated with a transjugular intrahepatic portosystemic shunt procedure as a bridge to hepatic transplantation.