ABSTRACT
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.
Subject(s)
Humans , Child , Exophthalmos , Vascular Malformations/therapy , Vascular Malformations/diagnostic imaging , Orbit/blood supply , Orbit/pathology , Visual Acuity , Hemorrhage/pathologyABSTRACT
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.
Subject(s)
Exophthalmos , Vascular Malformations , Humans , Child , Vascular Malformations/diagnostic imaging , Vascular Malformations/therapy , Orbit/blood supply , Orbit/pathology , Visual Acuity , Hemorrhage/pathologyABSTRACT
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.
Subject(s)
Lymphatic Abnormalities/therapy , Scrotum/surgery , Sirolimus/therapeutic use , Vascular Malformations/therapy , Administration, Oral , Child , Child, Preschool , Humans , Infant , Male , Postoperative Care , Premedication , Retrospective Studies , Scrotum/blood supply , Wound HealingABSTRACT
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.
