Overview
Racemose hemangioma is a rare, benign vascular malformation characterized by dilated, tortuous blood vessels without capillary architecture, often involving episcleral, bronchial, or systemic circulations 13.Diagnosis
Clinical Presentation: Dilated, tortuous blood vessels visible externally or detected via imaging 13.
Fluorescein Angiography: Reveals rapid flow and large caliber, curvilinear, twisted configuration in episcleral cases 1.
Imaging: CT scans useful for detecting fistulas and dilated vessels in systemic locations 3.
Histopathology: In cases of livedo racemosa, biopsies show intraluminal thrombi without vasculitis 2.Management
Thoracic Endovascular Aortic Repair (TEVAR): For sealing bronchial arteries in cases with bronchial-pulmonary artery fistulas 3.
Coil Embolization: Adjunctive treatment for occluding affected pulmonary arteries 3.
No Specific Pharmacological Treatment Mentioned: Management primarily surgical or interventional radiological 13.Special Populations
No Specific Guidance Provided: Abstracts do not cover pregnancy, pediatrics, elderly, or comorbidities 123.Key Recommendations
Utilize thoracic endovascular aortic repair (TEVAR) and coil embolization for managing racemose hemangiomas involving bronchial-pulmonary artery fistulas to prevent hemoptysis (Evidence: Moderate) 3.
Employ fluorescein angiography for diagnosing episcleral racemose hemangiomas to confirm vascular characteristics (Evidence: Weak) 1.
Consider imaging studies like CT scans for comprehensive assessment of systemic racemose hemangiomas (Evidence: Moderate) 3.References
1 Lim LA, Brower P, Shields JA, Shields CL. Episcleral racemose hemangioma. Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus 2019. link
2 Criado PR, Alavi A, Valente NY, Sotto MN. Amantadine-Induced Livedo Racemosa. The international journal of lower extremity wounds 2016. link
3 Matsubara Y, Morisaki K, Matsuda D, Aoyagi Y, Tanaka S, Okadome J et al.. Thoracic endovascular aortic repair and coil embolization of the pulmonary artery for primary racemose hemangioma of the bronchial artery with a bronchial-pulmonary artery fistula. Vascular 2015. link