Primary laryngeal lymphangioma

Primary laryngeal lymphangioma is an extremely rare benign vascular malformation characterized by abnormal proliferation of lymphatic vessels within the larynx. Lymphangiomas (also known as lymphatic malformations or cystic hygromas when large) are congenital malformations of the lymphatic system, and while they most commonly occur in the head and neck region, isolated primary involvement of the larynx is exceptionally uncommon. The condition affects the respiratory and upper aerodigestive systems, with the abnormal lymphatic tissue potentially involving the epiglottis, aryepiglottic folds, arytenoids, or other laryngeal structures. Key clinical features include progressive hoarseness, stridor (noisy breathing), dysphagia (difficulty swallowing), dyspnea (difficulty breathing), and a sensation of a lump in the throat. In severe cases, particularly in infants and young children, airway obstruction can be life-threatening. The lesions typically appear as soft, compressible, translucent or bluish swellings on laryngoscopic examination. Diagnosis is confirmed through imaging studies such as MRI or CT scan and histopathological examination of biopsy specimens. Treatment options depend on the size and location of the lesion and the severity of symptoms. Surgical excision, often performed endoscopically using CO2 laser, is the most common approach. However, complete resection can be challenging due to the infiltrative nature of lymphangiomas and the critical anatomy of the larynx. Sclerotherapy with agents such as OK-432 (picibanil) or bleomycin has been used as an alternative or adjunctive treatment. Recurrence after treatment is possible, and long-term follow-up is recommended. In cases of acute airway compromise, tracheostomy may be necessary as a temporizing measure.

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