A toddler with acute, life-threatening airway obstruction due to a retropharyngeal macrocytic lymphatic malformation: successful emergency sclerotherapy.
Case Report
Lymphatic malformations (LMs) are rare congenital abnormalities of the lymphatic system, occurring in approximately 1 in 4000 live births. These malformations can be classified as microcystic, macrocystic or mixed. The case report we present is exceptional as it involved a toddler with life-threatening airway obstruction, successfully treated with emergency sclerotherapy, demonstrating a life-saving approach./r/nThis case report highlights the efficacy of bleomycin sclerotherapy in treating a macrocystic lymphatic malformation in a child./r/nCase Presentation: A 23-month-old girl, weighing 12 kg, with no known secondary diagnoses or hereditary diseases, presented with increasing respiratory distress. Initial diagnoses included subglottic laryngitis, dehydration, and metabolic acidosis. Due to worsening inspiratory and expiratory stridor, the child was transferred to the intensive care unit, where a retropharyngeal lymphangioma was diagnosed. The patient underwent microlaryngoscopy with puncture and drainage of bloody fluid, followed by bleomycin sclerotherapy (10,000 IU, 6.6 ml) under general anesthesia. A Starplasty tracheostomy was also performed. Imaging and laboratory evaluations included bronchoscopy, echocardiography, head and neck sonography, MRI, and MIBG scintigraphy./r/nThree months post-sclerotherapy, follow-up MRI showed a 80 % reduction in the size of the lymphatic malformation. Seven months post-sclerotherapy follow-up showed no new pathological changes, no residual tumor formation, and a fully passable laryngeal aditus. The child’s respiratory and nutritional symptoms resolved, and no adverse effects from the bleomycin treatment were observed during the follow-up period. Given the positive outcome, no further sclerotherapy was required, and the tracheostomy was successfully closed./r/nThis case demonstrates the successful use of bleomycin sclerotherapy in treating a large macrocystic lymphatic malformation in a pediatric patient with life-threatening airway obstruction. The method proved effective, achieving a significant reduction in malformation size without notable side effects, underscoring its value as a non-surgical treatment option for LMs in children.
