Extensive lymphangioma presenting with upper airway obstruction

Objective: To describe the results of an outcome survey of 18 cases Of pedi atric lymphangioma with dyspnea from encroachment on the tongue base, parap haryngeal space, and/or larynx. Design: Retrospective review of files from January 1983 to September 1998. Setting: Pediatric otolaryngology departments from 2 referral centers. Patients: Eighteen patients were treated. The average age at initial surger y was 22 weeks (median, 5 weeks). All presented with at least unilateral su prahyoid and infrahyoid cavernous (microcystic) lymphangioma. The tongue ba se was involved in 11 patients, the parapharyngeal space in 12, and the lar ynx in 8. Interventions: Neck dissection was performed initially in all patients. Tra cheotomy was performed in 9 patients (50%). Macroglossia was treated by V g lossoplasty. Parapharyngeal extensions were treated by cervicotomy or endos copy, and larynx and tongue base extensions by carbon dioxide laser photoco agulation. Supraglottic laryngectomy was performed in 2 patients. Main Outcome Measures: Residual disease, decannulation, duration of tracheo tomy, and persistent respiratory symptoms. Results: The average follow-up was 4 years postoperatively. One postoperati ve death occurred. Sixteen (94%) of the remaining 17 patients had residual lymphangioma. Eight (89%) of the 9 patients with tracheotomy underwent deca nnulation (average duration, 22 months). Ten patients had persistent sympto ms, and 6 were asymptomatic. Conclusions: Involvement of the upper airway seems to be the determining pr ognostic factor in extensive lymphangioma; Patients with dyspnea by externa l compression of cervical lymphangioma on the airway responded well to surg ery. Aggressive surgical treatment did not seem to significantly improve th e prognosis in patients with intrinsic involvement of the upper airway. The natural evolution of untreated massive lymphangioma has not been documente d. Less aggressive, symptomatic therapy may be an alternative to avoid muti lating surgery in patients with intrinsic involvement of the airway.