Extensive lymphangioma presenting with upper airway obstruction.
OBJECTIVE
To describe the results of an outcome survey of 18 cases of pediatric lymphangioma with dyspnea from encroachment on the tongue base, parapharyngeal space, and/or larynx.


METHODS
Retrospective review of files from January 1983 to September 1998.


METHODS
Pediatric otolaryngology departments from 2 referral centers.


METHODS
Eighteen patients were treated. The average age at initial surgery was 22 weeks (median, 5 weeks). All presented with at least unilateral suprahyoid and infrahyoid cavernous (microcystic) lymphangioma. The tongue base was involved in 11 patients, the parapharyngeal space in 12, and the larynx in 8.


METHODS
Neck dissection was performed initially in all patients. Tracheotomy was performed in 9 patients (50%). Macroglossia was treated by V glossoplasty. Parapharyngeal extensions were treated by cervicotomy or endoscopy, and larynx and tongue base extensions by carbon dioxide laser photocoagulation. Supraglottic laryngectomy was performed in 2 patients.


METHODS
Residual disease, decannulation, duration of tracheotomy, and persistent respiratory symptoms.


RESULTS
The average follow-up was 4 years postoperatively. One postoperative death occurred. Sixteen (94%) of the remaining 17 patients had residual lymphangioma. Eight (89%) of the 9 patients with tracheotomy underwent decannulation (average duration, 22 months). Ten patients had persistent symptoms, and 6 were asymptomatic.


CONCLUSIONS
Involvement of the upper airway seems to be the determining prognostic factor in extensive lymphangioma. Patients with dyspnea by external compression of cervical lymphangioma on the airway responded well to surgery. Aggressive surgical treatment did not seem to significantly improve the prognosis in patients with intrinsic involvement of the upper airway. The natural evolution of untreated massive lymphangioma has not been documented. Less aggressive, symptomatic therapy may be an alternative to avoid mutilating surgery in patients with intrinsic involvement of the airway.
Hartl
D M
DM
Department of Pediatric Otolaryngology-Head and Neck Surgery, Hopital d'Enfants Armand Trousseau, 26 avenue du Dr Arnold Netter, 75571 Paris Cedex 12, France.
Roger
G
G
Denoyelle
F
F
Nicollas
R
R
Triglia
J M
JM
Garabedian
E N
EN
D000402:Airway Obstruction / Q000209:etiology*; D004417:Dyspnea / Q000209:etiology; D006258:Head and Neck Neoplasms / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D008197:Lymph Node Excision; D008202:Lymphangioma / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome