Treatment and outcomes for epistaxis in children with Glanzmann's thrombasthenia.

Objectives/hypothesis: :To understand Glanzmann's thrombasthenia and provide insight to the management of epistaxis in children with this disease.

Study Design: Retrospective chart review.

Methods: All children diagnosed with Glanzmann's thrombasthenia and treated for epistaxis at Children's Hospitals and Clinics of Minnesota were identified and a retrospective chart review was performed. Outpatient charts, hospitalization records, and operative reports were reviewed from 1999 up to 2009 and appropriate data were extracted. The episodes of epistaxis, therapy used, complications, and success rates for controlling hemorrhage were noted.

Results: Five children with a total of 63 clinical encounters for epistaxis were identified. Forty-seven encounters required hospitalization, close to half of which necessitated medical care in the intensive care unit. Nearly all encounters required infusion of hemostatic therapy with either single or multiple agents. Seventy-one procedures for life-threatening hemorrhage were performed. The most common (n = 24) intervention was administration of bovine collagen matrix, which was successful only half of the time (50%). Anterior and posterior nasal packing with or without hemostatic material completely resolved hemorrhage in 35% of the procedures.

Conclusions: To date, no form of medical or surgical intervention in children with this condition has consistently demonstrated its ability resolve nasal hemorrhage. The role of the otolaryngologist is to control bleeding during major episodes of nasal hemorrhage that do not respond to medical management. These patients usually have remarkable improvement in the frequency and severity of epistaxis in adolescence and then require much less aggressive therapy.