Pediatric hemophilic pseudotumor of the paranasal sinus.

ABSTRACT

OBJECTIVES/HYPOTHESIS: Hemophilic pseudotumors are rare clinical entities in otolaryngology. An unusual case of a pediatric hemophilic pseudotumor of the paranasal sinus in a previously undiagnosed hemophiliac is presented. STUDY DESIGN: Case report. METHODS: A 6-month-old, otherwise healthy boy was admitted for evaluation of a rapidly expanding left-sided cheek mass. The patient's initial presentation, imaging workup, and intraoperative and postoperative courses are discussed. RESULTS: Imaging workup revealed a large, heterogeneous, dense lesion in the patient's left-side maxillary sinus with distortion of the orbital floor and hard palate. Because of the concern for an aggressive malignancy, a biopsy was performed. After a Caldwell-Luc approach, a large amount of greenish fluid leaked from an otherwise empty maxillary sinus. The patient continued to bleed from his intraoral incision postoperatively and required two packed erythrocyte transfusions. Subsequent hematological testing led to a diagnosis of severe hemophilia type A with factor VIII levels less than 1% of normal. After treatment with recombinant coagulation factors hemostasis was achieved, and the patient clinically improved. CONCLUSION: The study presents the first report of a pediatric hemophilic pseudotumor in the paranasal sinuses leading to a diagnosis of hemophilia. It is a rare entity characterized by a cycle of repeated hemorrhage with subsequent encapsulation of the coagulum leading to pressure-induced distortion of adjacent bone. In the correct clinical setting, hemophilic pseudotumor should be considered in the differential diagnosis of a rapidly expanding mass in children.