Juvenile Paget's disease with paranasal sinus aplasia

Abstract

Juvenile Paget's disease (JPD) is a rare skeletal disorder that's characterized by bone demineralization and elevated levels of serum alkaline phosphatase. JPD involves the paranasal sinuses in extremely rare cases. We report here on a 25-month-old Asian male who was diagnosed of JPD associated with aplasia of the paranasal sinuses, but not the ethmoid sinuses. The patient was successfully treated by surgery and we undertook no medical intervention. This appears to be the first reported case of JPD associated with bilateral paranasal sinus aplasia.

Keywords: Osteitis deformans; Paget disease of bone; Paranasal sinus neoplasms; Paranasal sinuses.

Fig. 1

(A) Endoscopic view showing hypertrophic change of inferior turbinate & nasal cavity narrowing of right side. (B) Plain radiography showing thickening and sclerotic changes in the facial bone and skull vault, and unformed obliterate paranasal sinuses. (C) Coronal computed tomogram revealing complete agenesis of both paranasal sinuses but not the ethmoid sinuses. (D) Bone scan showing marked uptake of radionuclide in both paranasal sinuses, especially the maxillary sinuses. IT: inferior turbinate.

Fig. 2

Biopsy from the inferior nasal concha showing increased cellularity with irregular cement lines (black arrow) and loose fibrovascular tissue within the intertrabecular space (white arrow) (×200, H&E stain).