Pycnodysostosis患者でみられた上顎骨骨髄炎の1例

A Case of Pycnodysostosis complicated with Osteomyelitis of the Maxilla

　はじめに

　Pycnodysostosisとは，低身長，指趾短縮，頭蓋骨および上・下顎骨の形成障害など，特異的顔貌と外観をもち，全身のびまん性骨硬化と易骨折性を認める骨系統疾患である。同疾患に伴う下顎骨骨髄炎の報告は散見されるが，今回われわれは，抜歯後，長期にわたり上顎骨骨髄炎を認めた1例を経験したので若干の文献的考察を加えて報告する。

A 45-year-old female presented with pycnodysos-tosis complicated with chronic osteomyelitis of the right maxilla.

The patient complained of purulent nasal dis-charge and a swelling of the right cheek for several years.

CT and x-ray revealed a failure of closure of the cranial sutures and no formation of the maxillary sinus.

Right partial maxillectomy was performed to remove sequestra under general anesthesia.

There were no recurrent osteomyelitis nor other complications.