拍動を伴う眼球陥凹と眼球運動障害を認めた先天性蝶形骨欠損の1例

A case of congenital sphenoid bone defect with pulsating enophthalmos and ocular motility disorder

要約　目的：拍動性眼球陥凹と眼球運動障害を呈した先天性蝶形骨欠損の1例の報告。

症例：38歳女性が左眼の拍動と眼球陥凹で受診した。18歳の時から左眼に瞼裂狭小があり，3回の出産ごとに悪化し，眼球陥凹が徐々に出現した。

所見：矯正視力は左右眼とも1.2で，正位であった。左眼に上転と外転制限があり，拍動性眼球陥凹があった。安静時に眼球が陥凹し，怒責時に突出した。頭部のCTとMRIで左側の蝶形骨欠損と脳髄膜瘤があった。神経線維腫症1型の所見はなかった。左眼の先天性蝶形骨欠損と診断し，手術は行わなかった。

結論：蝶形骨欠損により，拍動を伴う眼球陥凹と眼球運動障害が生じることがある。

Abstract　Purpose: To report a case of congenital sphenoid bone defect manifesting pulsating enophthalmos and ocular motility disorder.

Case: A 38-year-old female presented with pulsation and enopthalmos in the left eye as chief complaint. She had had blepharophimosis in the left eye since the age of 18 years that aggravated after three childbirths. Enophthalmos was reportedly of slow progression.

Findings: Corrected visual acuity was 1.2 in either eye. The left eye showed restricted upper and lateral movements. Pulsating enophthalmos was also present. The enophthalmos became manifest at rest and exophthalmos appeared during exertion. Computed tomography and magnetic resonance imaging showed left sphnoid bone defect and encephalocele. Findings of neurofibromatosis 1 were absent. She was diagnosed with congenital sphenoid bone defect and received no surgery.

Conclusion: The present case illustrates that congenital sphenoid bone defect may manifest pulsating enophthalmos and impaired ocular motility.