脳室腹腔シャント術後に脊椎硬膜下血腫を来した中脳水道狭窄症を伴うvon Recklinghausen's diseaseの1例

A Case of the Spinal Subdural Hematoma Formation Following Ventriculoperitoneal Shunting for von Recklinghausen's Disease Associated with Aqueductal Stenosis

I．はじめに

　von Recklinghausen病には，多彩な中枢神経系の病変を合併することが知られている．そのなかで，中脳水道狭窄症はvon Recklinghausen病の1％程度にみられるが^8），そのほとんどが腫瘍性の中脳水道狭窄で，非腫瘍性のものはきわめて少ない^15）．一方，脳室腹腔シャント術後の合併症として頭蓋内硬膜下血腫は少なからず経験されるが，脊椎硬膜下血腫を形成することはきわめて稀で，過去に2例しか報告されていない^14,16）．

　われわれは，von Recklinghausen病に伴った中脳水道狭窄症に対して，脳室腹腔シャント術を施行後，腰部脊椎管に硬膜下血腫を来した1例を経験したので，文献的考察を加え報告する．

Von Recklinghausen's disease associated with hydrocephalus due to non-tumoral aqueductal stenosis is rare. Furthermore the formation of subdural hematoma within the spinal canal is also a very rare complica- tion of ventriculoperitoneal shunt. We presented a case of spinal subdural hematoma formation following ventriculoperitoneal shunting for von Recklinghausen's disease associated with aqueductal stenosis.

A 10-year-old girl with von Recklinghausen's disease was referred to our hospital for gait disturbance during the previous 8 months.Magnetic resonance imaging (MRI) revealed hydrocephalus due to non-tum-oral aqueductal stenosis, and ventriculoperitoneal shunt was instituted. Three months later, the patient de-veloped lumbar pain and paraplegia. MRI revealed subdural hematoma in the lumbosacral spinal canal, andbilateral intracranial subdural hematoma were shown on computerized tomography (CT) scans. Subduralhematoma in the lumbosacral spinal canal was evacuated by laminectomy. Improvement of her neurologic-al deficit was obtained postoperatively, and intracranial subdural hematomas disappeared spontaneously.

Spinal subdural hematoma is assumed to be formed by the migratory movement of intracranial subduralhematoma under the influence of gravity. A characteristic finding of MRI is that such a subdural hemato-ma in the lumbosacral canal is located around the cauda equina.