Arachnoid Cyst Associated with Isosexual Precocious Puberty

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A three-year and six-month-old girl was admitted to our hospital because of isosexual precocious puberty. She was a product of full term delivery weighing 3, 150 g. There was no past history of the central nervous system infection, head trauma or other serious illness. She had well developed breasts with areola. There was sparse pubic hair at the genital region. Deep tendon reflexes were slightly exaggerated but neither pathological reflexes nor ataxia were noted. Ophthalmological examinations were normal except mild bilateral optic atrophy. Plain X-ray films of the skull showed a "J-shaped sella turcica", thus suggesting suprasellar tumor. CT scan, however, showed two large solitary low density areas in the right middle fossa and the left occipital supratentorial region. These two low density areas showed the same density as CSF, and had a membranous structure around these lesions. According to these findings she was suspected to have congenital arachnoid cysts. Subsequent operation confirmed the diagnosis. CP shunt were performed for each lesion separately. Fluid in these two cysts was watery clear and showed no inflammatory signs. Histopathological findings of the cystic wall were those of arachnoid. Most of the reported cases of arachnoid cyst had single cyst in the middle or posterior fossa, and a few of them showed an isosexual precocious puberty.
一般社団法人日本小児神経学会の論文