鼻性髄液漏を呈したcloverleaf skull syndromeの1例—神経放射線学的検討

A CASE OF CLOVERLEAF SKULL SYNDROME WITH PERSISTENT CEREBROSPINAL FLUID RHINORRHEA

I．はじめに

　冠状縫合と人字縫合の早期癒合により，短頭と側頭部・頭頂部の突出を竪する水頭症は，その外見的特徴から，クローバー葉状頭蓋症候群といわれている^1,2）。

　今回我々は，鼻性髄液漏を呈した極めて高度の水頭症例を経験し，従来の報告では行われていなかつたアイソトープを用いて，神経放射線学的に検索したので報告する。

This 6 year-old female was first noted to havean enlarged and cloverleaf like skull soon afterbirth and then persistent rhinorrhea. Physical ex-amination revealed mental retardation with bubblingphonation and some attention to her surroundings.The throat, lungs, heart and abdomen were un-remarkable.

She could not hold her head or sit up, yet, butno paralysis of the extremities was noted.

The head bulged upward and laterally, resemblinga trilobe of cloverleaf. The elbow joints wereankylotic bilaterally.

The X ray of the skull showed craniolacuniae.No oxygen gas entered into by pneumoencephalo-graphy. Pneumoventriculography revealed markedenlargement of the lateral ventricles, probablepresence of the cavum septi pellucidii, and no gasinto the subarachnoid space.

Carotid angiography showed an unrolling of theanterior and middle cerebral arteries, and downwardcourse of the insular arteries. The sylvian cisternshifted downward and horizontal, and positivetamponade counting in the nostril (rhinorrhea) wasobserved, but no ventricular reflux was revealed incisternal scintigraphy. Therefore, cerebrospinalfluid rhinorrhea was ascertained. Ventricularscintigraphy showed large lateral ventricles, possiblepresence of the cavum septi pellucidii and presistentremaining of the radioisotope, indicating non-communicating hydrocephalus.