Craniofacial Dysostosis （crouzon氏病）に対する眼窩減圧術

ORBITAL DECOMPRESSION IN CRANIOFACIAL DYSOSTOSIS (CROUZON'S DISEASE):SPECIAL REFERENCE TO FRONTO-ZYGOMATICO-SPHENOIDAL APPROACH

Ｉ．緒言

　小児の頭部の奇形である狭頭症（頭蓋骨癒合症）のなかには，顔面頭蓋の発育不良の為，眼窩が浅く，その為に眼球突出が起こり，かつ両側眼窩の離開を認めるものがある。1912年Crouzonは頭蓋顔面異骨症（Dysostosecranio faciale héréditaire；Crouzon's disease）として，この疾患を，遺伝性・家族性に起こる狭頭症の特殊型であると提唱した。狭頭症の頭蓋骨に対する手術の術式は，1881年Lane以来数多くの先達によつて頭蓋開溝術が適用され，その開溝方式はほぼ確立された感がある^2）4）7）（第1図）。Crouzon氏病においては，眼科の立場からKrönlein （1888）が，また脳神経外科的にはNaffzlger （1935）が眼球突出の外科治療を試みて以来^8），頭蓋開溝術に加えて，眼窩減圧術が行なわれて来た^6）。

　我々は最近3例のCrouzon氏病に，眼窩減圧術と頭蓋開溝術とを行なう機会を得たので，我々の行なつている眼窩減圧術の手技，fronto-zygomatico-sphenoidalapproachを中心に述べてみる。

The methods of orbital decompression has been developing for the surgical therapy of malignant exophthalmos and intraorbital tumor, though it is not suitable to use those mothods on the patients of Crouzon's disease, because facial bones and skull of Crouzons' disease are anatomically different from the normal ones. The orbital decompression in Crouzon's disease has mainly two problems.

( 1 ) Relative intra-orbital high pressure due to shallow orbita is the most important pathognomy of exophthalmos in Crouzon's disease.

( 2 ) Orbital decompression in Crouzon's disease should be combined with linear craniectomy simul-taneously.

We developed an approach of orbital decompre-ssion named fronto-zygomatico-sphenoidal method. Because of shallow orbita of Crouzon's disesase, this junction is the most nearest points to reach the lateral wall and the roof of theorbita, and more over, this approach provides wide decompression of lateral wall of the orbita. As far as linear craniectomy is concerned, it is important to make free bone flap of cranium. If free bone flap is not made, increased intra-dural tension kills the effect of orbital decommpression. Simultaneous orbital decompression and linear craniectomy was carried out in three cases. The result has been satisfactory, and there has been no complications and no squelae so far.