|Year : 1960 | Volume
| Issue : 3 | Page : 77-80
Craniostenosis or oxycephaly
Schell Eye Hospital, C.M.C., Vellore, India
Schell Eye Hospital, C.M.C., Vellore
|How to cite this article:|
Ebenezer R. Craniostenosis or oxycephaly. Indian J Ophthalmol 1960;8:77-80
The diverse modifications of Craniostenosis are as follows
Mixed with Syndactylism
We shall consider of these Oxycephaly which is otherwise called Acrocephaly, Turricephaly or tower skull.
Oxycephaly exists in many different degrees of severity and is usually supposed to result from premature synostosis of the bones forming the carnial vault.
The incidence is more among males and shows a hereditary factor, the orbit is very shallow due to displacement of sphemoid leading to exophthalmos. In many cases there is divergent strabismus due to increased outward obliquity of the orbital axes. The skull is foreshortened and the vault is high more pointed and dome shaped. A short antero-posterior diameter with a high sloping forehead is rather typical of Oxycephaly.
Nystagmus is not uncommonly seen and partial optic atrophy of the post neuritic or secondary type is usually found. This may be due to an early papilloedema which arises early in life, from pressure by the brain growing in the confined space. However, absolute blindness seldom follows.
Radiological examination of the skull shows a silver beaten appearance of the bones of the vault due to raised intracranial pressure. The convolutional markings are present. Occipito parietal sutures are not visible. Middle fossa of skull is pushed downwards and forward. The optic foramen may be narrowed or normal.
Oxycephaly may be associated with Crouzon's Syndrome and a generalised disturbance of bones such as klipped Feil Syndrome or Brevicohis where the cervical vertebrae are absent or with arachnodactylism, congenital deformities of toes, syndactylism (Apert's disease) and patent parietal foramina. There is frequently prognathism and a high narrow palatine arch. The anterior fontanelles may remain widely open. Other associated malformations that may be present are deflected septum of nose, medullated nerve fibres with wide dilatation of pupil, coloboma of Iris and Choroid, irregular shape of pupil, congenital cataract, vitreous opacities, myelination of nerve fibres, detachment of retina and subluxation of lens.
There may be some features of Crouzon's disease associated with oxycephaly, The typical features of dysostosis craniofacialis are recession of maxilla with relative prognathism, marked exophthalmos, divergent strabismus and optic atrophy with diminution of vision.
Exophthalmos varies from a mild degree to extreme proptosis and extrusion of the globe. Pnosis is frequently seen, with lagophthalmos. A few cases of convergent strabismus have been reported. Frequently also there is a weakness or loss of convergence. Nystagmus which may be present is of the ocular type.
Headache is an almost constant complaint. It tends to disappear about the age of 8 years and comes back with severity in adult life. Convulsions may occur. The severe cases of Oxycephaly do not generally reach adult life. Mental development may be sub-normal, Gradual loss of vision is due to optic atrophy. The optic nerve is stretched due to malformation of the middle fossa of the skull. This mav account for the optic atrophy.
[Figure - 1],[Figure - 2],[Figure - 3],[Figure - 4],[Figure - 5] and [Figure - 6] to it illustrate the important clinical manifestations of two cases.
| Treatment|| |
Surgery is indicated before one year of life and preferably before the first 6 months if damage to the optic nerves is to be prevented. Artificial sutures in place of the fined ones are necessary on the cranium to allow more space for thee developing brain. 5-6 new sutures are cut in the skull. Burrholes are made which are connected by sutures cut with a gigli saw. The sutures may be left as such or tantallum implant or foil put in to keep the craniotomy openings patent. The foil covers the cut edges or the same effect may be achieved by wrapping polyethylene film over the cut.
There is amelioration of symptoms. The papilloedema if present subsides. The progress of optic atrophy is stayed. Headache is relieved and the vision ww hat remains of it, is preserved from further deterioration.
When there is atresia of the optic foramen as found out by radiological examination enlarging of the optic canal by means of an incision just above the brow and beneath the periosteum may be clone removing the fractured bits and incising the dural sheath of the nerve with good results.
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9], [Figure - 10], [Figure - 11]