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ARTICLE
Year : 1960  |  Volume : 8  |  Issue : 3  |  Page : 77-80
 

Craniostenosis or oxycephaly


Schell Eye Hospital, C.M.C., Vellore, India

Correspondence Address:
Roy Ebenezer
Schell Eye Hospital, C.M.C., Vellore
India
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How to cite this article:
Ebenezer R. Craniostenosis or oxycephaly. Indian J Ophthalmol 1960;8:77-80

How to cite this URL:
Ebenezer R. Craniostenosis or oxycephaly. Indian J Ophthalmol [serial online] 1960 [cited 2014 Nov 27];8:77-80. Available from: http://www.ijo.in/text.asp?1960/8/3/77/40684


The diverse modifications of Cranios­tenosis are as follows

Advanced oxycephaly
Brachycephaly
Plagiocephaly
Hyper-telorism
Acrocephaly
Incomplete Oxycephaly
Scaphocephaly
Craniofacial Dysostosis
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 Top


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.[8]

 
   References Top

1.Abeles MI. M. (1936), Archives Ophth. 16, 188-196.  Back to cited text no. 1    
2.Brontenbrenner A. N. (11931), Am. J.Ophth. 14.  Back to cited text no. 2    
3.Mann Ida (1937), Developmental ano­malie. of the eve. British Medical Asso­ciation, B. M. A. Hove Javistod Sq., London, W.C.I. 49.  Back to cited text no. 3    
4.Howwell Stacy C.(1954),Amer. J. Ophth. 37, 380-390.  Back to cited text no. 4    
5.Koziak P. H (1951), Anter., .J. Opht.h. 37. 380-390.  Back to cited text no. 5    
6.Lewis F. A. (1925) , Amer., J. Ophth.8, 513.  Back to cited text no. 6    
7.Peter A. L. (1946), Amer., J. Ophth.29, 685-698.  Back to cited text no. 7    
8.Walsh F. D. (1947), Clinical Neuro ophthalmology. The Williams Wilkins Co. Baltomore, .181. Amer., J. Dis child. 1931. 42, 837-857.  Back to cited text no. 8    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9], [Figure - 10], [Figure - 11]



 

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