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ARTICLE
Year : 1969  |  Volume : 17  |  Issue : 1  |  Page : 8-10

Oxycephaly with expulsion of eye balls


India

Date of Web Publication4-Jan-2008

Correspondence Address:
S Ramchand
India

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How to cite this article:
Ramchand S, Garg K C, Saxena R C. Oxycephaly with expulsion of eye balls. Indian J Ophthalmol 1969;17:8-10

How to cite this URL:
Ramchand S, Garg K C, Saxena R C. Oxycephaly with expulsion of eye balls. Indian J Ophthalmol [serial online] 1969 [cited 2020 Aug 9];17:8-10. Available from: http://www.ijo.in/text.asp?1969/17/1/8/37572

Oxycephaly is a developmental anomaly characterised by a vertically elongated head with short transverse and antero-posterior diameters. Though the clinical appearance of this condition has been recognized from ancient mythology (DUKE ELDER[2]), but true Oxycephaly was described by GREIG[4]` as a definite clinical entity with a germinal cause and predetermined course and symptoms. The term Pseudo-Oxycephaly was introduced to include all deformities which appear superficially as Tower or Steeple Skull.

Minor degree Oxycephaly is usually not associated with facial deformities and proptosis but in major form of Oxycephaly there is a vertical forehead, absence of superciliary arches, a prominent nose, hypoplasia of maxilla and relatively heavy lower jaw. Ocular changes are marked proptosis associated with divergence of eyeballs and restriction of movements. Rarely a dislocation of the eyeball may occur (SHERNIE[6]) Visual failure is due to papillmdema followed by optic atrophy. The disease is four times more common in males than females (DUKE-ELDER[2] t may be isolated in a family (WALSH[7]or may show a hereditary tendency (KING[5]; GREIG[4]; FR ANCESCHETTI AND KLEIN[3]).

Surgical interference is useful if symptoms of raised intracranial pressure persist in early childhood particularly if there is any evidence of papilloedema or optic atrophy (DANDY[1]). Once the active growth of the brain has stopped about the age of 7 or 8 years and it has become accommodated to its environment, symptoms cease to progress and the patient may continue life exhibiting his deformity without further harm.

In this paper a family of Oxycephaly is presented in which three out of four members suffered from this deformity. Rare presentation of expulsion of eyeballs and its occurrence in so many members, interests us to report this family.


  Case report Top


Patient V. P., Hindu, Male aged 9 years was first examined in the department of Ophthalmology, King George's Medical College, Lucknow, on 28th January, 1967 with the complaints of progressive elimination of vision in both the eyes since last 2 years associated with recurrent headache, pain and congestion of the eyes. Since the last month and a half his vision had markedly deteriorated. He also had abnormal shape of the skull and bulging of both the eyeballs since childhood which has been nonprogressive.

On examination, the child was of average build. He had Oxycephaly presenting a vertical elongation of skull [Figure - 1] with short anteroposterior and transverse diameter. Mentally he was of low intelligence.

Eye examination showed marked proptosis with expulsion of eyeballs from the orbits [Figure - 1]. The eyeballs were pushed downwards and outwards. The mobility was restricted in all directions. Palpebral apertures were wide through which the eyes were pushed out producing lagophthalmos. Bulbar and palpebral conjunctiva of both the eyes were congested, chemosed and fibrosed. Due to exposure the right cornea was completely opaque and dry. Cornea of the left eye was also white, opaque and dry except in the periphery and upper temporal quadrant which had macula grade of dry corneal opacity. Corneal sensitivity was absent in both the eyes. Perception and projection of light were present in the left eye but absent in the right eye.

The father [Figure - 2] aged 39 years also had similar deformity of the skull and bulging of both the eyeballs since birth. He also complained of progressive diminution of vision associated with pain and redness of both the eyes. He was of average build with eyes. skull deformity. Intelligence was also low.

Examination showed proptosis more in the left eye with divergence of the eyeballs and some downward displacement of the right eyeball. The palpebral apertures of both the eyes were wide, more in the left eye. Bulbar conjunctiva in the lower part was congested in both the eyes. The cornea of both the eyes showed opacities due to exposure keratitis in their lower halves. Pupillary reactions were sluggish and ophthalmoscopic examination showed secondary optic atrophy in both eyes. Vision in the left eye was 6/24 while in the Right eye 6/60.

The only sister of the patient aged 14, unmarried, who could not be brought for examination also had the same deformity of the skull and bulging of both the eyes since birth. The mother was completely normal.


  Investigation Top


Roentgenograms of both father and son [Figure - 3] and [Figure - 4] showed tower skull with decreased antero-posterior and transverse diameter and thinning of skull walls and marked digitations all over. All other laboratory investigation were normal.


  Discussion Top


The patient showed typical Oxycephaly with expulsion of both the eyeballs from the orbit. Due to constant exposure of both the eyeballs the conjunctiva were chemosed and fibrosed and the cornea were opaque and dry. The patient showed a strong hereditary background with similar involvement in father and sister.

Since in the son the right eye was blind and only projection of light was present in the left eye associated with expulsion of eyeballs and complete opacification of cornea of both the eyes, nothing could be done. Complete loss of vision in the right eye and markedly poor vision in left eye may be due to optic atrophy which is an associated feature of this disease. Poor vision in both the eyes of the father was due to secondary optic atrophy and since there were no signs of raised intracranial pressure, no surgical interference was advised.


  Summary Top


A family of Oxycephaly is presented in which the father and both the children (a son and daughter) showed this deformity associated with proptosis in the father and the daughter and a rare complication of expulsion of eyeballs in the son.

 
  References Top

1.
DANDY, W. E.: An operation for Scaphocephaly. Arch. Surg. 47: 274-249, (1943).  Back to cited text no. 1
    
2.
DUKE-ELDER, S.: Text Book of Ophthalmology., 5, Henry Kimpton London, 1952.  Back to cited text no. 2
    
3.
FRANCESCHETTI, A., AND KLEIN, D.: Oxycephaly in three pairs of uniovular twins, associated in one case with a cutis frontis gyrata (in French) Acta genet. med. et ;enel. 1: 48-66, (1937).  Back to cited text no. 3
    
4.
GREIG, D. M.: Oxycephaly Edin. M. Jr, 33, 189-218; 280-302: 357-376. (1926).  Back to cited text no. 4
    
5.
KING, J. E. J.: Oxycephaly. New York State Jr. Med. 41: 2119-2123. (1941).  Back to cited text no. 5
    
6.
SHERNE, J.: Dislocation of the Eyeball as a complication of oxycephaly. Case report. Brit. M. J. is 556, (1938).  Back to cited text no. 6
    
7.
WALSH, F. B.: Clinical Neurophthalmology. Ilnd Edition. The Williams and Wilkins Company Baltimore. (1957).  Back to cited text no. 7
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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