Indian Journal of Ophthalmology

ARTICLES
Year
: 1974  |  Volume : 22  |  Issue : 3  |  Page : 27--28

Crouzon's disease


IN Raizada1, VN Raizada1, VK Rohatgi2,  
1 Department of Ophthalmology, L.L.R.M. Medical College, Meerut, India
2 Department of Radiology, L.L.R.M. Medical College, Meerut, India

Correspondence Address:
I N Raizada
Department of Ophthalmology, L.L.R.M. Medical College, Meerut
India




How to cite this article:
Raizada I N, Raizada V N, Rohatgi V K. Crouzon's disease.Indian J Ophthalmol 1974;22:27-28


How to cite this URL:
Raizada I N, Raizada V N, Rohatgi V K. Crouzon's disease. Indian J Ophthalmol [serial online] 1974 [cited 2020 May 27 ];22:27-28
Available from: http://www.ijo.in/text.asp?1974/22/3/27/31358


Full Text

This is a rare clinical condition, where cran­iostenosis is associated with facial deformity. The combination of two conditions gives rise to a typical clinical picture. Slight variations from the typical picture are common, but fun­damental anomaly remains the same. [1] These cases come to the hospital for diminution of vision which is due to optic atrophy. The prognosis in such cases is poor both for the vision and life expectancy. These cases require decompression of orbit and cranium at the earliest possible stage to save vision and brain damage. [2]

 Case Report



S. D., 28 years old female, presented with the complaints of bilateral proptosis since birth, progres­sive diminution of vision and the heaviness of head. Proptosis had been stationary but for last one year there was a steady diminution of vision in both eyes, resulting in blindness in left eye and gross loss of vision in right eye, rendering the patient visually handicapped. Patient was shown in her infancy to many specialists who explained the mother about poor prognosis. No surgical interference was suggested. She had her education in normal way and passed the high school examination. It was the progressive diminution of vision that brought them back to hospital. On exami­nation the eyes were grossly proptosed (interorbital distance 105 mm. and proptosis 20 mm. with Hertel's exophthalmometer) and divergent. Both eyes were emmetropic and anterior segment showed no other positive finding. Both discs showed primary optic atrophy. Vision in the right eye was counting fingers upto two meters. Left eye had only perception and accurate projection of light Tension was normal in both eyes with grossly constricted fields.

Face showed deformity of the nose resembling Parrot's beak, prognathism of lower jaw, hypoplasia o maxilla and misalignment of teeth. Routine investiga­tions showed no other positive finding.

X-Ray Report : Skiagram of the skull, Posteroan­terior and lateral views), optic foramina, orbits, wrists hand and elbow joint were taken. The skull showed silver beaten appearance in the frontal, lower parietal and in occipital region. The skull showed disproportion between the large calvarium and small face. The maxilla was small and the mandible was large in size. The high vertex at the site of anterior fontanel was also visualised. The middle and posterior fossae were large in contrast to small flat anterior fossae. The optic foramina were normal. The screening of chest showed no abnormality.

 Summary



A case of Crouzon's anomaly has been described. Emphasis on early treatment in such cases was pointed out.

References

1Duke Elder, S. 1964, System of Ophthalmology,3 1052, Henry Kimpton, London.
2Sorsby, A. 1963, Modern Ophthalmology, 2 Systemic aspect, 50.