|Year : 1983 | Volume
| Issue : 1 | Page : 33-34
Glaucoma in crouzon's disease
M Krishnan, Rajini Kantha
Department of Ophthalmology, Lady Hardinge Medical College, New Delhi, India
Department of Ophthalmology, Lady Hardinge Medical College, New Delhi-110 001
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Krishnan M, Kantha R. Glaucoma in crouzon's disease. Indian J Ophthalmol 1983;31:33-4
Crouzon in 1912 described hereditary premature synostosis of the cranial sutures for the first time in a 3 year old male. This developmental cranial deformity which is similar to oxycephaly has an autosomal dominant hereditary tendency and manifests at birth. Though the typical features of Crouzon's disease have been reported by various authors, to our knowledge, glaucoma associated with crouzon's has been reported only by Naud, in the literature.
| Case report|| |
C.K. a 8 day old male baby was seen with the chief complaints of marked prominence of both eyes since birth and increased protrusion of right eye while crying.
Clinical examination revealed bitateral proptosis of the eyes, divergent strabismus [Figure - 1] A & B and luxation of the Right Eye on crying [Figure - 1] C. Exophthalmometry, gave a reading of 26 in the Right Eye and 24 in the Left Eye. Intra Ocular Pressure seemed digitally raised and Schiotz tonometry on three occasions recorded an average tension of 34 & 28 mm Hg. in Right Eye and Left Eye respectively. Fundus examination revealed bilateral optic atrophy.
The shape of the head was abnormal with marked increase in the vertical height of the anterior part of the skull, in the region of the anterior fontanelle. The fontanelles were closed.
Other features of Crouzon's disease like sittacorhina (Parrot beak nose), mandibular prognathism short upper lip with hypoplastic maxilla and large thickened ear lobes were also seen [Figure - 1] D.
Radiological examination revealed craniostenosis. Lacunae and large gaps were also seen in the skull. Orbits were shallow and roofs of the orbits were not seen. The maxilla was small and the mandible prominent. The appearance was consistent with crouzon's disease.
| Discussion|| |
Glaucoma associated with Crouzon's disease has been rarely reported. Though the exact cause of glaucoma in this case could not be ascertained, developmental anamoly of the angle of the anterior chamber could be the probable aetiological factor. Due to the age of the patient and nonavailability of direct gonioscope, gonioscopy though very relevant, could not be carried out. Optic atrophy seen in this case seems to be a part of the disease process, and not a sequelae of glaucoma.
| Summary|| |
A rare case of Crouzon's disease with associated glaucoma has been reported.
| References|| |
Naud. 1949, J. Med. Bordeaux. 126:350. Quoted by Duke Elder, S., 1967, system of Ophthalmology, Vol. III, part 2, Henry Kimpton, London PP 1048-1052.
[Figure - 1]