Year : 1972 | Volume
: 20 | Issue : 4 | Page : 191--193
Oxycephaly associated with cleft palate (a case report)
RC Saxena, KC Garg, Meena Bhatia
King George's Medical College, Lucknow, India
R C Saxena
King George«SQ»s Medical College, Lucknow
|How to cite this article:|
Saxena R C, Garg K C, Bhatia M. Oxycephaly associated with cleft palate (a case report).Indian J Ophthalmol 1972;20:191-193
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Saxena R C, Garg K C, Bhatia M. Oxycephaly associated with cleft palate (a case report). Indian J Ophthalmol [serial online] 1972 [cited 2021 Sep 27 ];20:191-193
Available from: https://www.ijo.in/text.asp?1972/20/4/191/34636
RAMCHAND, GARG AND SAXENA  reported a family of four in whom the father, his son and daughter were patients of Oxycephaly. The mother of these children was normal. This report mentions description of the father and the son but the authors could not examine the daughter. Recently we had an opportunity to examine this member of the family also. She showed oxycephaly with cleft palate. The association of cleft palate with oxycephaly is extremely rare in available ophthalmic literature. Moreover, her father and brother did not show this abnormality. In order to complete the series of RAMCHAND et al  and to present the rare association of cleft palate, we intend to report this case.
Patient K. Hindu, female aged 18 years was examined recently in the Department of Ophthalmology, K. G.'s Medical College, Lucknow. She complained of gradually increasing protrusion of the eye balls, poor vision, and nasal twang of voice since early childhood.
On examination the patient was of average build but mentally retarded. She had oxycephaly showing vertical elongation of skull with short anteroposterior and transverse diameter. Other associated features were, bulging of temporal fossae, and cleft palate [Figure 2]. The lips were normal.
The examination of eyes revealed bilateral proptosis with drooping of the lids. The eyes were divergent and the left eye was slightly displaced downwards (Plate 1). The palpebral aperture on left side was wider and the medial canthi of both eyes were a little higher than the lateral canthi. The movements of both the eye balls showed slight restriction in all directions. Pupillary reactions were sluggish on both sides. The fundus revealed signs of secondary optic atrophy. Distant vision of the right eye was 6/36 and that of the left eye was 6/18.
Peripheral field examinations showed concentric contraction of fields in both eyes.
Roentgenogram of skull showed vertical elongation, short anteroposterior diameter with no occipital protuberance and suture lines. Skull walls were thin and had marked digitations all over. The orbits were shallow (Plate 3). The maxilla showed hypoplasia but the mandible was heavy. The evidence of cleft palate (Plate 4) and defective occlusion (Plate 3) of teeth was also present.
This patient who had strong hereditary background showed typical oxycephaly with bilateral proptosis and ptosis. Her father had deformity of skull, bilateral proptosis with divergence of eye balls and exposure keratitis. He had no other developmental anomaly. Her younger brother had similar deformity of skull with expulsion of eye balls from the orbit. His eyes showed advanced changes of exposure in cornea and conjunctiva. He too had no other associated developmental anomaly. (RAMCHAND et al  ). This patient as against her father and brother had cleft palate. In the ophthalmic literature, association of high arched palate (WALSH) is more frequently mentioned but the association of cleft palate in oxycephaly is found to be rare. The fact that the two male members of this family had no cleft palate but the only female member had cleft palate, made us think of the sex difference in development of cleft palate.
The father and the son had signs of exposure keratitis. This patient was luckily free because there was no sign of lagophthalmos [Figure 3] inspite of proptosis.
The fundus changes in the present case resembled that of her father. She had fairly good working visual acuity in both eyes. She was, however, not advised any surgical interference.
A female member of a family of oxycephaly is reported who showed cleft palate as associated developmental anomaly. Other members of the family have already been reported by RAMCHAND et al. 
|1||Ramchand S, Garg K. C., & Saxena R. C.: Oxycephaly with Expulsion of eye balls., J. all India Ophthal. Soc. 17, 8-10, (1969).|
|2||Walsh F. B.: Clinical Neuro-Ophthalmology, llnd Edition, 407-409, The Williams & Wilkins Company, Baltimore, (1957).|