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Year : 1972  |  Volume : 20  |  Issue : 4  |  Page : 191-193

Oxycephaly associated with cleft palate (a case report)


King George's Medical College, Lucknow, India

Correspondence Address:
R C Saxena
King George's Medical College, Lucknow
India
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Source of Support: None, Conflict of Interest: None


PMID: 4671315

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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;20:191-3

How to cite this URL:
Saxena R C, Garg K C, Bhatia M. Oxycephaly associated with cleft palate (a case report). Indian J Ophthalmol [serial online] 1972 [cited 2021 Jul 27];20:191-3. Available from: https://www.ijo.in/text.asp?1972/20/4/191/34636

RAMCHAND, GARG AND SAXENA [1] 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 [1] and to present the rare association of cleft palate, we intend to report this case.


  Case Report Top


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.


  Discussion Top


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 [1] ). This patient as against her father and brother had cleft palate. In the ophthal­mic literature, association of high arched palate (WALSH[2]) 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.


  Summary Top


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]



 
  References Top

1.
Ramchand S, Garg K. C., & Saxena R. C.: Oxycephaly with Expulsion of eye balls., J. all India Ophthal. Soc. 17, 8-10, (1969).  Back to cited text no. 1
    
2.
Walsh F. B.: Clinical Neuro-Ophthal­mology, llnd Edition, 407-409, The Williams & Wilkins Company, Baltimore, (1957).  Back to cited text no. 2
    


    Figures

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



 

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