Indian Journal of Ophthalmology

CASE REPORT
Year
: 1983  |  Volume : 31  |  Issue : 2  |  Page : 85--86

Dyscrania excessiva with luxatio bulborum (A case report)


H Bhattacharjee 
 Deptt. of Ophthalmology, Gauhati, Medical College, India

Correspondence Address:
H Bhattacharjee
Sr. Research Fellow ICMR, Deptt, of Ophthalmology, Gauhati Medical College, Gauhati (Assam)
India




How to cite this article:
Bhattacharjee H. Dyscrania excessiva with luxatio bulborum (A case report).Indian J Ophthalmol 1983;31:85-86


How to cite this URL:
Bhattacharjee H. Dyscrania excessiva with luxatio bulborum (A case report). Indian J Ophthalmol [serial online] 1983 [cited 2021 Aug 6 ];31:85-86
Available from: https://www.ijo.in/text.asp?1983/31/2/85/27446


Full Text

A rare case of dyscrania Excessiva Luxatio bulborum is documented which was sporadic in occurrence.

 CASE REPORT



After 36 weeks of gestation a 26 years old priory mother (Father 31 years old) unevent­fully delivered a male infant. The mother had no associated history of any infection, intake of toxic drugs. exposure to radiation etc. during pregnancy. Available 18 members of three generation of his family (including father and mother) were examined and no congenital abnormality in any form was found in any member of the family.

The infant was examined 6 hours after birth and was found (a) Luxation of both eyes (which was evident at birth) with profuse chemosis of the conjunctiva and dry cornea and otherwise normal eyes-bear shaped' profile of the face from side characterised by extremely high skull, median prominance of the forehead, deep and impressed nose root, retrognath upper jaw, prominent inferior prognathism and depressed occiput-bulging pre and supra-auricular prominences of the squarno sphenoidal part of both side of the skull. The radiograph shows extremely shallow orbits. hypoplastic maxilla. open metopic sutures and fontanelles, digital impression over the formed bones and well developed skull base. No other skeletal abnormality could be detected [Figure 1].

 DISCUSSION



This is a rare and extreme degree of craniofacial dysostosis. Schulzbachz in the year 1938 described almost a similar case. In his case besides the craniofacial abnormali­ties there were other associated skeletal abnormalities like large clavicles, fixation in the elbows and the two distal joints of the second fingers and toes with premature ossification of the epiphyses near the joints. The present case had isolated abnormalities on the skull only. These cranial abnormali­ties bear some symmetry with Crouzon's disease and acrocephalia media. But a few characteristic features of Crouzon's disease like large hooked parrot's beak like profile of the nose. Frontal bossing of the skull and autosomal dominant inheritence were absent in the present case.

Spontaneous luxation of the globe is described in relation to other dyscranias like Crouzon's disease[2],[3] and Oxycephaly[4]. Shallowness of the orbit is responsible for this complication. The present case also shows very shallow orbits.

Influence of heredity over the various dyscranias are well known but sporadic­occurrence is also reported[5],[6]. In the present case no influence of heredity is detec­ted and no probable aetiologic factors could be detected. Parents of the infant are in biologically favourable age group. Localised affect of the injurious agent (if at all) at the cephalic end of the embryo over the skull bone is difficult to explain.

 SUMMARY



A case of dyscraner excessiva with Taxa­tion of globes is presented.

References

1Schulzbach. 1938. as described by Waardenburg P.J. Francesche HIA. Klein D. Genetics and Ophthalmology. Black Well Scientific Publication Ltd. Oxford 1961 P-320
2Sherne, 1938 Brit. Med. J. 1 : 565,
3Parks. N1.M: 1950. Ccstenbadcr. F. : Amer. J. Ophthalmol, 33 : 77.
4Robison. 13. Harley 1975 Paediatric Ophthal­mology W.B. Saunders Company P 886.
5Vorisek, 1941 Amer. J. Ophthalmol. 24; 1014,
6Duke Elder S. 1964 System of Ophthalmology, Vol. III. Part 2. Henry Kimpton. London P. 1049