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| Year : 1965 | Volume
: 13
| Issue : 3 | Page : 121-122 |
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Congenital bilateral anophthalmia
DL Maria, SP Shukla
Medical College, Aurangabad, India
Correspondence Address:
D L Maria
Medical College, Aurangabad
India
How to cite this article:
Maria D L, Shukla S P. Congenital bilateral anophthalmia. Indian J Ophthalmol 1965;13:121-2 |
Introduction
Anophthalmos is defined as a condition in which no eyeball, however small can be found in the orbit. To an embryologist the eye means the structures that owe their origin to neural ectoderm. To an anatomist, the mesodermal elements have to be added to the above to complete the eye in the proper anatomical sense.
According to Mann, (1937), there are three types of anophthalmia. The primary type is due to failure of appearance of the optic vesicle from the fore-brain and also of the optic pit to deepen, which happens at or before the 2 mm. stage in man. Secondary anophthalmos results from absence or abnormal development of the forebrain as a whole. The third type is due to the subsequent degeneration of a primarily formed optic vesicle.
 Case Report | |  |
A male child, thirteen days old was brought to the outpatient department of Government Medical College, Aurangabad, on 29th March, 1964. The child was the third son, the other two being also males, but without any congenital abnormality. The eldest son was eight years of age. The previous two males were full term normal births.
Examination:
The subject was a premature (7 months) male child weighing 1.6 kg.
Both the orbits were small, larger in the transverse diameter than the vertical. They were well developed; and so were the eye lashes. The conjunctival sac was reduced in size. The eyeball could not be felt on palpation, but movements of the conjunctival sac
were noticed, which must be due to the presence of extraocular muscles. The lids were kept closed. The palpebral fissures were markedly narrowed but equal on both sides and the lacrimal puncta were present.
No other congenital abnormality was noted in the child or in other members of the family. The orbit measured 17 x 10 mm. with orbital index of 170. Eyelids upper and lower were 6 mm. and 5 mm. respectively, and the palpebral fissure was 9 mm. wide on both sides.
The normal values for the orbit of the new born (6 months) given by Winckler are 27 x 27 with an orbital index of 100 and for the palpebral fissure 18.5 - 19 x 10 mm.
The anterior and posterior fontanelle were normal.
Respiratory, cardiovascular and urogenital systems were normal.
| Discussion | | |
From the clinical notes of various reported cases the following facts were noted. Anophthalmos in man is one of the rare congenital abnormalities. Mann and Collins, (1887) could find only 30 cases of bilateral and 12 cases of unilateral anophthalmos in the literature. Thirteen years later, Van Hippel, (1900) raised the reported number of bilateral cases to 64 and of unilateral to 23, a somewhat similar proportion. In 15 of this latter group, the other eye also showed some congenital anomalies. There does not appear to be any particular sex incidence, nor is there any evidence of parental transmission except in rare cases, although Mann remarked upon the anomaly having been transmitted through an affected male in one, and through an affected female in another instance, the conjugal partners in either case being normal. Transmission through the affected female seems to be commoner.
Anophthalmos is rarely associated with other defects, although Collins observed supernumerary digits, cleft palate and polydactylism in association with anophthalmos (Schwartzman and Maffia; (1939); Appelbaum and Marmelstein; (1943). The last named observer surveyed 66 case reports and found the condition to be bilateral in 72%. In about 50 percent of the unilateral cases the developed eye also showed some defect. Recordon and Griffiths (1938) reported a bilateral case with present foramen ovale.
The tendency to bilaterality and the sporadic incidence are in favour of an environmental cause, acting for a short time at the right moment. Rare familial and hereditary cases are obviously germinal in origin and several of these are unilateral - (Collins). Yudkin (1927) noticed hereditary anophthalmos in white rats, transmitted through affected female. Stockard (1910) produced anophthalmos in fish by subjecting the eggs to the action of a weak solution of alcohol, and Lewis by chemical methods produced anophthalmos in amphibia. Anophthalmos has been observed in young pigs when the sow has been fed on a diet-deficient in vitamin A as has been shown by Hale. (1935).[10]
| Summary | | |
A case of bilateral anophthalmos of the primary type in a 3rd male offspring of a family has been reported. The last case reported in literature was in 1938 and since then no case has been reported.
| References | | |
| 1. | Appelbaum. A & Marmelstein m. (1943), Arch. Ophthal. 29, 258.  |
| 2. | Falls, H.F. (1954), Cone. Ophthal. 17, 2060. |
| 3. | Hale, F. (1935), Amer. J. Ophthal., 18, 1087. |
| 4. | Hare, R., (1943), Arch. Ophthal., 30, 320. |
| 5. | Mann. I. (1937), "Developmental abnormalities of the eye", Cambridge. pp. 65-72. |
| 6. | Recordon, E. & Griffiths, G.M., (1938). Brit. J. Ophthal., 22, 353. |
| 7. | Schwartzman, J. & Maffia, A. (1939), Arch. Pediat., 56, 240. |
| 8. | Stockard, C.R., (1910), Amer. J. Anat., 10, 369. |
| 9. | Yudkin, A.M., (1927), Amer. J. Ophthal., 10, 341. |
| 10. | Wolff. E., (1958), "The anatomy of the eye and orbit", 4th Ed., Lewis London, pp. 16, 19, 155. |
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