Congenital ectropion of the upper lids.

This report of a case of congenital ectropion is presented not only because of its intrinsic interest and great rarity but also because, so far as one has been able to determine, it is the first case in which an attempt has been made to elucidate the aetiology of the condition by a detailed examination of the lids. Collins (1915) reported a case of a girl aged 4 months who had bilateral partial ptosis, well marked epicanthus and ectropion of the lower lids. The defect in the eyelids was stated to have been present from birth. The lower lacrymal puncta on each side were present but they stood away from the eyeballs. She was able to use the orbicularis muscles to close her eyes and to screw up her lids. In that case 'a wedge-shaped piece of the whole thickness of the right lower lid was cut out a little way internal to the outer canthus. The base of the wedge was at the free margin of the lid and measured about six millimetres in length. A stitch was inserted through the two raw surfaces left, just inside the line of the lashes, and by means of it they were drawn together and united'. The length of the lower lid was thus shortened and drawn up into a better position. Microscopical examination of sections of the lower lid showed a well-developed orbicularis muscle. Mackay (1915) described a similar case but with the distinction that in addition to bilateral eversion of the lower lids there was microphthalmos in both eyes and symmetrical coloboma iridum and a deepseated cyst; below the left lower lid. Von Herrenschwand (1916) reported a case of a man aged 48 years who had bilateral ectropion of the upper and lower eyelids since birth. In that case, even without the patient's history that the condition had been present since birth, attention would have been drawn to a congenital anomaly by the absence of the puncta lacrimalia, and by the presence in each eye of a symmetrically situated conjunctival fold which originated from the fundus of the conjunctival sac, and which was inserted in a pinguecular-like thickening encroaching slightly on to the outer limbus of the cornea just below a horizontal meridian. Gordon and Cragg (1944) reported a case of mild congenital ectropion of all four lids, associated with ptosis of the upper lids. Their patient was a man aged 23 years. He had six brothers and three sisters, of whom two brothers and one sister had a similar but less marked defect. The father of that family was stated to have had a defect of the same nature. Photographs of the four cases mentioned above show that they do not bear a close resemblance to the picture presented by our case and an exhaustive search of the world literature has revealed only two cases similar to the case reported here. Erb (1909) described a case of bilateral congenital ectropion of the upper eyelids in a 2-day-old infant. The father said the changes were seen immediately after birth and that they had increased since birth. Mertens (1920) also reported a case in which there was complete eversion of both upper lids immediately after birth. The mother, a healthy woman aged 42 years, was a seventh para with a generally contracted flat pelvis. All previous births had been difficult, and in all, labour had been prolonged. The ectropion was treated with boric acid and vaseline dressings and eventually completely disappeared but 'there persisted a narrowing of the palpebral fissure especially in the inner canthus and the palpebral fissures were oblique from upper outer towards lower inner angles'. This description, and the photograph of the child, strongly suggest that the case was a mongol, though this possibility was not put forward by Mertens. The child died with convulsions at the age of 4 months.