Congenital entropion with progeria: 4 going 40.

A 4-year-old male, with progeria, presented with pain, redness, and watering in the right eye since birth [Fig. 1a].

Figure 1

(a) Preoperative presentation with upper lid entropion. (b) Postoperative appearance with ocular surface improvement, at 1 month follow-up. (c) Postoperative outcome at 3 months follow-up

He had a biological age of 40 years, pinched nose facies, fat atrophy, senile and fragile skin. Right eye upper lid was inturned and shortened, inducing a 3 mm lagophthalmos. Posterior lamellar shortening was more than anterior lamellar shortening. The eyelashes rubbed on the cornea, causing superior corneal keratinization. Due to renal compromise, atherosclerosis, failure to thrive, as well as difficult intubation, he was considered high risk for anesthesia and surgery.

Surgery involved strategic placement of a double maternal donor graft as follows:

A mucus membrane graft[1] for posterior lamellar lengthening was placed just inside the upper lid margin [Fig. 2a and b].

Figure 2

(a) Diagrammatic presentation of strategic placement of grafts. (b) Maternal mucus membrane graft placed just inside the lid margin (upper lid everted). (c) Maternal skin graft positioned along the upper lid crease

A full-thickness skin graft was placed just below the brow for anterior lamella lengthening, ensuring that both grafts were not placed one over the other [Fig. 2a-c].

Corneal scraping was done and bandage contact lens was placed. On subsequent follow-up, both grafts integrated well and entropion was corrected [Fig. 1b and c]. At 2 years follow-up, his lid position and ocular surface were well maintained.

Progeria, also known as Hutchinson–Gilford progeria syndrome (HGPS), is a rare genetic condition (1 in 4 million births) that causes accelerated aging. Average life expectancy is 13 years. The commonest eye finding is nocturnal lagophthalmos and exposure keratopathy.[2] Only two cases of progeroid entropion have been reported.[34] Approach to such patients can be a challenge as they are pediatric patients with geriatric problems.

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