EVEN A SMALL STAR SHINES IN THE DARKNESS: REPORT OF PEDIATRIC MICROSPHEROPHAKIA SURGICAL TREATMENT

Microspherophakia is a rare, usually bilateral, abnormality of the crystalline lens. It is characterized by reduced equatorial diameter and increased lens thickness. Although the prevalence of this disorder is not entirely known, it may be present in various ocular and systemic conditions, including Weill-Marchesani syndrome, Alport syndrome, homocystinuria and Marfan syndrome. It is usually associated with lens subluxation, resulting in a high degree of lenticular myopia and defective accommodation. Angle-closure glaucoma is a common finding and may be found at the initial presentation.

Surgical procedure and reported footage show the treatment of the left eye. The surgery was performed on 8th August 2020 by Dr. Rui Carvalho and Dr. José Alberto Lemos, in Hospital Pedro Hispano – ULSM.

Report of the surgical treatment of a 3-year-old female patient diagnosed with microspherophakia secondary to Marfan Syndrome, associated lens subluxation and zonular instability.

A 3-year-old female patient with a direct familiar history of Marfan syndrome presented with very high bilateral myopia of -20.0D, objectified by retinoscopy, and variable angle alternating esotropia. During follow-up examinations, lens subluxation was perceived, along with severe myopization of both eyes, and she was proposed to bilateral surgery. During the procedure, lens aspiration and anterior vitrectomy were performed. Due to severe zonular instability with complete capsular bag detachment, the intra-ocular lens (Akreos Adapt AO, Bausch + Lomb^©) was implanted with scleral insertion, using a z-suture technique. During the 2-year follow-up period, the patient had 20/20 best-corrected visual acuity in both eyes, wearing bifocal glasses. She maintained a variable angle alternating esotropia.

Microspheraphakia is a rare but impactful condition mostly related to systemic syndromes that affect protein synthesis. Although most cases are associated with severe zonule instability, making it impossible to implant the intra-ocular-lens inside the capsular bag, the alternative method of scleral insertion shown in this report resulted in a positive functional outcome.