SURGICAL TECHNIQUE FOR TREATMENT OF SYMBLEPHARON AND MASSIVE PANNUS IN A YOUNG PATIENT WITH EPIDERMOLYSIS BULLOSA DYSTROPHIC HALLOPEAU-SIEMENS

To report our experience, surgical management, and results obtained with the use of amniotic membrane transplantation in a young patient with Epidermolysis Bullosa

 

Hospital Universitario Virgen de la Victoria, Málaga. Spain.

A 16-year-old boy with epidermolysis bullosa dystrophic (EBD) Hallopeau-Siemens presented to our hospital with unilateral loss of vision and impossibility to open or close his left eyelid. Best-corrected visual acuity (BCVA) was 20/20 in his right eye (OD) and hand motion in his left eye (OS). There was a limitation of OS motility secondary to extensive symblepharon from the upper and lower temporal lids to central and temporal corneal area and adjacent bulbar conjunctiva. The affected cornea showed massive neovascularization and opacification. The patient was planned for surgery consisting of symblepharon resection, lamellar keratectomy, and excision of the scarred conjunctiva. An amniotic membrane was used to cover the whole cornea and the bulbar and palpebral conjunctival surface. It was sutured to the corneal margins with a continuous circular suturing with a 10-0 monofilament nylon. It was also sutured to the eyelid margin with interrupted 8-0 polyglactin (Vycril). A silicone fitting symblepharon ring without suture was used to maintain the fornices. It was made a frost suture and a botulinum toxin-induced protective ptosis as the final step of the surgery.

Six months after surgery, the patient recovered full ductions with no symblepharon presence. His visual acuity in OS was 20/70, and the corneal surface was devoid of pannus with some superficial scarring. Fornices were free and well-formed.

Our surgery technique described was found effective in the treatment of massive symblepharon secondary to this rare disease. We present the possibility of avoiding sutures in the fornices, and the usefulness of frost suture and botulinum toxin-induced protective ptosis to improve postoperative outcomes.