Transmucosal Boston Keratoprosthesis Type II in a Case of Severe Bilateral Chemical Burn.

PURPOSE: The purpose of this report was to describe a new alternative surgical technique combining oral mucosa and the Boston Keratoprosthesis type II (BKPro II) in a patient with severe chemical burn. METHODS: We present a case of a 37-year-old man who suffered a severe bilateral chemical burn. Visual acuity was light perception and projection in both eyes. After many surgeries, including eyelid reconstruction, eyelashes electrolysis, and amniotic membrane transplants, he presented a corneal thinning in his right eye that was managed with an autologous oral mucosa graft. Considering the limited function of the eyelids, mild symblepharon, and dry ocular surface, we proposed the implantation of a BKPro II. The patient voiced his desire of avoiding complete tarsorrhaphy for better cosmesis. Osteo-odonto-keratoprosthesis or tibial bone keratoprothesis was also discarded at the patient's express wish. A year and a half later, the oral mucosa was transplanted, and once the ocular surface was stabilized, we lift the mucosa 270 degrees and performed the implantation of the BKPro II covering this device with the mucosa trephined avoiding the complete tarsorrhaphy and theraby modifying the standard procedure. RESULTS: After 34 months of follow-up, the patient did not develop any complications, his visual acuity is stable (0.3 decimal), and funduscopic examination showed a pale optic disk, vascular tortuosity, and an epiretinal membrane that remains stable up to this period. CONCLUSIONS: Transmucosal BKPro II may be considered as an alternative surgical technique for implant support in BKPro II carriers who want to improve their cosmesis avoiding, avoid complete tarsorrhaphy, and do not want to be exposed to dental or bone surgeries.

Surgical Management of Ocular Cicatricial Pemphigoid in a Pediatric Patient.

PURPOSE: The purpose of the report was to describe the first successful tarsal fracture surgery in a 1-year-old boy diagnosed with cicatricial ocular pemphigoid whose visual and psychomotor development were notably limited. METHODS: We present the case of a 1-year-old boy diagnosed with mucous membrane pemphigoid by biopsy who was treated with rituximab (375 mg/m 2 intravenous infusion at 2-week interval administered twice) and stable with oral dapsone (2 mg·kg -1 ·d -1 ). His eyelid cicatricial entropion and trichiasis in both eyes prevented him from opening his eyes, impeding visual development. After 1 year of clinical stability, we performed a tarsal fracture procedure in both eyes to restore eyelid anatomy and functionality, with the aim to prevent an inflammatory reaction, administrating intravenous dexamethasone before and after surgery. RESULTS: The intervention was successfully performed without postoperative complications. Excellent anatomic and functional results allowed him to develop normally in his daily life the first week after surgery. He is currently taking oral dapsone (2 mg·kg -1 ·d -1 ) as a maintenance treatment to stop the progression of the disease. CONCLUSIONS: Tarsal fracture surgery may be considered part of the treatment in pediatric patients with stable ocular cicatricial pemphigoid presenting with severe entropion and trichiasis.

Childhood ocular mucous membrane pemphigoid successfully treated with rituximab.

Ocular mucous membrane pemphigoid (MMP) is a chronic autoimmune blistering disease that usually affects elderly patients being extremely rare in pediatric population. Despite aggressive immunosuppressive therapy, ocular MMP may progress causing significant morbidity. Herein, we describe a toddler with ocular MMP successfully treated with rituximab.