Clinical use of cryopreserved ultra-thick human amniotic membrane for anophthalmic socket contracture.

PURPOSE: To study the use of ultra-thick human amniotic membrane for management anophthalmic socket contracture. METHODS: A prospective study done at King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia. Thirty-six patients (45 eyelids) were involved. Contracted socket caused by trauma, previous surgery or radiotherapy, delay in use of prosthesis, Congenital Anophthalmia/Microphthalmia, and Anophthalmia secondary to Enucleation/Evisceration were included in the study. RESULTS: Thirty-three patients (42 eyelids) underwent fornix reconstruction with cryopreserved ultra-thick human amniotic membrane. Mean ± SD age at surgery was (40.90 ± 17.32) years. Mean follow up was 10.5 months. Grade II fornix contracture was the most common type in 23 (54.8%) eyelids. The most common involved primary diagnosis was Anophthalmia secondary to Enucleation/Evisceration (n = 13). The incidence of pyogenic granuloma (PG) after surgery was seen in 8 eyelids (19.0%). CONCLUSION: Anophthalmic contracted socket secondary to significant history of multiple PG excision (> 5 times) and secondary to enucleation/evisceration were associated with good surgical outcome. Cryopreserved ultra-thick human amniotic membrane is an ideal material for the management of anophthalmic socket contracture.

Expression of Programmed Cell Death-L1 (PD-L1) Protein and Mismatch Repair Mutations in Orbital Tumours-a Pilot Study.

PURPOSE: Programmed cell death protein 1 (PD-1) and DNA mismatch repair (MMR) deficiency play an important role in tumour progression and response to treatment.Both markers have been studied in some ocular tumours but little is known about these markers in orbital tumours. This pilot study reports on PD-L1 expression and MMR mutations using next generation sequencing (NGS) in specific orbital tumours. METHODS: We reviewed surgical specimens from patients with rhabdomyosarcoma, adenoid cystic carcinoma (ACC), pleomorphic adenoma (PA) and biopsy negative tissue from orbital tumours used as a control. immunohistochemistry (IHC) was performed on Formalin fixed paraffin embedded tissue using a PD-L1 antibody. DNA was extracted for targeted gene panel NGS of the MMR genes PMS2, MLH1, MSH6 and MSH2. RESULTS: The study included 17 orbital specimens. Scattered membrane PD-L1 staining was noted in 3/6 rhabdomyosarcoma specimens without an accompanying lymphocytic infiltrate. PD-L1 immunostaining was absent in 3/3 ACC, and 5/6 PA specimens. PD-L1 immunostaining was not detected in 2/2 control specimens. 4/17 samples shared the same pathogenic mutation in the MLH1 gene, including 3/6 rhabdomyosarcoma and 1/3 ACC samples. 1/6 PA samples had a mutation in MSH6. CONCLUSIONS: Our study demonstrated scattered, non-quantifiable or absent PD-L1 staining in a limited sample of orbital tumours suggesting that PD-1/PD-L1 inhibitor therapy may not be useful in treatment of malignant orbital tumours (rhabdomyosarcoma and ACC) when refractory to conventional therapy. Our pilot study suggest that PD-L1/MMR axis might not play a major role in the pathogenesis of primary orbital tumour.

Orbital myeloid sarcoma (chloroma): Report of 2 cases and literature review.

PURPOSE: Myeloid sarcoma (MS) of the orbit is an uncommon condition in occurring in children, generally coupled to myeloproliferative neoplasms. OBSERVATIONS: We describe two rare cases of orbital MS in young boys with aggressive local symptoms but without evidence of acute myeloid leukemia (AML), both patients underwent orbitotomy for gross-tumor resection and biopsy. At follow up, there was no evidence of recurrence nor evolution of the myeloproliferative neoplasms clinically and by radiological and laboratory work-up. We also provide a detailed description of the magnetic resonance imaging presentation, with an extensive pathological analysis correlation. CONCLUSIONS AND IMPORTANCE: A comprehensive revision of the literature on isolated orbital MS was carried out with particular emphasis on clues for differential diagnosis and treatment options, stressing the need to consider MS even in the absence of sign and symptoms of an underlying myeloproliferative disorders.

ExPress mini shunt device with trabeculectomy surgery in patients with uncontrolled glaucoma of Middle Eastern descent.

PURPOSE: The aim of this study is to assess the efficacy and safety of ExPress mini shunt in glaucoma patients of Middle Eastern descent. METHODS: This is a prospective cohort study. Uncontrolled glaucoma patients were subjected to ExPress mini-shunt implant. Pre- and post-operative glaucoma clinical indices were measured and compared. Both intra- and postoperative complications and surgical success rates were assessed. In addition to comparing a group of combined ExPress mini-shunt implant with cataract surgery versus ExPress mini-shunt implant alone. RESULTS: A total of 35 eyes of 31 patients were involved. The most common type of glaucoma was primary open-angle glaucoma in 13 eyes (37.1%). Fourteen eyes (40.0%) were combined ExPress device with cataract surgery. The mean intraocular pressure (IOP) (±standard deviation) at the last visit dropped from 24.6 mmHg (±8.3) to 13.9 mmHg (±4.5). There was a significant reduction in the number of postoperative glaucoma medications from 3.0 ± 0.5 to 1.3 (±0.7). In cases combined with cataract extraction, the patients required fewer anti-glaucoma medications. Complete success was achieved in 22 eyes (63%) and qualified success was achieved in 9 eyes (26%), whereas 4 eyes (11%) were considered a failure. The two most common complications encountered were hypotony (28.6%) and hyphema (11.4%). CONCLUSION: Among the studied population of patients, ExPress offers IOP reduction that is comparable to reported rates following standard trabeculectomy. Postoperative hyphema was encountered at a slightly higher rate.

Conjunctival Pediatric Follicular Lymphoma: Case Report and Literature Review.

Follicular lymphoma (FL) is the second most commonly reported B-cell lymphoma and accounts for 6% of all orbital lymphomas. FL presentation in the pediatric population is rare. This study reported an 18-year-old male with a pink elevated lesion in the conjunctiva of the left eye and normal ocular parameters. Following a complete excisional biopsy, the lesion was oval and smooth grossly and showed dense proliferation with a high nuclear-cytoplasmic ratio microscopically. Immunohistochemical studies revealed positive CD20 and bcl-6 while bcl-2 was negative, which is a common feature for pediatric FL in contrast to bcl-2 positivity found by immunohistochemistry in FL occurring in adult patient population. No signs of recurrence were observed over 2 years of follow-up. This study adds a new case to the existing few reports. Interestingly, all reported cases were males including the patient, mean duration of onset was 5 months, and mean duration of follow-up was 18.4 months. The authors emphasize that total surgical excision with close monitoring is reasonable management of such clinical entity.Follicular lymphoma (FL) is a rare entity in pediatric patients. Six cases have been reported with conjunctival FL in the literature (age between 6 and 21 years). Interestingly, all of them were male including the patient.