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Treatment strategies and long-term outcomes in patients with congenital microphthalmia–anophthalmia with cyst
British Journal of Ophthalmology ( IF 3.7 ) Pub Date : 2023-06-01 , DOI: 10.1136/bjophthalmol-2020-318089
Sri Gore 1, 2 , Gabriela Grimaldi 2, 3 , Giacomilde Mazzone 2 , Richard Collin 3 , Gustavo Savino 4 , Mario Pagnoni 5 , Alessandra Modugno 2
Affiliation  

Background/Aims Microphthalmia and anophthalmia are rare conditions, which represent ocular maldevelopment; both may be associated with orbital cysts. Current literature recommends retention to stimulate orbital growth during socket rehabilitation but does not illustrate their potential to deform the periocular tissues. This study aims to illustrate the long-term outcomes when other elements, such as forniceal and lid development, are also considered when formulating bespoke treatment plans for patients. Methods Retrospective case series of 78 patients attending a single prosthetics clinic between 1988 and 2020. Clinical and surgical notes, radiological imaging, clinical photographs and patient/doctor satisfaction questionnaires were used to report patient outcomes and natural history data. Results 89 sockets of 78 patients (11 bilateral) were included; average age of presentation being 2.8 years (9 days to 29.5 years). Cysts were clinically detected (48%) or were incidental findings (52%). The mean follow-up time was 7.2 years (6 months to 28 years). Cysts in 46% of sockets underwent surgical excision while the remainder were retained. Satisfaction surveys were obtained for 75 patients, with cosmetic outcomes rated as ‘excellent’ or ‘good’ in 90% of cases by physicians and 97% of cases by patients or guardians. Conclusion The favourable long-term outcomes in this study have resulted from bespoke plans which considered periocular tissue development, regional orbital growth and orbital volume replacement. The authors contemplate cyst excision if the prosthetic fitting or retention is impeded by the cyst as this often heralds the increased risk of long-term periocular distortion. All data relevant to the study are included in the article or uploaded as online supplemental information.

中文翻译:

先天性小眼畸形-无眼畸形伴囊肿患者的治疗策略和长期结局

背景/目的 小眼畸形和无眼畸形是罕见的情况,代表眼部发育不良;两者都可能与眼眶囊肿有关。目前的文献建议保留以刺激眼窝修复期间的眼眶生长,但并未说明它们可能使眼周组织变形。本研究旨在说明在为患者制定定制治疗计划时还考虑其他因素(如穹窿和眼睑发育)时的长期结果。方法 回顾性病例系列,包括 1988 年至 2020 年间就诊于一家假肢诊所的 78 名患者。使用临床和手术记录、放射影像学、临床照片和患者/医生满意度调查问卷来报告患者结果和自然历史数据。结果 78例患者(11例双侧)的89个牙槽窝被纳入;平均发病年龄为 2.8 岁(9 天至 29.5 岁)。囊肿是临床检测到的 (48%) 或偶然发现的 (52%)。平均随访时间为 7.2 年(6 个月至 28 年)。46% 的牙槽囊肿接受了手术切除,其余的则保留了下来。对 75 名患者进行了满意度调查,90% 的病例被医生评为“优秀”或“良好”,97% 的病例被患者或监护人评为“优秀”或“良好”。结论 本研究的良好长期结果源于考虑了眼周组织发育、区域眼眶生长和眼眶体积置换的定制计划。如果假体安装或保留受到囊肿的阻碍,作者会考虑囊肿切除术,因为这通常预示着长期眼周变形的风险增加。与研究相关的所有数据都包含在文章中或作为在线补充信息上传。
更新日期:2023-05-19
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