Ophthalmic Biophysics, L V Prasad Eye Institute, Hyderabad, Telangana, India.
The Cornea Institute, L V Prasad Eye Institute, Hyderabad, Telangana, India.
Indian J Ophthalmol. 2021 Apr;69(4):860-864. doi: 10.4103/ijo.IJO_2188_20.
To evaluate the outcomes of keratoplasty for xeroderma pigmentosum (XP) performed at a tertiary eye care center.
A retrospective review of medical records of those patients who were clinically diagnosed to have XP (54 eyes of 36 patients) and underwent keratoplasty; either deep anterior lamellar keratoplasty (DALK, four eyes), endothelial keratoplasty (EK, eight eyes), or penetrating keratoplasty (PK, 42 eyes) from 1994 to 2018.
The median age at surgery was 20.6 years (interquartile range [IQR], 14.6-27.6 years) and 20 (55.6%) were males. Graft failure occurred in 15 eyes (35.7%) in the PK group and two eyes (50%) in the DALK group; none failed in the EK group. The probability of graft survival in the PK group was 97.2% ± 2.7% at 1 year, 74.0% ± 8.0% at 2 years, and 54.8% ± 11.7% at 5 years. In the PK group, 13 eyes needed antiglaucoma medications, 11 eyes developed graft infiltrate, and 13 eyes needed secondary interventions (cataract surgery, excision biopsy, and tarsorrhaphy). In the EK group, three eyes needed secondary interventions (excision biopsy). Median postoperative endothelial cell density at the last follow-up in the PK group was 1214 cells/mm (IQR, 623-2277 cells/mm).
Despite the complexities of the ocular surface and adnexal issues in XP, keratoplasty had reasonably good outcomes. More than half of the PK grafts survived 5 years with no failures in the EK group. Regular follow-up and timely management of suture-related infections raised intraocular pressure, and suspicious ocular surface lesions, in addition to solar protection, are important for the success of keratoplasty in these eyes.
评估在三级眼科中心进行的 Xeroderma Pigmentosum（XP）角膜移植的结果。
回顾性分析了 1994 年至 2018 年间，54 只眼（36 例患者）临床诊断为 XP 并接受角膜移植的患者的病历，包括深板层角膜移植术（DALK，4 只眼）、内皮角膜移植术（EK，8 只眼）和穿透性角膜移植术（PK，42 只眼）。
手术时的中位年龄为 20.6 岁（四分位距[IQR]，14.6-27.6 岁），20 只眼（55.6%）为男性。PK 组中有 15 只眼（35.7%）发生移植物失败，DALK 组中有 2 只眼（50%）发生移植物失败，而 EK 组中无移植物失败。PK 组 1 年时移植物存活率为 97.2%±2.7%，2 年时为 74.0%±8.0%，5 年时为 54.8%±11.7%。PK 组中，13 只眼需要抗青光眼药物治疗，11 只眼发生移植物浸润，13 只眼需要二次干预（白内障手术、切除活检和睑裂缝合）。EK 组中有 3 只眼需要二次干预（切除活检）。PK 组中最后一次随访时的中位术后内皮细胞密度为 1214 个细胞/mm（IQR，623-2277 个细胞/mm）。
尽管 XP 患者的眼表和附属器问题复杂，但角膜移植的结果相当不错。超过一半的 PK 移植物在 5 年内存活，而 EK 组无移植物失败。除了防晒外，定期随访和及时处理与缝线相关的感染性眼内压升高以及可疑的眼表病变对于这些患者角膜移植的成功也很重要。
