Ummar Shiji, Bhalekar Swapnil, Sangwan Virender
Cornea and Anterior Segment Services, LV Prasad Eye Institute, Hyderabad, Andhra Pradesh, India.
BMJ Case Rep. 2014 Jan 31;2014:bcr2013203091. doi: 10.1136/bcr-2013-203091.
A 6-year-old girl, a case of xeroderma pigmentosum (XP), presented with a visual acuity (VA) of 20/20 and 20/400 in the right and left eye, respectively. For a diagnosis of vascularised corneal scar, penetrating keratoplasty was performed in the left eye twice and ultimately graft failed following multiple episodes of rejection. Type I keratoprosthesis was performed in the left eye. She continues to maintain VA of 20/40 in the left eye for more than a year. Similarly, a 24-year-old man, a case of XP, presented with VA of finger counting at 1 metre in both eyes. Ocular examination showed bilateral vascularised corneal scar and conjunctivalisation. Type I keratoprosthesis was performed as primary procedure in the left eye. He maintains a VA of 20/30 for more than a year. Type I keratoprosthesis could be a primary procedure for visual rehabilitation in patients with XP with severe ocular surface disease.
一名6岁女童，患有着色性干皮病（XP），右眼视力（VA）为20/20，左眼为20/400。为诊断血管化角膜瘢痕，左眼进行了两次穿透性角膜移植术，但最终在多次排斥反应后移植失败。左眼进行了I型人工角膜植入术。她的左眼视力持续保持在20/40以上达一年多。同样，一名24岁男性，也是XP患者，双眼视力为1米处手指计数。眼部检查显示双侧血管化角膜瘢痕和结膜化。左眼首次手术即进行了I型人工角膜植入术。他的视力保持在20/30以上达一年多。对于患有严重眼表疾病的XP患者，I型人工角膜植入术可为视力康复的首选手术。
