Althaus C, Sundmacher R
Universitäts-Augenklinik, Heinrich-Heine-Universität, Düsseldorf, Germany.
Ger J Ophthalmol. 1996 Jan;5(1):31-5.
Severe Peters' anomaly with dense corneal opacities leads to blindness of the affected eye unless perforating keratoplasty is attempted. The optimal timing of this procedure has yet to be established. We performed keratoplasty early after birth in an attempt to optimally treat amblyopia. In eight eyes of five newborns with severe Peters' anomaly a first keratoplasty was performed at an average age of 54 days. A first control was done under general anesthesia 3 weeks thereafter, with subsequent controls being carried out according to the clinical course. Immunosuppressive therapy mostly consisted of topical steroid eye drops only. In two rekeratoplasty cases, systemic cyclosporin A was given in addition. Apart from the eight primary keratoplasties, three repeat keratoplasties, two lentectomies, and numerous glaucoma operations had to be performed. The average follow-up period was 46 months. As compared with the excellent results reported for penetrating keratoplasty in adults, the results obtained in this special group of newborns remain very poor. The observation of four eyes with a clear or partially clear graft and useful ambulatory vision might suggest a success rate of 50%. However, especially secondary glaucoma seems to be the limiting prognostic factor in the long run. At present, two of the four eyes continue to show uncontrolled intraocular pressure despite multiple surgical interventions, and their prognosis is poor. The performance of perforating keratoplasty in patients with Peters' anomaly early after birth is associated with a multitude of problems, especially glaucoma, and currently grafts can rarely be kept clear for an extended period. We would therefore conclude that it might be wise to postpone surgery until the patient is about 1 year old, in the hope that the overall chance for graft survival might be better at that point, even though persistent amblyopia might be quite severe and limit the functional success.
伴有致密角膜混浊的严重彼得斯异常会导致患眼失明,除非尝试进行穿透性角膜移植术。该手术的最佳时机尚未确定。我们在出生后尽早进行角膜移植术,以最佳地治疗弱视。在5例患有严重彼得斯异常的新生儿的8只眼中,首次角膜移植术的平均年龄为54天。3周后在全身麻醉下进行首次检查,随后根据临床病程进行检查。免疫抑制治疗主要仅包括局部类固醇眼药水。在2例再次角膜移植病例中,还加用了全身环孢素A。除了8例初次角膜移植术外,还必须进行3例重复角膜移植术、2例晶状体切除术和多次青光眼手术。平均随访期为46个月。与成人穿透性角膜移植术所报告的优异结果相比,这组特殊新生儿所获得的结果仍然很差。观察到4只眼的移植物清晰或部分清晰且有有用的可移动视力,这可能表明成功率为50%。然而,尤其是继发性青光眼似乎是长期的限制性预后因素。目前,尽管进行了多次手术干预,但4只眼中仍有2只眼的眼压持续不受控制,其预后很差。出生后早期对患有彼得斯异常的患者进行穿透性角膜移植术会带来诸多问题,尤其是青光眼,目前移植物很少能长时间保持清晰。因此,我们得出结论,将手术推迟到患者约1岁时可能是明智的,希望届时移植物存活的总体机会可能会更好,尽管持续性弱视可能相当严重并限制功能上的成功。