Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom.
Ophthalmology. 2013 May;120(5):892-8. doi: 10.1016/j.ophtha.2012.10.037. Epub 2013 Mar 6.
To describe the long-term outcomes of peripheral hypertrophic subepithelial corneal degeneration.
Retrospective case series.
Twenty-two patients under the care of the External Disease Service, Moorfields Eye Hospital.
All patients matching clinical diagnostic criteria were included. Symptomatic patients were managed either conservatively or were offered superficial keratectomy in progressive cases where symptom control was inadequate. All excised tissue was examined histologically.
Clinical phenotype, symptoms, recurrence rate after surgery, and histopathologic results.
Twenty-two white patients (20 women and 2 men; age range, 27-88 years; median age, 42 years) had peripheral, usually bilateral (20/22 [91%]), elevated circumferential peripheral subepithelial corneal opacities and adjacent abnormal limbal vasculature, with or without pseudopterygia in 9 patients (41%), among whom 7 (32%) patients had bilateral disease. Apart from the abnormal vasculature, there were no signs of chronic ocular surface inflammation. Symptoms were ocular surface discomfort in 10 patients (45%), reduced vision in 4 patients (18%), and both of these in 5 patients (23%). Three patients (14%) were asymptomatic. There was no treatment in 6 patients (27%), topical lubricants in 8 patients (36%), spectacles in 1 patient (5%), and superficial keratectomy in 7 patients (32%; 5 bilateral), which was repeated for incomplete primary excision in 1 eye of 2 patients (9%) and for a bilateral recurrence in 1 patient (5%). Median follow-up after excision was 5 years (range, 1-11 years). Histopathologic changes were similar to those observed in pterygia (vascular component) and Salzmann's nodular degeneration (corneal component).
Peripheral hypertrophic subepithelial corneal degeneration is an uncommon, usually bilateral, idiopathic disorder, occurring mostly in white women with a distinct phenotype. The condition was first described in 2003. Further cases among some patients have been described in more recent case series of Salzmann's nodular degeneration and diffuse keratoconjunctival proliferation, as well as in the previously described familial pterygoid corneal degeneration. Surgical excision was required in 30% of patients in this series, with infrequent short-term recurrences. The cause is uncertain.
FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
描述周边肥厚性上皮下角膜变性的长期结果。
回顾性病例系列。
莫尔菲尔德眼病医院外眼病科的 22 名患者。
所有符合临床诊断标准的患者均被纳入研究。有症状的患者根据症状的严重程度进行保守治疗或进行浅层角膜切除术，在症状控制不佳的进展性病例中进行手术。所有切除的组织均进行组织病理学检查。
临床表型、症状、手术后复发率以及组织病理学结果。
22 名白人患者（20 名女性和 2 名男性；年龄 27-88 岁；中位年龄 42 岁）患有周边性、通常为双侧性（20/22 [91%]）、环状周边性上皮下角膜混浊和邻近异常的角膜缘血管，9 名患者（41%）存在假性胬肉，其中 7 名患者（32%）患有双侧疾病。除了异常血管外，没有慢性眼表面炎症的迹象。10 名患者（45%）有眼部不适症状，4 名患者（18%）视力下降，5 名患者（23%）同时存在上述两种症状。3 名患者（14%）无症状。6 名患者（27%）未接受治疗，8 名患者（36%）使用眼用润滑剂，1 名患者（5%）配眼镜，7 名患者（32%；5 例为双侧）接受浅层角膜切除术，其中 2 名患者（9%）因初次切除不完全而重复手术，1 名患者（5%）因双侧复发而重复手术。切除后的中位随访时间为 5 年（1-11 年）。组织病理学改变与翼状胬肉（血管成分）和 Salzmann 结节状变性（角膜成分）相似。
周边肥厚性上皮下角膜变性是一种不常见的、通常为双侧的、特发性疾病，主要发生在白人女性中，具有独特的表型。该疾病于 2003 年首次描述。在最近的 Salzmann 结节状变性和弥漫性角膜结膜增殖的病例系列以及之前描述的家族性翼状胬样角膜变性的病例系列中，一些患者中有更多的病例被描述。在本系列研究中，30%的患者需要手术切除，且复发率较低。其病因尚不清楚。
