病例19　50岁中年女性，主诉左眼视力下降、异物感伴角膜变白5年

CASE 19　A 50-year-old female complaining of decreased vision, foreign body sensation and corneal whitening in her left eye for 5 years

见图1-32。See Fig. 1-32.

鉴别诊断

Differential Diagnosis

◎ 角膜带状变性：常发生于眼部慢性疾病或导致高钙血症的全身性疾病引起的角膜钙化沉着，在Bowman层、上皮基底膜及浅基质层均可见羟基磷灰石的沉积。角膜钙化多见于睑裂区，且在钙化带和角膜缘之间可见清晰的间隙。除引起不同程度视力下降外，还可引起角膜上皮糜烂，从而导致畏光、流泪、眼磨、眼痛等刺激症状。常见的眼部病因包括：慢性葡萄膜炎、晚期青光眼、角膜基质炎、复合性眼外伤、眼球萎缩、眼部结核和内眼手术史。高钙血症的全身病因包括：甲状旁腺功能亢进、痛风、恶性肿瘤、结节病、维生素D中毒等。特发性（如颇为常见的与年龄相关）或长期暴露于有毒蒸气或物质（如汞）也可能导致角膜带状变性。

◎ Band keratopathy: It is a corneal calcif ication caused by chronic diseases of the eye diseases or hypercalcemia. The deposition of calcium hydroxyapatite can be seen on the Bowman’s layer, the epithelium and the anterior stroma.Corneal calcif ication is more common in the interpalpebral area, and a clear gap can be seen between the calcif ication zone and the limbus. In addition to causing varying degrees of vision loss, it can cause corneal epithelial erosion accompanying with ocular irritation such as photophobia,tearing, and eye pain. Etiologies include chronic ocular inf lammation, chronic uveitis, advanced glaucoma, interstitial keratitis, ocular trauma, eyeball atrophy, phthisis bulbi,and history of internal eye surgery. Systemic causes of hypercalcemia include hyperparathyroidism, gout, malignant tumors, sarcoidosis, and vitamin D poisoning. Idiopathic (age related, fairly common) or chronic exposure to toxic vapors or substances (e.g., mercury) also can cause band keratopathy.

◎ 基质型角膜营养不良：为双侧发病的、遗传性角膜疾病，通常不伴炎症及角膜新生血管形成。如颗粒状角膜营养不良，角膜混浊呈“面包屑状”或颗粒状分布于角膜中央及旁中央区，混浊灶之间由透明角膜隔开，且角膜周边不受累。斑块状角膜营养不良的灰白色基质混浊边界不清晰，隔有云雾状混浊区，可累及全角膜及角膜全层。

◎ Corneal stromal dystrophies: It is a bilateral and inherited cor neal disorders without inf lammation or neovascula r i zation in general. Such as granular dystrophy, which appears deposits in the central and paracentral cornea, separated by discrete clear intervening spaces (“bread-crumb-like” opacities), but the corneal periphery is spared. Macular dystrophy appears graywhite stromal opacities with ill-def ined edges extending from limbus to limbus with cloudy intervening spaces, can involve the full thickness of the stroma, more superf icial centrally and deeper peripherally.

◎ 角膜白斑：多见于感染性角膜病、角膜创伤后的并发症及部分先天性角膜发育异常。角膜混浊无特殊形态及部位，混浊区不能透见虹膜，根据是否有原发病因较易鉴别。

◎ Corneal leukoma: It often occurs after infectious keratopathy, corneal trauma and some congenital corneal abnormalities. There is no special shape and location of corneal opacity. The iris can’t be seen at this area. It is easy to be identif ied based on the primary diseases.

病史询问

Asking History

◎ 需要询问眼部症状出现、进展及持续时间，是否伴随畏光、眼痛等刺激症状。

◎ It is necessary to ask about the onset and progre s s ion of ocular symptoms, such as photophobia, eye pain and other symptoms.

◎ 既往是否有慢性葡萄膜炎、晚期青光眼、角膜基质炎等病史，是否有内眼手术史及角膜外伤史，是否有引起高钙血症的全身性疾病，如甲状旁腺功能亢进、痛风、恶性肿瘤、结节病、维生素D中毒，有无长期接触有毒蒸气或物质（例如汞）等。有无家族性或遗传性眼病史。

◎ Any history of eye diseases such as chronic uveitis,advanced glaucoma, interstitial keratitis and history of internal eye surgery or ocular trauma. Patients should also be asked if they have any history of systemic diseases causing hypercalcemia, such as hyperparathyroidism, gout,malignant tumors, sarcoidosis, and vitamin D poisoning. Any history of chronic exposure to toxic vapors or substances (e.g.,mercury); any history of familial or hereditary eye diseases.

◎ 本例患者曾因左眼“孔源性视网膜脱离”行玻璃体切除术和硅油填充术。角膜带状变性是复杂玻璃体视网膜手术后的严重并发症之一。

◎ In this case, the patient had undergone vitrectomy and silicone oil f illing surgery for rhegmatogenous retinal detachment in her left eye. Band keratopathy is one of the severe complications after complicated vitreoretinal surgery.

检查

Examination

◎ 视力：视力受病灶部位影响。若病变区域不在视轴，通常无症状；若病灶居中，视力受损。

◎ Visual acuity: Often asymptomatic without invol v ed visual axial. If central, vision may be affected.

◎ 裂隙灯：可见角膜上皮下、Bowman层和前基质中的钙沉积，通常通过一条透明的角膜细线与角膜缘分开。斑块通常从鼻部和颞部角膜向中央延伸，通常包含小孔和裂缝，具有“瑞士奶酪”的外观。病变晚期可能变成斑块状、结节状和隆起状。

◎ Slit lamp examination: Anterior segment exam i n ation reveals calcium deposits in the subepithelial space, Bowman’s layer, and anterior stroma, and usually separated from the limbus by a thin line of clear cornea. The plaque typically begins at the nasal and temporal cornea and extends centrally.It often contains small holes and clefts, giving it a “Swiss cheese” appearance. Advanced lesions may become plaquelike, nodular, and elevated.

◎ 检查是否有其他眼部慢性疾病：以排除慢性眼部炎症、慢性葡萄膜炎、晚期青光眼、角膜基质炎、眼外伤、眼球萎缩等眼病。

◎ Check other chronic eye diseases: Exclude chronic ocular inf lammation, chronic uveitis, advanced glauc o ma, interstitial keratitis, eye trauma, eyeball atrophy and other eye diseases.

◎ 角膜共聚焦显微镜：于角膜前弹力层和浅基质层可见大量点状高反光沉积物。病变较重者，病变区角膜上皮下可见大量片状高反光结构，基质细胞结构不清，部分患者角膜内皮层可见不规则高反光物质沉积。

◎ Confocal microscope: A large number of spot-like hyperref lective deposits can be seen in the Bowman’s layer and anterior stromal of the cornea. In severe lesions, a large number of sheet-like hyperref lective deposits can be seen under the corneal epithelium, and the shape of stromal cells are unclear. In some patients, irregular hyperref lective deposits can be found in the endothelium.

实验室检查

Lab

◎ 如患者无慢性眼病，为排除引起高钙血症的全身性疾病，须检测血清钙、白蛋白、镁和磷酸盐水平、血尿素氮和肌酐水平。如果怀疑有痛风，检测尿酸水平。

◎ To rule out systemic diseases causing hyperc a l cemia,serum calcium, albumin, magnesium and phosphate levels,blood urea nitrogen and creatinine sho uld be measured if there isn’t any evidence of eye disor ders. Uric acid levels should be measured if gout is suspected.

诊断

Diagnosis

角膜带状变性。

Band keratopathy.

治疗

Management

◎ 轻症患者无须治疗。有轻微不适症状者可选择不含防腐剂的人工泪液点眼。部分角膜带状变性可以自行脱落，使角膜再次恢复透明。发生上皮糜烂引起刺激症状患者，可配戴角膜绷带镜。

◎ Mild lesion: No treatment for patients without any ocular symptoms. If patient feel uncomfortable, preservative free artif icial tear can be used (PRN). Calcium deposits can detach itself in some cases, and the cornea return to be transparent. Bandage contact lens can be used for patients with ocular irritation caused by epithelial erosion for comfort.

◎ 以下治疗可改善患者视力。

◎ The following treatments could improve visual acuity:

去除病灶区角膜上皮并应用0.37%依地酸二钠（乙二胺四乙酸二钠，EDTA）点眼可改善角膜混浊，提高视力。联合表面麻醉后去除角膜病灶区上皮有助于增强药物效果。

Removal of local epithelium and applying 0.37% disodium edetate (EDTA) can improve corneal opacif ication and vision.Removal of the epithelium of the focal area of the cornea after combined topical anesthesia can enhance the effect of the drug.

角膜混浊严重者可手术治疗：角膜表层病灶切除、治疗性准分子激光角膜切削术(PTK)、角膜板层移植术等。

Severe corneal opacity patients should be consi d ered to have surgery including excision of corneal lesions, phototherapeutic keratotomy (PTK), lamellar keratoplasty, etc.

◎ 对眼球萎缩无光感，并且有明显的眼部刺激症状者，为改善外观缓解症状，可以谨慎选择眼球摘除联合义眼植入术。

◎ In order to improve the appearance and relieve the obvious ocular irritation symptom, ophthalmectomy com bined with ocular prosthesis implantation should be con sidered carefully for the patients who have no light perception.

患者教育和预后

Patient Education & Prognosis

◎ 在原发病控制不良的情况下，本病可复发。应积极控制原发病。

◎ Band keratopathy can recur if the primary diseases are not controlled.

◎ 可出现角膜上皮愈合不良问题。

◎ Epithelial healing problems may occur.

◎ 由于斑块剥脱后残留的角膜瘢痕或因合并其他眼部病变，视力通常受损。

◎ Vision is often limited, as a result of residual corneal scarring or other ocular pathology.

◎ 须定期眼科复查，注意眼表保护。

◎ To pay more attention to corneal protection and do ocular examinations routinely.