Harrison D A, Mullaney P B, Mesfer S A, Awad A H, Dhindsa H
King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia.
Ophthalmology. 1998 Oct;105(10):1886-90. doi: 10.1016/S0161-6420(98)91035-1.
To determine the safety and efficacy of surgical versus medical management in the treatment of ophthalmic complications of homocystinuria, and also to document ocular complications of homocystinuria other than lens dislocation.
Retrospective case series.
Forty-five patients with ophthalmic complications of homocystinuria participated.
Eighty-four surgical procedures were performed on 40 patients. There were 82 procedures done with the patients under general anesthesia and 2 with the patients under local anesthesia. Medical therapy was attempted initially in all patients with lens dislocation and was the sole therapy used for five patients.
Complications resulting from medical or surgical treatment and final visual acuity were studied.
All patients had a history of lens subluxation or dislocation. Fourteen (31%) were receiving dietary treatment at the time of presentation and 29 (64%) were mentally retarded. Eighty-two procedures were performed with the patients under general anesthesia with 2 surgical complications and 1 postsurgical complication. Lens dislocation into the anterior chamber was the most frequent indication for surgery (50%) followed by pupillary block glaucoma (12%). Prophylactic peripheral iridectomy was not successful in preventing lens dislocation into the anterior chamber in five patients. Anesthetic precautions such as stockings to prevent deep venous thrombosis, preoperative hydration, or aspirin were given in 85% of cases. Other common ophthalmic complications found include optic atrophy (23%), iris atrophy (21%), anterior staphylomas (13%), lenticular opacities (9%), and corneal opacities (9%).
Laser iridectomy was unsuccessful in preventing lens dislocation into the anterior chamber. With appropriate anesthetic precautions and modern microsurgical techniques, the risks associated with the surgical management of ocular complications of homocystinuria are reduced. Surgical treatment should be considered, especially for cases of repeated lens dislocation into the anterior chamber or pupillary block glaucoma. If a conservative, nonsurgical approach is undertaken, these patients must be observed carefully for repeat episodes of lens dislocation.
确定手术治疗与药物治疗在同型胱氨酸尿症眼科并发症治疗中的安全性和有效性，并记录除晶状体脱位外的同型胱氨酸尿症眼部并发症。
回顾性病例系列。
45例患有同型胱氨酸尿症眼科并发症的患者参与。
对40例患者进行了84次外科手术。82次手术在全身麻醉下进行，2次在局部麻醉下进行。所有晶状体脱位患者最初均尝试药物治疗，5例患者仅采用药物治疗。
研究药物或手术治疗引起的并发症以及最终视力。
所有患者均有晶状体半脱位或脱位病史。14例（31%）就诊时正在接受饮食治疗，29例（64%）有智力障碍。82次手术在全身麻醉下进行，有2例手术并发症和1例术后并发症。晶状体脱入前房是最常见的手术指征（50%），其次是瞳孔阻滞性青光眼（12%）。预防性周边虹膜切除术在5例患者中未能成功预防晶状体脱入前房。85%的病例采取了如穿弹力袜预防深静脉血栓形成、术前补液或使用阿司匹林等麻醉预防措施。发现的其他常见眼科并发症包括视神经萎缩（23%）、虹膜萎缩（21%）、前葡萄肿（13%）、晶状体混浊（9%）和角膜混浊（9%）。
激光虹膜切除术未能成功预防晶状体脱入前房。采取适当的麻醉预防措施并采用现代显微外科技术，可降低同型胱氨酸尿症眼部并发症手术治疗的相关风险。应考虑手术治疗，尤其是对于晶状体反复脱入前房或瞳孔阻滞性青光眼的病例。如果采取保守的非手术方法，必须密切观察这些患者是否再次发生晶状体脱位。
