眼眶神经纤维瘤25例临床分析

Clinical review of twenty-five cases of orbital neurofibromas

目的 探讨眼眶神经纤维瘤患者的临床特点及诊治情况，进一步提高对眼眶神经纤维瘤的认识。方法 回顾性分析对中山大学中山眼科中心1999年5月至2006年7月收治的25例眼眶神经纤维瘤，对其临床资料进行整理、分析。结果 10岁以下患者89．5％为眼眶丛状型神经纤维瘤，其中63．2％属于I型神经纤维瘤病，10．5％并发先天性青光眼，10．5％具有阳性家族史。多数有全身皮肤咖啡色素斑、虹膜Lisch结节等改变，诊断容易，但手术难以彻底切除，术后容易复发。10岁以上患者多为孤立型神经纤维瘤，表现类似于一般的眼眶良性肿瘤，CT检查示神经纤维瘤大部分为中等偏低信号，也可表现为负值者。术前定性诊断不易，但手术常能完整切除，术后复发率低。结论 眼眶神经纤维瘤多发于10岁以下小儿，丛状型多见，临床表现多种多样，术前诊断容易，但治疗较为棘手，易复发。

Objective To evalulate the clinical features, diagnosis and treatment of orbital neurofibromas. Methods The medical records of 25 patients with orbital neurofibromas visited between May of 1995 and July of 2006 in Zhongshan Ophthalmic Center of Sun Yat-sen University were retrospectively reviewed. Results In the group of patients with their age of less than or equal to 10 years old, 89.5% of them are plexiform neurofibromas, 63.2% of them belong to type I neurofibromatosis with 10.5% having positive family history, 10.5% of them have congenital glaucoma. Frequently, most of them have typical cafe au lair spots, Lisch nodules （Iris hamartomas） and so on. It is not difficult to make a correct presurgical diagnosis. However, surgical excision is troublesome with a high rate of recurrence. In the group of patients with their age of more than 10 years old, Most of them have isolated neurofibromas. Their clinical features are similar to those of other orbital benign tumors, however, They often show a low to moderate, occasionally even a negative hounsfield value on CT scan. It is not easy to make a correct presurgical diagnosis. The mass can often be removed completely with a low rate of recurrence. Conclusion Orbital neurofibromas occur more often in patients with their age younger than ten years old. Most of them are plexiform neurofibromas with various clinical manifestations. It is easy to make a clinical diagnosis, however, surgical treatment is troublesome with a high rate of recurrence.