Pantalon Anca, Ștefănache Tudor, Danciu Mihai, Zurac Sabina, Chiseliță Dorin
Opthalmology Clinic, "Sf. Spiridon" University Hospital, Iași, Romania.
Ophthalmology Unit, "Gr. T. Popa" University of Medicine and Pharmacy, Iași, Romania.
Rom J Ophthalmol. 2017 Jul-Sep;61(3):229-236. doi: 10.22336/rjo.2017.41.
Juvenile xanthogranuloma (JXG) is a benign histiocytic skin disorder mainly encountered during infancy and childhood. Although with multiple potential localizations, less than 1% of the cases exhibit ocular manifestations. Some of these might lead to serious complications, specifically, secondary glaucoma that can result in severe and blinding eye disease. The aim of the present case report was to demonstrate typical clinical features, emphasize the difficulties attributed when managing these patients and literature review. We present the case of 4 months old female baby with spontaneous hyphema and secondary unilateral glaucoma due to ocular JXG. The natural history and treatment of the condition were extremely difficult to handle due to multiple opinions in histopathology related to other severe conditions that resembled with the lesions detected in this case: myelomonocytic leukemia and Langerhans cell histiocytosis. Although a minority of patients with JXG have ocular involvement, recognition of this condition is important because a treatment delay can lead to serious complications, such as glaucoma and spontaneous hyphema, as in our case. A thorough differential diagnosis represents the key to a proper management plan in these patients, both on short and long term. "Triple disease" defined as JXG plus neurofibromatosis type 1 (NF-1) and juvenile chronic myelogenous leukemia (JCML) has been reported, but it was not confirmed in our patient.
幼年性黄色肉芽肿（JXG）是一种主要在婴儿期和儿童期出现的良性组织细胞性皮肤疾病。尽管其可能有多个潜在发病部位，但不到1%的病例会出现眼部表现。其中一些可能会导致严重并发症，特别是继发性青光眼，可导致严重的致盲性眼病。本病例报告的目的是展示典型的临床特征，强调在管理这些患者时遇到的困难并进行文献综述。我们报告一例4个月大的女婴，因眼部JXG出现自发性前房积血和继发性单侧青光眼。由于组织病理学方面存在多种与该病例中检测到的病变相似的严重疾病（骨髓单核细胞白血病和朗格汉斯细胞组织细胞增多症）的观点，该疾病的自然病史和治疗极其难以处理。尽管少数JXG患者有眼部受累，但认识到这种情况很重要，因为治疗延迟可能导致严重并发症，如青光眼和自发性前房积血，就像我们的病例一样。全面的鉴别诊断是这些患者短期和长期合理管理计划的关键。已报道有“三联病”，定义为JXG加1型神经纤维瘤病（NF-1）和幼年慢性粒细胞白血病（JCML），但在我们的患者中未得到证实。
