Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Sankara Nethralaya, Medical Research Foundation, Chennai, Tamil Nadu, India.
Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Aditya Birla Sankara Nethralaya, Kolkata, West Bengal, India.
Indian J Ophthalmol. 2020 Aug;68(8):1604-1608. doi: 10.4103/ijo.IJO_1375_19.
To describe the clinical spectrum and management outcomes of Langerhans cell histiocytosis (LCH) involving the orbit.
Retrospective review of patients with orbital LCH presenting at the Sankara Nethralaya, Chennai, India, over the past 15 years. Demographic details, presenting features, radiology, histopathology, immunohistochemistry, and management outcomes were analyzed.
Nine patients were reviewed. The mean age of presentation was 10.12 ± 14.31 years (range: 6 weeks to 35 years). Eyelid swelling was the most common presenting feature (4, 44.4%), followed by proptosis (3, 33.3%). The mean duration of the presentation was 2.21 ± 2.77 months. Radiological investigations revealed orbital roof osteolytic defects in six (66.6%) patients. Six patients underwent near-complete excision of the mass while three underwent incisional biopsy. Histopathology revealed histiocytes with nuclear grooving and numerous eosinophils characteristic of LCH. The cells were positive for CD1a and S 100 antigens. None of the patients had any systemic involvement. Three received systemic steroids and four received systemic chemotherapy. At a mean follow-up of 17.85 ± 23.46 months, all had complete remission without any signs of recurrence. One patient was lost to follow-up after near-complete excision while one adult patient with a mass in the intraconal space had no recurrence after near-complete excision.
LCH is a rare disorder of the orbit that commonly occurs in children and should be considered a differential for osteolytic lesions involving the orbit. All patients should undergo a systemic evaluation to rule out multifocal disease. The treatment depends upon disease extent and risk factors.
描述累及眼眶的朗格汉斯细胞组织细胞增生症（LCH）的临床谱和治疗结果。
对过去 15 年在印度钦奈 Sankara Nethralaya 就诊的眼眶 LCH 患者进行回顾性分析。分析了患者的人口统计学资料、临床表现、影像学、组织病理学、免疫组织化学和治疗结果。
共回顾了 9 例患者。就诊时的平均年龄为 10.12±14.31 岁（范围：6 周至 35 岁）。最常见的表现为眼睑肿胀（4 例，44.4%），其次为眼球突出（3 例，33.3%）。表现的平均持续时间为 2.21±2.77 个月。放射学检查显示 6 例（66.6%）患者眶顶有溶骨性缺损。6 例患者行肿块近全切除术，3 例行切开活检。组织病理学显示具有核沟和大量嗜酸性粒细胞的组织细胞，具有 LCH 的特征。细胞对 CD1a 和 S100 抗原呈阳性。所有患者均无系统受累。3 例患者接受全身皮质类固醇治疗，4 例患者接受全身化疗。平均随访 17.85±23.46 个月后，所有患者均完全缓解，无复发迹象。1 例患者在接受近全切除术后失访，1 例成人患者眶内有肿块，在接受近全切除术后无复发。
LCH 是一种罕见的眼眶疾病，常见于儿童，应被视为累及眼眶的溶骨性病变的鉴别诊断。所有患者均应进行全身评估以排除多灶性疾病。治疗取决于疾病的范围和危险因素。
