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[Intraorbital extraocular Langerhans' cell granuloma: primary surgical approach. Why? - case report]
João Guilherme Valentim Neto1, Cristina Góes Schaurich
1Serviço de Neurocirurgia do Hospital São Lucas da Pontifícia Universidade Católica do Rio Grande do Sul - Porto Alegre (RS) - Brasil. jgvalentim@uol.com.br
Insights
A pediatric case of intraorbital Langerhans' cell granuloma (LCG) was successfully treated with urgent orbitotomy. This surgical intervention resolved proptosis, ophthalmoplegia, and optic disc edema, preserving visual function.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Neurosurgery
Background:
- Langerhans' cell granuloma (LCG) is a rare neoplastic proliferation.
- Intraorbital LCG can cause significant visual compromise due to mass effect and intraorbital hypertension.
- Urgent surgical intervention may be necessary to prevent irreversible vision loss.
Observation:
- A 2-year-old male presented with conjunctival proptosis, red eye, complete ophthalmoplegia, exophthalmos, non-reactive mydriasis, and blepharoptosis of the right eye.
- Fundoscopy revealed venous enlargement and optic disc edema, indicative of elevated intraorbital pressure.
- Imaging confirmed an intraorbital mass consistent with LCG.
Findings:
- Urgent orbitotomy via a cranial approach was performed to excise the intraorbital lesion.
- Histopathological examination confirmed Langerhans' cell granuloma.
- The patient experienced complete remission of clinical symptoms and restoration of normal orbital anatomy post-surgery.
Implications:
- Surgical decompression through orbitotomy can be a critical first-line treatment for intraorbital LCG presenting with severe visual compromise.
- This case highlights the importance of prompt surgical management in preventing permanent visual deficits from intraorbital LCG.
- Multidisciplinary management, including surgical, medical, and potentially radiation therapy, should be considered for LCG based on clinical presentation and extent.
Abstract:
We present a case in which a patient with intraorbital Langerhans' cell granuloma, with anatomical and functional skills compromised by intraorbital hypertension, which was treated with urgency orbitotomy by cranial approach. Male, two years-old, presented conjuntival proptosis, red eye, complete ophthalmoplegy, exophthalmos, with non-fotoreactive mydriasis and blepharoptosis of the right eye. Fundoscopy presented venous enlargement with optic disc edema. A cranial approach with orbitotomy was carried out, removed the lesion with total remission of clinical presentation and anatomic appearance as well. The handling of Langerhans' cell granuloma can vary from primary surgical approach as corticotherapy to chemotherapy and radiotherapy. In this case, the surgery was the first option due to the imminent risk of loss of visual function.
