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Atypical presentation of isolated orbital Langerhans cell histiocytosis
Nikisha Q Richards1, Matthew Young1, Kasey Pierson1
1Department of Ophthalmology, Virginia Commonwealth University, Richmond, VA.
Insights
A rare case of Langerhans cell histiocytosis (LCH) presented as eyelid swelling, mimicking orbital cellulitis in a child. Early diagnosis through biopsy and imaging is crucial for appropriate chemotherapy treatment.
Area of Science:
- Pediatric Ophthalmology
- Histiocytosis
- Orbital Diseases
Background:
- A 9-year-old female experienced a month of fluctuating upper eyelid swelling.
- Initial symptoms suggested orbital cellulitis, prompting an excisional biopsy via anterior orbitotomy.
Observation:
- The patient presented with periorbital edema and erythema.
- Symptoms were initially concerning for orbital cellulitis.
Findings:
- Ophthalmological examination, imaging, and excisional biopsy confirmed Langerhans cell histiocytosis (LCH).
- LCH is characterized by abnormal proliferation of polyclonal Langerhans cells.
Implications:
- This case highlights LCH as a rare cause of isolated orbital involvement in children.
- Prompt diagnosis and chemotherapy, guided by Histiocyte Society guidelines, are essential for managing LCH.
Background:
A 9-year old female presented with one month of waxing and waning upper eyelid swelling. An excisional biopsy via anterior orbitotomy was performed.
Objective:
To describe a patient presenting atypically with symptoms concerning for orbital cellulitis who was diagnosed with Langerhans cell histiocytosis (LCH).
Methods:
Description of case report.
Results:
We report a case of a 9-year old female with one month of periorbital edema and erythema suspected to be orbital cellulitis. A complete ophthalmological exam, subsequent imaging, and an excisional biopsy revealed the diagnosis of LCH. With a confirmed diagnosis, the patient started chemotherapy indicated by the Histiocyte Society Evaluation and Treatment Guidelines.
Conclusion:
Langerhans cell histiocytosis (LCH) embodies a spectrum of diseases with the primary pathologic process being the abnormal proliferation of polyclonal Langerhans cells. In children with isolated bony involvement, the most common presenting symptom is pain. Rarely is orbital involvement with associated periorbital edema and erythema the primary presentation.
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