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A high-pressure situation.

Matthew J Miller1, M John Hicks2, Rod Foroozan3

  • 1Baylor College of Medicine, Houston, Texas, USA.

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|August 15, 2022
PubMed
Summary
This summary is machine-generated.

A rare case of isolated orbital Langerhans cell histiocytosis (LCH) in a child caused acute vision loss. Successful chemotherapy resolved the LCH, but induced intracranial hypertension, which was managed with acetazolamide.

Keywords:
CytarabineIntracranial hypertensionLangerhans cell histiocytosis (LCH)Orbital apex syndrome

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Area of Science:

  • Ophthalmology
  • Pediatric Oncology
  • Neurology

Background:

  • Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder of myeloid cells.
  • Orbital involvement in LCH can lead to significant visual impairment, particularly when affecting the orbital apex.

Observation:

  • An 8-year-old boy presented with acute right eye visual loss, nausea, vomiting, and diplopia.
  • Imaging identified a right orbital apex mass, later diagnosed as LCH via biopsy.
  • The patient developed bilateral optic disc edema during cytarabine chemotherapy, indicative of intracranial hypertension.

Findings:

  • The patient was diagnosed with isolated orbital LCH presenting as orbital apex syndrome.
  • Cytarabine chemotherapy led to complete resolution of the LCH lesion.
  • Cytarabine-induced intracranial hypertension was successfully treated with acetazolamide.

Implications:

  • This case highlights the ophthalmologic manifestations of rare orbital LCH in children.
  • It underscores the importance of monitoring for intracranial hypertension during cytarabine chemotherapy.
  • Early diagnosis and multidisciplinary management are crucial for favorable outcomes in pediatric orbital LCH.