A Unique Case of Solitary Supraorbital/Intracranial Langerhans Cell Histiocytosis Presenting Primarily with Ocular Manifestations and a Review of Literature

Case: A 25 year-old male presented with acute onset periorbital edema associated with right eye pain waking him from sleep, conjunctival injection and 12 days of progressively worsening temporal headaches. He had right-sided ptosis but was otherwise neurointact and without proptosis. Noncontrasted CT maxillofacial at outside hospital demonstrated a soft tissue mass in the right intraorbital, extraconal, and intracranial (extradural) compartments. Lytic changes of the superior orbital rim, as well as the inner and outer table of the right frontal bone were noted. Ophthalmology was consulted who diagnosed ocular hypertension likely secondary to external compression. He was transferred to our institution for higher level of care where he underwent a right orbitozygomatic craniotomy with supraorbital osteotomy for resection of extradural skull base Langerhans' cell histiocytosis lesion with resolution of clinical symptoms.

Review of the Literature: Langerhans' cell histiocytosis (LCH) is a disease of dendritic cell proliferation that may vary along a spectrum of presentations, from incidental benign discovery to life-threatening multisystem disease. Adult onset LCH is rare, with peak incidence between 1 to 3 years of age.[1] The skeletal and pulmonary systems are the most commonly involved organ systems in adults.[2] Of these, approximately 51% of bony lesions are located in the skull, with 30% in the jaw.[3] LCH orbital lesions are significantly more common in pediatric patients than adults.[4] Unifocal orbital LCH with intracranial extension is an extremely rare condition, even more so in adult patients. Still, the condition should be considered in the differential diagnosis of orbital and skull base lesions. Approaches to treatment are driven by disparate patient reports and differing interpretations of the underlying disease process. While progress has been made in the understanding of the pathophysiology of the disease, much remains a matter of debate. We discuss different treatment options and review the literature.

Fig. 1 MRI brain with contrast demonstrating a right intraorbital LCH lesion with intracranial, extradural invasion. LCH, Langerhans' cell histiocytosis; MRI, magnetic resonance imaging.

No conflict of interest has been declared by the author(s).

• References

• 1 Carneiro I, Friande A, Araújo M. A case of orbital langerhans cell histiocytosis in an adult. Acta Med Port 2019; 32 (09) 617-620
• 2 de Menthon M, Meignin V, Mahr A, Tazi A. Adult Langerhans cell histiocytosis. Presse Med 2017; 46 (01) 55-69
• 3 Stockschlaeder M, Sucker C. Adult Langerhans cell histiocytosis. Eur J Haematol 2006; 76 (05) 363-368
• 4 Maia RC, de Rezende LM, Robaina M, Apa A, Klumb CE. Langerhans cell histiocytosis: differences and similarities in long-term outcome of paediatric and adult patients at a single institutional centre. Hematology 2015; 20 (02) 83-92

Publication History

Article published online:
12 February 2021