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Langerhans cell histiocytosis with presentation as orbital disease.

Bhanage AB, Katkar AD, Ghate PS - J Pediatr Neurosci (2015 Apr-Jun)

Bottom Line: Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs.In rare instances, the affection of the orbit is the only and the first symptom.He was treated with salvage therapy for risk patients achieving complete remission.

View Article: PubMed Central - PubMed

Affiliation: Department Neurosurgery, Ruby Hall Clinic, Pune, Maharashtra, India.

ABSTRACT
Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration) with acute inflammation (1-week duration) giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009) but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement) and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission.

No MeSH data available.


Related in: MedlinePlus

Preoperative computerized tomography brain showing lytic lesions
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Figure 1: Preoperative computerized tomography brain showing lytic lesions

Mentions: On admission, radiological investigations showed no evidence of hepatosplenomegaly or pulmonary involvement. The computerized tomography (CT) scan of brain showed a 2.1 cm × 2.0 cm well-defined expansile intensely homogenously enhancing mass lesion in the right superolateral orbital rim with breaching of the bony periosteum in the inferior aspect with extraconal orbital spread [Figure 1].


Langerhans cell histiocytosis with presentation as orbital disease.

Bhanage AB, Katkar AD, Ghate PS - J Pediatr Neurosci (2015 Apr-Jun)

Preoperative computerized tomography brain showing lytic lesions
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4489065&req=5

Figure 1: Preoperative computerized tomography brain showing lytic lesions
Mentions: On admission, radiological investigations showed no evidence of hepatosplenomegaly or pulmonary involvement. The computerized tomography (CT) scan of brain showed a 2.1 cm × 2.0 cm well-defined expansile intensely homogenously enhancing mass lesion in the right superolateral orbital rim with breaching of the bony periosteum in the inferior aspect with extraconal orbital spread [Figure 1].

Bottom Line: Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs.In rare instances, the affection of the orbit is the only and the first symptom.He was treated with salvage therapy for risk patients achieving complete remission.

View Article: PubMed Central - PubMed

Affiliation: Department Neurosurgery, Ruby Hall Clinic, Pune, Maharashtra, India.

ABSTRACT
Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration) with acute inflammation (1-week duration) giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009) but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement) and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission.

No MeSH data available.


Related in: MedlinePlus