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Orbital manifestations of Langerhans Cell Histiocytosis: A report of three cases.

Das JK, Soibam R, Tiwary BK, Magdalene D, Paul SB, Bhuyan C - Oman J Ophthalmol (2009)

Bottom Line: Langerhans Cell Histiocytosis (LCH) is a spectrum of disorders characterized by accumulation of histiocytes in various tissues.It is rarely encountered in ophthalmic practice and has an affinity for the orbit.We report three patients with LCH involving the lateral orbital wall, each with a different form of the condition.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Sri Sankaradeva Nethralaya, Guwahati.

ABSTRACT
Langerhans Cell Histiocytosis (LCH) is a spectrum of disorders characterized by accumulation of histiocytes in various tissues. It is rarely encountered in ophthalmic practice and has an affinity for the orbit. We report three patients with LCH involving the lateral orbital wall, each with a different form of the condition.

No MeSH data available.


Related in: MedlinePlus

Patient 1 – CT scan of the orbit showing an infiltrative mass with irregular margins arising from the lateral orbital wall
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Figure 0002: Patient 1 – CT scan of the orbit showing an infiltrative mass with irregular margins arising from the lateral orbital wall

Mentions: An eight-year-old girl presented with a painless, slowly progressive swelling in the lateral aspect of the left orbit of three months duration. On examination, visual acuity was 20/20 in both eyes (OU). There was a 3 mm proptosis of the left eye (OS) with restriction of abduction [Figure 1]. Palpation revealed a hard non-tender mass over the zygomatic bone. Systemic examination was normal except for raised absolute eosinophil count of 450 cell/mm. Computerized Tomography (CT) scan of orbit showed an infiltrative mass with irregular margins arising from left lateral orbital wall [Figure 2]. Radioisotope bone scan revealed an increased uptake of the involved bone [Figure 3]. Based on the clinical picture an infiltrative or neoplastic lesion was suspected. Curettage of the lateral orbital wall was done. Histopathological examination revealed the characteristic Langerhans cell – a large ovoid mononuclear cell, 15–25 μm in diameter, with a discrete nucleolus, slightly eosinophilic homogenous cytoplasm and Birbeck granules. The diagnosis of LCH was further confirmed by positive S100 protein and adenosine triphosphatase antibody staining on immunohistochemical studies.


Orbital manifestations of Langerhans Cell Histiocytosis: A report of three cases.

Das JK, Soibam R, Tiwary BK, Magdalene D, Paul SB, Bhuyan C - Oman J Ophthalmol (2009)

Patient 1 – CT scan of the orbit showing an infiltrative mass with irregular margins arising from the lateral orbital wall
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2903920&req=5

Figure 0002: Patient 1 – CT scan of the orbit showing an infiltrative mass with irregular margins arising from the lateral orbital wall
Mentions: An eight-year-old girl presented with a painless, slowly progressive swelling in the lateral aspect of the left orbit of three months duration. On examination, visual acuity was 20/20 in both eyes (OU). There was a 3 mm proptosis of the left eye (OS) with restriction of abduction [Figure 1]. Palpation revealed a hard non-tender mass over the zygomatic bone. Systemic examination was normal except for raised absolute eosinophil count of 450 cell/mm. Computerized Tomography (CT) scan of orbit showed an infiltrative mass with irregular margins arising from left lateral orbital wall [Figure 2]. Radioisotope bone scan revealed an increased uptake of the involved bone [Figure 3]. Based on the clinical picture an infiltrative or neoplastic lesion was suspected. Curettage of the lateral orbital wall was done. Histopathological examination revealed the characteristic Langerhans cell – a large ovoid mononuclear cell, 15–25 μm in diameter, with a discrete nucleolus, slightly eosinophilic homogenous cytoplasm and Birbeck granules. The diagnosis of LCH was further confirmed by positive S100 protein and adenosine triphosphatase antibody staining on immunohistochemical studies.

Bottom Line: Langerhans Cell Histiocytosis (LCH) is a spectrum of disorders characterized by accumulation of histiocytes in various tissues.It is rarely encountered in ophthalmic practice and has an affinity for the orbit.We report three patients with LCH involving the lateral orbital wall, each with a different form of the condition.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Sri Sankaradeva Nethralaya, Guwahati.

ABSTRACT
Langerhans Cell Histiocytosis (LCH) is a spectrum of disorders characterized by accumulation of histiocytes in various tissues. It is rarely encountered in ophthalmic practice and has an affinity for the orbit. We report three patients with LCH involving the lateral orbital wall, each with a different form of the condition.

No MeSH data available.


Related in: MedlinePlus