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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 2 – CT scan of the orbit showing osteolytic lesion in both lateral orbital wall
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Figure 0005: Patient 2 – CT scan of the orbit showing osteolytic lesion in both lateral orbital wall

Mentions: A seven-year-old female reported with periocular fullness OU. There was a bilateral proptosis of 3 mm right eye (OD) and 2 mm OS, respectively, with a palpable rubbery mass over the lateral wall of the orbit OU. The periocular cutaneous tissue and cervical lymph node was also involved on the left side. Ocular motility and ocular evaluation was unremarkable. Systemic examination revealed splenomegaly, which was confirmed by abdominal ultrasonography (USG). Routine blood examination did not show any abnormality except raised erythrocyte sedimentation rate (ESR) and eosinophil count. CT scan of the orbit showed bilateral lytic lesions of lateral orbital wall [Figure 5]. Curettage biopsy of the orbital mass was done in the right side. Histopathological examination and S100 protein test were consistent with a diagnosis of LCH. The presence of splenomegaly favored a diagnosis of Letter–Siwe disease. Considering the multisystem involvement, she was treated with radiotherapy (1000 cGy) in two fractions for orbital bony lesion and chemotherapy (vinblastine 6 mg/m2) to take care of the extra orbital involvement. Reduction in the size of spleen and lytic lesions of the bone were noted following therapy.


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 2 – CT scan of the orbit showing osteolytic lesion in both lateral orbital wall
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 0005: Patient 2 – CT scan of the orbit showing osteolytic lesion in both lateral orbital wall
Mentions: A seven-year-old female reported with periocular fullness OU. There was a bilateral proptosis of 3 mm right eye (OD) and 2 mm OS, respectively, with a palpable rubbery mass over the lateral wall of the orbit OU. The periocular cutaneous tissue and cervical lymph node was also involved on the left side. Ocular motility and ocular evaluation was unremarkable. Systemic examination revealed splenomegaly, which was confirmed by abdominal ultrasonography (USG). Routine blood examination did not show any abnormality except raised erythrocyte sedimentation rate (ESR) and eosinophil count. CT scan of the orbit showed bilateral lytic lesions of lateral orbital wall [Figure 5]. Curettage biopsy of the orbital mass was done in the right side. Histopathological examination and S100 protein test were consistent with a diagnosis of LCH. The presence of splenomegaly favored a diagnosis of Letter–Siwe disease. Considering the multisystem involvement, she was treated with radiotherapy (1000 cGy) in two fractions for orbital bony lesion and chemotherapy (vinblastine 6 mg/m2) to take care of the extra orbital involvement. Reduction in the size of spleen and lytic lesions of the bone were noted following therapy.

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