Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.
Adult ; Biopsy ; Case Report ; Exophthalmos/pathology* ; Exophthalmos/etiology* ; Exophthalmos/drug therapy ; Glucocorticoids/administration & dosage ; Human ; Magnetic Resonance Imaging ; Male ; Panniculitis, Nodular Nonsuppurative/pathology* ; Panniculitis, Nodular Nonsuppurative/drug therapy ; Panniculitis, Nodular Nonsuppurative/complications*

Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.
Adult ; Biopsy ; Case Report ; Exophthalmos/pathology* ; Exophthalmos/etiology* ; Exophthalmos/drug therapy ; Glucocorticoids/administration & dosage ; Human ; Magnetic Resonance Imaging ; Male ; Panniculitis, Nodular Nonsuppurative/pathology* ; Panniculitis, Nodular Nonsuppurative/drug therapy ; Panniculitis, Nodular Nonsuppurative/complications*

Hand-Schuller-Christian disease, eosinophilic granuloma and Letterer-Siwe disease are classified as histiocytosis-x, on the basis of the underlying proliferation of histiocytes in all three conditions and of their indefinite etiology. The authors experienced a case of Hand-Schuller-Christian disease with exophthalmos. A 17 months old boy was admitted to our Dept. of Ophthalmology because of left exophthalmos since 2 months ago. A biopsy demonstrated that the lesion was Hand-Schuller-Christian disease. The patient was treated with chemotherapy and low dose radiation therapy. We report our experience with this patient along with the review of other papers in this report.
Biopsy ; Drug Therapy ; Eosinophilic Granuloma ; Exophthalmos* ; Histiocytes ; Histiocytosis, Langerhans-Cell ; Humans ; Infant ; Male ; Ophthalmology

PURPOSE: A case of proptosis due to orbital metastasis of neuroblstoma and review of the current literature on neuroblastoma METHODS: A 8-month-old female presented with proptosis due to neuroblastoma involving skull and bone marrow. RESULTS: Proptosis was improved following chemotherapy. CONCLUSIONS: In case of infant who has a proptosis and periorbital ecchymosis without a primary lesion or a specific history, neuroblastoma may be considered as a differential diagnosis.
Bone Marrow ; Diagnosis, Differential ; Drug Therapy* ; Ecchymosis ; Exophthalmos* ; Female ; Humans ; Infant ; Neoplasm Metastasis ; Neuroblastoma* ; Orbit ; Skull

The orbital rhabdomyosarcoma is the most common primary malignant tumor of the orbit in childhood. It was first described in 1854 by Weber and has been increased possibly due to improvement of diagnostic procedures. A 7-year-old boy with the complaints of proptosis and visual disturbance coincidently had an ocular trauma. Incision and drainage of intraorbital hematoma was performed in the right orbit which was confirmed with orbital CT scan. Microscopic findings showed ovoid and spindle shaped rhabdoblasts, hyperchromatic nuclei, and characteristic cross striation in the cytoplasm of the giant cells. He was diagnosed as embryonal rhabdomyosarcoma. The intraorbital hematoma and surrounding tissue in the right orbit were excised and the right eye was enucleated. He received chemotherapy and radiation therapy.
Child ; Cytoplasm ; Drainage ; Drug Therapy ; Exophthalmos ; Giant Cells ; Hematoma ; Humans ; Male ; Orbit* ; Rhabdomyosarcoma* ; Rhabdomyosarcoma, Embryonal ; Tomography, X-Ray Computed

Leukemia accounts for 2 to 11 percent of orbital tumors in childhood. We experienced a case of unilateral proptosis by leukemic cell infiltration, involving the central nervous system in a 3 year old boy. Acute lymphoblastic leukemia was confirmed by bone marrow aspiration cytology and biopsy, and proptosis improved with chemotherapy alone. We report this very unusual condition with a review of pervious reports.
Biopsy ; Bone Marrow ; Central Nervous System ; Child, Preschool ; Drug Therapy ; Exophthalmos* ; Humans ; Leukemia ; Male ; Orbit ; Precursor Cell Lymphoblastic Leukemia-Lymphoma

In adults, it is known that tetastatic tumor to the eye and its adnexa is rare compared with other sites. Orbital tumors can be classified as primary, secondary, and metastatic. Secondary orbital tumors are those that extend to the orbit from a contiguous structure. Metastatic tumors are those that spread to the orbit from more distant site in the body. and their prognosis for life is poor because of widespread systemic malignancy. We experienced a case of metastatic uterine cervical carcinoma of the orbit in a 33-year-old female patient who came to us with a history of sudden proptosis and decreased vision. Eighteen months earlier, the patient had been diagnosed with squamous cell carcinoma of the uterine cervix, and she had undergone chemotherapy, irradiation and surgical intervention. Lung and parametrium metastases were also found six months previously. We report a case of metastatic uterine cervical cancer of the orbit with a review of the literature.
Adult ; Carcinoma, Squamous Cell ; Cervix Uteri ; Drug Therapy ; Exophthalmos ; Female ; Humans ; Lung ; Neoplasm Metastasis ; Orbit* ; Prognosis ; Uterine Cervical Neoplasms

A 3-year-old girl with rapidly progressive proptosis had an unusual malignant germ cell tumor of the orbit identified as an endodermal sinus tumor. The endodermal sinus tumor is a malignant germ cell neoplasm that usually arises in gonads, but on rare ocassion ocurrs in extragonadal locations. Histologic examination revealed pseudo papillary pattern that on occasion created peculiar organoid structure similar to the glomeruli(Shiller-Dubal bodies). The patient was treated with exenteration of left orbit and three drug chemotherapy. The present case is of particular interest because of the rarity of extragonadal endodermal sinus tumor.
Child, Preschool ; Drug Therapy ; Endoderm* ; Endodermal Sinus Tumor* ; Exophthalmos ; Female ; Germ Cells ; Gonads ; Humans ; Neoplasms, Germ Cell and Embryonal ; Orbit* ; Organoids

PURPOSE: Acute lympholastic leukemia is common in children but, rare in adults. Authors experienced a case of acute lympholastic leukemia with orbital relapse in adult, and we report this case with literature review. METHODS: A fifty-year-old woman complained of left eye pain and proptosis for 7 days. The superio-lateral subconjunctival mass excision and biopsy were performed and evisceration was done. The pathologic findings showed the lymphoblastic leukemia. Immunohistochemistry showed the original precursor T-lineage acute lymphoblastic leukemia. RESULTS: The mass was enlarged 40 days after surgery with pain, and subsided after radiation therapy. We recommended systemic chemotherapy, but she refused the therapy. She died 6months after surgery with systemic relapse.
Adult ; Biopsy ; Child ; Drug Therapy ; Exophthalmos ; Eye Pain ; Female ; Humans ; Immunohistochemistry ; Leukemia ; Orbit* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Recurrence*

PURPOSE: This study was carried out to evaluate the age and sex distribution, clinical manifestation, presence of the antithyroid antibody, the clinical outcome following antithyroid drug treatment in children with Graves' disease. METHODS: A total 45 children with Graves' disease were entered into the study. Diagnosis was based on clinical manifestation, elevated thyroid function and increased homogeneous 99mTc thyroid uptake on thyroid scan. All patient were treated with prophylthiouracil or methimazole, and assessed concerning about clinical symptoms and signs, existence of antithyroid antibody and states of thyroid function and outcome following antithyroid drug treatment during 3 years of therapy. Remission was defined as a euthyroid state without clinical manifestation and presence of TRAb, while relapse was defined as the recurrence of hyperthyroidism within 12 months of no medication after antithyroid therapy at least for 3 years. RESULTS: 1) The most prevalent age group was 10 to 15 years(82.8%) and female is more prevalent than male(M : F=1 : 14). 2) Goiter, emotional instability, hyperactivity, palpitation, sweating and exophthalmos are the most common symptoms in orders.3) The detection rate of TRAb, AMA and ATA before treatment were 93.3%, 86.7% and 62.2% respectively. 4) The euthyroid state showed in 36(80.0%) of 45 patients within 8 weeks after treatment. 5) The TRAb values remained positive in 27(60.0%) of 45 patients during the first 1 year of antithyroid therapy. 6) Remission rate was 58.8%(26 cases) and relapse rate of remission cases 73.1%. CONCLUSIONS: The present study suggests that children with Graves' disease will continue to require long term clinical and laboratory assessment after discontinuation of antithyroid drug therapy.
Child* ; Diagnosis ; Drug Therapy ; Exophthalmos ; Female ; Goiter ; Graves Disease* ; Humans ; Hyperthyroidism ; Methimazole ; Recurrence* ; Sex Distribution ; Sweat ; Sweating ; Thyroid Gland