Establishment of a combination scoring method for diagnosis of ocular adnexal lymphoproliferative disease.

Lymphoproliferative diseases (LPDs) of the ocular adnexa encompass the majority of orbital diseases and include reactive follicular hyperplasia (RFH), atypical lymphoid hyperplasia (ALH), and mucosa-associated lymphoid tissue lymphoma (MALToma). Lymphoid follicles (LFs) are usually observed during the histological examination of LPDs. Currently, because there is a lack of specific clinical signs and diagnostic immunohistochemical biomarkers, it is difficult for pathologists to distinguish MALToma from ocular RFH and ALH, which makes the clinical management of these conditions difficult. Here, we analyzed the clinical features of patients with ocular adnexal LPDs (n = 125) and investigated the structure of LFs in paraffin-embedded tissue samples using anti-CD23 and anti-IgD immunochemistry. We found that some clinical features including age, sex, and laterality were different among RFH, LFH, and MALToma. Additionally, immunohistochemistry revealed that the expression of IgD and CD23 was higher in RFH patients and decreased in patients with ALH and MALToma. Moreover, LFs in RFH were intact, whereas the structures of most LFs were disrupted in ALH. In MALToma specimens, few intact LFs were observed. In a further investigation, we combined the results for CD23/IgD immunohistochemistry and the structure of LFs to establish a scoring method for the differential diagnosis of LPDs. According to the BIOMED-2 protocol, we further detected IgH gene monoclonal rearrangement in 73 cases (35 RFH, 17 ALH, and 21 MALToma cases). The sensitivity of our scoring method, based on a comparison with the results of IgH gene monoclonal rearrangement detection, was 85.7% (18/21) for MALToma and 35.3% (6/17) for ALH. Our study provides a method that may be useful for the differential diagnosis of RFH, ALH, and MALToma.

[Clinical analysis of 14 cases of congenital orbital fibrosis].

OBJECTIVE: The purpose of this study was to show the clinical and imaging characteristics in patients with congenital orbital fibrosis. METHODS: A retrospective review of a series of 14 patients with congenital orbital fibrosis hospitalized in Institute of Orbital Disease during 2005 to 2009 have been characterized. Patients aged from 3 months to 18 years old, the median age was 7 years old, 6 cases (43%) were male. RESULTS: Ocular changes were found at birth in all patients, symptoms at onset of the disease included dysfunction of eye movement (12 cases), lagophthalmos (7 cases), diplopia (5 cases), exophthalmos (4 cases), conjunctival congestion (4 cases) and endophthalmos (2 cases). The results of medical examinations including visual acuity, exophthalmos or endophthalmos, diplopia with or without compensative head station, displacement of the eye, lagophthalmos, blephroptosis and ocular dyskinesia all have been record. In the 14 cases, 13 patients have undertaken the CT imaging examination, soft tissue lesions have been found in the orbit with high density and irregular shape, accompanied with thickened ocular muscles and anatomical changes. Of the 9 cases which underwent MRI examination, all of the lesions showed medium signals in T(1)WI, with medium to low signals in T(2)WI. Local excision has been performed in 2 patients, we found that the orbital lesions packaged with hard fibrous tissues around, and the ocular muscles were thick and stark. Pathological examination showed fibrosis and degeneration in the lesions, lacrimal gland and ocular muscles. Some lymphocyte infiltration also has been observed. CONCLUSIONS: Congenital orbital fibrosis is an orbital disease occurs at birth and is characterized with series of signs such as endophthalmos or exophthalmos, ocular dyskinesia and diplopia. Imaging examinations often find abnormal mass with irregular shape and soft tissue density in orbit. Till now, no efficient therapy has been established.

[Preliminary observations of embolization treatment of orbital varices with Glubran 2 acrylic glue].

OBJECTIVE: To evaluate the outcome of embolization via orbital surgical exposure for a series of orbital varices. METHODS: This study is a retrospective review of 28 patients (28 eyes, 11 men, 17 women) which were diagnosed as orbital varix from March to December in 2007, in the Institute of Orbital Diseases. Patient's age ranged from 11 to 54 years old, mean 32 years old. Right orbit 18 cases, left orbit 10 cases. All patients is under general anesthesia, operative approach included lateral orbitotomy (9 cases), anterior orbitotomy (11 cases), transconjunctival (7 cases) and percutaneous puncture (1 case). During the operation, venous malformations were dilatated and punctured with an 20-gauge catheter needle, then injected into 33% Glubran 2 with several punctures with different orientations of the needle were required to reach the different lobules of the venous pouch. RESULTS: Proptosis, visual acuity and eye movement for pre- and post-treatment were observed. All the patients treated with 100% efficiency, in which 16 cases (57%) were complete resolution of the signs and symptoms, no proptosis with Valsalva and correct completely or partially enophthalmos, 12 cases (43%) marked resolution. Six patients partial resection as lesion was stiffen after glue injection, and can touch in anterior part of the orbit. Complications included the vision acuity loss (1 case), limitation of ocular movement (3 cases), and proptosis (2 cases). CONCLUSION: The embolization with Glubran 2 after direct surgical exposure is the safe and effective treatment for orbital varix and can correct some enophthalmos.

[Analysis of the results of surgical management of orbital osteoma].

OBJECTIVE: To analyze the results and skill of surgical management of orbital osteoma retrospectively. METHODS: It was a retrospective case series. Nineteen cases of orbital osteoma (the diagnosis was confirmed by surgical and pathological studies) were analyzed retrospectively in the present study. RESULTS: The location of orbital osteoma was as following: 10 cases in medial wall of the orbit, 4 cases in orbital roof and 5 cases in lateral wall of the orbit. Surgical approaches included anterior orbitotomy (8 cases), lateral orbitotomy (7 cases), and medial orbitotomy (2 cases). Complete resection was performed in 12 cases,while partial resection was done in 7 cases. CONCLUSIONS: Orbital osteoma is easy to diagnosis but difficult in surgical treatment. Appropriate surgical approach and skilled surgical technique are able to reduce the surgical complications.

[Retrospectively analysis of diagnosis and treatment of the orbital neurilemomas].

OBJECTIVE: To evaluate the diagnosis and surgery of orbital neurilemoma. METHODS: Thirty-eight patients with pathologically confirmed orbital neurilemomas were analyzed retrospectively. The age of the patients ranged from 9 years to 69 years. Twenty-three patients were female and fifteen were male. Standardized A/B scan was performed in 37 cases. CT scanning was performed in 38 patients with contrast scanning in 15 patients and MR imaging was performed in 33 patients with contrast scanning in 32 patients. RESULTS: Neurilemoma was generally low to medium reflective or echo in the ultrasound. Of the 38 cases with orbital neurilemomas, location of tumors was superior of the orbit in 17 cases; lateral and inferior in 19 cases and medial in 2 cases. Shape of the tumors was elongate or round-like 26 cases, leaf-like and dumb-bell 12 cases. CT scanning showed soft tissue high density mass in 32 cases, 6 cases were heterogeneity (for cystoid change). MR imaging in 33 patients displayed a identical T1 and a long T2 signal intensity weighted images in 4 cases; identical T1 and T2 mixed signal (short, identical and long signal) in 28 cases; identical T1 and T2 in 1 cases. After Gd-DTPA enhancement in 32 cases, heterogeneity enhancement of the mass was seen in 28 cases, marked enhancement 4 cases. Four cases in all cases was found orbital-cranial communicate mass. Surgical approach was lateral orbitotomy in 26 cases, combined lateral and medial orbitotomy in 2 cases, anterior orbitotomy 4 cases and medial orbitotomy in 2 cases. CONCLUSIONS: Modern medical imaging, especial MR imaging can demonstrate the site, configuration, and characteristics of the tumors and contribute to the diagnosis and treatment. Skill of surgery can improve the rate of success and decrease the complications.

[The features of pathology and immunohistochemistry in orbital meningiomas].

OBJECTIVE: To study the biological characteristics of orbital meningiomas using histological and immunohistochemistrical analysis. METHODS: 19 specimens taking from orbital meningiomas were included in the study. HE staining was used for histology analysis and the immunohistochemistry was applied to demonstrate the expression of Vimentin, EMA, Ki-67, p53 in the tissue of orbital meningiomas. RESULTS: The main configuration of orbital meningiomas was meningothelial, transitional and fibrous meningioma. The expression rations of Vimentin and EMA were 94.75% (18/19), 52.63% (10/19), respectively. The detected rations of Ki-67 and p53 were very lower. CONCLUSIONS: Our study shows that the major histological configuration of orbital meningiomas are meningothelial, transitional and fibrous meningioma. Compare with intracranial meningiomas, the expression rate of EMA in orbital meningiomas is lower. No difference of the expression rates of p53 and Ki-67 are found in benignant, recrudescent and malignant of tumor.

[Diagnosis by ultrasonography and CT scan in orbital vascular tumors and malformations].

OBJECTIVE: To analyze the value of ultrasonography (standardized A/B scan) and CT scan in the diagnosis of orbital vascular tumors and vascular malformations. METHODS: One hundred and eight cases of orbital vascular tumors and malformations were studied, including 98 cases examined by ultrasonography, 108 cases examined by CT scan and 94 cases studied by orbital operation and pathological examination. There were 54 cases of cavernous hemangioma, 26 cases of varix, 13 cases of venous hemangioma, 6 cases of lymphangioma, 5 cases of infantile hemangioma and 4 cases of others disorders. RESULTS: The tumor could be detected in ultrasonography in 96/98 cases (98%), the false negative rate of ultrasonography was 2% (2/98). The nature of the tumor could be determined by ultrasonography in 93% (91/98) cases pre-operatively. The tumor could be found by CT in all cases (108/108). The nature of the tumor could be determined by CT in 92% (99/108) cases pre-operatively. CONCLUSIONS: Combined ultrasonography with CT scan can estimate the nature, extent and location of orbital vascular tumors and malformations and can be useful for selection of the route of orbital operation.

[The value and importance of CT and MRI for diagnosing optic nerve sheath meningiomas].

OBJECTIVE: To study the value and importance of CT and MRI for diagnosing optic nerve sheath meningiomas. METHODS: Fifteen patients with optic nerve sheath meningiomas were analyzed with CT and MRI and were confirmed by operation and pathological examination. RESULTS: The CT image was enhanced in 2 cases of intracranial extension of the optic nerve sheath meningiomas; for the examination of MRI without Gd-DTPA shown the lesion was extended in 2 cases and the positive result of MRI with Gd-DTPA was consistent with the diagnosis of the brain tumor in 9 cases. CONCLUSION: MRI with Gd-DTPA has been proved to be valuable in detecting intracranial extension of optic nerve sheath meningiomas that are not well visualized on enhanced CT and standard MRI, and should be used as a routinely examination of optic nerve sheath meningiomas before and after orbital surgery.