Effect of age at disease onset in the clinical profile of spondyloarthritis: a study of 1424 Brazilian patients.

OBJECTIVES: To analyse demographic and clinical variables in patients with disease onset before and after 40, 45 and 50 years in a large series of Brazilian SpA patients. METHODS: A common protocol of investigation was prospectively applied to 1424 SpA patients in 29 centres distributed through the main geographical regions in Brazil. The mean age at disease onset was 28.56 ± 12.34 years, with 259 patients (18.2%) referring disease onset after 40 years, 151 (10.6%) after 45 years and 81 (5.8%) after 50 years. Clinical and demographic variables and disease indices (BASDAI, BASFI, BASRI, MASES, ASQoL) were investigated. Ankylosing spondylitis was the most frequent disease (66.3%), followed by psoriatic arthritis (18%), undifferentiated SpA (6.7%), reactive arthritis (5.5%), and enteropathic arthritis (3.5%). RESULTS: Comparing the groups according to age of disease onset, those patients with later onset presented statistical association with female gender, peripheral arthritis, dactylitis, nail involvement and psoriasis, as well as negative statistical association with inflammatory low back pain, alternating buttock pain, radiographic sacroiliitis, hip involvement, positive familial history, HLA-B27 and uveitis. BASDAI, BASFI and quality of life, as well as physicians and patient's global assessment, were similar in all the groups. Radiographic indices showed worse results in the younger age groups. CONCLUSIONS: There are two different clinical patterns in SpA defined by age at disease onset: one with predominance of axial symptoms in the group with disease onset ≤ 40 years and another favouring the peripheral manifestations in those with later disease onset.

Orbital metastasis associated with primary breast carcinoma in a man detected during peribulbar anesthesia for cataract surgery.

PURPOSE: A case of orbital infiltration by breast carcinoma in a male patient causing mild enophthalmos and subcutaneous mass detected accidentally during peribulbar anesthesia for cataract surgery is discussed. METHODS: The authors report a case of a 65-year-old man who came to the Ophthalmology Department for cataract surgery. During the presurgery peribulbar injection, a hard palpable mass located under the inferior left eyelid was noted, together with mild enophthalmos. A historical clinical screening revealed that 5 years previously the patient had undergone a right radical mastectomy to treat a ductal carcinoma of the breast. RESULTS: The patient underwent an incisional biopsy of the orbital mass that confirmed the clinical hypothesis of a metastasis. DISCUSSION: This case highlights the importance of the collection and screening of detailed clinical information on the patient before every ophthalmic operation including cataract surgery. The occurrence of progressive enophthalmos can represent an unusual symptom of orbital metastasis, commonly presenting with proptosis and diplopia.

Identification of monoclonal B-cell populations in lymphoid infiltrates of lacrimal gland by rapid cycle polymerase chain-reaction.

Lymphoid hyperplasia of lacrimal gland may be difficult to be differentiated from lymphomas on the basis of morphology and immunohistochemistry. The results of this study indicate that polymerase chain reaction should be employed for confirming the diagnosis of lymphoma in cases with histological and immunophenotypical characteristics of lymphomas, and for detecting monoclonal lymphoid cells in an otherwise non-lymphomatous but dubious or borderline morphological context.

Unsuspected carcinoma of the gall bladder: case report of trocar-site metastasis following laparoscopic cholecystectomy.

Cancer of the gall bladder is a rare malignant neoplasm with an unfavourable prognosis. Laparoscopic surgery has brought about the emergence of possible neoplastic diffusion along trocar tracts in cases where unrecognized carcinoma of the gall bladder is present. The authors present a case of neoplastic abdominal diffusion discovered 4 months after laparoscopic cholecystectomy in which histologic examination of the surgical specimen revealed the presence of unrecognized carcinoma of the gall bladder.

Bile duct hamartomas: diagnostic problems and treatment.

Biliary hamartomas are rare, benign malformations of the biliary tract, usually discovered at autopsy or erroneously confused with metastasis or other cystic hepatic lesions. For these reasons it is necessary to consider them in the differential diagnosis. The authors describe three cases of biliary hamartoma in which the definitive diagnosis was obtained only with the use of histologic examination which represents the only means available that can draw conclusions about the real nature of this lesion.

Papillary cystic neoplasm of the pancreas: a description of two clinical cases.

Papillary-cystic neoplasm of the pancreas is a rare neoplasm principally effecting women in the 2nd and 3rd decades of life. It is considered a low grade malignancy due to it's favourable prognosis and the rarity of metastatic diffusion. Pre-operative diagnosis is rare due to the similarity with other cystic pancreatic lesions. It is for this reason that definitive diagnosis is made at histologic examination of the surgical specimen and total surgical excision represents the therapy of choice. The authors herein describe 2 cases of papillary-cystic neoplasm of the pancreas, thus bringing the total number of cases reported in the world literature to 294.

Epithelial malignancies of the lacrimal gland: survival rates after extensive and conservative therapy.

We reviewed 96 cases of lacrimal gland tumors; 20 patients had epithelial malignancies (11 adenoid cystic carcinomas, 7 carcinomas in pleiomorphic adenomas, 1 mucoepidermoid tumor, 1 adenocarcinoma, and 1 carcinoma in an epidermoid cyst). Classic computed tomographic scan changes associated with lacrimal gland malignancies were found in only 55% of cases. The median survival was 6.75 years in the seven patients treated with extensive surgery (orbital exenteration, possibly associated with osseous resections) and nine years in the 12 cases treated with eye-saving procedures (tumor excision, possibly followed by radiotherapy). Our data demonstrated that extensive surgery for lacrimal gland malignancies does not improve survival. Therefore, a therapeutic protocol is suggested, in which all suspected epithelial tumors (discriminated from inflammatory and lymphoid lesions by computed tomographic scan findings) undergo dacryoadenectomy, without a previous biopsy. Subsequently, in the case of carcinomas, the choice between extensive and eye-saving procedures is based on the extent and aggressiveness of the tumor and clinical findings.

Diagnosis and treatment of orbital hemorrhagic lesions.

Orbital hemorrhagic lesions include a group of heterogeneous conditions that may be idiopathic or caused by trauma, surgery, or preexisting vascular tumors or malformations. We report clinical, computed tomographic, and magnetic resonance imaging features of eight cases of orbital hemorrhage (2 cystic lymphangiomas, 2 subperiosteal hematomas, 1 traumatic hematoma, 1 hemorrhagic varix, 1 cholesterol granuloma, and 1 postsurgical hemosiderin deposit). The differential diagnosis of orbital hemorrhagic lesions may be difficult because of their relative rarity and nonspecific presentation. In particular, on computed tomography, cholesterol granulomas may simulate a lacrimal gland epithelial tumor and subperiosteal hematomas, a lymphoid tumor. Magnetic resonance imaging is an ideal imaging technique because of its ability to identify blood and blood products in all stages of degradation. Treatment can be limited to observation in varices, lymphangiomas, and trauma if vision is not threatened; otherwise, surgical blood drainage should be attempted. Cholesterol granulomas are best treated by curettage through an extraperiosteal orbitotomy. For subperiosteal acute or subacute hemorrhages, surgical drainage is recommended if the blood is not spontaneously reabsorbed within a few weeks.

Prognosis of orbital lymphoid hyperplasia.

BACKGROUND: Orbital lymphoid hyperplasia can be associated with systemic non-Hodgkin lymphoma (NHL), even when polyclonal proliferation is found in the orbit. Although irradiation is recommended, some orbital lymphoid hyperplasias are treated by steroids (when inflammation is clinically presumed) or left untreated. METHODS: The incidence of concurrent NHL and the incidence of future NHL after oral prednisone, radiotherapy, or no treatment were retrospectively evaluated in 33 cases of lymphoid hyperplasia (22 benign lymphoid hyperplasias, BLH, and 11 atypical lymphoid hyperplasias, ALH), after follow-up of 2-13 years. RESULTS: NHL occurred in 12 of 33 cases (36.4%). In seven patients it was concurrent; in five patients it occurred 2-6 years later. In the actuarial curve, at 5 years 55% of patients were free of lymphoma, at 10 years, 46%. NHL was more commonly observed when the lacrimal gland was involved (57% vs 21%; P = 0.03). Of the 13 patients treated with oral steroids, 46% had complete response, 39% partial response, and 15% future NHL. Of the seven irradiated patients, five (71%) had complete response, two (29%) partial response, and none future NHL. Of the eight untreated patients, five (63%) had partial response and three (37%) future NHL. Irradiated lacrimal gland BLHs only achieved partial response, one having radiation-induced orbital inflammation. CONCLUSION: Because of a high risk of NHL, in all orbital lymphoid tumors systemic staging and follow-up are mandatory. The advised management is irradiation, except for Sjögren syndrome, an initially inflammatory lacrimal gland BLH, where a course of steroid is suggested before considering radiotherapy.

Colobomatous ocular cyst excision with globe preservation.

Colobomatous ocular cyst is a rare malformation consisting of a cavity lined by neuroectodermic tissue and communicating with the vitreous cavity, generally associated with microphthalmos. The usual treatment for unsightly cases is enucleation and cyst excision, followed by prosthesis fitting. The authors treated a case of colobomatous ocular cyst associated with a mild microphthalmos by cyst excision and pedicle ligature, via a transconjunctival orbitotomy. The postoperative appearance was satisfactory. Pathology confirmed a thick fibrous wall with calcifications, lymphocytic infiltrates, and neurofilaments and internally lined by dysplastic retinal elements. A conservative approach is advised for colobomatous cysts associated with a globe almost normally in size, in order to avoid cosmetic complications associated with enucleation in childhood.

MRI in Graves orbitopathy: recognition of enlarged muscles and prediction of steroid response.

The purpose of this study is to compare MRI to CT in the recognition of Graves orbitopathy and to compare MRI to clinical examination in the prediction of steroid response. Sixteen patients with dysthyroid orbitopathy (21 orbits) were examined by CT and MRI; muscle enlargement was measured by ultrasonography. Sensitivity in recognizing enlarged muscles was 85.4% for CT and 61.2% for MRI; CT recognized all affected orbits but 1, while MRI failed in 4 cases. Clinical inflammatory signs (p = 0.17) were more reliable predictors of steroid response than muscular T2 hyperintensity on MRI (p = 0.64). In a patient where histological examination documented edematous changes, MRI failed to reveal edema. MRI adds no morphologic information to CT; moreover, T2 intensity is less specific than clinical examination in documenting active disease and forecasting therapeutic outcome.

Primary and secondary orbital melanomas: a clinical and prognostic study.

The clinical, radiologic, and histologic features of 14 cases of orbital melanoma are reviewed. Two cases were presumed to be primary orbital melanomas because no primary pigmented lesion was found in the globe, conjunctiva, or skin. Five cases originated from a conjunctival melanoma, one case from an eyelid cutaneous melanoma, and the remaining six patients had extrascleral extension of a choroidal melanoma. Four patients at the time of first diagnosis already had extraorbital metastases (one patient with extrascleral extension of a choroidal melanoma also had a metastasis to the opposite orbit). The median time interval between diagnosis and metastasis was 14 months. Patients without metastatic disease received radical surgery (partial or total orbital exenteration, in addition to regional lymph node resection if indicated) or conservative treatment (tumor excision plus chemotherapy and interferon). Log-rank test showed an equally poor prognosis for both groups (median time to metastasis was, respectively, 19.5 and 8 months), with no statistically significant difference between the two groups. The authors' reconstructive techniques (dermis-fat grafting and transplantation of temporalis muscle) following partial- and total orbital exenteration are described.

[Draize's test in assessment of eye tolerance to various cosmetic products]. / Tollerabilità oculare di taluni prodotti di cosmesi con il test di Draize.

The methods of Marzulli-Ruggles and Draize were used in an assessment of eye irritation on the part of three children's shampoos. Two products proved irritant with the Marzulli-Ruggles method. The most aggressive product was designated VAS-2-36/A. Better tolerance was observed in albino rabbits when the Draize method was used, though even here some ocular alterations were noted. Once again, VAS-2-36/A was the most irritant substance.

Cosmetic possibilities and problems in eyelid neurofibromas.

Neurofibromatosis type 1 (NF1) is an inherited systemic disease with frequent ocular involvement. A typical alteration observed in NF1 is the eyelid plexiform neurofibroma, often associated with facial homolateral hypertrophy. In such cases, surgical cosmetic results are rather unsatisfactory, since plexiform neurofibromas classically show a non-capsulated mass, with a marked tendency to local recurrence. The techniques for a cosmetic attempt are discussed on the basis of a personal series of eyelid reconstructions in NF1. The results can be considered satisfactory in some cases, when the orbital shape is preserved and the eyelid structure has not been excessively altered by the neurofibroma; in all other cases, the cosmetic outcome is inferior to the patient's and parents' expectations. The serious problem of recurrence is however a limitation to cosmetic surgery in eyelid neurofibromas.

Clinical and radiological presentation of 95 orbital lymphoid tumors.

BACKGROUND: Although most orbital lymphoid tumors are characterized by a slow, painless onset and a mass that molds to orbital structures, different presentations may occur. Intensity on T2-weighted MRI is a possible means for differentiating lymphoid tumors (hyperintense) from pseudotumors (hypointense). In addition, it is generally assumed that 75% of orbital lymphomas are at stage I on presentation. METHODS: The clinical, CT, and MRI presentation of 95 primary and secondary orbital lymphoid tumors (63 non-Hodgkin lymphomas and 32 lymphoid hyperplasias) and their histological grade and staging were reviewed. Immunohistochemistry and PCR were used to determine clonality. RESULTS: No significant differences were found between non-Hodgkin lymphomas and lymphoid hyperplasias, except for the mean age of the patients (6.3 years older in non-Hodgkin lymphomas). In both groups the most common presentation was a mass with an indolent course; visual impairment was seen in 13%, conjunctival redness in 25%, pain in 12%, and acute orbital inflammation in 15% of the patients. In most cases, the CT showed one or more lobulated or rounded masses, molding to adjacent structures, or a wedge-shaped enlargement of the lacrimal gland. Intramuscular lymphomas were rare and always associated with extramuscular masses. On T2-weighted MRI, only 35% of lymphoid tumors were hyperintense. Only 49% of lymphomas were at stage I on presentation. CONCLUSION: An inflammatory presentation is not uncommon in orbital lymphoid tumors. Shape, molding and multiple masses can help radiological diagnosis, whereas MRI T2 intensity is unreliable. Accurate staging can disclose systemic disease in more than 50% of cases of non-Hodgkin lymphoma.

Radical orbital resections.

The authors describe the techniques for subtotal, total and radical orbital exenteration. The aspects of primary and late reconstructive surgery are also discussed, with special reference to Frezzotti's personal technique for temporalis muscle transplantation after subtotal exenteration.