Orbital involvement in craniofacial brown tumors.

PURPOSE: To describe the clinical and radiologic features of orbital involvement in craniofacial brown tumors and to compare the rate of brown tumors in primary and secondary hyperparathyroidism. METHODS: A retrospective hospital-based study of 115 patients with chronic kidney disease and secondary hyperparathyroidism and 34 with primary hyperparathyroidism was conducted. Laboratory results such as serum levels of alkaline phosphatase, calcium, phosphorus, and parathyroid hormone were recorded. Demographic data (age, sex, duration of disease) and image findings (bone scan scintigraphy, skull and long bone x-rays, CT) were also obtained. The main outcome measures were analysis of clinical, biochemical, and radiologic findings of all patients. RESULTS: Of the 115 patients with chronic kidney disease, 10 (8.7%) had brown tumors in different bones of the skeleton. Five patients had lesions in the craniofacial bones. The maxilla, mandible, maxillary sinus, and nasal cavity were the most affected sites. The orbit was involved in 2 patients with lesions arising in the maxillary and ethmoid sinuses. One patient had facial leontiasis. All patients with brown tumors had extremely high levels of parathyroid hormone (>1,000 pg/ml, normal values 10-69 pg/ml) and alkaline phosphatase (>400 U/l, normal values 65-300 U/l). The mean serum levels of phosphorus and calcium were not abnormal among the patients with brown tumors. Age and time of renal failure were similar for patients with and without brown tumors. Among the patients with primary hyperparathyroidism, only 2 (5.8%) had brown tumors, and in just 1, the lesion was localized in the craniofacial skeleton. A 2-tailed Z test applied to compare the proportion of occurrence of brown tumors in the 2 groups revealed that the difference at the 90% of confidence level was not significant. CONCLUSIONS: Brown tumors are equally found in secondary and primary hyperparathyroidism. Craniofacial brown tumors involve the orbit, usually because of the osteodystrophy process that involves the maxilla and paranasal sinuses. The lesions do not necessarily need to be excised and may regress spontaneously after the control of hyperparathyroidism.

Tumor retro-ocular e abordagem interdisciplinar em cirurgia crânio-maxilo-facial

Objetivos: Tumores intra-orbitários são patologias com diagnóstico diferencial amplo e de abordagem diagnóstica e terapêutica muito específicos. O adenoma pleomórfico de glândula lacrimal faz parte das possibilidades diagnósticas e deve ser tratado cirurgicamente. Relato do caso: Relato de caso ilustrando paciente com tumoração retro-ocular, investigação realizada e condução clínica interdisciplinar. J.M.C., sexo masculino, 44 anos, natural e procedente de Salvador, Bahia. Paciente acompanhado em serviço de oftalmologia foi encaminhado para o Hospital Aristides Maltez(HAM), em setembro de 2007, apresentando tumoração em órbita esquerda associado a lacrimejamento e diminuição da acuidade visual por três meses. A tomografia computadorizada de face demonstrava lesão expansiva sólida em topografia de conteúdo orbitário esquerdo com íntimo contato com o teto da órbita, posterior ao globo ocular, este exoftálmico. Decidido por abordagem interdisciplinar com equipe de Cirurgia de Cabeça e Pescoço e Oftalmologia, em dezembro de 2007, sendo o paciente submetido a cirurgia por acesso supratarsal superior com exérese da tumoração e descompressão da órbita.

Superior oblique muscle amyloidosis mimicking myositis.

A 47-year-old man presented with complaints of progressive diplopia in downgaze and a painful firm mass on the left medial superior canthus. On examination, there was marked hyperemia of the superior bulbar conjunctiva of the left eye. Systemic examination revealed erythematous papules on his trunk and pulmonary infiltrates. CT of the orbits revealed a fusiform enlargement of the left superior oblique muscle and diffuse infiltration of the left temporal region. Biopsy of the left superior oblique muscle and temporal muscle disclosed Congo red deposits that show apple-green birefringence under polarized light. A comprehensive systemic investigation failed to show any disease that could explain the amyloid deposits. The patient was then diagnosed as having primary systemic amyloidosis. We think that this case highlights the necessity of a biopsy in any atypical extraocular muscle enlargement before a diagnosis of myositis.

Association between thyroid autoimmune dysfunction and non-thyroid autoimmune diseases.

PURPOSE: To assess the prevalence of the association between thyroid autoimmune dysfunction in patients with and without Graves orbitopathy and non-thyroid autoimmune diseases. METHODS: Retrospective review of the medical records of 254 consecutive patients with thyroid autoimmune disease with (n = 150) and without (n = 104) orbitopathy who had been followed at the same institution by ophthalmologists and general clinicians. All medical records contained information on any systemic diseases of the patients and a detailed description of their eye examinations. The mean follow-up period was 5.25 +/- 4.67 years. RESULTS: Non-thyroid autoimmune diseases were detected in 24 (9.4%) patients. Type 1 diabetes was the most prevalent non-thyroid autoimmune disease diagnosed in the patients without orbitopathy (7 patients, 6.7%). For the patients with orbitopathy, vitiligo was the most prevalent condition, affecting 6 patients (4%). Other diseases including systemic sclerosis, systemic lupus erythematosus, myasthenia gravis, Sjögren syndrome, and rheumatoid arthritis were seen in a few patients in both groups. The time intervals between the diagnoses of the orbitopathy and the non-thyroid autoimmune disease were highly variable, ranging from none (concomitance of the 2 conditions) to decades. CONCLUSIONS: The present data show that several non-thyroid autoimmune diseases may be associated with thyroid autoimmune dysfunction. Patients with Graves disease without orbitopathy are likely to develop polyglandular syndrome due to the occurrence of type 1 diabetes. Patients with Graves orbitopathy should be screened for other autoimmune conditions, especially vitiligo.