RESUMO -O acompanhamento a longo prazo de pacientes submetidos a tratamento de tumores da região parasselar é importante para detectar complicações tardias da terapêutica. Neste estudo avaliamos 6 pacientes com craniofaringioma, 1 com meningioma, 1 com germi¬ noma e 1 com cisto epidermóíde, localizados na região parasselar. Eles haviam sido tratados, em média, 3,8±3,2 anos antes, por cirurgia e radioterapia (6 casos) ou somente cirurgia (3 casos). Cinco pacientes eram do sexo feminino e a média de idade era de 24,3±18,8 anos. A avaliação consistiu na infusão endovenosa de TRH (200 mg), GnRH (100 mg) e insulina regular (0,1 UI Kg/peso), bem como na dosagem dos hormônios hipofisários antes (0) e após 20, 40, 60 e 80 minutos. Encontramos os seguintes resultados: (a) resposta deficitária do GH e do cortisol era todos pacientes; (b) 7/9 pacientes não tiveram respostas adequadas do FSH e 3/9 do L.H; (c) 4/9 tiveram respostas inadequadas da prolactina e 2/8 do TSH. Concluimos que: (a) o déficit de GH e cortisol são os mais frequentes nestes pacientes; (b) a lesão após radioterapia pode localizar-se tanto no hipotálamo quanto na hipófise ou, ainda, em ambos; (c) a sensibilidade das células hipofisárias e hipotalâmicas à irradiação é diferente de acordo com os hormônios que produzem; (d) é necessário o acompanhamento endocrinológico frequente dos pacientes submetidos a tratamento de tumores parasselares, a fim de detectar déficits hormonais tardios. SUMMARY -Long term follow-up of patients submitted to treatment of paraselar tumours region is important for the detection of late therapeutic complications. In this study the authors conducted an evaluation of six patients with craniopharyngioma, one with germinoma, one with meningioma, and one epidermoid cyst. All above tumours were localized at paraselar region. Six out of nine patients had been treated both by surgery and by radiotherapy and the other three surgically only, on an average 3.8±3.2 years before this observation was carried out. Five patients were female with their ages average 24.3±18.8 years old. Evaluation consisted: in the first place, an intravenous infusion of thyrotropin-releasing hormone (TRH, 200 mg), gonadotropin-releasing hormone (GnRH, 100 mg), and insulin tolerance test (0,1 IU/Kg, regular insulin); and secondly, in measurements of pituitary hormones secretion at different time points -0, 20,40, 60 and 80 minutes. We found both diminished response of growth hormone and Cortisol in all the patients. Seven out of nine patients did not have adequate response to follicle-stimulating hormone.Three out of nine responded unsatisfactory to luteinizing hormone. Four out of nine showed inadequate responses to prolactin as well as, two out of eight to thyrotropin.We concluded that: (a) growth hormone and Cortisol deficiency are the most frequent finding in these patients; (b) post-radiotherapy lesions can be located in the hypothalamus or pituitary, or even in Trabalho realizado na

The case of a male child with Russel's syndrome due to a pilocytic astrocytoma located in the diencephalic region is presented. The diagnosis was made in the 16th month of age, but symptoms began in the 4th months of life, when he started losing weight. By the time he was admitted weight was 6150g and he was 74cm tall, with an emaciated aspect, no panniculus adiposus, irritated, and with symptoms of intracranial hypertension. There was convergent strabismus, vertical nystagmus of the left eye and bilateral papilledema. Tendinous reflexes were exacerbated and he had spastic tetraparesis. The endocrine evaluation showed a basal raise of GH (23ng/ml), TSH (6.2mUI/1) and prolactin (26ng/ml). The first two hormones did not respond to the acute test with TRH, while prolactin had a poor response. He was submitted to radiotherapy with linear acceleration (total dose of 4000 rads) and surgery, during which the tumor could not be completely removed due to its large size. After 9 months, the child is doing well, with a considerable weight gain (2500g).

Seven cases of compromised pituitary fossa at the conventional skull X-ray, who had the final diagnosis of giant aneurysm of the intracavernous portion of the carotid artery (6 cases) and one of the anterior communicating artery, are reported. The main findings were: headache (7/7), complex ophthalmoplegia involving the III, IV and VI cranial nerves (5/7), compromised V cranial nerve (4/7) and eyeball pain (4/7). Other manifestations were: meningeal signs (2/7), unilateral blindness (1/7), hemiparesis (1/7), cacosmia (1/7) and inferior bitemporal quadrantanopsia (1/7). Five patients with intracavernous carotid artery aneurysm showed benefits with progressive occlusion of the internal carotid artery at the cervical level. One died before surgery. The case with anterior communicating artery aneurysm improved after its surgical clipping. Our data, in accord with the literature, support the conclusion that the differential diagnosis of aneurysms in the parasellar region remains a very difficult task. The accurate final diagnosis requires cerebral angiography and the surgical treatment with progressive occlusion at the cervical portion of the internal carotid artery has a relatively low risk with promising results.

RESUMO -Os autores apresentam o caso de uma paciente de 45 anos de idade com história de 15 meses de evolução, de hemiatrofia progressiva da face e língua à esquerda. A investigação laboratorial e de imagens foi normal, incluindo exames de sangue de rotina, líquido cerebro-espinhal, provas de função hepática, renal e reumáticas; além de raio X, tomografia computadorizada e ressonância magnética de crânio. A eletroneuromiografia mostrou alterações neurogênicas periféricas dos nervos trigêmio motor e hipoglosso, predominantemente à esquerda. Esses achados são compatíveis com o diagnóstico de hemiatrofia facial de Romberg, motivando a discussão de diversos aspectos desta rara doença de acordo com o relato e com a literatura.PALAVRAS-CHAVE: hemiatrofia facial, síndrome de Parry-Romberg, doença de Romberg. Romberg's facial hemiatrophy: case reportSUMMARY -The authors report the case of a 45-years-old female patient, with progressive hemiatrophy of the left face and tongue. The laboratorial and image studies were normal, including routine blood exams, cerebral-¬ spinal fluid, hepatic function tests, renal and rheumatic; besides X ray, computerized tomography and magnetic resonance of skull. The electroneuromyography showed peripheral neurogenic alterations of motor trigeminal and hypoglossal nerves, markedly on the left side. Those findings are compatible with Romberg's facial hemiatrophy diagnosis, motivating discussion of several aspects of this rare disease according to the account and literature.KEY WORDS: facial hemiatrophy, Parry-Romberg syndrome, Romberg's syndrome. O objetivo deste trabalho é relatar um caso de HFR e discutir alguns aspectos relevantesda doença. A hemiatrofia facial de Romberg (HFR),

RESUMO -Relatamos caso de tratamento cirúrgico com instrumentação anterior em espondilite tuberculosa (mal de Pott), em mulher de 71 anos, que se encontrava em tratamento para tuberculose na forma pulmonar e evoluiu com dor lombar, dificuldade progressiva para deambular, cifose e disfunção vesical. A ressonância magnética revelou lesão nos corpos vertebrais de T12 e L1 com abcesso paravertebral. A paciente foi tratada cirurgicamente por acesso tóraco-abdominal lateral. Os corpos vertebrais foram ressecados, foi instalado uma gaiola vertical (mesh cage) e a coluna foi estabilizada com placa lateral (Z plate) e parafusos fixados em T11 e L2. Essa técnica permite boa artrodese dos corpos vertebrais remanescentes e boa estabilidade da coluna, sem comprometer a eficácia dos tuberculostáticos. A paciente foi avaliada um ano após a operação e não apresentava déficit motor, dor residual e a cifose foi desfeita.PALAVRAS-CHAVE: mal de Pott, tratamento cirúrgico, instrumentação anterior.Anterior instrumentation in tuberculous spondylitis (P Anterior instrumentation in tuberculous spondylitis (P Anterior instrumentation in tuberculous spondylitis (P Anterior instrumentation in tuberculous spondylitis (P Anterior instrumentation in tuberculous spondylitis (Pott's disease): case report ott's disease): case report ott's disease): case report ott's disease): case report ott's disease): case report ABSTRACT -We report a case of a surgical treatment with anterior instrumentation in tuberculous spondylitis (Pott's disease), in a 71 years old woman, that was in treatment for pulmonary tuberculosis, with lumbar pain, progressive disability to walk, kyphotic deformit and vesical dysfunction. Magnetic resonance image presents a lesion in the bodies of T12 and L1, with paravertebral abscess. The patient was treated surgically by transthoracic-abdominal approach. The vertebral bodies were cut off and the spine were instrumented anteriorly with a mesh cage and a Z plate. This procedure permits a good arthrodesis and a immediately stabilization of the spine, without any complication of the infection. The patient was seen a year after the surgery and is free of infection, without motor deficit, pain or reminiscent kyphosis.KEY WORDS: Pott's disease, surgical treatment, anterior instrumentation. 1 . Dentre os vários locais atingidos pela doença, o pulmão é o mais frequente e a afecção óssea representa a quinta mais comum nas formas extrapulmonares 2 . Dentre estes casos 50% apresentam doença na coluna vertebral [2][3][4] . A concomitância pulmonar e vertebral ocorre em 10-15% dos pacientes 2,3,5 . As vértebras torácicas inferiores e lombares são as mais frequentemente afetadas, seja por contigüidade pleural ou por disseminação hematogênica. A lesão inicial é na porção anterior do corpo vertebral acarretando a destruição deste e o aparecimento da cifose (giba), característica do mal de Pott 2,3,5 .O tratamento ideal para TB espinhal quando existe déficit neurológico, resistência bacteriana, imunossupressão, instabilidade ou deformidade impor...

The case of a three-years-old male with intracranial hypertension is reported. Without a clear clinical diagnosis, a gradual deterioration ocurred before his death, 8 days later. The neuropathological examination of the brain revealed a tumor with indistint borders at the right lateral ventricle, with granulous surface, pink-gray colour, irregular necrotic and hemorragic areas, invading the adjacent brain tissue. The histopathological study revealed a malignant variant of the choroid plexus tumor, of extreme rarity in the central nervous system. The biological and histological criteria accepted by Lewis, Rubinstein and Polak for the correct diagnosis of this type of tumour are discussed.

Durante o período de seis meses (janeiro a julho de 1991), 66 pacientes portadores de traumatismo craniencefálico (TCE) grave (graduados na Escala de Coma de Glasgow em igual ou menor que 9) foram analisados de forma prospectiva quanto aos procedimentos a que foram submetidos du¬rante o tratamento e as complicações resultantes destes procedimentos. Os procedimentos realizados mais frequentemente foram: a venóclise em 100%, a sondagem vesical em 96%, a entubação orotraqueal em 92%, o uso do respirador mecânico em 89%, a traqueostomia em 83% e a sondagem nasogástrica em 75% dos pacientes. As complicações que envolveram o aparelho respiratório foram as mais frequentes: na fase precoce, a obstrução do tubo orotraqueal ocorreu em 78,69% e a infecção pulmonar em 67,27%; na fase tardia, a estenose subglótica foi diagnosticada em 56,5% dos casos sobreviventes. Complicações, em geral, relacionadas com estes procedimentos, ocorreram em 72,73% dos pacientes internados nas enfermarias convencionais e em 27,27% dos internados na terapia intensiva (x2 = 11, p < 0,0008). A taxa de óbito desses pacientes nas enfermarias convencionais foi de 62,50%, enquanto que, na terapia intensiva, de 16,57% (x2 = 11, P < 0,0009). Sabemos que existem vários outros fatores que influenciam a evolução, mas a forte correlação entre os índices de complicações e óbitos, tanto nas enfermarias como na terapia intensiva, sugerem que a taxa de complicações também influenciou o resultado do tratamento do traumatizado craniencefálico grave.

A case of chondrosarcoma of the orbit is reported. The patient complained of marked deterioration of her vision, pain and presented exophatalmus of the left eye. Chondrosarcoma of the orbit are rare and this is the first case reported in the Brazilian literature. Diagnostic and prognostic are discussed.