-Objective: To determine if there was a relationship between intrassellar pressure (ISP) and p i t u i t a ry tumor volume. Method: Between August 2002 and May 2004, 60 patients aged between 13 and 75 years old (39 males), having a pituitary adenoma were submitted to an endoscope transseptal appro a c h . During the surg e ry and before tumor resection, 2 mm of the sella's floor were removed and a 1.5 mm dural opening made to place a transducer into the pituitary adenoma. The transducer was connected to a p re s s u re monitor. Results: The intrasellar pre s s u re, ranged from 2-51 mmHg and was measured based on the classification of Hard y -Vezina. The most elevated was in the type II macro adenomas with 32.6 mmHg, sharply superior to the value of a normal intracranial pre s s u re . Conclusion: These values showed that the m a c roadenomas confined to the sella, without destruction of the floor and integrity of the diaphragm, type II of Hardy-Vezina, presented a value of ISP much higher than intra-extrasellar macroadenoma's.KEY WORDS: pituitary adenoma, intrasellar pressure, endonasal approach. Relação entre pressão intra-selar e volume de tumor de hipófiseObjetivo: Determinar se existia uma relação entre a pressão intraselar (ISP) e o volume de tumor de hipó-f i s e . Método: E n t re agosto de 2002 e maio de 2004, 60 pacientes com idades variando entre 13 e 75 anos (39 homens), port a d o res de adenoma hipofisários foram operados por via transesfenoidal. Durante o ato c i r ú rgico e antes da resseção do tumor, uma osteotomia de 2 mm foi realizada no assoalho selar e uma a b e rtura de 1,5 mm na duramater para a introdução de um transdutor dentro do tumor. O transdutor foi conectado a um monitor de pressão e esta foi medida por 2 minutos. Resultados: A pressão intra-selar variou entre 2-51 mmHg e a correlação entre tamanho do tumor e ISP foi baseada na classificação de Hard yVezina. A média da ISP mais elevada foi encontrada nos macroadenomas tipo II com 32,6 mmHg, nitidamente superior a pressão intra selar norm a l . Conclusão: Estes valores mostram que os macro a d e n o m a s confinados a sela sem destruição do assoalho selar e com integridade do diafragma, classificados como tipo II de Hardy-Vezina, apresentam uma ISP muito superior aos outros adenomas. PALAVRAS-CHAVE: adenoma hipofisário, pressão intra-selar, via transesfenoidal.T h e re are studies in the literature corre l a t i n g intrasellar pre s s u re(ISP) and adenoma blood flow 1 , ISP and stalk compression syndrome 2 , ISP and endocrine function 3 , 4 , ISP and headaches 3 , pituitary volume and headache 5 , ISP and pituitary tumor apop l e x y 6 , but there are no specific studies corre l a t i n g ISP and tumor volume.The walls of the sella turcica are a relatively rigid s t ru c t u reand under normal circumstances, may serv e to protect the pituitary gland from trauma and surrounding pressure fluctuations. The growth of a tumor within the sella, a normally inelastic space, is likely to cause an increase of ISP. It has been conje...
Relatamos nossa experiência com 11 pacientes portadores de meningiomas do forame magno, oito cranioespinhais e três espinocraniais. A média de idade foi 50,8 anos, o sintoma mais comum foi cefaléia occipital com duração média de 18,6 meses. Os principais achados neurológicos foram tetraparesia e comprometimento dos nervos cranianos baixos. O tratamento foi cirúrgico, sempre com exposição da artéria vertebral em sua entrada na dura-máter da fossa posterior e ressecção de parte do côndilo occipital apenas em três casos. Ressecção total foi possível em sete pacientes e parcial nos demais, devido às aderências a vasos e nervos. O prognóstico esteve relacionado com as condições neurológicas pré-operatórias.
Aneurismas cerebrais são raros na faixa etária pediátrica. A apresentação destas lesões difere significativamente em relação á população adulta no que se refere a tamanho, localização e incidência. Relatamos caso de menimo de três anos de idade que apresentou quadro de hemorragia subaracnóidea sem história de infecções ou traumatismos prévios sendo, posteriormente, diagnosticado aneurisma gigante localizado na artéria cerebral média. Foi submetido a tratamento cirúrgico com boa evolução pós-operatória.
Meningeomas da goteira olfatória representam 8-18% dos meningeomas intracranianos e geralmente são diagnosticados quando alcançam grande tamanho. Relatamos uma série de 13 casos consecutivos de meningeomas de goteira olfativa operados nos Hospitais São Paulo (UNIFESP) e Professor Edmundo Vasconcelos no período de 1995 a 2003, estudados retrospectivamente quanto ao quadro clínico, resultados cirúrgicos e complicações. Os pacientes foram submetidos a ressecção cirúrgica destes tumores pela via subfrontal, em 9 casos a ressecção foi completa incluindo dura-máter e osso infiltrado por tumor e em 4 a dura-máter foi somente coagulada. Um paciente morreu devido a infarto cerebral e três pacientes evoluíram com fístulas liquóricas. Não houve recorrência sintomática no período que variou de 11 meses a 8 anos. Com as técnicas microcirúrgicas atuais, estes tumores podem ser removidos com baixa morbidade.
SUELY KAZUE NAGAHASHI MARIE** Mitochondrial disorders are a heterogeneous group of diseases characterized by the dysfunction of the oxidative phosphorilation. As the protein complexes of the respiratory chain are coded by both nuclear and mitochondrial genomes, there are additional difficulties on the clinical and diagnostic approaches to those disorders. The RRF detected on muscle biopsy, a hallmark of mitochondrial proliferation, is not always present on those conditions, neither is the increase of lactic acid level. Since the first description of pathogenic mutation of mitochondrial DNA (mtDNA), in 1988, a molecular criteria has been used for the classification of mitochondriopathies. Among 377 patients with clinical diagnosis of mitochondrial disease and patients with maternal inheritance of diabetes mellitus, and patients with maternal risk factors of dementia, followed-up between the period of 1991 to 2001, by our group, 145 met the clinical criteria for MELAS (40cases), MERRF (27 cases), Leigh Syndrome (LS) (32 cases), Kearns-Sayre Syndrome (KSS) (14 cases), and Chronic Progressive External Ophthalmoplegia (CPEO) (32 cases). Measurements of lactic acid level, muscle biopsy, and neuroimaging were performed on the majority of the cases. Molecular analysis were done on all patients looking for 8 point mutations of mtDNA in MELAS, 3 in MERRF, 6 in LS, 1 in KSS/CPEO, and mtDNA rearrangements on KSS/CPEO. A3243G point mutation of mtDNA was detected in 18 cases with MELAS, including 10 asymptomatic proband's family members. A8344G mutation was observed in 5 cases with MERRF; T8993G and T8993C in two cases with LS; 14 different mtDNA deletions in KSS and 11 deletions in CPEO, including 3 multiple deletions. Phenotypic variability was observed in all 5 groups, with overlap of MELAS, MERRF and LS in one patients, and MELAS and LS in another. An autopsy study of MELAS case showed an universal distribution of A3243G mutation on all tissues studied. The neuropathological findings showed a diffuse degeneration of cerebral cortex with neovascularization, a similar picture observed on LS. Surprisingly the neurons were apparently well preserved even when surrounded by degeneration. Atrophy and deep grey matter lesion were the most frequent neuroimaging findings, particularly in KSS and LS, when compared to MELAS and MERRF. The characteristic distribution of lesion on the tegmen of brain stem was observed in KSS and LS, which is similar to the presentation of Wilson's disease. Interestingly, those 3 diseases share a common alteration: mutation in gene coing for the complex V of the respiratory chain. Although the central nervous system is diffusely lesioned on mitochondriopathies, as show the most sensitive diagnostic methods as functional imaging, and immunohistochemical methods, the order, the degree, and the velocity of those alterations seen to present some differences between the groups, as observed in our cases. A careful analysis concerning those parameters could reveal new details for better understanding of the pat...
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