Eyelid liquoric fistula secondary to orbital meningocele

Liquoric fistula (LF) is defined as the communication of the subarachnoid space with the external environment, which main complication is the development of infection in the central nervous system. We reported the case of a patient with non-traumatic eyelid liquoric fistula secondary to orbital meningocele (congenital lesion), which main clinical manifestation was unilateral eyelid edema. Her symptoms and clinical signs appeared in adulthood, which is uncommon. The patient received surgical treatment, with complete resolution of the eyelid swelling. In conclusion, eyelid cerebrospinal fluid (CSF) fistula is a rare condition but with great potential deleterious to the patient. It should be considered in the differential diagnosis of unilateral eyelid edema, and surgical treatment is almost always mandatory.

Fístula liquórica (FL) é definida como a comunicação do espaço subaracnóide com o ambiente externo, cuja principal complicação é o desenvolvimento da infecção no sistema nervoso central. Relatamos o caso de um paciente com fístula liquórica palpebral não traumática secundária à meningocele orbitária (lesão congênita) sendo que a principal manifestação clínica foi o edema palpebral unilateral. Os sintomas e sinais clínicos da paciente apareceram apenas na idade adulta, o que é incomum. A paciente recebeu tratamento cirúrgico, com resolução completa do edema palpebral. Concluimos que fístula liquórica palpebral é uma condição rara, mas com grande potencial deletério para o paciente. Deve ser sempre considerarada no diagnóstico diferencial do edema da pálpebra unilateral, e o tratamento cirúrgico é quase sempre obrigatório.

Intradural lumbar disc herniation: report of five cases with literature review.

INTRODUCTION: Intradural lumbar disc herniations are uncommon presentations of a relatively frequent pathology, representing less than 1% of all lumbar disc hernias. They show specific features concerning their clinical diagnosis, with a higher incidence of cauda equina syndrome, and their surgical treatment requires a transdural approach. METHODS: In this article, we describe five cases of this pathology and review the literature as well as some considerations about the difficulties in the preoperative diagnostic issues and the surgical technique. CONCLUSION: We concluded that for intradural disc herniations the diagnosis is mainly intraoperative, and the surgical technique has some special aspects.

Results of microsurgical treatment of medulla oblongata and spinal cord hemangioblastomas: a comparison of two distinct clinical patient groups.

PURPOSE: To analyze the surgical outcome of a consecutive and single center series of medulla oblongata (MO) and spinal cord hemangioblastomas (HB). PATIENT AND METHODS: We retrospectively reviewed the medical charts of all MO and spinal HB patients operated on in our institution between 1985 and 2002. All patients had pre- and at least one post-operative MRI. McCormick classification was used to assess neurological status and functional outcome. RESULTS: Forty surgical procedures have been performed on 34 patients (19 females and 15 males, mean age of 41 years). Twenty-five (73%) patients had Von Hippel Lindau (VHL) disease confirmed by genetic screening, and nine patients had sporadic disease. Complete clinical, radiological, and genetic studies were done in all cases. The most frequent clinical symptom was pain (28 patient, 85%) followed by motor (42%) and sensitive deficits (42%). Fourteen lesions (19%) were located in the MO, 28 (38%) in the cervical spine, 25 (34%) in the thoracic spine, 4 (5%) in the lumbar spine and 3 (4%) in the Cauda Equina. In the VHL group, 15 patients (60%) presented multiple lesions and 10 a single neurological lesion (40%). A cyst was present in 23% of VHL patients and in 55% of the non-VHL group. A complete removal was achieved in 85% of all cases. No deaths related to surgery occurred. At the end of the follow-up period (mean 60 months) 50% of patients were stabilized, while the condition of 32.35% was improved and of 17.65% worsened. Comparing the clinical evolution considering the presence or not of VHL we have seen that there are no differences in terms of functional outcome between VHL and non-VHL groups. CONCLUSION: Our results confirmed that surgery remains a safe and effective treatment for medulla oblongata and spinal hemangioblastoma. Only symptomatic lesion required surgical treatment. In other cases, especially in VHL patients, a close and regular follow-up (clinical and MRI) is necessary.

[Crouzon syndrome: factors related to the neuropsychological development and to the quality of life]. / Síndrome de crouzon: fatores envolvidos no desenvolvimento neuropsicologico e na qualidade de vida.

Crouzon syndrome is characterized by cranial and facial abnormalities and exophtalmos. Mental retardation is sometimes observed. The objective of this study was to correlate brain malformations, timing for surgery and also social classification of families and parents education to the neuropsychological evaluation and to the quality of life of these families. Eleven patients with Crouzon syndrome were studied, whose ages were between 16 and 132 months. The multidisciplinary evaluation included : social evaluation, cognitive evaluation, brain studies by magnetic resonance imaging and quality of life evaluation. The intelligence quotient values observed were between 46 and 102 (m=84.2) and was correlated (inverted correlation) to the factor IV of the short-form of the Questionnaire on Resources and Stress. Mental development was not correlated to brain malformation, neither to the age at time of operation or to the level of family environment and parents education.

Síndrome de crouzon: fatores envolvidos no desenvolvimento neuropsicologico e na qualidade de vida / Crouzon syndrome: factors related to the neuropsychological development and to the quality of life

A síndrome de Crouzon é caracterizada por deformidade craniana, alterações faciais e exoftalmia. O retardo no desenvolvimento neuropsicomotor é observado em alguns casos. Este estudo tem como objetivo analisar a influência do momento da cirurgia, da classe sócio-econômica associada ao nível educacional dos pais e da ocorrência de malformações do sistema nervoso central no desenvolvimento cognitivo destes pacientes correlacionando estes achados à qualidade de vida deles e de suas famílias. Foram estudados 11 pacientes com diagnóstico de síndrome de Crouzon com idade entre um ano e quatro meses e treze anos. A avaliação multidisciplinar dos pacientes incluiu, avaliação social, avaliação cognitiva, estudo do encéfalo por ressonância magnética e avaliação da qualidade de vida. O quociente de inteligência variou de 46 a 102 (m=84,2) e foi correlacionado de forma inversa com o Fator 4 do Questionário de Recursos e Estresse Simplificado (incapacidade da criança); não se correlacionou com as alterações encefálicas, com a condição sócio-econômica dos pais e nem com o momento do tratamento neurocirúrgico.

Crouzon syndrome is characterized by cranial and facial abnormalities and exophtalmos. Mental retardation is sometimes observed. The objective of this study was to correlate brain malformations, timing for surgery and also social classification of families and parents education to the neuropsychological evaluation and to the quality of life of these families. Eleven patients with Crouzon syndrome were studied, whose ages were between 16 and 132 months. The multidisciplinary evaluation included : social evaluation, cognitive evaluation, brain studies by magnetic ressonance imaging and quality of life evaluation. The intelligence quotient values observed were between 46 and 102 (m=84.2) and was correlated (inverted correlation) to the factor IV of the short-form of the Questionnaire on Resources and Stress. Mental development was not correlated to brain malformation, neither to the age at time of operation or to the level of family environment and parents education.

Apert syndrome: factors involved in the cognitive development

A síndrome de Apert é caracterizada por cranioestenose, sindactilia simétrica e outras malformações sistêmicas. O retardo no desenvolvimento neuropsicomotor é freqüentemente observado. Este trabalho tem como objetivo analisar as malformações do sistema nervoso central, o momento da cirurgia e a classe sócio-econômica associada ao nível educacional dos pais como variáveis que possam influenciar no desenvolvimento cognitivo. Foram estudados 18 pacientes com diagnóstico de síndrome de Apert com idade entre 14 e 322 meses e as alterações encefálicas foram observadas em 55,6%. O quociente de inteligência variou de 45 a 108 e estava correlacionado com a classe sócio-econômica e com o nível de instrução dos pais; não se correlacionou com as alterações encefálicas nem com o momento do tratamento neurocirúrgico. Em conclusão, a condição sócio-econômica e o nível de instrução dos pais foram relevantes na determinação do desenvolvimento cognitivo destes pacientes.

Apert syndrome: factors involved in the cognitive development.

Apert syndrome is characterized by craniosynostosis, symmetric syndactyly and other systemic malformations, with mental retardation usually present. The objective of this study was to correlate brain malformations and timing for surgery with neuropsychological evaluation. We also tried to determine other relevant aspects involved in cognitive development of these patients such as social classification of families and parents education. Eighteen patients with Apert syndrome were studied, whose ages were between 14 and 322 months. Brain abnormalities were observed in 55.6% of them. The intelligence quotient or developmental quotient values observed were between 45 and 108. Mental development was related to the quality of family environment and parents education. Mental development was not correlated to brain malformation or age at time of operation. In conclusion, quality of family environment was the most significant factor directly involved in mental development of patients with Apert syndrome.

[Prototyping as an alternative to cranioplasty using methylmethacrylate: technical note]. / Prototipagem como forma alternativa para realização de cranioplastia com metilmetacrilato: nota técnica.

The prototyping is a method for reconstruction of human body segments by computer software. It has been used in neurosurgery for cranial reproduction in patients allowing the programming of surgical procedures and the production of prosthesis to reconstruct bone failures in the skull. We present two cases of cranioplasty performed with the use of acrylic prosthesis constructed by prototyping. After 10 months of follow-up, they do not present signs of infection and show good aesthetic result. The advantages pointed at the literature for this method (reduction of surgical time, easy technical handle, and good aesthetic result) were confirmed.

Prototipagem como forma alternativa para realização de cranioplastia com metilmetacrilato: nota técnica / Prototyping as an alternative to cranioplasty using methylmethacrylate: technical note

A prototipagem, método de reconstrução de segmentos do corpo humano através de programas de computação, tem sido usada na neurocirurgia para reproduzir o crânio de pacientes permitindo a programação de atos cirúrgicos e a produção de próteses para reconstruir falhas ósseas no crânio. Apresentamos dois casos de cranioplastia realizadas com o uso de próteses de acrílico construídas por prototipagem. Após 10 meses de acompanhamento, os pacientes não apresentaram sinais de infecção e apresentam bom resultado estético. As vantagens apontadas na literatura para este método (redução do tempo cirúrgico, facilidade técnica e bom resultado estético) foram observadas.

Hérnia diafragma congênita bilateral associada a hipoplasia pulmonar severa e óbito neonatal / Bilateral congenital diaphragmatic hernia associated with severe pulmonary hypoplasia and neonatal death

Os autores apresentam um caso de recém-nascido a termo de gestaçao normal com quadro de insuficiência respiratória aguda rapidamente progressiva e óbito neonatal. Autópsia revelou hérnia diafragmática bilateral associada a hipoplasia pulmonar. O presente trabalho procura destacar a presença incomum da bilateralidade dessa anomalia diafragmática e a importância do diagnóstico precoce na conduçao imediata do caso.