Helder Zambelli

Desenvolvimento cerebral em recém-nascidos prematuros

OBJECTIVE:To review the current literature about brain growth and development of premature children, as well as the motor and cognitive changes that may result from prematurity. DATA SOURCES: Medline and Lilacs were searched between 2000 and 2007 along with textbooks whose content was relevant. DATA SYNTHESIS: The development of preterm infants differs from term neonates. Studies have shown that children born prematurely have anatomical changes related to cognitive impairments in the central nervous system. Some regions seem vulnerable, such as white and gray matter, corpus callosum, caudate nucleus, hippocampus and cerebellum, when evaluated by volumetric neuroimaging techniques. Thus, one would expect some functional and/or learning impairment in children, adolescents and adults born prematurely. When evaluated in late childhood and adolescence, they show deficits in the intelligence quotient, memory, calculations skills and in the overall cognitive function. Motor coordination, attention, planning and association deficits are also reported in the literature. CONCLUSIONS: Prematurity can lead to structural and anatomical changes of the brain due to interruption of the prenatal development. These changes can cause functional deficits and children born prematurely are more vulnerable to cognitive and motor problems as well as its effects on their daily activities, even in adolescence and adulthood.Objective: To review the current literature about brain growth and development of premature children, as well as the motor and cognitive changes that may result from prematurity. Data sources: Medline and Lilacs were searched between 2000 and 2007 along with textbooks whose content was relevant. Data synthesis: The development of preterm infants differs from term neonates. Studies have shown that children born prematurely have anatomical changes related to cognitive impairments in the central nervous system. Some regions seem vulnerable, such as white and gray matter, corpus callosum, caudate nucleus, hippocampus and cerebellum, when evaluated by volumetric neuroimaging techniques. Thus, one would expect some functional and/ or learning impairment in children, adolescents and adults born prematurely. When evaluated in late childhood and adolescence, they show deficits in the intelligence quotient, memory, calculations skills and in the overall cognitive function. Motor coordination, attention, planning and association deficits are also reported in the literature. Conclusions: Prematurity can lead to structural and anatomical changes of the brain due to interruption of the prenatal development. These changes can cause functional deficits and children born prematurely are more vulnerable to cognitive and motor problems as well as its effects on their daily activities, even in adolescence and adulthood.

Cisticercose medular: relato de dois casos, revisäo da literatura e comentários sobre a patogenia

A case of spinal intramedullary cysticercosis in a 13-year-old Brazilian female and a case of spinal leptomeningeal infestation by cysticercosis in a 51 year-old Brazilian female are presented. A review of 95 published cases of medullar cysticercosis since 1856 shows the incidence of this condition. Extramedullary forms are explained by the downward migration of larvae from the cerebral to the spinal subarachnoid space and most larvae are expected to be stopped in the upper portions of the spinal canal due to peculiarities of the anatomy of the spinal cord.A case of spinal intramedular cysticercosis in a 13-year-old Brazilian female and a case of spinal leptomeningeal infestation by cysticercosis in a 51 year-old Brazilian female are presented. A review of 95 published cases of medullar cysticercosis since 1856 shows the incidence of this condition. Extramedullar forms are explained by the downward migration of larvae from the cerebral to the spinal subarachnoid space and most larvae are expected to be stopped in the upper portions of the spinal canal due to peculiarities of the anatomy of the spinal cord.

Congenital hydrocephalus: gestational and neonatal outcomes

PurposeTo evaluate gestational and neonatal outcomes in pregnancies complicated by fetal hydrocephalus.MethodsRetrospective analysis of 287 cases of fetal hydrocephalus followed at the Fetal Medicine Unit of the University of Campinas in the period of 1996 to 2006.ResultsMean maternal age was 25 years, mean gestational age at diagnosis was 27 weeks. There were 50 cases of isolated ventriculomegaly, 95 cases of Chiari II malformation and 142 cases of ventriculomegaly associated with other malformations. Preterm delivery and vaginal delivery were more frequent in the group of ventriculomegaly associated with other malformations. Cardiac, skeletal and renal malformations were the most common associated malformations. Cesarean section was common (95%) in the Chiari II group. Fetal and neonatal death occurred more frequently (29 and 68%, respectively) in the group of ventriculomegaly associated with other malformations. Chromosomal anomalies were present in 15% of 165 investigated cases.ConclusionsFetal and neonatal prognosis and outcome are associated with the presence of associated anomalies and aneuploidy.

[Anesthesia for intrauterine myelomeningocele correction: case report].

JUSTIFICATIVA E OBJETIVOS: A cirurgia fetal constitui tratamento de malformacoes no periodo pre-natal, que nao sao adequadamente corrigidas apos o nascimento e tem como objetivo tratar ou evitar a progressao das anomalias. O objetivo deste relato e apresentar um caso de anestesia para correcao intra-utero de mielomeningocele. RELATO DO CASO: Paciente com 19 anos, 23 semanas de idade gestacional, sem antecedentes anestesicos, estado fisico ASA I, submetida a cirurgia fetal intra-uterina, sob anestesia geral associada a peridural continua. No pre-operatorio utilizaram-se indometacina (50 mg por via retal), metoclopramida (10 mg por via venosa), cimetidina (50 mg por via venosa), e como medicacao pre-anestesica midazolam (2 mg por via venosa). No espaco peridural injetou-se bupivacaina a 0,25% com adrenalina (25 mg) associada a fentanil (100 µg), seguida de passagem de cateter cefalico, para analgesia pos-operatoria. O utero foi mantido deslocado para esquerda com auxilio da cunha de Crawford. Inducao anestesica em sequencia rapida, com fentanil, propofol e rocuronio e manutencao com isoflurano em concentracao de 2,5% a 3% veiculado em O2 e N2O (50%). Apos histerotomia, realizada com staplin (grampeadores) para promover hemostasia, a regiao fetal a ser operada foi exposta e a analgesia e imobilidade fetal, foram obtidas com a associacao fentanil (10 µg.kg-1) e pancuronio (0,1 mg.kg-1) administrada na regiao glutea fetal. A pressao arterial sistolica materna foi mantida acima de 100 mmHg, com efedrina em bolus (5 mg), coloides e cristaloides. O liquido amniotico perdido foi substituido por solucao fisiologica aquecida. Apos correcao do defeito fetal, procedeu-se ao fechamento uterino e da membrana amniotica em dois planos, com fio de vicryl e cola de fibrina. Seguiu-se a diminuicao gradativa da concentracao do isoflurano, e para a manutencao do relaxamento uterino utilizou-se sulfato de magnesio (4 g/20minutos), seguido de infusao continua (2 g/hora). Ao final da cirurgia injetou-se morfina (2 mg) pelo cateter peridural para analgesia pos-operatoria. CONCLUSOES: A anestesia para cirurgia fetal envolve dois seres, mae e feto, e o manuseio anestesico exige seguranca materno-fetal, anestesia e imobilidade fetal, relaxamento uterino, prevencao do trabalho de parto prematuro e analgesia pos-operatoria.

Evolução de 58 fetos com meningomielocele e o potencial de reparo intra-útero

RESUMO - Introducao:O diagnostico pre natal da meningomielocele (MM) permite melhor planejamento de sua abordagem e,mais recentemente ,um possivel reparo intra-utero. Objetivo:Descricao da evolucao perinatal de fetos com MM,acompanhadosem um centro de referencia em Medicina Fetal,identificando os possiveis fetos candidatos a cirurgia intra-uterina. Metodo:Analiseretrospectiva descritiva de 58 casos de MM fetal, atendidos no CAISM-UNICAMP, de janeiro de 1997 a dezembro de 2001,identificando-se os casos cuja indicacao de cirurgia fetal seria possivel. Resultados: Media da idade gestacional ao diagnosticode 29 semanas (17-39);nivel da lesao acima da regiao sacral em 85%;associacao com hidrocefalia em 86%;taxa de complicacoescirurgicas de 39%.Na evolucao,98% apresentaram bexiga neurogenica e 60% deficiencia neuro-mental.O potencial reparo intra-utero foi de 42%. Conclusao:MM esta associada a graves e frequentes sequelas.Quase um terco dos nossos casos poderiam tertido a cirurgia fetal como opcao terapeutica.PALAVRAS-CHAVE: diagnostico pre-natal, meningomielocele fetal, hidrocefalia, cirurgia fetal.Fetal myelomeningocele and the potential in-utero repair: follow-up of 58 fetusesABSTRACT - Introduction:Prenatal diagnosis of myelomeningocele (MM) allows planning its management and,recently,a possiblein utero repair. Objective: To describe the perinatal outcome of fetuses with MM, in a Fetal Medicine Unit, identifying possiblecandidates for the in utero surgical repair. Methodo:Retrospective and descriptive study of 58 cases of prenatally diagnosed MM,at CAISM-UNICAMP, from January 1997 to December 2001, identifying possible fetal candidates for in utero repair. Results: thediagnosis mean gestacional age was 29 weeks (17-39);level of lesions was above sacral region in 85%,association with hydrocepha lyin 86%. Surgical complications were present in 39% of the neonates. During follow-up, 98% presented neurogenic bladder and60% neurological/mental handicap. Twenty eight fetuses (42%) could have indication of in utero repair.Conclusion:MM isassociated with severe and frequent poor results.Almost one third of our cases could had fetal repair as a treatment choice.KEY WORDS: prenatal diagnosis, myelomeningocele, hydrocephaly, fetal surgery.

Analysis of 52 patients with head trauma assisted at pediatric Intensive Care Unit: considerations about intracranial pressure monitoring

OBJECTIVES Analysis of 52 pediatric patients with head trauma assisted at Intensive Care Unit; to present considerations about epidemiologic factors of trauma, clinical presentation, tomografic aspects, hemodynamic changes and treatment options of intracranial hypertension; to present considerations about the intracranial pressure (ICP) monitoring. METHOD Retrospective study involving 52 patients with head trauma and 17 patients submitted to a ICP monitoring. RESULTS We found a male predominance, mean age 7.75 years-old, main cause was run over (38.5%); 21.2% patients presentd arterial hypotension; 67.3% were considered severe head trauma. According to Marshall tomografic grading we had 19.2% type I, 65.4% type II, 3.8% type III, 3.8% type IV and 7.7% type V. Seizures occurred in 25% children ICP monitoring was made in 32.7% of all patients. Mortality rate was 11.5%. In 58% the maximum ICP level occured at the second day of trauma. CONCLUSION Prognosis was related to severity of trauma, arterial hypotension, Marshalls tomografic gradind III and IV and ICP high values. The ICP monitoring was considered useful to allow the identification and treatment of intracranial hypertension.

Cerebral development in preterm newborn infants

OBJECTIVE:To review the current literature about brain growth and development of premature children, as well as the motor and cognitive changes that may result from prematurity. DATA SOURCES: Medline and Lilacs were searched between 2000 and 2007 along with textbooks whose content was relevant. DATA SYNTHESIS: The development of preterm infants differs from term neonates. Studies have shown that children born prematurely have anatomical changes related to cognitive impairments in the central nervous system. Some regions seem vulnerable, such as white and gray matter, corpus callosum, caudate nucleus, hippocampus and cerebellum, when evaluated by volumetric neuroimaging techniques. Thus, one would expect some functional and/or learning impairment in children, adolescents and adults born prematurely. When evaluated in late childhood and adolescence, they show deficits in the intelligence quotient, memory, calculations skills and in the overall cognitive function. Motor coordination, attention, planning and association deficits are also reported in the literature. CONCLUSIONS: Prematurity can lead to structural and anatomical changes of the brain due to interruption of the prenatal development. These changes can cause functional deficits and children born prematurely are more vulnerable to cognitive and motor problems as well as its effects on their daily activities, even in adolescence and adulthood.Objective: To review the current literature about brain growth and development of premature children, as well as the motor and cognitive changes that may result from prematurity. Data sources: Medline and Lilacs were searched between 2000 and 2007 along with textbooks whose content was relevant. Data synthesis: The development of preterm infants differs from term neonates. Studies have shown that children born prematurely have anatomical changes related to cognitive impairments in the central nervous system. Some regions seem vulnerable, such as white and gray matter, corpus callosum, caudate nucleus, hippocampus and cerebellum, when evaluated by volumetric neuroimaging techniques. Thus, one would expect some functional and/ or learning impairment in children, adolescents and adults born prematurely. When evaluated in late childhood and adolescence, they show deficits in the intelligence quotient, memory, calculations skills and in the overall cognitive function. Motor coordination, attention, planning and association deficits are also reported in the literature. Conclusions: Prematurity can lead to structural and anatomical changes of the brain due to interruption of the prenatal development. These changes can cause functional deficits and children born prematurely are more vulnerable to cognitive and motor problems as well as its effects on their daily activities, even in adolescence and adulthood.

MULTI-MINICORE DISEASE REVISITED

Multi-minicore disease (MmD) is an infrequent congenital myopathy, defined by structural changes in optic and electron microscopy, namely, multiple small areas lacking oxidative enzyme activity and focal disorganization of contractile proteins involving at most a few sarcomeres. The classical form of the disease manifests as more or less severe hypotonia and generalized weakness with predominance in axial and proximal limb muscles. Clinical variants also exist. Usually MmD is inherited as an autosomal recessive trait. Genetic heterogeneity is recognized and up to now mutations in the genes of RYR1 and SEPN1 have been detected. We record three unrelated cases of MmD. Case 1, with the classical benign form, was followed-up for 15 years. Case 2, presenting pharyngolaryngeal involvement and severe delay of head control, improved gradually, until independent gait was acquired at age of six years. A moderate restriction of daily life activities remains. Case 3, of antenatal-onset, was expressed by arthrogryposis of hands, predominance of scapular girdle deficit and a stable course after ten years on physiotherapy. All cases were selected by the characteristic morphological abnormalities in biceps brachii samples, including electron microscopy. Emphasis is given to case 2 due to type 1 fiber uniformity and mild endomysial fibrosis, posing a difficult differential diagnosis with congenital muscular dystrophy were it not for the significant number of multi-minicores.

Trombose da artéria carótida interna devida a trauma penetrante no pálato mole: relato de caso

Stroke following intraoral trauma is a rare complication of a common childhood injury. In the literature these complications have been well documented, however this condition is still infrequent. In order to alert the physicians about this possible injury we report our experience with one case. Computer tomography and magnetic resonance imaging evidenced complete occlusion of the internal carotid artery. Pathogenesis of this oral trauma is discussed.

Perfil de doadores efetivos do serviço de procura de órgãos e tecidos

Objective To characterize the profile of effective organ and tissue donors and to understand which organs and tissues were donated for transplantation. Methods This was a quantitative, descriptive, exploratory, retrospective study that analyzed clinical data from 305 donors between January 2006 to December 2010. The data were then analyzed using descriptive analyses, generating frequency tables, measures of position (mean, minimum and maximum) and measures of dispersion (standard deviation) for data that was social and clinical in nature. Results There was an overall predominance of white (72%) and male (55%) individuals between the ages of 41 and 60 years (44%). The primary cause of brain death was cerebrovascular accident (55%). In the patient history, 31% of the patients were classified as overweight, 27% as hypertensive and only 4.3% as having diabetes mellitus. Vasoactive drugs were used in 92.7% of the donors, and the main drug of choice was noradrenaline (81.6%). Hyperglycemia and hypernatremia were diagnosed in 78% and 71% of the donors, respectively. Conclusion Significant hemodynamic changes were found, and the results indicate that the use of vasoactive drugs was the main strategy used to control these changes. Furthermore, most donors presented with hyperglycemia and hypernatremia, which were frequently reported in association with brain death. The persistent nature of these findings suggests that the organ donors were inadequately maintained.Objective: To characterize the profile of effective organ and tissue donors and to understand which organs and tissues were donated for transplantation. Methods: This was a quantitative, descriptive, exploratory, retrospective study that analyzed clinical data from 305 donors between January 2006 to December 2010. The data were then analyzed using descriptive analyses, generating frequency tables, measures of position (mean, minimum and maximum) and measures of dispersion (standard deviation) for data that was social and clinical in nature. Results: There was an overall predominance of white (72%) and male (55%) individuals between the ages of 41 and 60 years (44%). The primary cause of brain death was cerebrovascular accident (55%). In the patient history, 31% of the patients were classified as overweight, 27% as hypertensive and only 4.3% as having diabetes mellitus. Vasoactive drugs were used in 92.7% of the donors, and the main drug of choice was noradrenaline (81.6%). Hyperglycemia and hypernatremia were diagnosed in 78% and 71% of the donors, respectively. Conclusion: Significant hemodynamic changes were found, and the results indicate that the use of vasoactive drugs was the main strategy used to control these changes. Furthermore, most donors presented with hyperglycemia and hypernatremia, which were frequently reported in association with brain death. The persistent nature of these findings suggests that the organ donors were inadequately maintained.