Enfermedades raquimedulares congénitas en Teletón Santiago: factores demográficos, sociales, clínicos y funcionales asociados / Congenital spine pathologies in Teleton Santiago: associated demographic factors, social, clinical and functional

Introduction: In our country there is no information on factors related to independence and participation in children with congenital spine pathology. Objective: To describe the demographic, clinical, social and functional characteristics of patients aged 2 to 18 years with neural tube defects, explore the association between age and neurological level and compare with international literature. Materials and Methods: This is a retrospective descriptive study were we review the clinical protocolized evaluation forms from the Integral Spine Polyclinic of Teletón Santiago Institute, from children seen between March 2008 and March 2009. 255 individuals with chronic spine pathology met the inclusion criteria. The data was processed using SPSS version 17.0. Association tests were applied between variables based on a x2 distribution with p < 0.05. Results: Of the 255 patients studied, 92 percent of the patients had myelomeningocele, 50 percent of them with a thoracic level; 58 percent were women; 49 percent were in the group of 8-13 years; 82.4 percent attended school. Thirty three percent of them were obese; 89 percent used intermittent bladder catheterization and 77 percent achieve regular bowel movements; 60-80 percent were independent in feeding and minor hygiene, but less than 40 percent were independent in dressing, bathing and transfers; 62 percent used a wheelchair, 50 percent did not leave their home alone and 30.6 percent did not have any participation in household tasks. Conclusion: Demographic profile similar to patients with the same disease and age in other countries, high neurological level prevalence, obesity tendency in the group from 2-7 years, poor independence in more complex daily life activities and social participation.

Introducción: En el país se carece de información sobre factores relacionados con independencia, participación en el hogar y comunidad, en niños con patología raquimedular congénita. Objetivo: Describir las características demográficas, clínicas funcionales y sociales de pacientes entre 2 y 18 años con mielodisplasia y explorar asociación con edad y nivel neurológico. Materiales y Método: Estudio descriptivo transversal, mediante revisión de formularios de evaluación clínica protocolizados del Policlínico Integral Raquimedular de Instituto Teletón Chile, de niños atendidos entre marzo 2008 y marzo 2009. Doscientos cincuenta y cinco individuos con patología raquimedular cumplieron con requisitos de inclusión. Los datos se procesaron con SPSS versión 17.0. Se aplicaron pruebas de asociación entre variables basado en distribución X2 con p < 0,05. Resultados: De los 255 pacientes estudiados, 92 por ciento eran portadores de mielomeningocele siendo el 50 por ciento clasificados en nivel torácico; 58 por ciento fueron mujeres; 49 por ciento en el grupo de 8-13 años, el 82,4 por ciento asistía al colegio. El 33 por ciento era obeso; 89 por ciento usaba cateterización intermitente y 77 por ciento tenía ritmo intestinal normal; 60-80 por ciento eran independientes en alimentación e higiene menor, pero menos del 40 por ciento eran independientes en vestuario, baño y transferencias, el 62 por ciento usaba silla de ruedas, el 50 por ciento no salía sólo de su casa y el 30,6 por ciento no tenía tareas en el hogar. Conclusión: Perfil demográfico similar a pacientes con igual patología y edad en otros países; nivel neurológico alto, mayor a lo habitual; la independencia en actividades diarias y participación en el hogar y comunidad, fueron bajas en esta población.

Funcionalidad en movilidad y transferencias según edad y nivel de lesión en niños con mielomeningocele / Functionality in mobility and transference according to the neurological level of injury and age in children with myelomeningocele

Introducción: Los niños con mielomenigocele presentan limitaciones en su movilidad lo que incide en su independencia e integración social. Objetivo: evaluar la independencia lograda en el área de movilidad y transferencias según nivel de lesión neurológica y edad, Materiales y método: estudio retrospectivo de revisión de 577 fichas clínicas, de pacientes de 3 a18 años, con evaluación WeeFIM entre los años 2000-2004; 296 cumplieron requisitos de inclusión. Se registraron variables biodemográficas, puntaje WeeFIM, nivel de lesión neurológica que se clasificó: alto (dorsales, Ll, L2), medio (L3, L4) Y bajo (L5, sacro); edad dicotomizada en menores de 7 y ≥ 7 años, usando criterio de la edad mediana. Se usó SPSS para pruebas de normalidad, asociación y ANOVA con p

Introduction: Children with Myelomeningocele show mobility limitations that affect their independence and social integration. Objective: To assess the independence reached in the mobility and transfer area according to the neurological level of injury and age in children with myelomeningocele. Material and method: Retrospective study, where 577 clinical records of patients between 3 and 18 years old were reviewed, with WeeFIM evaluation registered between 2000 and 2004 years; 296 met the inclusion criteria. The data registered were: biodemographic variables, WeeFIM score, neurologicallevel of injury: high (thoracic, L1 and L2), middle (L3 and L4) and low (L5 and sacrum), and age dichotomized at younger ®7 years old) and older (≥ 7 years old), using median age criteria. The SPSS was used for normality, association, and ANOVA tests, with p< 0.05. Results: Functional independence was related significantly with neurological level of injury and age. Younger children showed complete dependence for upstairs, transference toilet, and hygiene. Older children with neurological middle or low level of injury showed a higher independence; on the other hand, children with high neurological level of injury continue being dependent. The mobility item was related significantly with neurological level of injury in younger children. Conclusions: A significant association between functionality, neurological level of injury, and age was found. Patients with myelomeningocele didn't have reached the functional independence expected; in a subgroup of patients of 12 years old and older a decreased functionality was found when compared to studies of reference.

Acompanhamento ambulatorial de pacientes com mielomeningocele em um hospital pediátrico / Ambulatory follow-up of patients with myelomeningocele in a pediatric hospital

A mielomeningocele constitui a mais freqüente malformaçäo congênita do sistema nervosos e, a despeito de sua complexidade e acometimento de múltiplos órgäos, é compatível a sobrevida prolongada. Por esses motivos, o acompanhamento de pacientes com essa malformaçäo assume vital importância no que dis respeito à qualidade de sua sobrevida. Com o objetivo de avaliar a qualidade do acompanhamento de portadores de spina bifida cística tratados em um hospital pediátrico, 54 pacientes foram examinados e seus familiares entrevistados. A inexistência de um centro multidisciplinar de tratamento de portadores de defeito de fechamento do tubo neural na cidade do Rio de Janeiro obrigou os pacientes a se deslocarem para locais geograficamente distantes enter si em busca de tratamento complementar em várias especialidades correlatas, com reflexos negativos na qualidade de sobrevida. Desta maneira, apenas uma quarta parte dos pacientes era capaz de se locomover e a chamada deambulaçäo comunitária era praticamente nenhuma. Apenas 2/3 se submetiam regularmente a fisioterapia e apenas a metade era regularmente acompanhada por ortopedistas. Quase 50 por cento dos pacientes näo foram orientados a procurar assistência urológica e 75 por cento apresentavam incontinência urinária. A incidência de infecçöes urinárias de repetiçäo foi 72,2 por cento. As complicaçöes e intercorrências neurocirúrgicas propriamente ditas tiveram incidências comparáveis às observadas na literatura. Concluímos que a qualidade de sobrevida dos pacientes estudados é significativamente afetada por fatores sócio-econômicos e pela ausência de centros multidisciplinares de tratamento