Functional independence of oneto four-year-old children with myelomeningocele

Corresponding address: Mariana Callil Voos – Universidade Ibirapuera – Interlagos Avenue, 1329 – São Paulo (SP), Brazil – Zip Code: 04661100 – E-mail: ftmarivoos@gmail.com – Financing source: Nothing to declare – Conflict of interests: Nothing to declare – Presentation: Sept. 16th, 2017 – Accepted for publication: Mar. 4th, 2018 – Approved by the Ethics Committee of Universidade Santa Cecília under protocol no 13555913.7.0000.5513. Functional independence of oneto four-year-old children with myelomeningocele Independência funcional de crianças de um a quatro anos com mielomeningocele Independencia funcional de niños de un a cuatro años con mielomeningocele Fabiane Ramos Ferreira1, Fernanda Pinheiro Bexiga2, Vivian Vargas de Moraes Martins3, Francis Meire Favero4, Cristina Dallemole Sartor4, Mariana Cunha Artilheiro4, Mariana Callil Voos4


INTRODUCTION
Myelomeningocele is a form of spinal dysraphism, caused by a defect in the neural tube closure during the fourth week of gestation.In Brazil, the incidence is 2.28 for each 1000 childbirths 1,2 .It results in tetraparesis or paraparesis, neurogenic bladder, and cognitive changes.Many children have difficulty in performing daily life activities.The decreased mobility may affect the cardiovascular system and generate difficulties in social relationships [3][4][5][6] .The injury occurs during the embryonic development of the nervous system.The neural tube, which later becomes the central nervous system, remains open in the cranial and caudal ends around the 24th day of gestation 1 .
Myelomeningocele is the second most common cause of chronic disability in the locomotor system in children.Recent studies indicate the influence of genetic, environmental, and nutritional factors, such as folic acid deficiency at the beginning of gestation 1,5 .Patients can also present other complications, like Arnold-Chiari II malformation and hydrocephalus.The Arnold-Chiari II malformation involves cerebellum, medulla oblongata, and the cervical part of the spinal cord.Herniation of the posterior lobe of cerebellum by the foramen magnum results in caudal displacements of brainstem structures 1 .The fourth ventricle is obstructed by these abnormally located structures of the foramen magnum, and the flow of cerebrospinal fluid is stopped.Hydrocephalus is characterized by the abnormal accumulation of cerebrospinal fluid due to excessive production, circulatory obstruction, or failed absorption 1,6 .
Incapacity severity and degree depends in the location where the spinal cord injury occurred, as well as on other neurological factors, mainly the hydrocephalus.It may occur in any region of the spinal cord, but most injuries (about 75%) are located in the lumbar area, which results in difficulty in standing up, prowling, and bladder and bowel controlling 5,7,8 .The diagnosis can be done still in intrauterine stage, which increase the chances of treatment.In such cases, the option is made for a caesarean section, to reduce the risk of myelomeningocele rupture and infection.After birth, a simple radiograph, computerized tomography, and magnetic resonance imaging are complementary examinations that identify anatomic malformations 1,6 .
The motor and cognitive framework influences the sensory-motor development and the functional independence (self-care, mobility, and social function).Trunk control is essential for these acquisitions and allows better development of bimanual skills 3,9-11 .
Sirzai et al. (2014) investigated the functional performance in children with different levels of injury, using the Pediatric Evaluation Disability Inventory (PEDI) 12 .They considered the functional performance low in most children and concluded that lower-level injuries partially preserved muscle strength and mobility 12 .
The aim of this study was to describe the functional independence in children with myelomeningocele from one to four years of age.Our hypothesis is that those with higher-level lesions would present less functional independence.

METHODOLOGY
This study was conducted in accordance with the Resolution 196/96 of the National Health Council and approved by the Ethics Committee in Research with Human Beings of the Santa Cecília University -Santos/ SP, under the protocol No. 13555913.7.0000.5513.
Criteria for inclusion were: children between one and four years of age, with complete spinal cord injury according to the International Standards for Neurological Classification of Spinal Cord Injury of the American Spinal Injury Association, and an informed consent form signed by one of the responsible.The exclusion criterion was having presented clinical complications in the last six months (bladder infection, changes in the functioning of the ventriculoperitoneal shunt, etc.).
The evaluation was performed through PEDI, with questions directed to the patients' parents, to assess functional independence.Parents were informed about the research procedures and signed the informed consent form prior to participation.The PEDI measures the functional skills and the need for assistance during daily living activities.It has three areas: self-care, mobility, and social function 3,12,13 , totaling 197 items with scores from 0 to 1 (0: Incapable of limited in performing the item in most situations; 1: Able to perform the item in most situations, or the item has been already mastered and the functional skills have progressed beyond this level).
The International Standards for Neurological Classification of Spinal Cord Injury scale of the American Spinal Injury Association was used to evaluate the motor and sensory level of injury and to classify it (complete or incomplete).The sensory examination consisted of testing 28 dermatomes in the right and left sides of the body, for soreness (tested with a disposable needle) and light touch (tested with a brush).Motor examination tested ten muscles, bilaterally.The strength of each muscle was scored in an ordinal scale from zero (total paralysis) to five (normal active movement, full range of motion against total resistance).Scores of myotomes on both sides of the body are combined to generates a single total motor score (TM).The rectal examination involved sensation evaluation and contraction of the anal external sphincter A professional trained in this evaluation and classification method performed these examinations 14 .
Injury classification is based on the motor and sensory scores.It determines whether the injury is complete or incomplete.Classification A means a complete loss of sensory and motor function below the level of injury.B means that the sensitivity is preserved below the level of the injury but the motor function is lost.C denotes that the motor function is preserved below the level of injury, with more than half of the main muscles reaching less than 3 in the motor score.D means that the motor function below the level of injury is preserved, with more than half of the main muscles scoring at least 3 or more in the motor score.E is attributed when both motor and sensory functions are preserved.The complete spinal injury occurs when there is complete loss of motor and sensory function below the level of injury.A complete injury affects both sides of the body equally.In incomplete injuries, some functions and sensations remain below the level of injury. 14

RESULTS
15 children were evaluated, six boys and nine girls, aged from 1 to 4 years and diagnosed with myelomeningocele.All of them were subjected to surgery to fix the injury within 48 hours after birth.According the evaluation by the International Standards for Neurological Classification of Spinal Cord Injury scale, of the American Spinal Injury Association, three children presented a thoracic level, nine showed high lumber injury, two had low lumber injury, and one featured a sacral level.They all featured complete spinal injury.
The PEDI scores in the areas self-care, mobility, and social function varied according to age and level of injury of the participants.Overall, the smaller the age and the higher the injury, the greater the functional dependence detected.However, there was great variability and some exceptions to this general rule (Table 1).PEDI scores ranged from 15 to 60% in the area self-care, from 10 to 15% in mobility, and from 19% to 58% in the social function.PEDI scores ranged from 15 to 60% in the area self-care, from 10 to 15% in mobility, and from 19% to 58% in the social function.Therefore, mobility was the most affected aspect.There are few studies on the functional independence of children with myelomeningocele 3,12,15,16 .Overall, the higher the level of the lesion, the greater the functional impairmentwhich was the hypothesis of this study.These findings are not in accordance with this study since some children with thoracic injury showed higher scores than those with lumbar injury in the areas self-care and social function.
Physical therapists have a fundamental role in stimulating the functional independence of children, through the continuous monitoring with the multidisciplinary team [15][16][17][18] .It is essential to guide the families so that children acquire the greatest independence possible, considering the scores of each age group.Physical therapy also aims to prevent secondary deformities, decubitus ulcers, and secondary cognitive changes 3,[9][10][11] .
Physical therapy should promote the learning of motor skills, postural adjustments, independent locomotion (if necessary, with resources such as crutches or a wheelchair), to ensure the maximum functional independence possible 3,5,9,12 .Muscle stretching and strengthening should be continuously carried out to assure the development and maintenance of strength and mobility.Consequently, is part of the physical therapist's job to promote quality of life during all phases of development 5,19 .
According to Lundberg (2011), the higher the neurological level of injury, the worse will the quality of life of these patients be 14 .This can be explained by the fact that higher levels tend to cause greater motor sensory, and cognitive damage; hence, the impact of the disease is greater, especially on mobility and selfcare 4,5,11,20 .In this study, though patients with thoracic injury have had, indeed, low mobility scores, they varied greatly in self-care and social function.Given these findings, we emphasize the importance of encouraging early the used of wheelchairs in children with higher lesions, as well as the independence in self-care and social function 11,19 .
The study of Seitzber et al. (2008) indicates that a significant factor for the ambulance prognosis in children with myelomeningocele is age 21 .In this study, the level of injury was more influential than age on the PEDI area of mobility.It is important to note that, overall, the children had low scores on mobility.That is, they did not show great mobility acquisition over the years.This did not occur for self-care and social function, in which there was greater independence since the first year of life.
In addition, the study shows that, even among complete injuries, the outcomes may vary, especially regarding self-care and social function.It is essential to use not only compensatory strategies but also stimulate motor gains, including the control below the level of injury, even in children with complete injuries. 22unctional independence should be always optimized so there is better quality of life. 23

CONCLUSION
The level of injury in patients with myelomeningocele interferes with their mobility but has a smaller impact on self-care and social function in the age group from one to four years.It is essential to always used scores corrected by age when evaluating this group because it has a significant interference on functional acquisitions.The monitoring with the multidisciplinary team is of paramount importance, especially including physical therapists to promote the neuropsychomotor performance.

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Pediatric Evaluation of Disability Inventory, PEDI) con los padres para evaluar la independencia funcional en las actividades de vida diaria de los niños.La escala de Estándares Internacionales para la Clasificación Neurológica de la Lesión Medular de la

Table 1 .
Level of injury, classification, age, and score in the Pediatric Evaluation of Disability Inventory