Child: care, health and development, 1991,17, 367-380
Occupational therapy intervention guidelines for children and adolescents with spina bifida DIANE WATSON Formerly ofGlenrose Rehabilitation Hospital, Pediatric Rehabilitation Unit, Edmonton, Alberta, Canada
Accepted for publication 10 July 1991
Summary Children and adolescents with spina bifida present with a number of characteristics that affect functional performance and developmental skills. The focus of this paper will be to share information about spina bifida and the overall approach to care within the Glenrose Rehabilitation Hospital Spina Bifida Clinic and, more specifically, the role of the occupational therapist. INTRODUCTION With recent advances in medical and surgical techniques, the life span and quality of life of people with spina bifida has expanded. The Glenrose Rehabilitation Hospital in Edmonton, Alberta, Canada, provides a co-ordinated and comprehensive assessment and treatment clinic that serves the children of northern Alberta and the North-west Territories who have congenital or acquired spinal-cord anomalies. SPINA BIFIDA Spina bifida is a congenital malformation of the vertebral arches resulting in failure of closure with or without dysplasia of the spinal cord. Wolraich (1983) reports that the incidence of spina bifida in the United States is 0-7 to 1-0 cases per 1000 live births. The prevalence of neural tube defects varies over time and by geographic location. Although specific causes are not yet known, both genetic and environmental factors play an interactive role (Williamson 1987). Correspondence: Diane Watson, Box 25 Site 471 R.R. 4, Sherwood Park, Alberta T8A 3K4, Canada.
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Spina bifida is classified into types according to the degree of involvement and resultant deficit. Spina bifida occulta is an opening in the vertebrae without displacement of tissues. Neurological deficits are uncommon. Spina bifida cystica describes a sac-like protrusion through the defective vertebral arches and includes two types: meningocele and meningomyelocele. Surgical closure of the protrusion is necessary and often occurs shortly after birth. Meningocele is a condition where meninges and cerebral spinal fluid protrude through the vertebral opening. As the spinal cord remains intact, neurological deficit is uncommon. Meningomyelocele occurs in 94% of spina bifida cystica cases and is the most severe form of spina bifida (Menelaus 1980). The protrusion includes meninges and neural tissue.
CLINICAL FEATURES The most obvious clinical features of spinal neurological damage are motor impairment, orthopaedic deformities and loss of sensation and genito-urinary control (Atkins & Chapman 1975). The extent of neurological damage and resultant functional problems is dependent on the level and type of lesion. Motor impairment of the vertebral opening The site usually indicates the level of neurological damage but typically is not the only source of insult. Meningomyelocele is most common in the lumbosacral region and can result in scattered and asymmetrical innervation. Orthopaedic deformities Scattered congenital neurological impairment, accompanied by limited movement in utero, can result in orthopaedic deformities present at birth. After birth, children are susceptible to additional lower extremity joint deformities and contractures. Scoliosis, kyphosis and gibbus are also common. Sensory loss Hunt (1981) documented 87 injuries in 100 spina bifida children that were secondary to sensory loss. These injuries included fractured legs, pressure sores and damage resulting from heat or cold.
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Loss of genito-urinary control Bladder and bowel control is also compromised. Incontinence and incomplete bladder emptying can potentially cause infections and compromise kidney function. Fifty-one per cent of the children studied by Hunt (1981) had faecal incontinence which caused more distress in the classroom than any other aspect of their disability. As children grow, incontinence is tolerated less and can affect social acceptance and self-esteem. ASSOCIATED FEATURES Tethered cord, hydrocephalus, Chiari II malformation, oculomotor irregularities and upper-extremity anomalies are conditions commonly associated with spina bifida that place the child at risk for additional neurological complications or loss of functional status. Spinal-cord tethering Spinal anomalies, such as diastematomyelia, hydromyelia and lipoma are common and may cause spinal-cord tethering. Cord tethering describes a clinical condition where there is deterioration of motor and/or sensory function, progressive scoliosis, changes in urological status and back or leg pain. Hydrocephalus Estimates on the incidence of hydrocephalus and spina bifida vary from 70% to 86% (Hunt 1981, Knowlton et al. 1985). Ventricular shunt treatment has increased survival rates and improved the quality of outcome of children born with spina bifida cystica and hydrocephalus. Dennis et al. (1981) suggest that hydrocephalus may affect gross motor function by deforming the cerebellum, fine motor control by disturbing the basis of hand control, and bimanual motor function by stretching the corpus callosum. Impairment of intellectual and perceptual abilities may be caused by damage to association tracts after ventricular enlargement (Schneider 1985). McLone et al. (1982), however, found that both shunted and non-shunted children without a history of central nervous system infections have intelligence quotients within the normal range. Hydrocephalus is also known to be associated with distractibility
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(Horn et al. 1985), information processing deficits (Miller & Sethi 1971a,b, Zeiner & Prigatano 1982), visuo-spatial relationship problems (Miller & Sethi 1971b, Spain 1974), interhemispheric communication deficits (Miller & Sethi 1971a,b), uneven cognitive growth and abnormalities in brain development (Dennis et al. 1981). Several authors describe the hyperverbal behaviour or 'cocktail party speech syndrome' in children with spina bifida and hydrocephalus. Schwartz (1974) defines this syndrome as: excessive verbalizations, utterances out of context, automatic phrases and cliches, and verbal intelligence quotients higher than performance quotients. If verbal skills are higher than performance, a child's comprehension should be questioned. Many people erroneously think that these children are more capable because of their high verbal skills and become discouraged when the child cannot perform. There is little literature on the effectiveness of speech and language therapy in this area. Chiari II malformation The Arnold-Chiari malformation, specifically Chiari II, is a consistent clinical and pathological finding in spina bifida cystica (McCullough 1986, Naidich et al. 1986). The Chiari II malformation is a congenital anomaly in which the cerebellum and medulla oblongata are elongated and protrude into the spinal canal through the foramen magnum. The malformation may be associated with apnoea, stridor, vocal cord paralysis, retrocollis, nystagmus, upper body weakness, hypotonia or spasticity and feeding problems (Stark 1977). Regurgitation, bronchial aspiration and a depressed or absent gag reflex may be evident (Cardarelli et al. 1986). The nurses at the Glenrose Clinic have more frequently reported a hyperactive gag. Feeding problems may be compounded by poor positioning, proximal instability, deficiencies in oral motor control and difficulties with the upper extremity control needed for self-feeding. The presence of feeding problems may interfere with health, nutrition and general well-being. Oculomotor irregularities Hunt (1981) and Turner (1985) found that 59% to 42% of their subjects had strabismus. Early detection and treatment is important as binocular vision is established in infancy. Binocular vision assists us in making judgements of distance, size and direction. Oculomotor
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control is also important for efficient tracking and scanning (Williamson 1987). Upper extremity anomalies Upper extremity anomalies that are documented in the literature include: immature fine motor development, impaired strength, bilateral inco-ordination, apraxia, poor eye-hand co-ordination and manipulative abilities, reduced reaction time, poor tactile perception, hypotonia, hypertonia, tremors and ataxia and poor initiation and cessation of movement (Brunt 1980, Grimm 1976, Sand et al. 1974, Stephens 1982, Turner 1985). Sand et al. (1974) found that hand impairment occurred more often in children where hydrocephalus or an intelligence quotient of 79 or less was present. Williamson (1987) suggests that upper extremity dysfunction may be attributable to cord cavitation, malformation of the spine above the lesion, cerebellar damage secondary to the Chiari II malformation, hydrocephalus and pyramidal tract abnormalities. He also suggests that upper extremity dysfunction can be further complicated by delayed gross motor development, proximal instability, lack of opportunity and oculomotor anomalies. THE GLENROSE SPINA BIFIDA CLINIC The paediatric Spina Bifida Clinic at the Glenrose Rehabilitation Hospital was established in 1972 to provide care for children aged 3 months to 18 years. The team includes health care professionals from medicine (genetics, paediatrics, neurosurgery, physiatry, urology and orthopaedics), nursing, occupational therapy, physical therapy, psychology, social work, speech therapy, special education, dietary and orthotics. In 1988, the Adolescent Spina Bifida Clinic was created to provide a specialized service to children aged 12 to 18 years. This clinic was established to help adolescents: develop social emotional support systems, increase self-esteem and peer relations, discover and use community resources and increase levels of independence in anticipation of community living. Medical monitoring and information about medical conditions and reproductive potential is also provided. These goals are met through educational sessions, personal interviews, individual and group therapy and community referral and liaison. The
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clinic stresses self-responsibility for health, discourages dependency roles and encourages participation in organized events and spina bifida associations to facilitate contact with positive role models.
THE ROLE OF OCCUPATIONAL THERAPY Occupational therapy assessments determine whether physical, neurological, social, psychological or environmental problems impede participation or performance in life activities. Therapists aim to maintain health by developing or restoring daily living skills to enable children to engage in age-appropriate occupational roles. Atkins & Chapman (1975) and Knowlton et al. (1985) provide detailed outlines of concerns of the spina bifida cystica child based on a growth and developmental model. Rather than re-evaluate every 6 months, as these authors suggest, our children are reviewed by occupational therapy at infancy (9 months), preschool age (3'/2 to 5 years), school age (8 to 9 years) and early adolescence (12 to 15 years). These ages reflect the relative transitional periods when the occupational roles and expectations of children are changing and becoming more challenging (Clark 1985). In infancy, children engage in feeding, socialization and play. The occupation of play is a long, active learning process that contributes to the development of locomotion, cognition, language and social maturity. By preschool age, children are beginning to refine their motor, manual and cognitive skills in preparation for formalized learning. Social and community activities are increasing and active participation in self-care is encouraged. By school age, academic, play and social roles become complex as children are expected to become more self-sufficient. During adolescence, the balance of prevocational, self-care and leisure roles is compounded by issues of sexuality and the emergence of community independence. We propose that these four ages reflect stages in the life of the spina bifida child when occupational therapy assessment is critical. Table 1 illustrates the types of functional problems that may become the focus of assessment and treatment at these age levels. Home programmes, short-term treatment and referrals for community intervention are provided as needed. Between reviews, children can be scheduled at the discretion of the occupational therapist or by referral from parents, educators or other
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TABLE 1. Commoti functional issues in children and adolescents with spina bifida* Age
Common functional issues
9 months
Developmental delay Feeding difficulties Oculomotor irregularities Orthopaedic anomalies Positioning problems Impaired gross motor function and mobility Delayed fine motor development Upper extremity irregularities Perceptual problems 'Cocktail party' speech Disability awareness Advancing orthopaedic deformities School problems Delayed independence in daily living skills Classroom continence Community mobility Delayed independence in community living skills Emotional and financial dependence on parents Limited peer relationships Unemployment Questions on health and sexuality Skin breakdown and back pain Social continence
Preschool (3Vi to 5 years)
School age (8 to 9 years)
Adolescence
•Although issues are addressed at different ages, they may be lifespan treatment objectives.
health care professionals. Children are followed by other team members, such as paediatrics, physiatry, physiotherapy and urology, on a more regular basis as monitoring of neurological status is important. Once the newborn is medically stable, the nurse clinic co-ordinator meets with the family to discuss spina bifida and the future role of the Glenrose Spina Bifida Clinic. From birth to 6 rnonths of age, a baseline occupational therapy assessment is completed if there are orthopaedic concerns requiring splinting, concerns regarding feeding or the potential for the development of upper extremity dysfunction. Occupational therapy can provide recommendations for oral motor exercises, oral sensitization or desensitization techniques, pre-feeding regimes, food selection, preparation and provide feeding aids (Walsh 1988). Children with a greater potential for impaired hand function are
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those with higher level lesions (above TIO to T12) (Sand et al. 1973, Grimm 1976). At 6 months of age, the therapist and family meet to discuss the future role of occupational therapy with their child. General caregiving needs can be addressed and information on normal development provided. Early motor development is monitored in conjunction with the clinic physical therapist. Home programmes and treatment are generally not provided until the child is 9 months of age, as numerous medical procedures usually occupy most of the parents' time and energies. A thorough developmental evaluation is completed at 9 months. Assessment areas include behaviour, oculomotor control, muscle tone, reflex integration, fine and gross motor abilities, positioning for play and transportation and overall play development skills. Assessments are based on clinical observation and comparison to normative developmental profiles of movement quality and motor milestones. Between Wi to 5 years, a preschool or school readiness assessment is completed. The assessment includes the areas listed previously but also includes standardized evaluations of visual perceptual motor development and refinement of fine motor control. The tests most commonly used at the clinic include the Motor Free Visual Perceptual Test, Gardner's Test of Visual Perceptual Skills and Test of Visual Motor Skills, the Beery Developmental Test of Visual Motor Integration, Gesell's Draw A Person and the Peabody Developmental Motor Scales (fine motor index). Independence in daily living skills is encouraged. Evaluation with a questionnaire is used to compare abilities with accepted norms. Sousa et al. (1976) developed a functional activities scoring system to evaluate daily living skills in children with spina bifida. The mean scores of 256 patients, age 1 day to 72 years, is graphically plotted to depict the average age of development of functional activities of daily living. This graph, therefore, provides guidelines for developmental expectations and a means of setting realistic treatment goals. Mastery of stool and urinary self-care was found to be more delayed than any other activity of daily living. Sousa et al. (1976) stress that parental co-operation in accepting, learning and teaching self-toileting is essential. Parents should be encouraged to transfer the responsibility for self-cleanliness as soon as the child is physically and mentally capable. Children as young as 3 and 5 years can learn intermittent catheterization and may benefit from learning tools like dolls for
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practice or mirrors for self-checking (Hannigan 1979). Adequate fine motor skills and eye-hand co-ordination are prerequisites to independence in this area. Between 8 and 9 years, school becomes the primary occupation of the spina bifida child. Subtle deficits in visual perceptual motor skills can begin to affect school success as the demands for quality and quantity of school work increases. Culatta (1980) found that 70% to 80% of spina bifida children had marked educational handicaps not attributed to retardation and which required special education programmes. These handicaps were in the areas of: sensory integration, visual perception, organization and attention, language and academics. Sand et al. (1973) and Tew & Laurence (1975) found a higher than average probability of visual perceptual impairment in children with spina bifida. Gluckman & Barling (1980), however, found significant improvements in performance on the Frostig Developmental Test of Visual Perception following administration of the Frostig Program for the Development of Visual Perception. Horn et al. (1985), Spain (1974) and Tew & Laurence (1975) describe a number of other deficits and learning problems including reading comprehension, mathematical reasoning, spelling, distractibility and inattention. Stephens (1982) describes difficulties with spatial and time orientation, direction and social perception. Occupational therapists are able to use tests such as Gardiner's Test of Visual Perceptual Skills, the Test of Visual Motor Skills and parts of the Sensory Integration and Praxis Tests to assist educators in teaching spina bifida children. With the current trend of academic mainstreaming, occupational therapists provide remedial, preventive and compensatory strategies for school programming. Consulting on adaptive seating, perceptual motor remediation and overall therapeutic goals ensures that therapy continues in the classroom and that the children are able to participate in regular curricular activities. As children with spina bifida differ greatly in their degree of physical impairment and potential for development, individualized assessment, treatment and educational programming is essential. Hunt (1981) found that most teachers regard the presence of a handicapped child in the class as positive, as other children developed a sense of consideration, responsibility and kindness. At 12 years, children begin the Adolescent Clinic by being assigned to a social worker, psychologist or occupational therapist who will
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serve as a primary contact. Personal interviews are held to assess needs in sexuality, social skills, independent living skills, transportation and prevocational exploration. Concerns of the spina bifida adolescent parallel normal development, but issues are compounded by the special challenges of a disabled person. Strax (1976) describes four developmental goals of adolescence: consolidating identity, achieving emotional and financial independence from parents, establishing new peer relationships and finding a vocation. Limited mobility, prolonged dependence, limited life experiences, social isolation, poor peer relation and limited role models place an added challenge on the adolescent with spina bifida (Strax 1976, Sherman et al 1985, Dorner 1975). Hayden et al. (1979) found that adolescents with myelodysplasia tend to suffer low self-esteem, and have doubts about their health and sexuality. There was no significant difference between 20 teenagers with myelodysplasia and controls in frequency of perceived depression or sadness. Social immaturity, however, was prevalent and may be secondary to frequent or prolonged hospitalizations and environmental barriers to independence. The role of the occupational therapist on this clinic as a contact person is primarily that of an educator, facilitator and counsellor. Referrals for occupational therapy, specific assessment and treatment are made by the contact person following personal interviews. CASE EXAMPLE The Glenrose Spina Bifida Clinic nurse co-ordinator first met P.N.'s parents 3 days after his birth. Back closure surgery for a high lumbar meningomyelocele and shunting for hydrocephalus was complete. Information about the role of the clinic and questions regarding spina bifida were addressed. At 6 months of age, P.N. attended his first clinic. The neurologist noticed persisting primitive head and neck reflex patterns. Baby P.N. could not sit without support and was suckling from a bottle. The occupational therapist introduced herself to his parents and discussed her future role with the child. The therapist recommended using a Fisher Price head support in the child's care seat to promote midline head position and reduce the effects of the delayed reflex integration on his motor development. At 9 months, a developmental assessment was completed. The
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persisting reflex pattern noted at 6 months was integrated. Baby P.N.'s fine motor play skills were delayed and he continued to require support in sitting. A small positioning aid was loaned to the family to enable P.N. to spend some time sitting alone with his hands free for play. Toy ideas were provided to give P.N. the fine motor experience he needed. At 21 months, P.N.'s mother requested an occupational therapy assessment as P.N. had some 'unusual eating habits'. P.N. would only chew food with his front teeth. A hyperreflexic gag could be elicited through stimulation on the back 2/3 of P.N.'s tongue. This finding, in combination with other neurological signs, prompted the neurosurgeon to monitor and eventually perform surgery. P.N. was experiencing pressure to his brainstem from his Arnold-Chiari malformation. P.N.'s next visit was at age 3. By this time, P.N. was mobile in his wheelchair. P.N. was found to have low tone in his upper limbs and had not yet established a hand dominance. P.N. did not like crayon work. Due to these fine motor irregularities a review was scheduled at age 4. Self-care was significantly delayed and his mother thought it was time for P.N. to learn to become more independent. P.N. was seen monthly for 6 months to improve his dressing skills, and transfers in and out of bed. At 4 years, P.N.'s fine motor skills, as assessed with the Peabody Developmental Motor Scales, were significantly delayed. A fine motor home programme was given and a school consultation visit was done when P.N. entered kindergarten. P.N. was seen again at age 8. He had done well in school academics but was easily distracted and verbal in class, particularly during mathematics, and had little concept of time when he was told to hurry and complete an assignment. His fine motor and self-care skills were within age expectations. On the Gardner Test of Visual Perceptual Skills, P.N.'s performance fell to low average. He had particular difficulty with the visual sequencing memory subtests. This visual memory skill was effecting P.N.'s ability to copy efficiently mathematics questions from the board. P.N.'s family was given a number of memory games designed to develop P.N.'s visual sequencing memory skills. The teacher tried hand-out printed questions, and P.N. was eventually able to keep up with the workload expected of his peers. Strategies for dealing with a child who is easily distracted in the classroom was discussed with the teacher. P.N. was seated at the front
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of the class and students no longer arranged their desks in small groups. P.N. was given a small desk timer to learn self-time management within the class. P.N. was seen on three occasions between the ages of 8 and 12 at his parents' request. Self-catheterization, bathtub transfers and dressing skills were all taught. At the age of 12, P.N. was interviewed by the occupational therapist upon entry into the Adolescent clinic. P.N. seemed to have a lot of anxieties around dating and establishing friends at his new school. P.N. was referred to a summer programme organized by the social worker and occupational therapist. The topics included: assertiveness training, establishing and developing relationships, organizing social outings and visiting community recreational centres. CONCLUSION Children with spina bifida present with a number of features that affect functional performance and developmental potential. As the spina bifida population increases, occupational therapists are establishing their role in the care of these clients. By evaluating children's physical, neurological, social, psychological and environmental status, occupational therapists monitor and effect change in performance of activities of daily living. Consultation and treatment maximize functional independence and adaptation. Treatment focuses on remediating deficits that are amenable to change and teaching compensation for those that are not.
ACKNOWLEDGEMENTS The author wishes to thank Kate Yelle Watt BScOT from the Glenrose Rehabilitation Hospital for sharing her clinical expertise, and Christy L.A. Nelson MS OTR from the Glenrose Rehabilitation Hospital for her assistance in literature search and editing. REFERENCES i Atkins J.A. & Chapman R.L. (1975). Occupational therapy in a myelomeninocele clinic. American Joumal of Occupational Therapy 229(7), 403-406
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Brunt D. (1980) Characteristics of upper limb movement in a sample of meningomyelocele children. Perceptual and Motor Skills 51. 431-437 Cardarelli M., Di Rocco C. & McLone D.G. (1986) Chiari II malformation: clinical manifestations and indications for decompression. In Spina Bifida: A Multidisciplinary Approach, eds R.L. McLaurin, S. Oppenheimer, L. Dias & W.E. Kaplan. Praeger. New York Clark R.N. (1985) Occupational therapy in paediatrics. In Occupational Therapy for Children, eds R. N. Clark & A. S. Allen. C. V. Mosby, St Louis Culatta B. (1980) Perceptual and linguistic performance of spina bifida hydrocephalic children. Spina Bifida Therapy 2, 235 Dennis M., Fitz C.R.. Netley C.T., Sugar J.. Derek C.K., Harwood-Nash D.C.F. et at. (1981) The intelligence of hydrocephalic children. Archives of Neurology 38, 607-615 Dorner S\ (1975) The relationship of physical handicap to stress in families with an adolescent with spina bifida. Developmental Medicine and Child Neurology 17. 765-776 Gluckman S. & Barling J. (1980) Effects of a remedial program on visual-motor perception in spina bifida children. Journal of Genetic Psychology 136, 195-202 Grimm R.A. (1976) Hand function and tactile perception in a sample of children with myelomeningocele. American Journal of Occupational Therapy 30, 234-240 Hannigan K.F. (1979) Teaching intermittant self catheterization to young children with myelodysplasia. Developmental Medicine and Child Neurology 21(3), 365-368 Hayden P.W., Davenport S. & Campbell M. (1979) Adolescents with myelodysplasia: impact of physical disability on emotional maturation. Pediatrics 64(1), 53-59 Horn D.G., Lorch E., Lorch R.F. & Culatta B. (1985) Distractibility and vocabulary deficits in children with spina bifida and hydrocephalus. Developmental Medicine and Child Neurology 27, 713-720 Hunt G.M. (1981) Spina bifida: implications for 100 children at school. Developmental Medicine and Child Neurology 23, 160-172 Knowlton D.D., Peterson K. & Putbrese A. (1985) Team management of cognitive dysfunction in children with spina bifida. Rehabilitation Literature 46(9-10). 259-263 McCullough D.C. (1986) Theories of development of the Arnold Chiari malformation. In Spina Bifida: A Multidisciplinary Approach, eds R. L. McLaurin, S. Oppenheimer, L. Dias & W. E. Kaplan. Praeger, New York, pp. 159-163 McLone D., Czyzewski P., Raimondi A. J. & Sommers R. C. (1982) Central nervous system infections as a limiting factor in the intelligence of children with myelomeningocele. Paediatrics 70(3), 338-342 Menelaus M. (1980) The Orthopaedic Management of Spina Bifida Cystica 2nd end. Churchill Livingstone, New York Miller E. & Sethi L. (1971a) Tactile matching in children with hydrocephalus. Neuropediatrics 3(2), 191-194 Miller E. & Sethi L. (1971b) The effect of hydrocephalus on perception. Developmental Medicine and Child Neurology 13(25), 77-81 Myers G.J. (1984) Myelomeningocele: the medical aspects. In Children With Spina Bifida: Early Intervention and Preschool Programming, ed. G. Williamson. Brookes, Baltimore Naidich T.P., Maravilla D. & McLone D.G. (1986) Radiology of the Chiari malformation. In Spina Bifida: A Multidisciplinary Approach, eds R. L. McLaurin, S. Oppenheimer, L. Dias & W. E. Kaplan. Praeger, New York, pp. 164-173 Sand P.L., Taylor N., Hill M., Kosky N. & Rawlings M. (1974) Hand function in children with myelomingocele. American Journal of Occupational Therapy 28(2), 87-90 Sand P.L., Taylor N., Rawlings M. & Chitnis S., (1973) Performance of children with spina biflda manifesta on the Frostig developmental test of visual perception. Perceptual and Motor Skills 37, 539-546 Schneider J. W. (1985) Spina bifida. In Neurological Rehabilitation vol. Ill, ed. D. A. Umphred. C. V. Mosby, St Louis, pp. 289-314
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Schwartz E. (1974) Characteristics of speech and language development in children with myelomeningocele and hydrocephalus. Journal of Speech and Hearing Disorders 39, 465468 Sherman R.G., Berling B.S. & Oppenheimer S. (1985) Increasing community independence for adolescents with spina bifida. Adolescence 20(77). 1-13 Sousa J . C . Gordon L.H. & Shurtleff D.B. (1976) Assessing the development of daily living skills in patients with spina bifida. Developmental Medicine and Child Neurology 18(6. 37). 134142 Spain B. (1974) Verbal and performance ability in pre-school children with spina bifida. Developmental Medicine and Child Neurology 16. 773-780 Stark G.D. (1977) Spina Bifida: Problems and Management. Blackwell Scientific. Boston. Stephens S. (1982) Learning difficulties and children born with neural tube defect. Spina Bifida Therapy 4(2). 63-76 Strax T.E. (1976) Adolescence: a period of stress, the search for an identity. In Birth Defects. Original Article Series, eds D. Bergma & A. Pulver. A. R. Liss. New York Tew B. & Laurence K.M. (1975) The effects of hydrocephalus on intelligence, visual perception and school attainment. Development Medicine and Child Neurology 17(35). 129-134 Turner A, (1985) Hand function in children with myelomeningocele. Journal of Bone and Joint Surgery 67-B(2). 268-272 Walsh M. (1988) Chiari can cause feeding problems. Insights into spina bifida. Newsletter of the Spina Bifida Association of America, March/April Williamson G. (ed.) (1987) Children with Spina Bifida: Early Intervention and Preschool Programming. Brookes. Baltimore Wolraich M, (1983) The Needs of Children With Spina Bifida: A Comprehensive Review. University of Iowa. Iowa City Zeiner H.K. & Prigatano G.P. (1982) Information processing deficits in hydrocephalic and letter reversal children. Neuropsychotogy 20(4). 483-492
Occupational therapy intervention guidelines for children and adolescents with spina bifida DIANE WATSON Formerly ofGlenrose Rehabilitation Hospital, Pediatric Rehabilitation Unit, Edmonton, Alberta, Canada
Accepted for publication 10 July 1991
Summary Children and adolescents with spina bifida present with a number of characteristics that affect functional performance and developmental skills. The focus of this paper will be to share information about spina bifida and the overall approach to care within the Glenrose Rehabilitation Hospital Spina Bifida Clinic and, more specifically, the role of the occupational therapist. INTRODUCTION With recent advances in medical and surgical techniques, the life span and quality of life of people with spina bifida has expanded. The Glenrose Rehabilitation Hospital in Edmonton, Alberta, Canada, provides a co-ordinated and comprehensive assessment and treatment clinic that serves the children of northern Alberta and the North-west Territories who have congenital or acquired spinal-cord anomalies. SPINA BIFIDA Spina bifida is a congenital malformation of the vertebral arches resulting in failure of closure with or without dysplasia of the spinal cord. Wolraich (1983) reports that the incidence of spina bifida in the United States is 0-7 to 1-0 cases per 1000 live births. The prevalence of neural tube defects varies over time and by geographic location. Although specific causes are not yet known, both genetic and environmental factors play an interactive role (Williamson 1987). Correspondence: Diane Watson, Box 25 Site 471 R.R. 4, Sherwood Park, Alberta T8A 3K4, Canada.
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Spina bifida is classified into types according to the degree of involvement and resultant deficit. Spina bifida occulta is an opening in the vertebrae without displacement of tissues. Neurological deficits are uncommon. Spina bifida cystica describes a sac-like protrusion through the defective vertebral arches and includes two types: meningocele and meningomyelocele. Surgical closure of the protrusion is necessary and often occurs shortly after birth. Meningocele is a condition where meninges and cerebral spinal fluid protrude through the vertebral opening. As the spinal cord remains intact, neurological deficit is uncommon. Meningomyelocele occurs in 94% of spina bifida cystica cases and is the most severe form of spina bifida (Menelaus 1980). The protrusion includes meninges and neural tissue.
CLINICAL FEATURES The most obvious clinical features of spinal neurological damage are motor impairment, orthopaedic deformities and loss of sensation and genito-urinary control (Atkins & Chapman 1975). The extent of neurological damage and resultant functional problems is dependent on the level and type of lesion. Motor impairment of the vertebral opening The site usually indicates the level of neurological damage but typically is not the only source of insult. Meningomyelocele is most common in the lumbosacral region and can result in scattered and asymmetrical innervation. Orthopaedic deformities Scattered congenital neurological impairment, accompanied by limited movement in utero, can result in orthopaedic deformities present at birth. After birth, children are susceptible to additional lower extremity joint deformities and contractures. Scoliosis, kyphosis and gibbus are also common. Sensory loss Hunt (1981) documented 87 injuries in 100 spina bifida children that were secondary to sensory loss. These injuries included fractured legs, pressure sores and damage resulting from heat or cold.
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Loss of genito-urinary control Bladder and bowel control is also compromised. Incontinence and incomplete bladder emptying can potentially cause infections and compromise kidney function. Fifty-one per cent of the children studied by Hunt (1981) had faecal incontinence which caused more distress in the classroom than any other aspect of their disability. As children grow, incontinence is tolerated less and can affect social acceptance and self-esteem. ASSOCIATED FEATURES Tethered cord, hydrocephalus, Chiari II malformation, oculomotor irregularities and upper-extremity anomalies are conditions commonly associated with spina bifida that place the child at risk for additional neurological complications or loss of functional status. Spinal-cord tethering Spinal anomalies, such as diastematomyelia, hydromyelia and lipoma are common and may cause spinal-cord tethering. Cord tethering describes a clinical condition where there is deterioration of motor and/or sensory function, progressive scoliosis, changes in urological status and back or leg pain. Hydrocephalus Estimates on the incidence of hydrocephalus and spina bifida vary from 70% to 86% (Hunt 1981, Knowlton et al. 1985). Ventricular shunt treatment has increased survival rates and improved the quality of outcome of children born with spina bifida cystica and hydrocephalus. Dennis et al. (1981) suggest that hydrocephalus may affect gross motor function by deforming the cerebellum, fine motor control by disturbing the basis of hand control, and bimanual motor function by stretching the corpus callosum. Impairment of intellectual and perceptual abilities may be caused by damage to association tracts after ventricular enlargement (Schneider 1985). McLone et al. (1982), however, found that both shunted and non-shunted children without a history of central nervous system infections have intelligence quotients within the normal range. Hydrocephalus is also known to be associated with distractibility
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(Horn et al. 1985), information processing deficits (Miller & Sethi 1971a,b, Zeiner & Prigatano 1982), visuo-spatial relationship problems (Miller & Sethi 1971b, Spain 1974), interhemispheric communication deficits (Miller & Sethi 1971a,b), uneven cognitive growth and abnormalities in brain development (Dennis et al. 1981). Several authors describe the hyperverbal behaviour or 'cocktail party speech syndrome' in children with spina bifida and hydrocephalus. Schwartz (1974) defines this syndrome as: excessive verbalizations, utterances out of context, automatic phrases and cliches, and verbal intelligence quotients higher than performance quotients. If verbal skills are higher than performance, a child's comprehension should be questioned. Many people erroneously think that these children are more capable because of their high verbal skills and become discouraged when the child cannot perform. There is little literature on the effectiveness of speech and language therapy in this area. Chiari II malformation The Arnold-Chiari malformation, specifically Chiari II, is a consistent clinical and pathological finding in spina bifida cystica (McCullough 1986, Naidich et al. 1986). The Chiari II malformation is a congenital anomaly in which the cerebellum and medulla oblongata are elongated and protrude into the spinal canal through the foramen magnum. The malformation may be associated with apnoea, stridor, vocal cord paralysis, retrocollis, nystagmus, upper body weakness, hypotonia or spasticity and feeding problems (Stark 1977). Regurgitation, bronchial aspiration and a depressed or absent gag reflex may be evident (Cardarelli et al. 1986). The nurses at the Glenrose Clinic have more frequently reported a hyperactive gag. Feeding problems may be compounded by poor positioning, proximal instability, deficiencies in oral motor control and difficulties with the upper extremity control needed for self-feeding. The presence of feeding problems may interfere with health, nutrition and general well-being. Oculomotor irregularities Hunt (1981) and Turner (1985) found that 59% to 42% of their subjects had strabismus. Early detection and treatment is important as binocular vision is established in infancy. Binocular vision assists us in making judgements of distance, size and direction. Oculomotor
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control is also important for efficient tracking and scanning (Williamson 1987). Upper extremity anomalies Upper extremity anomalies that are documented in the literature include: immature fine motor development, impaired strength, bilateral inco-ordination, apraxia, poor eye-hand co-ordination and manipulative abilities, reduced reaction time, poor tactile perception, hypotonia, hypertonia, tremors and ataxia and poor initiation and cessation of movement (Brunt 1980, Grimm 1976, Sand et al. 1974, Stephens 1982, Turner 1985). Sand et al. (1974) found that hand impairment occurred more often in children where hydrocephalus or an intelligence quotient of 79 or less was present. Williamson (1987) suggests that upper extremity dysfunction may be attributable to cord cavitation, malformation of the spine above the lesion, cerebellar damage secondary to the Chiari II malformation, hydrocephalus and pyramidal tract abnormalities. He also suggests that upper extremity dysfunction can be further complicated by delayed gross motor development, proximal instability, lack of opportunity and oculomotor anomalies. THE GLENROSE SPINA BIFIDA CLINIC The paediatric Spina Bifida Clinic at the Glenrose Rehabilitation Hospital was established in 1972 to provide care for children aged 3 months to 18 years. The team includes health care professionals from medicine (genetics, paediatrics, neurosurgery, physiatry, urology and orthopaedics), nursing, occupational therapy, physical therapy, psychology, social work, speech therapy, special education, dietary and orthotics. In 1988, the Adolescent Spina Bifida Clinic was created to provide a specialized service to children aged 12 to 18 years. This clinic was established to help adolescents: develop social emotional support systems, increase self-esteem and peer relations, discover and use community resources and increase levels of independence in anticipation of community living. Medical monitoring and information about medical conditions and reproductive potential is also provided. These goals are met through educational sessions, personal interviews, individual and group therapy and community referral and liaison. The
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clinic stresses self-responsibility for health, discourages dependency roles and encourages participation in organized events and spina bifida associations to facilitate contact with positive role models.
THE ROLE OF OCCUPATIONAL THERAPY Occupational therapy assessments determine whether physical, neurological, social, psychological or environmental problems impede participation or performance in life activities. Therapists aim to maintain health by developing or restoring daily living skills to enable children to engage in age-appropriate occupational roles. Atkins & Chapman (1975) and Knowlton et al. (1985) provide detailed outlines of concerns of the spina bifida cystica child based on a growth and developmental model. Rather than re-evaluate every 6 months, as these authors suggest, our children are reviewed by occupational therapy at infancy (9 months), preschool age (3'/2 to 5 years), school age (8 to 9 years) and early adolescence (12 to 15 years). These ages reflect the relative transitional periods when the occupational roles and expectations of children are changing and becoming more challenging (Clark 1985). In infancy, children engage in feeding, socialization and play. The occupation of play is a long, active learning process that contributes to the development of locomotion, cognition, language and social maturity. By preschool age, children are beginning to refine their motor, manual and cognitive skills in preparation for formalized learning. Social and community activities are increasing and active participation in self-care is encouraged. By school age, academic, play and social roles become complex as children are expected to become more self-sufficient. During adolescence, the balance of prevocational, self-care and leisure roles is compounded by issues of sexuality and the emergence of community independence. We propose that these four ages reflect stages in the life of the spina bifida child when occupational therapy assessment is critical. Table 1 illustrates the types of functional problems that may become the focus of assessment and treatment at these age levels. Home programmes, short-term treatment and referrals for community intervention are provided as needed. Between reviews, children can be scheduled at the discretion of the occupational therapist or by referral from parents, educators or other
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TABLE 1. Commoti functional issues in children and adolescents with spina bifida* Age
Common functional issues
9 months
Developmental delay Feeding difficulties Oculomotor irregularities Orthopaedic anomalies Positioning problems Impaired gross motor function and mobility Delayed fine motor development Upper extremity irregularities Perceptual problems 'Cocktail party' speech Disability awareness Advancing orthopaedic deformities School problems Delayed independence in daily living skills Classroom continence Community mobility Delayed independence in community living skills Emotional and financial dependence on parents Limited peer relationships Unemployment Questions on health and sexuality Skin breakdown and back pain Social continence
Preschool (3Vi to 5 years)
School age (8 to 9 years)
Adolescence
•Although issues are addressed at different ages, they may be lifespan treatment objectives.
health care professionals. Children are followed by other team members, such as paediatrics, physiatry, physiotherapy and urology, on a more regular basis as monitoring of neurological status is important. Once the newborn is medically stable, the nurse clinic co-ordinator meets with the family to discuss spina bifida and the future role of the Glenrose Spina Bifida Clinic. From birth to 6 rnonths of age, a baseline occupational therapy assessment is completed if there are orthopaedic concerns requiring splinting, concerns regarding feeding or the potential for the development of upper extremity dysfunction. Occupational therapy can provide recommendations for oral motor exercises, oral sensitization or desensitization techniques, pre-feeding regimes, food selection, preparation and provide feeding aids (Walsh 1988). Children with a greater potential for impaired hand function are
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those with higher level lesions (above TIO to T12) (Sand et al. 1973, Grimm 1976). At 6 months of age, the therapist and family meet to discuss the future role of occupational therapy with their child. General caregiving needs can be addressed and information on normal development provided. Early motor development is monitored in conjunction with the clinic physical therapist. Home programmes and treatment are generally not provided until the child is 9 months of age, as numerous medical procedures usually occupy most of the parents' time and energies. A thorough developmental evaluation is completed at 9 months. Assessment areas include behaviour, oculomotor control, muscle tone, reflex integration, fine and gross motor abilities, positioning for play and transportation and overall play development skills. Assessments are based on clinical observation and comparison to normative developmental profiles of movement quality and motor milestones. Between Wi to 5 years, a preschool or school readiness assessment is completed. The assessment includes the areas listed previously but also includes standardized evaluations of visual perceptual motor development and refinement of fine motor control. The tests most commonly used at the clinic include the Motor Free Visual Perceptual Test, Gardner's Test of Visual Perceptual Skills and Test of Visual Motor Skills, the Beery Developmental Test of Visual Motor Integration, Gesell's Draw A Person and the Peabody Developmental Motor Scales (fine motor index). Independence in daily living skills is encouraged. Evaluation with a questionnaire is used to compare abilities with accepted norms. Sousa et al. (1976) developed a functional activities scoring system to evaluate daily living skills in children with spina bifida. The mean scores of 256 patients, age 1 day to 72 years, is graphically plotted to depict the average age of development of functional activities of daily living. This graph, therefore, provides guidelines for developmental expectations and a means of setting realistic treatment goals. Mastery of stool and urinary self-care was found to be more delayed than any other activity of daily living. Sousa et al. (1976) stress that parental co-operation in accepting, learning and teaching self-toileting is essential. Parents should be encouraged to transfer the responsibility for self-cleanliness as soon as the child is physically and mentally capable. Children as young as 3 and 5 years can learn intermittent catheterization and may benefit from learning tools like dolls for
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practice or mirrors for self-checking (Hannigan 1979). Adequate fine motor skills and eye-hand co-ordination are prerequisites to independence in this area. Between 8 and 9 years, school becomes the primary occupation of the spina bifida child. Subtle deficits in visual perceptual motor skills can begin to affect school success as the demands for quality and quantity of school work increases. Culatta (1980) found that 70% to 80% of spina bifida children had marked educational handicaps not attributed to retardation and which required special education programmes. These handicaps were in the areas of: sensory integration, visual perception, organization and attention, language and academics. Sand et al. (1973) and Tew & Laurence (1975) found a higher than average probability of visual perceptual impairment in children with spina bifida. Gluckman & Barling (1980), however, found significant improvements in performance on the Frostig Developmental Test of Visual Perception following administration of the Frostig Program for the Development of Visual Perception. Horn et al. (1985), Spain (1974) and Tew & Laurence (1975) describe a number of other deficits and learning problems including reading comprehension, mathematical reasoning, spelling, distractibility and inattention. Stephens (1982) describes difficulties with spatial and time orientation, direction and social perception. Occupational therapists are able to use tests such as Gardiner's Test of Visual Perceptual Skills, the Test of Visual Motor Skills and parts of the Sensory Integration and Praxis Tests to assist educators in teaching spina bifida children. With the current trend of academic mainstreaming, occupational therapists provide remedial, preventive and compensatory strategies for school programming. Consulting on adaptive seating, perceptual motor remediation and overall therapeutic goals ensures that therapy continues in the classroom and that the children are able to participate in regular curricular activities. As children with spina bifida differ greatly in their degree of physical impairment and potential for development, individualized assessment, treatment and educational programming is essential. Hunt (1981) found that most teachers regard the presence of a handicapped child in the class as positive, as other children developed a sense of consideration, responsibility and kindness. At 12 years, children begin the Adolescent Clinic by being assigned to a social worker, psychologist or occupational therapist who will
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serve as a primary contact. Personal interviews are held to assess needs in sexuality, social skills, independent living skills, transportation and prevocational exploration. Concerns of the spina bifida adolescent parallel normal development, but issues are compounded by the special challenges of a disabled person. Strax (1976) describes four developmental goals of adolescence: consolidating identity, achieving emotional and financial independence from parents, establishing new peer relationships and finding a vocation. Limited mobility, prolonged dependence, limited life experiences, social isolation, poor peer relation and limited role models place an added challenge on the adolescent with spina bifida (Strax 1976, Sherman et al 1985, Dorner 1975). Hayden et al. (1979) found that adolescents with myelodysplasia tend to suffer low self-esteem, and have doubts about their health and sexuality. There was no significant difference between 20 teenagers with myelodysplasia and controls in frequency of perceived depression or sadness. Social immaturity, however, was prevalent and may be secondary to frequent or prolonged hospitalizations and environmental barriers to independence. The role of the occupational therapist on this clinic as a contact person is primarily that of an educator, facilitator and counsellor. Referrals for occupational therapy, specific assessment and treatment are made by the contact person following personal interviews. CASE EXAMPLE The Glenrose Spina Bifida Clinic nurse co-ordinator first met P.N.'s parents 3 days after his birth. Back closure surgery for a high lumbar meningomyelocele and shunting for hydrocephalus was complete. Information about the role of the clinic and questions regarding spina bifida were addressed. At 6 months of age, P.N. attended his first clinic. The neurologist noticed persisting primitive head and neck reflex patterns. Baby P.N. could not sit without support and was suckling from a bottle. The occupational therapist introduced herself to his parents and discussed her future role with the child. The therapist recommended using a Fisher Price head support in the child's care seat to promote midline head position and reduce the effects of the delayed reflex integration on his motor development. At 9 months, a developmental assessment was completed. The
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persisting reflex pattern noted at 6 months was integrated. Baby P.N.'s fine motor play skills were delayed and he continued to require support in sitting. A small positioning aid was loaned to the family to enable P.N. to spend some time sitting alone with his hands free for play. Toy ideas were provided to give P.N. the fine motor experience he needed. At 21 months, P.N.'s mother requested an occupational therapy assessment as P.N. had some 'unusual eating habits'. P.N. would only chew food with his front teeth. A hyperreflexic gag could be elicited through stimulation on the back 2/3 of P.N.'s tongue. This finding, in combination with other neurological signs, prompted the neurosurgeon to monitor and eventually perform surgery. P.N. was experiencing pressure to his brainstem from his Arnold-Chiari malformation. P.N.'s next visit was at age 3. By this time, P.N. was mobile in his wheelchair. P.N. was found to have low tone in his upper limbs and had not yet established a hand dominance. P.N. did not like crayon work. Due to these fine motor irregularities a review was scheduled at age 4. Self-care was significantly delayed and his mother thought it was time for P.N. to learn to become more independent. P.N. was seen monthly for 6 months to improve his dressing skills, and transfers in and out of bed. At 4 years, P.N.'s fine motor skills, as assessed with the Peabody Developmental Motor Scales, were significantly delayed. A fine motor home programme was given and a school consultation visit was done when P.N. entered kindergarten. P.N. was seen again at age 8. He had done well in school academics but was easily distracted and verbal in class, particularly during mathematics, and had little concept of time when he was told to hurry and complete an assignment. His fine motor and self-care skills were within age expectations. On the Gardner Test of Visual Perceptual Skills, P.N.'s performance fell to low average. He had particular difficulty with the visual sequencing memory subtests. This visual memory skill was effecting P.N.'s ability to copy efficiently mathematics questions from the board. P.N.'s family was given a number of memory games designed to develop P.N.'s visual sequencing memory skills. The teacher tried hand-out printed questions, and P.N. was eventually able to keep up with the workload expected of his peers. Strategies for dealing with a child who is easily distracted in the classroom was discussed with the teacher. P.N. was seated at the front
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of the class and students no longer arranged their desks in small groups. P.N. was given a small desk timer to learn self-time management within the class. P.N. was seen on three occasions between the ages of 8 and 12 at his parents' request. Self-catheterization, bathtub transfers and dressing skills were all taught. At the age of 12, P.N. was interviewed by the occupational therapist upon entry into the Adolescent clinic. P.N. seemed to have a lot of anxieties around dating and establishing friends at his new school. P.N. was referred to a summer programme organized by the social worker and occupational therapist. The topics included: assertiveness training, establishing and developing relationships, organizing social outings and visiting community recreational centres. CONCLUSION Children with spina bifida present with a number of features that affect functional performance and developmental potential. As the spina bifida population increases, occupational therapists are establishing their role in the care of these clients. By evaluating children's physical, neurological, social, psychological and environmental status, occupational therapists monitor and effect change in performance of activities of daily living. Consultation and treatment maximize functional independence and adaptation. Treatment focuses on remediating deficits that are amenable to change and teaching compensation for those that are not.
ACKNOWLEDGEMENTS The author wishes to thank Kate Yelle Watt BScOT from the Glenrose Rehabilitation Hospital for sharing her clinical expertise, and Christy L.A. Nelson MS OTR from the Glenrose Rehabilitation Hospital for her assistance in literature search and editing. REFERENCES i Atkins J.A. & Chapman R.L. (1975). Occupational therapy in a myelomeninocele clinic. American Joumal of Occupational Therapy 229(7), 403-406
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Brunt D. (1980) Characteristics of upper limb movement in a sample of meningomyelocele children. Perceptual and Motor Skills 51. 431-437 Cardarelli M., Di Rocco C. & McLone D.G. (1986) Chiari II malformation: clinical manifestations and indications for decompression. In Spina Bifida: A Multidisciplinary Approach, eds R.L. McLaurin, S. Oppenheimer, L. Dias & W.E. Kaplan. Praeger. New York Clark R.N. (1985) Occupational therapy in paediatrics. In Occupational Therapy for Children, eds R. N. Clark & A. S. Allen. C. V. Mosby, St Louis Culatta B. (1980) Perceptual and linguistic performance of spina bifida hydrocephalic children. Spina Bifida Therapy 2, 235 Dennis M., Fitz C.R.. Netley C.T., Sugar J.. Derek C.K., Harwood-Nash D.C.F. et at. (1981) The intelligence of hydrocephalic children. Archives of Neurology 38, 607-615 Dorner S\ (1975) The relationship of physical handicap to stress in families with an adolescent with spina bifida. Developmental Medicine and Child Neurology 17. 765-776 Gluckman S. & Barling J. (1980) Effects of a remedial program on visual-motor perception in spina bifida children. Journal of Genetic Psychology 136, 195-202 Grimm R.A. (1976) Hand function and tactile perception in a sample of children with myelomeningocele. American Journal of Occupational Therapy 30, 234-240 Hannigan K.F. (1979) Teaching intermittant self catheterization to young children with myelodysplasia. Developmental Medicine and Child Neurology 21(3), 365-368 Hayden P.W., Davenport S. & Campbell M. (1979) Adolescents with myelodysplasia: impact of physical disability on emotional maturation. Pediatrics 64(1), 53-59 Horn D.G., Lorch E., Lorch R.F. & Culatta B. (1985) Distractibility and vocabulary deficits in children with spina bifida and hydrocephalus. Developmental Medicine and Child Neurology 27, 713-720 Hunt G.M. (1981) Spina bifida: implications for 100 children at school. Developmental Medicine and Child Neurology 23, 160-172 Knowlton D.D., Peterson K. & Putbrese A. (1985) Team management of cognitive dysfunction in children with spina bifida. Rehabilitation Literature 46(9-10). 259-263 McCullough D.C. (1986) Theories of development of the Arnold Chiari malformation. In Spina Bifida: A Multidisciplinary Approach, eds R. L. McLaurin, S. Oppenheimer, L. Dias & W. E. Kaplan. Praeger, New York, pp. 159-163 McLone D., Czyzewski P., Raimondi A. J. & Sommers R. C. (1982) Central nervous system infections as a limiting factor in the intelligence of children with myelomeningocele. Paediatrics 70(3), 338-342 Menelaus M. (1980) The Orthopaedic Management of Spina Bifida Cystica 2nd end. Churchill Livingstone, New York Miller E. & Sethi L. (1971a) Tactile matching in children with hydrocephalus. Neuropediatrics 3(2), 191-194 Miller E. & Sethi L. (1971b) The effect of hydrocephalus on perception. Developmental Medicine and Child Neurology 13(25), 77-81 Myers G.J. (1984) Myelomeningocele: the medical aspects. In Children With Spina Bifida: Early Intervention and Preschool Programming, ed. G. Williamson. Brookes, Baltimore Naidich T.P., Maravilla D. & McLone D.G. (1986) Radiology of the Chiari malformation. In Spina Bifida: A Multidisciplinary Approach, eds R. L. McLaurin, S. Oppenheimer, L. Dias & W. E. Kaplan. Praeger, New York, pp. 164-173 Sand P.L., Taylor N., Hill M., Kosky N. & Rawlings M. (1974) Hand function in children with myelomingocele. American Journal of Occupational Therapy 28(2), 87-90 Sand P.L., Taylor N., Rawlings M. & Chitnis S., (1973) Performance of children with spina biflda manifesta on the Frostig developmental test of visual perception. Perceptual and Motor Skills 37, 539-546 Schneider J. W. (1985) Spina bifida. In Neurological Rehabilitation vol. Ill, ed. D. A. Umphred. C. V. Mosby, St Louis, pp. 289-314
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Schwartz E. (1974) Characteristics of speech and language development in children with myelomeningocele and hydrocephalus. Journal of Speech and Hearing Disorders 39, 465468 Sherman R.G., Berling B.S. & Oppenheimer S. (1985) Increasing community independence for adolescents with spina bifida. Adolescence 20(77). 1-13 Sousa J . C . Gordon L.H. & Shurtleff D.B. (1976) Assessing the development of daily living skills in patients with spina bifida. Developmental Medicine and Child Neurology 18(6. 37). 134142 Spain B. (1974) Verbal and performance ability in pre-school children with spina bifida. Developmental Medicine and Child Neurology 16. 773-780 Stark G.D. (1977) Spina Bifida: Problems and Management. Blackwell Scientific. Boston. Stephens S. (1982) Learning difficulties and children born with neural tube defect. Spina Bifida Therapy 4(2). 63-76 Strax T.E. (1976) Adolescence: a period of stress, the search for an identity. In Birth Defects. Original Article Series, eds D. Bergma & A. Pulver. A. R. Liss. New York Tew B. & Laurence K.M. (1975) The effects of hydrocephalus on intelligence, visual perception and school attainment. Development Medicine and Child Neurology 17(35). 129-134 Turner A, (1985) Hand function in children with myelomeningocele. Journal of Bone and Joint Surgery 67-B(2). 268-272 Walsh M. (1988) Chiari can cause feeding problems. Insights into spina bifida. Newsletter of the Spina Bifida Association of America, March/April Williamson G. (ed.) (1987) Children with Spina Bifida: Early Intervention and Preschool Programming. Brookes. Baltimore Wolraich M, (1983) The Needs of Children With Spina Bifida: A Comprehensive Review. University of Iowa. Iowa City Zeiner H.K. & Prigatano G.P. (1982) Information processing deficits in hydrocephalic and letter reversal children. Neuropsychotogy 20(4). 483-492
