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Adult IssuesAvailable Topics: January 2005 - March 2005 Research Reports Table of Contents
January 2005 - March 2005 This excellent review article focuses on urological and sexual issues, but also surveys general aspects of spina bifida from birth to young adulthood. Jackson AB, Sipski ML. Reproductive issues for women with spina bifida. J Spinal Cord Med. 2005;28(2):81-91. Review. PMID: 15889694 [PubMed - indexed for MEDLINE] The purpose of this article was to review previously published reports on the reproductive issues facing female adolescents and women and to ascertain the current knowledge so that future research needs can be established. This study concluded that little research has examined the reproductive issues of women with spina bifida and women with other neurologic disabilities. Future controlled prospective research studies are needed to examine issues related to puberty and sexual and gynecologic issues throughout the life span and pregnancy in these populations.
April 2005 - June 2005 In this study, 48% of young adults with spina bifida were unsatisfied with their present sex life; 53% expressed a lack of confidence. (What percent of individuals without spina bifida would be unsatisfied or lacking confidence?) Males with hydrocephalus were least likely to have had sexual encounters. Having an IQ > 70 and being continent both predicted better sexual functioning. Females have more sexual encounters; however, sexual abuse or being taken-advantage-of sexually is more likely to occur in women who have spina bifida than in the general population. Lingman G. Management of pregnancy and labour in cases diagnosed with major fetal malformation. Curr Opin Obstet Gynecol. 2005 Apr;17(2):143-6. Recent studies confirm previous findings that caesarean section does not improve the outcome in cases of fetal gastroschisis or spina bifida. Recent research has demonstrated the development of severe fetal malformations. New studies have shown the positive effects of fetal supervision in cases of gastroschisis. Furthermore, caesarean section is not beneficial compared with vaginal delivery in such cases. Fetal surgery is still undergoing rapid development and has the potential to improve outcome in the near future.
April 2006 - June 2006 This study of adults with spinal cord injuries and spina bifida from Sweden found that individuals with spinal cord injury were somewhat more likely to be working than were adults with spina bifida. As might be expected, adults with spina bifida who had lower level lesions and higher cognitive functioning were more likely to be employed.
January 2007 - June 2007 Abstract: This study concerns life satisfaction and its determinants in Dutch young adults with spina bifida (SB). Data on life satisfaction (Life Satisfaction Questionnaire [LiSat-9]) were related to hydrocephalus, lesion level, disabilities, and demographic variables. In total, 179 young adults with SB participated (41% male, age range 16-25y; 79% SB aperta, 67% hydrocephalus [HC], 39% wheelchair-dependent). Most were satisfied with their life as a whole (24% dissatisfied). No difference was found from a population reference group (28% dissatisfied). Highest proportions of dissatisfaction were found for financial situation (44%), partnership relations (49%), and sex life (55%). Least dissatisfaction was found for contact with friends (17%) and families (15%). Young adults with SB and HC were more satisfied with their financial situation and family life but were less satisfied with self-care ability and partnership relations than those without HC and the reference group. However, except for self-care ability, relationships between life satisfaction and having SB were weak. In conclusion, self-care ability and partnership relations were rated least favourable and may need more attention from care providers. Overall, SB does not seem to be an important determinant of life satisfaction Verhoef M, Post MW, Barf HA, van Asbeck FW, Gooskens RH, Prevo AJ. Perceived health in young adults with spina bifida. Dev.Med.Child Neurol. 2007 Mar;49(3):192-7. Abstract: The aims of this study were to compare the perceived health of young adults with spina bifida with a population without disability, and to determine the effect of the disease characteristics and resulting impairments on perceived health. This cross-sectional study is part of the Adolescents with Spina Bifida in the Netherlands study. Data were collected by physical examination and a questionnaire. In total, 179 patients (age range 16-25y) participated in the study and perceived health data were completed for 164 participants (92 females, 72 males; mean age 20y 7mo [SD 2y 9mo]). Twenty-six participants had spina bifida occulta and 138 had spina bifida aperta, of whom 115 also had hydrocephalus. Perceived health was measured with the Medical Outcome Study 36-item Short-form Health Survey (SF-36), a generic health status measure. SF-36 scores of young adults with spina bifida were below those of an age-matched population group for six of the eight domains. This difference was largest for the physical functioning domain. Although these differences were statistically significant they were small. Findings for the emotional health domains (vitality, mental health, role problems due to emotional problems) did not differ at all from the population group [These two articles confirm that adults with spina bifida are dissatisfied with their sex life (55%), relationships with partners (49%), and their financial situation (44%). In terms of their "overall satisfaction," they were no different from people without spina bifida. However, all studies on "overall satisfaction with life" need to be viewed with caution. People do not know how happy or satisfied they are with life in the way they know their height or telephone number. The answers to global life satisfaction questions are constructed only when asked, and are, therefore, susceptible to bias. We do not continuously think about our circumstances, whether positive or negative. Instead, we spend most of our time attending to and drawing pleasure or displeasure from experiences such as having breakfast or watching television. The real question that these studies ask is, 'How can we improve the health and well-being of adults with spina bifida?' One way is to address specific aspects of their lives, like relationships.] Dennis M, Jewell D, Drake J, Misakyan T, Spiegler B, Hetherington R, Gentili F, Barnes M. Prospective, declarative, and nondeclarative memory in young adults with spina bifida. J.Int.Neuropsychol.Soc. 2007 Mar;13(2):312-23. Abstract: The consequences of congenital brain disorders for adult cognitive function are poorly understood. We studied different forms of memory in 29 young adults with spina bifida meningomyelocele (SBM), a common and severely disabling neural tube defect. Nondeclarative and semantic memory functions were intact. Working memory was intact with low maintenance and manipulation requirements, but impaired on tasks demanding high information maintenance or manipulation load. Prospective memory for intentions to be executed in the future was impaired. Immediate and delayed episodic memory were poor. Memory deficits were exacerbated by an increased number of lifetime shunt revisions, a marker for unstable hydrocephalus. Memory status was positively correlated with functional independence, an important component of quality of life [This study found that certain types of memory, including those linked with functional independence, are impaired in adults with spina bifida-just as they are in children with spina bifida. Adults with spina bifida should consider using assistive technology (like PDAs) to help with memory and with organization and planning.] Bellin MH, Sawin KJ, Roux G, Buran CF, Brei TJ. The experience of adolescent women living with spina bifida part I: self-concept and family relationships. Rehabil.Nurs. 2007 Mar;32(2):57-67. Abstract: Adolescent women with spina bifida (SB) face unique and diverse challenges. The purpose of this qualitative component of a larger mixed-method study on adaptation was to heighten rehabilitation nurses' understanding of self-concept and family relationships during adolescence. Interviews were conducted with 31 adolescent women and analyzed for themes. The women described a range of experiences, including challenges of typical adolescence, specific concerns about living with SB, school-based stressors, and incidences of teasing and bullying. The overall self-concept was primarily positive, despite the diverse stressors encountered. A significant source of strength was the close relationships with parents, although an undercurrent of tension related to independence was also expressed. Results from this study support the need for rehabilitation nurses to address not only the functional status but also the well-being and psychosocial challenges of adolescent women with SB. Roux G, Sawin KJ, Bellin MH, Buran CF, Brei TJ. The experience of adolescent women living with spina bifida. Part II: Peer relationships. Rehabil.Nurs. 2007 May;32(3):112-9. Abstract: Relationships are much more complex for those with disabilities than for those without disabilities. This study was part of a larger mixed-method investigation that explored comprehensive aspects of adaptation in adolescents with spina bifida (SB). The purpose of this qualitative component of the study was to explore the experiences of peer relationships in 31 adolescent women with SB. The participants were interviewed, and analysis was conducted for common themes. The five major themes and one subtheme were peers without disabilities (subtheme: peers with disabilities), normalization, challenges in peer connectedness, peer connectedness with adults, and romantic connectedness. Whereas some participants voiced close connections with peers, others described prejudices, stereotyping, and limited dating experiences. Results from this study support the need for comprehensive assessment of social relationships in adolescent women with SB and active interventions to address problems identified. Rehabilitation nurses are in a key position to implement social interventions in adolescents and young women with SB [Teenagers with spina bifida must go through the same developmental stages as those without spina bifida. These include finding an identity and developing intimacy (versus becoming isolated). Although having close relationships with parents can help diminish isolation (as documented in the first study), it does not tend to enhance independence. From the time a baby with spina bifida is born, we should be focusing on that child's future and doing everything we can to optimize independence skills. For example, it is very reasonable to ask the parents of a newborn what they envision for the child's future when he or she is a young adult. That helps them focus on the child's functional abilities and not just on his or her disabilities and impairments.] Olsson I, Dahl M, Mattsson S, Wendelius M, Astrom E, Westbom L. Medical problems in adolescents with myelomeningocele (MMC): an inventory of the Swedish MMC population born during 1986-1989. Acta Paediatr. 2007 Mar;96(3):446-9. Abstract: AIM: To describe the prevalence of myelomeningocele (MMC) and the medical needs of adolescents, 15-18 years, with MMC in Sweden , at a time when they are on the threshold of adulthood, leaving paediatrics. METHODS: In a retrospective study, we identified all adolescents with MMC, born during 1986-1989 and living in Sweden on July 1, 2004. An inventory was agreed upon with questions concerning their medical problems and need for medical care. RESULTS: There were 175 persons 15-18 years of age, born with MMC or lipoMMC (prevalence 3.8 per 10,000). Hydrocephalus was seen in 86%, 31% had been operated because of tethered cord syndrome, and 6% for Chiari malformation symptoms. The majority had motor impairments. Clean intermittent catheterisation for bladder emptying was used by 85%, and 59% used enemas on a regular basis because of the neurogenic bowel dysfunction. Renal dysfunction was seen in 1.7% of the adolescents. CONCLUSION: Lifelong follow-up by many specialists, among others neurologists and neurosurgeons, urotherapists and urologists, orthopaedic surgeons and orthotists, is necessary for individuals with MMC. The complex medical situation, often in combination with cognitive difficulties, makes it necessary to coordinate medical services for this increasing group of adults with multiple impairments [This study documents that adolescents with spina bifida who are 15 to 18 years of age have involvement of multiple body systems. This implies that they require long-term follow-up by urologists, neurosurgeons, orthopaedists, therapists, orthotists, and others. Unfortunately, in many communities, individuals who are expert in those fields and are willing and able to take care of adults are hard to find. In addition, finding programs that can coordinate such complex care is even more difficult.]
June 2007 - January 2008 Abstract: OBJECTIVES: To address the lack of synthesis regarding the factors, processes, and outcomes specific to the transition from child-centered to adult-centered health care for people with cerebral palsy (CP) and spina bifida (SB); more specifically, to identify barriers, to outline key elements, to review empirical studies, and to make clinical and research recommendations. DATA SOURCES: We searched Medline and CINAHL databases from 1990 to 2006 using the key words: transition, health care transition, pediatric health care, adult health care, health care access, health care use, chronic illness, special health care needs, and physical disability. The resulting studies were reviewed with a specific focus on clinical transition for persons with CP and SB, and were supplemented with key information from other diagnostic groups. STUDY SELECTION: All studies meeting the inclusion criteria were included. DATA EXTRACTION: Each article classified according to 5 criteria: methodology, diagnostic group, country of study, age group, and sample size. DATA SYNTHESIS: We identified 149 articles: 54 discussion, 21 case series, 28 database or register, 25 qualitative, and 34 survey articles (some included multiple methods). We identified 5 key elements that support a positive transition to adult-centered health care: preparation, flexible timing, care coordination, transition clinic visits, and interested adult-centered health care providers. There was, however, limited empirical evidence to support the impact of these elements. CONCLUSIONS: This review summarizes key factors that must be considered to support this critical clinical transition and sets the foundation for future research. It is time to apply prospective study designs to evaluate transition interventions and determine long-term health outcomes. [In this review, published articles about the health care transition (from adolescence to adulthood) of people with CP and spina bifida are reviewed. While many pediatric programs provide comprehensive care, continuity, coordination across disciplines, and family engagement, adult-oriented programs appear to be fragmented and unprepared for young adults with spina bifida. The goals of transition should be to optimize activity, encourage participation in society, and enhance quality of life. To do this young people need to develop skills, self-confidence, and self-management, as well as problem-solving and decision-making skills and experiences. Support for prospective research that examines programs designed to improve transition is encouraged.] Bong GW, Rovner ES. Sexual health in adult men with spina bifida. ScientificWorldJournal. 2007;7:1466-9. Abstract: Medical and surgical advances in the treatment of spina bifida (SB) have resulted in increasing numbers of patients reaching adulthood. As such, issues related to sexual maturity are being investigated to offer optimal healthcare to men with spina bifida. This report constitutes a review of the current literature relating to adults with spina bifida and issues of sexuality, erectile dysfunction and fertility. In general, adult males with spina bifida have normal sexual desires and an interest in addressing these issues with healthcare providers. Sexual education and access to intimacy are delayed compared to the general population. 75% of men achieve erections, but maintaining erections is a problem and some may be merely reflexive in nature. Many of these men show marked improvement with sildenafil. In SB erectile dysfunction and infertility are related to the level of neurological lesion with the best performance status in those with sacral lesions and intact reflexes. Men with lesions higher than T10 are at risk for azoospermia. There is an increased risk of neural tube defects in the children of men with spina bifida, but the current incidence with modern folic acid therapy is unknown. As the number of males with spina bifida reaching sexual maturity increases, further investigation into sexuality, sex education, intimacy, and treatments for erectile dysfunction and infertility will be needed. [Men who have meningomyelocele often have several problems with sexual function including an inability to achieve and maintain an erection and problems with ejaculation. They also do not receive adequate sex education. This article reviews the published literature on sexual function in men. One of their findings is that sildenafil (Viagra ® ) can enhance sexual function. Further research on ways of optimizing sexual functioning for these men is needed.] Cardenas DD, Topolski TD, White CJ, McLaughlin JF, Walker WO. Sexual functioning in adolescents and young adults with spina bifida. Arch Phys Med Rehabil. 2008 Jan;89(1):31-5. Abstract: OBJECTIVE: To assess sexual education and sexual functioning in adolescents and young adults with spina bifida. DESIGN: Survey, inception cohort. SETTING: The community. PARTICIPANTS: A cohort of adolescents and young adults (N=121; range, 15-35 y; 58% women) enrolled in a longitudinal pediatric database. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Questions on sexual function, reproductive function, bladder and bowel continence, the Perceived Quality of Life Scale, and the Satisfaction With Life Scale. RESULTS: Almost all adolescents and young adults with spina bifida in our study received sexual education at school, less at home, or by physicians. Twenty-five percent of men and 68% of women were informed about reproductive function by their physicians. Participants who reported that they smoked were 10 times more likely to report being sexually active and women were 2.3 times more likely to be sexually active than men. Hydrocephalus was a significant predictor of sexual activity among women but not men. Participants with urinary incontinence were less likely to be sexually active. Women without hydrocephalus were significantly more satisfied with life than women with hydrocephalus. CONCLUSIONS: Adolescents and young adults with spina bifida in this sample were only slightly satisfied with life and sexual activity was only associated with life satisfaction among women. Dissatisfaction with life often leads to engagement in health-risk behaviors, which may, in part, account for the association between sexual activity and smoking behavior observed in these data. Further studies of health risk behaviors among youth with spina bifida are warranted and interventions aimed at reducing health risk behaviors among adolescents and young adults should specifically include spina bifida as a target group. Lassmann J, Garibay GF, Melchionni JB, Pasquariello PS, Jr., Snyder HM, III. Sexual function in adult patients with spina bifida and its impact on quality of life. J.Urol. 2007 Oct;178(4 Pt 2):1611-4. Abstract: PURPOSE: We evaluated sexual function in adult patients with spina bifida and its impact on quality of life. MATERIALS AND METHODS: Between March 2005 and May 2006, 76 adult patients with spina bifida, including 34 women and 42 men, with a mean age of 24.4 years (range 18 to 37) were seen for followup at our institution. Following institutional review board approval data were collected from medical records and validated questionnaires were completed, including the Watts Sexual Function Questionnaire to assess sexual function and SF-36 to assess quality of life. RESULTS: Of the 76 patients 18 (24%), including 9 women and 9 men, achieved sexual intercourse at least once in the last 2 months. There was no difference regarding gender distribution and mean age +/- SD in sexually active vs not sexually active patients (25.8 +/- 4.2 vs 24 +/- 4.5 years, p = 0.13). All levels (thoracic to sacral) of myelomeningocele were seen in the 2 groups with significant higher lesions of neurological impairment in not sexually active than in sexually active patients. No difference was seen in relation to ambulatory status and urinary incontinence. Overall total Watts Sexual Function Questionnaire scores in sexually active patients were similar in men and women. When comparing the 4 domain scores on desire, arousal, orgasm and satisfaction, women scored similar to men. Only 2 men tried to achieve paternity but failed and 1 woman gave birth. When evaluating SF-36 for quality of life, there was no significant difference in physical health (42.4 +/- 11.9 vs 38.7 +/- 7.2, p = 0.11) and mental health (54.1 +/- 11.3 vs 58.6 +/- 10.7, p = 0.12) subscores in sexually active vs not sexually active patients. CONCLUSIONS: In our cohort 24% of adult patients with spina bifida were sexually active. Sexual activity was not related to gender, level of urinary incontinence or extent of physical disability but it was more likely in patients with more caudal levels of neurological impairment. Sexual function seems not to affect health related quality of life in these patients. [These two studies examine the sexual activity of youth and young adults with spina bifida. Both found that the rate of sexual activity was lower than reported for people of similar ages but without spina bifida. Only in the first study was sexual activity associated with life satisfaction, and then only among women. Being incontinent was negatively correlated with sexual function in the first study but not in the second. In the first study women reported that they were more sexually active than men. This difference was not found in the second study (which had a smaller sample size.) Although both studies examined similar factors, some of their conclusions differ. This may be related to differences in their samples.]
September 2009 Abstract: OBJECT: Although postsurgical neurological outcomes in patients with tethered cord syndrome (TCS) are well known, the rate and development of neurological improvement after first-time tethered cord release is incompletely understood. The authors reviewed their institutional experience with the surgical management of adult TCS to assess the time course of symptomatic improvement, and to identify the patient subgroups most likely to experience improvement of motor symptoms. METHODS: The authors retrospectively reviewed 29 consecutive cases of first-time adult tethered cord release. Clinical symptoms of pain and motor and urinary dysfunction were evaluated at 1 and 3 months after surgery, and then every 6 months thereafter. Rates of improvement in pain and motor or urinary dysfunction over time were identified, and presenting factors associated with improvement of motor symptoms were assessed using a multivariate survival analysis (Cox model). RESULTS: The mean patient age was 38 +/- 13 years. The causes of TCS included lipomyelomeningocele in 3 patients (10%), tight filum in 3 (10%), lumbosacral lipoma in 4 (14%), intradural tumor in 3 (10%), previous lumbosacral surgery in 2 (7%), and previous repair of myelomeningocele in 14 (48%). The mean +/- SD duration of symptoms before presentation was 5 +/- 7 months. Clinical presentation included diffuse pain/parasthesias in both lower extremities in 13 patients (45%), or perineal distribution in 18 (62%), lower extremity weakness in 17 (59%), gait difficulties in 17 (59%), and bladder dysfunction in 14 (48%). Laminectomy was performed in a mean of 2.5 +/- 0.7 levels per patient, and 9 patients (30%) received duraplasty. At 18 months postoperatively, 47% of patients had improved urinary symptoms, 69% had improved lower extremity weakness and gait, and 79% had decreased painful dysesthesias. Median time to symptomatic improvement was least for pain (1 month), then motor (2.3 months), and then urinary symptoms (4.3 months; p = 0.04). In patients demonstrating improvement, 96% improved within 6 months of surgery. Only 4% improved beyond 1-year postoperatively. In a multivariate analysis, the authors found that patients who presented with asymmetrical lower extremity weakness (p = 0.0021, hazard ratio 5.7) or lower extremity hyperreflexia (p = 0.037, hazard ratio = 4.1) were most likely to experience improvement in motor symptoms. CONCLUSIONS: In the authors' experience, pain and motor and urinary dysfunction improve postoperatively in the majority of patients. The rate of symptomatic improvement was greatest for pain resolution, followed by motor, and then urinary improvement. Patients who experienced improvement in any symptom had done so by 6 months after tethered cord release. Patients with asymmetrical motor symptoms or lower extremity hyperreflexia at presentation were most likely to experience improvements in motor symptoms. These findings may help guide patient education and surgical decision-making. [In this study of tethered spinal cord repair, which included 14 patients with repaired spina bifida, the authors found that pain, weakness and urinary dysfunction improved in more than half the individuals who had surgery. If improvement did occur, 96% improved within 6 months of the surgery. This study is consistent with findings from tethered spinal cord repair in children.]
January 2010 to January 2011 Dicianno BE, Wilson R. Hospitalizations of adults with spina bifida and congenital spinal cord anomalies. Arch Phys Med Rehabil 2010 April;91(4):529-35. Abstract: OBJECTIVE: To examine hospital admission records from a large cohort of persons with spina bifida (SB) with a variety of insurers to provide descriptive detail about adult hospital use for persons with SB and associated disorders in terms of primary diagnosis for hospitalization, age, sex, payer source, lengths of stay, and total charges. DESIGN: Retrospective secondary data analysis from the Nationwide Inpatient Sample (NIS) from the Healthcare Cost and Utilization Project for 2004 and 2005 of hospitalizations for adults with SB or associated spinal cord anomalies. SETTING: Records from U.S. inpatient hospital admissions. PARTICIPANTS: Persons with SB age 18 years and older. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Diagnoses associated with hospitalizations and death. RESULTS: The most common primary diagnosis for hospitalization was urinary tract infection, followed by complications from devices/grafts/implants and skin wounds. Sepsis accounted for the most deaths. Approximately one third of hospitalizations were for primary diagnoses of potentially preventable conditions. Hospitalizations associated with a primary diagnosis of a potentially preventable condition occurred most often in those less than 51 years of age and in rural or urban nonteaching hospitals. CONCLUSIONS: Reducing the number of secondary medical conditions with proactive and preventative approaches to health care could reduce the morbidity, mortality, and cost for health care for this group. [Adults with spina bifida continue getting secondary conditions that may lead to hospitalization. In this study based on a national database, the most common reason for hospitalization was urinary tract infection (UTI), followed by complications from devices/grafts/implants and skin wounds. Sepsis (blood infection–e.g., from an infected decubitus ulcer or UTI) led to the most deaths. One third of hospitalizations were for potentially preventable conditions. This study confirms the importance of preventive care for adults with spina bifida. However, for many adults accessing adequate care is difficult since the multidisciplinary clinics that had been available to them when they were younger do not exist. Many adults continue to need advocates to insure that they are getting adequate care.] Jenkinson MD, Campbell S, Hayhurst C, Clark S, Kandasamy J, Lee MK, Flynn A, Murphy P, Mallucci CL. Cognitive and functional outcome in spina bifida-Chiari II malformation. Childs Nerv Syst 2010 December 31. Abstract: PURPOSE: The long-term outcome in spina bifida-Chiari II-hydrocephalus complex is poorly understood. Traditional neurosurgical outcome measures are crude. Neuropsychological testing is increasingly important in outcome assessment. We investigated the health, disability, lifestyle and cognitive function in adults who had myelomeningocoele closure at birth. METHODS: Adult patients under routine follow-up were assessed in a joint neurosurgery/neuropsychology clinic. Patients completed lifestyle questionnaires, the hydrocephalus outcome questionnaire (HOQ) and underwent cognitive testing. Clinical variables including number of shunt revisions, shunt infection and surgical decompression of foramen magnum, which may influence outcome, were investigated. RESULTS: Twenty-one adults with a median age of 35 years were investigated. All had treated hydrocephalus, and eight had foramen magnum decompression for headache or progressive brainstem symptoms with stabilisation of symptoms in seven and improvement in one. Only eight patients were living independently, five were in paid employment and five work voluntarily. HOQ scores for cognitive function were lower (0.56 +/- 0.20; mean +/- standard deviation (SD)) than those for physical (0.64 +/- 0.15) and social-emotional (0.65 +/- 0.17) health. Cognitive function varied across the cohort with attention most severely affected (73.9 +/- 17.0; mean +/- SD). Repeated episodes of shunt malfunction or foramen magnum decompression were not associated with a worse cognitive function. CONCLUSIONS: Despite intervention in childhood and adequate cerebrospinal fluid diversion the prognosis for independent living into adulthood remains poor. All patients have elements of cognitive impairment. Structural brain abnormalities may be more important determinants of cognitive outcome than shunt malfunction. [In this study 38% of adults with spina bifida and Chiari malformation were living independently; all had some evidence of cognitive problems. The congenital abnormalities of the brain found in spina bifida as well as shunted hydrocephalus are associated with cognitive difficulties.] Roach JW, Short BF, Saltzman HM. Adult Consequences of Spina Bifida: A Cohort Study. Clin Orthop Relat Res 2010 September 28. Abstract: BACKGROUND: Patients with spina bifida potentially experience social isolation with limited employment opportunities and restriction of independent living, neurologic and intellectual impairment, and orthopaedic and urologic consequences of their condition. However, the degree of disability as these individuals age into adulthood has not been completely delineated. QUESTIONS/PURPOSE: In adults with spina bifida we determined the cause of death for those who had died, IQ, level of education, work history, independent living status, physical disabilities, and urologic status; we then identified surgical procedures that led to better adult function. METHODS: We retrospectively reviewed 84 individuals with myelomeningocele, examining social, cognitive, and physical disabilities. The minimum age at followup was 20 years (mean, 31 years; range, 20-64 years). RESULTS: Forty-two percent had normal IQs. Seventy percent never needed a neurosurgical shunt and those without shunts had higher IQs. Forty-four percent had regular education and 8% achieved college degrees. Fifty-six percent were unemployed. Thirty percent lived independently. Twenty-three percent were either married or divorced with nine normal offspring. Eighty-five percent dressed themselves, 65% shopped independently, 54% drove. Thirty-one percent were at the thoracic neurologic level (all used wheelchairs), 12% were at L1-L3 (all used a wheelchair except one), 33% were at L4-L5 (78% used a wheelchair at least part-time), and 24% were at S1 and below (all walked). Fifty-four percent experienced decubiti and as a consequence, four required major extremity amputations. Spinal fusions protected sitting balance, but hip surgery did not produce congruent hips and occasionally resulted in debilitating stiffness. Pressure sores resulted in partial foot amputations despite plantigrade feet. CONCLUSIONS: Our observations confirm the consequences associated with the physical and cognitive disabilities of patients with spina bifida. Many of these consequences can likely be mitigated by modern multidisciplinary care and special education to increase independence. [This study describes a cohort of adults with spina bifida. This is a very select group, considering that 70% never had a ventricular shunt. This is critical because the most important single factor in determining outcomes in adults like independence and cognition is the presence of a shunt. From an orthopaedic standpoint, having had hip surgery did not have many positive effects, and often was associated with stiff hips. Spinal fusion for kyphoscoliosis, on the other hand, helped with sitting balance.] |