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Myelomeningocele: neglected aspects

Myelomeningocele: neglected aspects Pediatr Nephrol (2008) 23:1223–1231 DOI 10.1007/s00467-007-0663-3 EDUCATIONAL FEATURE Christopher R. J. Woodhouse Received: 6 June 2007 /Revised: 28 August 2007 /Accepted: 21 September 2007 / Published online: 17 January 2008 IPNA 2007 . . Abstract The commonest cause of neurogenic bladder in Keywords Neurogenic bladder Myelomeningocele . . children is myelomeningocele. Survival of children is much Long-term outcome Paediatric nephrology Education improved in the Western world, but by 35 years old, about 50% will have died. In adults, the commonest causes of death are lung and heart diseases. All physical aspects deteriorate Introduction with age, especially in those with thoracic lesions. Those who walk in childhood have a 20–50% chance of becoming Children who are born with myelomeningocele grow up to wheelchair dependent as adults. Immobility, poor respiratory become adults–a truth which should be self-evident but reserve, obesity, latex allergy and worsening kyphoscoliosis often seems to be overlooked by doctors and by some contribute to the increased risks of surgery. It is essential that health care systems. In contrast, it is a preoccupation with safe and manageable urine drainage is established in their parents. Passage to adult life brings with it the childhood: the bladder never improves with time, and surgical difficulties of adolescence that are common to all: the reconstruction becomes progressively more difficult. Inde- beginning of sexual interest but a continuing deterioration pendence in adult life will only be possible with intense in the body as a whole. Sadly, nothing in spina bifida gets preparation in childhood. Children must be allowed to join in better with age. Preparation for adulthood is frequently with family chores and events. Education, both academic and neglected in childhood, and the consequences of growing practical, must be encouraged. Skills such as driving, up with spina bifida are poorly researched. shopping and birth control must be taught. However, even with the best support, less than 40% will have gainful employment. Children who are continent and have lesions Survival below L2 are likely to have normal sexual function. Sexual activity in adolescents, especially in those with hydrocepha- For all babies born with open spina bifida, the prognosis is lus, is limited (but not absent). However, by adult life, about poor. In underdeveloped countries, congenital anomalies, two thirds will have established a regular partnership. All especially spina bifida, account for nearly 50% of infant females and those males who are naturally potent are likely to deaths, and the rate is inversely correlated with the per be fertile. There is a high risk of neural tube defects in their capita gross domestic product [1]. In the UK, only 60% of offspring unless the female partner takes prophylactic folic such children have survived into adulthood (Fig. 1)[2]. At acid for 3 months before pregnancy and for first trimester. all ages, renal failure is the commonest cause of death. In children, the risk of renal failure is strongly related to the sensory level (which may not be the same as the anatomical level in the spine or the level suggested by X-ray). Renal C. R. J. Woodhouse (*) failure is rare with sensory levels at or below L4 and Adolescent Urology, University College, common at or above T10 [3]. However, renal failure can 235 Euston Road, occur even with apparently minor neural tube defects, such London NW1 2PR, UK e-mail: [email protected] as occult spinal dysraphism. In a group of 55 such cases, 1224 Pediatr Nephrol (2008) 23:1223–1231 100% continue to walk, there may be damage to insensate joints (Charcot arthropathy), which has a prevalence of one in 100 80% up to the age of 42 years [9]. The percentage of children 60% who start ambulation (with or without aids) and the 40% percentage who continue to walk up to 9 years 1 month, by spinal level, is shown in Fig. 3 [10]. Loss of mobility 20% encourages obesity, which, combined with the collapsed 0% spine, makes much of the lower half of the body invisible to 1 7 14 21 28 35 the patient. Attempts to impose weight loss in spina bifida Fig. 1 Survival curve for children born with spina bifida adolescents can lead to eating disorders just as it can in others [11]. Respiratory reserve worsens, making mobility more difficult. only 24 patients (43%) had urological symptoms at presentation, and yet all eventually became incontinent, and eight developed renal failure [4]. Surgery in adulthood In an unselected series of 695 adults in the UK, 56 were known to have died before their expected time. In the 30 The decision to undertake major surgery on an adult with patients whose cause of death could be determined, renal spina bifida should not be taken lightly, and preparation failure accounted for one third. Thirteen died of cardiac or must be meticulous. respiratory disease, three committed suicide and one died of If there is to be any significant period of immobility, it cancer (Fig. 2)[5]. There is an increased risk of atheroscle- may be necessary to have a water bed and nursing by rosis in patients with spina bifida, even in the absence of appropriately trained individuals. Even prior to surgery, obesity [6]. some patients are limited in the positions in which they can lie; after surgery, the possible positions may be even more limited. There is anecdotal evidence that there is an Mobility increased risk of fracture with minimal trauma in patients with spina bifida. Great care must be taken in moving As the child grows, there is a natural tendency for physical patients in the hospital. Special attention must be given to anomalies to deteriorate. The spinal deformity becomes the position of metal work in the spine, parts of which may more pronounced. Those who could just walk tend to relapse come dangerously close to the surface of the skin. into a wheelchair. This is illustrated in Fig. 3: those with Latex allergy is common in patients with spina bifida, high lesions, who also have a considerably delayed age of the incidence increasing with age (see below). If there is ambulation, have a 20–50% chance of loosing the ability to any suggestion of such a problem, surgery must be walk by the age of 10 years. Furthermore, adolescents with performed in a strictly latex-free environment. spina bifida spend about a half the time in dynamic physical With age, respiratory reserve declines. Presumably, activity as do their normal contemporaries [7]. increasing kyphoscoliosis is at least a part of the cause. In Unfortunately, just being able to walk does not mean that the author’s own series of adult patients presenting for independence is assured. Even those with sacral lesions who urinary tract reconstruction, 10% were thought to be unfit have good muscle strength in the hips may require help with for anaesthesia on the basis of lung-function tests [12]. mobility and bowel and bladder care [8]. For those who Cardiopulmonary exercise testing (CPX) is rapidly becom- Fig. 2 Histogram to show the 50% causes of death in children and adults born with spina bifida. 40% CNS Central nervous system 30% Children 20% Adults 10% 0% CNS Renal Heart/lung Suicide Other Pediatr Nephrol (2008) 23:1223–1231 1225 Fig. 3 Percentage of children 100% who start ambulation (with or without aids) and percentage 80% who continue to walk up to 9 years 1 month, by spinal level 60% Started walking [10] Still walking 40% 20% 0% Thoracic L1/2 L3 L4/5 Sacral Latex allergy ing a standard assessment of fitness for major surgery [13]. CPX requires adaptation for use in patients with spina bifida, as it normally is performed on a static bicycle. We Aetiology are presently investigating the use of upper-limb pedals for CPX testing, especially to measure the anaerobic threshold. Paediatric urologists with a large practice of spina bifida Worsening kyphoscoliosis also makes access to the patients are well aware of the problems of latex allergy. abdominal cavity more difficult. Patients may be unable Sophisticated strategies have been established to identify to lie flat on the operating table. The distance between the patients at risk and to avoid the serious problems of costal margin and the pubis decreases. Rotational deformity anaphylaxis, especially under anaesthetic. In the United of the spine results in a changing relationship between States, the Shriners Myelodysplasia Hospitals are entirely abdomen and chest so that when lying flat on the back at latex free. Adult urologists are much less aware of this chest level, the abdomen may be rotated to the side. The potentially fatal problem. Although there is a general kidneys may be particularly inaccessible. recognition that spina bifida children are particularly at risk, Neither bowel nor bladder function improves at puberty, the reasons are unclear. It is tempting to think that the though bladder outflow resistance increases (which may increased number of surgical procedures is to blame. Ells- bring more danger than advantage). Because of the worth et al. found an overall incidence of latex allergy of 60% difficulties with surgery, it is particularly important to in 50 spina bifida children. The mean number of operations in establish stable systems in childhood. It should be positive patients was 9.5 compared with 6.7 in negative remembered that the lower half of the abdomen may children (P=0.03). There was no difference in the age of the become invisible to the patient, particularly in women, so children, the number of years that they had been on clean that stomata and catheterization sites must be put above the intermittent catheterisation or the number of abdominal umbilicus (Fig. 4). operations that they had undergone [14]. Fig. 4 Clinical photograph to illustrate the difficulty that adults with spina bifida have in visualizing the lower half of the abdomen Correct site of stoma within the girl’s field of view Actual site which she cannot see 1226 Pediatr Nephrol (2008) 23:1223–1231 However, there are probably other factors to consider. operations have been uneventful in a latex-free operating The presence of latex-specific immunoglobulin (Ig)G was theatre (unpublished data). found in 25% of a group of European spina bifida patients aged between 2 and 40 years old. Multivariate analysis showed that atopy, especially to pears and kiwi fruit and a Preparation for adulthood history of five or more operations were significantly and independently associated with latex allergy [15]. On the Childhood is a preparation for adulthood. This is true of other hand, in a controlled study of the incidence of latex children with spina bifida as it is for those who are more allergy, it was found that that 26% of spina bifida patients normal. It should come as no surprise to find that if those with had symptomless latex allergy compared with 5% of spina bifida are inadequately prepared, they will not become patients from an ear, nose and throat clinic, even though independent. As with everything else in this condition, it is both groups had had the same number of operations (mean very hard to do. Expectations must be appropriate and special of 1.65 and 1.95, respectively). There were no positive tests needs met, but without such work in childhood, dependence in a control group of urological patients who had had no will continue into adult life. Few countries have adequate operations [16]. Latex allergy has also been found in 1.1% arrangements for young dependent adults, and much of the of patients on regular haemodialysis [17]. burden will, therefore, continue to fall on the family. It is seldom appropriate to wait for symptoms to “improve with Presentation age”. Anything that is not sorted out in childhood will be more difficult to manage in adulthood. About one third of spina bifida patients will have a history Autonomy of the disabled can be considerably improved of allergic reactions to latex products [14]. The level of by an active but realistic programme of education [19]. awareness of the problem amongst spina bifida children and Parents who have a positive and hopeful attitude are able to their families is low, and so the symptoms should be improve their adolescent’s quality of life by about 25% over specifically sought. In some cases, the association is that which would be predicted for the disability at birth obvious, but in others it may be obscure, such as itchy [20]. Unfortunately, even with the highest levels of eyes while using washing-up gloves or genital reactions to expectation and ambition, participation in the full range of condoms. In occasional patients, there is acute anaphylaxis adolescent activities or household chores is uncommon under anaesthetic. All patients once identified should be [21]. Although incontinence is not always a barrier to a made aware of the risks and should wear a medic-alert fulfilled life, those who are continent have an improved bracelet. During medical procedures, latex use should be view of self worth. In girls, social acceptance and views of reduced to a minimum. There are important warning signs global self-worth and in boys, scholastic competence, social that may alert the clinician to the problem in a previously acceptance, physical appearance and behaviour are all unsuspected case. In a group of five children (from 17) who improved by continence [22]. were undergoing transurethral bladder stimulation and In addition, throughout childhood, a whole set of urodynamics, all had coughing or sneezing for several emotional and social problems emerge, though they cannot minutes before bronchospasm and generalised allergic totally be separated from the physical. Patients become reactions developed [18]. sexually interested without necessarily having the means to indulge or control their desires. Maturity brings new Management interests. For those who are reasonably intelligent, there is a desire to have normal friends, to go out to the cinema or The most important part of management is identification of club and to socialise with a peer group. Many patients are patients at risk and prophylaxis. As the incidence is so high able to work, some in very demanding areas including in spina bifida patients, a specific enquiry should be made medicine, though special training or work facilities may be for symptoms in all patients. Minor reactions can be treated needed. For the mentally retarded, appropriately stimulating with bronchodilators, antihistamines and, occasionally, occupations must be found. All of this requires help. At the steroids. It should be remembered that minor reactions very least, there has to be an infrastructure of sympathetic may be the forerunner of major anaphylaxis. All who have friends who will take a wheelchair-bound person to places a suggestive history or unexplained hypotensive episodes that often are not too well equipped to accommodate them. under anaesthetic should undergo skin testing and mea- Social research shows that buildings and transport systems surement of latex-specific IgE. A latex-free medical are poorly adapted to the needs of the handicapped. environment should be created for patients found to be at All children who are born with congenital anomalies risk. In all of four patients on the author’s service who have a desire to be normal and to be treated as such. It is presented with anaphylaxis under anaesthetic, further essential to encourage children with spina bifida to look Pediatr Nephrol (2008) 23:1223–1231 1227 after themselves and to take part in normal family life right sexual sensations, as are most with urinary continence. Only from the start. about 20% of those with higher levels or with urinary incontinence have normal sexual function [27]. Some women appear to have powerful contractions of the detrusor in Sexual development response to sexual stimulation or orgasm. The contractions are painful if the bladder is empty and cause incontinence if Sexual development of handicapped patients has been the any urine is present. They are not abolished by clam subject of little research. The most obvious public attention cystoplasty, but they are if the bladder is removed and a arises when there is a moral or ethical problem. For example, a substitution cystoplasty performed (unpublished data). difficult area of community policy is how to prevent unwanted A recent study from the Netherlands has shown that, in pregnancies and sexual abuse in affected girls of low practice, sexual activity is less common than might have intelligence. Those with severe congenital disabilities often been suspected, especially in those with hydrocephalus fail to develop normal sexuality because of lack of privacy and (Fig. 5)[28]. Erection and ejaculation in males is largely dependence on others for normal daily living. They have low under neurological control. All males with intact sacral social and sexual confidence. Surprisingly, however, even reflexes and urinary continence are potent. With absent those who can walk and whose spina bifida is “hidden” have sacral reflexes, 64% with levels below D10 and 14% with major sexual problems. They will have uncertain bladder and levels above D10 are potent. There is doubt about the true bowel control, which leads to an unwillingness to mix with sexual nature of such erections [27]. their peers on an equal basis. It is most important not to Again, male patients in a Dutch study had less than imagine that an apparently minor level of neurological expected sexual activity, especially if they had hydroceph- disability means that sexuality is normal [23]. The physical alus (Fig. 6)[28]. Impotence responds to conventional aspects of sexual function that depend on the brain are management such as intracorporeal injection [29]. Sildena- generally intact, whereas those that depend on the spinal fil may be used with appropriate dose reduction. In the only cord will be damaged in line with the neurological level. trial to date in this group, dose escalation was used with patients as their own controls. Eighty percent of men When children are brought up in the mainstream of education and integrated in school, the social results are responded to a dose of 50 mg. Although one patient excellent. In a study to compare 11 dimensions of self-image subsequently responded to 100 mg, it was recommended in adolescents with spina bifida with those of their peers, that such a high dose should not be used in spina bifida. there was no difference in ten. Unfortunately, the 11th was Five of the 11 responders in the series were wheelchair the dimension of sexuality, which was significantly below bound [30, 31]. In view of the possibility that impotence is normal, especially in females [24]. Spina bifida adolescents associated with azoospermia, the prospects for fertility may are often ignorant of even very straightforward aspects of not be improved. sexuality, which should be taught within the family. The ordinary facts of reproductive life often are not given. Up to 23% of girls do not know about the hygienic management Fertility of menstruation [25, 26]. It is not surprising, therefore, that adolescents have very little sexual contact. Fertility in females is thought to be normal. The main In females, as in males, sexual function, as defined by problem is, of course, the risk of a neural tube defect in the sexual sensation and orgasm, is dependent on neurological offspring, which is discussed below. Folic acid prophylaxis level. Most with levels below L2 are thought to have normal considerably reduces the risk. A secondary cause is the Fig. 5 Histogram showing sex- 100% No hydrocephalus n=24 ual activity in women with With hydrocephalus n=69 spina bifida [28]. SI Sexual 80% intercourse 60% 40% 20% 0% Masturbation SI last year SI ever Partner (ever) Satisfaction 1228 Pediatr Nephrol (2008) 23:1223–1231 Fig. 6 Histogram showing sex- 100% No hydrocephalus n=22 ual activity in men with spina With hydrocephalus n=42 bifida [28]. SI Sexual intercourse 80% 60% 40% 20% 0% Masturbation SI last year SI ever Partner (ever) Satisfaction The incidence of all neural tube defects in babies in the obesity commonly seen in spina bifida adolescents. Unfor- tunately, the incidence of neural tube defect pregnancies is Western world has diminished in the last 30 years. Even nearly double in women who are obese at the time of before the discovery of the protective effects of folic acid, the conception (body mass index >29 kg/m )[32]. overall incidence had been falling. In a recent study from the There have been several reports of favourable pregnancy United Kingdom, it was shown that the incidence of neural outcomes in women with spina bifida, though one group tube defects started to fall about 18 years before the use of summarised their experiences with difficult pregnancies in folic acid began to rise. The incidence fell from about 225/ (medically) difficult patients from difficult (socially deprived) 100,000 live births to about 48/100,000 live births between families [33]. Several specific problems have been identified. 1972 and 1990. The number of sales of folic acid was less Urinary tract infections are almost invariable; bladder than 100,000 per year until 1990, rising to 1.2 million per function and mobility often deteriorate, though not perma- year by 1996 (Fig. 7)[37]. However, the main protection nently; the deformed and, often, small pelvis makes against the conception of a baby with a neural tube defect is accommodation of the foetus difficult, leading to premature to give the mother folic acid supplements in the 3 months labour and an increased need for caesareans [33, 34]. In a before conception and for the first trimester [36, 38]. The series of 20 pregnancies, 35% were delivered before Department of Health in the United Kingdom now 37 weeks. Eight required a caesarean section, all for obstetric recommends that women who are at high risk of conceiving indications, most commonly disproportion [33]. Four of five a baby with a neural tube defect should take 5 mg per day wheelchair-bound women required caesarean section com- whereas 0.8 mg per day is sufficient for other women [39]. pared with eight of 18 walkers [35]. Despite this prophylaxis, there remains a small risk of an For females in general and spina bifida girls in particular, affected pregnancy. At least one cause appears to be an in- one of the great medical success stories of the last 25 years has born error of folic acid metabolism, which was found in 16 been the discovery of the prophylactic role of folic acid. In a women who gave birth to two successive babies with double-blind placebo-controlled trial in 1,195 women with myelomeningocele in spite of prophylaxis [40]. high-risk pregnancy (previous birth of a child with neural tube The use of selective pregnancy termination in cases of defect or affected parent), there were six affected foetuses in neural tube defect is as much a social as a medical issue. In the treated group versus 21 in the untreated group [36]. If the a series in South Australia between 1966 and 1991, the father has spina bifida, the pregnancy is also at risk. prevalence of affected pregnancies did not change, but the Fig. 7 Graph showing the inci- dence of births of children with neural tube defects by year. The black line shows the sales of Spina bifida folic acid Anencephaly Folic Acid Sales x10000 1972 1980 1986 1990 1996 Pediatr Nephrol (2008) 23:1223–1231 1229 number of affected live births fell by 84% from 2.29 to We now need to establish how aggressive it is necessary to 0.35/1,000 with an active programme of prenatal diagnosis be in childhood to produce a good bladder in adults. Sadly, and termination [41]. inadequate attention has been paid to preparation for In males infertility appears to be a common problem. In independent adult life for these individuals. Families and those with higher lesions, it might be thought that it would support agencies must remember that childhood is only due to impotence. Although this is undoubtedly true in part, about 25% of the human lifespan. It must be used, as it is preliminary results of a continuing study have identified with normal children, to prepare for the remaining 75%. another problem. In ten impotent males with spina bifida, all were found to be azoospermic on analysis of semen Questions obtained by electroejaculation. On testicular biopsy, all had Sertoli cells only [42]. Poor semen quality has also been Multiple choice questions (answers appear following reported in men with acquired spinal lesions using electro- reference list) ejaculation, especially if ejaculation is infrequent [23]. No figures are available for the overall incidence of 1. On present data, what percentage of babies born with infertility in males. However, it is interesting to note a study myelomeningocele have survived into adult life? of 49 adults with spina bifida in South Wales (UK): 16 men a. 75–80% and 15 women were married or had a regular partner. b. 65–70% Twenty-eight (90%) of these married couples had children. c. 60–64% Success in partnership and fertility were said to be d. 40–45% unrelated to continence or mobility [43]. 2. In adults born with myelomeningocele, what is the commonest cause of death? Independence and work a. Renal failure Few data are available on levels of independence and b. Suicide employment of adults with spina bifida. The greater the c. Infected ventriculoperitoneal shunt handicap, both physical and mental, the less likely is a d. Respiratory failure successful outcome. Even so, many are able to occupy themselves with work at home or in protected environments, 3. In wheelchair-bound males born with myelomeningo- with or without pay. The social outcome in an unselected cele, which of the following may improve with puberty? group of adults is shown in Table 1 [44]. a. Bowel control In a Swedish study, 38% of young adults with spina bifida b. Manual dexterity were in gainful employment (compared with 47% with c. Respiratory reserve traumatic paraplegia). Not surprisingly, a better outcome was d. Bladder outflow resistance found in walkers with higher educational achievements [45]. 4. In which men with myelomeningocele are erections most likely to be normal? Conclusion a. Sensory level below D10 The management of children with spina bifida has b. Absent history of hydrocephalus improved considerably in the last 50 years. In particular, c. Normal serum testosterone understanding of the neuropathic bladder has lead to less d. In-tact sacral reflexes urinary infection, less renal damage and better continence. 5. Which of the following is the most important prophy- Table 1 Social outcomes in adults with spina bifida laxis to reduce the risk of neural tube defects in babies Below L3 L3-T11 Above T11 conceived by women born with myelomeningocele? n=24 n=15 n=12 a. Folic acid 5 mg daily in the first trimester IQ> 80 21 11 6 b. Folic acid 5 mg daily in the first two trimesters Walker 16 0 0 c. Folic acid 5 mg daily for at least 3 months before Independent 14 5 2 conception and in the first trimester Driver 14 4 2 d. Folic acid 5 mg daily for at least 3 months before Employed 9 4 2 conception 1230 Pediatr Nephrol (2008) 23:1223–1231 bifida: importance of parental hope. Arch Dis Child 83:293– References 21. Buran CF, Sawin KJ, Brei TJ, Fastenau PS (2004) Adolescents 1. Rosano A, Botto LD, Botting B, Mastroiacovo P (2000) Infant with myelomeningocoele: activities, beliefs, expectations and mortality and congenital anomalies from 1950 to 1994: an interna- perceptions. Dev Med Child Neurol 46:244–252 tional perspective. J Epidemiol Community Health 54:660–666 22. Moore C, Kogan BA, Parekh A (2004) Impact of urinary 2. Hunt GM (2000) Open spina bifida: outcome for a complete incontinence on self concept in children with spina bifida. J Urol cohort treated unselectively and followed into adulthood. Dev 171:1659–1662 Med Child Neurol 32:108–118 23. Glass C, Soni B (1999) Sexual problems of disabled patients. Br 3. Hunt GM, Lewin WS, Gleave J, Gairdner D (1973) Predictive Med J 318:518–521 factors in open myelomeningocele with special reference to 24. Cartwright DB, Joseph AS, Grenier CE (1993) A self image sensory level. BMJ 4:197–201 profile analysis of spina bifida adolescents in Louisiana. J La State 4. Silveri M, Capitanucci ML, Capozza N, Mosiello G, Silvano A, Med Soc 145:394–402 Gennaro G (1997) Occult spinal dysraphism: neurogenic voiding 25. Dorner S (1997) Sexual interest and activity in adolescents with dysfunction and long-term urologic follow-up. Pediatr Surg Int spina bifida. J Child Psychol Psychiatry 18:229–237 12:148–150 26. Blum RW, Resnick MD, Nelson R, St Germaine A (1991) Family 5. Singhal B, Mathew KM (1999) Factors affecting mortality and and peer issues among adolescents with spina bifida and cerebral morbidity in adult spina bifida. Eur J Pediatr Surg 9:31–32 palsy. Pediatrics 88:280–285 6. Rendeli C, Castorina M, Ausili E, Giradi E, Fundaro C, Calderelli 27. Cass AS, Bloom DA, Luxenberg M (1986) Sexual function in M, Savaggio E (2004) Risk factors for atherogenesis in children adults with myelomeningocele. J Urol 136:425–426 with spina bifida. Childs Nerv Syst 20:392–396 28. Verhoef M, Vroege JA, Post MW, van Asbeck FW, Gooskens RH, 7. van den Berg-Emons HJ, Bussman JB, Brobbel AS, Roebroeck Prevo AJ (2005) Sex education, relationships and sexuality in ME, van Meeteren J, Stam HJ (2001) Everyday physical activity young adults with spina bifida. Arch Phys Med Rehabil 86: in adolescents and young adults with myelomeningocele as 979–987 measured with a novel activity monitor. J Pediatr 139:880–886 29. Lue TF, Tanagho EA (1987) Physiology of erection and 8. Schoemakers MA, Gulmans VA, Gooskens RH, Helders PJ pharmacological management of impotence. J Urol 137:829–836 (2004) Spina bifida at the sacral level: more than minor gait 30. Palmer JS, Kaplan WE, Firlit CF (1999) Erectile dysfunction in disturbances. Clin Rehabil 18:178–185 spina bifida is treatable. Lancet 354:125–126 9. Nagarkatti DG, Banta JV, Thomson JD (2000) Charcot arthrop- 31. Palmer JS, Kaplan WE, Firlit CF (2000) Erectile dysfunction in athy in spina bifida. J Pediatr Orthop 20:82–87 patients with spina bifida is a treatable condition. J Urol 164: 10. Williams EN, Broughton NS, Menelaus MB (1999) Age related 958–961 walking in children with spina bifida. Dev Med Child Neurol 32. Shaw GM, Velie EM, Schaffer D (1996) Risk of neural tube 41:446–449 defect-affected pregnancies among obese women. JAMA 11. Silber TJ, Shaer C, Atkins D (1999) Eating disorders in adolescents 275:1093–1096 and young women with spina bifida. Int J Eat Disord 25:457–461 33. Richmond D, Zaharievski I, Bond A (1987) Management of 12. Woodhouse CR (1992) Reconstruction of the lower urinary tract pregnancy in mothers with spina bifida. Eur J Obstet Reprod Biol for neurogenic bladder: lessons from the adolescent age group. Br 25:341–345 J Urol 69:589–593 34. Rietberg CC, Lindhout D (1993) Adult patients with spina bifida 13. Ross RM (2003) ATS/ACCP statement on cardiopulmonary cystica: genetic counselling, pregnancy and delivery. Eur J Obstet exercise testing. Am J Respir Crit Care Med 167:1451 Reprod Biol 52:63–70 14. Ellsworth PI, Merguerian PA, Klein RB, Rozycki AA (1993) 35. Arata M, Grover S, Dunne K, Bryan D (2000) Pregnancy outcome Evaluation and risk factors of latex allergy in spina bifida patients: and complications in women with spina bifida. J Reprod Med is it preventable? J Urol 150:691–693 45:743–748 15. Bernardini R, Novembre E, Lombardi E, Mezzetti P, Cianferroni 36. MRC vitamin study research group (1991) Prevention of neural A, Danti AD, Mercurella A, Vierucci A (1998) Prevalence of and tube defects: results of the MRC vitamin study. Lancet 338: risk factors for latex sensitization in patients with spina bifida. J 131–135 Urol 160:1775–1778 37. Kadir RA, Sabin C, Whitlow B, Brockbank E, Economides D 16. Ziylan HO, Ander AH, Alp T, Kadioglou TC, Esen T, Besisik TA, (1999) Neural tube defects and periconceptional folic acid in Cuhadaroglou C (1996) Latex allergy in patients with spinal England and Wales: retrospective study. Br Med J 319:92–93 dysraphism: the role of multiple surgery. Br J Urol 78:777–779 38. Czeizel AE, Dudas I (1992) Prevention of the first occurrence of 17. Kalpaklioglou AF, Aydin G (1999) Prevalence of latex sensitivity neural tube defects by periconceptional vitamin supplementation. among patients with chronic renal failure: a new risk group? Artif N Engl J Med 327:1832–1835 Organs 23:139–142 39. Lloyd J (1992) Folic acid and the prevention of neural tube 18. Schneck FX, Bellinger M (1993) The innocent cough or sneeze: defects. London: Department of Health the harbinger of serious latex allergy in children during bladder 40. Wild J, Seller MJ, Schorah CJ, Smithells RW (1994) Investigation stimulation and urodynamics. J Urol 150:687–690 of folate intake and metabolism in women who have had two 19. Reiter S, Goldman T (1999) A programme for the enhancement of pregnancies complicated by neural tube defects. Br J Obstet autonomy in young adults with physical disabilities. Int J Rehabil Gynaecol 101:197–202 Res 22:71–74 41. Chan A, Robertson EF, Haan EA (1993) Prevalence of neural tube 20. Kirpalani HM, Parkin PC, Willan AR, Fehlings DL, Rosenbaum defects in South Australia,1966–91: effectiveness and impact of PL, King D, van Nie AJ (2000) Quality of life in spina prenatal diagnosis. Br Med J 307:703–706 Pediatr Nephrol (2008) 23:1223–1231 1231 42. Reilly JM, Oates RD (1992) Preliminary investigation of the cord injury and meningomyelocele. J Rehabil Med 38: potential fertility status of postpubertal males with myelodysplasia. 192–200 J Urol 147:75 A (abstract 150) 43. Laurence KM, Beresford A (1975) Continence, friends, marriage Answers and children in 51 adults with spina bifida. Dev Med Child Neurol 17:123–128 44. Hunt GM, Oakeshott P (2003) Outcome in people with spina 1. c 1. c bifida at age 35: prospective community based cohort study. Br 2. a 2. a Med J 326:1365–1366 3. d 3. d 45. Valtonen K, Karlsson AK, Alaranta H, Viikari-Jantura E 4. d 4. d (2006) Work participation among persons with traumatic spinal 5. c 5. c http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Pediatric Nephrology Unpaywall

Myelomeningocele: neglected aspects

Woodhouse, Christopher R. J.
Pediatric Nephrology – Aug 1, 2008
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Pediatr Nephrol (2008) 23:1223–1231 DOI 10.1007/s00467-007-0663-3 EDUCATIONAL FEATURE Christopher R. J. Woodhouse Received: 6 June 2007 /Revised: 28 August 2007 /Accepted: 21 September 2007 / Published online: 17 January 2008 IPNA 2007 . . Abstract The commonest cause of neurogenic bladder in Keywords Neurogenic bladder Myelomeningocele . . children is myelomeningocele. Survival of children is much Long-term outcome Paediatric nephrology Education improved in the Western world, but by 35 years old, about 50% will have died. In adults, the commonest causes of death are lung and heart diseases. All physical aspects deteriorate Introduction with age, especially in those with thoracic lesions. Those who walk in childhood have a 20–50% chance of becoming Children who are born with myelomeningocele grow up to wheelchair dependent as adults. Immobility, poor respiratory become adults–a truth which should be self-evident but reserve, obesity, latex allergy and worsening kyphoscoliosis often seems to be overlooked by doctors and by some contribute to the increased risks of surgery. It is essential that health care systems. In contrast, it is a preoccupation with safe and manageable urine drainage is established in their parents. Passage to adult life brings with it the childhood: the bladder never improves with time, and surgical difficulties of adolescence that are common to all: the reconstruction becomes progressively more difficult. Inde- beginning of sexual interest but a continuing deterioration pendence in adult life will only be possible with intense in the body as a whole. Sadly, nothing in spina bifida gets preparation in childhood. Children must be allowed to join in better with age. Preparation for adulthood is frequently with family chores and events. Education, both academic and neglected in childhood, and the consequences of growing practical, must be encouraged. Skills such as driving, up with spina bifida are poorly researched. shopping and birth control must be taught. However, even with the best support, less than 40% will have gainful employment. Children who are continent and have lesions Survival below L2 are likely to have normal sexual function. Sexual activity in adolescents, especially in those with hydrocepha- For all babies born with open spina bifida, the prognosis is lus, is limited (but not absent). However, by adult life, about poor. In underdeveloped countries, congenital anomalies, two thirds will have established a regular partnership. All especially spina bifida, account for nearly 50% of infant females and those males who are naturally potent are likely to deaths, and the rate is inversely correlated with the per be fertile. There is a high risk of neural tube defects in their capita gross domestic product [1]. In the UK, only 60% of offspring unless the female partner takes prophylactic folic such children have survived into adulthood (Fig. 1)[2]. At acid for 3 months before pregnancy and for first trimester. all ages, renal failure is the commonest cause of death. In children, the risk of renal failure is strongly related to the sensory level (which may not be the same as the anatomical level in the spine or the level suggested by X-ray). Renal C. R. J. Woodhouse (*) failure is rare with sensory levels at or below L4 and Adolescent Urology, University College, common at or above T10 [3]. However, renal failure can 235 Euston Road, occur even with apparently minor neural tube defects, such London NW1 2PR, UK e-mail: [email protected] as occult spinal dysraphism. In a group of 55 such cases, 1224 Pediatr Nephrol (2008) 23:1223–1231 100% continue to walk, there may be damage to insensate joints (Charcot arthropathy), which has a prevalence of one in 100 80% up to the age of 42 years [9]. The percentage of children 60% who start ambulation (with or without aids) and the 40% percentage who continue to walk up to 9 years 1 month, by spinal level, is shown in Fig. 3 [10]. Loss of mobility 20% encourages obesity, which, combined with the collapsed 0% spine, makes much of the lower half of the body invisible to 1 7 14 21 28 35 the patient. Attempts to impose weight loss in spina bifida Fig. 1 Survival curve for children born with spina bifida adolescents can lead to eating disorders just as it can in others [11]. Respiratory reserve worsens, making mobility more difficult. only 24 patients (43%) had urological symptoms at presentation, and yet all eventually became incontinent, and eight developed renal failure [4]. Surgery in adulthood In an unselected series of 695 adults in the UK, 56 were known to have died before their expected time. In the 30 The decision to undertake major surgery on an adult with patients whose cause of death could be determined, renal spina bifida should not be taken lightly, and preparation failure accounted for one third. Thirteen died of cardiac or must be meticulous. respiratory disease, three committed suicide and one died of If there is to be any significant period of immobility, it cancer (Fig. 2)[5]. There is an increased risk of atheroscle- may be necessary to have a water bed and nursing by rosis in patients with spina bifida, even in the absence of appropriately trained individuals. Even prior to surgery, obesity [6]. some patients are limited in the positions in which they can lie; after surgery, the possible positions may be even more limited. There is anecdotal evidence that there is an Mobility increased risk of fracture with minimal trauma in patients with spina bifida. Great care must be taken in moving As the child grows, there is a natural tendency for physical patients in the hospital. Special attention must be given to anomalies to deteriorate. The spinal deformity becomes the position of metal work in the spine, parts of which may more pronounced. Those who could just walk tend to relapse come dangerously close to the surface of the skin. into a wheelchair. This is illustrated in Fig. 3: those with Latex allergy is common in patients with spina bifida, high lesions, who also have a considerably delayed age of the incidence increasing with age (see below). If there is ambulation, have a 20–50% chance of loosing the ability to any suggestion of such a problem, surgery must be walk by the age of 10 years. Furthermore, adolescents with performed in a strictly latex-free environment. spina bifida spend about a half the time in dynamic physical With age, respiratory reserve declines. Presumably, activity as do their normal contemporaries [7]. increasing kyphoscoliosis is at least a part of the cause. In Unfortunately, just being able to walk does not mean that the author’s own series of adult patients presenting for independence is assured. Even those with sacral lesions who urinary tract reconstruction, 10% were thought to be unfit have good muscle strength in the hips may require help with for anaesthesia on the basis of lung-function tests [12]. mobility and bowel and bladder care [8]. For those who Cardiopulmonary exercise testing (CPX) is rapidly becom- Fig. 2 Histogram to show the 50% causes of death in children and adults born with spina bifida. 40% CNS Central nervous system 30% Children 20% Adults 10% 0% CNS Renal Heart/lung Suicide Other Pediatr Nephrol (2008) 23:1223–1231 1225 Fig. 3 Percentage of children 100% who start ambulation (with or without aids) and percentage 80% who continue to walk up to 9 years 1 month, by spinal level 60% Started walking [10] Still walking 40% 20% 0% Thoracic L1/2 L3 L4/5 Sacral Latex allergy ing a standard assessment of fitness for major surgery [13]. CPX requires adaptation for use in patients with spina bifida, as it normally is performed on a static bicycle. We Aetiology are presently investigating the use of upper-limb pedals for CPX testing, especially to measure the anaerobic threshold. Paediatric urologists with a large practice of spina bifida Worsening kyphoscoliosis also makes access to the patients are well aware of the problems of latex allergy. abdominal cavity more difficult. Patients may be unable Sophisticated strategies have been established to identify to lie flat on the operating table. The distance between the patients at risk and to avoid the serious problems of costal margin and the pubis decreases. Rotational deformity anaphylaxis, especially under anaesthetic. In the United of the spine results in a changing relationship between States, the Shriners Myelodysplasia Hospitals are entirely abdomen and chest so that when lying flat on the back at latex free. Adult urologists are much less aware of this chest level, the abdomen may be rotated to the side. The potentially fatal problem. Although there is a general kidneys may be particularly inaccessible. recognition that spina bifida children are particularly at risk, Neither bowel nor bladder function improves at puberty, the reasons are unclear. It is tempting to think that the though bladder outflow resistance increases (which may increased number of surgical procedures is to blame. Ells- bring more danger than advantage). Because of the worth et al. found an overall incidence of latex allergy of 60% difficulties with surgery, it is particularly important to in 50 spina bifida children. The mean number of operations in establish stable systems in childhood. It should be positive patients was 9.5 compared with 6.7 in negative remembered that the lower half of the abdomen may children (P=0.03). There was no difference in the age of the become invisible to the patient, particularly in women, so children, the number of years that they had been on clean that stomata and catheterization sites must be put above the intermittent catheterisation or the number of abdominal umbilicus (Fig. 4). operations that they had undergone [14]. Fig. 4 Clinical photograph to illustrate the difficulty that adults with spina bifida have in visualizing the lower half of the abdomen Correct site of stoma within the girl’s field of view Actual site which she cannot see 1226 Pediatr Nephrol (2008) 23:1223–1231 However, there are probably other factors to consider. operations have been uneventful in a latex-free operating The presence of latex-specific immunoglobulin (Ig)G was theatre (unpublished data). found in 25% of a group of European spina bifida patients aged between 2 and 40 years old. Multivariate analysis showed that atopy, especially to pears and kiwi fruit and a Preparation for adulthood history of five or more operations were significantly and independently associated with latex allergy [15]. On the Childhood is a preparation for adulthood. This is true of other hand, in a controlled study of the incidence of latex children with spina bifida as it is for those who are more allergy, it was found that that 26% of spina bifida patients normal. It should come as no surprise to find that if those with had symptomless latex allergy compared with 5% of spina bifida are inadequately prepared, they will not become patients from an ear, nose and throat clinic, even though independent. As with everything else in this condition, it is both groups had had the same number of operations (mean very hard to do. Expectations must be appropriate and special of 1.65 and 1.95, respectively). There were no positive tests needs met, but without such work in childhood, dependence in a control group of urological patients who had had no will continue into adult life. Few countries have adequate operations [16]. Latex allergy has also been found in 1.1% arrangements for young dependent adults, and much of the of patients on regular haemodialysis [17]. burden will, therefore, continue to fall on the family. It is seldom appropriate to wait for symptoms to “improve with Presentation age”. Anything that is not sorted out in childhood will be more difficult to manage in adulthood. About one third of spina bifida patients will have a history Autonomy of the disabled can be considerably improved of allergic reactions to latex products [14]. The level of by an active but realistic programme of education [19]. awareness of the problem amongst spina bifida children and Parents who have a positive and hopeful attitude are able to their families is low, and so the symptoms should be improve their adolescent’s quality of life by about 25% over specifically sought. In some cases, the association is that which would be predicted for the disability at birth obvious, but in others it may be obscure, such as itchy [20]. Unfortunately, even with the highest levels of eyes while using washing-up gloves or genital reactions to expectation and ambition, participation in the full range of condoms. In occasional patients, there is acute anaphylaxis adolescent activities or household chores is uncommon under anaesthetic. All patients once identified should be [21]. Although incontinence is not always a barrier to a made aware of the risks and should wear a medic-alert fulfilled life, those who are continent have an improved bracelet. During medical procedures, latex use should be view of self worth. In girls, social acceptance and views of reduced to a minimum. There are important warning signs global self-worth and in boys, scholastic competence, social that may alert the clinician to the problem in a previously acceptance, physical appearance and behaviour are all unsuspected case. In a group of five children (from 17) who improved by continence [22]. were undergoing transurethral bladder stimulation and In addition, throughout childhood, a whole set of urodynamics, all had coughing or sneezing for several emotional and social problems emerge, though they cannot minutes before bronchospasm and generalised allergic totally be separated from the physical. Patients become reactions developed [18]. sexually interested without necessarily having the means to indulge or control their desires. Maturity brings new Management interests. For those who are reasonably intelligent, there is a desire to have normal friends, to go out to the cinema or The most important part of management is identification of club and to socialise with a peer group. Many patients are patients at risk and prophylaxis. As the incidence is so high able to work, some in very demanding areas including in spina bifida patients, a specific enquiry should be made medicine, though special training or work facilities may be for symptoms in all patients. Minor reactions can be treated needed. For the mentally retarded, appropriately stimulating with bronchodilators, antihistamines and, occasionally, occupations must be found. All of this requires help. At the steroids. It should be remembered that minor reactions very least, there has to be an infrastructure of sympathetic may be the forerunner of major anaphylaxis. All who have friends who will take a wheelchair-bound person to places a suggestive history or unexplained hypotensive episodes that often are not too well equipped to accommodate them. under anaesthetic should undergo skin testing and mea- Social research shows that buildings and transport systems surement of latex-specific IgE. A latex-free medical are poorly adapted to the needs of the handicapped. environment should be created for patients found to be at All children who are born with congenital anomalies risk. In all of four patients on the author’s service who have a desire to be normal and to be treated as such. It is presented with anaphylaxis under anaesthetic, further essential to encourage children with spina bifida to look Pediatr Nephrol (2008) 23:1223–1231 1227 after themselves and to take part in normal family life right sexual sensations, as are most with urinary continence. Only from the start. about 20% of those with higher levels or with urinary incontinence have normal sexual function [27]. Some women appear to have powerful contractions of the detrusor in Sexual development response to sexual stimulation or orgasm. The contractions are painful if the bladder is empty and cause incontinence if Sexual development of handicapped patients has been the any urine is present. They are not abolished by clam subject of little research. The most obvious public attention cystoplasty, but they are if the bladder is removed and a arises when there is a moral or ethical problem. For example, a substitution cystoplasty performed (unpublished data). difficult area of community policy is how to prevent unwanted A recent study from the Netherlands has shown that, in pregnancies and sexual abuse in affected girls of low practice, sexual activity is less common than might have intelligence. Those with severe congenital disabilities often been suspected, especially in those with hydrocephalus fail to develop normal sexuality because of lack of privacy and (Fig. 5)[28]. Erection and ejaculation in males is largely dependence on others for normal daily living. They have low under neurological control. All males with intact sacral social and sexual confidence. Surprisingly, however, even reflexes and urinary continence are potent. With absent those who can walk and whose spina bifida is “hidden” have sacral reflexes, 64% with levels below D10 and 14% with major sexual problems. They will have uncertain bladder and levels above D10 are potent. There is doubt about the true bowel control, which leads to an unwillingness to mix with sexual nature of such erections [27]. their peers on an equal basis. It is most important not to Again, male patients in a Dutch study had less than imagine that an apparently minor level of neurological expected sexual activity, especially if they had hydroceph- disability means that sexuality is normal [23]. The physical alus (Fig. 6)[28]. Impotence responds to conventional aspects of sexual function that depend on the brain are management such as intracorporeal injection [29]. Sildena- generally intact, whereas those that depend on the spinal fil may be used with appropriate dose reduction. In the only cord will be damaged in line with the neurological level. trial to date in this group, dose escalation was used with patients as their own controls. Eighty percent of men When children are brought up in the mainstream of education and integrated in school, the social results are responded to a dose of 50 mg. Although one patient excellent. In a study to compare 11 dimensions of self-image subsequently responded to 100 mg, it was recommended in adolescents with spina bifida with those of their peers, that such a high dose should not be used in spina bifida. there was no difference in ten. Unfortunately, the 11th was Five of the 11 responders in the series were wheelchair the dimension of sexuality, which was significantly below bound [30, 31]. In view of the possibility that impotence is normal, especially in females [24]. Spina bifida adolescents associated with azoospermia, the prospects for fertility may are often ignorant of even very straightforward aspects of not be improved. sexuality, which should be taught within the family. The ordinary facts of reproductive life often are not given. Up to 23% of girls do not know about the hygienic management Fertility of menstruation [25, 26]. It is not surprising, therefore, that adolescents have very little sexual contact. Fertility in females is thought to be normal. The main In females, as in males, sexual function, as defined by problem is, of course, the risk of a neural tube defect in the sexual sensation and orgasm, is dependent on neurological offspring, which is discussed below. Folic acid prophylaxis level. Most with levels below L2 are thought to have normal considerably reduces the risk. A secondary cause is the Fig. 5 Histogram showing sex- 100% No hydrocephalus n=24 ual activity in women with With hydrocephalus n=69 spina bifida [28]. SI Sexual 80% intercourse 60% 40% 20% 0% Masturbation SI last year SI ever Partner (ever) Satisfaction 1228 Pediatr Nephrol (2008) 23:1223–1231 Fig. 6 Histogram showing sex- 100% No hydrocephalus n=22 ual activity in men with spina With hydrocephalus n=42 bifida [28]. SI Sexual intercourse 80% 60% 40% 20% 0% Masturbation SI last year SI ever Partner (ever) Satisfaction The incidence of all neural tube defects in babies in the obesity commonly seen in spina bifida adolescents. Unfor- tunately, the incidence of neural tube defect pregnancies is Western world has diminished in the last 30 years. Even nearly double in women who are obese at the time of before the discovery of the protective effects of folic acid, the conception (body mass index >29 kg/m )[32]. overall incidence had been falling. In a recent study from the There have been several reports of favourable pregnancy United Kingdom, it was shown that the incidence of neural outcomes in women with spina bifida, though one group tube defects started to fall about 18 years before the use of summarised their experiences with difficult pregnancies in folic acid began to rise. The incidence fell from about 225/ (medically) difficult patients from difficult (socially deprived) 100,000 live births to about 48/100,000 live births between families [33]. Several specific problems have been identified. 1972 and 1990. The number of sales of folic acid was less Urinary tract infections are almost invariable; bladder than 100,000 per year until 1990, rising to 1.2 million per function and mobility often deteriorate, though not perma- year by 1996 (Fig. 7)[37]. However, the main protection nently; the deformed and, often, small pelvis makes against the conception of a baby with a neural tube defect is accommodation of the foetus difficult, leading to premature to give the mother folic acid supplements in the 3 months labour and an increased need for caesareans [33, 34]. In a before conception and for the first trimester [36, 38]. The series of 20 pregnancies, 35% were delivered before Department of Health in the United Kingdom now 37 weeks. Eight required a caesarean section, all for obstetric recommends that women who are at high risk of conceiving indications, most commonly disproportion [33]. Four of five a baby with a neural tube defect should take 5 mg per day wheelchair-bound women required caesarean section com- whereas 0.8 mg per day is sufficient for other women [39]. pared with eight of 18 walkers [35]. Despite this prophylaxis, there remains a small risk of an For females in general and spina bifida girls in particular, affected pregnancy. At least one cause appears to be an in- one of the great medical success stories of the last 25 years has born error of folic acid metabolism, which was found in 16 been the discovery of the prophylactic role of folic acid. In a women who gave birth to two successive babies with double-blind placebo-controlled trial in 1,195 women with myelomeningocele in spite of prophylaxis [40]. high-risk pregnancy (previous birth of a child with neural tube The use of selective pregnancy termination in cases of defect or affected parent), there were six affected foetuses in neural tube defect is as much a social as a medical issue. In the treated group versus 21 in the untreated group [36]. If the a series in South Australia between 1966 and 1991, the father has spina bifida, the pregnancy is also at risk. prevalence of affected pregnancies did not change, but the Fig. 7 Graph showing the inci- dence of births of children with neural tube defects by year. The black line shows the sales of Spina bifida folic acid Anencephaly Folic Acid Sales x10000 1972 1980 1986 1990 1996 Pediatr Nephrol (2008) 23:1223–1231 1229 number of affected live births fell by 84% from 2.29 to We now need to establish how aggressive it is necessary to 0.35/1,000 with an active programme of prenatal diagnosis be in childhood to produce a good bladder in adults. Sadly, and termination [41]. inadequate attention has been paid to preparation for In males infertility appears to be a common problem. In independent adult life for these individuals. Families and those with higher lesions, it might be thought that it would support agencies must remember that childhood is only due to impotence. Although this is undoubtedly true in part, about 25% of the human lifespan. It must be used, as it is preliminary results of a continuing study have identified with normal children, to prepare for the remaining 75%. another problem. In ten impotent males with spina bifida, all were found to be azoospermic on analysis of semen Questions obtained by electroejaculation. On testicular biopsy, all had Sertoli cells only [42]. Poor semen quality has also been Multiple choice questions (answers appear following reported in men with acquired spinal lesions using electro- reference list) ejaculation, especially if ejaculation is infrequent [23]. No figures are available for the overall incidence of 1. On present data, what percentage of babies born with infertility in males. However, it is interesting to note a study myelomeningocele have survived into adult life? of 49 adults with spina bifida in South Wales (UK): 16 men a. 75–80% and 15 women were married or had a regular partner. b. 65–70% Twenty-eight (90%) of these married couples had children. c. 60–64% Success in partnership and fertility were said to be d. 40–45% unrelated to continence or mobility [43]. 2. In adults born with myelomeningocele, what is the commonest cause of death? Independence and work a. Renal failure Few data are available on levels of independence and b. Suicide employment of adults with spina bifida. The greater the c. Infected ventriculoperitoneal shunt handicap, both physical and mental, the less likely is a d. Respiratory failure successful outcome. Even so, many are able to occupy themselves with work at home or in protected environments, 3. In wheelchair-bound males born with myelomeningo- with or without pay. The social outcome in an unselected cele, which of the following may improve with puberty? group of adults is shown in Table 1 [44]. a. Bowel control In a Swedish study, 38% of young adults with spina bifida b. Manual dexterity were in gainful employment (compared with 47% with c. Respiratory reserve traumatic paraplegia). Not surprisingly, a better outcome was d. Bladder outflow resistance found in walkers with higher educational achievements [45]. 4. In which men with myelomeningocele are erections most likely to be normal? Conclusion a. Sensory level below D10 The management of children with spina bifida has b. Absent history of hydrocephalus improved considerably in the last 50 years. In particular, c. Normal serum testosterone understanding of the neuropathic bladder has lead to less d. In-tact sacral reflexes urinary infection, less renal damage and better continence. 5. Which of the following is the most important prophy- Table 1 Social outcomes in adults with spina bifida laxis to reduce the risk of neural tube defects in babies Below L3 L3-T11 Above T11 conceived by women born with myelomeningocele? n=24 n=15 n=12 a. Folic acid 5 mg daily in the first trimester IQ> 80 21 11 6 b. Folic acid 5 mg daily in the first two trimesters Walker 16 0 0 c. Folic acid 5 mg daily for at least 3 months before Independent 14 5 2 conception and in the first trimester Driver 14 4 2 d. Folic acid 5 mg daily for at least 3 months before Employed 9 4 2 conception 1230 Pediatr Nephrol (2008) 23:1223–1231 bifida: importance of parental hope. Arch Dis Child 83:293– References 21. Buran CF, Sawin KJ, Brei TJ, Fastenau PS (2004) Adolescents 1. Rosano A, Botto LD, Botting B, Mastroiacovo P (2000) Infant with myelomeningocoele: activities, beliefs, expectations and mortality and congenital anomalies from 1950 to 1994: an interna- perceptions. Dev Med Child Neurol 46:244–252 tional perspective. J Epidemiol Community Health 54:660–666 22. Moore C, Kogan BA, Parekh A (2004) Impact of urinary 2. Hunt GM (2000) Open spina bifida: outcome for a complete incontinence on self concept in children with spina bifida. J Urol cohort treated unselectively and followed into adulthood. Dev 171:1659–1662 Med Child Neurol 32:108–118 23. Glass C, Soni B (1999) Sexual problems of disabled patients. Br 3. Hunt GM, Lewin WS, Gleave J, Gairdner D (1973) Predictive Med J 318:518–521 factors in open myelomeningocele with special reference to 24. Cartwright DB, Joseph AS, Grenier CE (1993) A self image sensory level. BMJ 4:197–201 profile analysis of spina bifida adolescents in Louisiana. J La State 4. Silveri M, Capitanucci ML, Capozza N, Mosiello G, Silvano A, Med Soc 145:394–402 Gennaro G (1997) Occult spinal dysraphism: neurogenic voiding 25. Dorner S (1997) Sexual interest and activity in adolescents with dysfunction and long-term urologic follow-up. Pediatr Surg Int spina bifida. J Child Psychol Psychiatry 18:229–237 12:148–150 26. Blum RW, Resnick MD, Nelson R, St Germaine A (1991) Family 5. Singhal B, Mathew KM (1999) Factors affecting mortality and and peer issues among adolescents with spina bifida and cerebral morbidity in adult spina bifida. Eur J Pediatr Surg 9:31–32 palsy. Pediatrics 88:280–285 6. Rendeli C, Castorina M, Ausili E, Giradi E, Fundaro C, Calderelli 27. Cass AS, Bloom DA, Luxenberg M (1986) Sexual function in M, Savaggio E (2004) Risk factors for atherogenesis in children adults with myelomeningocele. J Urol 136:425–426 with spina bifida. Childs Nerv Syst 20:392–396 28. Verhoef M, Vroege JA, Post MW, van Asbeck FW, Gooskens RH, 7. van den Berg-Emons HJ, Bussman JB, Brobbel AS, Roebroeck Prevo AJ (2005) Sex education, relationships and sexuality in ME, van Meeteren J, Stam HJ (2001) Everyday physical activity young adults with spina bifida. Arch Phys Med Rehabil 86: in adolescents and young adults with myelomeningocele as 979–987 measured with a novel activity monitor. J Pediatr 139:880–886 29. Lue TF, Tanagho EA (1987) Physiology of erection and 8. Schoemakers MA, Gulmans VA, Gooskens RH, Helders PJ pharmacological management of impotence. J Urol 137:829–836 (2004) Spina bifida at the sacral level: more than minor gait 30. Palmer JS, Kaplan WE, Firlit CF (1999) Erectile dysfunction in disturbances. Clin Rehabil 18:178–185 spina bifida is treatable. Lancet 354:125–126 9. Nagarkatti DG, Banta JV, Thomson JD (2000) Charcot arthrop- 31. Palmer JS, Kaplan WE, Firlit CF (2000) Erectile dysfunction in athy in spina bifida. J Pediatr Orthop 20:82–87 patients with spina bifida is a treatable condition. J Urol 164: 10. Williams EN, Broughton NS, Menelaus MB (1999) Age related 958–961 walking in children with spina bifida. Dev Med Child Neurol 32. Shaw GM, Velie EM, Schaffer D (1996) Risk of neural tube 41:446–449 defect-affected pregnancies among obese women. JAMA 11. Silber TJ, Shaer C, Atkins D (1999) Eating disorders in adolescents 275:1093–1096 and young women with spina bifida. Int J Eat Disord 25:457–461 33. Richmond D, Zaharievski I, Bond A (1987) Management of 12. Woodhouse CR (1992) Reconstruction of the lower urinary tract pregnancy in mothers with spina bifida. Eur J Obstet Reprod Biol for neurogenic bladder: lessons from the adolescent age group. Br 25:341–345 J Urol 69:589–593 34. Rietberg CC, Lindhout D (1993) Adult patients with spina bifida 13. Ross RM (2003) ATS/ACCP statement on cardiopulmonary cystica: genetic counselling, pregnancy and delivery. Eur J Obstet exercise testing. Am J Respir Crit Care Med 167:1451 Reprod Biol 52:63–70 14. Ellsworth PI, Merguerian PA, Klein RB, Rozycki AA (1993) 35. Arata M, Grover S, Dunne K, Bryan D (2000) Pregnancy outcome Evaluation and risk factors of latex allergy in spina bifida patients: and complications in women with spina bifida. J Reprod Med is it preventable? J Urol 150:691–693 45:743–748 15. Bernardini R, Novembre E, Lombardi E, Mezzetti P, Cianferroni 36. MRC vitamin study research group (1991) Prevention of neural A, Danti AD, Mercurella A, Vierucci A (1998) Prevalence of and tube defects: results of the MRC vitamin study. Lancet 338: risk factors for latex sensitization in patients with spina bifida. J 131–135 Urol 160:1775–1778 37. Kadir RA, Sabin C, Whitlow B, Brockbank E, Economides D 16. Ziylan HO, Ander AH, Alp T, Kadioglou TC, Esen T, Besisik TA, (1999) Neural tube defects and periconceptional folic acid in Cuhadaroglou C (1996) Latex allergy in patients with spinal England and Wales: retrospective study. Br Med J 319:92–93 dysraphism: the role of multiple surgery. Br J Urol 78:777–779 38. Czeizel AE, Dudas I (1992) Prevention of the first occurrence of 17. Kalpaklioglou AF, Aydin G (1999) Prevalence of latex sensitivity neural tube defects by periconceptional vitamin supplementation. among patients with chronic renal failure: a new risk group? Artif N Engl J Med 327:1832–1835 Organs 23:139–142 39. Lloyd J (1992) Folic acid and the prevention of neural tube 18. Schneck FX, Bellinger M (1993) The innocent cough or sneeze: defects. London: Department of Health the harbinger of serious latex allergy in children during bladder 40. Wild J, Seller MJ, Schorah CJ, Smithells RW (1994) Investigation stimulation and urodynamics. J Urol 150:687–690 of folate intake and metabolism in women who have had two 19. Reiter S, Goldman T (1999) A programme for the enhancement of pregnancies complicated by neural tube defects. Br J Obstet autonomy in young adults with physical disabilities. Int J Rehabil Gynaecol 101:197–202 Res 22:71–74 41. Chan A, Robertson EF, Haan EA (1993) Prevalence of neural tube 20. Kirpalani HM, Parkin PC, Willan AR, Fehlings DL, Rosenbaum defects in South Australia,1966–91: effectiveness and impact of PL, King D, van Nie AJ (2000) Quality of life in spina prenatal diagnosis. Br Med J 307:703–706 Pediatr Nephrol (2008) 23:1223–1231 1231 42. Reilly JM, Oates RD (1992) Preliminary investigation of the cord injury and meningomyelocele. J Rehabil Med 38: potential fertility status of postpubertal males with myelodysplasia. 192–200 J Urol 147:75 A (abstract 150) 43. Laurence KM, Beresford A (1975) Continence, friends, marriage Answers and children in 51 adults with spina bifida. Dev Med Child Neurol 17:123–128 44. Hunt GM, Oakeshott P (2003) Outcome in people with spina 1. c 1. c bifida at age 35: prospective community based cohort study. Br 2. a 2. a Med J 326:1365–1366 3. d 3. d 45. Valtonen K, Karlsson AK, Alaranta H, Viikari-Jantura E 4. d 4. d (2006) Work participation among persons with traumatic spinal 5. c 5. c

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Published: Aug 1, 2008

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