Skip to main content
SpringerLink
Log in

Neurogenic LUT symptoms in Children with Rare Diseases

  • Reference work entry
  • First Online:
Handbook of Neurourology

  • 425 Accesses

Abstract

Obtaining control over bladder and bowel function is a complex process, and especially for children with intellectual or motor disability, this may be more difficult. Some congenital anomalies may also have a neurological compound affecting normal bladder function. It is important to rule out anatomical and neurological abnormalities in these children.

Different rare diseases, such as acute transverse myelitis, Guillain–Barré syndrome, adrenoleukodystrophy, Williams–Beuren syndrome, Menkes disease, congenital Zika syndrome, and surfer’s myelopathy, are described, as well as cerebral palsy and mental retardation. The most common presenting symptoms and expected “normal” development in these children are discussed as well as diagnostic and specific therapeutic approaches. In the vast majority, the diagnostic work-up is not different from children with urinary incontinence without developmental, neurological, or anatomic abnormalities. The same is true for the different treatment options, although this may be extremely challenging. Because most children with special needs have an insufficient fluid intake, it is extremely important to normalize fluid intake, as this will significantly increase continence and maximum voided volume. Poor mobility is an equally important factor in achieving continence, as children can be faced with both organic incontinence due to their underlying disorder and functional incontinence due to the inability to reach and adequately get on the toilet.

Although often thought to be a minor problem, urinary incontinence remains an important issue for every child with special needs or rare disease. It can lead to limited self-esteem and independence and may be a considerable health care challenge. Often it is a multifactorial problem, but with adequate therapy, these children are amenable to continence rehabilitation. The majority can become continent, but some will only achieve a passive form of continence.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 999.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 999.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. M.C. Bax, D.P. Smyth, A.P. Thomas, Health care of physically handicapped young adults. Br. Med. J. (Clin. Res. Ed.) 296(6630), 1153–1155 (1988)

    Article  CAS  PubMed  Google Scholar 

  2. G. Mosiello, C. Gatti, M. De Gennaro, Neurovesical dysfunction in children after treating pelvic neoplasms. BJU Int. 92(3), 289–292 (2003)

    Article  CAS  PubMed  Google Scholar 

  3. M. Knebusch, H.M. Strassburg, K. Reiners, Acute transverse myelitis in childhood: nine cases and review of the literature. Dev. Med. Child Neurol. 40(9), 631–639 (1998)

    Article  CAS  PubMed  Google Scholar 

  4. C.W. Hicks, B. Kay, S.E. Worley, M.J. Moodley, A clinical picture of Guillain-Barré syndrome in children in the United States. Child Neurol. 25(12), 1504–1510 (2010)

    Article  Google Scholar 

  5. J.R. Scheepe, Y.Y.M. Wong, E.D. van Pelt, I.A. Ketelslegers, Neurogenic lower urinary tract dysfunction in the early disease phase of paediatric multiple sclerosis. Mult. Scler. J. 22(11), 1490–1494 (2016)

    Article  Google Scholar 

  6. M. Silveri, M. De Gennaro, C. Gatti, C. Bizzarri, Voiding dysfunction in x-linked adrenoleukodystrophy: symptom score and urodynamic finding. J. Urol. 171(6), 2651–2653 (2004)

    Article  CAS  PubMed  Google Scholar 

  7. L.A. de Araújo, A.F. de Arruda Musegante, E. de Oliveira Damasceno, B. Barroso, Investigation into neurogenic bladder in arthrogryposis multiplex congenita. J. Pediatr. Urol. 9(6), 895–899 (2013)

    Article  Google Scholar 

  8. A. von Gontard, J. Niemczyk, S. Borggrefe-Moussavian, C. Wagner, Incontinence in children, adolescents and adults with Williams syndrome. Neurourol. Urodyn. 35(8), 1000–1005 (2016)

    Article  Google Scholar 

  9. Z.M. Sammour, C.M. Gomes, R.J. Duarte, F.E. Trigo-Rocha, M. Srougi, Voiding dysfunction and the Williams-Beuren syndrome: a clinical and urodynamic investigation. Urology 175(4), 1472–1476 (2006)

    Article  Google Scholar 

  10. S.L. Schulman, S. Zderic, P. Kaplan, Increased prevalence of urinary symptoms and voiding dysfunction in Williams syndrome. J. Pediatr. 129(3), 466–469 (1996)

    Article  CAS  PubMed  Google Scholar 

  11. C. Wagner, J. Niemczyk, M. Equit, L. Curfs, A. von Gontard, Incontinence in persons with Angelman syndrome. Eur. J. Pediatr. 176(2), 225–232 (2017)

    Article  CAS  PubMed  Google Scholar 

  12. M. Radstaake, R. Didden, S. Giesbers, H. Korzilius, N. Peters-Scheffer, R. Lang, A. von Gontard, L.M.G. Curfs, Incontinence in individuals with Angelman syndrome: a comparative study. Res. Dev. Disabil. 34(11), 4184–4193 (2013)

    Article  PubMed  Google Scholar 

  13. M.Y. Kim, J.H. Kim, M.H. Cho, Y.H. Choi, S.H. Kim, Y.J. Im, K. Park, H.G. Kang, J.H. Chae, H.I. Cheong, Urological problems in patients with Menkes disease. J. Korean Med. Sci. 34(1), e4 (2018)

    Article  PubMed  PubMed Central  Google Scholar 

  14. J. Niemczyk, A. von Gontard, M. Equit, K. Bauer, T. Naumann, C. Wagner, L. Curfs, Detailed assessment of incontinence in boys with fragile-X-syndrome in a home setting. Eur. J. Pediatr. 175(10), 1325–1334 (2016)

    Article  CAS  PubMed  Google Scholar 

  15. A. Von Gontard, R. Didden, M. Sinnema, L. Curfs, Urinary incontinence in persons with Prader-Willi syndrome. BJU Int. 106(11), 1758–1762 (2010)

    Article  Google Scholar 

  16. M. Equit, A. Piro-Hussong, J. Niemczyk, L. Curfs, A. von Gontard, Elimination disorders in persons with Prader-Willi and Fragile-X syndromes. Neurourol. Urodyn. 32(7), 986–992 (2013)

    Google Scholar 

  17. S. Giesbers et al., Incontinence in individuals with Rett syndrome: a comparative study. J. Dev. Phys. Disabil. 24(3), 287–300 (2012)

    Article  PubMed  PubMed Central  Google Scholar 

  18. J. Niemczyk, M. Equit, S. Borggrefe-Moussavian, L. Curfs, A. von Gonthard, Incontinence in persons with Noonan syndrome. J. Pediatr. Urol. 11(4), 201–205 (2015)

    Article  Google Scholar 

  19. L.M. Costa Monteiro, G.N.O. Cruz, A.C. Monteiro, M.E.L. Moreira, et al., Neurogenic bladder in the settings of congenital Zika syndrome: a confirmed and unknown condition for urologists. J. Pediatr. Urol. 15(4), 434–435 (2019)

    Article  CAS  PubMed  Google Scholar 

  20. J. Gandhi, Surfer’s myelopathy: a review of etiology, pathogenesis, evaluation, and management. J. Spinal Cord Med. 15, 1–6 (2019)

    Google Scholar 

  21. L. von Wendt et al., Development of bowel and bladder control in the mentally retarded. Dev. Med. Child Neurol. 32(6), 515–518 (1990)

    Article  Google Scholar 

  22. N. Yildiz, Y. Akkoc, M. Ersoz, et al., Cross-sectional study of urinary problems in adults with cerebral palsy: awareness and impact on the quality of life. Neurol. Sci. 38, 1193–1203 (2017)

    Article  PubMed  Google Scholar 

  23. L.E. Roijen et al., Development of bladder control in children and adolescents with cerebral palsy. Dev. Med. Child Neurol. 43(2), 103–107 (2001)

    Article  CAS  PubMed  Google Scholar 

  24. C.J. Reid, M. Borzyskowski, Lower urinary tract dysfunction in cerebral palsy. Arch. Dis. Child. 68(6), 739–742 (1993)

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. B. Samijn et al., Lower urinary tract symptoms and urodynamic findings in children and adults with cerebral palsy: a systematic review. Neurourol. Urodyn. 36(3), 541–549 (2017)

    Article  PubMed  Google Scholar 

  26. M.K. Powers et al., Trends in toilet training and voiding habits among children with Down syndrome. J. Urol. 194(3), 783–787

    Google Scholar 

  27. J. Niemczyk, A. von Gontard, M. Equit, D. Medoff, C. Wagner, L. Curfs, Incontinence in persons with Down syndrome. Neurourol. Urodyn. 36, 1550–1556 (2017)

    Article  CAS  PubMed  Google Scholar 

  28. A. Kitamura et al., Assessment of lower urinary tract function in children with Down syndrome. Pediatr. Int. 56(6), 902–908 (2014)

    Article  PubMed  PubMed Central  Google Scholar 

  29. M.C. de Carvalho et al., Prevalence of lower urinary tract symptoms in individuals with Down syndrome. J. Pediatr. Urol. 10(5), 844–849 (2014)

    Article  Google Scholar 

  30. A. von Gontard et al., Do we manage incontinence in children and adults with special needs adequately? ICI-RS 2014. Neurourol. Urodyn. 35(2), 304–306 (2016)

    Google Scholar 

  31. E. Van Laecke et al., Voiding disorders in severely mentally and motor disabled children. J. Urol. 166(6), 2404–2406 (2001)

    Article  PubMed  Google Scholar 

  32. S. Bross et al., Correlation between motor function and lower urinary tract dysfunction in patients with infantile cerebral palsy. Neurourol. Urodyn. 26(2), 222–227 (2007)

    Article  PubMed  Google Scholar 

  33. P.K. Chiu et al., Does selective dorsal rhizotomy improve bladder function in children with cerebral palsy? Int. Urol. Nephrol. 46(10), 1929–1933 (2014)

    Article  PubMed  Google Scholar 

  34. S.U. Delialioglu et al., Evaluation of lower urinary system symptoms and neurogenic bladder in children with cerebral palsy: relationships with the severity of cerebral palsy and mental status. Turk. J. Med. Sci. 39(4), 571–578 (2009)

    Google Scholar 

  35. A.M. Houle et al., Bladder function before and after selective dorsal rhizotomy in children with cerebral palsy. J. Urol. 160(3 Pt 2), 1088–1091 (1998)

    Article  CAS  PubMed  Google Scholar 

  36. J.A. Fernandes Silva, F. Borges Carrerette, R. Damiao, Uroflowmetry in the management of lower urinary tract symptoms of children and adolescents with cerebral palsy. J. Pediatr. Urol. 10(3), 413–417 (2014)

    Article  CAS  PubMed  Google Scholar 

  37. M. Ersoz et al., Noninvasive evaluation of lower urinary tract function in children with cerebral palsy. Am. J. Phys. Med. Rehabil. 88(9), 735–741 (2009)

    Article  PubMed  Google Scholar 

  38. C.J. Fowler, D. Griffiths, W.C. de Groat, The neural control of micturition. Nat. Rev. Neurosci. 9(6), 453–466 (2008)

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. R.M. Decter et al., Urodynamic assessment of children with cerebral palsy. J. Urol. 138(4 Pt 2), 1110–1112 (1987)

    Article  CAS  PubMed  Google Scholar 

  40. B. Azzarelli, U. Roessmann, Diffuse “anoxic” myelopathy. Neurology 27(11), 1049–1052 (1977)

    Article  CAS  Google Scholar 

  41. M.E. Mayo, Lower urinary tract dysfunction in cerebral palsy. J. Urol. 147(2), 419–420 (1992)

    Article  CAS  PubMed  Google Scholar 

  42. Â. de Medeiros Francilaide Campos, L.A. de Souza, A. Mattos, Urological profile of children with microcephaly and congenital Zika syndrome. J. Pediatr. Urol. 14(20), 30567–30562 (2020)

    Google Scholar 

  43. H.A. Allen et al., Initial trial of timed voiding is warranted for all children with daytime incontinence. Urology 69(5), 962–965 (2007)

    Article  PubMed  Google Scholar 

  44. P. Drigo et al., Neurogenic vesico-urethral dysfunction in children with cerebral palsy. Ital. J. Neurol. Sci. 9(2), 151–154 (1988)

    Article  CAS  PubMed  Google Scholar 

  45. E. Van Laecke et al., Adequate fluid intake, urinary incontinence, and physical and/or intellectual disability. J. Urol. 182(4 Suppl), 2079–2084 (2009)

    Article  PubMed  Google Scholar 

  46. M.I. Karaman et al., Urodynamic findings in children with cerebral palsy. Int. J. Urol. 12(8), 717–720 (2005)

    Article  PubMed  Google Scholar 

  47. B. Samijn, C. Van den Broeck, E. Deschepper, et al., Risk factors for daytime or combined incontinence in children with cerebral palsy. J. Urol. 198, 937–943 (2017)

    Article  PubMed  Google Scholar 

  48. J.A. Fernandes Silva et al., Lower urinary tract dysfunction in children with cerebral palsy. Neurourol. Urodyn. 28(8), 959–963 (2009)

    Article  Google Scholar 

  49. A. von Gontard, Urinary incontinence in children with special needs. Nat. Rev. Urol. 10, 667–674 (2013)

    Article  Google Scholar 

  50. E.A. Anigilaje, T.T. Bitto, Prevalence and predictors of urinary tract infections among children with cerebral palsy in Makurdi, Nigeria. Int. J. Nephrol., 937268 (2013)

    Google Scholar 

  51. K.P. Murphy, S.A. Boutin, K.R. Ide, Cerebral palsy, neurogenic bladder, and outcomes of lifetime care. Dev. Med. Child Neurol. 54(10), 945–950 (2012)

    Article  PubMed  Google Scholar 

  52. G. Gundogdu et al., Relationship of bladder dysfunction with upper urinary tract deterioration in cerebral palsy. J. Pediatr. Urol. 9(5), 659–664 (2013)

    Article  PubMed  Google Scholar 

  53. J.W. Chase et al., Functional constipation in children. J. Urol. 171(6 Pt 2), 2641–2643 (2004)

    Article  CAS  PubMed  Google Scholar 

  54. C.J. Bohmer et al., The prevalence of constipation in institutionalized people with intellectual disability. J. Intellect. Disabil. Res. 45(Pt 3), 212–218 (2001)

    Article  CAS  PubMed  Google Scholar 

  55. K. Krogh, P. Christensen, S. Laurberg, Colorectal symptoms in patients with neurological diseases. Acta Neurol. Scand. 103(6), 335–343 (2001)

    Article  CAS  PubMed  Google Scholar 

  56. N.R. Bhatt, L. Murchison, G. Yardy, Bladder bowel dysfunction in children with Down’s syndrome. Pediatr. Surg. Int. 36(7), 763–772 (2020)

    Article  PubMed  Google Scholar 

  57. L. Szymanski, B.H. King, Practice parameters for the assessment and treatment of children, adolescents, and adults with mental retardation and comorbid mental disorders. American Academy of Child and Adolescent Psychiatry Working Group on Quality Issues. J. Am. Acad. Child Adolesc. Psychiatry 38(12 Suppl), 5S–31S (1999)

    Article  CAS  PubMed  Google Scholar 

  58. A. von Gontard, M. Equit, Comorbidity of ADHD and incontinence in children. Eur. Child Adolesc. Psychiatry 24(2), 127–140 (2015)

    Article  Google Scholar 

  59. K. Dhondt et al., Sleep fragmentation and increased periodic limb movements are more common in children with nocturnal enuresis. Acta Paediatr. 103(6), 268–272 (2014)

    Article  Google Scholar 

  60. K. Dhondt et al., Abnormal sleep architecture and refractory nocturnal enuresis. J. Urol. 182(4 Suppl), 1961–1965 (2009)

    Article  PubMed  Google Scholar 

  61. K. Dhondt et al., Sleep fragmentation and periodic limb movements in children with monosymptomatic nocturnal enuresis and polyuria. Pediatr. Nephrol. 30(7), 1157–1162 (2015)

    Article  PubMed  Google Scholar 

  62. J.E. Jan, R.D. Freeman, Melatonin therapy for circadian rhythm sleep disorders in children with multiple disabilities: what have we learned in the last decade? Dev. Med. Child Neurol. 46(11), 776–782 (2004)

    Article  PubMed  Google Scholar 

  63. C.J. Newman, M. O’Regan, O. Hensey, Sleep disorders in children with cerebral palsy. Dev. Med. Child Neurol. 48(7), 564–568 (2006)

    Article  PubMed  Google Scholar 

  64. S. Kotagal, V.P. Gibbons, J.A. Stith, Sleep abnormalities in patients with severe cerebral palsy. Dev. Med. Child Neurol. 36(4), 304–311 (1994)

    Article  CAS  PubMed  Google Scholar 

  65. L.E. Svedberg et al., Parental perception of cold extremities and other accompanying symptoms in children with cerebral palsy. Eur. J. Paediatr. Neurol. 12(2), 89–96 (2008)

    Article  PubMed  Google Scholar 

  66. A.J. Wright, O. Fletcher, D. Scrutton, G. Baird, Bladder and bowel continence in bilateral cerebral palsy: a population study. J. Pediatr. Urol. 12, 381–383 (2016)

    Article  Google Scholar 

  67. P.F. Austin et al., The standardization of terminology of lower urinary tract function in children and adolescents: update report from the Standardization Committee of the International Children’s Continence Society. J. Urol. 191(6), 1863–1865 (2014)

    Article  PubMed  Google Scholar 

  68. J.F. Martinez-Lage et al., Midline cutaneous lumbosacral lesions: not always a sign of occult spinal dysraphism. Childs Nerv. Syst. 22(6), 623–627 (2006)

    Article  PubMed  Google Scholar 

  69. S.B. Bauer, R.J.M. Nijman, B.A. Drzewiecki, U. Sillen, P. Hoebeke, International Children’s Continence Society standardization report on urodynamic studies of the lower urinary tract in children. Neurourol. Urodyn. 34, 640–647 (2015)

    Article  PubMed  Google Scholar 

  70. P. Rosier, W. Schaefer, G. Lose, et al., International Continence Society Good Urodynamic Practices and Terms 2016: Urodynamics, uroflowmetry, cystometry, and pressure-flow study. Neurourol. Urodyn. 36, 1243–1260 (2017)

    Article  PubMed  Google Scholar 

  71. S.J. Chang et al., Treatment of daytime urinary incontinence: a standardization document from the International Children’s Continence Society. Neurourol. Urodyn. 36(1), 43–50 (2017)

    Article  PubMed  Google Scholar 

  72. S. Vande Velde, K. Van Renterghem, M. Van Winkel, R. De Bruyne, S. Van Biervliet, Constipation and fecal incontinence in children with cerebral palsy. Overview of literature and flowchart for a stepwise approach. Acta Gastroenterol. Belg. 81, 415–418 (2018)

    CAS  PubMed  Google Scholar 

  73. R.E. Burgers et al., Management of functional constipation in children with lower urinary tract symptoms: report from the Standardization Committee of the International Children’s Continence Society. J. Urol. 190(1), 29–36 (2013)

    Article  PubMed  Google Scholar 

  74. I. Franco, A. von Gontard, M. De Gennaro, Evaluation and treatment of nonmonosymptomatic nocturnal enuresis: a standardization document from the International Children's Continence Society. J. Pediatr. Urol. 9(2), 234–243 (2013)

    Article  PubMed  Google Scholar 

  75. E. Van Laecke et al., The daytime alarm: a useful device for the treatment of children with daytime incontinence. J. Urol. 176(1), 325–327 (2006)

    Article  PubMed  Google Scholar 

  76. G.E. Lancioni, I. Van Bergen, F. Furniss, Urine alarms and prompts for fostering daytime urinary continence in a student with multiple disabilities: a replication study. Percept. Mot. Skills 94(3 Pt 1), 867–870 (2002)

    Article  CAS  PubMed  Google Scholar 

  77. R.J. Nijman, Role of antimuscarinics in the treatment of nonneurogenic daytime urinary incontinence in children. Urology 63(3 Suppl 1), 45–50 (2004)

    Article  PubMed  Google Scholar 

  78. K. McKeage, Propiverine: a review of its use in the treatment of adults and children with overactive bladder associated with idiopathic or neurogenic detrusor overactivity, and in men with lower urinary tract symptoms. Clin. Drug. Investig. 33(1), 71–91 (2013)

    Article  CAS  PubMed  Google Scholar 

  79. T. Neveus et al., Evaluation of and treatment for monosymptomatic enuresis: a standardization document from the International Children’s Continence Society. J. Urol. 183(2), 441–447 (2010)

    Article  PubMed  Google Scholar 

  80. X. Game et al., Botulinum toxin-A (Botox) intradetrusor injections in children with neurogenic detrusor overactivity/neurogenic overactive bladder: a systematic literature review. J. Pediatr. Urol. 5(3), 156–164 (2009)

    Article  PubMed  Google Scholar 

  81. P. Hoebeke et al., The effect of botulinum-A toxin in incontinent children with therapy resistant overactive detrusor. J. Urol. 176(1), 328–330 (2006)

    Article  CAS  PubMed  Google Scholar 

  82. Y.F. Rawashdeh et al., International Children’s Continence Society’s recommendations for therapeutic intervention in congenital neuropathic bladder and bowel dysfunction in children. Neurourol. Urodyn. 31(5), 615–620 (2012)

    Article  CAS  PubMed  Google Scholar 

  83. J.C. Kupferman, C.M. Druschel, G.S. Kupchik, Increased prevalence of renal and urinary tract anomalies in children with Down syndrome. Pediatrics 124(4), 615–621 (2009)

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Urology, University Medical Centre Groningen, Groningen, The Netherlands

    Rien J. M. Nijman

  2. Department of Urology, University Hospital Ghent, Ghent, Belgium

    Erik van Laecke

  3. Department of Neuro-Urology, Bambino Gesù Children’s Hospital, Rome, Italy

    Giovanni Mosiello

Authors
  1. Rien J. M. Nijman
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Erik van Laecke
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Giovanni Mosiello
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Rien J. M. Nijman .

Editor information

Editors and Affiliations

  1. Department of Urology Department of Neurourology, China Rehabiliation Research Center China Rehabilitation Science InstituteCapital Medical University, Beijing, China

    Limin Liao

  2. Department of Urology, Medical University Innsbruck, University Hospital, Innsbruck, Austria

    Helmut Madersbacher

Rights and permissions

Reprints and permissions

Copyright information

© 2023 Springer Nature Singapore Pte Ltd.

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Nijman, R.J.M., van Laecke, E., Mosiello, G. (2023). Neurogenic LUT symptoms in Children with Rare Diseases. In: Liao, L., Madersbacher, H. (eds) Handbook of Neurourology. Springer, Singapore. https://doi.org/10.1007/978-981-99-1659-7_80

Download citation

  • DOI: https://doi.org/10.1007/978-981-99-1659-7_80

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-99-1658-0

  • Online ISBN: 978-981-99-1659-7

  • eBook Packages: MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation