Tülay BECERİR, İlknur GİRİŞGEN, Selçuk YÜKSEL

Spinal disrafizmli çocuklarda üriner sistem bulgularının değerlendirilmesi

Amaç: Bu çalışmada Pamukkale Üniversitesi Çocuk Nefroloji Kliniği’nde ‘spinal disrafizm' nedeniyle izlenen çocuk hastaların üriner sistem bulgularının retrospektif olarak değerlendirilerek, böbrek hasarı sıklığı ve olası risk faktörlerini değerlendirmek amaçlanmıştır.Gereç ve yöntem: Ocak 2014 ile Ağustos 2021 tarihleri arasında ‘spinal disrafizme bağlı nörojen mesane’ tanısı ile izlenen çocuk hastaların verileri retrospektif olarak değerlendirildi. Hastaların demografik ve klinik özellikleri, temiz aralıklı katater (TAK) tedavisi alıp almadığı, alıyorsa başlanma zamanı ve son kontroldeki üriner sistem ultrasanografi bulguları (hidronefroz ve mesane duvar kalınlığı), vezikoüreteral reflü (VUR) varlığı, Dimerkaptosüksinik asit (DMSA) sintigrafi bulguları, Shwartz’a göre tahimini glomeruler filtrasyon hızı (eGFR) düzeyleri kayıt edildi.Bulgular: Çalışmaya 17 erkek, 27 kız olmak üzere toplam 44 hasta dahil edildi. Ortalama yaş 7,2±4,2 yaş olarak bulundu. Çocukların 20’si (%45,5) 5 yıldan uzun süredir takip edilmekteydi. Olgular arasında meningomyelosel (%88,6) en çok saptanan defekt olarak bulundu. Spinal disrafizme yönelik operasyon geçirmeyen sadece 2 hasta vardı. Olguların yaklaşık %90’ı 1 yaşından önce opere edilmişti. Hastaların yaklaşık %40’ında DMSA sintigrafide böbrekte hasar, %52’sinde hidronefroz, yaklaşık %16’sında ise eGFR’nin düşük olduğunu saptandı. Ürodinami bulgularında 18 (%40,9) hastada yüksek basınçlı mesane (mesane basıncı >40 cmH2O), 11 (%25) hastada arefleks mesane, 13 (%29,5) hastada normal ürodinami bulguları vardı. Onbeş (%34,1) hastada VUR vardı. Operasyon zamanı, TAK başlama yaşı ve gergin kord gelişimi ile böbrekte hasarlanma arasında anlamlı ilişki saptanmadı.Sonuç: Spinal disrafizm hastalarının ürolojik problemlerinin yönetiminde temel hedef üst üriner sistemin korunmasıdır. Bu hastalarda ürolojik problemlerin erken tanınıp tedavilerin başlanması yönünde öneriler olsa da halen bu hastaların yönetimi tartışmalı ve zordur.

Anahtar Kelimeler:

Spinal disrafizm, nörojen mesane, çocuk, meningomiyelosel

Evaluation of urinary system findings in children with spinal dysraphism

Purpose: It was aimed to evaluate the frequency of kidney damage and possible risk factors by retrospectively evaluating the urinary system findings of pediatric patients followed up for 'spinal dysraphism' in Pamukkale University Pediatric Nephrology Clinic.Materials and methods: The data of pediatric patients who were followed up with the diagnosis of neurogenic bladder due to spinal dysraphism between January 2014 and August 2021 were evaluated retrospectively. Demographic and clinical characteristics of the patients, whether they received clean intermittent catheter (CIC) treatment, if so, when to start and at the last follow-up, urinary system ultrasound findings (hydronephrosis and bladder wall thickness), presence of vesicoureteral reflux (VUR), dimercaptosuccinic acid scintigraphy findings, according to Shwartz Glomerular filtration rate (eGFR) levels were recorded.Results: A total of 44 patients, 17 male and 27 female, were included in the study. The mean age was 7.2±4.2 years. Twenty (45.5%) of the children were followed for more than 5 years. Among the cases, meningomyelocele (88.6%) was found to be the most common defect. There were only 2 patients who did not undergo surgery for spinal dysraphism. Approximately 90% of the cases were operated before 1 year of age. Approximately 40% of the patients were found to have kidney damage on DMSA scintigraphy, 52% had hydronephrosis, and approximately 16% had low eGFR. In urodynamic findings, 18 (40.9%) patients had high pressure bladder (bladder pressure >40 cmH2O), 11 (25%) patients had areflex bladder, 13 (29.5%) patients had normal urodynamic findings. Fifteen (34.1%) patients had VUR. There was no significant relationship between operation time, age at onset of CIC, tethered cord development and kidney damage.Conclusion: The main goal in the management of urological problems in patients with spinal dysraphism is the protection of the upper urinary tract. Although there are suggestions for early diagnosis and treatment of urological problems in these patients, the management of these patients is still controversial and difficult.

Keywords:

Spinal dysraphism, neurogenic bladder, child, meningomyelocele,

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1. Copp AJ, Adzick NS, Chitty LS, Fletcher JM, Holmbeck GN, Shaw GM. Spina bifida. Nat Rev Dis Primers. 2015;1:15007. https://doi.org/10.1038/nrdp.2015.7
2. Tunçbilek E. Türkiye’deki yüksek nöral tüp defekti sıklığı ve önlemek için yapılabilecekler. Çocuk Sağlığı ve Hastalıkları Dergisi 2004:47;79-84.
3. Le HK, Cardona-Grau D, Chiang G. Evaluation and Long-term Management of Neurogenic Bladder in Spinal Dysraphism. Neoreviews 2019;20:e711-e724. https://doi.org/10.1542/neo.20-12-e711
4. Proesmans W. The neurogenic bladder: introducing four contributions. Pediatr Nephrol 2008;23:537-40. https://doi.org/10.1007/s00467-008-0768-3
5. Cardona-Grau D, Chiang G. Evaluation and Lifetime Management of the Urinary Tract in Patients with Myelomeningocele. Urol Clin North Am 2017;44:391-401. https://doi.org/10.1016/j.ucl.2017.04.006 6. Moldenhauer JS, Adzick NS. Fetal surgery for myelomeningocele: After the Management of Myelomeningocele Study (MOMS). Semin Fetal Neonatal Med 2017;22:360-66. https://doi.org/10.1016/j.siny.2017.08.004
7. Parizi JLG, Leal da Cruz M, et al. Comparative Analysis of Bladder Pattern of Patients who Underwent In Utero Versus Postnatal Myelomeningocele Repair. J Urol 2020;203:194-9. https://doi.org/10.1097/JU.0000000000000521
8. Taşkapılıoğlu MÖ, Türedi B, Altunyuva O, Utanğaç MM, Balkan ME, Kılıç N. Retrospective analysis of early- and late-operated meningomyelocele patients. Childs Nerv Syst 2021;37:539-43. https://doi.org/10.1007/s00381-020-04860-8
9. Clayton DB, Tanaka ST, Trusler L, Thomas JC, Pope JC 4th, Adams MC, Brock JW 3rd. Long-term urological impact of fetal myelomeningocele closure. J Urol 2011;186:1581-5. https://doi.org/10.1016/j.juro.2011.04.005
10. Snow-Lisy DC, Yerkes EB, Cheng EY. Update on Urological Management of Spina Bifida from Prenatal Diagnosis to Adulthood. J Urol 2015;194:288-96. https://doi.org/10.1016/j.juro.2015.03.107
11. Klose AG, Sackett CK, Mesrobian HG. Management of children with myelodysplasia: urological alternatives. J Urol 1990;144:1446-9. https://doi.org/10.1016/s0022-5347(17)39763-x
12. Park JM, Bauer SB, Freeman MR, Peters CA. Oxybutynin chloride inhibits proliferation and suppresses gene expression in bladder smooth muscle cells. J Urol 1999;162:1110-4. https://doi.org/10.1097/00005392-199909000-00048
13. Teichman JM, Scherz HC, Kim KD, Cho DH, Packer MG, Kaplan GW. An alternative approach to myelodysplasia management: aggressive observation and prompt intervention. J Urol 1994;152:807-11. https://doi.org/10.1016/s0022-5347(17)32716-7
14. Girişgen İ, Yüksel S, Akbulut EE, Karcılı Yalçın K, Şenol H. Temiz aralıklı kateterizasyon yapan nörojen mesaneli hastalar ile normal mesaneli ve tekrarlayan idrar yolu enfeksiyonu olan çocukların idrar kültürlerindeki mikroorganizma türleri ve antibiyotik direnç farkları. Pamukkale Tıp Dergisi 2019;12:133-140. https://doi.org/10.31362/patd.472717
15. Hopps CV, Kropp KA. Preservation of renal function in children with myelomeningocele managed with basic newborn evaluation and close followup. J Urol 2003;169:305-8. https://doi.org/10.1097/01.ju.0000040590.35948.bc 16. Woo J, Palazzi K, Dwek J, Kaplan G, Chiang G. Early clean intermittent catheterization may not prevent dimercaptosuccinic acid renal scan abnormalities in children with spinal dysraphism. J Pediatr Urol 2014;10:274-7. https://doi.org/10.1016/j.jpurol.2013.09.001
17. Ab E, Dik P, Klijn AJ, van Gool JD, de Jong TP. Detrusor overactivity in spina bifida: how long does it need to be treated? Neurourol Urodyn 2004;23:685-8. https://doi.org/10.1002/nau.20044
18. Mehta VA, Bettegowda C, Ahmadi SA et al. Spinal cord tethering following myelomeningocele repair. J Neurosurg Pediatr 2010;6:498-505. https://doi.org/10.3171/2010.8
19. Tarcan T, Onol FF, Ilker Y, Simsek F, Ozek M. Does surgical release of secondary spinal cord tethering improve the prognosis of neurogenic bladder in children with myelomeningocele? J Urol 2006;176:1601-6. https://doi.org/10.1016/j.juro.2006.06.036
20. Khoury A. Does surgery improve outcomes for secondary tethering of the spinal cord after myelomeningocele repair? Nat Clin Pract Urol 2007;4:418-9. https://doi.org/10.1038/ncpuro0853
21. Abrahamsson K, Olsson I, Sillén U. Urodynamic findings in children with myelomeningocele after untethering of the spinal cord. J Urol 2007;177:331-4. https://doi.org/10.1016/j.juro.2006.08.146