Clinical Condition of Patients with Neural Tube Defects
Authors:
L. Šabová 1; F. Horn 2; T. Drdulová 3; K. Viestová 4; P. Bartoň 5; M. Kabát 2; L. Kovács 1
Authors place of work:
II. detská klinika Lekárskej fakulty Univerzity Komenského a Detskej fakultnej nemocnice
s poliklinikou v Bratislave, Slovenská republika, prednosta: prof. MUDr. L. Kovács, DrSc., MPH
1; Klinika detskej chirurgie Lekárskej fakulty Univerzity Komenského a Detskej fakultnej nemocnice
s poliklinikou v Bratislave, Slovenská republika, prednosta: doc. MUDr. J. Trnka, CSc.
2; Slovenská spoločnosť pre spina bifida a/alebo hydrocefalus o. z. v Smoleniciach, Slovenská republika
3; Klinika detskej neurológie Lekárskej fakulty Univerzity Komenského a Detskej fakultnej nemocnice
s poliklinikou v Bratislave, Slovenská republika, prednosta: doc. MUDr. P. Sýkora, CSc.
4; Klinika pediatrickej urológie Slovenskej zdravotníckej univerzity a Detskej fakultnej nemocnice
s poliklinikou v Bratislave, Slovenská republika, primár: MUDr. V. Polák, Ph. D.
5
Published in the journal:
Rozhl. Chir., 2010, roč. 89, č. 8, s. 471-477.
Category:
Monothematic special - Original
Summary
Introduction:
Neural tube defects are the most common congenital anomalies of central nervous system. Their onset is at the embryonic age of 21 to 28 days. Periconceptional administration of folic acid may reduce the occurrence of neural tube defects up to 70%. Clinical features depend on localization of the defect and malformation of central nervous system, as well as on intensive multidisciplinary care in early stages of development. Open defects (meningomyelocele) present with more severe neurological deficiency early after birth. Closed defect manifestation occurs usually later in life with tethered cord syndrome.
Purpose:
This study evaluates clinical status of the patients with neural tube defects, who are followed in the Children’s Faculty Hospital in Bratislava
Methods:
Cross-sectional and also retrospective study was conducted by questionnaire that was given to mothers of children with neural tube defect. Data about perinatal and family history, occurrence of hydrocephalus, scoliosis, joint deformities, dysfunction of urinary system and bowel, as well as social care, were collected. Clinical features were statistically evaluated depending on beginning of the defect or locomotion status.
Results:
94 patients with mean age of 12.7 years ± 6.59 SD were included in the study. Patients with open defects had more severe neurological deficiency with hydrocephalus, more common epilepsy, skeleton deformities, wheelchair-dependency, and dysfunction of urinary tract and bowel. Scoliosis and ability of locomotion significantly correlated with higher lever of defect, while hydrocephalus, continence, urinary infections, clean intermittent catheterization, constipation, scoliosis and joint deformities significantly correlated with the ability of locomotion (p < 0.05).
Conclusion:
For patients with neural tube defects, the level of neurological deficiency is the most important prognostic factor for many other clinical characteristics.
Key words:
neural tube defects – clinical features – neurological deficiency – Slovak population – spina bifida
Zdroje
1. McComb, J. G. Spinal and cranial neural tube defects. Semin. Pediatr. Neurol., 1997; 4: 156–166.
2. Horn, F. Spina bifida. Kaudálne defekty neurálnej rúry. 1. vydanie. Prešov, VMV, 2005.
3. Danielsson, A. J., Bartonek, A., Levey, E., McHalle, K., Sponseller, P., Saraste, H. Associations between orthopaedic findings, ambulation and health-related quality of life in children with myelomeningocele. J. Child. Orthop., 2008; 2: 45–54.
4. Oktem, I. S., Menku, A., Ozdemir, A. When Should Ventriculoperitoneal Shunt Placement Be Performed in Cases with Myelomeningocele and hydrocephalus? Turk. Neurosur., 2008; 18: 387–391.
5. Woodhouse, C. R. J. Myelomeningocele: neglected aspects. Pediatr. Nephrol., 2008; 23: 1223–1231.
6. Horn, F., Babala, J., Smrek, M., Hajnovič, Ľ., Pekarovič, E., Siman, J. V akom veku operovať pacientov so spinálnym lipómom? Čes.-slov. Pediat., 1999; 54: 632–634.
7. Barkovich, A. J. Pediatric neuroimaging. 4th edn. Philadelphia, Lippincott Williams and Wilkkins, 2005.
8. Trumble, E. Chiari malformation clinical quandaries: When to operate. First World Congress on Spina Bifida Research and Care. Orlando, March 15-18 2009. Neurosurgery Abstracts. Dostupný na www:
http://medicalconference.spinabifidaassociation.org/atf/cf/%-7B10221c89-6b69-45bd-81bf-3194b0be6fa5%7D/NEUROSU-R04.PDF
9. Tubbs, R. S., Oakes, W. J. Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs. Nerv. Sys., 2004; 20: 375–381.
10. Vandertop, W. P., Hoffman, H. J., Drake, J. M., Humphreys, R. P., Rutka, J. P., Becker, L. E. Surgical decompression for symptomatic chiari II malformation in neonates with myelomeningocele. J. Neurosurg., 1992; 77: 541–744.
11. Verpoorten, C., Buyse, G. M. The neurogenic bladder: medical treatment. Pediatr. Nephrol., 2008; 23: 717–725.
12. Kass, E. J., Koff, S. A., Diokno, A. C., Lapides, J. The significance of bacilluria in children on long-term intermittent catheterization. J. Urol., 1981; 126: 223–225.
13. Perrouin-Verbe, B., Labat, J. J., Richard, I., Mauduyt de la Greve, I., Buzelin, J.M., Mathe, J. F. Clean intermittent catheterization from the acute period in spinal cord injury patients. Long term evaluation of uretheral and genital tolerance. Paraplegia, 1995; 33: 619–624.
14. Spindel, M. R., Bauer, S. B., Dyro, F. M., Krarup, C., Koshbin, S., Winston, K. R., Lebowitz, R. L., Colodny, A. H., Retik, A. B. The changing neurourologic lesion in myelodysplasia. JAMA. 1987; 258: 1630–1633.
15. Valanský, L., Kurcinová, Z., Sádová, E., Podracká, Ľ. Urologické komplikácie detí s myelomeningokélou v prvom roku života. Klin. urol., 2005; 1: 52–55.
16. Johnsen, V., Skattebu, E., Aamot-Andersen, A., Thyberg, M. Problematic aspects of faecal incontinence according to the experience of adults with spina bifida. J. Rehabil. Med., 2009; 41: 506–511.
17. Kajbafzadeh, A., Mahboubi, A. H., Pavabvash, S. Concomitant repeated intravesical injections of botulinum toxin – type A and laparoscopic antegrade continence enema; a new solution for an old problem. BJU Int., 2009; 103: 1248–1254.
18. van de Velde, S., van Biervliet, S., van Renterghem, K., van Laecke, E., Hoebeke, P., van Winckel, M. Achieving fecal continence in patients with spina bifida: a descriptive cohort study. J. Urol., 2007; 178: 2640–2644.
19. Hoffer, M., Feiwell, E., Perry, R., Perry, J., Bonnet, Ch. Functional Ambulation in Patients with myelomeningocele. J. Bone Joint Surg., 1973; 55: 137–148.
20. Specht, E. E. Myelomeningocele. Part I. Orthopedic management in children. West. J. Med., 1974; 121: 281–291.
21. Danzer, E., Finkel, R. S., Rintoul, N. E., Bebbington, M. W., Schwartz, E. S., Zarnow, D. M., Adzick, N. S., Johnson, M. P. Reversal of hindbrain herniation after maternal-fetal surgery for myelomeningocele subsequently impacts on brain stem function. Neuropediatrics, 2008; 39: 369–362.
22. Gregor, V., Šípek, A., Horáček, J., Šípek, A. jr., Langhammer, P. Prenatální diagnostika vybraných typů vrozených vývojových vad v České republice: 1994–2006. Čes. Gynek. 2008; 73: 169–178.
23. Wilson, R. D., Johnson, J. A., Wyatt, P., Allen, V., Gagnon, A., Langlois, S., Blight, C., Audibert, F., Désilets, V., Brock, J. A., Koren, G., Goh, I., Nguyen, P., Kapur, B. Pre-conceptional Vitamin/Folic Acid Supplementation 2007: The Use of Folic Acid in Combination With a Multivitamin Supplement for the Prevention of Neural Tube Defects and Other Congenital Anomalies. J. Obstet. Gynaecol. Can., 2007; 29: 1003–1013.
24. Kovács, L. Prevencia vrodených vývojových chýb. Pediatr. prax., 2008; 2: 63.
25. Behunová, J., Podracká, Ľ. Rázštepy nervovej trubice – súčasné pohľady na etiopatogenézu a možnosti prevencie kyselinou listovou. Čes.-slov. Pediat., 2008; 68: 38–45.
26. Rendeli, C., Ausili, E., Tabacco, F., Caliandro, P., Aprile, I., Tonali, P., Salvaggio, E., Padua, L. Assessment of health status in children with spina bifida. Spinal. Cord. 2005; 43: 230–235.
Štítky
Surgery Orthopaedics Trauma surgeryČlánok vyšiel v časopise
Perspectives in Surgery
2010 Číslo 8
- Metamizole at a Glance and in Practice – Effective Non-Opioid Analgesic for All Ages
- Metamizole vs. Tramadol in Postoperative Analgesia
- Spasmolytic Effect of Metamizole
Najčítanejšie v tomto čísle
- Syndroma Caudae Equinae of Rare Etiology
- Possibilities and Results of Surgical Treatment of Benign Tracheoesophageal Fistula
- How Long to Wait with Operations for Necrotizing Pancreatitis?
- Urgent Thyroidectomy for a Mechanical Syndrome Caused by a Toxic Nodular Goiter – A Case Report