Abstract
Purpose
To analyze the outcome after untethering surgery in patients with spinal dysraphism (SD), with or without associated anorectal malformation (ARM).
Methods
Patients operated on for SD, with (Group A) or without (Group B) associated ARM (1999–2015), were included. The post-operative outcome was analyzed in the two groups in terms of improving of clinical symptoms (neuro-motor deficits, bladder dysfunction, bowel dysfunction) and of instrumental examinations (urodynamics, bladder ultrasound, neurophysiology). Fisher’s exact test and χ2 test were used as appropriate; p < 0.05 was considered statistically significant.
Main results
Ten patients in Group A and 24 in Group B were consecutively treated. One patient was lost at follow up. Six patients (25%) in Group B underwent prophylactic surgery. The analysis of the pre-operative symptoms in the two groups showed that a significantly higher number of patients in group A needed bowel management and presented with neuro-motor deficits, compared to group B (p = 0.0035 and p = 0.04, respectively). Group A showed a significant post-operative neuro-motor improvement as compared to group B (p = 0.002).
Conclusions
Based on our results, untethering seems to be effective in neuro-motor symptoms in selected patients with ARM. In ARM patients, untethering surgery does not seem to benefit intestinal and urinary symptoms. The presence of the associated ARM does not seem to impact the medium-term outcome of patients operated for SD.
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References
Rittler M, Paz JE, Castilla EE (1996) VACTERL association, epidemiologic definition and delineation. Am J Med Genet 63(4):529–536
Van den Hondel D, Sloots C, de Jong TH, Lequin M, Wijnen R (2016) Screening and treatment of tethered spinal cord in anorectal malformation patients. Eur J Pediatr Surg 26(1):22–28. https://doi.org/10.1055/s-0035-1563673
Totonelli G, Catania VD, Morini F, Fusaro F, Mosiello G, Iacobelli BD, Bagolan P (2015) VACTERL association in anorectal malformation: effect on the outcome. Pediatr Surg Int 31(9):805–808. https://doi.org/10.1007/s00383-015-3745-5.
Tuuha SE, Aziz D, Drake J, Wales P, Kim PC (2004) Is surgery necessary for asymptomatic tethered cord in anorectal malformation patients? J Pediatr Surg 39(5):773–777
Uchida K, Inoue M, Matsubara T, Otake K, Koike Y, Okugawa Y et al (2007) Evaluation and treatment for spinal cord tethering in patients with anorectal malformations. Eur J Pediatr Surg 17(6):408–411
Totonelli G, Morini F, Catania VD, Schingo PM, Mosiello G, Palma P et al (2016) Anorectal malformations associated spinal cord anomalies. Pediatr Surg Int 32(8):729–735
Totonelli G, Messina R, Morini F, Mosiello G, Palma P, Scuglia M et al (2017) Embryological and clinical implications of the association between anorectal malformations and spinal dysraphism. Pediatr Surg Int 33(8):843–847. https://doi.org/10.1007/s00383-017-4104-5
Scottoni F, Iacobelli BD, Zaccara AM, Totonelli G, Schingo AM, Bagolan P (2014) Spinal ultrasound in patients with anorectal malformations: is this the end of an era? Pediatr Surg Int 30(8):829–831. https://doi.org/10.1007/s00383-014-3546-2
Holschneider A, Hutson J, Pen˜a A, Beket E, Chatterjee S, Coran A et al (2005) Preliminary report on the international conference for the development of standards for the treatment of anorectal malformations. J Pediatr Surg 40(10):1521–1526
Levitt MA, Patel M, Rodriguez G, Gaylin DS, Pena A (1997) The tethered spinal cord in patients with anorectal malformations. J Pediatr Surg 32(3):462–468
Kyrklund K, Pakarinen MP, Taskinen S, Kivisaari R, Rintala RJ (2016) Spinal cord anomalies in patients with anorectal malformations without severe sacral abnormalities or meningomyelocele: outcomes after expectant, conservative management. J Neurosurg Spine 25(6):782–789
Fanjul M, Samuk I, Bagolan P, Leva E, Sloots C, Giné C et al (2017) Tethered cord in patients affected by anorectal malformations: a survey from the ARM-Net Consortium. Pediatr Surg Int 33(8):849–854. https://doi.org/10.1007/s00383-017-4105-4
Valentini LG, Selvaggio G, Erbetta A, Cordella R, Pecoraro MG, Bova S et al (2013) Occult spinal dysraphism: lessons learned by retrospective analysis of 149 surgical cases about natural history, surgical indications, urodynamic testing, and intraoperative neurophysiological monitoring. Childs Nerv Syst 29(9):1657–1669. https://doi.org/10.1007/s00381-013-2186-5
Nijman R, Tekgul S, Chase J et al (2017) Diagnosis and management urinary incontinence in childhood. In continence 6th ICS-ICUD edn. pp 959–1092
Mosiello G, Capitanucci ML, Gatti C, Adorisio O, Lucchetti MC, Silveri M et al (2003) How to investigate neurovescical dysfunction in children with anorectal malformations. J Urol 170(4 Pt 2):1610–1613
Morota N, Ihara S, Ogiwara H (2017) New classification of spinal lipomas based on embryonic stage. J Neurosurg Pediatr 19(4):428–439
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Totonelli, G., Messina, R., Morini, F. et al. Impact of the associated anorectal malformation on the outcome of spinal dysraphism after untethering surgery. Pediatr Surg Int 35, 227–231 (2019). https://doi.org/10.1007/s00383-018-4400-8
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DOI: https://doi.org/10.1007/s00383-018-4400-8