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Tethered cord in patients affected by anorectal malformations: a survey from the ARM-Net Consortium

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Abstract

Purpose

The goal of this study was to determine the degree of consensus in the management of spinal cord tethering (TC) in patients with anorectal malformation (ARM) in a large cohort of European pediatric centers.

Methods

A survey was sent to pediatric surgeons (one per center) members of the ARM-Net Consortium.

Results

Twenty-four (86%) from ten different countries completed the survey. Overall prevalence of TC was: 21% unknown, 46% below 15, and 29% between 15 and 30%. Ninety-six agreed on screening all patients for TC regardless the type of ARM and 79% start screening at birth. Responses varied in TC definition and diagnostic tools. Fifty percent of respondents prefer ultrasound (US), 21% indicate either US or magnetic resonance (MRI) based on a pre-defined risk of presenting TC, and 21% perform both. Discrepancy exists in complementary test: 82% carry out urodynamic studies (UDS) and only 37% perform somatosensory-evoked potentials (SSEP). Prophylactic untethering is performed in only two centers (8%).

Conclusions

Survey results support TC screening in all patients with ARM and conservative management of TC. There is discrepancy in the definition of TC, screening tools, and complementary test. Protocols should be developed to avoid such variability in management.

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References

  1. Lew SM, Kothbauer KF (2007) Tethered cord syndrome: an update review. Pediatr Neurosurg 43:236–248

    Article  PubMed  Google Scholar 

  2. van den Hondel D, Sloots C, de Jong TH, Lequin M, Wijnen R (2015) Screening and treatment of tethered spinal cord in anorectal malformation patients. Eur J Pediatr Surg 26(1):22–28

    Article  PubMed  Google Scholar 

  3. Uchida K, Inoue M, Matsubara T, Otake K, Koike Y, Okugawa Y, Kawamoto A, Miki C, Kusunoki M (2007) Evaluation and treatment for spinal cord tethering in patients with anorectal malformation. Eur J Pediatr Surg 17(6):408–411

    Article  CAS  PubMed  Google Scholar 

  4. Levitt MA, Peña A (2005) Outcomes from the correction of anorectal malformations. Curr Opin Pediatr 17(3):394–401

    Article  PubMed  Google Scholar 

  5. Di Cesare A, Leva E, Macchini F, Canazza L, Carrabba G, Fumagalli M, Mosca F, Torricelli M (2010) Anorectal malformations and neurospinal dysraphism: is this association a major risk for continence? Pediatr Surg Int 26(11):1077–1081

    Article  PubMed  Google Scholar 

  6. Borg HC, Holmdahl G, Gustavsson K, Doroszkiewicz M, Sillén U (2013) Longitudinal study of bowel function in children with anorectal malformation. J Pediatr Surg 48(3):597–606

    Article  PubMed  Google Scholar 

  7. Kyrklund K, Pakarinen MO, 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

    Article  PubMed  Google Scholar 

  8. Totonelli G, Morini F, Catania VD, Schingo PM, Mosiello G, Palma P, Iacobelli BD, Bagolan P (2016) Anorectal malformations associated spinal cord anomalies. Pediatr Surg Int 32(8):729–735

    Article  PubMed  Google Scholar 

  9. 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

    Article  PubMed  Google Scholar 

  10. 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

    Article  CAS  PubMed  Google Scholar 

  11. Teo AT, Gan BK, Tung JS, Low Y, Seow WT (2012) Low-lying spinal cord and tethered cord syndrome in children with anorectal malformations. Singap Med J 53(9):570–576

    Google Scholar 

  12. Miyasaka M, Nosaka S, Kitano Y, Ueoka K, Tsutsumi Y, Kuroda T, Honna T (2009) Utility of spinal MRI in children with anorectal malformation. Pediatr Radiol 39(8):810–816

    Article  PubMed  Google Scholar 

  13. Podberesky DJ, Towbin AJ, Eltomey MA, Levitt MA (2013) Magnetic resonance imaging of anorectal malformations. Magn Reson Imaging Clin N Am 21(4):791–812

    Article  PubMed  Google Scholar 

  14. Taskinen S, Valanne L, Rintala R (2002) Effect of spinal cord abnormalities on the function of the lower urinary tract in patients with anorectal abnormalities. J Urol 168(3):1147–1149

    Article  PubMed  Google Scholar 

  15. Frainey B, Yerkes E, Menon V, Gong E, Meyer T, Bowman R, McLone D, Cheng E (2014) Predictors of urinary continence following tethered cord release in children with occult spinal dysraphism. J Pediatr Surg 10:627–633

    Google Scholar 

  16. Bischoff A, Levitt MA, Peña A (2013) Update on the management of anorectal malformations. Pediatr Surg Int 29(9):899–904

    Article  PubMed  Google Scholar 

  17. Borg H, Holmdahl G, Olsson I, Wiklund LM, Sillén U (2009) Impact of spinal cord malformation on bladder function in children with anorectal malformations. J Pediatr Surg 44(9):1778–1785

    Article  PubMed  Google Scholar 

  18. 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

    Article  PubMed  Google Scholar 

  19. Kim SM, Chang HK, Lee MJ, Shim KW, Oh JT, Kim DS, Kim MJ, Han SJ (2010) Spinal dysraphism with anorectal malformation: lumbosacral magnetic resonance imaging evaluation of 120 patients. J Pediatr Surg 45(4):769–776

    Article  PubMed  Google Scholar 

  20. Golonka NR, Haga LJ, Keating RP, Eichelberger MR, Gilbert JC, Hartman GE, Powell DM, Vezina G, Newman KD (2002) Routine MRI evaluation of low imperforate anus reveals unexpected high incidence of tethered spinal cord. J Pediatr Surg 37(7):966–969

    Article  PubMed  Google Scholar 

  21. Chern JJ, Aksut B, Kirkman JL, Shoja MM, Tubbs RS, Royal SA, Wellons JC 3rd, Rozzelle CJ (2012) The accuracy of abnormal lumbar sonography findings in detecting occult spinal dysraphism: a comparison with magnetic resonance imaging. J Neurosurg Pediatr 10(2):150–153

    Article  PubMed  Google Scholar 

  22. Tsuji H, Okada A, Nakai H, Azuma T, Yagi M, Kubota A (2002) Follow-up studies of anorectal malformations after posterior sagittal anorectoplasty. J Pediatr Surg 37(11):1529–1533

    Article  PubMed  Google Scholar 

  23. Eltomey MA, Donnelly LF, Emery KH, Levitt MA, Peña A (2008) Postoperative pelvic MRI of anorectal malformations. AJR Am J Roentgenol 191(5):1469–1476

    Article  PubMed  Google Scholar 

  24. Mosiello G, Capitanucci ML, Gatti C, Adorisio O, Lucchetti MC, Silveri M, Schingo PS, De Gennaro M (2003) How to investigate neurovesical dysfunction in children with anorectal malformations. J Urol 170(4 Pt 2):1610–1613

    Article  PubMed  Google Scholar 

  25. Tsuda T, Iwai N, Kimura O, Kubota Y, Ono S, Sasaki Y (2007) Bowel function after surgery for anorectal malformations in patients with tethered spinal cord. Pediatr Surg Int 23(12):1171–1174

    Article  PubMed  Google Scholar 

  26. Suppiej A, Dal Zotto L, Cappellari A, Traverso A, Castagnetti M, Drigo P, Midrio P (2009) Tethered cord in patients with anorectal malformation: preliminary results. Pediatr Surg Int 25(10):851–855

    Article  CAS  PubMed  Google Scholar 

  27. Morimoto K, Takemoto O, Wakayama A (2003) Tethered cord associated with anorectal malformation. Pediatr Neurosurg 38(2):79–82

    Article  PubMed  Google Scholar 

  28. Severino M, Manara R, Faggin R, Dalle Nogare C, Gamba P, Midrio P (2008) Anorectal malformation and spinal dysraphism: the value of diffusion-weighted imaging in detecting associated intradural (epi)dermoid cyst. J Pediatr Surg 43(10):1935–1938

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

Aminoff: AIMAR (patient-organization), Italy; Bagolan: Ospedale Bambino Gesú-Roma-Ped. Surgery, Italy; De Blaauw: Radboudumc Nijmegen, Netherlands; Deluggi: Kepler Universitätsklinikum GmbH-Linz, Austria; Fanjul: Hospital Gregorio Maranon-Madrid, Spain; Fascetti Leon: University of Padua-Pediatric surgery, Italy; García: University Hospital 12 de Octubre, Madrid, Spain; Giné: Hospital Vall d’Hebron-Barcellona, Spain; Giuliani: St.George’s University Hospitals NHS Foundation Trust-London, UK; Grano: University Sapienza-Rome, Italy; Grasshoff-Derr: Buergerhospital and Clementine Childrens Hospital-Frankfurt, Germany; Haanen: VA (patient organization), Netherlands; Holland-Cunz: University Children’s Hospital, Basel, Switzerland; Jenetzky: DKFZ-Uni Heidelberg, Germany; Lacher: Universitätsklinikum Leipzig, Germany; Leva: Policlinico Milano-Pediatric Surgery, Italy; Lisi: Santo Spirito” Civil Hospital of Pescara, Italy; Makedonsky: Children’s Hospital Dnepropetrovsk, Ukraine; Marcelis: Radboudumc Nijmegen, Netherlands; Midrio: Ospedale Ca’ Foncello, Treviso, Italy; Miserez: UZ Leuven-Dept. of Abdominal Surgery, Belgium; Ozen: Gazi University Faculty of Medicine Department of Pediatric Surgery Ankara, Turkey; Percin: Gazi University Faculty of Medicine Department of Medical Genetics, Turkey; Reutter: Cure-Net University of Bonn, Germany; Rohleder: Universitat Medizin Mainz, Germany; Samuk: Department of Pediatric and Adolescent Surgery, Schneider Children’s Medical Center-Petach Tikva, Israel; Schmiedeke: Klinikum Bremen Mitte, Germany; Schwarzer: SoMA e.V.-Munich (patient organization), Germany; Sloots: Sophia Children’s Hospital-Erasmus Medical Centre Rotterdam, Netherlands; Stenström: Lund University, Skane University Hospital, Sweden; Till: Universitats Klinik fur Kinder-Graz, Austria; Van der Steeg: Children’s Hospital AMC and VU University , Netherlands; Van Rooij: Radboudumc Nijmegen, dept Health Evidence, Netherlands; Volk: Klinik für Chirurgie, Universitätsklinik Heidelberg, Germany; Wester: Department of Pediatric Surgery, Karolinska University Hospital, Sweden; Zwink: DKFZ-Uni Heidelberg, Germany

Author information

Authors and Affiliations

  1. Department of Pediatric Surgery, Gregorio Marañón University General Hospital, Calle del Dr. Esquerdo, 46, 28007, Madrid, Spain

    María Fanjul

  2. Department of Pediatric Surgery, Schneider Children’s Medical Center of Israel, Sackler Medical School, University of Tel Aviv, Tel Aviv, Israel

    I. Samuk

  3. Department of Medical and Surgical Neonatology, Bambino Gesù Children’s Hospital, Rome, Italy

    P. Bagolan

  4. Department of Pediatric Surgery, Fondazione Ca’ Granda-Ospedale Maggiore Policlinico, Milan, Italy

    E. Leva

  5. Department of Pediatric Surgery, Erasmus MC-Sophia Children’s Hospital, Rotterdam, The Netherlands

    C. Sloots

  6. Department of Pediatric Surgery, Vall d’Hebron Hospital, Barcelona, Spain

    C. Giné

  7. Italian Association for Anorectal Malformations, Rome, Italy

    D. Aminoff

  8. Mother and Child Department-Section of Pediatric Surgery, Cá Foncello Hospital, Treviso, Italy

    P. Midrio

Authors
  1. María Fanjul
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  2. I. Samuk
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  3. P. Bagolan
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  4. E. Leva
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  5. C. Sloots
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  6. C. Giné
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  7. D. Aminoff
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  8. P. Midrio
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Consortia

On behalf of the ARM-Net Consortium

  • Dalia Aminoff
  • , Piero Bagolan
  • , Ivo De Blaauw
  • , Stefan Deluggi
  • , María Fanjul
  • , Francesco Fascetti Leon
  • , Araceli García
  • , Carles Giné
  • , Stefano Giuliani
  • , Caterina Grano
  • , Sabine Grasshoff-Derr
  • , Michel Haanen
  • , Stephan Holland-Cunz
  • , Ekkehart Jenetzky
  • , Martin Lacher
  • , Ernesto Leva
  • , Grabiele Lisi
  • , Igor Makedonsky
  • , Carlos Marcelis
  • , Paola Midrio
  • , Marc Miserez
  • , Onur Ozen
  • , Ferda Percin
  • , Heiko Reutter
  • , Stephan Rohleder
  • , Inbal Samuk
  • , Eberhard Schmiedeke
  • , Nicole Schwarzer
  • , Pim Sloots
  • , Pernilla Stenström
  • , Holger Till
  • , Lideke Van der Steeg
  • , Iris Van Rooij
  • , Patrick Volk
  • , Tomas Wester
  •  & Nadine Zwink

Corresponding author

Correspondence to María Fanjul.

Additional information

Members of ARM-Net Consortium are listed in acknowledgements.

Appendix: Current ARM-Net institutions

Appendix: Current ARM-Net institutions

Name

Institution

Country

Aminoff

AIMAR (patient organization)

Italy

Bagolan

Ospedale Bambino Gesú-Roma-Ped. Surgery

Italy

De Blaauw

Radboudumc Nijmegen

Netherlands

Deluggi

Kepler Universitätsklinikum GmbH-Linz

Austria

Fanjul

Hospital Gregorio Maranon-Madrid

Spain

Fascetti Leon

University of Padua-Pediatric surgery

Italy

García

University Hospital 12 de Octubre, Madrid

Spain

Giné

Hospital Vall d’Hebron-Barcellona

Spain

Giuliani

St.George’s University Hospitals NHS Foundation Trust-London

UK

Grano

University Sapienza-Rome

Italy

Grasshoff-Derr

Buergerhospital and Clementine Children Hospital-Frankfurt

Germany

Haanen

VA (patient organization)

Netherlands

Holland-Cunz

University Children’s Hospital, Basel

Switzerland

Jenetzky

DKFZ-Uni Heidelberg

Germany

Lacher

Universitätsklinikum Leipzig

Germany

Leva

Policlinico Milano-Pediatric Surgery

Italy

Lisi

Santo Spirito” Civil Hospital of Pescara

Italy

Makedonsky

Children’s Hospital Dnepropetrovsk

Ukraine

Marcelis

Radboudumc Nijmegen

Netherlands

Midrio

Ospedale Ca’ Foncello, Treviso

Italy

Miserez

UZ Leuven-Dept. of Abdominal Surgery

Belgium

Ozen

Gazi University Faculty of Medicine Department of Pediatric Surgery Ankara

Turkey

Percin

Gazi University Faculty of Medicine Department of Medical Genetics

Turkey

Reutter

Cure-Net University of Bonn

Germany

Rohleder

Universitat Medizin Mainz

Germany

Samuk

Department of Pediatric and Adolescent Surgery, Schneider Children’s Medical Center-Petach Tikva

Israel

Schmiedeke

Klinikum Bremen Mitte

Germany

Schwarzer

SoMA e.V.-Munich (patient organization)

Germany

Sloots

Sophia Children’s Hospital-Erasmus Medical Centre Rotterdam

Netherlands

Stenström

Lund University, Skane University Hospital

Sweden

Till

Universitats Klinik fur Kinder-Graz

Austria

Van der Steeg

Children’s Hospital AMC and VU University

Netherlands

Van Rooij

Radboudumc Nijmegen, dept Health Evidence

Netherlands

Volk

Klinik für Chirurgie, Universitätsklinik Heidelberg

Germany

Wester

Department of Pediatric Surgery, Karolinska University Hospital

Sweden

Zwink

DKFZ-Uni Heidelberg

Germany

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Fanjul, M., Samuk, I., Bagolan, P. et al. Tethered cord in patients affected by anorectal malformations: a survey from the ARM-Net Consortium. Pediatr Surg Int 33, 849–854 (2017). https://doi.org/10.1007/s00383-017-4105-4

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  • DOI: https://doi.org/10.1007/s00383-017-4105-4

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