Causes of death among people with myelomeningocele: A multi-institutional 47-year retrospective study

Author:

Szymanski Konrad M.1,Adams Cyrus M.1,Alkawaldeh Mohammad Y.2,Austin Paul F.3,Bowman Robin M.4,Castillo Heidi5,Castillo Jonathan5,Chu David I.6,Estrada Carlos R.7,Fascelli Michele8,Frimberger Dominic C.9,Gargollo Patricio C.10,Hamdan Dawud G.11,Hecht Sarah L.12,Hopson Betsy13,Husmann Douglas A.10,Jacobs Micah A.14,MacNeily Andrew E.15,McLeod Daryl J.16,Metcalfe Peter D.17,Meyer Theresa6,Misseri Rosalia1,O’Neil Joseph18,Rensing Adam J.9,Routh Jonathan C.19,Rove Kyle O.12,Sawin Kathleen J.20,Schlomer Bruce J.14,Shamblin Isaac13,Sherlock Rebecca L.2,Slobodov Gennady9,Stout Jennifer21,Tanaka Stacy T.22,Weiss Dana A.11,Wiener John S.19,Wood Hadley M.8,Yerkes Elizabeth B.6,Blount Jeffrey13

Affiliation:

1. Division of Pediatric Urology, Riley Hospital for Children at Indiana University Health, Indianapolis, IN, USA

2. Spina Bifida Center, Boston Children’s Hospital, Boston, MA, USA

3. Division of Urology, Texas Children’s Hospital, Houston, TX, USA

4. Division of Neurosurgery, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA

5. Section of Developmental Pediatrics, Texas Children’s Hospital, Houston, TX, USA

6. Division of Urology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA

7. Department of Urology, Boston Children’s Hospital, Boston, MA, USA

8. Department of Urology, Glickman Urological and Kidney Institute Cleveland Clinic Foundation, Cleveland, OH, USA

9. Department of Urology, Oklahoma University Medical Center, Oklahoma City, OK, USA

10. Division of Urology, Mayo Clinic, Rochester, MN, USA

11. Division of Urology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

12. Department of Pediatric Urology, Children’s Hospital Colorado, Aurora, CO, USA

13. Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, AL, USA

14. Department of Urology, University of Texas Southwestern Medical Center, Dallas, TX, USA

15. Division of Pediatric Urology, British Columbia Children’s Hospital, Vancouver, BC, Canada

16. Section of Pediatric Urology, Nationwide Children’s Hospital, Columbus, OH, USA

17. Department of Surgery, University of Alberta, Edmonton, Canada

18. Department of Pediatrics, Riley Hospital for Children at Indiana University Health, Indianapolis, IN, USA

19. Division of Urologic Surgery, Duke University, Durham, NC, USA

20. Department of Nursing Research and Evidence-Based Practice, Children’s Hospital of Wisconsin, Milwaukee, WI, USA

21. Surgery Office of Clinical Research, Duke University, Durham, NC, USA

22. Division of Pediatric Urology, Vanderbilt University Medical Center, Nashville, TN, USA

Abstract

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.

Publisher

IOS Press

Subject

Rehabilitation,Physical Therapy, Sports Therapy and Rehabilitation,Pediatrics, Perinatology and Child Health

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