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PubMed This is a summary of 17 peer-reviewed journal articles Updated
Pediatrics · VACTERL Association

Beyond the NICU: Long-Term Monitoring and Growth

At a Glance

Children with VACTERL association can lead active lives with proactive long-term care. Key ongoing priorities include monitoring kidney health, managing bowel function after ARM repair, tracking spinal growth for tethered cord issues, and utilizing early intervention for neurodevelopment.

Once the initial surgeries of infancy are complete, the focus for a child with VACTERL association shifts from acute survival to long-term monitoring and optimizing their quality of life. While the path involves ongoing medical vigilance, most children with VACTERL lead full, active lives and report a psychological well-being similar to their peers [1].

Early Intervention and Neurodevelopment

Recent research has shown that children with VACTERL have a higher risk for certain neurodevelopmental conditions compared to the general population [2].

  • Conditions to Watch For: These include ADHD (Attention-Deficit/Hyperactivity Disorder), Autism Spectrum Disorder (ASD), and intellectual disabilities [2].
  • The Power of Early Intervention (EI): It is highly recommended to enroll your child in an Early Intervention program right away. These programs provide physical, occupational, and speech therapy at a young age, which can significantly improve long-term social and academic outcomes [2].

Kidney Health: A Lifelong Priority

Because renal (kidney) anomalies are a core feature, protecting kidney function is a primary goal throughout childhood and adulthood [3][4].

  • Vigilant Monitoring: Many children with VACTERL require regular ultrasounds and blood tests to monitor for Chronic Kidney Disease (CKD) [5].
  • Renal Failure and Transplant: While some children have completely healthy kidneys, others may face a risk of end-stage renal disease [6]. It is important to know that kidney transplantation is a successful and viable option for VACTERL patients, though it requires close coordination with urologists to ensure the urinary tract is structurally prepared for a new kidney [5].

Bowel Management After ARM Repair

For children who had an Anorectal Malformation (ARM) repaired as infants, bowel function is a significant focus of childhood care [7].

  • Functional Challenges: Many children experience chronic constipation or challenges with fecal incontinence (difficulty controlling bowel movements) [8][9].
  • Bowel Management Programs (BMP): Specialized programs involving targeted diets, medications, or occasionally small procedures (like an ACE/Malone stoma) can help children achieve social continence and independence [10][11]. Bowel function often continues to improve as children grow, particularly in boys [8][12].

Growth and the Spine

As your child hits growth spurts, the medical team will monitor their spine (V - Vertebral) and spinal cord.

  • Tethered Cord and Syrinx: Doctors use MRI to check for a tethered cord (where the spinal cord is pulled tight by tissue at the base) or a syrinx (a fluid-filled cyst in the cord) [13]. If not addressed, these can lead to changes in leg strength, foot shape, or bladder control as the child grows [14][15].

Looking Toward Adulthood

VACTERL is not just a pediatric condition; it is a lifelong one. Transitioning to adult care involves finding specialists who understand the unique structural history of VACTERL patients, particularly in Urology, Cardiology, and Gynecology [16][17]. Navigating the daily routines of medical care can be exhausting, but with comprehensive support and early intervention, individuals with VACTERL can thrive in their adult lives [1].

Common questions in this guide

What is the long-term monitoring schedule for my child's kidney function?
Children with VACTERL association typically require regular ultrasounds and blood tests to monitor for Chronic Kidney Disease. This vigilant screening is a lifelong priority to protect kidney function and prepare for potential treatments like transplantation if needed.
How do we support my child's neurodevelopment and track milestones?
Children with VACTERL have a higher risk for neurodevelopmental conditions like ADHD, autism, and intellectual disabilities. Enrolling your child in an Early Intervention program for physical, occupational, and speech therapy can significantly improve their long-term social and academic outcomes.
How do we manage bowel challenges after an anorectal malformation repair?
If your child experiences chronic constipation or fecal incontinence, specialized Bowel Management Programs can help. These programs involve targeted diets, medications, or small procedures to help your child achieve bowel control and independence.
What signs of a tethered cord should I watch for as my child grows?
A tethered cord or spinal cyst can cause changes in leg strength, foot shape, or bladder control, especially during growth spurts. Doctors monitor the spine with MRI scans to catch and address these structural issues before they cause complications.
When and how do we transition from pediatric to adult specialists?
Because VACTERL is a lifelong condition, you will eventually need to transition your child to adult care. This involves finding adult specialists, particularly in urology, cardiology, and gynecology, who understand the unique structural history of VACTERL patients.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What is the specific long-term monitoring schedule for my child's kidney function (e.g., annual ultrasounds or blood work)?
  2. 2.How will we track my child's neurodevelopmental milestones to ensure we catch signs of ADHD or ASD early?
  3. 3.Who should I call if my child experiences a sudden change in bowel habits or signs of constipation?
  4. 4.At what age should we begin discussing the transition from pediatric to adult specialists?
  5. 5.Are there specific signs of 'tethered cord' syndrome I should watch for as my child grows taller?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (17)
  1. 1

    Children and adolescents with VACTERL association: health-related quality of life and psychological well-being in children and adolescents and their parents.

    Kassa AM, Dellenmark-Blom M, Thorsell Cederberg J, et al.

    Quality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation 2020; (29(4)):913-924 doi:10.1007/s11136-019-02364-w.

    PMID: 31741214
  2. 2

    Neurodevelopmental outcomes in individuals with VACTERL association. A population-based cohort study.

    Kassa AM, Lilja HE

    PloS one 2023; (18(6)):e0288061 doi:10.1371/journal.pone.0288061.

    PMID: 37384789
  3. 3

    Maternal risk associated with the VACTERL association: A case-control study.

    van de Putte R, de Walle HEK, van Hooijdonk KJM, et al.

    Birth defects research 2020; (112(18)):1495-1504 doi:10.1002/bdr2.1773.

    PMID: 33179873
  4. 4

    A case report of intrahepatic bile duct confluence anomalies in VACTERL syndrome.

    Yoon Y, Kim K, Yeom SK, et al.

    Medicine 2018; (97(39)):e12411 doi:10.1097/MD.0000000000012411.

    PMID: 30278516
  5. 5

    Outcomes of kidney transplants in pediatric patients with the vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, limb abnormalities association.

    Diaz J, Chavers B, Chinnakotla S, Verghese P

    Pediatric transplantation 2019; (23(2)):e13341 doi:10.1111/petr.13341.

    PMID: 30597716
  6. 6

    Kidney transplantation in an adult patient with VACTERL association.

    Cimen S, Nantais J, Guler S, Lawen J

    BMJ case reports 2015; (2015()).

    PMID: 26106170
  7. 7

    HSPA6: A new autosomal recessive candidate gene for the VATER/VACTERL malformation spectrum.

    Kause F, Zhang R, Ludwig M, et al.

    Birth defects research 2019; (111(10)):591-597 doi:10.1002/bdr2.1493.

    PMID: 30887706
  8. 8

    Long-term (> 10 years) bowel function of anorectal malformations: a retrospective single-center study.

    Wang K, Peng C, Pang W, et al.

    Pediatric surgery international 2024; (40(1)):138 doi:10.1007/s00383-024-05731-y.

    PMID: 38796646
  9. 9

    Assessment of long-term quality of life, bowel and voiding function outcomes in patients with anorectal malformation at a single UK centre.

    Beattie H, Subramanian T, Scudamore E, et al.

    Pediatric surgery international 2024; (40(1)):95 doi:10.1007/s00383-024-05684-2.

    PMID: 38565744
  10. 10

    Long-Term Bowel and Urinary Function Outcomes and Quality of Life in Patients with Anorectal Malformations: 20 Years of Experience.

    Baldanza F, Grasso F, Pensabene M, et al.

    Children (Basel, Switzerland) 2025; (12(8)) doi:10.3390/children12081042.

    PMID: 40868494
  11. 11

    Identification of urological anomalies associated with anorectal malformation in southwestern Uganda: Limitations and opportunities.

    Oyania F, Eze N, Aturinde M, et al.

    Journal of pediatric urology 2024; doi:10.1016/j.jpurol.2024.07.027.

    PMID: 39147608
  12. 12

    Anorectal malformation patients' outcomes after definitive surgery using Krickenbeck classification: A cross-sectional study.

    Makrufardi F, Arifin DN, Afandy D, et al.

    Heliyon 2020; (6(2)):e03435 doi:10.1016/j.heliyon.2020.e03435.

    PMID: 32095653
  13. 13

    Incidence of idiopathic syrinx in pediatric patients diagnosed with VACTERL association.

    Papadakis JE, Weber D, Albanese JS, et al.

    Journal of neurosurgery. Pediatrics 2025; (35(4)):385-390 doi:10.3171/2024.10.PEDS24242.

    PMID: 39793014
  14. 14

    Terminal myelocystocele: Surgical management.

    Massa DS, Montivero NA, Medina SAP

    Surgical neurology international 2022; (13()):234 doi:10.25259/SNI_299_2022.

    PMID: 35855164
  15. 15

    Cartilage within lipomyelomeningocele and ulnar longitudinal deficiency syndrome as VACTERL association, alliance in SHH/GLI3, and Wnt pathway: illustrative case.

    Shimekit MA, Yesuf EF, Teferi SM, Lemma MG

    Journal of neurosurgery. Case lessons 2024; (7(18)).

    PMID: 38684130
  16. 16

    In vitro fertilization outcomes in VACTERL association (vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies and limb anomalies): report of 2 cases.

    LePoidevin L, Dunn T, Arian SE, et al.

    F&S reports 2022; (3(3)):280-284 doi:10.1016/j.xfre.2022.06.006.

    PMID: 36212569
  17. 17

    Long overlooked: Adult VACTERL association unmasked by a large patent ductus arteriosus.

    Tamilselvan A, Mohan MK

    Radiology case reports 2025; (20(6)):2758-2762 doi:10.1016/j.radcr.2025.02.050.

    PMID: 40165847

This page provides educational information on the long-term management of VACTERL association. Always consult your pediatric specialists for personalized medical advice regarding your child's specific monitoring schedule.

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