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PubMed This is a summary of 23 peer-reviewed journal articles Updated
Neonatology · VACTERL Association

The First Steps: Neonatal Surgeries and Immediate Care

At a Glance

For newborns with VACTERL association, immediate NICU care focuses on stabilizing breathing, feeding, and waste elimination. The top surgical priorities usually involve repairing esophageal atresia (TEF/EA), managing anorectal malformations, and stabilizing any severe heart defects.

For parents of a newborn with VACTERL association, the first few days of life are often a whirlwind of surgical consultations, beeping monitors, and intensive care jargon. Because VACTERL involves multiple systems, the medical team’s primary goal is to stabilize your baby and prioritize repairs that are essential for breathing, feeding, and waste elimination [1][2].

A Note on the NICU Environment: Seeing your baby connected to breathing machines, feeding tubes, and IV lines is deeply traumatic for any parent. Please know that beneath the wires, they are still your baby. Ask your nurses how you can safely touch them, change their diapers, or provide skin-to-skin contact. Your presence and scent are vital components of their healing.

The Surgical “Priority List”

In the neonatal period, two of the most common core features requiring immediate attention are Tracheoesophageal Fistula (TEF) and Anorectal Malformations (ARM).

1. Repairing the Airway and Esophagus (TEF/EA)

If your baby has Esophageal Atresia (EA) with or without a Tracheoesophageal Fistula (TEF), it means their food pipe is either improperly connected to their windpipe or it ends in a pouch [3]. They cannot feed normally, and saliva can dangerously enter their lungs.

  • The Procedure: Surgeons typically perform a repair to close the connection (fistula) between the windpipe and the food pipe and then carefully sew the two ends of the esophagus together [4].
  • Preoperative Checks: Before surgery, doctors must use an echocardiogram to see if the main artery from the heart (the aorta) curves to the right instead of the left. This occurs in about 5% of these babies and changes which side of the chest the surgeon must open [5][6].
  • Airway Imaging: A bronchoscopy (using a tiny camera to look inside the airway) or a chest CT is critical to check for other hidden airway differences that could complicate the surgery [7][8].

2. Managing Anorectal Malformations (ARM)

If a baby is born with anal atresia, they do not have a standard opening for stool to safely leave the body.

  • Staged Approach: In many cases, especially if the malformation is “high” inside the pelvis or complex, a surgeon will first perform a colostomy [9]. This creates a temporary opening on the baby’s belly for stool to exit into a bag, allowing the baby to grow and get stronger before the final reconstructive surgery [10].
  • Single-Stage Repair: In some instances, a primary repair (doing the final reconstruction right away without a colostomy) is possible [11]. This is more common if the malformation is “low” [12]. Preoperative imaging like an ultrasound helps the team decide which path is safest [13][9].

Coordinating Care with Cardiac Needs

Structural heart defects are very common in VACTERL [14]. While some heart issues (like small holes) can wait and heal over time, others require immediate medication (like prostaglandins) to keep specific blood vessels open until the baby is stable enough for open-heart surgery [15][16].

If a baby has a severe heart condition, the surgical team may choose a staged approach for other repairs—such as placing a feeding tube (gastrostomy) or a colostomy first—to minimize the time the baby spends under anesthesia while their heart is fragile [10][17].

Recovery and the Path Forward

Recovery in the NICU is a marathon, not a sprint.

  • Esophageal Healing: After TEF/EA repair, babies are often monitored for acid reflux and narrowing of the esophagus (strictures), which may require gentle stretching procedures in the future [18][19].
  • Bowel Management: For those with ARM, long-term follow-up is essential to help with bowel control as the child grows [20][21].

While this journey is medically complex, modern neonatal surgery is highly advanced. Each procedure is a step toward your baby being able to grow, eat, and thrive [22][23]. To prepare for the transition home, read about Building Your Child’s Multidisciplinary Care Team.

Common questions in this guide

What is the first surgery a baby with VACTERL association usually needs?
The timing of surgeries depends on the baby's specific needs, but repairing the airway and esophagus (TEF/EA) or managing anorectal malformations are often the highest priorities. Severe heart defects may also require immediate medical or surgical attention to keep the baby stable.
Why does my baby need an echocardiogram before esophageal (TEF/EA) surgery?
An echocardiogram helps the surgical team see if the main artery from the heart curves to the right instead of the left. This structural difference determines which side of the chest the surgeon must open to perform the esophagus repair safely.
Will my baby need a colostomy for an anorectal malformation?
Many babies with complex or 'high' anorectal malformations will first need a temporary colostomy to allow stool to exit the body safely. Once the baby grows and gets stronger, surgeons will perform a final reconstructive surgery. Some babies with less complex malformations may qualify for a single-stage repair right away.
How do heart defects affect the timing of other VACTERL surgeries?
If a baby has a severe or fragile heart condition, surgeons may choose to do quicker, temporary procedures first, such as placing a feeding tube or colostomy. This minimizes the time the baby spends under anesthesia until their heart is strong enough for more extensive reconstructive surgeries.
Why is a bronchoscopy performed before a fistula repair?
A bronchoscopy uses a tiny camera to look inside the airway before surgery. This is critical to check for other hidden airway differences that could complicate the esophageal repair and ensure the surgical team is fully prepared.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Does my child have a right-sided aortic arch, and how will that affect the surgical approach for the TEF/EA repair?
  2. 2.Has a bronchoscopy been performed to check for other airway issues before we proceed with the fistula repair?
  3. 3.Is my child a candidate for a single-stage repair of their anorectal malformation, or do they need a temporary colostomy first?
  4. 4.How do my child's heart findings affect the timing of their other surgeries?
  5. 5.What is the plan for managing pain and acid reflux during the recovery period?

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 (23)
  1. 1

    Anorectal malformations (ARM) and VACTERL association and severity of congenital heart diseases (CHD): Experience of 396 consecutive patients in a tertiary center.

    Moras P, Zarfati A, Bagolan P, et al.

    Pediatrics and neonatology 2024; (65(4)):381-385 doi:10.1016/j.pedneo.2023.08.011.

    PMID: 38163746
  2. 2

    Single-Stage Surgery for Persistent Cloaca With Vertebral Defects, Anal Atresia, Cardiac Defects, Tracheoesophageal Fistula or Atresia, Renal Anomalies, and Limb Defects (VACTERL) Association: A Case Report on Avoiding Temporary Colostomy.

    Masuko T, Yanai T, Toma M

    Cureus 2025; (17(4)):e82487 doi:10.7759/cureus.82487.

    PMID: 40385907
  3. 3

    VACTERL association in a fetus with multiple congenital malformations - Case report.

    Pariza PC, Stavarache I, Dumitru VA, et al.

    Journal of medicine and life 2021; (14(6)):862-867 doi:10.25122/jml-2021-0346.

    PMID: 35126759
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    Surgery for intrathoracic tracheoesophageal and bronchoesophageal fistula.

    Bibas BJ, Cardoso PFG, Minamoto H, Pêgo-Fernandes PM

    Annals of translational medicine 2018; (6(11)):210 doi:10.21037/atm.2018.05.25.

    PMID: 30023373
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    Infants with esophageal atresia and right aortic arch: Characteristics and outcomes from the Midwest Pediatric Surgery Consortium.

    Lal DR, Gadepalli SK, Downard CD, et al.

    Journal of pediatric surgery 2019; (54(4)):688-692 doi:10.1016/j.jpedsurg.2018.08.002.

    PMID: 30224238
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    Management of neonates with right-sided aortic arch and esophageal atresia: International survey on IPEG AND ESPES members´ experience.

    Aguilera-Pujabet M, Gahete JAM, Guillén G, et al.

    Journal of pediatric surgery 2018; (53(10)):1923-1927 doi:10.1016/j.jpedsurg.2017.11.051.

    PMID: 29241961
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    VACTERL association complicated with multiple airway abnormalities: A case report.

    Yang L, Li S, Zhong L, et al.

    Medicine 2019; (98(42)):e17413 doi:10.1097/MD.0000000000017413.

    PMID: 31626096
  8. 8

    Expect the unexpected: neuroblastoma in a patient with the VACTERL association.

    Braungart S, Daff C

    BMJ case reports 2025; (18(4)) doi:10.1136/bcr-2025-265123.

    PMID: 40199601
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    Identification of an occult recto-prostatic fistula with cystoscopy-assisted air colostogram.

    Shaughnessy MP, Park CJ, Hittelman AB, Cowles RA

    International journal of surgery case reports 2019; (65()):349-353 doi:10.1016/j.ijscr.2019.11.019.

    PMID: 31770712
  10. 10

    The boy who lived: staged repair of congenital diaphragmatic hernia with esophageal atresia and tracheoesophageal fistula in a 32-week, 1.5 kg infant, and review of the literature.

    Woodward JM, Corujo Avila P, Mathew B, et al.

    Journal of surgical case reports 2025; (2025(5)):rjaf333 doi:10.1093/jscr/rjaf333.

    PMID: 40453740
  11. 11

    Short and Long-Term Outcomes of PSARP versus LAARP and Single versus Staged Repair for Infants with High-Type Anorectal Malformations: A Systematic Review and Meta-Analysis.

    Miscia ME, Lauriti G, Di Renzo D, et al.

    Children (Basel, Switzerland) 2024; (11(3)) doi:10.3390/children11030376.

    PMID: 38539411
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    The management of anorectal malformation with congenital vestibular fistula: a single-stage modified anterior sagittal anorectoplasty.

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    Pediatric surgery international 2015; (31(9)):809-14 doi:10.1007/s00383-015-3749-1.

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    The comparison of magnetic resonance and fluoroscopic imaging options in the preoperative assessment of boys with anorectal malformations and a colostomy.

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    Pediatric radiology 2024; (54(1)):68-81 doi:10.1007/s00247-023-05816-1.

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    Congenital Heart Defects in Patients with Anorectal Malformations: A Retrospective Cohort Study of 281 Patients.

    de Beaufort CMC, Mackay TM, Stevens MF, et al.

    Pediatric cardiology 2025; (46(5)):1202-1210 doi:10.1007/s00246-024-03536-3.

    PMID: 38836880
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    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
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    A Rare Case of Pulmonary Artery Sling with the VACTERL Association in a 20-Month-Old Infant.

    Ghandi Y, Shafiee A, Sharifi M, Bolandnazar NS

    The journal of Tehran Heart Center 2017; (12(3)):131-133.

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

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This page provides educational information about neonatal surgeries for VACTERL association. Always consult your pediatric surgeon and neonatology team for medical advice specific to your baby's condition.

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