Difference between revisions of "VACTERL association"
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VACTERL is a constellation of birth defects that tend to co-occur. It is considered an association rather than a syndrome as there is no known pathogenetic cause for the grouped symptoms. | |||
== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. --> == | == Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. --> == | ||
| Line 27: | Line 27: | ||
* V – Vertebral anomalies | * V – Vertebral anomalies | ||
** Usually hypoplastic vertebrae or hemivertebrae. | |||
** Around 80% have vertebral anomalies. | |||
* A – Anal atresia | * A – Anal atresia | ||
** Seen in 55% | |||
* C – Cardiovascular anomalies | * C – Cardiovascular anomalies | ||
** Most commonly ASD, VSD, or Tetralogy of Fallot | |||
** Less commonly truncus arteriosus and transposition of the great arteries | |||
* T – Tracheoesophageal fistula | * T – Tracheoesophageal fistula | ||
** Seen in 70% | |||
* E – Esophageal atresia | * E – Esophageal atresia | ||
* R – Renal (Kidney) and/or radial anomalies | * R – Renal (Kidney) and/or radial anomalies | ||
** May have incomplete formation of one or both kidneys | |||
** May have urologic abnormalities including obstruction of outflow of urine from kidneys or severe reflux of urine from bladder back into the kidney. | |||
** Seen in 50% | |||
* L – Limb defects | * L – Limb defects | ||
** Seen in 70% | |||
** May have displaced or hypoplastic thumb, polydactyly, fusion of digits, or forearm defects (radial aplasia). | |||
== Diagnosis<!-- Describe how this comorbidity is diagnosed. --> == | == Diagnosis<!-- Describe how this comorbidity is diagnosed. --> == | ||
VACTERL is typically defined as >=3 of the above defects. | |||
== Treatment<!-- Summarize the treatment of this comorbidity. Add subsections as needed. --> == | == Treatment<!-- Summarize the treatment of this comorbidity. Add subsections as needed. --> == | ||
| Line 45: | Line 57: | ||
== Epidemiology<!-- Describe the epidemiology of this comorbidity --> == | == Epidemiology<!-- Describe the epidemiology of this comorbidity --> == | ||
No specific genetic or chromosome problem has been identified with VACTERL association. VACTERL can be seen with some chromosomal defects such as Trisomy 18. VACTERL association, however, is most likely caused by multiple factors. | |||
The incidence of VACTERL association is estimated to be approximately 1 in 10,000 to 1 in 40,000 live-born infants. It is seen more frequently in infants born to diabetic mothers. While most cases are sporadic, there are clearly families who present with multiple involved members. | |||
== References == | == References == | ||
[[Category:Comorbidities]] | [[Category:Comorbidities]] | ||
Latest revision as of 11:19, 7 December 2021
VACTERL association
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| Anesthetic management |
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| Signs and symptoms | |
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VACTERL is a constellation of birth defects that tend to co-occur. It is considered an association rather than a syndrome as there is no known pathogenetic cause for the grouped symptoms.
Anesthetic implications
Preoperative optimization
Intraoperative management
Postoperative management
Related surgical procedures
Pathophysiology
Signs and symptoms
- V – Vertebral anomalies
- Usually hypoplastic vertebrae or hemivertebrae.
- Around 80% have vertebral anomalies.
- A – Anal atresia
- Seen in 55%
- C – Cardiovascular anomalies
- Most commonly ASD, VSD, or Tetralogy of Fallot
- Less commonly truncus arteriosus and transposition of the great arteries
- T – Tracheoesophageal fistula
- Seen in 70%
- E – Esophageal atresia
- R – Renal (Kidney) and/or radial anomalies
- May have incomplete formation of one or both kidneys
- May have urologic abnormalities including obstruction of outflow of urine from kidneys or severe reflux of urine from bladder back into the kidney.
- Seen in 50%
- L – Limb defects
- Seen in 70%
- May have displaced or hypoplastic thumb, polydactyly, fusion of digits, or forearm defects (radial aplasia).
Diagnosis
VACTERL is typically defined as >=3 of the above defects.
Treatment
Medication
Surgery
Prognosis
Epidemiology
No specific genetic or chromosome problem has been identified with VACTERL association. VACTERL can be seen with some chromosomal defects such as Trisomy 18. VACTERL association, however, is most likely caused by multiple factors.
The incidence of VACTERL association is estimated to be approximately 1 in 10,000 to 1 in 40,000 live-born infants. It is seen more frequently in infants born to diabetic mothers. While most cases are sporadic, there are clearly families who present with multiple involved members.