VATER / VACTERL ASSOCIATION

• The VACTERL association is presumed to arise from a disorder of mesodermal development occurring between the fifth and seventh week of gestation (1-4). Incidence = 1:6250 births.
• The number of anomalies required to establish a diagnosis is arbitrary (5), however most authors require three (or two if tracheo-esophageal fistula is present) (1).

ULTRASOUND

Anomalies	Frequency
V = vertebral anomalies, vascular anomalies (1). A = anorectal anomalies (imperforate anus), auricular. C = cardiac anomalies (2). TE = tracheo-esophageal and esophageal atresia. R = renal anomalies (3). L = limb malformations (4).	70% 70% 40% 40% 65%

1. Spinal fusion, segmental errors, scoliosis, vascular rings, right sided aortic arch.
2. VSD, PDA, complex cyanotic heart disease.
3. Renal agenesis, dysplasia, hypoplasia or ectopia. Less commonly there may calyceal diverticuli, hydronephrosis, Prune belly syndrome, persistent urachus, cryptorchidism, hypospadias and posterior urethral valves.
4. Radial hypoplasia and aplasia, polydactyly or triphalangeal thumbs.
• Single umbilical artery is reported in a high percentage of fetuses (2).

VACTERL versus MURCS association
		VACTERL		MURCS
Mullerian duct failure		-		+
Vertebral defects		+		+
Limb abnormalities		+		+
Anorectal anomalies		+		-
Renal defects		+		+
Cardiovascular anomalies		+		-
Tracheo-esophageal anomalies		+		-
	Relative frequency (%) (1,2)
Type or location of defect	Caudal Regression Syndrome (N 153)		VACTERL (N 120)		Sirenomelia (N 134)
Vertebrae / sacrum / pelvis	100		100		100
Lower limb	73		30		100
Anorectal anomalies	0		88		97
Renal anomalies	26		85		93
Genital anomalies	3		55		85
Lower urinary tract	0		48		57
Single umbilical artery	0		10		79
Cardiac anomalies	0		24		26
Radial ray anomalies	0		29		21
Esophageal atresia ± tracheo esophageal fistula	0		28		5
Other Gastrointestinal defects	0		21		44
Respiratory tract (excluding tracheo esophageal fistula)	0		11		24
Central Nervous System	69		16		8

REFERENCES

1. Harris RD, Nyberg DA, Mack LA et.al. Anorectal atresia: Prenatal sonographic diagnosis. AJR 1987;149:395.
2. Khoury MJ, Cordero JF, Greenberg F et.al. A population study of the VACTERL association: Evidence for its etiologic heterogenicity. Pediatrics 1983;71:815.
3. Temtany SA, Miller JD. Extending the scope of the VATER association: Definition of the VATER syndrome. J Pediatr 1974;85:345.
4. Barnes JC, Smith WL. The VATER association. Radiology 1978;126:445.
5. Miller SF, Angtuaco TL, Quirk JG et.al. Anorectal atresia presenting as an abdominopelvic mass. J Ultrasound Med 1990;9:669-672.
6. Duncan PA, Shapiro LR, Klein RM. Sacrococcygeal dysgenesis association. Am J Med Genet 1991;411-53