VA(C)TER(L) Association

Acronym stands for: Vertebral Anomalies, Anal Atresia, (Cardiac Defects), Tracheo-Esophageal Fistula, Renal (and Limb Anomalies). It already describes the most important defects of this disorder. Other coexisting anomalies must be excluded.

VATERS Association; VACTER Syndrome; Kaufman Syndrome; Quan-Smith Syndrome; Say-Gerald Syndrome; Hydronephrocolpos-Postaxial Polydactyly-Congenital Heart Disease Syndrome.

Unknown.

1:3,500 to 1.6:10,000 live births.

Sporadic (some are familial, transmitted as an autosomal dominant trait).

Unknown; a disruption of blastogenesis seems to be the origin of the association. Multiple exogenous causes have been evocated; familial occurrence has been reported.

Association can be evocated in the antenatal period. Prematurity is frequent. Diagnosis is evocated on the association of the different signs included in the acronym of VATER or VACTERL: vertebral abnormalities (70% of cases), anal atresia (80% of cases), cardiovascular defects (53% of cases), tracheoesophageal fistula with esophageal atresia (70% of cases), renal agenesis or dysplasia (53% of cases), and limb abnormalities (65% of cases).

The acronyms emphasize only the most frequent malformations and a wide variety of other defects, affecting most parts of the body, may be associated, including head (prominent epicanthus, flat nasal bridge, choanal atresia, micrognathia, low-set malformed ears), thorax (spondylocostal dysplasia, hypoplastic lungs, laryngeal stenosis, ventricular septal defects, patent ductus arteriosus, tetralogy of Fallot, transposition of the great arteries), gastrointestinal tract (absent rectum and anus, duodenal atresia), urogenital system (urethral atresia, renal agenesis, hydronephrocolpos, hydronephrosis, hypospadias), and skeleton (polydactyly and proximally placed thumbs, humeral hypoplasia, radial aplasia, vertebral dysgenesis, spondylocostal dysplasia, scoliosis, and hemivertebrae).

Anesthetic management must consider the emergency of the surgical procedure. Evaluate cardiac function (clinical, chest radiographs, echocardiography, ECG), respiratory function (chest radiographs, arterial blood gas analysis), and renal function (echography, laboratory investigations, including urea, creatinine, electrolytes).

Various anesthetic management procedures have been described for these patients. Challenge is greater if tracheal fistula ligature is initially closed. Careful intraoperative positioning is necessary but may be difficult. Venous and arterial access can be a challenge in cases of severe limb anomalies. Avoid manual-assisted ventilation to limit aspiration risk. Direct laryngoscopy can be difficult because of micrognathia, and tracheal intubation can be hazardous because of laryngeal stenosis. Consider existence of a tracheoesophageal fistula to determine exact position of tube's end (placement just near a tracheal carina can be useful if fistula is located over the carina). Control chest radiographs or with fiberoptic can be interesting after intubation. Spontaneous ventilation should be preserved until fistula is closed or after confirmation of the position of the tracheal tube past the fistula and absence of gastric air entry. Medullar regional anesthesia should be avoided because of vertebral anomalies. Intraoperative monitoring should include artery, double pulse oximetry or transcutaneous O2 (one in preductal and the other in postductal).

Anesthetic medications should be chosen to preserve spontaneous ventilation and cardiac function. Prophylactic antibiotics should be used in cases of cardiac defect. Intraoperative fluid regimen must be strict in cases of renal anomalies.

VACTERL Association with Hydrocephalus: Probably autosomal recessive with aqueductal stenosis, hydrocephalus, encephalocele, meningocele, and agenesis of corpus callosum; prognosis is poor.

X-Linked VACTERL Association with Hydrocephalus: X-linked instead of autosomal recessive.