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.
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 ...