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At a glance

An acronym that 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.

Synonyms

VATERS Association; VATER(2) Syndrome VACTER Syndrome; Kaufman Syndrome; Quan-Smith Syndrome; Say-Gerald Syndrome; Hydrometrocolpos-Postaxial Polydactyly-Congenital Heart Disease Syndrome.

Nature

Unknown.

Incidence

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

Genetic inheritance

Sporadic (some are familial, transmitted as an autosomal dominant trait). Initially described in 1973 as a statistically nonrandom co-occurrence of a group of congenital malformations.

Pathophysiology

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

Diagnosis

Association can be evocated in the antenatal period. Prematurity is frequent. Diagnosis is evocated on the association of the different clinical signs included in both acronyms. VATER or VACTERL are acronyms that stands for Vertebral abnormalities (70% of cases), Anal atresia (80% of cases), Cardiovascular defects (53% of cases), Tracheo-Esophageal fistula with or without esophageal atresia (70% of cases), Renal agenesis or dysplasia (53% of cases), and Limb abnormalities (65% of cases). The acronyms refer to the most frequent malformations and other defects (see Clinical aspects).

Clinical aspects

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, hydrometrocolpos, hydronephrosis, hypospadias), and skeleton (polydactyly and proximally placed thumbs, humeral hypoplasia, radial aplasia, vertebral dysgenesis, spondylocostal dysplasia, scoliosis, and hemivertebrae). Hydrometrocolpos is the result of accumulation of secretions in the vagina and uterus. It is caused by excessive intrauterine stimulation of the infant’s cervical mucous glands by maternal oestrogen in the presence of an intact hymen.

Precautions before anesthesia

Anesthetic management must consider the emergency of the surgical procedure and focus on the principle underlying abnormalities. 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).

Anesthetic considerations

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 ...

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