Brunner Winter Syndrome

Bipolar syndrome composed of digestive atresia and malformations of the extremities.

Esophageal Duodenal Atresia, Abnormalities of Hands and Feet Syndrome.

Congenital genetic disorder.

Very rare.

Probably autosomal dominant. Gene located on 2p23-p24.

Unknown. Phenotypes are similar to those observed with 13q22-qter and 13q14 deletion.

Characterized by abnormalities of the hands and feet with short palpebral fissures, variable microcephaly with learning disability, and esophageal and/or duodenal atresia.

Features involve head with microcephaly, prominent occiput, microretrognathia, high-vaulted narrow palate, telecanthus, short palpebral fissures, low-set long ears, anteverted nares, and broad nasal root. Cry and voice are abnormal. Limbs can present syndactyly of toes, brachydactyly, thinning of the proximal end of the first metacarpal with shortening of that metacarpi, and abnormal dermatoglyphs with capillary hemangioma. Esophageal atresia, duodenal atresia, or both are frequent.

Evaluate tracheal intubation (clinical, radiographs) because of facial malformations.

Both direct laryngoscopy and tracheal intubation can be difficult because of microretrognathia. Patients with tracheoesophageal fistula are at high risk for aspiration. Venous access and pulse oximetry can be difficult because of limb malformations. Regional anesthesia is not contraindicated, but perimedullar blockade should be avoided in case of angioma.

Anesthetic drugs used for induction must authorize spontaneous ventilation until airway management is realized.

Feingold Syndrome: Anomalies of the digits with short palpebral fissures and atresia of esophagus or duodenum.