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Polymalformative condition characterized by esophageal/duodenal atresia, limb abnormalities (hand/foot), short palpebral fissures, microcephaly and learning disabilities.

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Microcephaly-Oculo-Digito-Esophago-Duodenal,Syndrome; MODED Syndrome; Brunner-Winter Syndrome; Oculodigitoesophagoduodenal Syndrome.

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Incidence unknown. Autosomal dominant with gene map locus at 2p24.

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Most common clinical features are microcephaly with prominent occiput, mental retardation, narrow palpebral fissures, telecanthus, retrognathia and low-set ears. The presence of a laryngeal cleft/vocal cord palsy gives an abnormal cry and voice. The gastrointestinal defects include esophageal atresia (EA) [with or without tracheoesophageal fistula (TEF) or duodenal atresia (DA)], occurs in 30% of patients. Other features include a patent ductus arteriosus, sacral spine anomalies (sagittal cleft), and blockage of C5-C6 vertebrae. Neural arch fusion at C6-C7 has been reported. Short stature, syndactyly of toes, brachydactyly type A, clinodactyly, slender thumbs with limited flexion in the distal interphalangeal joint.

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Evaluate the airway due to facial malformations. Mental retardation may limit patient cooperation. Sedative premedication and/or the presence of the primary caregiver may be helpful.

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Airway management should be expected to be difficult due to microretrognathia and potential cervical motility problems. Patients with DA and/or TEF are at increased risk for aspiration. The association of EA and TEF requires that the tracheal tube be positioned below the level of the fistula to avoid gastric distention during positive pressure ventilation. Venous access may be difficult. Regional anesthesia is not contraindicated, but caudal anesthesia may be difficult in presence of sacral anomalies.

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Avoid neuromuscular blockers until the airway has been secured.

Celli J, van Bokhoven H, Brunner HG: Feingold syndrome: clinical review and genetic mapping. Am J Med Genet 122:294, 2003.

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