Achalasia-Microcephaly Syndrome

Characterized by microcephaly, mental deficiency and early onset of symptoms of achalasia. Patients present with recurrent pulmonary aspirations and frequent respiratory infections.

Asherson Syndrome; Cricopharyngeal Achalasia.

First described in 1978 in a Mexican family located in a small area in Northwest Mexico. There is no epidemiological study available to establish the incidence. Autosomal recessive inheritance is suggested.

Recurrent vomiting and pulmonary infections in a child with microcephaly, mental deficiency, achalasia.

It is recommended to consider prophylaxis administration of sodium citrate to reduce the risk of pulmonary aspiration at time of induction of anesthesia. Evaluate serum electrolytes and arterial blood gases, if recurrent vomiting evident. A complete medical history and physical examination must be obtained to rule out the presence of reactive airways disease as a result of chronic aspiration. A chest x-ray and pulmonary function tests are indicated (when applicable).

Patients must be considered at significant risk for pulmonary aspiration of gastric contents. It is recommended to use an anesthesia technique to prevent this complication during induction of anesthesia and instrumentation of the airway. Reactive airway disease may be present as a result of chronic pulmonary aspiration. Intravascular volume depletion, serum electrolytes imbalances and arterial blood gases anomalies as a result of chronic vomiting must be corrected preoperatively in all patients.

There are no known pharmacological implications in this medical condition. However, the use of anesthetic agents releasing histamine should be considered contraindicated in the presence of severe reactive airway disease.