Anesthetic management of a patient with Cri Du Chat syndrome. Case report.

BACKGROUND AND

OBJECTIVES

Cri Du Chat syndrome is a chromosomal disorder with peculiar clinical characteristics including airways abnormalities that require special care by anesthesiologists when handling those patients.

OBJECTIVE

To present a case of outpatient anesthesia in a patient with Cri Du Chat syndrome and discuss the anesthetic aspects related to this disorder.

CASE REPORT: Male patient, 14 years old, 25 kg, with Cri Du Chat syndrome, physical status ASA P2, was admitted for upper gastrointestinal endoscopy and esophageal dilation. The patient had mental retardation, a few episodes of seizures, and marked hypertonia of the limbs. Airways exam showed limited cervical mobility and thyromental distance less than six centimeters. The patient was unable to comprehend verbal commands, making it difficult to undertake a complete assessment of the airways. Other findings on physical exam included microcephaly, micrognathism, subtle strabismus, limb hypertonia with flexion, and protrusion of the tip of the tongue. Intravenous fentanyl 50 μg, midazolam 1 mg, and propofol 60 mg were administered. The patient was maintained on spontaneous ventilation. The procedure lasted 5 minutes, without intercurrences.

CONCLUSIONS

Patients with Cri Du Chat syndrome have clinical characteristics that are very important for their anesthetic management, being the responsibility of the anesthesiologist to consider carefully the structural particularities of each patient.