Anesthesia in a patient with Job's syndrome (hyper IgE). Case report.

BACKGROUND AND OBJECTIVES

Job's syndrome (JS), one of the presentations of the Hyper IgE Syndrome, is a rare immunodeficiency. It includes cutaneous abscesses, recurring pneumonias, pneumatoceles, eosinophilia, hyperimmunoglobulinemia E (> 2,000 IU.mL(-1)), and craniofacial and bone growth changes. This report describes the disease and its anesthetic management.

CASE REPORT

The patient is a 13 year old black male, 40 kg, ASA II, with Job's Syndrome diagnosed 6 months prior to this admission. The patient was admitted for elongation of the right femur. He denied use of drugs and prior surgeries; he presented good cervical mobility, interincisive distance greater than 3 cm, Mallampati II, without signs of infection. Preoperative exams were within normal limits. He was monitored with electrocardioscope, SpO(2), non-invasive blood pressure, and P(ET)CO(2). After pre-oxygenation, general anesthesia was induced and he was maintained with sevoflurane. At the end of the procedure, the patient was extubated after reversal of the neuromuscular blockade, and the patient was transferred to the PACU with Aldrete 9, He was discharged from the hospital 72 hours later, without complications.

CONCLUSIONS

The choice of anesthetic technique is guided by rigorous observation among risks and benefits for each patient, according to respiratory sequelae, risk of infection, and surgical site. In the patient described here, we considered that neuroaxis anesthesia could be associated with an increased risk of severe infections due to the patient immunologic background. The procedure was safely performed with general anesthesia.