Anesthetic management during orchiopexy in Prader-Willi syndrome. Case report

Abstract

Introduction: Prader-Willi syndrome is a non-hereditary genetic disease, associated with disorders of the chromosome 15 of paternal origin. Although the diagnosis is based on suspicion of characteristic symptoms, it is only confirmed by genetic testing. The anesthetic peculiarities of these patients are derived from their characteristics at the time of the intervention; however, special attention should be paid to several aspects, including difficulties in the doctor-patient relationship, the possibility of a patient having a full stomach, difficult venous access, difficulty in managing the airway, increased anesthetics sensitivity and delayed awakening.

Case presentation: a nine year old male patient, white skin, 64 Kg of weight and 1,50 m of height, diagnosed with Prader-Willi syndrome. The examinations of the respiratory and cardiovascular systems did not provide positive data; the results of the supplementary examinations were within normal limits. On physical examination, characteristic features were found: large head and short neck with good flexion-extension, small mouth and large tongue, moderate mental retardation and bilateral cryptorchidism, reason for which surgical treatment was indicated by bilateral orchidopexy with the use of general orotracheal anesthesia. The patient had no anesthetic complications; he was hospitalized for 48 hours with a satisfactory evolution.

Conclusions: in the anesthetic management of patients with Prader-Willi syndrome, the associated comorbidities must be taken into account; Proper postoperative surveillance is necessary to prevent the occurrence of respiratory complications.