Postępowanie anestezjologiczne z zastosowaniem deksmedetomidyny u chorego z zespołem Prader-Williego – opis przypadku

*Tomasz Gaszyński¹, Janusz Strzelczyk², Waldemar Machała¹, Wojciech Gaszyński¹

Summary
Background.Prader-Willi syndrome (PWS) is characterized by hypothalamic dysfunction resulting in obesity, hypotonia, hypogonadism, and behavioural disturbance. It may be associated with hyperlipidemia, diabetes, and hypertension. Intubation in these patients may be difficult due to the obesity, short neck, and limited jaw mobility. Dexmedetomidine is a new a₂-adrenergic receptor agonist that offers a unique ”cooperative sedation,” anxiolysis, and analgesia with no respiratory depression. We describe a case when it was used for intraoperative and postoperative sedation in a patient with Prader-Willi syndrome.
Case report. A 24-yr-old man, height 155 cm, body weight 141 kg, BMI 59 kg m^-2, was scheduled for gastric reduction surgery. Due to impaired respiratory function (hypercapnia and hypoxia at rest), the surgery was postponed and a gastric balloon introduced under 0.2 ?g kg^-1min^-1 dexmedetomidine sedation. The drug produced satisfactory (Ramsay score 5) sedation, without respiratory depression and the procedure was uneventful. Boussignac CPAP was used in the postoperative period.
After 9 months and a weight loss of 21 kg, the patient´s condition improved markedly and the signs of respiratory insufficiency were no longer present. It was decided to proceed with the gastric reduction surgery, which was performed under general anaesthesia with sevoflurane, fentanyl and cisatracurium. Before extubation, the patient received 100 mg dexmedetomidine and 100 mg tramadol. He was extubated and transferred to the recovery area, where sudden apnoea occurred. An LMA was inserted and after 20 min of ventilation, the patient regained consciousness. Mask CPAP was then applied. The rest of the postoperative period was uneventful, and opioids were not required.
Discussion and conclusion. Morbidly obese patients have severe respiratory impairment such as hypoventilation and obstructive sleep apnoea. Opioids can be associated with potentially profound respiratory depressant effects in these patients, as occurred in the described case (fentanyl). The analgesic effects of dexmedetomidine have been demonstrated in numerous studies, producing clinically effective sedation and reduction of analgesic requirements. Dexmedetomidine can be recommended for sedation and analgesia in cases similar to the one described.

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