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2019

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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REGIONAL ANAESTHESIA IN A PATIENT WITH PROGERIA

Luísa Valente, Eduardo Barata Correia, Teresa Rocha.

Serviço de Anestesiologia, Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central, E.P.E.

- V encontro de anestesia pediátrica (apresentação oral).

Background: Hutchinson-Gilford progeria is a rare genetic disorder characterised by accelerated ageing developing during childhood, with an average life span of 13 years.
The clinical manifestations include growth failure, characteristic facies, and alterations of the skin, skeleton, and cardiovascular system, which ultimately become the most common cause of death. We report a case of a surgical patient with progeria who was submitted to regional anesthesia, which use in this population, as far as we know, has never been reported.

Case report: A 7-year-old boy with progeria was proposed for bilateral orchidopexy and surgical treatment of phimosis. He presented features of difficult airway, osteoporosis, limited joint mobility and cerebral vascular atherosclerotic disease, and was under a clinical trial that contraindicated the use of numerous drugs, namely benzodiazepines,
opioids, barbiturates and neuroleptics.
The surgery was performed under a spinal block with levobupivacaine 0,5%, combined with inhalation anaesthesia with sevoflurane by facemask, maintaining spontaneous ventilation. An ilioinguinal bilateral block with levobupivacaine 0,25% for postoperative analgesia was performed. The surgery and postoperative period underwent without complications.

Discussion and Conclusion: Progeria presents important anaesthetic implications: difficult airway and intravenous access, the need for careful intraoperative positioning and the high probability of cardiovascular disease. By choosing a regional technique we
avoided the manipulation of the airway and the use of drugs that were contra-indicated by the clinical trial, reduced the risk of postoperative nausea and vomiting, and obtained an excellent control of postoperative pain. Regional anesthesia seems to be a safe and good alternative to general anaesthesia in this population.

Keywords: progeria.