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Background and Aims Rett syndrome is a rare genetic neurodevelopmental disorder caused by mutations on MECP2 gene on chromosome X, which encodes a protein essential for the normal function of nerve cells. Hence, females are primarily affected. It is characterized by normal early growth and development followed by loss of previously acquired skills at about 6-18 months of life. Symptoms may include: loss of speech, mobility and muscle tone, involuntary hand movements, seizures, breathing and sleep disturbances and slowed rate of growth for head, hands and feet. This is the first case report of a patient with Rett syndrome who underwent surgery under regional anesthesia.
Methods A 38 year old woman, ASA physical status III, presented for elective equinovarus foot surgery. She was diagnosed with Rett syndrome at 2 years of age. Spinal anesthesia with 0.5% hyperbaric bupivacaine was combined with femural and sciatic nerve blocks with 0.375% ropivacaine.
Results The surgery lasted about 2,5 hours and went out uneventfully. No complications were reported in postoperative visit, no need of rescue analgesics registered, and the patient was discharged home on postoperative day 2.
Conclusions Rett syndrome is a rare genetic disorder and therefore recommendations regarding anesthetic management are scarce and there are no reports of regional anesthesia. Anesthetic considerations should include: possibility of a difficult airway; risk of prolonged QT interval and T wave changes; increased sensitivity to sedative drugs; and anatomical malposition of vessels. In this case report we show that regional anesthesia can be an effective and safe approach in patients with Rett syndrome.
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