Anaesthesia and Rett syndrome: a case report

Maguire, Doug; Bachman, C.

doi:10.1007/bf03005353

Cited by 15 publications

(11 citation statements)

References 9 publications

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“…Many experts believe that the brainstem function of patients with Rett syndrome is immature and might be a factor in causing unexpected sudden death in some patients. 25,26,[28][29][30] This feature might have played a role in our patients with prolonged apnea. This is the largest study to date documenting the effects of deep sedation in patients with Rett syndrome.…”

Section: Discussionmentioning

confidence: 89%

Deep Sedation With Propofol in Patients With Rett Syndrome

et al. 2006

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Herein we present the largest retrospective case-control series of deep sedation in patients with Rett syndrome, including discussion of the unique aspects of Rett syndrome that make these patients at high risk for sedation. Twenty-one patients with Rett syndrome and 21 control patients who received propofol for deep sedation to facilitate lumbar puncture were compared. Patients with Rett syndrome required significantly less propofol than control patients when standardized for weight and the duration of the procedure (P = .004). Seven of the 21 patients with Rett syndrome compared with none of the control patients experienced a serious adverse event, most of which were due to prolonged apnea (P = .004). All adverse events were transient, and all patients returned to their baseline after the procedure was completed. Sedation of patients with Rett syndrome is associated with a relatively high rate of complications and should not be done without appropriate personnel available who recognize the risks of sedating this unique population.

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Section: Discussionmentioning

confidence: 89%

Deep Sedation With Propofol in Patients With Rett Syndrome

et al. 2006

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“…Therefore, we determined that the timing of her operation was appropriate. Patients with Rett syndrome reportedly have individual variability in sensitivity to anesthetic agents and muscle relaxants [9,10]. Therefore, it is strongly recommended that the BIS monitor and muscle relaxation monitor be used to avoid both insufficient anesthesia and delayed awaking.…”

Section: Discussionmentioning

confidence: 99%

Anesthetic management of a Rett syndrome patient with apnea and epilepsy: a case report

et al. 2018

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Rett syndrome, which is a progressive, central nervous system disease that is caused by a gene mutation, is known to present with various symptoms. This case is that of a 15-year-old girl who was diagnosed with Rett syndrome at the age of 2 years. Laryngotracheal isolation under general anesthesia was planned due to recurrent aspiration pneumonia. Since the patient's nutritional status and control of convulsions were good, this was deemed an appropriate time for the surgery. Following careful preoperative evaluation of her airway, we performed oral endotracheal intubation using a video laryngoscope after rapid induction. Since postoperative pain control was important to prevent apneic attacks and convulsions, we used a multimodal analgesic regimen including carefully titrated fentanyl, acetaminophen, nonsteroidal anti-inflammatory drug, and wound infiltration with a local anesthetic. Postoperatively, the patient returned to the intensive care unit under spontaneous ventilation and followed a good course. Patients with Rett syndrome present several symptoms. Thus, several points must be considered during the preoperative evaluation, anesthetic management, and postoperative care of these patients.

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“…These children may be at risk of recurrent aspiration and impaired respiratory function due to muscle weakness and thoracic deformity. In addition, abnormal patterns of respiration are commonly seen 10 . Cirignotta et al (8) have suggested that the frequent desaturations may cause permanent hypoxic damage and contribute to the progressive cerebral deterioration.…”

Section: Consentmentioning

confidence: 99%

“…Rett syndrome (RS) is a progressive developmental syndrome that occurs at the rate of 10,000-23,000 throughout the world and courses with autism, dementia, ataxia and loss of manual dexterity in girls 1 . It is the second leading cause of mental retardation among girls following Down syndrome 2 .…”

Section: Introductionmentioning

confidence: 99%