Background
Hutchinson‐Gilford progeria syndrome is a rare disease in childhood that results in premature aging. The presence of multisystem derangements including skin, bone, and joint diseases and possibly a difficult airway makes the anesthetic management challenging. Because of the extremely low prevalence, experience is limited even for experienced pediatric anesthesiologists.
Objective
To review the available literature on anesthesia for patients with Hutchinson‐Gilford progeria syndrome and to give recommendations for establishing the best practice for patients with Hutchinson‐Gilford progeria syndrome.
Design
A narrative review of the rare existing literature.
Date sources
CENTRAL (Cochrane), EMBASE, Google Scholar, MEDLINE and PubMed.
Eligibility criteria
Articles addressing anesthesia in patients with Hutchinson‐Gilford progeria syndrome were included.
Results
An overview of the current literature was made on anesthesia care for patients with Hutchinson‐Gilford progeria syndrome. After screening the literature, only ten articles were found to be of interest and include some case reports and a correspondence. The focus points on how to perform anesthesia care in patients with Hutchinson‐Gilford progeria syndrome and the entire perioperative care are suggested. The available data are limited and results need to be interpreted with caution.
Conclusion
The patients with Hutchinson‐Gilford progeria syndrome are not just “frail” patients. Awareness concerning intubation difficulties is mandatory, and airway strategies must be addressed in advance. Although these patients present with the physiology of an elderly with accompanying comorbidities, emotionally they are only children and should be approached as such.
We describe a patient who presented with a bilateral pulmonary artery sarcoma, initially treated as pulmonary embolism, that necessitated concomitant pulmonary endarterectomy and pneumonectomy. We reviewed the anesthetic management used for this procedure, which bears many similarities to the management of patients undergoing pulmonary thromboendarterectomy. Right ventricular failure, pulmonary hemorrhage, and cerebral ischemia due to circulatory arrest are life-threatening perioperative complications. The anesthesiologist can play a key role in the prevention (or timely recognition and treatment) of these perioperative complications by establishing adequate hemodynamic, echocardiographic, and neurologic monitoring and by optimizing cardiopulmonary function and coagulation.
IntroductionIn the context of enhanced recovery after cardiac surgery, surgical techniques for mitral valve surgery have witnessed substantial modifications, from approaching the heart using open approaches with traditional sternotomy to thoracoscopic access via minithoracotomy. After cardiac surgery, acute postoperative pain is frequent and caused by surgical incision and retraction. Perioperative analgesia in cardiac surgery still relies mainly on opioids. Although neuraxial techniques could be a valuable non-opioid-based analgesia regimen, they can be associated with devastating complications in situations with (iatrogenic) coagulation abnormalities. Only two randomised clinical trials describe the erector spinae plane (ESP) block to provide sufficient postoperative analgesia following cardiac surgery with median sternotomy. Regarding postoperative analgesia after cardiac surgery with a minithoracotomy approach, adequately designed trials are still lacking. We, therefore, designed a double-blind, placebo-controlled trial to prove the hypothesis that the ESP block reduces opioid consumption in patients undergoing minimally invasive mitral valve surgery (MIMVS).Methods and analysisSixty-four patients undergoing MIMVS will be included in this double-blind, prospective, placebo-controlled trial. Patients will be randomised to receive an ESP block with a catheter with either intermittent ropivacaine 0.5% (ropi group) or normal saline 0.9% (placebo group). Both groups will receive patient-controlled intravenous analgesia with morphine following extubation. Primary endpoint is the 24-hour cumulative morphine consumption after extubation. Multiple secondary endpoints will be evaluated.Ethics and disseminationThe study is approved by the ethics committee of the University Hospitals Leuven, the Clinical Trials Centre of the University Hospitals Leuven and the ‘Federaal Agentschap voor Geneesmiddelen en Gezondheidsproducten’. Dissemination of the study results will be via scientific papers.Trial registration numberEudraCT identifier: 2019-001125-27.
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