Risk Of Malignant Hyperthermia Research Articles

A Single-Center Retrospective Review of Patients with Suspected Malignant Hyperthermia Susceptibility.

Purpose The diagnosis of malignant hyperthermia susceptibility (MHS) has significant implications for the perioperative period that may persist for generations. Anesthetic medication options are reduced, anesthetic workstations require preparation to reduce exposure to inhaled volatile anesthetics, and patients may be excluded from surgery at ambulatory centers. In this study, we sought to better characterize the etiology of MHS diagnoses in our health system and the downstream effects of this diagnosis on anesthetic care. Methods We retrospectively reviewed the electronic medical records of 55 patients with a documented concern for MHS who received care at University of Florida (UF) Health between 2014 and 2020. We characterized the etiology of the patient's MHS diagnosis, whether this diagnosis was supported by formal genetic or muscle contracture testing, and the details of the recorded anesthetics that were delivered to these patients. Results The 55 patients with suspected MHS were evenly split between those with a family history of malignant hyperthermia (MH) (28/55) and those with a concern for MHS in their personal medical history (27/55). Of the 28 patients with a family history of MH, 16 reported that the affected family member was a first-degree relative, and two of these 16 reported that the affected family member had undergone confirmatory muscle contracture testing. Of the 27 patients with a personal history suspicious for MHS, two had undergone confirmatory genetic testing, and two patients had anesthetic records available for review where intraoperative MH was suspected and treated with dantrolene. An additional four patients were told of a concern about MHS due to another underlying diagnosis. No patients with a personal history suspicious of MHS had undergone confirmatory muscle contracture testing. These 55 patients underwent 87 anesthetics, and exclusively non-triggering anesthetic techniques were utilized in nearly all cases. In pediatric patients, some perioperative challenges were identified, related to the avoidance of mask inhalational induction. Only six of these 87 anesthetics occurred at our ambulatory surgery centers, a proportion (6.9%) lower than that of the general surgical population at UF Health (20.0%). Conclusions Among patients suspected to be MH susceptible in our health system over a six-year period, a minority (8/55) were supported by clear records of a prior MH event, confirmatory genetic or muscle contracture testing, or an underlying diagnosis closely linked to MH. The vast majority had limited documentation supporting their MH risk but continued to be treated with non-triggering anesthetics and were less likely to have surgery at an ambulatory surgery center than our overall surgical population. Among pediatric patients, some anesthetic challenges related to delivering non-triggering anesthetics were identified. Improving the documentation of index cases of MH and increasing referrals to clinical geneticists and genetic testing may be a viable route to decreasing the proportion of suspected MHS patients with a poorly characterized risk profile.

Anaesthetic Management of Pediatric Patient with Osteogenesis Imperfecta Posted for Radius Ulna Nailing: A Case Report

Osteogenesis Imperfecta is a collagen disorder of autosomal dominant type caused by mutation of type COLIA-1 and COLIA-2 genes. Orthopaedic surgery in paediatric patient patients of OI is challenging mainly due to difficult airway and risk of malignant hyperthermia. Here we report, a paediatric case of OI with similar family history posted for radius ulna nailing which was managed with total intravenous anaesthesia and supraclavicular brachial plexus block.

Trigger-free anesthesia : Indications and safe application

The safe anesthesiological care of patients with neuromuscular diseases poses aparticular challenge for anesthetists. Only asmall group of muscle diseases and syndromes are associated with an increased risk of malignant hyperthermia and therefore require trigger-free anesthetic procedures avoiding volatile anesthetics and succinylcholine. These diseases are frequently associated with mutations in the RYR1, CACNA1S or STAC-3 genes. In other neuromuscular diseases, anesthetic-induced rhabdomyolysis can occur. Therefore, volatile anesthetics should be avoided in these patients in addition to the contraindication for succinylcholine. In other neuromuscular diseases the risk of a propofol infusion syndrome or myotonic crises can be elevated or the duration of the effect of non-depolarizing muscle relaxants can be changed in an incalculable way. In every case an individual anesthetic aproach including the avoidance of potential pharmacological or non-pharmacological triggers is essential for the safety of the patients.

Friedreich Ataksisi Olan Hastaların Skolyoz Cerrahisinde Anestezi Yönetimi: Dört Olgu Sunumu

Friedreich's ataxia (FRDA) is an autosomal recessive neurodegenerative disease, which may be accompanied by scoliosis, cardiac, endocrine and pulmonary comorbidities. We present our anesthesia experiences using total intravenous anesthesia (TIVA) method in scoliosis surgery of four patients with Friedreich ataxia. Patients were monitored with central venous pressure (CVP), bispectral index (BIS),invasive blood pressure and near infrared spectroscopy (NIRS). The risk of malignant hyperthermia and difficult airway are increased in patients with Friedreich's ataxia. We were prepared for difficult airway and provided dantrolene preoperatively. Neuromuscular blocker was used for anesthesia induction in one case, we did not use it in the other cases. Hypertrophic cardiomyopathy may accompany patients with Friedreich's ataxia. Therefore, detailed preoperative examination, intraoperative close bleeding, blood gas analysis, hemodynamic monitoring and postoperative multimodal analgesia for hemodynamic stability in patients with Friedreich's ataxia were performed. We aimed to discuss our anesthesia management with four patients with Friedreich's ataxia who underwent scoliosis surgery.

Malignant Hyperthermia in PICU-From Diagnosis to Treatment in the Light of Up-to-Date Knowledge.

Malignant Hyperthermia (MH) is a rare, hereditary, life-threatening disease triggered by volatile anesthetics and succinylcholine. Rarely, MH can occur after non-pharmacological triggers too. MH was detected more often in children and young adults, which makes this topic very important for every pediatric specialist, both anesthesiologists and intensivists. MH crisis is a life-threatening severe hypermetabolic whole-body reaction. Triggers of MH are used in pediatric intensive care unit (PICU) as well, volatile anesthetics in difficult sedation, status asthmaticus or epilepticus, and succinylcholine still sometimes in airway management. Recrudescence or delayed onset of MH crisis hours after anesthesia was previously described. MH can also be a cause of rhabdomyolysis and hyperpyrexia in the PICU. In addition, patients with neuromuscular diseases are often admitted to PICU and they might be at risk for MH. The most typical symptoms of MH are hypercapnia, tachycardia, hyperthermia, and muscle rigidity. Thinking of the MH as the possible cause of deterioration of a patient's clinical condition is the key to early diagnosis and treatment. The sooner the correct treatment is commenced, the better patient´s outcome. This narrative review article aims to summarize current knowledge and guidelines about recognition, treatment, and further management of MH in PICU.

Risk of malignant hyperthermia in patients carrying a variant in the skeletal muscle ryanodine receptor 1 gene

Malignant hyperthermia is a life-threatening disorder, which can be prevented by avoiding certain anesthetic agents. Pathogenic variants in the skeletal muscle ryanodine receptor 1-gene are linked to malignant hyperthermia. We retrospectively studied 15 patients who presented to our clinic with symptoms of muscle dysfunction (weakness, myalgia or cramps) and were later found to have a variant in the skeletal muscle ryanodine receptor 1-gene. Symptoms, creatine kinase levels, electromyography, muscle biopsy and in vitro contracture test results were reviewed. Six out of the eleven patients, with a variant of unknown significance in the skeletal muscle ryanodine receptor 1-gene, had a positive in vitro contracture test, indicating malignant hyperthermia susceptibility. In one patient, with two variants of unknown significance, both variants were required to express the malignant hyperthermia-susceptibility trait. Neurologists should consider screening the skeletal muscle ryanodine receptor 1-gene in patients with myalgia or cramps, even when few to no abnormalities on ancillary testing.

Malignant Hyperthermia and Absence of Ryanodine Receptors in a Child Presenting for Ptosis Repair

Introduction: Malignant hyperthermia (MH) is a hypermetabolic condition caused by a genetic cell membranes channel disruption leading to increased calcium release from the sarcoplasmic reticulum after exposure to triggering agents. Central Core Disease (CCD) is a rare inherited neuromuscular disorder with a wide spectrum of phenotypic presentations. Clinically, there is muscle weakness of variable degree and histopathologically there is evidence of core formation in the muscle fibers. Additional features may occur, including skeletal abnormalities such as foot deformities, scoliosis, or hip displacement. Association of CCD with a mutation of the ryanodine receptor RYR1 and MH is high. If not properly treated, MH could be fatal. Purpose: This study aims towards increasing the awareness of pediatricians, surgeons and anesthesiologists about possible occurrence of MH in patients presenting with only mild “clinical” signs of congenital myopathies such as CCD. Careful diagnostic investigations should be performed prior to surgical interventions and extreme caution during anesthesia. Material and Methods: We report the case of a 3 years old girl, with a history of ptosis, scoliosis, and good muscle tone, who developed unexpected MH during the surgical repair of her ptosis. The critically ill child was transferred to Pediatric Intensive Care Unit (PICU) for management of her MH. The management was done following the guidelines of the European Malignant Hyperthermia Group published in the British Journal of Anesthesia 2010. The patient responded well to treatment. She had a muscle biopsy done after recovery. Discussion: Early management and dantrolene administration are the most important factors to minimize MH morbidity and mortality. MH susceptibility is known in patients carrying mutations in the RYR1 receptors. In our case, RYR1 staining was negative on the muscle biopsy specimen suggesting abnormal or absent RYR1 function, with the presence of occasional cores. These findings, in addition to the ophthalmological and orthopedic history were diagnostic of CCD. In Vitro Contracture Testing (IVCT) was not done because it was not available. Genetic testing was refused by the parents due to financial reasons. Conclusion: In patients presenting with symptoms suggestive of muscle weakness, such as ptosis and scoliosis, and variable muscle tone; congenital myopathies should be considered. CCD and RYR1 receptors deficiency or mutation, if present, may lead to a possible risk of fatal MH, which can be prevented.

PHARMACOGENETIC ASPECTS OF THE USE OF PERIOPERATIVE DRUGS IN PEDIATRICS. Review

Relevance. It is known that in children the frequency of perioperative critical events due to the introduction of drugs is more than 5%. For example fentanyl, which is used as an adjunct to surgical anesthesia, is a major factor in increasing the number of deaths from opioid overdoses. Among the areas of reducing the frequency of severe adverse reactions to perioperative drugs - taking into account the pharmacogenetic variations of the patient.
 Objective is to consider current data on pharmacogenetic aspects of pharmacokinetics and pharmacodynamics of perioperative drugs.
 Methods. Analysis of the data presented in PubMed by keywords "pharmacogenetics", "general anesthetics", "analgesics", "muscle relaxants", "children". Search depth – 7 years (2014-2020), with a retrospective deepening of some positions until 2002.
 Results. The pharmacogenetic aspects of the pharmacokinetics of perioperative drugs are related to the genes of the enzymes that metabolize them and their transporters. Current data on the prevalence of polymorphic alleles of CYP2C9 genes (provides metabolism of nonsteroidal anti-inflammatory and anticonvulsant drugs) and CYP2D6 (metabolizes opioids, antidepressants, antiemetics) in Europe and, in particular, in Ukraine were presented. Thus, the inactive allele CYP2C19 * 2 was found in 13%, while the allele of increased activity CYP2C19 * 17 - in 25% of the population of Ukraine; allele with lost CYP2D6 function (CYP2D6 * 4) - in 18.6% of Ukrainians. Homozygotes with CYP2C9 * 3 polymorphism metabolize nonsteroidal anti-inflammatory drugs much more slowly than wild-type carriers, which may lead to their accumulation and side effects. The analgesic effect of codeine occurs only after it is metabolized in the liver by CYP2D6 to morphine. Respiratory depression, apnea and death may occur in patients with excessive metabolic rate even after a single dose of codeine; however, was noted the ineffectiveness of ondansetron due to a decrease in its concentration in the blood in thesepatients. Concomitant use of midazolam and fentanyl may prolong the effect of fentanyl by competing metabolism of midazolam by the hepatic enzyme CYP3A4, especially in patients with low metabolism. Plasma butyrylcholinesterase deficiency reduces succinylcholine inactivation in 1 in 1,500 people. Changing the genotype of uridine diphosphate glycosyltransferase causes increased glucuronidation of morphine, which may lead to a decrease in its effectiveness. As an example of pharmacogenetic changes in pharmacodynamics can be considered malignant hyperthermia, which occurs due to exposure to volatile anesthetics and depolarizing muscle relaxants in mutations in the RYR1 gene. Carriers of the minor allele (G) of the 5HT2A rs6313 gene require less propofol and 40% less time to start induction of anesthesia.
 Conclusions. The efficacy and safety of perioperative drugs are largely related to the pharmacogenetic aspects of their pharmacokinetics, especially mutations in genes of enzymes of the metabolism. Mutations in the genes of volatile anesthetic receptors and depolarizing muscle relaxants when their used cause a risk of malignant hyperthermia. The application of pharmacogenetics is the need to titrate drugs that have significant variability of action depending on the genotype. It is advisable to avoid the use of drugs with a high risk of pharmacogenetic reactions, if other alternative drugs are available.

Ontogeny of Pediatric Pharmacogenetics: Celebrating the Past and Vision for the Future.

Ontogeny of Pediatric Pharmacogenetics: Celebrating the Past and Vision for the Future.

The effect of statins on RyR and RyR-associated disease.

We sought to review the effects of statins on the ryanodine receptor (RyR) and on RyR-associated diseases, with an emphasis on catecholaminergic polymorphic ventricular tachycardia (CPVT). Statins can affect skeletal muscle and produce statin-associated muscle symptoms (SAMS) but have no adverse effects on cardiac muscle. These contrasting effects may be due to differences in how statins affect the skeletal (RyR1) and cardiac (RyR2) RyR. We searched PubMed to identify English language articles reporting the pathophysiology of the RyR, the effect of statins on RyR function, and on RyR-associated genetic diseases. We selected 150 articles for abstract review, 96 of which provided sufficient information to be included and were reviewed in detail. Fifteen articles highlighted the interaction of statins with the RyR. Nine identified the interaction of statins with RyR1, six addressed the interaction of statins with RyR2, 13 suggested that statins reduce ventricular arrhythmias (VA), and seven suggested that statins increase the risk of malignant hyperthermia (MH). In general, statins increase RyR1 and decrease RyR2 activity. We identified no articles examining the effect of statins on CPVT, a condition often caused by defects in RyR2. Statins appear to increase the risk of MH and decrease the risk of ventricular arrhythmia. The effect of statins on CPVT has not been directly examined, but statins' reduction in RyR2 function and their apparent reduction in VA suggest that they may be beneficial in this condition.

Rapid screening for mutations associated with malignant hyperthermia using high-resolution melting curve analysis.

Objectives:The diagnosis of malignant hyperthermia (MH) is based on clinical signs or laboratory testing. The gold standard laboratory test is the in vitro contracture test, although it is invasive, expensive, and only performed at specialized centers. Genetic diagnosis is another option, although direct mutation screening is a laborious task. Therefore, we evaluated whether high-resolution melting (HRM) curve analysis could be used as a rapid screening tool to target MH-associated mutations.Materials and Methods:The feasibility of HRM analysis was evaluated using plasmids that were constructed by cloning wild-type or mutated versions of the ryanodine receptor 1 (RYR1) gene into the pCR2.1 plasmid. We obtained engineered plasmids and patient DNA extracted from blood samples with known wild-type or mutated sequences that are associated with MH. Amplicon lengths were kept relatively short (<250 bp) to improve discrimination between the engineered and patient plasmids. Real-time polymerase chain reaction (PCR) cycling and HRM analysis of the engineered plasmids and patient DNA were performed using the LightCycler 480 System (Roche).Results:The HRM results were clearly different from those obtained using real-time PCR. Furthermore, the HRM analysis provided sufficient resolution to identify two single-nucleotide variants in the tested RYR1 exons.Conclusion:We conclude that HRM analysis can provide high resolution for identifying single-nucleotide variants in RYR1, which might be useful for predicting the risk of MH in the preanesthesia setting.

Anaesthesia and orphan disease: A patient with neuromyotonia undergoing single lung ventilation.

Anaesthesia and orphan disease: A patient with neuromyotonia undergoing single lung ventilation.

Anesthetic Management for Emergency Cesarean Section Considering Risk of Malignant Hyperthermia

Anesthetic Management for Emergency Cesarean Section Considering Risk of Malignant Hyperthermia

Significance of Asymptomatic Hyper Creatine-Kinase Emia.

Whether asymptomatic hyper-CKemia (AHCE) should prompt a thorough work-up for muscle disease or not is controversially discussed. This review aims at summarizing and discussing recent findings concerning the cause, frequency, evolution, and work-up of conditions manifesting as AHCE and normal or abnormal electromyography (EMG) respectively muscle biopsy. Systematic PubMed search. There are numerous primary (hereditary) and acquired myopathies that manifest with permanent, recurrent, or temporary AHCE with/without myopathic EMG or muscle biopsy. AHCE particularly occurs at onset of these conditions, which include dystrophinopathies, myotilinopathies, calpainopathy, caveolinopathy, dysferlinopathy, central core disease, multicore disease, desminopathy, MD1, MD2, hypoPP, malignant hyperthermia susceptibility, Pompe disease, McArdle disease, myoadenylate deaminase-deficiency, CPT2-deficiency, mitochondrial disorders, or myopathy with tubular aggregates. Most likely, other primary myopathies manifest with AHCE as well, without having been reported. Patients with AHCE should be taken seriously and repeated CK determination must be conducted. If hyper-CKemia is persisting or recurrent, these patients should undergo an EMG and eventually muscle biopsy. If noninformative, genetic work-up by a panel or whole exome sequencing should be initiated, irrespective of the family history. Patients with AHCE should avoid excessive exercise, require sufficient hydration, require counseling with regard to the risk of malignant hyperthermia, and should inform anesthesiologists and surgeons about their condition before elective surgery. Recurrent AHCE should be taken seriously and managed with conventional work-up. If noninformative, genetic work-up should follow irrespective of the family history.

Risk-Stratifying Patients at an Increased Risk of Malignant Hyperthermia: Improving Safety by Expanding Beyond Classic Tenets

Malignant hyperthermia (MH) is classically described as an autosomal dominant myopathy involving type 1 ryanodine receptor (RYR1), which predisposes affected individuals to potentially life-threatening sequela when exposed to volatile anesthetics or depolarizing skeletal muscle relaxants, such as succinylcholine. In cases where malignant hyperthermia or anesthetic induced rhabdomyolysis (AIR) is triggered, significant morbidity and often mortality can result. In an ambulatory anesthetic setting, family history of malignant hyperthermia and history of anesthetic-related events is typically utilized to screen out susceptible individuals. Furthermore, in cases where suspicion of increased risk for MH susceptibility is present, volatile agents and depolarizing muscle relaxants are avoided, per recommendations of Malignant Hyperthermia Association of US. However, MH phenotype has been found to be inherited with incomplete penetrance and variable expression. Investigations into the molecular genetics of MH have revealed over 400 different variants in the RYR1 gene, with fewer than 40 variants that have been functionally characterized. Exome studies have suggested that the incidence of RYR1 variants in general population is as high as 1 in 2000-3000 persons. These data suggest that potential for triggering malignant hyperthermia is much greater than previously estimated, even if a fulminant reaction is not encountered. Estimates based on genomic analysis call for increased index of suspicion in patient selection for ambulatory anesthesia, compelling for improved risk stratification, as well as improved level of preparedness to treat malignant hyperthermia. This is especially pertinent for providers that administer anesthesia to pediatric populations, as pediatric patients have been documented to exhibit MH/AIR response in the absence of volatile anesthetic exposure.

Postpartum psychosis – case report on effective treatment of electroconvulsive therapy in a patient with a history of neuroleptic malignant syndrome

In this paper, we present a case of a 31-year-old patient with postpartum psychosis of an unusual course. In patients with antipsychotics, there have been significant complications of treatment in the form of a neuroleptic malignant syndrome. Because of the complications of pharmacological treatment, it was decided to withdraw antipsychotics and to use electroconvulsive therapy. Propofol was used for the anaesthesia of the patient; cisatracurium was used for muscle relaxation. In case of neuroleptic malignant syndrome, succinylcholine is contraindicated because of the increased risk of malignant hyperthermia. Due to the use of long-term anaesthetic agents, the patient required temporary intubation and respiratory support. A total of 8 ECT procedures were performed, complete disappearance of positive symptoms was achieved. After electroconvulsive treatment, aripiprazole 5 mg/d was administered, no significant complications were observed. The patient was discharged home with full mental status.

Neuromuscular Blockers and Reversal Agents

Neuromuscular blocking drugs, which include depolarizing and nondepolarizing drugs, are used to facilitate intubation and provide skeletal muscle relaxation during surgery and in the intensive care unit. The agents differ in their mechanism, duration of action, side-effect profile, and metabolism. Succinylcholine is the only depolarizing agent in clinic use and is typically used for emergent control of the airway, rapid sequence intubations, and short surgical procedures. The risk of hyperkalemia in certain clinical conditions and risk of malignant hyperthermia in susceptible individuals limit the use of succinylcholine in specific patient populations. Nondepolarizing agents vary in their duration of action, but all provide muscle relaxation for a longer duration than succinylcholine. Clinical effects of neuromuscular blocking drugs can be assessed with neuromuscular monitoring, although there is significant variability among providers in the regular use of neuromuscular monitoring. Reversal agents are used to restore neuromuscular transmission, as residual neuromuscular blockade after extubation has been associated with multiple adverse events, including hypoxemia, atelectasis, and aspiration. Sugammadex is an encapsulating agent capable of immediately reversing the effects of rocuronium-induced neuromuscular blockade that will likely impact the way many providers administer rocuronium and may decrease the future use of succinylcholine. This review contains 4 figures, 6 tables, and 41 references. Keywords: hyperkalemia, neuromuscular monitoring, neuromuscular transmission, nondepolarizing neuromuscular blocking agents, residual neuromuscular blockade, reversal agents, succinylcholine sugammadex, sugammadex

Comment on "Schwartz-Jampel syndrome: Is risk of malignant hyperthermia the same as that of the general population?"

Comment on "Schwartz-Jampel syndrome: Is risk of malignant hyperthermia the same as that of the general population?"

Epidural Anesthesia for Caesarean Section in a Patient with Risk of Malignant Hyperthermia

Abstract Malignant hyperthermia is a hypermetabolic disorder of skeletal muscle that occurs in genetically susceptible individuals after exposure to anesthetic. Basic disorder is an increase of calcium ions inside the skeletal muscle, increasing metabolism and reducing cell energy supplies leading to development of acidosis, cell membrane destruction and cell death. Due to the increased metabolism occurs hypercarbia and strong stimulation of the sympathetic nervous system (tachycardia, hypertension, ventricular arrhythmia, tachypnea dropped for the neuromuscular blockade). Sweating, cyanosis, muscle rigidity and hyperthermia are also present. This work presents the case of a female patient aged 32 who was heterozygous for the mutation RYR1 gene and therefore has an increased risk of malignant hyperthermia. Per anamnesis we got data that patient’s brother suffers from central core disease (myopathy). Patient has no muscle disease, 41st week of pregnancy and was admitted to the hospital for childbirth. Vaginal delivery in epidural analgesia was planned. Epidural catheter is placed in the space L3 - L4, through which she received 0.25% levobupivacaine 10 ml. Due to adverse obstetric findings cesarean section underwent after two hours. Given the increased risk of malignant hyperthermia, the safest type of anesthesia for cesarean is epidural anesthesia. Over the epidural catheter has received 0.5% levobupivacaine 18 ml. Anesthesia machine was verified, hoses were replaced with new ones, CO2 absorber system was replaced, and whole system is flushed with pure oxygen, before surgery started. During the operation the patient had stable vital parameters that are monitored. She got a male child Apgar score of 9/10 and saw her child at birth. After the operation was transferred to the intensive care unit where we monitored the vital parameters, laboratory analysis, the amount and color of urine. Since all parameters were satisfactory, following day she was transferred to the ward, and she was discharged with a child on the fourth day after the surgery.

Difficult airway management in children and young adults with arthrogryposis.

ObjectiveTo review current evidence and experience with anesthesia and airway management issues in children and young adults with arthrogryposis.Data sourcesReview of existing world literature and description of personal experience at a center for children's orthopedic surgery and rehabilitation over 2 decades.MethodsDescription of common problems and their solutions in this unusual and diverse group of patients.ResultsArthrogryposis multiplex congenital includes more than 400 conditions that lead to congenital joint contractures affecting more than one body area. Among the many causes of arthrogryposis, 50%–65% fall into two large categories – amyoplasia and distal arthrogryposis. There is general agreement that best function in children with arthrogryposis is achieved through early mobilization of joint contractures. Children with arthrogryposis average >5 operative procedures during childhood. Anesthesia for these procedures may be complicated by limited jaw mobility and mouth opening, restricted lung development, positioning difficulties, difficult venous access and concerns about increased risk for malignant hyperthermia. 75% of arthrogryposis patients do not have a difficult airway. For those with a history of airway problems or those meeting criteria for a difficult airway, careful advanced planning helps to assure safe and successful surgery. We describe several specialized techniques for endotracheal intubation of children with arthrogryposis.ConclusionsChildren and young adults with arthrogryposis are a diverse group. Many pose unique challenges for airway and surgical management. Review of individual anesthesia records and careful advanced planning by a coordinated, experienced airway team can lead to best outcomes from arthrogryposis surgery.