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1.
Cureus ; 13(9): e18168, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34707951

ABSTRACT

Propionic acidemia is a rare genetic disorder of metabolism that predisposes patients to metabolic acidosis, lethargy, neurologic dysfunction, developmental delays, and cardiomyopathy. Perioperative anesthetic management is guided toward mitigating the effects of preoperative fasting times, maintaining normovolemia, and preventing cardiovascular complications secondary to underlying cardiomyopathy. Commonly used anesthetic agents may have undesirable side effects in these patients. Propofol, the lactate in Lactated Ringer's, and neuromuscular blocking agents that undergo ester hydrolysis are poorly metabolized and can lead to metabolic acidosis. Opioids, such as fentanyl and morphine, should be used judiciously in patients with coexisting developmental delays to avoid oversedation and delayed time to resuming oral intake postanesthesia. In addition, inhaled anesthetics have direct myocardial depressive effects and can compromise cardiac function in the setting of pre-existing cardiomyopathy. The perioperative period represents a critical time in this population and appropriate planning is crucial to prevent perioperative morbidity. We present a case of an eight-year-old child undergoing esophagogastroduodenoscopy under general anesthesia and describe the anesthetic concerns we addressed to provide a safe perioperative course.

2.
J Pediatr Genet ; 10(2): 152-155, 2021 Jun.
Article in English | MEDLINE | ID: mdl-33996187

ABSTRACT

Surgical correction for scoliosis is undertaken to avoid progression to cardiopulmonary compromise as well as improve the patient's overall quality of life. In this case report, we presented a case of a 14-year-old girl with epidermolysis bullosa simplex and Gitelman's syndrome who underwent posterior spinal fusion for scoliosis. The perioperative planning and intraoperative management of a patient with this unique combination of comorbidities undergoing a complex, high-risk surgical procedure were not previously chronicled in the literature. We detailed the steps undertaken to optimize the patient prior to surgery and the unique intraoperative surgical and anesthetic considerations that led to a successful completion of the surgery and recovery.

3.
Lancet ; 396(10266): 1905-1913, 2020 12 12.
Article in English | MEDLINE | ID: mdl-33308472

ABSTRACT

BACKGROUND: Orotracheal intubation of infants using direct laryngoscopy can be challenging. We aimed to investigate whether video laryngoscopy with a standard blade done by anaesthesia clinicians improves the first-attempt success rate of orotracheal intubation and reduces the risk of complications when compared with direct laryngoscopy. We hypothesised that the first-attempt success rate would be higher with video laryngoscopy than with direct laryngoscopy. METHODS: In this multicentre, parallel group, randomised controlled trial, we recruited infants without difficult airways abnormalities requiring orotracheal intubation in operating theatres at four quaternary children's hospitals in the USA and one in Australia. We randomly assigned patients (1:1) to video laryngoscopy or direct laryngoscopy using random permuted blocks of size 2, 4, and 6, and stratified by site and clinician role. Guardians were masked to group assignment. The primary outcome was the proportion of infants with a successful first attempt at orotracheal intubation. Analysis (modified intention-to-treat [mITT] and per-protocol) used a generalised estimating equation model to account for clustering of patients treated by the same clinician and institution, and adjusted for gestational age, American Society of Anesthesiologists physical status, weight, clinician role, and institution. The trial is registered at ClinicalTrials.gov, NCT03396432. FINDINGS: Between June 4, 2018, and Aug 19, 2019, 564 infants were randomly assigned: 282 (50%) to video laryngoscopy and 282 (50%) to direct laryngoscopy. The mean age of infants was 5·5 months (SD 3·3). 274 infants in the video laryngoscopy group and 278 infants in the direct laryngoscopy group were included in the mITT analysis. In the video laryngoscopy group, 254 (93%) infants were successfully intubated on the first attempt compared with 244 (88%) in the direct laryngoscopy group (adjusted absolute risk difference 5·5% [95% CI 0·7 to 10·3]; p=0·024). Severe complications occurred in four (2%) infants in the video laryngoscopy group compared with 15 (5%) in the direct laryngoscopy group (-3·7% [-6·5 to -0·9]; p=0·0087). Fewer oesophageal intubations occurred in the video laryngoscopy group (n=1 [<1%]) compared with in the direct laryngoscopy group (n=7 [3%]; -2·3 [-4·3 to -0·3]; p=0·028). INTERPRETATION: Among anaesthetised infants, using video laryngoscopy with a standard blade improves the first-attempt success rate and reduces complications. FUNDING: Anaesthesia Patient Safety Foundation, Society for Airway Management, and Karl Storz Endoscopy.


Subject(s)
Airway Management/statistics & numerical data , Intubation, Intratracheal , Laryngoscopy/statistics & numerical data , Video Recording , Australia , Esophagus , Female , Hospitals, Pediatric , Humans , Infant , Intention to Treat Analysis , Male , United States
4.
Paediatr Anaesth ; 30(10): 1149-1152, 2020 10.
Article in English | MEDLINE | ID: mdl-32761718

ABSTRACT

Stiff skin syndrome is a rare genetic disorder that is present in infancy or early childhood. It is characterized by hard, inflexible skin and limited joint mobility making anesthetic management of these patients challenging. Their limited neck flexibility and chest wall rigidity make intubation and mask ventilation difficult. Intraoperative positioning can be challenging due to joint contractures and potential entrapment peripheral neuropathy. Even though peripheral intravenous access can be relatively easy, central venous cannulation may be problematic due to the hard skin overlying the entry sites. Our case report details the anesthetic management and considerations of a pediatric patient with stiff skin syndrome.


Subject(s)
Anesthetics , Contracture , Child , Child, Preschool , Humans , Skin Diseases, Genetic
5.
Anesth Analg ; 131(1): 61-73, 2020 07.
Article in English | MEDLINE | ID: mdl-32287142

ABSTRACT

The severe acute respiratory syndrome coronavirus 2 (coronavirus disease 2019 [COVID-19]) pandemic has challenged medical systems and clinicians globally to unforeseen levels. Rapid spread of COVID-19 has forced clinicians to care for patients with a highly contagious disease without evidence-based guidelines. Using a virtual modified nominal group technique, the Pediatric Difficult Intubation Collaborative (PeDI-C), which currently includes 35 hospitals from 6 countries, generated consensus guidelines on airway management in pediatric anesthesia based on expert opinion and early data about the disease. PeDI-C identified overarching goals during care, including minimizing aerosolized respiratory secretions, minimizing the number of clinicians in contact with a patient, and recognizing that undiagnosed asymptomatic patients may shed the virus and infect health care workers. Recommendations include administering anxiolytic medications, intravenous anesthetic inductions, tracheal intubation using video laryngoscopes and cuffed tracheal tubes, use of in-line suction catheters, and modifying workflow to recover patients from anesthesia in the operating room. Importantly, PeDI-C recommends that anesthesiologists consider using appropriate personal protective equipment when performing aerosol-generating medical procedures in asymptomatic children, in addition to known or suspected children with COVID-19. Airway procedures should be done in negative pressure rooms when available. Adequate time should be allowed for operating room cleaning and air filtration between surgical cases. Research using rigorous study designs is urgently needed to inform safe practices during the COVID-19 pandemic. Until further information is available, PeDI-C advises that clinicians consider these guidelines to enhance the safety of health care workers during airway management when performing aerosol-generating medical procedures. These guidelines have been endorsed by the Society for Pediatric Anesthesia and the Canadian Pediatric Anesthesia Society.


Subject(s)
Airway Management/methods , Anesthesiology/methods , Coronavirus Infections/therapy , Intubation, Intratracheal/methods , Pediatrics/methods , Pneumonia, Viral/therapy , Adolescent , Anesthesia/methods , Anesthesiology/standards , COVID-19 , Child , Child, Preschool , Consensus , Guidelines as Topic , Humans , Infant , Infant, Newborn , Infection Control , Infectious Disease Transmission, Patient-to-Professional/prevention & control , Intubation, Intratracheal/standards , Pandemics , Pediatrics/standards
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