Gloria Y Chang1, Tate Salazar2, Abhishek Karnwal3,4, Sheila S Kun5, Josephine Ellashek5, Cathy E Shin3,6, J Gordon McComb3,7, Thomas G Keens3,5, Iris A Perez8,9. 1. Department of Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA. 2. , Los Angeles, CA, USA. 3. Keck School of Medicine of University of Southern California, Los Angeles, CA, USA. 4. Department of Anesthesia and Critical Care Medicine, Children's Hospital Los Angeles, Los Angeles, CA, USA. 5. Division of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, MS 83, 4650 Sunset Blvd, Los Angeles, CA, 900217, USA. 6. Department of Surgery, Children's Hospital Los Angeles, Los Angeles, CA, USA. 7. Department of Surgery/Division of Neurosurgery, Children's Hospital Los Angeles, Los Angeles, CA, USA. 8. Keck School of Medicine of University of Southern California, Los Angeles, CA, USA. iaperez@chla.usc.edu. 9. Division of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, MS 83, 4650 Sunset Blvd, Los Angeles, CA, 900217, USA. iaperez@chla.usc.edu.
Abstract
PURPOSE: Patients with congenital central hypoventilation syndrome (CCHS) have autonomic dysfunction and lack ventilatory responses to hypoxemia and hypercarbia and thus are prone to adverse events during general anesthesia. The objective of this study was to describe the perioperative outcomes of patients with CCHS who were undergoing diaphragm pacer (DP) implantation surgeries under general anesthesia. METHODS: A retrospective cohort study was conducted on patients with CCHS who underwent DP implantation surgeries at CHLA between January 2000 and May 2016. Charts were reviewed for demographics, PHOX2B genotype, ventilatory support, comorbidities, anesthesia administered, and perioperative courses. RESULTS: Of 19 patients with CCHS (58% female) mean age at surgeries was 8.6 ± 5.8 years. Seventeen patients were ventilator-dependent during sleep only; two were ventilator dependent 24 h per day. Mean surgery duration was 3.1 ± 0.5 h. Seventeen patients were extubated to PPV via tracheostomy in the OR. Two patients were extubated to NPPV on postoperative day (POD) 1. Mean transition time to home ventilator or NPPV was 3.0 ± 2.2 days, and mean hospital stay was 5.0 ± 2.1 days. One patient premedicated without ventilatory support developed hypoxemia and hypoventilation. Ten patients (52%) had intraoperative events such as bradycardia, hypotension, significant hypoxemia, and bronchospasm. Fifteen patients had postoperative events. Hypoxemia, pneumonia, and atelectasis accounted for most of perioperative complications. One patient experienced seizure on POD 2 due to hypercarbia. CONCLUSION: Patients with CCHS are vulnerable to the cardiorespiratory effects of sedative and anesthetic agents. Therefore, they require vigilant monitoring and optimal ventilatory support in the perioperative period.
PURPOSE: Patients with congenital central hypoventilation syndrome (CCHS) have autonomic dysfunction and lack ventilatory responses to hypoxemia and hypercarbia and thus are prone to adverse events during general anesthesia. The objective of this study was to describe the perioperative outcomes of patients with CCHS who were undergoing diaphragm pacer (DP) implantation surgeries under general anesthesia. METHODS: A retrospective cohort study was conducted on patients with CCHS who underwent DP implantation surgeries at CHLA between January 2000 and May 2016. Charts were reviewed for demographics, PHOX2B genotype, ventilatory support, comorbidities, anesthesia administered, and perioperative courses. RESULTS: Of 19 patients with CCHS (58% female) mean age at surgeries was 8.6 ± 5.8 years. Seventeen patients were ventilator-dependent during sleep only; two were ventilator dependent 24 h per day. Mean surgery duration was 3.1 ± 0.5 h. Seventeen patients were extubated to PPV via tracheostomy in the OR. Two patients were extubated to NPPV on postoperative day (POD) 1. Mean transition time to home ventilator or NPPV was 3.0 ± 2.2 days, and mean hospital stay was 5.0 ± 2.1 days. One patient premedicated without ventilatory support developed hypoxemia and hypoventilation. Ten patients (52%) had intraoperative events such as bradycardia, hypotension, significant hypoxemia, and bronchospasm. Fifteen patients had postoperative events. Hypoxemia, pneumonia, and atelectasis accounted for most of perioperative complications. One patient experienced seizure on POD 2 due to hypercarbia. CONCLUSION: Patients with CCHS are vulnerable to the cardiorespiratory effects of sedative and anesthetic agents. Therefore, they require vigilant monitoring and optimal ventilatory support in the perioperative period.
Authors: Bonnie Diep; Annie Wang; Sheila Kun; J Gordon McComb; Donald B Shaul; Cathy E Shin; Thomas G Keens; Iris A Perez Journal: Respiration Date: 2015-04-25 Impact factor: 3.580
Authors: Debra E Weese-Mayer; Elizabeth M Berry-Kravis; Isabella Ceccherini; Thomas G Keens; Darius A Loghmanee; Ha Trang Journal: Am J Respir Crit Care Med Date: 2010-03-15 Impact factor: 21.405
Authors: Jean M Silvestri; Maida L Chen; Debra E Weese-Mayer; John M McQuitty; Holly J Carveth; Dennis W Nielson; Drucy Borowitz; Frank Cerny Journal: Am J Med Genet Date: 2002-09-15