Marco Corridore

Eteplirsen for the treatment of Duchenne muscular dystrophy

In prior open‐label studies, eteplirsen, a phosphorodiamidate morpholino oligomer, enabled dystrophin production in Duchenne muscular dystrophy (DMD) with genetic mutations amenable to skipping exon 51. The present study used a double‐blind placebo‐controlled protocol to test eteplirsens ability to induce dystrophin production and improve distance walked on the 6‐minute walk test (6MWT).

Need for emergency surgical airway reduced by a comprehensive difficult airway program.

BACKGROUND: Inability to intubate and ventilate patients with respiratory failure is associated with significant morbidity and mortality. A patient is considered to have a difficult airway if an anesthesiologist or other health care provider experienced in airway management is unable to ventilate the patients lungs using bag-mask ventilation and/or is unable to intubate the trachea using direct laryngoscopy. METHODS: We performed a retrospective review of a departmental database to determine whether a comprehensive program to manage difficult airways was associated with a reduced need to secure the airway surgically via cricothyrotomy or tracheostomy. The annual number of unplanned, emergency surgical airway procedures for inability to intubate and ventilate reported for the 4 yr before the program (January 1992 through December 1995) was compared with the annual number reported for the 11 yr after the program was initiated (January 1996 through December 2006). RESULTS: The number of emergency surgical airways decreased from 6.5 ± 0.5 per year for 4 yr before program initiation to 2.2 ± 0.89 per year for the 11-yr period after program initiation (P < 0.0001). During the 4-yr period from January 1992 through December 1995, 26 surgical airways were reported, whereas only 24 surgical airways were performed in the subsequent 11-yr period (January 1996 through December 2006). CONCLUSIONS: A comprehensive difficult airway program was associated with a reduction in the number of emergency surgical airway procedures performed for the inability of an anesthesiologist to intubate and ventilate, a reduction that was sustained over an 11-yr period. This decrease occurred despite an increase in the number of patients reported to have a difficult airway and an overall increase in the total number of patients receiving anesthesia per year.

Dexmedetomidine-ketamine sedation during bone marrow aspirate and biopsy in a patient with duchenne muscular dystrophy

Sedation during invasive procedures not only provides appropriate humanitarian care for patients, but also facilitates the completion of invasive procedures. Although generally safe and effective, adverse effects may occur especially in patients with co-morbid diseases. We present the successful use of a combination of dexmedetomidine and ketamine to provide sedation and analgesia in a 21-year-old patient with Duchenne muscular dystrophy (DMD) undergoing bone marrow aspiration and biopsy. Co-morbidities included both depressed myocardial function and impaired respiratory function. Dexmedetomidine was administered as a loading dose of 1 μg/kg over 5 min followed by an infusion of 1 μg/kg/h. Ketamine (20 mg) was administered along with the dexmedetomidine loading dose. An additional 10 mg of ketamine was administered to treat the pain experienced during the placement of the local anesthetic agent prior to the procedure. No clinically significant hemodynamic or respiratory changes were noted. The patient tolerated the procedure well and was discharged home. A review of previously published reports of dexmedetomidine and ketamine for procedural sedation are reviewed.

Dexmedetomidine-Ketamine Sedation in a Child With a Mediastinal Mass

Sedation during invasive procedures provides appropriate humanitarian care as well as facilitates the completion of procedures. Although generally safe and effective, adverse effects may occur especially in patients with comorbid diseases. One particularly challenging situation is the child with an anterior mediastinal mass who requires sedation during performance of a biopsy to obtain a tissue diagnosis. When there is evidence of airway compromise, it is generally accepted that the maintenance of spontaneous ventilation is necessary as complete airway obstruction may occur, if positive pressure ventilation is chosen. We present the use of a dexmedetomidine–ketamine combination for procedural sedation in a three-year-old child who presented with a large mediastinal mass and respiratory compromise. Previous reports regarding the use of dexmedetomidine and ketamine for procedural sedation are reviewed and the potential efficacy of this combination is discussed.

Perioperative care of an infant with an anomalous left innominate artery arising from the main pulmonary artery.

A 1.4-kilogram, male infant was born at 27 weeks gestation at an outside hospital. After birth, the patient’s trachea was intubated and surfactant administered. Initial echocardiogram showed a patent ductus arteriosus (PDA) and a ventricular septal defect (VSD), with a possible aberrant vessel arising from the carotid artery. The patient was transferred to our hospital for further management. Repeat echocardiogram showed a right aortic arch with the left common carotid/innominate artery arising from the pulmonary artery. The infant was subsequently brought to the operating room where following sternotomy, the aberrant left innominate artery was reimplanted into the aorta. The origin of the left carotid artery or innominate artery from the pulmonary artery is an extremely rare form of congenital heart disease with a limited number of reports in the literature. The differential pressure gradient between the systemic and pulmonary beds may lead to a steal phenomenon, with shunting of blood from the carotid system into the pulmonary vascular bed, resulting in decreased cerebral blood flow. The left-to-right shunting from the systemic to pulmonary bed may also lead to volume overload of the left side of the heart. In the perioperative care of such patients, control of physiologic factors that control the balance between the pulmonary and systemic vascular resistance is mandatory to ensure that cerebral blood flow is maintained.

A Multidisciplinary Approach to a Pediatric Difficult Airway Simulation Course

Objective To design and assess an advanced pediatric airway management course, through simulation-based team training and with multiple disciplines, to emphasize communication and cooperation across subspecialties and to provide a common skill set and knowledge base. Methods Trainees from anesthesiology, emergency medicine, critical care, pediatric surgery, and otolaryngology at a tertiary children’s hospital participated in a 1-day workshop emphasizing airway skills and complex airway simulations. Small groups were multidisciplinary to promote teamwork. Participants completed pre- and postworkshop questionnaires. Results Thirty-nine trainees participated over the 3-year study period. Compared with their precourse responses, participants’ postcourse responses indicated either agreement or strong agreement that the multidisciplinary format (1) helped in the development of team communication skills and (2) was preferred over single-discipline training. Improvement in confidence in managing critical airway situations and in advanced airway management skills was significant (P < .05). Eighty-one percent of participants had improved confidence in following the hospital’s critical airway protocol, and 64% were better able to locate advanced airway management equipment. Discussion Multiple subspecialists manage pediatric respiratory failure, where successful care requires complex handoffs and teamwork. Multidisciplinary education to teach advanced airway management, teamwork, and communication skills is practical and preferred by learners and is possible to achieve despite differences in experience. Future study is required to better understand the impact of this course on patient care outcomes. Implications for Practice Implementation of a pediatric difficult airway course through simulation-based team training is feasible and preferred by learners among multiple disciplines. A multidisciplinary approach exposes previously unrecognized knowledge gaps and allows for better communication and collaboration among the fields.

Double-Blind Randomized Placebo-Controlled Trial of Single-Dose Intravenous Acetaminophen for Pain Associated With Adenotonsillectomy in Pediatric Patients With Sleep-Disordered Breathing

OBJECTIVES Adequate pain control is an important component in the postoperative outcome for pediatric adenotonsillectomy patients with sleep-disordered breathing (SDB). Intravenous acetaminophen appears to be a favorable analgesic adjunct owing to its predictable pharmacokinetics and opioid-sparing effects; however, its role in pediatric adenotonsillectomy pain management remains unclear. METHODS In this prospective, randomized, double-blinded, controlled study, subjects with the diagnosis of SDB, aged 2 to 8 years, who required extended postoperative admission, received intravenous acetaminophen (15 mg/kg) or saline placebo intraoperatively in addition to morphine (0.1 mg/kg) for postoperative surgical analgesia. Pain scores in the postanesthesia care unit (PACU) using the FLACC (Faces, Leg, Activity, Cry, Consolability) score were used to determine the need for supplemental analgesic agents in the PACU. The PACU time and time to the first request for pain medication on the inpatient ward were also measured. RESULTS A total of 239 patients were included in the final data analysis (118 in the intravenous acetaminophen group and 121 in the saline placebo group). The 2 groups did not differ in the proportion of patients reaching FLACC scores = 4 in the PACU (p = 0.223); mean FLACC scores in the PACU (p = 0.336); mean PACU time (p = 0.883); or time to requesting pain medication on the inpatient ward (p = 0.640). CONCLUSIONS A single intraoperative dose of intravenous acetaminophen did not alter the postoperative course of pediatric patients with SDB following adenotonsillectomy.

Reply to Adam Adler and Arvind Chandrakantan regarding their comment “Nursing initiated tracheal extubation in PACU, the risk of delegating critical anesthesiology tasks in the interest of speed”

Sir—We would like to thank Drs. Adler and Chandrakantan for their interest in our manuscript regarding tracheal extubation practices following adenotonsillectomy in children. Their first query concerns the unclear reasons for excluding some cases from the control institution when analyzing the duration of postanesthesia care unit (PACU) stay. As noted in our original manuscript, these patients were discharged home directly from the PACU and therefore their duration of PACU stay was exceptionally long compared to data from Nationwide Children’s Hospital where the patients were discharged to an individual room where they received discharge and home care instructions in quiet environment from the nurses prior to discharge. As PACU stay durations were also more skewed in the control institution, we used the data reported in our manuscript to calculate the median postoperative length of stay (LOS) as 69 minutes at Nationwide Children’s Hospital (interquartile range [IQR]: 55, 88) compared to 80 minutes at the control institution (IQR: 60, 111). The 95% confidence interval (CI) of this difference in medians was 6-14 (P < .001 on Wilcoxon rank-sum test). If we had added the 167 control institution cases excluded from our original analysis, median PACU LOS in the control institution would have increased to 95 minutes (IQR: 65, 229), and the 95% CI of the difference in medians would have become 22-35. Clearly, the exclusion described in our manuscript led to a conservative estimate of differences in PACU LOS between the 2 institutions. Aside from this consideration, PACU LOS was calculated from the arrival in the PACU for both groups. The PACU discharge criteria were not standardized in the 2 institutions, and as noted in our manuscript, this was one of the limitations of our retrospective study. Our primary objective was to determine differences in operating room (OR) time, especially time from completion of the surgical procedure to exit from the OR, according to the institutional practice of tracheal extubation. The primary analyses from our study hold even when the cases with unusually prolonged PACU stay in the control institution were excluded, due to variation in the practice of discharging patients directly from the PACU. Drs. Adler and Chandrakantan also expressed a concern regarding the patient safety implications of tracheal extubation in the PACU and that such a practice eliminates a key component of what an anesthesiologist does. As perioperative physicians, however, our role is much greater in the overall care of patients during the many phases of anesthetic care. Over the years, our role has grown from merely an intraoperative care provider to a consultant during the entire perioperative process. As anesthesiologists, one of our roles is to determine when and where it is appropriate to extubate the trachea and also, who should perform this task. If we deem a patient safe for tracheal extubation in the PACU with a well-trained nurse, whom we have personally trained, we believe that this remains consistent with the role of a perioperative physician. The mere task of tracheal extubation should not define us. Equally as important, is to ensure that we continue to perform tracheal extubation in the OR in specific patient populations. Tracheal extubation in the PACU does not mean sacrificing deliberate practice and patient safety to save money and gain speed. They can exist together. As Drs. Adler and Chandrakantan mentioned, production pressure has been shown to relate to perioperative medical errors and adverse events. By performing tracheal extubation in the PACU, we remove the production pressure in the OR, where we believe tracheal extubation at times is hurried and perhaps performed at an inappropriate plane of anesthesia. Tracheal extubation in the PACU allows the patient enough time to fully emerge without production pressure. As outlined in our article, the process of tracheal extubation must be deliberate, with clear policies and procedures including training and recredentialing of the nursing staff. We do not routinely allow parents in the PACU, thereby eliminating that source of distraction or concern. Finally, what Adler et al. called “PACU nursing driven extubation” is not without supervision and direction of the anesthesiology staff. We carry walkie-talkies for instant communication, ensuring rapid response times for all phases of recovery in the PACU. While tracheal extubation is a key time point in the postoperative process, it is well known that sudden adverse airway events may occur before or following tracheal extubation. This mandates our constant ability to respond to problems in the PACU. The process of PACU tracheal extubation is only one aspect of team-based anesthesia care where anesthesiologists and nurses work together to provide high-quality and safe perioperative care as well as efficiency.

Perioperative Management of the Fontan Operation in an Adolescent With a Single Lung

The Fontan operation is performed to correct complex cardiac malformations characterized by single ventricle physiology. Following the procedure, the systemic venous blood flows directly into the lungs without passing through the single ventricle. Consequently, the Fontan circulation requires passive venous flow to the lungs, making it imperative for the pulmonary vascular resistance to be kept low in order to maintain adequate pulmonary blood flow and cardiac output. Given that the pulmonary circulation is important for a successful Fontan physiology, it would be intuitive that a single lung Fontan operation would do poorly because of the major loss in pulmonary vascular bed and the subsequent increase in pulmonary vascular resistance. The authors present a 14-year-old adolescent who was born with tricuspid atresia, pulmonary atresia, and right ventricular hypoplasia who underwent a successful Fontan operation into a single right lung, the left pulmonary artery being hypoplastic and disconnected from the...