Neonatal Carotid Artery and Internal Jugular Vein Management Practices at Extracorporeal Membrane Oxygenation Decannulation: No Standard Approach.

BACKGROUND: There are currently no commonly accepted standardized guidelines for management of cervical vessels at neonatal extracorporeal membrane oxygenation (ECMO) decannulation. This study investigates neonatal ECMO decannulation practices regarding management of the carotid artery and internal jugular vein, use of post-repair anticoagulation, and follow-up imaging. METHODS: A survey was distributed to the 37 institutions in the Children's Hospitals Neonatal Consortium. Respondents reported their standard approach to carotid artery and internal jugular vein management (ligation or repair) at ECMO decannulation by their pediatric surgery and cardiothoracic (CT) surgery teams as well as post-repair anticoagulation practices and follow-up imaging protocols. RESULTS: The response rate was 95%. Pediatric surgeons performed most neonatal respiratory ECMO cannulations (88%) and decannulations (85%), while all neonatal cardiac ECMO cannulations and decannulations were performed by CT surgeons. Pediatric surgeons overwhelmingly ligate both vessels (90%) while CT surgeons typically repair both vessels at decannulation (83%). Of the responding centers that repair, 28% (7) have a standard anticoagulation protocol after neck vessel repair. While 52% (13) of centers routinely image cervical vessel patency at least once post repair, most do not subsequently repeat neck vessel imaging. CONCLUSIONS: Significant practice differences exist between pediatric and CT surgeons regarding the approach to cervical vessels at neonatal ECMO decannulation. For those centers that do repair the vessels there is little uniformity in post-repair anticoagulation or imaging protocols. There is a need to develop standardized cervical vessel management guidelines for neonatal ECMO patients and to study their impact on both short- and long-term outcomes. LEVEL OF EVIDENCE: IV.

Analgesia, Sedation, and Neuromuscular Blockade in Infants with Congenital Diaphragmatic Hernia.

OBJECTIVE: The aim of this study was to describe the use, duration, and intercenter variation of analgesia and sedation in infants with congenital diaphragmatic hernia (CDH). STUDY DESIGN: This is a retrospective analysis of analgesia, sedation, and neuromuscular blockade use in neonates with CDH. Patient data from 2010 to 2016 were abstracted from the Children's Hospitals Neonatal Database and linked to the Pediatric Health Information System. Patients were excluded if they also had non-CDH conditions likely to affect the use of the study medications. RESULTS: A total of 1,063 patients were identified, 81% survived, and 30% were treated with extracorporeal membrane oxygenation (ECMO). Opioid (99.8%), sedative (93.4%), and neuromuscular blockade (87.9%) use was common. Frequency of use was higher and duration was longer among CDH patients treated with ECMO. Unadjusted duration of use varied 5.6-fold for benzodiazepines (median: 14 days) and 7.4-fold for opioids (median: 16 days). Risk-adjusted duration of use varied among centers, and prolonged use of both opioids and benzodiazepines ≥5 days was associated with increased mortality (p < 0.001) and longer length of stay (p < 0.001). Use of sedation or neuromuscular blockade prior to or after surgery was each associated with increased mortality (p ≤ 0.01). CONCLUSION: Opioids, sedatives, and neuromuscular blockade were used commonly in infants with CDH with variable duration across centers. Prolonged combined use ≥5 days is associated with mortality. KEY POINTS: · Use of analgesia and sedation varies across children's hospital NICUs.. · Prolonged opioid and benzodiazepine use is associated with increased mortality.. · Postsurgery sedation and neuromuscular blockade are associated with mortality..

Perinatal Care of Infants with Congenital Birth Defects.

Prenatal diagnosis has changed perinatal medicine dramatically, allowing for additional fetal monitoring, referral and counseling, delivery planning, the option of fetal intervention, and targeted postnatal management. Teams participating in the delivery room care of infants with known anomalies should be knowledgeable about specific needs and expectations but also ready for unexpected complications. A small number of neonates will need rapid access to postnatal interventions, such as surgery, but most can be stabilized with appropriate neonatal care. These targeted perinatal interventions have been shown to improve outcome in selected diagnoses.

Thoracoscopic Repair of Congenital Diaphragmatic Hernia After Extracorporeal Membrane Oxygenation: Feasibility and Outcomes.

INTRODUCTION: Thoracoscopic repair of congenital diaphragmatic hernia (CDH) has been associated with faster recovery, earlier extubation, and decreased morbidity. Nevertheless, thoracoscopic repair is rarely attempted in the post-extracorporeal membrane oxygenation (ECMO) patient. Commonly cited reasons for not attempting thoracoscopy include concerns that the patients' respiratory status is too tenuous to tolerate insufflation pressures or that presumed defect size is so large that it precludes thoracoscopic repair. Our purpose is to review our experience with post-ECMO thoracoscopic CDH repair and evaluate the success of this approach. METHODS: We performed retrospective analysis of attempted thoracoscopic CDH repairs after ECMO decannulation at our institution from 2001 to 2015. Primary outcome was rate of conversion. Secondary outcomes were intraoperative end-tidal CO2, time to extubation, and rate of recurrence. RESULTS: We identified 21 post-ECMO patients in whom thoracoscopic CDH repair was attempted. Thoracoscopic repair was successfully completed in 28%. No patients had reported intolerance to insufflation at 3-7 mmHg. Average end-tidal CO2 at 15 operative minutes was 36.9 mmHg in the thoracoscopic group versus 50.7 mmHg in the open group and at 60 minutes was 34.25 mmHg versus 45.6 mmHg, respectively. One patient in the thoracoscopic group died and 1 experienced a large pneumothorax. In the converted group there was one clinically significant pneumothorax and three pleural effusions. Survivors after thoracoscopy were extubated an average of 5.6 ± 2.6 days after surgery versus 19.4 ± 10 days in the converted group (P < .05). Recurrence rates at last follow-up were equal between the two groups at 20%. CONCLUSIONS: Thoracoscopic CDH repair is both safe and feasible after ECMO with no increase in operative morbidity or mortality. Insufflation pressures of 3-7 mmHg are well tolerated without undue increase in end-tidal CO2. When compared to conversion cases, thoracoscopic repair is associated with significantly decreased time to extubation with no difference in recurrence.

Identical twins with lethal congenital pulmonary airway malformation type 0 (acinar dysplasia): further evidence of familial tendency.

We report a case of identical twins with lethal congenital pulmonary airway malformation (CPAM) type 0. Twin A expired several hours after birth, and twin B was sustained by extra-corporeal membrane oxygenation (ECMO) support; however, care was withdrawn from twin B following the autopsy of twin A, which revealed a diagnosis of CPAM type 0. Both twins showed pulmonary hypoplasia, histologically consistent with CPAM type 0 and pulmonary hypertension. Furthermore, the family also had a previous male who presented with pulmonary hypoplasia and respiratory failure and died shortly after birth; however, no autopsy was performed to confirm a diagnosis of CPAM. Here, in discussing our case, as well as previously reported cases, we demonstrate CPAM type 0's high prevalence among females (9:1 ratio). From the reported cases, it appears that CPAM type 0's tendency to recur in families is up to 40%, suggesting an autosomal recessive inheritance pattern. However, the actual tendency of familial recurrence is hard to assess due to the rarity of the disease and the potential lack of reporting CPAM type 0 cases. To our knowledge, our report represents the first description of CPAM type 0 in identical twins.