Successful on-ECLS Repair of CDH and Omphalocele in a Newborn.

Both congenital diaphragmatic hernias (CDHs) and omphaloceles show relevant overall mortality rates as individual findings. The combination of the two has been described only sparsely in the literature and almost always with a fatal course. Here, we describe a term neonate with a rare high-risk constellation of left-sided CDH and a large omphalocele who was successfully treated on extracorporeal life support (ECLS). Prenatally, the patient was diagnosed with a large omphalocele and a left CDH with a lung volume of ∼27% and an observed to expected lung-to-head ratio of 30%. Due to respiratory insufficiency, an ECLS device was implanted. As weaning from ECLS was not foreseeable, the female infant underwent successful surgery on ECLS on the ninth day of life. Perioperative high-frequency oscillatory ventilation and circulatory and coagulation management under point-of-care monitoring were the main anesthesiological challenges. Over the following 3 days, ECLS weaning was successful, and the patient was extubated after another 43 days. Surgical treatment on ECLS can expand the spectrum of therapy in high-risk constellations if potential risks are minimized and there is close interdisciplinary cooperation.

Whole liver transplantation in children under 10 kg: How to minimize the high risks of a still challenging procedure.

BACKGROUND: Whole liver transplantation in infants <10 kg is a rare procedure with moderate outcomes (67%-79% graft survival at 1 year) and high rates of vascular complications (hepatic artery thrombosis 5-26%). METHODS: Retrospective single-center analysis of whole liver transplantation in infants <10 kg and systematic review of the literature focused on survival rates and surgical complications. RESULTS: Between January 2005 and December 2020, 175 liver transplantations in 173 children were performed at our center. A total of 92 (53%) children weighed less than 10 kg; 19 (21%) of them underwent WLT and constitute the study population. Median age of the recipients was 10 months (21 days-24 months) and median body weight 6.5 (3.1-9.8) kg. Median age of the donors was 5 (1-84) months and median body weight 6.1 (4-21) kg. Median donor-to-recipient body weight ratio was 1.2 (range: 0.6-4.5). Postoperatively, neither hepatic artery nor portal vein thrombosis occurred. A biliary complication occurred in 4 cases: 1 bile leak (early), 3 anastomotic stenoses (1 delayed and 2 late), and 1 non-anastomotic stenosis (late). Patient survival rate at 1, 5, and 10 years was 100%, 92%, and 92%, respectively. Overall, death-censored graft survival after 1, 5, and 10 was 100%. CONCLUSION: Our results are excellent in terms of complications and graft and patient survival. This involves not only high-end surgical performance but also close interdisciplinary perioperative cooperation based on strong standard operating procedures and mainly focused on fluid management, hemostasiology, and strict monitoring of vessel patency.

Impaired Autoregulation Following Resuscitation Correlates with Outcome in Pediatric Patients: A Pilot Study.

In children with a traumatic brain injury, the duration of autoregulation impairment correlates with the neurological outcome. This pilot study explored whether a similar relation exists in nontraumatic hypoxic-ischemic brain injury following resuscitation.We investigated 11 children after resuscitation. Blood pressure and intracranial pressure (ICP) were monitored with ICM+ software and actively managed to maintain optimal cerebral perfusion pressure (CPP), using the pressure reactivity index (PRx). Outcomes were scored according to the Glasgow Outcome Scale.Three children died within 24 h. Three survivors had an unfavorable outcome and five had a favorable outcome. In the first 72 h, ICP and CPP values did not differ between, or predict, children with favorable or unfavorable outcomes. The duration of a PRx value ≥0.2 was significantly greater in children with an unfavorable outcome. A PRx value ≤0 was associated with a favorable outcome in all except one child. Children with an unfavorable outcome had areas of ischemic brain tissue on magnetic resonance imaging.The duration of poor autoregulation within the first 72 h is associated with an unfavorable outcome. Prognostic signs for insult severity are initially poor autoregulation plus inability to restore autoregulation despite active attempts to do so. Limited ischemia, especially in the basal ganglia, cannot be detected by ICP-based monitoring of autoregulation and may still result in an unfavorable outcome despite good global autoregulation.

Pre- and postnatal findings in a patient with a recombinant chromosome rec(8)(qter→q21.11::p23.3→qter) due to a paternal pericentric inversion inv(8)(p23.3q21.11) and review of the literature.

Recombinant chromosome 8 (Rec8) syndrome (San Luis Valley [SLV] syndrome; OMIM #179613) is a rare chromosome disorder associated with intellectual disability, congenital heart defects, variable skeletal and urogenital anomalies, and dysmorphic features. It is characterized by a partial terminal deletion of 8p and a partial terminal duplication of 8q, which is usually due to meiotic recombination of a pericentric inversion of chromosome 8 of a healthy carrier parent. There are only few reports of cases with breakpoints defined at the molecular level by molecular karyotyping. We report on a case of Rec8 syndrome with previously unreported breakpoints in a male fetus with intrauterine growth restriction, hypogenesis of the corpus callosum, bilateral cleft lip/palate, and congenital heart defect. Cytogenetic analysis revealed a recombinant chromosome 8 [46,XY,rec(8)(qter→q21.11::p23.3→qter)] secondary to a paternal pericentric inversion [46,XY,inv(8)(p23.3q21.11)]. Molecular karyotyping correspondingly showed a terminal copy number loss of 1.4 Mb (arr[hg19] 8p23.3(158048_1514749)×1) and a terminal copy number gain of chromosome band 8q21.11q24.3 of 69.8 Mb (arr[hg19] 8q21.11q24.3(76477367_146295771)×3). To our knowledge, this is the fourth reported case diagnosed prenatally. We describe the postnatal clinical course of the male newborn. Furthermore, we review and compare the phenotypic features and breakpoints of 74 reported Rec8/SLV cases.

Cerebral Oxygen Metabolism Before and After RBC Transfusion in Infants Following Major Surgical Procedures.

OBJECTIVE: Although infants following major surgery frequently require RBC transfusions, there is still controversy concerning the best definition for requirement of transfusion in the individual patient. The aim of this study was to determine the impact of RBC transfusion on cerebral oxygen metabolism in noncardiac and cardiac postsurgical infants. DESIGN: Prospective observational cohort study. SETTING: Pediatric critical care unit of a tertiary referral center. PATIENTS: Fifty-eight infants (15 after pediatric surgery and 43 after cardiac surgery) with anemia requiring RBC transfusion were included. INTERVENTIONS: RBC transfusion. MEASUREMENTS AND MAIN RESULTS: We measured noninvasively regional cerebral oxygen saturation and microperfusion (relative cerebral blood flow) using tissue spectrometry and laser Doppler flowmetry before and after RBC transfusion. Cerebral fractional tissue oxygen extraction and approximated cerebral metabolic rate of oxygen were calculated. Fifty-eight RBC transfusions in 58 patients were monitored (15 after general surgery, 24 after cardiac surgery resulting in acyanotic biventricular physiology and 19 in functionally univentricular hearts including hypoplastic left heart following neonatal palliation). The posttransfusion hemoglobin concentrations increased significantly (9.7 g/dL vs 12.8 g/dL; 9.7 g/dL vs 13.8 g/dL; 13.1 g/dL vs 15.6 g/dL; p < 0.001, respectively). Posttransfusion cerebral oxygen saturation was significantly higher than pretransfusion (61% [51-78] vs 72% [59-89]; p < 0.001; 58% [35-77] vs 71% [57-88]; p < 0.001; 51% [37-61] vs 58% [42-73]; p = 0.007). Cerebral fractional tissue oxygen extraction decreased posttransfusion significantly 0.37 (0.16-0.47) and 0.27 (0.07-039), p = 0.002; 0.40 (0.2-0.62) vs 0.26 (0.11-0.57), p = 0.001; 0.42 (0.23-0.52) vs 0.32 (0.1-0.42), p = 0.017. Cerebral blood flow and approximated cerebral metabolic rate of oxygen showed no significant change during the observation period. The increase in cerebral oxygen saturation and the decrease in cerebral fractional tissue oxygen extraction were most pronounced in patients after cardiac surgery with a pretransfusion cerebral fractional tissue oxygen extraction greater than or equal to 0.4. CONCLUSION: Following RBC transfusion, cerebral oxygen saturation increases and cerebral fractional tissue oxygen extraction decreases. The data suggest that cerebral oxygenation in postoperative infants with cerebral fractional tissue oxygen extraction greater than or equal to 0.4 may be at risk in instable hemodynamic or respiratory situations.

Nurse-driven analgesia and sedation in pediatric patients with univentricular hearts requiring extracorporeal life support after first-stage palliation surgery: A pilot study.

BACKGROUND: Few data are available regarding requirements of sedation and analgesia in children during extracorporeal life support. AIMS: The aim of this study was to evaluate if children with functionally univentricular hearts on extracorporeal life support after first-stage palliation surgery have higher requirement of analgesics and sedatives compared with children without extracorporeal life support using a goal-directed nurse-driven analgesia and sedation protocol. METHODS: This prospective observational matched case-control pilot study was conducted at a cardiac pediatric intensive care unit of a tertiary referral center. Seventeen patients with functionally univentricular hearts including hypoplastic left heart syndrome who were on extracorporeal life support after first-stage palliation surgery were enrolled from July 2012 to January 2017. Seventeen matched patients served as controls. Doses of morphine, midazolam, clonidine, and muscle relaxants as well as sedation scores (COMFORT behavior scale and the nurse interpretation of sedation scale) were assessed according to a nurse-driven protocol every 8 hours up to 120 hours after first-stage palliation surgery. RESULTS: Sedation scores were equal in the extracorporeal life support group and in the control group at most points in time. There was no significant difference in cumulative doses of morphine and midazolam. However, children of the extracorporeal life support group received higher doses of midazolam and morphine at some points in time. CONCLUSION: A nurse-driven protocol for analgesia and sedation of children with extracorporeal life support is feasible. Patients with extracorporeal life support do not need deeper sedation levels and have not higher cumulative sedation requirements than children without extracorporeal life support.

Ketamine Infusion as a Counter Measure for Opioid Tolerance in Mechanically Ventilated Children: A Pilot Study.

BACKGROUND: Drug rotation to prevent opioid tolerance is well recognized in chronic pain management. However, ketamine infusion as a counter measure for opioid tolerance is rarely described in mechanically ventilated children developing tolerance from prolonged opioid infusion. PATIENTS AND METHODS: We performed a retrospective study in a 14-bed medical-surgical-cardiac pediatric intensive care unit. Thirty-two mechanically ventilated children who had developed tolerance from prolonged intravenous infusion of opioids received a continuous intravenous infusion of ketamine as an opioid substitute for more than 2 days, scheduled in a drug rotation protocol. RESULTS: Thirty-two children (median age 2.5 years, range 0.1-16.0; weight 11.2 kg [3.8-62.0]) were included. Patients had received continuous intravenous infusion of opioids and benzodiazepines for 16.0 days (4.0-34.0) when drug rotation was started. The median dose of continuous intravenous infusion of ketamine was 4.0 mg·kg-1·h-1 (1.8-6.0) and the median duration was 3.0 days (2.0-6.0). After having restarted opioids, fentanyl doses were significantly lower compared with the time before the drug rotation began (after, 2.9 µg·kg-1·h-1 [0.8-4.9] vs before, 4.15 µg·kg-1·h-1 [1.2-10.0]; p < 0.001). Continuous intravenous infusion of midazolam and clonidine were unchanged during drug rotation. COMFORT-B scoring was significantly lower after having started drug rotation (after, 14.5 [8-19] vs before, 16 [11-22]; p < 0.001). CONCLUSION: Drug rotation with ketamine in mechanically ventilated children with opioid tolerance is feasible and seems to reduce the rate of fentanyl infusion.

Analgesia and Sedation Protocol for Mechanically Ventilated Postsurgical Children Reduces Benzodiazepines and Withdrawal Symptoms-But Not in All Patients.

Background We demonstrated recently that the implementation of a nurse-driven analgesia and sedation protocol (pediatric analgesia and sedation protocol [pASP]) for mechanically ventilated nonpostsurgical patients reduces the total dose of benzodiazepines and the withdrawal symptoms significantly. It has not been investigated if these results can also be expected in the group of patients undergoing surgery. Objectives To evaluate the effects of the pASP in mechanically ventilated postsurgical children regarding drug dosage, duration of mechanical ventilation, length of stay, and rate of withdrawal symptoms. Methods This is a two-phase prospective observational control study. The preimplementation group was managed by the physician's order and the postimplementation group was managed with the pASP including COMFORT-B, nurse interpretation of sedation, and Sophia observation withdrawal symptoms scale scoring. Measurements and Main Results One hundred and sixteen patients were included before and one hundred and ten patients after implementation. The pASP had no effect on length of pediatric intensive care unit stay or duration of mechanical ventilation. The protocol reduced total (5.0 mg/kg [0.5-58.0] vs 4.0 mg/kg [0.0-47.0]; p = 0.021) and daily doses (4.4 mg/kg/d [1.1-33.9] vs 2.9 mg/kg/d [0.0-9.9]; p < 0.001) of benzodiazepines significantly. No difference was observed in total and daily doses of opioids (5.0 mg/kg [0.1-67.0] vs 3.0 mg/kg [0.1-71.0]; p = 0.81) and (0.7 mg/kg/d [0.0-7.0] vs. 0.8 mg/kg/d [0.0-3.7]; p = 0.35), respectively. Rate of withdrawal symptoms was significantly lower after implementation (35.3 vs 20.0%; p = 0.01), but not in patients after solid organ transplantation or oncological patients. Conclusion The nurse-driven analgesia and sedation protocol is an effective procedure to reduce the total doses of benzodiazepines and occurrence of withdrawal symptoms in postsurgical children, which are naïve to opioids or benzodiazepines.

State of Cerebrovascular Autoregulation Correlates with Outcome in Severe Infant/Pediatric Traumatic Brain Injury.

OBJECTIVE: It could be shown in adults with severe traumatic brain injury (TBI) that the functional status of cerebrovascular autoregulation (AR), determined by the pressure reactivity index (PRx), correlates with and even predicts outcome. We investigated PRx and its correlation with outcome in infant and pediatric TBI. Methods Ten patients (median age 2.8 years, range 1 day to 14 years) with severe TBI (Glasgow Coma Scale score <9 at presentation) underwent long-term computerized intracranial pressure (ICP) and mean arterial pressure (MAP) monitoring using dedicated software for continuous determination of cerebral perfusion pressure (CPP) and PRx. Outcome was determined at discharge and at follow-up at 6 months using the Glasgow Outcome Scale (GOS) score. RESULTS: Median monitoring time was 182 h (range 22-355 h). Seven patients underwent decompressive craniectomy to control ICP during treatment in the intensive care unit. Favorable outcome (GOS 4 and 5) was reached in 4 patients, an unfavorable outcome (GOS 1-3) in 6 patients. When dichotomized to outcome, no correlation was found with ICP and CPP, but median PRx correlated well with outcome (r = -0.79, p = 0.006) and tended to be lower for GOS 4 and 5 (-0.04) than for GOS 1-3 (0.32; p = 0.067). CONCLUSION: The integrity of AR seems to play the same fundamental role after TBI in the pediatric population as in adults and should be determined routinely. It carries an important prognostic value. PRx seems to be an ideal candidate parameter to guide treatment in the sense of optimizing CPP, aiming at improvement of cerebrovascular autoregulation (CPPopt concept).

Nurse-driven pediatric analgesia and sedation protocol reduces withdrawal symptoms in critically ill medical pediatric patients.

BACKGROUND: While several analgesia and sedation guidelines and protocols have been developed and implemented for adults, there is still little evidence of clinical use of analgesia and sedation protocols and the impact on withdrawal symptoms in critically ill children. OBJECTIVE: The aim of this study was to evaluate the effects of a nurse-driven goal-directed analgesia and sedation protocol for mechanically ventilated pediatric patients (pASP) on duration of mechanical ventilation, pediatric intensive care unit (PICU) length of stay, total doses of opioids and benzodiazepines, and occurrence of withdrawal symptoms. PATIENTS AND METHODS: This is a before and after protocol implementation study in a 14-bed medical-surgical-cardiac pediatric intensive care unit at a university children's hospital. A total of 337 medical pediatric patients requiring mechanical ventilation with PICU length of stay for at least 24 h were included. Prior to implementation of the protocol, analgesia and sedation was managed by the attending physician's order. Afterwards, postimplementation, nurses managed analgesia and sedation following a pASP, including COMFORT 'behavioral' Scale, Nurse Interpretation Sedation Scale, and Sophia Observation Withdrawal Symptoms Scale. RESULTS: One hundred and sixty-five patients were included in the 15-month period before and 172 patients were included in the 15-month period after implementation of the pASP. Median duration of mechanical ventilation was 2.02 (0.96-25.0) days in the group preceding protocol implementation and 1.71 (0.96-66.0) days afterwards (P = 0.23). Median PICU length of stay was 5.8 (1-37.75) days in the preimplementation and 5.0 (1-120) days in the postimplementation group (P = 0.14). Total doses of opioids and benzodiazepines were 3.9 mg·kg(-1) ·day(-1) (0.1-70) vs 3.1 mg·kg(-1) ·day(-1) (0.05-56); P = 0.38 and 5.9 mg·kg(-1) ·day(-1) (0-82.0) vs 4.2 mg·kg(-1) ·day(-1) (0-66); P = 0.009 after implementation. Incidence of withdrawal was significantly lower over the postimplementation period (12.8% vs 23.6%; P = 0.005). CONCLUSION: Implementation of a nurse-driven pASP reduced the total dose of benzodiazepines and the occurrence of withdrawal symptoms significantly.