Multiparametric Imaging for Presurgical Planning of Craniopagus Twins: The Experience of Two Tertiary Pediatric Hospitals with Six Sets of Twins.

Conjoined twins are rare and pose a challenge to radiologists and surgeons. Craniopagus twins, where conjunction involves the cranium, are especially rare. Even in large pediatric centers, radiologists are unlikely to encounter more than one such event in their medical careers. This rarity makes it daunting to select a CT and MRI protocol for these infants. Using the experience of two tertiary pediatric hospitals with six sets of craniopagus twins, this multidisciplinary and multimodal integrated imaging approach highlights the key questions that need addressing in the decision-making process for possible surgical intervention.

Early Hybrid Extracorporeal Therapies in Pediatric Acute Liver Failure of Unknown Etiology.

We describe a 9-year-old boy with acute liver failure of unknown etiology, unresponsive to standard medical therapy, with increasing hyperammonemia blood level, lactate elevation, a pediatric end liver stage of 20, a hepatic encephalopathy (HE) score of 2, and scheduled for emergent liver transplantation on the waiting list. We admitted him in the pediatric intensive care unit and managed him in the early stages with continuous renal replacement therapy and therapeutic plasma exchange as soon as neurologic impairment started to worsen. He recovered from his HE after 3 days of blood purification and was removed from the transplantation waiting list due to progressive liver function improvement.

Early Leukapheresis Depletion in an Ex-Premature with Severe Acute Respiratory Distress Syndrome Due to Bordetella Pertussis and Coronavirus Infection.

We describe a 2 weeks corrected gestational age infant admitted in pediatric intensive care unit (PICU) for severe acute respiratory distress syndrome (ARDS) associated to Bordetella pertussis and Coronavirus infection. He developed leukocytosis as soon as ARDS required intubation and aggressive mechanical ventilation: hence he underwent 3 early therapeutic leukapheresis treatments in order to avoid the worsening of related cardiopulmonary complications, according to recent literature on pertussis infection in infants. The infant was discharged from PICU healthy.

Propofol Formulation Affects Myocardial Function in Newborn Infants.

This study aimed to evaluate the effects of propofol in diluted and undiluted formulations on cardiac function in infants. Infants > 30 days received propofol sedation for central line insertion. Cases were divided into two groups: those who received undiluted 1% propofol (P1%); and those who received a diluted formulation (Pd) of equal volumes propofol 1% and 0.9% NaCl. Echocardiograms were performed pre (t0)-, immediately post (t1)-, and 1-h post (t2) propofol administration. Myocardial deformation was assessed with tissue Doppler imaging (TDI) analysis and peak longitudinal strain (LS). 18 cases were included: nine (50%) P1% and nine (50%) Pd. In the P1% group, TDI velocities and LS were significantly reduced at t1 and t2. In the Pd Group, only TDI velocities in the left ventricle were reduced at t1, but not at t2. Dilution of propofol may minimize myocardial dysfunction while maintaining adequate sedation in infants. Further comparative studies are needed to investigate the safety and efficacy of this approach.

Liver transplant in ethylmalonic encephalopathy: a new treatment for an otherwise fatal disease.

Ethylmalonic encephalopathy is a fatal, rapidly progressive mitochondrial disorder caused by ETHE1 mutations, whose peculiar clinical and biochemical features are due to the toxic accumulation of hydrogen sulphide and of its metabolites, including thiosulphate. In mice with ethylmalonic encephalopathy, liver-targeted adeno-associated virus-mediated ETHE1 gene transfer dramatically improved both clinical course and metabolic abnormalities. Reasoning that the same achievement could be accomplished by liver transplantation, we performed living donor-liver transplantation in an infant with ethylmalonic encephalopathy. Unlike the invariably progressive deterioration of the disease, 8 months after liver transplantation, we observed striking neurological improvement with remarkable achievements in psychomotor development, along with dramatic reversion of biochemical abnormalities. These results clearly indicate that liver transplantation is a viable therapeutic option for ETHE1 disease.

The use of nasotracheal Airtraq® laryngoscope with reinforced endotracheal tube: our experience.

The authors describe the use of nasotracheal AirtraQ(®) laryngoscope to perform orotracheal intubation with reinforced endotracheal tube in patients with difficult airways. The choice of nasotracheal AirtraQ(®) instead of orotracheal device resulted from the difficulty that they sometimes found using orotracheal AirtraQ(®) with reinforced endotracheal tube. The nasotracheal AirtraQ(®) may be successfully used to perform orotracheal intubation with styletted reinforced endotracheal tube, specially in case of difficulty in advancing it inside the tube-guiding channel of orotracheal AirtraQ(®).

Comparison of regional vs. systemic analgesia for post-thoracotomy care in infants.

BACKGROUND: In infants, post-thoracotomy analgesia traditionally consists of systemic opiates, while regional techniques have gained more favor in recent years. We compare the two techniques for thoracotomy in infants. METHODS: All consecutive patients below 6 months of age who underwent thoracotomy for congenital pulmonary malformations in the study period were retrospectively divided according to the chosen postoperative analgesia: Group S systemic opiates, Group R continuous regional (epidural or extrapleural paravertebral) block. We studied the following outcomes: need for NICU and mechanical ventilation, pain score, requirement for additional analgesics, heart rate 1 h postsurgery, time to pass first stool and to full feed, complications, and duration of hospitalization. RESULTS: Forty consecutive patients were included, 19 in Group S and 21 in Group R. Median age at surgery was 89 days (40-110) and 90 days (46-117), respectively. Five of 19 patients in Group S vs none in Group R required postoperative intensive care (P = 0.017). Patients in Group R had significantly lower postoperative heart rate (145 [138-150] vs. 160 [152-169] b·min(-1) , P = 0.007), earlier passage of first stools (24 h [12-24] vs. 36 h [24-48] P = 0.004), and earlier time to full feed (36 h [24-48] vs. 84 h [60-120] P = 0.0001) than those in Group S. The only observed complication was one catheter dislocation. CONCLUSION: In infants undergoing thoracotomy, loco-regional analgesia is effective and associated with a reduced intensity of postoperative care and earlier full feeding than systemic analgesia; it should therefore be considered a better option.

Impact of severe sepsis on serum and urinary biomarkers of acute kidney injury in critically ill children: an observational study.

BACKGROUND/AIMS: We hypothesized that sepsis could have an impact on the sensitivity of serum and urinary neutrophil gelatinase-associated lipocalin (NGAL) and cystatin C (CysC) for acute kidney injury (AKI) diagnosis in critically ill children. METHODS: Serum NGAL (sNGAL) and urinary NGAL (uNGAL) and CysC were measured daily in the first 48 h from pediatric intensive care unit admission in 11 consecutive critically ill children with severe sepsis; a single measurement was made in a population of 10 healthy controls undergoing minor ambulatory surgery to exclude possible biases in the laboratory methods. RESULTS: uNGAL, serum CysC (sCysC), and urinary CysC (uCysC) levels were significantly increased in patients with septic AKI compared with septic patients without AKI, while sNGAL levels were not significantly different between septic patients with and without AKI. Median serum creatinine levels did not show significant differences between AKI and non-AKI patients. CONCLUSIONS: uNGAL, sCysC and uCysC were not altered by sepsis and were good predictors of AKI. In a septic state, sNGAL alone did not discriminate patients with AKI from those without AKI.

Performance of the pediatric index of mortality 2 (PIM-2) in cardiac and mixed intensive care units in a tertiary children's referral hospital in Italy.

BACKGROUND: Mortality rate of patients admitted to Intensive Care Units is a widely adopted outcome indicator. Because of large case-mix variability, comparisons of mortality rates must be adjusted for the severity of patient illness at admission. The Pediatric Index of Mortality 2 (PIM-2) has been widely adopted as a tool for adjusting mortality rate by patients' case mix. The objective of this study was to assess the performance of PIM-2 in children admitted to intensive care units after cardiac surgery, other surgery, or for other reasons. METHODS: This was a prospective cohort study, conducted in a 607 inpatient-bed tertiary-care pediatric hospital in Italy, with three pediatric intensive care Units (PICUs) and one cardiac Unit (CICU). In 2009-11, all consecutive admissions to PICUs/CICU of children aged 0-16 years were included in the study. Discrimination and calibration measures were computed to assess PIM-2 performance. Multivariable logistic regression analysis was used to assess the association of patients' main reason for intensive care admission (cardiac-surgical, other-surgical, medical), age, Unit and year with observed mortality, adjusting for PIM-2 score. RESULTS: PIM-2 data collection was completed for 91.2% of total PICUs/CICU patient admissions (2912), and for 94.8% of patients who died in PICUs/CICU (129). Overall observed mortality was 4.4% (95% CI, 3.7-5.2), compared to 6.4% (95% CI, 5.5-7.3) expected mortality. Standardised mortality ratio was 0.7 (95% CI: 0.6-0.8). PIM-2 discrimination was fair (area under the curve, 0.79; 95% CI: 0.75-0.83). Calibration was less satisfactory, mainly because of the over two-fold overprediction of deaths in the highest risk group (114.7 vs 53; p < 0.001), and particularly in cardiac-surgical patients. Multivariable logistic analysis showed that risk of death was significantly reduced in cardiac-surgical patients and in those aged 1 month to 12 years, independently from PIM-2. CONCLUSIONS: The children age distribution and the proportion of cardiac-surgical patients should be taken into account when interpreting SMRs estimated using the PIM-2 prediction model in different Units. A new calibration study of PIM-2 score might be needed, and more appropriate cardiac-focused risk-adjustment models should be developed. The role of age on risk of death needs to be further explored.

Pain prevalence and pain management in children and adolescents in an italian third level pediatric hospital: a cross-sectional study.

BACKGROUND: In 2016, we performed a one-day investigation to analyze the prevalence of pain, pain intensity, and pain therapy in the Departments of Surgery and Onco-Hematology of the Ospedale Pediatrico Bambino Gesù. To improve the knowledge gap highlighted in the previous study, refresher courses and even personalized audits have been carried out during these years. The purpose of this study is to evaluate if, after 5 years, there have been improvements in the management of pain. METHODS: The study was conducted on 25 January 2020. Pain assessment, pain therapies, pain prevalence and intensity in the preceding 24 h and during the recovery period were recorded. Pain outcomes were compared with previous audit results. RESULTS: Out of the 63 children with at least one documented pain assessment (starting from 100 eligible), 35 (55.4%) experienced pain: 32 children (50.7%) experienced moderate /severe pain while 3 patients (4%) felt mild pain. In the preceding 24 h, 20 patients (31.7%) reported moderate/severe pain while 10 (16%) reported moderate or severe pain during the interview. The average value of the Pain Management Index (PMI) was - 1.3 ± 0.9 with a minimum of -3 and a maximum of 0. 28 patients (87%) undergoing analgesic therapy for moderate/severe pain had a PMI of less than 0 (undertreated pain), while 3 patients (13%) scored value of 0 or higher (adequate pain therapy), 4 patients (12.5%) received multimodal analgesia with opioids and 2 patients (6%) opioids alone. Time-based therapy was prescribed to 20 patients (62.5%), intermittent therapy was prescribed to 7 patients (22%) and 5 patients (15.5%) did not receive any therapy. The prevalence of pain was higher during hospitalization and 24 h before the interview, while at the time of the interview, the proportion was the same. In this audit, the daily prescription modality of the therapy had some improvements (time-based: 62.5% vs. 44%; intermittent: 22%vs 25%; no therapy: 15.5% vs. 31%). CONCLUSION: Pain management in hospitalized children constantly requires special daily attention from health professionals aimed at mitigating the components of intractable pain and resolving those of treatable pain. TRIAL REGISTRATION: This study is registered with ClinicalTrials.gov, number (NCT04209764), registered 24 December 2019, https://clinicaltrials.gov/ct2/show/NCT04209764?term=NCT04209764&draw=2&rank=1 .

Unplanned admissions after day-case surgery in an Italian third-level pediatric hospital: a retrospective study.

BACKGROUND: Increasing procedures in day-case surgery can mitigate the costs of health service, without reducing safety and quality standards. The Ospedale Pediatrico Bambino Gesù has adopted an educational program for healthcare personnel and patients' families to increase the number of day-case surgery procedures performed without reducing the level of safety. The unplanned admission rate after day-case surgery can be a quality benchmark for pediatric day-case surgery, and in literature, there are no Italian data. METHODS: We made a retrospective analysis of the hospital database and focused on children requiring unplanned admission to the central venue of the hospital for the night. The audit covered the period from September 2012 to April 2018. RESULTS: We performed general anesthesia for 8826 procedures (urology 33.60%, plastic surgery 30.87%, general surgery 17.44%, dermatology 11.66%, dentistry 3.16%, orthopedics 1.64%, digestive endoscopy 1.63%). Unplanned admission for anesthetic reasons resulted in two cases: one case of syncope and one case of vomit (0.023% rate). No one major complication. CONCLUSIONS: Good quality of patient selection, the safety of the structure, family education, and an efficient organizational model combined with an educational program for anesthesiologists can improve the safety of anesthesia for day-case surgery.

Neutrophil gelatinase-associated lipocalin levels during extracorporeal membrane oxygenation in critically ill children with congenital heart disease: preliminary experience.

OBJECTIVE: To evaluate diagnostic and prognostic significance of neutrophil gelatinase-associated lipocalin during extracorporeal membrane oxygenation in children with congenital heart disease. DESIGN: Prospective observational study. SETTING: Pediatric cardiac intensive care unit. PATIENTS: Ten pediatric patients with congenital heart disease requiring venoarterial extracorporeal membrane oxygenation for postoperative cardiorespiratory support were prospectively enrolled. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Five patients were successfully weaned from extracorporeal membrane oxygenation and survived to intensive care unit discharge; five children died during extracorporeal therapy or within 12 hrs from extracorporeal membrane oxygenation stop. Continuous renal replacement therapy after extracorporeal membrane oxygenation start was indicated for three patients. When patients on continuous venovenous hemofiltration were compared with patients not on continuous venovenous hemofiltration, diuretic administration was lower (1.5 vs. 10 mg/kg/day), extracorporeal membrane oxygenation duration was longer (7 [range, 6-14]) vs. 4 [range, 3-8] days), and survival decreased from 60% (four of seven) to 33% (one of three). Neutrophil gelatinase-associated lipocalin levels were higher at the first extracorporeal membrane oxygenation day in patients who underwent continuous venovenous hemofiltration, 285 (range, 181-513) vs. 130 (range, 81-277) ng/mL, in patients who did not undergo continuous venovenous hemofiltration (p = .045). Neutrophil gelatinase-associated lipocalin levels remained higher among patients on continuous venovenous hemofiltration than others throughout the examined extracorporeal membrane oxygenation period (p = .0007), whereas creatinine levels tended to be equivalent between the two groups (p = .38). However, a trend toward increasing neutrophil gelatinase-associated lipocalin levels was noticed also in patients not on continuous venovenous hemofiltration. CONCLUSIONS: Neutrophil gelatinase-associated lipocalin levels at the first extracorporeal membrane oxygenation day may have predictive value for continuous venovenous hemofiltration. During the course of extracorporeal membrane oxygenation support, creatinine levels were normalized in patients with acute kidney injury undergoing continuous venovenous hemofiltration; in these patients, neutrophil gelatinase-associated lipocalin levels may provide further information on kidney injury.

Paraplegia after thoracotomy: a single center experience with pediatric patients and a review of the literature.

AIM: Paraplegia is an infrequent although fearsome complication of anesthesia and surgical procedures, such as epidural anesthesia and thoracotomy. It may occur in both adults and children and a medullary lesion may be confirmed by magnetic resonance imaging, rather than computed tomography. The aim of this study is to describe the experience of two pediatric tertiary centers, contextualizing it with the other cases reported in literature. MATERIAL AND METHODS: We reported three pediatric cases of post-operative paraplegia in oncological patients, focusing on the potential causes and underling the possible strategies to prevent this complication. RESULTS: From our study, two principal features emerged: 1) Epidural anesthesia may expose children to a greater risk of spinal cord permanent damage due to the execution of the procedure under general anesthesia, which deprives the anesthesiologist of an important feedback about the position of the device; 2) In thoracotomy, the risk of paraplegia tends to increase along with the proximity to the costo-vertebral angle, especially if electrocautery or hemostatic materials are used. CONCLUSIONS: A prompt post-surgical neurological status routine assessment in pediatric patients undergoing epidural anesthesia or thoracotomy should be mandatory and, associated with the correct imaging study, may lead to the most appropriate therapeutic pathway and to a better prognosis. KEY WORDS: Anesthesia, Children, Epidural, Thoracotomy, Paraplegia.

Case report of anesthesia for free fibula flap reconstruction in Ewing sarcoma: Safety and efficacy of continuous popliteal sciatic nerve block and very low doses of intravenous heparin.

A child with a maxillary Ewing sarcoma was operated for tumor asportation and reconstruction with free fibula flap. Adequate anticoagulation was achieved with lower doses of heparin and monitored with multiple ACT values. We used NIRS monitoring to avoid hypoperfusion. Post-operative pain relief was guarantited by local anestethic continous infusion.

Therapeutic Drug Monitoring of Amphotericin-B in Plasma and Peritoneal Fluid of Pediatric Patients after Liver Transplantation: A Case Series.

Fungal infections represent a serious complication during the post-liver transplantation period. Abdominal infections can occur following pre-existing colonization, surgical procedures, and permanence of abdominal tubes. In our center, liposomal amphotericin-B is used as antifungal prophylaxis in pediatric patients undergoing liver transplantation. The aim of this study is to evaluate peritoneal levels of amphotericin-B following intravenous administration. Six liver recipients received liposomal amphotericin-B. Three of them were treated as prophylaxis; meanwhile, three patients received liposomal amphotericin-B to treat Candida albicans infection. Plasma and peritoneal amphotericin-B levels were measured by LC-MS/MS in two consecutive samplings. Cmin (pre-dose) and Cmax (2 h after the end of infusion) were evaluated as drug exposure parameters for both plasma and peritoneum. Our results showed that peritoneal amphotericin-B levels were significantly lower than plasma and that the correlation coefficient was 0.72 (p = 0.03) between plasma and peritoneal Cmin. Moreover, although peritoneal levels were within the therapeutic range, they never reached the PK/PD target (Cmax/MIC > 4.5). In conclusion, PK exposure parameters could be differently used to analyze amphotericin-B concentrations in plasma and peritoneum. However, liposomal amphotericin-B should be preferred in these patients as prophylactic rather than therapeutic treatment for fungal infections.