Anesthetic Chalenges And Managment In Patient With Ataxia- Telangiectasia.

Louis-Bar Syndrome is a synonym for a very rare complex neurodegenerative disorder ataxia-telangiectasia (A-T). This is an autosomal recessive inherited disease that encompasses abnormalities in the cerebellum, multisystem degeneration, immunodeficiency, increased risk for malignancy and consecutive respiratory insufficiency. Most of the patients are radiosensitive and any exposing to ionization may lead to progression of the disease. Potential risks from anesthesia, mechanical ventilation, and postoperative complications in these patients have been insufficiently discussed in the literature. We present a successful anesthetic and respiratory management with one-lung ventilation in a patient with Louis-Bar Syndrome who underwent video assisted thoracoscopy (VATS) for recurrent pleural effusion.

Clinical Importance of Potential Genetic Determinants Affecting Propofol Pharmacokinetics and Pharmacodynamics.

Interindividual variability in response to drugs used in anesthesia has long been considered the rule, not the exception. It is important to mention that in anesthesiology, the variability in response to drugs is multifactorial, i.e., genetic and environmental factors interact with each other and thus affect the metabolism, efficacy, and side effects of drugs. Propofol (2,6-diisopropylphenol) is the most common intravenous anesthetic used in modern medicine. Individual differences in genetic factors [single nucleotide polymorphisms (SNPs)] in the genes encoding metabolic enzymes, molecular transporters, and molecular binding sites of propofol can be responsible for susceptibility to propofol effects. The objective of this review (through the analysis of published research) was to systematize the influence of gene polymorphisms on the pharmacokinetics and pharmacodynamics of propofol, to explain whether and to what extent the gene profile has an impact on variations observed in the clinical response to propofol, and to estimate the benefit of genotyping in anesthesiology. Despite the fact that there has been a considerable advance in this type of research in recent years, which has been largely limited to one or a group of genes, interindividual differences in propofol pharmacokinetics and pharmacodynamics may be best explained by the contribution of multiple pathways and need to be further investigated.

ERECTOR SPINAE PLANE BLOCK AND PLACEMENT OF PERINEURAL CATHETER FOR DEVELOPMENTAL HIP DISORDER SURGERY IN CHILDREN.

The ultrasound-guided erector spinae plane (ESP) block is a novel interfascial plane block technique providing analgesic effects in different localizations of the body, in accordance with the level of administration. Although ESP block is usually performed in the thoracic region in pediatric patients, it is possible to achieve ESP block in the lumbar region as well. Postoperative pain management is essential in patients undergoing operative hip treatment, one of the most common procedures in pediatric orthopedic surgery. We report on a case of effective intraoperative analgesia achieved by ultrasound-guided lumbar ESP block and another case of effective intra- and postoperative analgesia accomplished with perineural catheter placement in addition to lumbar ESP block, both performed in children surgically treated for developmental hip disorders.

The Effect of UGT1A9, CYP2B6 and CYP2C9 Genes Polymorphism on Propofol Pharmacokinetics in Children.

PURPOSE: This study was conducted to determine the effect of UGT1A9 98T>C, CYP2B6 516G>T and CYP2C9 430C>T genetic polymorphisms on the pharmacokinetics of propofol in children of different sexes and ages who undergone total intravenous anesthesia (ТIVA) and deep sedation during diagnostic and therapeutic procedures. PATIENTS AND METHODS: The prospective study included 94 children, ASA I-II status, 1 to 17 years of age, who undergone standard anesthetic protocol for TIVA, which implied the continuous use of propofol. Before the administration of propofol, venous blood was sampled to determine the presence of genetic variations in UGT1A9, CYP2B6 and CYP2C9 gene using a polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). From each patient included in the study blood samples were taken: 10 mins after the induction of anesthesia, immediately before the discontinuation of the propofol infusion, 10 mins after discontinuation of the propofol infusion and 20 mins after discontinuation of the propofol infusion to determine the pharmacokinetics of the drug in the plasma of the subjects The plasma propofol concentration was determined by HPLC analytical technique. RESULTS: UGT1A9 genotype is an independent predictor of the propofol concentration in children immediately after the end of the continuous infusion and 10 mins afterwards. In the carriers of the polymorphic UGT1A9 C allele, the propofol distribution constant was higher. The carriers of the polymorphic CYP2B6 T allele received a significantly lower overall and initial dose of propofol. Unlike polymorphism of the UGT1A9 gene, the tested CYP2C9 and CYP2B6 gene polymorphisms are not independent predictors of the pharmacokinetics of propofol. CONCLUSION: Further investigations of UGT1A9, CYP2B6 and CYP2C9 and other genes that participate in propofol metabolism as well as detailed analyses of the general conditions, administered therapies and associated diseases could explain the large interindividual variability of propofol metabolism in children.

Topical anesthetics for pediatric laser treatment.

Topical anesthetic agent causes transient insensibility to pain in a limited area of skin, and provides effective anesthesia in a short onset time, short duration, with seldom local or systemic side effects on intact skin and is simple to use. Topical formulations may offer significant benefits for prevention of procedural pain. Currently, they are considered to be the most effective anesthesia for laser treatments. Unfortunately, there is no standard anesthetic technique for this procedure. Lasers are being widely used in numerous dermatological and esthetics treatments in childhood. The advancement of new knowledge in laser technology have contributed to the development of new lasers that are commonly used in a pediatric population, such as Pulsed Dye, Carbon-dioxide and Nd:YAG laser. The most commonly used topical anesthetics in young patients for minimally or moderately painful laser cutaneous procedures are Lidocaine, Prilocaine, Tetracaine gel and combinations thereof.

Diagnosis of Eosinophilic Esophagitis in Children: A Serbian Single-Center Experience from 2010 to 2017.

OBJECTIVES: The aim of this study was to assess the epidemiological, clinical, endoscopic, and pathohistological characteristics of pediatric eosinophilic esophagitis (EoE) in Serbia. METHOD: All children aged 0-18 years diagnosed with EoE in the period between 2010 and 2017 at the University Children's Hospital in Belgrade, Serbia, were retrospectively enrolled. RESULTS: EoE was diagnosed in 35 children (12.45 ± 3.77 years) with a male predominance (74%). The median incidence rate was estimated to be 0.85 per 100,000 children per year with the highest rate estimated at 3.17 per 100,000 children in 2017. Dysphagia (71.4%) and food impaction (40%) were dominant symptoms. Inflammatory endoscopic changes were found in 74.3% and fibrostenotic changes in 62.9% of the children. The esophageal biopsy rate was low (6.8%), especially in children with reflux and nonspecific symptoms. Subepithelial fibrosis was found in only 20% of the patients. Since 2016, the number of biopsy samples has increased, but the sampling rate of lamina propria is still low (<50%). The correlation between the number of biopsies and lamina propria acquisition was strong (rs = 0.773, p < 0.05). In 2 immunocompetent adolescents, EoE was diagnosed after successful treatment of infectious esophagitis. CONCLUSIONS: An increase in the incidence of EoE in Serbian children is evident. The biopsy rate in children with nonspecific and reflux symptoms should be increased, as well as the number of biopsy samples for the detection of subepithelial fibrosis. In immunocompetent children with infectious esophagitis, EoE should be suspected and endoscopy may be recommended after successful treatment of infection.

Evaluation of factors for poor outcome in preterm newborns with posthemorrhagic hydrocephalus associated with late-onset neonatal sepsis.

PURPOSE: Preterm newborns, due to many factors, are at increased risk for poor neural development, intraventricular hemorrhages, infections, and higher rate of mortality. The aim of this study was to evaluate the risk factors associated with poor outcome in preterm neonates with late-onset neonatal sepsis (LONS) who had posthemorrhagic hydrocephalus and underwent neurosurgical procedures for treatment of the hydrocephalus. PATIENTS AND METHODS: Preterm neonates who had undergone insertion of ventriculoperitoneal shunt or Ommaya reservoir, during the 10-year period at University Children's Hospital, were retrospectively analyzed. According to the presence or absence of LONS, patients were divided into LONS group and non-LONS group. In both groups, we analyzed demographic and clinical data as well as nondependent factors. Additionally, we evaluated the patients who had lethal outcome in respect to all the analyzed factors. RESULTS: A total of 74 patients were included in the study, 35 in LONS group and 39 in control group. Patients in LONS group were born significantly earlier with lower birth weight, needed significantly higher O2 inspiratory concentration, and had longer duration of mechanical ventilation when compared to the nonseptic group. Five patients in LONS group had lethal outcome, and for these patients we identified a grade American Society of Anaesthesiologists score of 4 (P=0.000), ductus arteriosus persistens (P=0.000), bronchopulmonary dysplasia (P=0.003), and pneumothorax (P=0.003) as independent preoperative risk factors for lethal outcome. CONCLUSION: Neurosurgical procedures are relatively safe in neonates with posthemorrhagic hydrocephalus without LONS after birth. However, if LONS is present, various conditions such as preoperative high grade American Society of Anaesthesiologists score, ductus arteriosus persistens, bronchopulmonary dysplasia, and pneumothorax markedly increase the risk for a lethal outcome after the operation.

The Safety and Efficacy of the Continuous Peripheral Nerve Block in Postoperative Analgesia of Pediatric Patients.

Postoperative analgesia is imperative in the youngest patients. Pain, especially if experienced during childhood, has numerous adverse effects-from psychological, through complications of the underlying disease (prolonged treatment, hospital stay, and increased costs of the treatment) to an increase in the incidence of death due to the onset of the systemic inflammatory response. Peripheral blocks provide analgesia for 12-16 h, and are safer due to rare side effects that are easier to treat. The continuous peripheral block (CPNB) has been increasingly used in recent years for complete and prolonged analgesia of pediatric patients, as well as a part of multidisciplinary treatment of complex regional pain syndrome. It has been shown that outpatient CPNB reduces the need for parenteral administration of opioid analgetics. It has also been proved that this technique can be used in pediatric patients in home conditions. Safety of CPNB is based on the increasing use of ultrasound as well as on the introduction of single enantiomers local anesthetics (ropivacaine and levobupivacaine) in lower concentrations. It is possible to discharge patient home with catheter, but it is necessary to provide adequate education for staff, patients, and parents, as well as to have dedicated anesthesiology team. Postoperative period without major pain raises the morale of the child, parents. and medical staff.

The comparative analyses of different diagnostic approaches in detection of gastroesophageal reflux disease in children.

OBJECTIVES: The aim of this study was to compare the different diagnostic approaches in detection of gastroesophageal reflux disease in children presented with symptoms suggesting gastroesophageal reflux disease. METHODS: The study design was cross sectional. The study retrospectively included all children who underwent combined multiple intraluminal impedance and pH (pH-MII) monitoring due to gastrointestinal and/or extraesophageal symptoms suggesting gastroesophageal reflux disease at University Children's Hospital in Belgrade, from July 2012 to July 2016. RESULTS: A total of 218 (117 boys/101 girls), mean age 6.7 years (range 0.06-18.0 years), met the inclusion criteria. Gastroesophageal reflux disease was found in 128 of 218 children (57.4%) by pH-MII and in 76 (34.1%) children by pH metry alone. Using pH-MII monitoring as gold standard, sensitivity of pH-metry was lowest in infants (22.9%), with tendency to increase in older age groups (reaching 76.4% in children ≥ 9 years). The sensitivity of pH-metry alone in children with extraesophageal symptoms was 38.1%, while the sensitivity of pH-metry in children with gastrointestinal symptoms was 63.8%. Reflux esophagitis was identified in 31 (26.1%) of 119 children who underwent endoscopy. Logistic regression analysis showed that best predictors of endoscopic reflux esophagitis are the longest acid episode (OR = 1.52, p<0.05) and DeMeester reflux composite score (OR = 3.31, p<0.05). The significant cutoff values included DeMeester reflux composite score ≥ 29 (AUC 0.786, CI 0.695-0.877, p<0.01) and duration of longest acid reflux ≥ 18 minutes (AUC 0.784, CI 0.692-0.875, p<0.01). CONCLUSIONS: The results of our study suggested that compared with pH-metry alone, pH-MII had significantly higher detection rate of gastroesophageal reflux disease, especially in infants. Our findings also showed that pH-MII parameters correlated significantly with the endoscopically confirmed erosive esophagitis.

Comparison of ketamine and ketofol for deep sedation and analgesia in children undergoing laser procedure.

The aim of our study was to research and evaluate cardiovascular and respiratory stability, clinical efficacy, and safety of two different anesthetic agents in pediatric patients who underwent Pulse dye (wavelength 595 nm, pulse duration 0-40 ms, power 0-40 J) and CO2 (wavelength 10,600 nm, intensity-fraxel mod with SX index 4 to 8, power 0-30 W) laser procedure. This prospective non-blinded study included 203 pediatric patients ASA I-II, aged between 1 month and 12 years who underwent short-term procedural sedation and analgesia for the laser procedure. After oral premedication with midazolam, 103 children were analgo-sedated with ketamine and fentanyl (K group) and 100 with ketofol and fentanyl (KT group). Vital signs, applied drug doses, pulse oximetry, and parental satisfaction questionnaire were used to compare these two groups. Statistical differences were tested using Student's t test, Mann-Whitney U test, chi-square test, and Fisher's exact test. Receiver operating characteristic (ROC) curve analysis was used to assess the cut-off value of the duration of anesthesia predicting apnea. Tachycardia was recorded in a significantly higher number of patients who received ketamine as the anesthetic agent (35.9 vs. 3% respectively). Hypertension was also significantly more frequent in patients who received ketamine in comparison with patients who received ketofol (25.2 vs. 3%). Laryngospasm was not observed in both examined groups. There was no statistically significant difference between groups in satisfaction of parents and doctors. Apnea and respiratory depression occurred significantly more frequent in ketofol than in ketamine group (12 vs. 0.97% and 13 vs. 0%). Based on ROC analysis for apnea, we found a significantly higher number of patients with apnea in the ketofol group when duration of anesthesia was longer than 17 min. Our study has shown that ketofol is more comfortable than ketamine in short-term laser procedures in children, causing less hemodynamic alteration with mild respiratory depression and less post-procedural adverse events.

A Comparison of Three Different Volumes of Levobupivacaine for Caudal Block in Children Undergoing Orchidopexy and Inguinal Hernia Repair.

OBJECTIVE: The aim of this study was to compare the efficacy of 3 different volumes of 0.25% levobupivacaine caudally administered on the effect of intra- and postoperative analgesia in children undergoing orchidopexy and inguinal hernia repair. SUBJECTS AND METHODS: Forty children, aged 1-7 years, American Society of Anesthesiologists (ASA) physical status I and II, were randomized into 3 different groups according to the applied volumes of 0.25% levobupivacaine: group 1 (n = 13): 0.6 mL∙kg-1; group 2 (n = 10): 0.8 mL∙kg-1; and group 3 (n = 17): 1.0 mL∙kg-1. The age, weight, duration of anesthesia, onset time of intraoperative analgesic, dosage, and addition of intraoperative fentanyl were compared among the groups. The time to first use of the analgesic and the number of patients who required analgesic 24 h after surgery in the time intervals within 6 h, between 6 and 12 h, and between 12 and 24 h postoperatively were evaluated among the groups. Statistical analyses were performed with a Dunnett t test, ANOVA, or Kruskal-Wallis test and χ2 test. Logistic regression analysis was used in order to examine predictive factors on duration of postoperative analgesia. RESULTS: Age, weight, duration of anesthesia, onset time of intraoperative analgesic, dosage, and addition of intraoperative fentanyl were similar among the groups. The time to first analgesic use did not differ among the groups, and logistic regression modelling showed that using the 3 different volumes of levobupivacaine had no predictive influence on duration of postoperative analgesia. The numbers of patients who required analgesics within 6 h (3/2/3), between 6 and 12 h (3/1/3), and between 12 and 24 h (1/0/2) after surgery were similar among the groups. CONCLUSION: The 3 different volumes of 0.25% levobupivacaine provided the same quality of intra- and postoperative pain relief in pediatric patients undergoing orchidopexy and inguinal hernia repair.

MOEBIUS SYNDROME: CHALLENGES OF AIRWAY MANAGEMENT.

Moebius syndrome is a rare nonprogressive congenital neurological disorder with a wide range of severity and variability of symptoms. This diversity is a consequence of dysfunction of different cranial nerves (most often facial and abducens nerves), accompanying orofacial abnormalities, musculoskeletal malformations, congenital cardiac diseases, as well as specific associations of Moebius and other syndromes. The authors present anesthesia and airway management during the multiple tooth extraction surgery in a 10-year-old girl with Moebius syndrome associated with Poland and trigeminal trophic syndromes.

Management of Anesthesia in a Child with a Large Neck Rhabdoid Tumor.

OBJECTIVE: The aim of this paper was to report the management of anesthesia of a child with a large neck rhabdoid tumor. CLINICAL PRESENTATION AND INTERVENTION: A 9-month- old female patient underwent urgent neck tumor excision due to intratumoral bleeding from a large tumor that compressed and dislocated the trachea; therefore, intubation was expected to be difficult. Sevoflurane inhalation induction was utilized to maintain spontaneous respiration. Oral laryngoscopy revealed Cormack-Lehane grade 3 laryngeal view. The trachea was intubated using a reinforced tube on the third attempt. Fiberoptic bronchoscope-assisted intubation was planned as an alternative in case of conventional intubation failure. Anticipation of massive blood loss necessitated central venous catheterization. CONCLUSION: Establishing a safe airway, intubation during spontaneous breathing and invasive hemodynamic monitoring are crucial factors in the anesthetic management of pediatric patients with a large neck tumor.

Unpredictable drug reaction in a child with Cornelia de Lange syndrome.

CASE DESCRIPTION: Preoperative use of midazolam sedation is mandatory during induction of anesthesia in noncooperative and hyperactive children to prevent possible obstacles. Unusual drug reactions rarely occur in patients undergoing anesthesia or in intensive care unit. This report describes an unpredictable drug reaction after a routine midazolam premedication in a patient with no history of allergy. There has been no literature data yet to show that midazolam can provoke respiratory problems in patients with Cornelia de Lange Syndrome. CONCLUSION: In our opinion midazolam should be avoided in patients with Cornelia de Lange Syndrome, which we enforced after first unpredictable reaction.