Objectives: We sought to evaluate dexmedetomidine efficacy in assuring comfort and sparing conventional drugs when used for prolonged sedation (≥24 hr) in critically ill patients, by using validated clinical scores while systematically collecting drug dosages. We also evaluated the safety profile of dexmedetomidine and the risk factors associated with adverse events. Design: Observational prospective study. Setting: Nine tertiary-care PICUs. Patients: Patients less than 18 years who received dexmedetomidine for greater than or equal to 24 hours between January 2016 and December 2017. Interventions: None. Measurements and Main Results: One-hundred sixty-three patients (median age, 13 mo; interquartile range, 4–71 mo) were enrolled. The main indication for dexmedetomidine use was as an adjuvant for drug-sparing (42%). Twenty-three patients (14%) received dexmedetomidine as monotherapy. Seven percent of patients received a loading dose. The median infusion duration was 108 hours (interquartile range, 60–168 hr), with dosages between 0.4 (interquartile range, 0.3–0.5) and 0.8 µg/kg/hr (interquartile range, 0.6–1.2 µg/kg/hr). At 24 hours of dexmedetomidine infusion, values of COMFORT-B Scale (n = 114), Withdrawal Assessment Tool-1 (n = 43) and Cornell Assessment of Pediatric Delirum (n = 6) were significantly decreased compared with values registered immediately pre dexmedetomidine (p < 0.001, p < 0.001, p = 0.027). Dosages/kg/hr of benzodiazepines, opioids, propofol, and ketamine were also significantly decreased (p < 0.001, p < 0.001, p = 0.001, p = 0.027). The infusion was weaned off in 85% of patients, over a median time of 36 hours (interquartile range, 12–48 hr), and abruptly discontinued in 15% of them. Thirty-seven percent of patients showed hemodynamic changes, and 9% displayed hemodynamic adverse events that required intervention (dose reduction in 79% of cases). A multivariate logistic regression model showed that a loading dose (odds ratio, 4.8; CI, 1.2–18.7) and dosages greater than 1.2 µg/kg/hr (odds ratio, 5.4; CI, 1.9–15.2) increased the odds of hemodynamic changes. Conclusions: Dexmedetomidine used for prolonged sedation assures comfort, spares use of other sedation drugs, and helps to attenuate withdrawal syndrome and delirium symptoms. Adverse events are mainly hemodynamic and are reversible following dose reduction. A loading dose and higher infusion dosages are independent risk factors for hemodynamic adverse events.

Hepatic pulmonary fusion is a rare malformation associated with right congenital diaphragmatic hernia (CDH), often only discovered during surgical repair of the defect. Fourteen previous cases have been reported in the literature. We describe a case of a full term male newborn with prenatal ultrasound diagnosis of right CDH who underwent a thoracoscopy converted to a thoracotomy, due to this rare aforementioned intraoperative incidental finding. We reviewed the previous reported literature, especially focusing on the chosen surgical approach, concluding that an early and appropriate preoperative imaging investigation may be crucial for the best management of these kinds of patients.

This report describes a complication of post-operative oedema of the palate, tongue and pharynx after a Perko-revised cleft palate repair, which resulted in a life-threatening airway obstruction in an infant with Pierre Robin syndrome. Although infants experiencing airway problems after Wardill-Kilner, von Langenbeck and Furlow palatoplasty have been described, airway complications in a group of Perko-revised repair children have not been previously reported. We speculate that this complication, which occurred in the absence of a history of previous airway problems, is due to prolonged operating time and excessive pressure exerted on the base of the tongue by the Kilner-Doughty retractor. Acknowledgments of this risk permits to identify those patients prior to surgery so that they can be managed appropriately.

Prenatal ultrasound (US) diagnosis and postnatal outcome are reviewed in three babies with the complex form of meconium peritonitis (MP), the cystic type. Perinatal management is discussed. Large intra-abdominal cysts with signs of calcifications were detected during the second mid-trimester. Meconium ascites and polyhydramnios appeared between 32 and 35 weeks of gestation. Signs of anaemia were assessed on median cerebral artery peak systolic velocity. Sudden appearance of hydrops and anaemia required preterm delivery, neonatal resuscitation and urgent abdominal drainage. Postnatal US imaging confirmed prenatal sonographic evidence. Abdominal X-ray showed calcifications and no free abdominal air. Intestinal diversion was performed in two patients on their first day of life and evolution was uneventful. Hospital death occurred in one baby, who was submitted to delayed surgery due to unstable hemodynamic conditions. Distal ileal perforation walled off by pseudocysts was detected in all cases. One baby was found to be affected by cystic fibrosis. Ileal intussusception was described in the non-surviving infant. The cystic type of MP may have a potentially rapid lethal course and the onset of foetal anaemia and polyhydramnios is a bad prognostic factor. Severe evolution in hydrops and foetal distress may occur at any moment suggesting the persistence of a leakage or re-rupture of the cysts with new meconium spillage into the abdomen. Prenatal detection of ascites, polyhydramnios and pseudocysts requires a strict follow-up, and timing of delivery has to be planned in a tertiary centre. Postnatal radiological imaging does not offer further information over prenatal imaging and surgical decision should not be influenced by the absence of abdominal free air. Urgent abdominal drainage at birth, followed by intestinal diversion of persistent intestinal perforation on the first day of life, may prevent bacterial colonisation and improve prognosis.