Sofia Apostolidou

Analgesie und Sedierung sind Grundpfeiler sowohl der prähospitalen als auch der innerklinischen Notfallversorgung. Akuter Schmerz ist eines der häufigsten Symptome von Kindern, die in der Notaufnahme vorgestellt werden. Die Sorge vor Komplikationen führt häufig zur Zurückhaltung in der medikamentösen Versorgung von kindlichem Schmerz, was erhebliche Langzeitfolgen haben kann [1]. Multimodale Strategien haben sich in der Behandlung von akutem Schmerz und zur Anxiolyse bewährt. Ein systematisches Vorgehen ist obligat [2]. Article published online: 22 May 2025 © 2025. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

Background Bilateral diaphragmatic agenesis (DA) is a rare congenital defect leading to a bilateral herniation of abdominal organs into the thorax cavity [1]. Diaphragmatic defects are often associated with congenital anomalies and genetic syndromes. However, the occurrence of DA in association with Cornelia de Lange (CDL) Syndrome is very rare [2]. Aim To discuss the current diagnosis and treatment options of DA associated with CDL Syndrome. Methods This report describes the case of a male, late preterm neonate with prenatally diagnosed DA and CDL Syndrome. Results The infant presented with severe respiratory distress after birth, bilateral pulmonary hypoplasia, and herniation of abdominal organs into the chest cavity. The postnatal management was performed according to the recommendations of the Congenital Diaphragmatic Hernia EURO Consortium, including immediate intubation and invasive mechanical ventilation [3]. Chest auscultation revealed a decreased air entry, only reaching the upper lungs. Physical examination confirmed congenital malformations associated with CDL Syndrome, including microcephaly, facial dysmorphia with micro- and retrognathia, long philtrum, long eyelashes, and cleft palate. In addition, the x-ray showed bilateral diaphragm agenesis with herniation of the liver, spleen, and stomach into the thorax resulting in pronounced bilateral pulmonary hypoplasia. Despite inhaled nitric oxide, the application of intensive high-frequency oscillatory ventilation oxygenation gradually deteriorated. Due to the unfavorable prognosis, an interdisciplinary consensus was reached to forego extracorporeal membrane oxygenation (ECMO). The boy died at four hours of age. The parents declined an autopsy, photographic documentation, and postmortem computer tomography. Conclusion DA remains a life-threatening disease despite early prenatal MRI diagnosis, comprehensive treatment of respiratory insufficiency, including intubation, lung-protective ventilation, extensive supportive therapy, and ECMO use [4]. Article published online: 06 June 2023 © 2023. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

The number of pediatric procedural sedations for diagnostic and minor therapeutic procedures performed outside the operating room has increased. Therefore, we established a specialized interdisciplinary team of pediatric anesthesiologists and intensivists (Children’s Analgosedation Team, CAST) at our tertiary-care university hospital and retrospectively analyzed the first year after implementation of the CAST. Within one year, 784 procedural sedations were performed by the CAST; 12.2% of the patients were infants <1 year, 41.9% of the patients were classified as American Society of Anesthesiologists (ASA) grade III or IV. Most children received propofol (79%) and, for painful procedures, additional esketamine (48%). Adverse events occurred in 51 patients (6.5%), with a lack of professional experience (OR 0.60; 95% CI 0.42–0.81) and increased propofol dosage (OR 1.33; 95% CI 1.17–1.55) being significant predictors. Overall, the CAST enabled safe and effective procedural sedation in children outside the operating room.

Critical Coronavirus disease 2019 (COVID-19) developed in a 7-year-old girl with a history of dystrophy, microcephaly, and central hypothyroidism. Starting with gastrointestinal symptoms, the patient developed severe myocarditis followed by progressive multiple organ failure complicated by Pseudomonas aeruginosa bloodstream infection. Intensive care treatment consisting of invasive ventilation, drainage of pleural effusion, and high catecholamine therapy could not prevent the progression of heart failure, leading to the implantation of venoarterial extracorporeal life support (VA-ECLS) and additional left ventricle support catheter (Impella^® pump). Continuous venovenous hemofiltration (CVVH) and extracorporeal hemadsorption therapy (CytoSorb^®) were initiated. Whole exome sequencing revealed a mutation of unknown significance in DExH-BOX helicase 30 (DHX30), a gene encoding a RNA helicase. COVID-19 specific antiviral and immunomodulatory treatment did not lead to viral clearance or control of hyperinflammation resulting in the patient’s death on extracorporeal life support-(ECLS)-day 20. This fatal case illustrates the potential severity of pediatric COVID-19 and suggests further evaluation of antiviral treatment strategies and vaccination programs for children.

Demands in procedural sedation and analgesia in children are growing as the number of diagnostic and minor therapeutic procedures performed on paediatric patients outside the operating room setting has increased. We established a specialized interdisciplinary team of paediatric anaesthesiologists and paediatric intensivists (Children’s Analgosedation Team, CAST) for diagnostic and therapeutic procedures and aimed to analyse the incidence and risk factors of adverse events. A retrospective analysis of data collected in the first year after implementation of the interdisciplinary CAST at our tertiary care university hospital was conducted. Within one year, 784 procedural sedations were performed by the CAST. 7.4% of the patients were infants < 1 year of age. 53% of the patients were classified as American Society of Anesthesiologists (ASA) status III or IV. Most children received propofol (79%) and for painful procedures, additional esketamine (48%). Adverse events occurred in 51 patients (6.5%), most frequently apnoea (1.7%), airway obstruction (1%), and problems with the intravenous access (1%). Cancellation of the procedure occurred in four cases (0.5%). Lack of experience (OR 0.60; 95% CI 0.42-0.81) and increasing propofol dosage of 1mg kg^-1 (OR 1.33; 95% CI 1.17-1.55) were identified as predictors for adverse events. ASA classification did not reveal a significant difference in complication rates. Conclusion: Increasing dosage of propofol and lack of experience were associated with adverse events in paediatric analgosedation for brief diagnostic or therapeutic procedures. Trial registration number: NCT04760249 (retrospectively registered on February 7th, 2021)

Reifes männliches Neugeborenes, Gestationsalter 39+0 Schwangerschaftswochen (SSW), Geburtsgewicht 4200 g, Nabelarterien-pH 7,31, APGAR 9/10/10. Geburt per primärer Sectio bei bekannter fetaler Tachykardie. Die Herzfrequenz lag postnatal bei 220/min; die „probatorische“ Gabe von Adenosin führte nicht zur Konversion in regulären Sinusrhythmus. Die pränatale Verdachtsdiagnose Vorhofflattern ließ sich im 12-Kanal-EKG bestätigen: Die Kammerfrequenz betrug (unter postnataler Fortsetzung der intrauterin begonnenen Flecainid-Therapie) 160/min, die Vorhoffrequenz 320/min mit überwiegender 2:1-Überleitung ([Abb. 1a]). Durch Adenosin kam es zur Demaskierung des Vorhofflatterns mit typischem Sägezahnmuster ([Abb. 1b]). Nach Ausschluss eines Herzvitiums und intraatrialer Thromben erfolgte bei persistierender Tachykardie die elektrische Kardioversion unter Analgosedierung. Ein erster Versuch mit 1 J/kg (4 J) zeigte keinen Effekt; die Wiederholung mit 1,5 J/kg (6 J) führte zur Konversion in stabilen Sinusrhythmus ([Abb. 1c]). Im Verlauf zeigte sich eine supraventrikuläre Extrasystolie, weshalb eine antiarrhythmische Therapie mit Propafenon 3×70 mg/m² angeschlossen wurde. Bereits mit 35+1 SSW war bei der 40-jährigen Erstgravida mit leerer Familien- und Eigenanamnese und zuvor unauffälligem Schwangerschaftsverlauf ein schwer ableitbares Cardiotokogramm (CTG) aufgrund einer fetalen Tachykardie aufgefallen. Nach stationärer Aufnahme zeigte sich in der Sonographie ein zeitgerecht entwickelter Fet mit unauffälliger Herzanatomie und Verdacht auf supraventrikuläre Tachykardie. Die fetale Herzfrequenz lag um 210/min. Dem M-Mode war ein Vorhofflattern mit einer atrialen Frequenz um 440/min und überwiegender 2:1-Überleitung zu entnehmen ([Abb. 2]). Nach kardiologischer Abklärung der Schwangeren wurde eine antiarrhythmische Therapie mit Flecainid (2×100 mg, nach zwei Tagen auf 3×100 mg gesteigert) initiiert. Ab 36+0 SSW war erstmalig ein CTG mit einer Baseline von 178/min ableitbar, im weiteren Verlauf stellte sich eine Kammerfrequenz um 159/min ein. Darunter zeigte sich ein unauffälliges fetales Wachstum ohne Hydropszeichen.