ABSTRACT
La pentalogía de Cantrell es una rara malformación congénita caracterizada por un amplio defecto de la región inferior del esternón, el diafragma y la región anterior abdominal. Se caracteriza por la migración de órganos y su pronóstico depende de la presencia de malformaciones cardiacas. Presentamos el caso de un varón de 18 años sometido a corrección programada de un onfalocele gigante. Se empleó monitorización invasiva y del gasto cardiaco para evitar (como había sucedido en dos intervenciones previas) episodios de inestabilidad por aumento de la presión intraabdominal y disminución del retorno venoso. El pronóstico depende de múltiples factores; lo más importante es el buen control hemodinámico y respiratorio. A pesar de la monitorización avanzada, la mortalidad es muy alta(AU)
Pentalogy of Cantrell is a rare congenital malformation characterised by a large defect in the lower region of the sternum, diaphragm, and lower abdominal region. It is also characterised by the migration of organs, and its prognosis depends on the presence of cardiac malformations. We present the case of an 18 year-old male subjected to a scheduled giant omphalocele correction. Invasive monitoring, including cardiac output, was used to avoid episodes of instability due to the increase in abdominal pressure and the increase in venous return (as had happened on two previous operations). The prognosis depends on multiple factors, with good haemodynamic and respiratory control being the most important. Mortality is high despite the advances in monitoring(AU)
Subject(s)
Humans , Male , Young Adult , Pentalogy of Cantrell/drug therapy , Thoracic Surgery , Cardiovascular Surgical Procedures/methods , Cardiovascular Surgical Procedures/trends , Hernia, Umbilical/complications , Hernia, Umbilical/physiopathology , Hernia, Umbilical/surgery , Pentalogy of Cantrell/physiopathology , Pentalogy of Cantrell/therapy , Pentalogy of Cantrell , PrognosisSubject(s)
Obstetric Labor Complications , Umbilical Cord/pathology , Adult , Female , Humans , PregnancyABSTRACT
No dipsonible
Subject(s)
Humans , Female , Pregnancy , Infant, Newborn , Adult , Umbilical Cord/physiology , Delivery, Obstetric , Bradycardia/complications , Bradycardia/diagnosis , Cesarean Section/methods , Twin Studies as Topic/methods , Cardiopulmonary Resuscitation/methods , Cardiopulmonary Resuscitation/trends , Umbilical Cord/physiopathology , Umbilical Cord , Natural Childbirth , Labor, Obstetric/physiology , Cardiopulmonary Resuscitation/instrumentation , Cardiopulmonary Resuscitation , Intubation/methods , Intubation, Intratracheal/methods , Intubation, IntratrachealSubject(s)
Anesthesia, Inhalation/adverse effects , Angelman Syndrome/diagnosis , Delayed Emergence from Anesthesia/etiology , Laryngostenosis/etiology , Receptors, GABA-A/drug effects , Retrognathia/etiology , Anesthesia, Inhalation/methods , Anesthetics, General/pharmacology , Angelman Syndrome/complications , Angelman Syndrome/pathology , Benzodiazepines/pharmacology , Brain/pathology , Child, Preschool , Contraindications , Diagnostic Errors , Humans , Incidental Findings , Intellectual Disability/etiology , Intubation, Intratracheal/adverse effects , Magnetic Resonance Imaging , Male , Methyl Ethers/pharmacokinetics , Mucopolysaccharidoses/diagnosis , Muscle Hypotonia/etiology , Receptors, GABA-A/deficiency , Receptors, GABA-A/genetics , SevofluraneABSTRACT
No disponible
