Anestesia en paciente pediátrico con síndrome de Rohhad / Anesthesia in a pediatric patient with ROHADD syndrome

El síndrome obesidad de rápida progresión, hipoventilación alveolar, disfunción hipotalámica y disregulación autonómica (ROHHAD) es una entidad infrecuente caracterizada por un comienzo en niños sanos a los 2-4 años. Se trata de un síndrome complejo caracterizado por una rápida ganancia de peso con hiperfagia, disfunción hipotalámica, hipoventilación central y disregulación autonómica, entre otros síntomas. Presentamos el caso de un niño de 10 años con diagnóstico de síndrome de ROHHAD a quien se colocó un porth-a-cath bajo anestesia general y que desarrolló complicaciones durante el procedimiento anestésico relacionadas con su enfermedad. El manejo perioperatorio de estos pacientes supone todo un reto para el anestesista dada la afectación de múltiples sistemas y las frecuentes comorbilidades respiratorias que asocian. Se resumen algunas de las implicaciones y consideraciones anestésicas que hay que tener en cuenta en el manejo de estos pacientes

Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome is a rare entity that is characterised by its onset in healthy children at 2-4 years of age. It is a complex syndrome that includes, among other symptoms, rapid weight gain with hyperphagia, hypothalamic dysfunction, central hypoventilation, and autonomic dysregulation. The case is presented of a 10-year-old boy with a diagnosis of ROHHAD syndrome undergoing insertion of a port-a-cath under general anaesthesia, who developed complications during the anaesthetic procedure related to his illness. The peri-operative management of these patients represents a challenge for the anaesthetist, given the involvement of multiple systems and the frequent respiratory comorbidities associated with them. A summary is presented of some of the implications and anaesthetic considerations that must be taken into account in the management of these patients

Anesthesia in a pediatric patient with ROHADD syndrome. / Anestesia en paciente pediátrico con síndrome de Rohhad.

Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome is a rare entity that is characterised by its onset in healthy children at 2-4 years of age. It is a complex syndrome that includes, among other symptoms, rapid weight gain with hyperphagia, hypothalamic dysfunction, central hypoventilation, and autonomic dysregulation. The case is presented of a 10-year-old boy with a diagnosis of ROHHAD syndrome undergoing insertion of a port-a-cath under general anaesthesia, who developed complications during the anaesthetic procedure related to his illness. The peri-operative management of these patients represents a challenge for the anaesthetist, given the involvement of multiple systems and the frequent respiratory comorbidities associated with them. A summary is presented of some of the implications and anaesthetic considerations that must be taken into account in the management of these patients.

[Practice Guidelines of the Spanish Society of Cardiology in the postoperative course of congenital heart diseases]. / Guías de práctica clínica de la Sociedad Española de Cardiología en el postoperado de cardiopatía congénita.

Improvements in myocardial protection, surgical techniques, and perioperative care have made it possible to achieve better prognosis in most congenital heart defects. This requires a coordinated, multidisciplinary approach to patient care, based on the preservation of adequate oxygen delivery to vital organs. It is important to have an understanding of normal postoperative status after cardiac surgery so that abnormal postoperative convalescence can be identified and treated.The causes of abnormal convalescence may be grouped into three categories: a) the pathophysiology of the defect before surgery and the acute changes in physiology that result from surgery; b) the effects of hypothermic cardiopulmonary bypass and deep hypothermic circulatory arrest on organ function, and c) the presence of residual anatomic defects. These conditions may result in prolonged convalescence as well as increased morbidity and mortality. Three primary hemodynamic pathophysiologic disturbances may occur during the postoperative period and lead to abnormal convalescence: left ventricular dysfunction, right ventricular dysfunction and pulmonary hypertension. Though sometimes not directly related to either the cardiac defect or surgery, specific problems involving different organs may alter the normal postoperative period. Neurologic, pneumologic, renal, gastrointestinal and infective complications are discussed separately.

[Anesthesia in a patient with toxic epidermal necrolysis]. / Anestesia en un paciente cón necrólisis epidérmica tóxica.

We report the case of a 16-month-old boy weighing 11 kg who presented toxic epidermal necrolysis secondary to phenobarbital administration and underwent emergency laparotomy under general anesthesia because of gastrointestinal bleeding. The boy presented epidermal detachment on the slightest friction (Nikolsky's sign) so monitoring was difficult; in these cases it is advisable to use padded sphygmomanometers and weighted electrodes. Owing to the great extension of denuded skin, these patients must be considered as hypovolemic and ketamine is the drug of choice for anesthesia induction. The maintenance of airways and the proper replacement of fluids and electrolytes are essential for the correct anesthetic treatment of these patients.