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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.

Resumen El sindrome obesidad de rapida progresion, hipoventilacion alveolar, disfuncion hipotalamica y disregulacion autonomica (ROHHAD) es una entidad infrecuente caracterizada por un comienzo en ninos sanos a los 2-4 anos. Se trata de un sindrome complejo caracterizado por una rapida ganancia de peso con hiperfagia, disfuncion hipotalamica, hipoventilacion central y disregulacion autonomica, entre otros sintomas. Presentamos el caso de un nino de 10 anos con diagnostico de sindrome de ROHHAD a quien se coloco un porth-a-cath bajo anestesia general y que desarrollo complicaciones durante el procedimiento anestesico relacionadas con su enfermedad. El manejo perioperatorio de estos pacientes supone todo un reto para el anestesista dada la afectacion de multiples sistemas y las frecuentes comorbilidades respiratorias que asocian. Se resumen algunas de las implicaciones y consideraciones anestesicas 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.

El objetivo del tratamiento quirurgico para la esco-liosis, cifosis y otro tipo de deformidades del raquis esla fusion de las vertebras para que la columna verte-bral no pueda curvarse. Se colocan implantes metali-cos (barras, tornillos, alambres, placas, etc.), paramantener el raquis mientras las vertebras se fusionan.La artrodesis vertebral se potencia con injerto (autoge-nico y/o alogenico) de hueso. El abordaje quirurgicopuede ser posterior, anterior o combinacion de ambos.La cirugia toracoscopica se puede utilizar en el abor-daje anterior del raquis dorsal.La cirugia correctora del raquis en pediatria y adultoses un procedimiento prolongado (en general mas de 4horas), tiene las complicaciones potenciales de la ciru-gia mayor (hemorragia grave, hipotermia, infeccion,embolismo graso o aereo, hipotension arterial, etc.)

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.

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.