ABSTRACT
Pheochromocytomas, which derive from chromaffin cells, can secrete catecholamines in large amounts. The incidence of these tumors is low at 2 to 8 cases per million population and only 10% of cases occur in children. A thorough understanding of the pathophysiology of these tumors and the consequent effect of drugs will be necessary for treating these patients during the perioperative period. A great deal has been published on drugs that can be used in this setting, but only a small portion of the information is relevant to children. Esmolol, a short-action beta-blocker, figures among the drugs that have been developed in recent years. We report a case in which esmolol was used during surgery in a 10-year-old girl undergoing scheduled removal of a bilateral pheochromocytoma.
Subject(s)
Adrenal Gland Neoplasms/surgery , Adrenergic beta-Antagonists/therapeutic use , Hemodynamics/drug effects , Intraoperative Care/methods , Pheochromocytoma/surgery , Propanolamines/therapeutic use , Child , Female , HumansABSTRACT
Los feocromocitomas son tumores derivados de lascélulas cromafines, que pueden secretar grandes cantidadesde catecolaminas. Su incidencia es baja, 2-8 casospor millón de habitantes, solamente un 10% de éstos sedan en edad infantil. Para el tratamiento perioperatoriocorrecto se requiere un conocimiento profundo desu fisiopatología, y de la farmacología implicada. Haygran cantidad de información publicada sobre fármacosque pueden utilizarse pero no tan extensa en niños.En los últimos años se han ido desarrollando nuevosfármacos entre los que se encuentran el esmolol, unbeta bloqueante de acción corta. Presentamos un casode tratamiento intraoperatorio con esmolol en unapaciente de 10 años programada para la extirpación deun feocromocitoma bilateral(AU)
Pheochromocytomas, which derive from chromaffincells, can secrete catecholamines in large amounts. Theincidence of these tumors is low at 2 to 8 cases permillion population and only 10% of cases occur inchildren. A thorough understanding of thepathophysiology of these tumors and the consequenteffect of drugs will be necessary for treating thesepatients during the perioperative period. A great dealhas been published on drugs that can be used in thissetting, but only a small portion of the information isrelevant to children. Esmolol, a short-action -blocker,figures among the drugs that have been developed inrecent years. We report a case in which esmolol was usedduring surgery in a 10-year-old girl undergoingscheduled removal of a bilateral pheochromocytoma(AU)
Subject(s)
Humans , Female , Child , Pheochromocytoma/diagnosis , Pheochromocytoma/surgery , Intraoperative Period/instrumentation , Anesthesia/methods , Laparoscopy/methods , Isoflurane/therapeutic use , Hypnosis, Anesthetic/trends , Preanesthetic Medication/methods , Preanesthetic Medication , Hemodynamics , Anesthesia , Intraoperative Period/methods , Hemodynamics/physiology , Laparoscopy/trends , Preoperative Care/instrumentationABSTRACT
Cornelia de Lange syndrome involves multiple malformations with particular phenotypic features (craniofacial abnormalities such as microcephaly or hypertrichosis with synophrys; cutaneous abnormalities such as hirsutism, and limb anomalies) and it is associated with a high percentage of mental retardation and complications such as digestive tract abnormalities, cardiac defects, and endocrine disorders. We report the case of a 2-month-old infant girl who underwent a laparoscopic antireflux procedure, with closure of a diaphragmatic hernia and a gastric stoma. The medical history included repeated episodes of aspiration pneumonia and hypertension. Early in the procedure, 2 episodes of sudden desaturation, hypotension, and bradycardia with a probable diagnosis of air embolism. The complications resolved with specific treatment. Anesthetic management for laparoscopic surgery in these patients is truly complex and must be informed by a thorough understanding of the disease and complications that may develop.
Subject(s)
De Lange Syndrome/complications , Embolism, Air/etiology , Laparoscopy/adverse effects , Female , Humans , InfantABSTRACT
El síndrome de Cornelia de Lange es un cuadro polimalfomativopoco frecuente, con características fenotípicasparticulares (alteraciones craneofaciales comomicrocefalia o hipertricosis con sinofridia; alteracionescutáneas como hirsutismo; anomalías de las extremidades),asociado en un alto porcentaje a retraso mental yotro tipo de anomalías (digestivas, cardiacas, endocrinas).Presentamos el caso de una paciente de 2 meses deedad en la que se realizó por vía laparoscópica, una plastiaantirreflujo, cierre de defecto diafragmático y gastrostomía.Como antecedentes a destacar neumoníasaspirativas de repetición e hipertensión arterial. Duranteel inicio de la intervención quirúrgica, hubo dos episodiosde desaturación brusca, hipotensión y bradicardia,con diagnóstico probable de embolia aérea, que seresolvieron aplicando tratamiento específico. El manejoanestésico en estos pacientes para cirugía laparoscópicaes realmente complejo, por lo que hay que conocer demanera profunda la patología y complicaciones que puedansurgir(AU)
Cornelia de Lange syndrome involves multiplemalformations with particular phenotypic features(craniofacial abnormalities such as microcephaly orhypertrichosis with synophrys; cutaneous abnormalitiessuch as hirsutism, and limb anomalies) and it isassociated with a high percentage of mental retardationand complications such as digestive tract abnormalities,cardiac defects, and endocrine disorders. We report thecase of a 2-month-old infant girl who underwent alaparoscopic antireflux procedure, with closure of adiaphragmatic hernia and a gastric stoma. The medicalhistory included repeated episodes of aspirationpneumonia and hypertension. Early in the procedure, 2episodes of sudden desaturation, hypotension, andbradycardia with a probable diagnosis of air embolism.The complications resolved with specific treatment.Anesthetic management for laparoscopic surgery in thesepatients is truly complex and must be informed by athorough understanding of the disease and complicationsthat may develop(AU)
