The pentalogy of Cantrell is a disorder characterized by congenital abnormalities in the abdominal wall, lower sternum, anterior diaphragm, diaphragmatic pericardium, and cardiac anomalies. It is a rare disease with 250 cases registered around the world. The anesthetic implications will require a specialized management given the ventilatory mechanics and cardiac function which are compromised by the disease in the newborn. We present the case of a female patient with pentalogy of Cantrell without prenatal diagnosis, who had an operative procedure to correct patent ductus arteriosus and abdominal mesh placement under balanced general anesthesia with sevoflurane and fentanyl plus caudal block. This case is reported to provide our experience in the anesthetic management of this type of patients.
Introduction: Ichthyosis are hereditary disorders of keratinization which are characterized for the presence of hyperkeratosis and/or peeling. This disorder group can put in danger the life of the patient because of the severe alteration of the skin barrier, associated with a severe transdermic loss of water, different grades of hypothermia and a hypernatremic dehydration. Objective: Introducing the clinic case of an infant patient with the diagnosis of ichthyosis plus severe dehydration who is scheduled for placement of CVC. Clinical case: Male infant, 3 months old, with the diagnosis of ichthyosis and severe dehydration, scheduled for placement of CVC. Conclusions: Due to the urgency of our patient, who presented severe dehydration, an inhaled general anesthesia with sevoflurane and oxygen was decided, without instrumenting the airway which in these patients has the risk of being potentially difficult, from the placement of the face mask itself, until finding lesions within the oral cavity, ventilatory assistance should be maintained by the pediatric anesthesiologist, once venous access is achieved, the fluids restitution is performed with loads of 10 to 20 ml/kg of the patient's weight, hyperthermia was controlled with physical means.
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