Glutaric aciduria type I (GA-1) is an inborn error of metabolism caused by a deficiency of glutaryl-CoA dehydrogenase. It presents early in life, usually after an episode of fever, dehydration, infection or fasting, and results in metabolic decompensation and neurologic damage. We report the perioperative management of a 5-year-old boy admitted to the hospital for surgery because of neurogenic hip dislocation. Here we present the preoperative preparation, which focused on appropriate fluid administration and therapy intensification, as well as the safe anesthetic management with inhalation anesthesia and remifentanil, taking into consideration the mitochondrial basis of the disease. Furthermore, the role of postoperative care is emphasized in relation to stress response prophylaxis and the avoidance of complications related to the disorder.

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We describe the case of a 5-year-old boy with phaeochromocytoma of the left adrenal gland, treated surgically by removal of the tumour under general anaesthesia. Phaeochromocytoma is a particularly rare tumour in children and surgical excision is the definitive treatment. We discuss the clinical and laboratory characteristics of the case, the diagnostic approach, the preoperative and intraoperative management and the postoperative course.

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