Anesthesiological risks in mucopolysaccharidoses.

Background: Patients suffering from mucopolysaccharidosis are among the most complex from the anesthesiological point of view, especially regarding the management of the airway. The evidence base for anesthesia management is often limited to case reports and small case series.

Aims: To identify useful information about experience with each subtype of mucopolysaccharidosis reported in the literature and propose a guide on the best options for airway management to the anesthesiologists who take care of these patients.

Safety of anesthesia for children with mucopolysaccharidoses: A retrospective analysis of 54 patients.

Background: Complications are common during anesthesia for patients with mucopolysaccharidoses. San Gerardo Hospital (Italy) is a reference center for mucopolysaccharidoses with a dedicated pediatric anesthesia service.

Aims: This study aims to evaluate the safety of anesthesia for mucopolysaccharidoses patients, describe their anesthetic management at our institution, and assess risk factors for complications.

Induction Dosage of Propofol for Repeated Sedations in Children With Hematological Disorders.

Pediatric patients with hematologic malignancies require several procedural sedations by means of propofol infusion. We retrospectively analyzed the medical records of leukemic pediatric patients who had undergone procedural sedations at an Italian tertiary referral center (San Gerardo Hospital, Monza) from January 2011 to November 2013. We retrieved the following: demographics; diagnosis; chemotherapy phase; use of corticosteroids; induction dosage of propofol, fentanyl and/or ketamine; and the type of procedure.

Sedation and general anesthesia for patients with Cornelia De Lange syndrome: A case series.

Cornelia De Lange syndrome (CdLS) is a rare congenital disease characterized by typical facial dysmorphism, developmental disability, and limb deficiency defects. Various congenital malformations and medical complications have been described with gastroesophageal reflux as the major one. CdLS patients often require multiple high-risk anesthetic procedures.

Caesarean delivery of a patient with truncus arteriosus.

Truncus arteriosus Type IV is a rare congenital cardiac malformation characterized by agenesis of the pulmonary arteries. Pulmonary perfusion is ensured by bronchial arteries. One common arterial trunk arises from both ventricles and systemic pulmonary and coronary arteries originate from this common vessel.