Patients with mucopolysaccharidoses are at high risk for anesthesia-related complications. Remote location anesthesia is associated with increased risk for complications, and older age is associated with increased risk for difficult intubation.
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. At San Gerardo Hospital (Monza, Italy) the management of CdLS patients is routinely organized through a standard protocol and a dedicated pediatric anesthesia team has been implemented. We report on a retrospective descriptive analysis of the anesthetic records of the CdLS patients admitted to San Gerardo Hospital from January 2010 to December 2015. We retrieved: demographics, genetic profiles, type of procedures, anesthetic approaches, anesthetics usage and complications. Data are reported as median (interquartile range) values. Twenty-seven patients (11 female), with age 12 (7-15) years old, weight 24 (14-35) kg, and severity score of 25 (18-32) were included. NIBPL mutations were the most frequently represented. We analyzed 58 procedures (30 esophagogastroduodenoscopies, 8 evoked auditory potential tests, 5 radiodiagnostics, 5 catheters positioning, 4 bronchoscopies) managed by sedation (36) and general anesthesia (6). Each patient underwent one (1-2) anesthetic procedure. Propofol (59%), sevoflurane (31%), fentanyl (24%), and ketamine (10%) were used. Three out of six endotracheal intubations were difficult. The only documented intraoperative complications were three episodes of desaturation (oxygen saturation <90%) occurring during sedations and were managed without the need for an invasive control of the airways. Implementation of a specific management protocol and a dedicated allowed to provide anesthesia to CdLS patients without the occurrence of major complications. © 2016 Wiley Periodicals, Inc.
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. We used a multivariate linear mixed model to evaluate the factors affecting induction propofol dose. We analyzed 1459 procedures (59% lumbar punctures, 31% bone marrow aspirations) performed on 96 children (7 [4-10] y old, 24 [16-34] kg, 37% female) admitted for acute lymphoblastic leukemia (80%), lymphoma (11%), and acute myeloid leukemia (7%). The induction propofol dose increased by 0.03 mg/kg per each procedure (P<0.05), from 2.6 (2.0-3.2) to 3.5 (2.6-4.3) mg/kg at the first and the last procedure, respectively. Higher age, weight, and use of ketamine were associated to lower propofol dosage (P<0.01), while combined procedures increased propofol dosage (P<0.01). In a large cohort of leukemic pediatric patients undergoing procedural sedation, the induction dose of propofol was increased over time, regardless of weight, age, use of corticosteroids, diagnosis, and treatment phase.
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