Perioperative Care for Children With Syndromic Craniofacial Synostosis Undergoing Le Fort III Surgery: A Retrospective Cohort Study.

OBJECTIVE: To present characteristics, surgical variables, complications, and postoperative care in pediatric patients with craniofacial synostosis undergoing Le Fort III osteotomy. BACKGROUND: Craniofacial synostoses are a group of genetic syndromes that result in premature fusion of cranial and facial sutures, leading to craniofacial deformities and associated complications. Midface advancement through Le Fort III osteotomy is the most frequent surgical option for these conditions. METHODS: Retrospective monocentric cohort study including patients with syndromic craniofacial synostosis who underwent Le Fort III osteotomy between 2009 and 2022 in a specialized referral center. Data collection encompassed surgical time, blood loss, intraoperative transfusions, fluid balance, and postoperative parameters such as duration of invasive mechanical ventilation and intensive care unit (ICU) length of stay. RESULTS: Twenty-six children were included in the analysis. The median surgical time was 345 minutes (300-360), with an estimated blood loss of 15 (9.9-24) mL/kg. Patients required a median transfusion of 12.63 (7.1-24.5) mL/kg of packed red blood cells and 19.82 (11.1-33) mL/kg of fresh frozen plasma. Intraoperative fluid balance was + 12.5 (0.8-22.8) mL/kg, with a median infusion of 30.4 (23.9-38.7) mL/kg of crystalloids. All patients were transferred to the ICU after surgery to ensure a safe environment for extubation. The median duration of mechanical ventilation in the ICU was 30 (20.25-45) hours, and postoperative ICU length of stay was 2 (2-4) days, and complications were infrequent, with only one extubation failure recorded. CONCLUSION: Le Fort III osteotomy in craniofacial synostosis patients may be characterized by a complex perioperative course. A multidisciplinary approach in the care of these patients allows for minimizing complications in the perioperative phase. Further research is needed to enhance perioperative management in this unique patient population.

Cervicomedullary Gliomas in Pediatric Age: A Systematic Review of the Literature and Tertiary Care Center Experience.

INTRODUCTION: Cervicomedullary gliomas (CMGs) are usually low-grade tumors often found in the pediatric age. Histological findings, treatments, and classification have been much the same for 40 years, although histological and molecular classifications have largely been developed for other pediatric CNS tumors. The management and treatment of pediatric CMG are still conducted by many authors according to their anatomical location and characteristics, independently from histology. METHODS: We conducted a literature review in PubMed (Medline) to identify relevant contributions about pediatric CMG published until December 31, 2021. We also analyzed a series of 10 patients with CMG treated from 2006 to 2021 at IRCCS Istituto Nazionale dei Tumori. The aim of the present review was to see whether and how the diagnosis, treatment, and classification of CMGs in children have developed over time, especially in the context of molecular advancements, and to analyze our single-center experience in the last 15 years. RESULTS: Thirty articles have been included in the review. Articles have been divided into two historical periods (1981-2000 and 2001-2021) and data from different series were analyzed to see how much the management and treatment of pediatric CMG have changed during years. Analysis of our series of 10 patients affected by CMG was also performed to compare it with the literature. DISCUSSION: Management and classification of CMG in children have not dramatically changed during years. However, new insight from molecular diagnostics and target therapies and the development of radiological, neurophysiological, and radiotherapy techniques have updated treatment modalities in the last 20 years. Treatment modalities and their innovations have been reviewed and discussed. Further studies are needed to standardize and customize treatment protocols for these tumors.

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. METHODS: A query of the PubMed database specific for "anesthesia" and "mucopolysaccharidosis" and a further query specific for "mucopolysaccharidosis and difficult airway management" was conducted. We looked for those items that offered practical guidance to anesthesiological management. We did not exclude case reports, especially those that reported a specific technique, because of their practical suggestions. RESULTS: We identified 15 reviews, 17 retrospective case series, 5 prospective studies, and 28 case reports that focused on airway managements in anesthesia or had practical suggestions for preoperative evaluation and risk assessment. An accurate preoperative evaluation and the need for an experienced team are emphasized in all the reviewed articles and for each type of mucopolysaccharidosis. Many suggestions on how to plan the perioperative period have been highlighted. Insertion of a laryngeal mask airway generally improves ventilation and facilitates intubation with a fiberoptic bronchoscope. Furthermore, the videolaryngoscope is very useful in making intubation easier and facilitating bronchoscope passage. CONCLUSIONS: Patients with mucopolysaccharidosis are at high risk for anesthesia-related complications and require a high level of attention. However, a multidisciplinary approach, combined with expertise in the use of new techniques and new devices for airway management, makes anesthesiological management safer. Further research with prospective studies would be useful.

Data on thermal and hydrolytic stability of both domiphen bromide and para-bromodomiphen bromide.

In this data article, HPLC analyses were applied to investigate the hydrolytic and thermal stability of domiphen bromide a FDA approved OTC ingredients endowed with antimicrobial activity. The data obtained by stressing domiphen bromide in acid, base and thermal conditions enlarge the research article published by Fumagalli et al. [2]. The chromatograms herein presented reveal that domiphen bromide is stable under acidic and thermal stress while the treatment with base yield to a by-product. The para-bromo derivative, p-bromodomiphen bromide shows the same behavior under the up mentioned stressed conditions.

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. METHODS: The anesthetic charts of mucopolysaccharidoses patients admitted from January 1999 to December 2014 were retrospectively analyzed. We retrieved patients' demographics; location and type of the procedure; anesthetic approach airway management and occurrence of difficult intubation and complications and outcome at hospital discharge. A generalized linear mixed model was performed to assess risk factors for complications and difficult intubation. RESULTS: Fifty-four consecutive children were included. The anesthetic charts of 232 procedures (52% radio-diagnostics, 15% orthopedics, 15% ear-nose-throat surgery, 10% neurosurgery, and 8% general surgery) were analyzed. Each patient underwent a median of 4 (1-6) procedures. The median age at the first procedure was 2 (1-5), and overall age was 5 (2-8) years old. One hundred and twenty-five (54%) procedures were performed in remote locations. General anesthesia was utilized for 100 (43%) procedures. No death was recorded. Twenty-one (9%) procedures had respiratory complications. Remote location anesthesia was associated with increased risk for complications (odds ratio 5.405 [1.355-28.571], P = .016). All planned intubations (n = 65) were successful. Nineteen (29%) of those were defined difficult. All emergency intubations (n = 3) failed and were rescued by laryngeal mask airways. Older age was associated with an increased risk of difficult intubation (OR 1.200 [1.019-1.436], P = .028). CONCLUSION: 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.

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. 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.

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. 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.