Morbidity and mortality after anesthesia in early life in Italy. A subgroup analysis of the NECTARINE Trial.

BACKGROUND: Recent literature on neonatal anesthesia focuses on the importance of keeping physiology within the ranges of normality to improve the long-term neurological outcome. The Neonate and Children audit of Anesthesia pRactice IN Europe (NECTARINE) showed a derangement of one or more than one physiological parameters during anesthesia needing a medical intervention in 35.2% of 6592 anesthesia procedure performed in infants up to 60 weeks postmenstrual age. METHODS: Subanalysis of the Italian NECTARINE cohort providing a snapshot of anesthesia management, incidence of clinical events requiring intervention during anesthesia, and morbidity and mortality at 30 and 90 days. Secondary aim was to compare outcomes between Italy and Europe. RESULTS: Twenty-three Italian centers recruited 501 patients (63% male, 37% female) undergoing 611 procedures (441 surgical and 170 non-surgical) with a mean gestational age at birth of 38 weeks. Events requiring a medical intervention during anesthesia occurred in 177 cases (28.9%), lower than those reported in Europe (35.3%). The majority of events concerned episodes of cardiovascular instability, most commonly due to hypotension. The incidence of mortality at 30 days was 2.7%, consistent with the European incidence. CONCLUSIONS: Anesthetizing neonates is challenging. It is crucial that neonatal anesthesia practice is performed in specialized centers to maximize the potential positive outcome. We recommend a certification of quality for Institutions providing care for very young patients.

From Cortical and Subcortical Grey Matter Abnormalities to Neurobehavioral Phenotype of Angelman Syndrome: A Voxel-Based Morphometry Study.

Angelman syndrome (AS) is a rare neurogenetic disorder due to loss of expression of maternal ubiquitin-protein ligase E3A (UBE3A) gene. It is characterized by severe developmental delay, speech impairment, movement or balance disorder and typical behavioral uniqueness. Affected individuals show normal magnetic resonance imaging (MRI) findings, although mild dysmyelination may be observed. In this study, we adopted a quantitative MRI analysis with voxel-based morphometry (FSL-VBM) method to investigate disease-related changes in the cortical/subcortical grey matter (GM) structures. Since 2006 to 2013 twenty-six AS patients were assessed by our multidisciplinary team. From those, sixteen AS children with confirmed maternal 15q11-q13 deletions (mean age 7.7 ± 3.6 years) and twenty-one age-matched controls were recruited. The developmental delay and motor dysfunction were assessed using Bayley III and Gross Motor Function Measure (GMFM). Principal component analysis (PCA) was applied to the clinical and neuropsychological datasets. High-resolution T1-weighted images were acquired and FSL-VBM approach was applied to investigate differences in the local GM volume and to correlate clinical and neuropsychological changes in the regional distribution of GM. We found bilateral GM volume loss in AS compared to control children in the striatum, limbic structures, insular and orbitofrontal cortices. Voxel-wise correlation analysis with the principal components of the PCA output revealed a strong relationship with GM volume in the superior parietal lobule and precuneus on the left hemisphere. The anatomical distribution of cortical/subcortical GM changes plausibly related to several clinical features of the disease and may provide an important morphological underpinning for clinical and neurobehavioral symptoms in children with AS.

Open versus closed management of burn wounds in a low-income developing country.

Over a 38 month period, 264 patients were admitted for management of burns at the Emergency referral hospital in the resource-poor West African country of Sierra Leone. 244 charts and records were available for review, and 196 met the study's inclusion criteria. For the first 27 months, 158 patients were treated with the closed method and for the last 11 months of the study, 86 patients were treated with the open (exposure) method. Overall, the open method had as good or better early outcomes than the closed method, at significantly lower costs, and is the recommended treatment for burns in this particular type of environment.

Corrosive esophageal injuries in children. A shortlived experience in Sierra Leone.

OBJECTIVE: Children with caustic ingestions in developing countries are often treated at home, sometimes by traditional healers, or are referred, frequently late, to tertiary hospitals, which only seldom offer adequate endoscopic and dilatation facilities. Therefore, when dilatations are performed, the stricture is often already well established, making dilatation more difficult. The aim of this paper is to report our experience in the management of corrosive injuries in a group of children of Sierra Leone, all complaining accidental ingestion of caustic soda, many of them treated months after the ingestion. METHOD: We considered all children admitted after corrosive ingestion, from November 2001 to November 2005, to the "Emergency" Surgical Center in Goderich-Freetown, Sierra Leone. In December 2005 the hospital was supplied with endoscopes and dilatation devices. The children still followed up clinically were recalled to submit them to an endoscopic follow-up and to a dilatation, if needed. RESULTS: Forty children were admitted (mean age: 4.5 years): 16 (group A) after an esophageal perforation during dilatation performed elsewhere (death rate: 56%). Twenty-four children (group B) were observed after ingestion, 58% being submitted to a surgical gastrostomy. Death rate after ingestion was nil. The mean interval between ingestion and endoscopy was 8.8 months. Fifty-three dilatations were carried out in 17 children over a 3 months period. We report three perforations (17.6%) and a death rate of 5.8% (1/17). Two patients were lost to follow-up. Three patients (17.6%) did not show any improvement. Four children complained recurrent dysphagia after the first dilatation cycle. Overall, 10 children (58.8%) showed a clear-cut improvement at 6 months. CONCLUSIONS: The majority of treated strictures were late, therefore difficult to dilate and at higher risk of perforation. Dilatation with Savary bougies seems safer than with balloon catheters. Recurrent strictures and a long-term dilatation treatment should be expected. Retrograde dilatations through gastrostomies should be the preferred method of treatment and surgical gastrostomies should be performed without hesitation. Esophageal replacement is unlikely in these countries, except in very few referral centres. Therefore, any effort should be made to treat caustic strictures by timely dilatation programs.