Prevalence and survival of patients with anorectal malformations: A population-based study.

BACKGROUND: Anorectal malformations (ARMs) are the most frequent congenital intestinal anomalies. The aim of this study was to describe the epidemiology of anorectal defects between 1981 and 2014 and to evaluate patients' survival. METHODS: A population-based study using data collected by an Italian, regional registry of birth defects and by the local Pediatric Surgery Units. RESULTS: A total of 428 individuals with ARM were identified, with an overall prevalence of 3.09 per 10,000 births. Characteristics associated with decreased survival were low birth weight (<2500 g) (HR 6.4; 95% CI, 2.3-17.9), the presence of two or more additional major defects (HR 7.9; 95% CI, 2.2-27.8), and birth before year 2000 (HR 4.7; 95% CI, 1.8-11.8). The 10-year survival probability was 100% for individuals with isolated ARM, regardless of their birth weight. Survival of patients with non-isolated ARM varied according to their year of birth and birth weight: 73.3% (≥2500 g) and 23.8% (<2500 g) in children born before 2000; 97.9% (≥2500 g) and 68.8% (<2500 g) in children born after year 2000. CONCLUSIONS: This study found a significant improvement in the survival of individuals with anorectal malformations over the past decades and identified the strongest predictors of mortality. LEVEL OF EVIDENCE (PROGNOSIS STUDY): Level II.

Erector spinae plane block combined with a novel technique for selective brachial plexus block in breast cancer surgery -a case report.

BACKGROUND: The breast is innervated by the intercostal nerves and the brachial plexus. We propose a technique to perform breast surgery without general anesthesia using the erector spinae plane (ESP) block and selective block of four nerves that arise from the brachial plexus innervate the breast and the axilla (SBP block). CASE: A 77-year-old man with breast cancer was scheduled for radical mastectomy and axillary clearance. He had a previous history of myocardial infarction with dilated cardiomyopathy and severely impaired ejection fraction. The surgery was performed under regional anesthesia with combined ESP and SBP block. The patient did not require opioids or other supplemental analgesics intra- or postoperatively and was discharged uneventfully. CONCLUSIONS: SBP is a novel block that selectively blocks branches of the brachial plexus that innervate the breast.

Prevalence, characteristics, and survival of children with esophageal atresia: A 32-year population-based study including 1,417,724 consecutive newborns.

BACKGROUND: Esophageal atresia (EA) is a congenital malformation of the upper gastrointestinal tract with an estimated prevalence varying from 1 in 2500 to 1 in 4500 births. The aim of this study was to describe the epidemiology of EA between 1981 and 2012 and evaluate patients' survival. METHODS: This study used data from a population-based Italian Congenital Malformation Registry. The survival status was ascertained by linking the registry records, vital records and the regional registries of patients. Kaplan-Meier methods were used to estimate survival probabilities up to 25 years and Cox proportional hazards regression was used to evaluate factors that affected survival. RESULTS: A total of 407 cases of EA were identified among 1,417,724 total births. After the exclusion of cases with chromosomal anomalies, 49.9% of the patients presented with at least one associated congenital anomaly. The 25-year survival probability was 85.1% (95% confidence interval [CI], 80.8-89.4), with most deaths occurring during the first months of life. Patients' characteristics associated with decreased survival probability were low birth weight (hazard ratio, 3.7; 95% CI, 1.7-8.3) and presence of additional major defects (hazard ratio, 2.8; 95% CI, 1.3-6.0). A significant improvement in survival over the decades was observed for patients with nonisolated EA. CONCLUSION: This study detected a significant improvement in survival of individuals with EA over the past decades and identified the strongest predictors of mortality. These results will be important for the planning of the clinical management and formulation of prognosis when EA is diagnosed in a newborn. Birth Defects Research (Part A) 106:542-548, 2016. © 2016 Wiley Periodicals, Inc.

Diaphragm Repair with a Novel Cross-Linked Collagen Biomaterial in a Growing Rabbit Model.

BACKGROUND: Neonates with congenital diaphragmatic hernia and large defects often require patch closure. Acellular collagen matrices (ACM) have been suggested as an alternative to synthetic durable patches as they are remodeled by the host or could also be used for tissue engineering purposes. MATERIALS AND METHODS: 2.0x1.0 cm diaphragmatic defects were created in 6-weeks old New-Zealand white rabbits. We compared reconstruction with a purpose-designed cross-linked ACM (Matricel) to 4-layer non-cross-linked small intestinal submucosa (SIS) and a 1-layer synthetic Dual Mesh (Gore-Tex). Unoperated animals or animals undergoing primary closure (4/0 polyglecaprone) served as age-matched controls. 60 (n = 25) resp. 90 (n = 17) days later, animals underwent chest x-ray and obduction for gross examination of explants, scoring of adhesion and inflammatory response. Also, uniaxial tensiometry was done, comparing explants to contralateral native diaphragmatic tissue. RESULTS: Overall weight nearly doubled from 1,554±242 g at surgery to 2,837±265 g at obduction (+84%). X-rays did show rare elevation of the left diaphragm (SIS = 1, Gore-Tex = 1, unoperated control = 1), but no herniation of abdominal organs. 56% of SIS and 10% of Matricel patches degraded with visceral bulging in four (SIS = 3, Matricel = 1). Adhesion scores were limited: 0.5 (Matricel) to 1 (SIS, Gore-Tex) to the left lung (p = 0.008) and 2.5 (Gore-Tex), 3 (SIS) and 4 (Matricel) to the liver (p<0.0001). Tensiometry revealed a reduced bursting strength but normal compliance for SIS. Compliance was reduced in Matricel and Gore-Tex (p<0.01). Inflammatory response was characterized by a more polymorphonuclear cell (SIS) resp. macrophage (Matricel) type of infiltrate (p<0.05). Fibrosis was similar for all groups, except there was less mature collagen deposited to Gore-Tex implants (p<0.05). CONCLUSIONS: Matricel induced a macrophage-dominated inflammatory response, more adhesions, had appropriate strength but a lesser compliance compared to native tissue. The herein investigated ACM is not a viable option for CDH repair.