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PLoS Medicine Sep 2020Following a reduction in global child mortality due to communicable diseases, the relative contribution of congenital anomalies to child mortality is increasing.... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Following a reduction in global child mortality due to communicable diseases, the relative contribution of congenital anomalies to child mortality is increasing. Although infant survival of children born with congenital anomalies has improved for many anomaly types in recent decades, there is less evidence on survival beyond infancy. We aimed to systematically review, summarise, and quantify the existing population-based data on long-term survival of individuals born with specific major congenital anomalies and examine the factors associated with survival.
METHODS AND FINDINGS
Seven electronic databases (Medline, Embase, Scopus, PsycINFO, CINAHL, ProQuest Natural, and Biological Science Collections), reference lists, and citations of the included articles for studies published 1 January 1995 to 30 April 2020 were searched. Screening for eligibility, data extraction, and quality appraisal were performed in duplicate. We included original population-based studies that reported long-term survival (beyond 1 year of life) of children born with a major congenital anomaly with the follow-up starting from birth that were published in the English language as peer-reviewed papers. Studies on congenital heart defects (CHDs) were excluded because of a recent systematic review of population-based studies of CHD survival. Meta-analysis was performed to pool survival estimates, accounting for trends over time. Of 10,888 identified articles, 55 (n = 367,801 live births) met the inclusion criteria and were summarised narratively, 41 studies (n = 54,676) investigating eight congenital anomaly types (spina bifida [n = 7,422], encephalocele [n = 1,562], oesophageal atresia [n = 6,303], biliary atresia [n = 3,877], diaphragmatic hernia [n = 6,176], gastroschisis [n = 4,845], Down syndrome by presence of CHD [n = 22,317], and trisomy 18 [n = 2,174]) were included in the meta-analysis. These studies covered birth years from 1970 to 2015. Survival for children with spina bifida, oesophageal atresia, biliary atresia, diaphragmatic hernia, gastroschisis, and Down syndrome with an associated CHD has significantly improved over time, with the pooled odds ratios (ORs) of surviving per 10-year increase in birth year being OR = 1.34 (95% confidence interval [95% CI] 1.24-1.46), OR = 1.50 (95% CI 1.38-1.62), OR = 1.62 (95% CI 1.28-2.05), OR = 1.57 (95% CI 1.37-1.81), OR = 1.24 (95% CI 1.02-1.5), and OR = 1.99 (95% CI 1.67-2.37), respectively (p < 0.001 for all, except for gastroschisis [p = 0.029]). There was no observed improvement for children with encephalocele (OR = 0.98, 95% CI 0.95-1.01, p = 0.19) and children with biliary atresia surviving with native liver (OR = 0.96, 95% CI 0.88-1.03, p = 0.26). The presence of additional structural anomalies, low birth weight, and earlier year of birth were the most commonly reported predictors of reduced survival for any congenital anomaly type. The main limitation of the meta-analysis was the small number of studies and the small size of the cohorts, which limited the predictive capabilities of the models resulting in wide confidence intervals.
CONCLUSIONS
This systematic review and meta-analysis summarises estimates of long-term survival associated with major congenital anomalies. We report a significant improvement in survival of children with specific congenital anomalies over the last few decades and predict survival estimates up to 20 years of age for those born in 2020. This information is important for the planning and delivery of specialised medical, social, and education services and for counselling affected families. This trial was registered on the PROSPERO database (CRD42017074675).
Topics: Adult; Age Factors; Child; Child Mortality; Child, Preschool; Congenital Abnormalities; Female; Humans; Infant; Live Birth; Parturition; Pregnancy; Registries; Young Adult
PubMed: 32986711
DOI: 10.1371/journal.pmed.1003356 -
Pediatric Surgery International Mar 2024Sandifer syndrome (SS), which combines gastroesophageal reflux (GER) and a neurological or psychiatric disorder, is an uncommon condition that often takes a long time to... (Meta-Analysis)
Meta-Analysis
PURPOSE
Sandifer syndrome (SS), which combines gastroesophageal reflux (GER) and a neurological or psychiatric disorder, is an uncommon condition that often takes a long time to diagnosis. We aimed to systematically review available papers regarding SS.
METHODS
After presenting our two cases of SS, we systematically reviewed articles published in MEDILINE/PubMed, Cochrane Library, and Web of Science.
RESULTS
The meta-analysis included 54 reported cases and 2 of our own cases. Our results showed that all cases achieved symptom improvement with appropriate treatment for GER. Notably, 19 of the 56 cases exhibited anatomical anomalies, such as hiatal hernia and malrotation. Significantly more patients with than without anatomical anomalies required surgery (pā<ā0.001). However, 23 of the 29 patients without anatomical anomalies (79%) achieved symptom improvement without surgery. Patients who did not undergo surgery had a median (interquartile range) duration to symptom resolution of 1 (1-1) month.
CONCLUSION
The primary care providers should keep SS in the differential diagnosis of patients presenting with abnormal posturing and no apparent neuromuscular disorders. Fundoplication may be effective especially for patients with anatomical anomalies or those whose symptoms do not improve after more than 1 month with nonsurgical treatment.
Topics: Child; Humans; Gastroesophageal Reflux; Torticollis; Fundoplication; Hernia, Hiatal; Treatment Outcome; Laparoscopy
PubMed: 38526644
DOI: 10.1007/s00383-024-05683-3 -
Journal of Clinical GastroenterologyWith the advent of high-resolution esophageal manometry, it is recognized that the antireflux barrier receives a contribution from both the lower esophageal sphincter...
With the advent of high-resolution esophageal manometry, it is recognized that the antireflux barrier receives a contribution from both the lower esophageal sphincter (intrinsic sphincter) and the muscle of the crural diaphragm (extrinsic sphincter). Further, an increased intra-abdominal pressure is a major force responsible for an adaptive response of a competent sphincter or the disruption of the esophagogastric junction resulting in gastroesophageal reflux, especially in the presence of a hiatal hernia. This review describes how the pressure dynamics in the lower esophageal sphincter were discovered and measured over time and how this has influenced the development of antireflux surgery.
Topics: Esophageal Sphincter, Lower; Esophagogastric Junction; Gastroesophageal Reflux; Hernia, Hiatal; Humans; Manometry; Pressure
PubMed: 36084164
DOI: 10.1097/MCG.0000000000001756 -
Updates in Surgery Jun 2024A Morgagni hernia is a congenital diaphragmatic hernia that is rarely diagnosed in adults, and the technique for its repair has not yet been standardized. This review...
A Morgagni hernia is a congenital diaphragmatic hernia that is rarely diagnosed in adults, and the technique for its repair has not yet been standardized. This review will give an overview of the different laparoscopic methods reported by other authors, highlighting the key points indicating a good repair to help standardize the technique. A systematic review of the available articles on PubMed was conducted according to PRISMA 2020 by two authors independently in May 2022. Only articles written in English were included. A total of 180 case reports of laparoscopic Morgagni's hernia repair procedures were found; direct repair was performed in 59 patients, mesh was used in 119 patients, and mesh was not used in 2 patients. The hernia sac was removed in 71 patients, and the defect was closed before mesh placement in 49 patients. Nonabsorbable, dual or biologic mesh was used. The mean operative time was 92.65 min for direct repair and 84.11 min for mesh repair. One recurrence was reported in the direct repair series. The optimal method of repair has not yet been identified. The laparoscopic approach is associated to fewer complications and facilitates a faster recovery than the open approach. Several manoeuvres have been reported to help surgeons, who are not trained in laparoscopic knotting, perform extracorporeal knotting. Mesh should be placed when tension is too high after a direct repair or when primary closure cannot be achieved.
Topics: Adult; Female; Humans; Male; Hernias, Diaphragmatic, Congenital; Herniorrhaphy; Laparoscopy; Operative Time; Recurrence; Surgical Mesh; Treatment Outcome
PubMed: 37924436
DOI: 10.1007/s13304-023-01677-3