Congenital diaphragmatic hernia survival in an English regional ECMO center

Introduction Congenital diaphragmatic hernia(CDH)remains a cause of neonatal death.Our aims are to describe contemporary rates of survival and the variables associated with this outcome,contrasting these with our study of two decades earlier and recent reports.Materials and methods A retrospective review of all infants diagnosed in a regional center between January 2000 and December 2020 was performed.The outcome of interest was survival.Possible explanatory variables included side of defect,use of complex ventilatory or hemodynamic strategies(inhaled nitric oxide(iNO),high-frequency oscillatory ventilation(HFOV),extracorporeal membrane oxygenation(ECMO),and Prostin),presence of antenatal diagnosis,associated anomalies,birth weight,and gestation.Temporal changes were studied by measuring outcomes in each of four consecutive 63-month periods.Results A total of 225 cases were diagnosed.Survival was 60%(134 of 225).Postnatal survival was 68%(134 of 198 liveborn),and postrepair survival was 84%(134 of 159 who survived to repair).Diagnosis was made antenatally in 66%of cases.Variables associated with mortality were the need for complex ventilatory strategies(iNO,HFOV,Prostin,and ECMO),antenatal diagnosis,right-sided defects,use of patch repair,associated anomalies,birth weight,and gestation.Survival has improved from our report of a prior decade and did not vary during the study period.Postnatal survival has improved despite fewer terminations.On multivariate analysis,the need for complex ventilation was the strongest predictor of death(OR=50,95%CI 13 to 224,p<0.0001),and associated anomalies ceased to be predictive.Conclusions Survival has improved from our earlier report,despite reduced numbers of terminations.This may be related to increased use of complex ventilatory strategies.

Predictors of surgical complications in boys with hypospadias:data from an international registry

Background Complications are frequently reported after hypospadias repair and there is a need to understand the factors that influence their occurrence.Methods Data from boys with hypospadias born between 2000 and 2020 were obtained from the International Disorders of Sex Development(l-DSD)Registry.Logistic regressions,fisher's exact tests and spearman's correlation tests were performed on the data to assess associations between clinical factors and complication rates.Results Of the 551 eligible boys,data were available on 160(29%).Within the cohort,the median(range)External Masculinization Score(EMS)was 6(2,9).All presented with one or more additional genital malformation and 61(38%)presented with additional extragenital malformations.Disorders of androgen action,androgen synthesis and gonadal development were diagnosed in 28(18%),22(14%)and 9(6%)boys,respectively.The remaining 101(62%)patients were diagnosedas having non-specific 46,XY Disorders of Sex Development.Eighty(50%)boys had evidence of abnormal biochemistry,and gene variants were identified in 42(26%).Median age at first hypospadias surgery was 2 years(0,9),and median length of follow-up was 5 years(0,17).Postsurgical complications were noted in 102(64%)boys.There were no significant associations with postsurgical complications.Conclusions Boys with proximal hypospadias in the I-DSD Registry have high rates of additional comorbidities and a high risk of postoperative complications.No clinical factors were significantly associated with complication rates.High complication rates with no observable cause suggest the involvement of other factors which need investigation.