ObjectiveTo determine predictors of poor outcome in patients with heterotaxy syndrome.MethodsA retrospective review of children with heterotaxy syndrome, in a single tertiary paediatric cardiology centre, was conducted between 1 January 1997 and 1 January 2014 to determine predictors of poor outcome. Poor outcome was defined as death, cardiac transplantation or New York Heart Association (NYHA) functional class III or IV.ResultsThere were 35 patients diagnosed with heterotaxy syndrome, 17 of whom were diagnosed antenatally. 22 patients had right atrial isomerism and 13 had left atrial isomerism. The median age of postnatal diagnosis was 2.5 days old (1 day to 19 months). 12 patients had a poor outcome; 6 patients died, 1 underwent cardiac transplantation and 5 had an NYHA functional class of >III. 5 patients had a biventricular repair and the remaining 30 had a univentricular repair. Type of atrial isomerism, univentricular or biventricular anatomy, severity of atrioventricular valve regurgitation or ventricular dysfunction, obstructed pulmonary venous return, occurrence of arrhythmia and presence of pulmonary atresia did not predict poor outcome. Fetal diagnosis also did not confer a survival advantage. The median duration of follow-up in this cohort was 65 months (2 days to 16.8 years).ConclusionsSurvival for patients with heterotaxy syndrome was 83% over a median follow-up of 65 months. 34% of patients had a poor outcome. None of the variables studied were predictive of death, transplantation or NYHA classification III or IV.

Following the introduction of fully endoscopic techniques for the resection of pituitary tumors, there was a rapid expansion of the indications for endonasal endoscopic surgery to include extrasellar tumors of the skull base. These techniques offer significant advantages over traditional open surgical approaches to the skull base, including improved tumor resection, and better post-operative neurological outcomes. Following their introduction, however, the initial rate of post-operative CSF leak was unacceptably high. Post-operative CSF leak following skull base surgery is a major source of morbidity, and can lead to the development of life-threatening intracranial infection. The use of vascularized naso-septal flaps transformed the management of these patients, significantly reducing the rate of post-operative CSF leak and increasing the number of patients that could benefit from this less invasive treatment modality. Adequate repair of iatrogenic defects in the skull base is of crucial importance for patients with skull base tumors, as the development of a post-operative CSF leak, and the associated complications can significantly delay the administration of the adjunctive oncological therapies these patients require. In this review, we provide an overview of the latest evidence regarding skull base reconstruction following endoscopic skull base surgery, and describe the skull base repair technique in use at our institution.

In recent years, three-dimensional printing has demonstrated reliable reproducibility of several organs including hearts with complex congenital cardiac anomalies. This represents the next step in advanced image processing and can be used to plan surgical repair. In this study, we describe three children with complex univentricular hearts and abnormal systemic or pulmonary venous drainage, in whom three-dimensional printed models based on CT data assisted with preoperative planning. For two children, after group discussion and examination of the models, a decision was made not to proceed with surgery. We extend the current clinical experience with three-dimensional printed modelling and discuss the benefits of such models in the setting of managing complex surgical problems in children with univentricular circulation and abnormal systemic or pulmonary venous drainage.