Anaesthesia concerns and perioperative management in a child with DiGeorge syndrome with corrected tetralogy of Fallot with pulmonary atresia posted for laparoscopic orchidopexy: Case report.

DiGeorge syndrome is afflicted with multiple congenital anomalies such as conotruncal and craniofacial anomaly, immune system dysfunction and hypoplasia/aplasia of parathyroid glands. Laparoscopy is a preferred surgical approach over open orchidopexy due to better visualisation of impalpable testis avoiding long incision, minimal tissue damage and a faster recovery. We report a case of DiGeorge syndrome with corrected tetralogy of Fallot with pulmonary atresia in a 1-year-old male child posted for laparoscopic orchidopexy. The anaesthesiologists face unique challenges due to the multisystem involvement and the effects of laparoscopic surgery on multiple organs. Thorough understanding of DiGeorge syndrome is essential for a good perioperative outcome.

Intraoperative customized double-patch device with twin sutures for multiple muscular septal defects.

OBJECTIVES: Closure of multiple muscular ventricular septal defects (VSDs) remains a challenge because of anatomical complexity. METHODS: We mapped all the VSDs using en face reconstruction of the right ventricular septal surface through echocardiography and then performed an 'Intraoperative Customized Double-Patch Device' technique to surgically close them in 39 patients (male:female = 25:14). The median age of the patients was 6 months (2 months-10 years), and mean weight was 5.98 ± 4.21 kg. A patch of polytetrafluoroethylene was placed on the left ventricular side of the defect and another on the right ventricular side, and they were anchored to each other using 2 polypropylene sutures. Residual shunts were evaluated using intraoperative echocardiography and measurements of right atrial-pulmonary arterial saturation were taken in all patients. RESULTS: The distribution of muscular VSDs was as follows: anterior muscular 12, posterior muscular 18, mid-muscular 11 and apical 9. The associated lesions included perimembranous VSD (n = 28), tetralogy of Fallot (n = 6), double-outlet right ventricle (n = 2) and supramitral membrane (n = 2). Mean clamp time and bypass time were 93 ± 19 min and 147 ± 26 min, respectively. Mean hospital stay was 11 ± 3.39 days with no in-hospital mortality. Five patients with significant residual shunts needed concomitant PA banding. All patients remained in New York Heart Association Class I. There was either no residual shunt (n = 3) or trivial shunt (n = 2) among the banded patients. All patients remained symptom-free and continued to thrive well at the most recent follow-up (3.48 ± 1.51 years). CONCLUSIONS: Muscular VSDs can be mapped through en face reconstruction and closed using intraoperative customized double-patch device technique in a variety of situations with satisfactory immediate and short-term results.

Complex aortopulmonary window in a single ventricle setting: Technical considerations for staged palliation.

We report a successful surgical management of a case presented with a combination of aortopulmonary window (APW) with large ventricular septal defect (VSD) amounting to a single ventricle, with a view to highlight technical considerations during staged single-ventricle palliation.