We present the case of a 2.8 kg baby born with Axial Mesodermal Disruption syndrome that encompasses features of Oculo-Auriculo-Vertebral spectrum disorder and VACTERL. OAV spectrum disorder is also known as Goldenhar. The patient was diagnosed with a left pulmonary artery sling, long segment tracheal stenosis, atrial septal defect and bilateral superior vena cavae. The patient underwent a colostomy immediately after birth due to an imperforate anus. Patient was also diagnosed with cleft palate, microtia, micrognathia and encephalocele. Patient then underwent a slide tracheoplasty with reimplantation of left pulmonary artery. Post operative course was uneventful. We acknowledge the assistance of Dr. Manning with the management of this patient.
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Nice demonstration.
Would you recommend pericardial patch over tracheal suture line? any additional tracheal release needed?
Thank you. We have not found pericardial patch necessary and prefer slide tracheoplasty for most tracheal reconstruction. Also we did not need any additional release like hilar release.
I think Dr Ghassan Baslaim question was related to the placement of pericardium along the suture line to avoid dehiscence of the tracheoplasty. Pericardium wrap is a good strategy. I like to move the thymus posteriorly to the aorta and on top of the tracheoplasty suture line to reduce the risk of fistula formation between airways and head and neck vessels.
By the way, very nice repair Dr Vinod Sebastian.