There can be different configurations of the vessels, but, by and large, a true double aortic arch surrounds the trachea, and requires surgery. The reason for this is that it remains a fixed external obstruction to the trachea, not only at rest, but also with growth, so it will impinge on it eventually if it isn't doing it now.
With regard to his baclofen therapy, you will need to discuss this with your neurologist.
hi there, we are waiting to see our Cardia surgeon in Leeds, can I just ask are most DAA acceptable for surgery, someone has mentioned about if vessels/branches are coming off both arches it is difficult to seperate/impossible to seperate as you wouldnt be able to cut and keep the branches - can that be true?
thanks agin for the reply, I was kinda hoping you would say that as his trachea grows then the surrounding Arch would grow with it.... the thought of surgery and the risk of losing him frightens me to death after all he has been through recently.
I was talking to a 28 year old lady the other day who has had a DAA and diagnosed from 6MO and never had surgery so was hoping for the same.
Thank you so much for your swift reply,we have also had calcium levels checked and all appears to be ok. We have had his lymposite count done and its fine but waiting for his T and B cell split which hopefully will be tomorrow. Is there a chance that surgical intervention is not always needed in all cases and is there ever an instance where it can't be operated on. My consultant not heart just general says it depends on where the vessels are located on the arch so you could have 2 on one side and 2 on the other or even in this case would a vascular ring split work? Sorry a lot of questions. We should no soon about digeorge but I would hope that if he had no immune at all he would be sick a lot more often.
On another matter whjat do you know about baclofen if anything, my son has been weaned off it due to no spasms for months but today after withdrawl he has been all unsettled
Dear Dave and Julie,
Early on as the embryo forms, there are a number of double sided arches. Various parts of these arches disappear or become incorporated into other structures that eventually make up the aorta, pulmonary artery, and ductus arteriosus. A double-aortic arch is seen when there is a remnant of the embryologic structures that make up the aorta. The two sides of the arch surround the trachea and can compress it, causing respiratory distress. This is most frequently seen in infancy. However, in your son’s case, it appears that the combination of the compression and the internal airway swelling associated with the parainfluenza infection led to his respiratory problems and the subsequent discovery of this congenital anomaly.
As you found, it is associated with DiGeorge syndrome, or chromosome 22q11.2 deletion. If a thymus was seen on his imaging studies, the likelihood that he has this chromosome deletion is lower (but not zero). A genetic test will be needed to fully determine this, though. Unfortunately, the manifestations of DiGeorge can be quite variable, including both their appearance as well as which organs are affected and how much, so I cannot fully predict whether he has it, or not.
Without having all of the information in your son’s case, I cannot say for sure, but typically repair of a double-aortic arch is a simple procedure, depending on the skill of the surgeon as well as the experience of the institution. He should otherwise do well afterward. That said, because his airway was externally compressed for so long, there is a chance that he has a fixed obstruction that may require relief later when he grows up. In the end, this, as well as the neurologic outcome from the basal ganglia damage, will require observation over time.