case for discussion

[amyl]

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Young girl in late teens w h/o Loeys-Dietz syndrome for R vertebral-carotid reinsertion d/t many aneursyms. aside from the syndrome and its associated problems she is otherwise heathly, mild asthma.
as is c/w loeys-dietz she is full up of aneursyms including h/o thoracic ascending
aortic aneursym w aortic root dilitation s/p repair and AVR a few years ago and the above procedure on the L side that ended up getting into the subclavian as well.
pt reports poor functional class -- states she would be a little winded after climbing a flight of stairs. normal BMI.

the ;ast surgery came with several complications:
1. compartment syndrome of R forearm d/t combination of IV infiltrating + brachial arterial line placement w residual neuropathy and paresthesias -- R arm is mottled with muscle atropy in forearm and hand
2. severe intractable NV
3. uncontrolled pain
4. residual R LE paresthesias and pain apparently d/t femoral central line placement --

-- basic labs (cbc, cmp, coags all wnl)
anything else you care about in preop h and p, tests you wanna order?
what access you want and where?
thoughts?

 

[Idiopathic]

ugh

preop echo, decent IV access, no central line, art line in the left arm (is the chronic pain a component of the syndrome?), cerebral oximetry, consider TIVA/BIS (propofol/ketamine/dexmedetomidine/lidocaine/cisatracurium infusions)

id be okay with any access not in the right arm given proximity to the operation: left sided IVs or foot IVs. would be fine using ultrasound for a left neck central line if no decent veins in the extremities. i would avoid brachial art line placement

i would have nicardipine/nitroglycerine/nipride in the room (one hanging) as well as neo.

TEE ready to go, sternal saw on standby...do these aneurysms have higher risk of rupture_/

 

[Idiopathic]

thats probably totally wrong

 

[sevoflurane]

OK... I'll give it a go.

Issues:

• Loeys-Dietz
• Pre-existing heart disease
• Poor functional capacity
• Surgical Approach.
• Asthma
• RUE Neuropathy/RLE parasthesias/chronic pain

Pre-Op:

1. Loeys-Dietz: Where exactly are these anurysms and what is the surgical plan? Does it involve the posterior circulation? Is it complex and is circ arrest something that needs to be discussed? Other anurysms in the body? How about other manifestations of the disease? scoliosis (what's her cobb angle), congenital heart anomalies (Echo), craniocynastosis, arnold chiari formations or other head and neck anatomical issues (CT/MRI)?
2. Poor Functional Capacity. Is it heart related, lung related or due to some other process such as her neuropathy? A "young girl" should not have SOB when climbing one flight of stairs. Sounds like more than mild asthma.
3. What does her new valve look like? Perivalular leak causing SOB? Any other issues regarding her repair. EF? Dilated cardiomyopathy from previous AI?
4. Mild Asthma: Is she optimized?
5. What was done last time?
6. Careful documentation of neuromuscular function

Intra-op:

1. If she has disuse atrophy I would avoid Sux. O/W standard induction.
2. Femoral Access would be my route. I would have good access to it intra-op and would decrease the possibility of increasing ICP by obstructing venous flow (IJ). If she has cardiac dysfxn, you could also float a femoral PAC. She also has had this surgery on the left with involvement of the subclavian. I'd stay away. I might also put in a "just in case 18g or 16g" peripheral.
3. A-line: Radial on the good extremity and would consider femoral if the surgical approach puts the subclavian artery at risk (sounds like this happened during her previous operation).
4. Avoid anything that would increase ICP/anurysm rupture such as light anesthesia, bucking, brochospasm, etc. If she has vascular insufficiency then I would be careful not to drop BP all the while avoiding HTN with said anurysms.
5. Inotropes and vasodilators ready to fire.
6. Neuromonitoring and cerebral oxymetry.
7. If ICP was a potential issue, TIVA has advantages in reducing ICP and CBF while Halogenated agents increase both.
8. Type and Cross, products available.
9. Treat PONV with multiple drugs s/p repair.

Routine post-op ICU care with careful neuromonitoring.

 

[Planktonmd]

Would be nice to know more about the airway and how it was managed last time.
As for access I think a femoral line and a femoral arterial line would be a good idea here.
It seems that with every surgery we do this patient is getting closer to the grave!
I hope they stop trying to fix her before it's too late.

 

[proman]

Preop: needs an echo and recent CT scan (likely already has the CT for the vascular surgery). Functional status is a concern. Knowing whether or not she has cerebral aneurysms would be nice.

Intraop: I'd approach this similar to a CEA: 2 IVs and an arterial line. Cerebral oximeter, nitroprusside and phenylephrine gtt ready. She'll likely have a period of carotid occlusion. Assuming her echo shows normal LV function, I'd use propofol for induction and vecuronium for paralysis with 1/2 MAC isoflurane and 50% nitrous.

Postop: She's obviously high risk for PONV. I would minimize opioids, give preop scopolamine patch, dexamethasone and ondansetron at the end. ICU post op.

Couple of points:
I'm probably not worried about her ascending aneurysm because she's had her root replaced (again, depends on imaging obtained preop).
This is all extra-cranial so likely no major concerns re: ICP
Loeys and Dietz are at Hopkins so we encounter these patients frequently (usually for their root replacements). Vascular access is challenging because of aberrant vessels as well as abnormal connective tissue. My first choice for arterial access would be left radial artery, with ultrasound, keeping in mind she's had what sounds like a left vert/carotid/subclavian bypass. Femoral artery would be a good back up.

As for the genetics of the disorder, Loeys-Dietz is a caused by a defect in TGF beta receptor 1 or 2 and share many characteristics of Marfan's and vascular Ehlers Danlos. Because of the overlap in presenting signs and symptoms, many LDS patients are initially misdiagnosed. Interestingly, fibrillin activates TGF beta.

 

[proman]

TEE ready to go, sternal saw on standby...do these aneurysms have higher risk of rupture_/

She'd be a redo sternotomy so a sternal saw isn't going to help. I think the preop imaging/echo is key here.

 

[sevoflurane]

Lots of neuro during residency, but I have done a couple of these at most and proman has more experience than I do.

I could be wrong here, but isn’t the distal segment of the vertebral artery intracranial (before it becomes the basilar artery)? Isn’t this area the one most susceptible to aneurysms? Or is it extra cranial? The answer to this questions carries significant implications on ICP management.

Just wondering... 🙄

 

[proman]

Good point, I'm assuming it's being done by vascular surgeons extra-cranial. I am assuming that the area of interest is where the lowest arrow is pointing to (take-off of the vert at the subclavian):

Love the chriopracticeinjurylawyer.com source of the image!

 

[sevoflurane]

Did not notice that...

Interesting case report:

http://thejns.org/doi/abs/10.3171/jns.1989.70.3.0489?journalCode=jns

 

[amyl]

thanks for playing everyone. even tho loey-dietz comes w cleft-palate, etc she did not have either. and yes this was via vascular surgeons extracranial at the caudal end of these vessels.
believe it or not her echo was remarkably normal aside from what was to be expected from her surgical hx. the poor functional capacity was probably a combination of things: one probably a little more than mild asthma (one ER visit, no hospitalizations, no inutbutations but she was on xopenex which is typically first line) and i really think she was treated with kid gloves. when i asking her about physical activity, she kept saying i am not supposed to do this or that... i know you are thinking deconditioning should not affect a young person like that but i also suspect they were blowing this "shortness of breath" out of proportion. ( i am not judging i don't know what it was like for her or her parents to deal w this awful disease, these surgeries or their complications.... )
it came up about having the surgeons place the arterial line under direct visualization in one of the arteries in the exposed surgical site. some people were hesitant to place a L sided arm line d/t what happened with the last one. you guys ever use that approach to a line placement?
also, any thoughts on options post-operatively for regional for pain control?

 

[Idiopathic]

ive placed brachial arterial lines and never felt comfortable until they were out. would probably rather do an axillary line, honestly, but thats just anecdotal, i guess.

as far as my comment about sternotomy, id only be worried if the proximal subclavian A/V got injured and you had to emergently open the chest.

good point on the possibility of intracranial aneurysm from the vert, ive never seen this case and didnt think of it. however, increased ICP is not an issue unless something ruptures, so my concern would be keeping MAP in line with normal to avoid that. id still likely do a TIVA but for PONV and adjunct benefits rather than ICP.

 

[proman]

as far as my comment about sternotomy, id only be worried if the proximal subclavian A/V got injured and you had to emergently open the chest.

I get what you're saying but 2 points:

1) A supraclavicular incision can give adequate exposure up to the brachiocephalic artery if there's an injury to the subclavian. If needed they can remove the clavicle.

2) In patients who have had prior sternotomy, there's no easy way to enter the chest rapidly. Even a careful dissection sometimes leads to heart injury. Are you going to have a pump on standby? A cardiac surgeon available?

 

[proman]

i really think she was treated with kid gloves. when i asking her about physical activity, she kept saying i am not supposed to do this or that...

Good point about how some patients remain somewhat infantile (particulary the pediatric oncology patients who grow up).

it came up about having the surgeons place the arterial line under direct visualization in one of the arteries in the exposed surgical site. some people were hesitant to place a L sided arm line d/t what happened with the last one. you guys ever use that approach to a line placement?

I think the only reason to do this is to share the pain/blame if something goes wrong.

also, any thoughts on options post-operatively for regional for pain control?

She's going to hurt no matter what. A supraclavicular incision shouldn't be too painful, and I'd have the surgeons infiltrate with bupi. Ketamine may be helpful. Sounds like her chronic pain is related to the comparment syndrome more than her prior operation. Make sure she doesn't skip her pain meds. I'd also recommend she see interventional pain management if she doesn't already.