Interesting Headache Case - Test Your Skills

[jmj11]

I don't have too many interesting cases, but this one is. I will start by saying that this patient’s diagnosis was missed in the ED, and, but the grace of God, I would have missed it but was able to finalize it yesterday. I had to fight up-hill to get to the answers and almost gave up during the process. I will do this as a puzzle to solve (although I know the answer now) rather than giving you the whole story at once.

I saw a 29-year-old G-1 P-1 lady, who had given birth, under an epidural, to her daughter on September 6^th without complication. She noticed a low-grade headache the day of delivery and the subsequent two days, not thinking much about it, considering what she just went through. On day 3 post-partum, she woke up with a much more severe headache, level 6-8/10. The headache continued to build to about a 9/10 and stayed at that level. The only associated symptoms were photo phobia, phono phobia, no nausea. She had some transient right face numbness for a few days. The patient went to the ED (holding off because she was trying to recover at home after just having her first baby) the next week. She had a CT scan of the brain, which was normal. She was diagnosed with “Tension-Headache” and treated with hydromorphone.

The hydromorphone gave her a few hours of modest relief and the headache returned. The headache remained unchanged, so she went to her PCP the first week of October. The dx was “tension-migraine” related to being a new mother and was offered T-3, but she declined as she does not like to take medications.

She headache continued, and two weeks and she called her PCP. The PCP then made a referral to the headache clinic. The first available was November 20^th and the headache had remained unchanged until then. On that day I saw her, she gave the above HPI, plus no personal history of headache except for a couple of headaches in middle school, around menarche. She had no family history of headache. She has no other personal medical problems and is on no medications.

Her exam was completely normal, except that she looked like she was in pain and emotionally desperate for help.

What is your working differential?

What tests do you order?

I will do a follow up (if interest) with the “rest of the story.”

If someone figures it out now, or after additional information you will get a reward. I considered a Lamborghini or a trip for two to Venice, however, I decided to give lots of praise, a free copy of my up-coming novel Waters of Bimini (a medical mystery with a PA hero) and maybe a Starbucks card.

[pastudentw]

I'm student but...

Given the post partum history, rarely you can have postpartum preeclampsia for a while after delivery. Up to 6 weeks (more commonly within 48 hours) so this may not fit given the time frame, but since you are saying this is an out of the ordinary case, it could be considered.

What was her BP? Urinalysis? 

Just a thought but maybe I'm way off. 

[jmj11]

That was a worthy consideration, especially around the time of delivery, her B/P (don't have it in front of me right now) was very normal and I think a UA was done in the ED and was normal.

[sk732]

Post epidural HA...odd presentation for it, but if she kept having a slow leak, would make sense.  Blood patch or a gift card for Tim Horton's or Starbucks for couple large coffees...or both.

 

SK

[jmj11]

That was one of my top three thoughts, would have gone looking for a leak, if I had not found the cause. The headaches were never positional, which isn't an absolute in spinal leaks but would have raised more suspicion. Those post-epidural leaks are often missed, and the leak often eventually seals on its own. But leaks do occur from 1 / 100-500 of cases, depending on the study. But that was a very reasonable and was second on my differential. 

[narcan]

I'm still tripping over the fact that the ED treated her headache with hydromorphone.

Differential without additional data:
1. post-LP headache (still the most likely until you patch it, positional or not)
2. HELLP syndrome (although presumably the ED checked labs)
2. missed SAH (CT not sensitive enough after 6 hours)
3. dural venous thrombosis (although CT often sensitive)
4. atypical migraine (always in the differential)
5. viral meningitis (I hope you would've said if she were febrile)
6. Idiopathic intracranial hypertension

Workup depends on where you're at and what you have available. In in ideal world, and because I know this is likely a rare or bizarre diagnosis, MRI of the brain and MRA of the head/neck would be the ideal tests of choice. If those are negative, you're stuck doing a tap, although check with your local radiologist's comfort level ruling out SAH from an MRI.

[sk732]

Headache global or unilateral?  Changes with movement?  I've seen the odd post-partum migraine sort of thing where the trigger was hormonal changes associated with childbirth...transient numbness can be "aura"...rotational stuff could be occipital neuralgia...could have some sort of meningo-encephalitis too if their was poor asceptic technique.

[Guest ral]

"had some transient right face numbness for a few days"

Makes me consider varicella/herpes virus infection, or trigeminal neuralgia variant.

I did have an interesting case about ten years ago, with a young female (late 20's) who presented to our ER with a persistent headache, and hers turned out to be an internal carotid dissection.  Considering your patient delivered a baby, a prolonged/repeated valsalva could certainly result in such.

[jmj11]

34 minutes ago, sk732 said:

Headache global or unilateral?  Changes with movement?  I've seen the odd post-partum migraine sort of thing where the trigger was hormonal changes associated with childbirth...transient numbness can be "aura"...rotational stuff could be occipital neuralgia...could have some sort of meningo-encephalitis too if their was poor asceptic technique.

The headache was mostly occipital and favored the left side. What you described as partum-triggered status migraine was my choice # 1, as it is the most common reason for this kind of headache, but it, the final diagnosis, turned out to be my # 3 on my list. meningitis or encephalitis would have been a remote possibility, however, while in severe pain, and emotionally worn out, she was otherwise not "sick" during this ordeal.  

[jmj11]

43 minutes ago, narcan said:

I'm still tripping over the fact that the ED treated her headache with hydromorphone.

Differential without additional data:
1. post-LP headache (still the most likely until you patch it, positional or not)
2. HELLP syndrome (although presumably the ED checked labs)
2. missed SAH (CT not sensitive enough after 6 hours)
3. dural venous thrombosis (although CT often sensitive)
4. atypical migraine (always in the differential)
5. viral meningitis (I hope you would've said if she were febrile)
6. Idiopathic intracranial hypertension

Workup depends on where you're at and what you have available. In in ideal world, and because I know this is likely a rare or bizarre diagnosis, MRI of the brain and MRA of the head/neck would be the ideal tests of choice. If those are negative, you're stuck doing a tap, although check with your local radiologist's comfort level ruling out SAH from an MRI.

Good guesses. It WAS one of the six above.

[Kaepora]

My very first thought (like everyone else it seems) was csf leak, but I see that was r/o.  I think a sinus occlusive disease d/t pregnancy/puerperium would be top of my list.  Doesn't present like a typical SAH.  I would think HELLP would have been ruled in or out right away.  Same with meningitis.  All good thoughts here.

[Guest ral]

"She had a CT scan of the brain, which was normal."

Would they not see a clot sign? (if it was a CVST, along with the duration)

I am asking, as I am not well versed in these.

[jmj11]

6 minutes ago, ral said:

"She had a CT scan of the brain, which was normal."

Would they not see a clot sign? (if it was a CVST, along with the duration)

I am asking, as I am not well versed in these.

For a venous clot, a non-contrast CT could miss it unless there has been an associated hemorrhage or infarction. 

[Guest ral]

11 minutes ago, jmj11 said:

For a venous clot, a non-contrast CT could miss it unless there has been an associated hemorrhage or infarction. 

Ahh...okay, thanks.

[sk732]

Did you do anything that made it better for her?  If unilateral, and person is having pain to palpation in the occipital nerve distribution, I'll offer an occipital nerve block - have a few ED regulars that get them from me actually.  I take it CBC, biochem were normal?

[jmj11]

I have given it partially away that is one of Narcan's six.

This is where this gets tricky. I will tell you the data that came in and explain at the end.

Her fundi were normal without any evidence of papilledema. An opening pressure was not done.

The MRI and MR-V (very hard to get them prior authorized) were read by the local radiologist as normal (notice the emphasis on read).

 

[jmj11]

4 minutes ago, sk732 said:

Did you do anything that made it better for her?  If unilateral, and person is having pain to palpation in the occipital nerve distribution, I'll offer an occipital nerve block - have a few ED regulars that get them from me actually.  I take it CBC, biochem were normal?

My first treatment, thinking that it was status migraine, was steriods + daily naratriptan, did give her 2-3 days of much relief, but then the headache came right back. My # 1 differential (status migraine) was not correct.

Her CBC was slightly elevated (I'm not at work and can't remember the numbers) and her D Dimer was elevated (again, can't remember the numbers).  All other labs were normal.

[sk732]

Hmm - CBC out to lunch 2 months out doesn't sound good...I'd expect the D-Dimer up just post partum, but not by now.  No jaw claudication or visual disturbance aside from photophobia? 

Interesting Smithers...

SK

[Guest Samhain_Grim]

14 minutes ago, jmj11 said:

I have given it partially away that is one of Narcan's six.

This is where this gets tricky. I will tell you the data that came in and explain at the end.

Her fundi were normal without any evidence of papilledema. An opening pressure was not done.

The MRI and MR-V (very hard to get them prior authorized) were read by the local radiologist as normal (notice the emphasis on read).

 

You're giving lots of hints, here. Did they run all typical sequences with the MRI/MRV (including DWI/GRE)? Were you able to review the images yourself?

The facial numbness lasted a few days and, even if transient, would make me suspicious for some sort of small infarct. Assuming this is CVST, this could make sense in the setting of a small thalamic venous infarct or edema. However, deep cerebral veins can be hard to pick up on MRI. The transverse sinuses can appear to have normal flow-voids even in the setting of a thrombus due to the image acquisition, but I think MRV would have remedied that?

I'd want to review the images myself, but barring that I'd consider a CTV with con to get a better look at deep cerebral veins. 

Otherwise, like others have mentioned: I'd consider a slow CSF leak or a small SAH (though I'd expect to see this after a few weeks on the MRI, even if it was missed on CT). If the CTV was normal, I'd start to think about a tap..

[jmj11]

I don't know when Narcan is coming back and if he/she knows the answer. I will post the rest of the story below. I'm not at work today, but if can figure out how to save the images without the name attached, I will try to post them when I'm back at work next week.

 

So, I will tell this story in real-world, the things that we all have to put up with.

When I saw her on November 20th, as I alluded to, my differential was 1) postpartum status migraine (by far most common), 2) possible persistent dural leak from an epidural, that accidently went subdural, as least during the placement. and 3) peri-partum central venous thrombosis. I was worried enough about # 2 (but the headache was never positional) and # 3 (no real personal or family history of migraine and not positional to support # 1 or # 2 so # 3 plausible as she was quite distressed with 8 weeks of significant pain. Her CT was negative and she had none of the dismal associated symptoms with a central venous thrombosis, including infarct,  seizure, other neurological signs.

I started her on a prednisone burst and daily naratriptan, (expecting # 1 above) and ordered a brain MRI / MR-venogram and scheduled her back in four days because she was not doing well.

Her insurance rejected both the MRI and MR-V. I started a prior authorization appeal (we rescheduled her until the MRI / V could be done). Second time around, both exams rejected by the insurance. At this juncture, I came very close to giving up on the exams. I asked for a "peer to peer" with a AIM radiologist (AIM contracts with insurance companies to try and disapprove of radiological exams). I spoke to the radiologist and came in with my guns a blazing. It turns out that the exam was ordered by the hospital prior auth department as "Migraine." Nowhere, except in my billing sheet for the visit, did I list status migraine as one of three diagnoses. On the radiology request form, along with a brief HPI I ordered the MRI for "New worsening, daily headache." For the MR-V I listed central venous thrombosis. I have no clue why the exam was then sent to the insurance as "migraine," but I'm looking into it.  So, the AIM radiologist approved the MRI, but chuckled at the MR-V, stating that only big, university settings and said he is not approving it. I argued for it.

We immediately schedule the MRI and to see me back the following day (yesterday). Just before her visit, I went to look her images online. First I noticed that the MRI and MR-V were both done and read as normal. I again came very close to moving on and treating this as status migraine.

I pulled up the images and viewed the MRI. I didn't see anything abnormal. I viewed the MR-V. It is typical to have a dominant transverse venous sinus. Her right was much larger than her left. However, one segment of the left was very small and I saw no patent lumen.

I called the reading radiologist and spoke to him. He said again that the MR-V shows a normal right-dominant transverse sinus. I pointed out that I see no patent lumen. He (in the "reading room" with HD screens and more views than I can see) looked closer and commented that in his view he can see a tiny flow, but started to agree that it was tiny. He asked if I wanted it sent to the neuroradiologist and I said I did.

I saw the patient in the meantime. The prednisone and naratriptan did help for two-three days with the pain much less, but by the third day, it was back to thes status quo and no new symptoms.

I had her wait while I saw another patient. When the neuroradiologist never called back, so I called her. She reviewed the image in real time with me on the phone. She agreed that the left transverse venous sinus was not normal and appeared to have a clot, a clot that was probably fully occlusive (her guess listening to the story) in the beginning and now is starting to recanalize. 

I called her OB, who is also my hospital's Chief of Medicine. I do not feel comfortable managing this patient. I wanted to send her out of town to a stroke neurologist to follow her. Her OB, who had only seen one other case his entire 45-year career, wanted me to send her to the hospital internist for management. Unfortunately, later in the afternoon (I had the patient still in my office) the internist said he could not see her (too busy) and that there is no reason her PCP could not follow her. If this had been the first couple of weeks, she would be admitted and heparinized or even directly "clot busted." I did research and apparently, at this stage, with recanalization and no neurological complication, it is reasonable to treat with oral warfarin. I order coag labs, D dimer and started her on 2 MG of warfarin. I will see her back in five days with an new INR. I hope that her PCP has seen her by then to take over management.

So, one take home is to look at the patient and not the image report. I almost let this go twice. For the other take home, I will leave a link to remind us to thing CVT in peripartum patients with new headache.

I'm not at work but will try to insert the images next week.

 

[sk732]

Cool Case - thanks for that.

 

SK

[Guest JMPA]

this highlights the importance of communicating with the radiologist what you are suspecting. clinical history and findings can help with image interpretation.

Very nice catch

[Marinejiujitsu]

I'm puttingg my money on HELLP syndrome.

I have seen SAH 6 weeks out of delivery thinking most likely hellp but found 2 subarachnoid hemorrhages.

Sent from my SM-N950U using Tapatalk

[SERENITY NOW]

Jeez...Add it to the list of crappy things that can happen to women surrounding pregnancy... they're at an increased risk for everything!  

 

But anyways, excellent case - thanks for sharing.  Keep the cases coming when you have more!

[d2305]

2mg warfarin sounds kind of low.