Sharing Mayo Clinic

Stories from patients, family, friends and Mayo Clinic staff

Share this:

Oct 17, 2016 · 30 Replies

Diagnosis and Treatment of Chiari Malformation Spurs Nikki Prins to Reach Out to Others

By SharingMayoClinic @SharingMayoClinic

Nikki Prins enjoying time outdoors.Oct. 23, 2015, is a date that Nikole Prins will always remember. It’s the day she finally learned the reason for the bizarre symptoms she had been dealing with for more than half her life. Nikki precisely recalls the time she heard the news. It was 1:24 in the afternoon when her doctor called with the results of an MRI she had the previous day.

“I was diagnosed with Chiari malformation,” says Nikki, who lives in Owatonna, Minnesota, and received care at Mayo Clinic Health System in Owatonna. “No one wants to be told at 21 that you have a brain malformation. I cried a lot.”

For years, Nikki had experienced a range of symptoms that included lightheadedness, fainting and extremities that tingled when she stood up, as well as migraines that got worse while she was standing. As a preteen, she was told she was having syncope episodes when she got lightheaded. Later, physicians made the diagnosis of postural orthostatic tachycardia syndrome. But neither diagnosis nor any treatment had an effect on her symptoms.

In the fall of 2015, her incidences of passing out were followed by a total loss of feeling in her limbs. She was also having problems breathing and swallowing, and she noticed odd fluctuations in her voice. It was then Nikki decided to ask her family practice physician to investigate further. He recommended the MRI that uncovered the true cause of Nikki’s symptoms. 

“Chiari malformation isn’t an easy diagnosis to take on,” Nikki says. “People find themselves alone in that kind of diagnosis, and it’s important to me that people get the proper care because it can go wrong. I’m in so many Chiari support groups, and I’ve heard many stories where people end up in the wrong hands and something takes a turn for the worse.”

Reassuring care

Of the three types of Chiari malformation, Nikki was diagnosed with type 1, which is the most common and least severe. It usually develops in late childhood or early adulthood. Chiari malformation is estimated to occur in about one in 1,000 births. It happens when the lower portion of part of the brain called the cerebellum extends into the bony opening of the spinal canal that’s meant for the spinal cord. As a result, spinal fluid builds up, putting pressure on the brain and brain stem. That leads to problems with balance, coordination and movement.

After she was diagnosed, Nikki was directed to the Department of Neurology at Mayo Clinic’s Rochester campus, where she met with a neurologist and with neurosurgeon Grant Mallory, M.D. Within 10 minutes of meeting Dr. Mallory, Nikki was informed that surgery was imperative. While she was initially skeptical, Dr. Mallory’s bedside manner and his ability to convey facts in a relatable way convinced Nikki that she was in the right place.

“I can’t say enough about Dr. Mallory. He was phenomenal,” Nikki. “I trusted him with everything, and I still do.”


“Everyone was really good about being personable when they talked to me. They were really sensitive about how they said everything.” – Nikki Prins


In early January 2016, Nikki underwent a type of brain surgery called a suboccipital craniectomy. Surgeons removed part of the bottom of Nikki’s skull and a portion of her first cervical vertebra. The dura matter — the outermost membranes surrounding the brain and spinal cord — was opened and a bovine patch was sewed in to give the brainstem more space.

Nikki was discharged two days after her surgery, hopeful that the treatment would put an end to her problems with head pain, numbness and breathing. But not quite two weeks later, she awoke with a debilitating migraine.

“I thought it would get better through the day. I was still on leave from work, so I just took the day to take it easy,” Nikki says. “Finally at 5 p.m., I was trying to fold laundry, and I ended up passing out on my couch.”

Nikki called her mom, Karla Prins, who drove her to the hospital in Owatonna. At the emergency room, doctors put Nikki in an ambulance and sent her to Rochester, where she was immediately admitted. Her care team found that Nikki had contracted a staph infection at the incision site. In addition, the inflamed and swollen wound was leaking spinal fluid.

Setbacks and solutions

A second surgery was necessary, but first Nikki’s infection had to be addressed. A culture of the infection yielded a diagnosis of methicillin sensitive staph aureus, or MSSA. Nikki received alternative antibiotics to combat it, and the next day, she underwent surgery. Surgeons washed out the wound and replaced the bovine patch with collagen.

A lumbar puncture, or spinal tap, was also conducted as a cautionary measure to check for meningitis — an inflammation of the membranes surrounding the brain and spinal cord. The test was negative for any additional infections, but Nikki was outfitted with a PICC line and given an intravenous antibiotic. With directions to continue the IV therapy as an outpatient, Nikki was discharged after five days.

“I felt like a human pincushion,” Nikki says. “I thought I was going to have one IV during surgery, but I ended up having 23.”

On Feb. 17, the PICC line was removed. Nikki attempted to pick up where her life had left off. And she did — for three weeks.

“On March 6, I sprung a spinal fluid leak,” Nikki says, explaining that the event was more annoying than painful. This time, the fluid draining from the incision site was trickling outside of her body and covered two towels with spinal fluid.


“A lot of people don’t know what the next steps should be and some people think there are only a few doctors who know about Chiari. But that’s not the case because I went to a place where they know a ton.” – Nikki Prins


“I called the clinic at Rochester and said, ‘I need someone to get Dr. Mallory to call me,’” Nikki says. “So he calls me back and says, ‘What’s going on?’ And he’s really funny, and he’s always liked to joke around with me, so I said, ‘Well, I’m leaking from my neck.’ And he said, ‘Well, that’s not good!’”

He directed Nikki to get back to the hospital in Rochester, where she was prepped for a third operation. During that procedure, Nikki’s wound was again cleansed. Her surgeons replaced the collagen patch with a fascial graft from her thigh and also inserted a lumbar drain to prevent future leaks. Five days later, Nikki was released from the hospital.

On April 1, she was cleared to go back to work, Nikki says, adding that Dr. Mallory jokingly told her that he didn’t want her coming back to see him unless it was for a social visit.

“Everyone was really good about being personable when they talked to me. They were really sensitive about how they said everything,” Nikki recalls. “It was a really sensitive time for me and for my parents.”

Empowered through education

While Chiari malformation may cause progressive and debilitating symptoms, they often can be halted and even improved with surgery. And rather than embarrassment or fear about her diagnosis, Nikki has embraced her future and hopes her success story helps others with Chiari malformation. She encourages people affected by the disorder to learn more, seek out resources, and connect with others through organizations such as Conquer Chiari.

“A lot of people don’t know what the next steps should be, and some people think there are only a few doctors who know about Chiari. But that’s not the case because I went to a place where they know a ton,” she says.

“It really does matter where you go with this malformation,” Nikki says. “You’re going to find yourself with a little bit of frustration just because of the condition, but don’t give up. Keep fighting and finding the doctors that you’re comfortable with and who can communicate with you on the same level.”


HELPFUL LINKS

Tags: Chiari Malformation, Dr. Grant Mallory, Mayo Clinic Health System, Mayo Clinic Health System in Owatonna, Neurology & Neurosurgery, Neurology & Neurosurgery

abbydabby

Posted by @abbydabby, Oct 28, 2016

I had spinal fluid leaks too...waking up soaking wet with it!

nikoleprins

Posted by @nikoleprins, Nov 3, 2016

It's a weird and gross feeling to wake up to that!!!!

jimm45

Posted by @jimm45, Nov 2, 2016

My wife had the same surgery several years ago at Mayo with excellent results. However, she has been having recurring symptoms. Does this ever happen. What would be the best next steps?

nikoleprins

Posted by @nikoleprins, Nov 3, 2016

I do have some recurring symptoms now, the best thing to do would probably be to consult her primary care, in my opinion. Don't stop fighting for answers, Jim.

jimm45

Posted by @jimm45, Nov 5, 2016

Nikki thank you for your reply.

Dr John Atkinson was the Mayo surgeon (very happy with the surgery). Fortunately she did quite well - very well in fact. St Mary's was amazing! I got to watch the medical Helicoptor come and go from Sharon's room! Sharon was seen after the surgery by one of Mayo's neurologists and was later diagnosed with a small aneurysm as well as a "silent" stroke. We weren't happy with him and asked to be seen by a different neurologist. (He couldn't answer any of our questions (re: stroke) which we were very concerned about.) Unfortunately he has to "approve" our request and will not see her or allow her to be seen by another neurologist. He also informed us that Mayo has not seen a patient after the surgery with recurring symptoms. 🙁

Sharon also had another surgery at St Mary's and ended up with a major infection and an additional trip and week long "stay" as well as return trips every 2 weeks for a couple of months! BUT we wouldn't go anywhere else!!

Sharon and I are so glad that you are doing better! Although we don't know you personally we do understand.

Nikole Prins likes this
eappleberry

Posted by @eappleberry, Nov 3, 2016

Nikki, I'm so very sorry to read about the multiple problems you experienced. I underwent surgery for a Chiari malformation in 2010 at West Jefferson Medical Center in Marrero, Louisiana. My surgery was a complete success. Prior to my surgery, I experienced symptoms much like the symptoms you experienced. My headaches were called "cluster headaches:". The headaches began when I was a teenager and got progressively worse. For a long time, I thought I had a brain tumor. I started losing my grip and my balance. Dr. John Steck of Cuclicchia Neurological Clinic saved my life with the surgery performed. It's been 6 years since the surgery and now I am started to experience strange tingling in my hands and feet. Your article is giving me the courage to face my problems. Thanks so much.

nikoleprins

Posted by @nikoleprins, Nov 3, 2016

I am always here to help and support in anyway that I can. I think it's great that you're gaining that courage. Just remember to keep fighting, you are Worth The Fight.

dboen

Posted by @dboen, Nov 12, 2016

I was recently diagnosed with arnold chiari malformation. I was told i need surgery fairly soon. I am very nervous and scared. Can anyone please tell me about their experience with the surgery and recovery process.

nikoleprins

Posted by @nikoleprins, Nov 14, 2016

Hi! I was out of work for about 3 weeks after my third surgery, but all together had been out for 3 months because of all the emergent cases. I would take about 6-8 weeks for recovery. I got tired even standing waiting for food at the microwave. You will have a lot of things that make you very tired, quickly. I still do and I am 8 months post operation of my last surgery. Take your time, don't rush.

dbcb98

Posted by @dbcb98, Dec 12, 2016

My daughter is 17 and a senior in high school. She has suffered from headaches ever since she was little, but nothing compared to what she started suffering in October of this year. She was tired all the time (she is a night owl), so I knew something wasn't right. We went to the clinic and had lab work done, nothing odd showed up. In the middle of November, she experienced severe upper neck and shoulder pain, a nasty headache, blurred and double vision, dizziness upon standing, vomiting and ringing in her ear. I was worried about meningitis. She missed 2 weeks of school. We had plenty of visits to the chiropractor, 2 trips to the ER, a trip to ENT. The second trip to ER did the CT scan and discovered the Chiari Malformation. The dr. Did not seemed too concerned. We scheduled a MRI. The day before we went to eye dr., he dilated her eyes and told us it was good we had the scan the next day because there was swelling and it was pushing on her optic nerve...called papilledum. Fortunately, the MRI only showed Chiari Malformation and no tumors. The appointment with Neurology was moved up an entire month and we go tomorrow. I am hoping they will do more than just prescribe pain meds, she is too young for that and I don't want her to experience these headaches while in college. I am not sure how many, if any doctors in Fargo treat this condition so was glad to hear of your experience with Mayo. I will pray for continued healing for you.

brendak

Posted by @brendak, Dec 14, 2016

My daughter is having Chiari Decompression next week in Fargo with Dr.K.
My daughter was diagnosed at 14- she's 22 now. Initially the only symptoms she had were the headaches & surgery was deemed not necessary. In the last year she was sent to the chiropractor, physical therapy, and then to the eye dr.
He referred her to a specialist in Fargo.
The specialist sent her directly to the ER... as it turned out- it's her chiari that's causing her vision issues, balance & dexterity, dizziness & vocal changes.
Her neurologist is Dr. Brekken (sp?) at Sanford. Very personable young man.
Best wishes.

dbcb98

Posted by @dbcb98, Dec 15, 2016

Best of luck with her surgery, I will pray for your daughter. This journey sucks for such young people!!! We are currently using Essentia and Dr. Christenson. He does not seem to worried about the Chiari Malformation. He is looking more at the fluid in her head, which no one has told us about. So she is luckily enough to get the spinal tap (to measure the pressure), an appointment with opthamalogy to make sure there is no lasting damage to her vision and another MRI with contrast to show the veins. Is this normal protocol for this? Have all you you gone on this same path?

The dr. told me her Chiari isn't that bad and most of the time surgery causes more complications than it solves. I can understand that based on what I've read, but I don't know if he is an expert on the condition. My plan is once this is all completed to have our regular dr. send all results to Mayo and make sure we are on the right path.

brendak

Posted by @brendak, Dec 15, 2016

I'll pray for your daughter too!
My daughter doesn't have fluid in her head that I'm aware of...and she didn't need the spinal tap. I'm sorry, I don't know what normal protocol would be as this is my only experience with it.
My daughter was initially told that surgical procedures at the stage where she was diagnosed- was seldom helpful. Her symptoms are progressing to the point that it's not at option to forego the procedure now...but there are still no promises that it will help at all, or even if it more procedures will be required.
There's a young gal in Breckenridge that has had the procedure and it was a huge success for her though- so that's encouraging. I've read several horror stories- I would stay away from those...and focus on the positives.
Best of luck to you and your daughter. I know it's stressful for the family as well because we want so badly to help and fix things, and we can't.

brendak

Posted by @brendak, Tue, Jan 3 at 3:00pm CDT

My daughter had the surgery on the 23rd. It was much more than we thought. The surgeon estimated between 3-5 hours, but it took 7 hours. He knew before the surgery she would be in ICU, but not for 5 days. She was just transferred out of ICU to a regular room on Thursday last week. She's really had a time of it. She had a lot of issues with pain control, and getting out of bed has been a real wow factor. She's having physical therapy 3 hours a day. She can't see well enough to read or watch tv, and she's having trouble pee-ing so they had to cath her again. Think very carefully before having this procedure...it's not the in & out we were told. We were told she would be in the hospital for a couple days. I asked for the scripts for pain meds and antibiotics prior to surgery so I would have them on hand when she came home. He told us she wouldn't need anything. I'm not going to bash the surgeon, but I'm not dazzled or happy.

nikoleprins

Posted by @nikoleprins, Wed, Jan 4 at 7:52am CDT

I guess when I think Brain surgery I don't think of being in and out and I had enough prep by my other friends with Chiari and did enough research to know the possibilities of surgery. I had friends that told me this would probably be the hardest recovery I could think of. I had more than one surgery, but how was my surgeon supposed to know that would happen? I stayed 2 days the first time, 5 days the second and 5 days the third. I don't think he knew how much it would take. They aren't psychic. It's different for every patient. If she just had her surgery it will take a while to regain all of her past milestones. I don't think I could look at a TV without a headache for the first two-three weeks after surgery. The going to the bathroom thing, I'm pretty sure I had that same issue, I can't remember because my biggest deficit after surgery is memory loss. I would take blurred vision over memory loss anyday. I also have hearing problems.

I will send prayers your way for your daughter's recovery. This isn't an easy thing. The biggest thing I had to learn was to listen to my body, and learn how to deal with pain the best way I knew how. I'm almost a year post op from surgery number one, and the problems I was having back then and the ones I'm having now are much different.

It's a learning process for everyone. This surgery is not common enough to know how much it will impact the outcome for each patient.

brendak

Posted by @brendak, Wed, Jan 4 at 8:09am CDT

I certainly wasn't placing blame anywhere, but when he stated answers as nearly factual, we tend to lean our expectations in that direction. I would rather have heard, "I don't know..." as opposed to stats."
She's strong, she'll get through it.

brendak

Posted by @brendak, Tue, Jan 10 at 4:37am CDT

Just a quick update...after 17 days, she's home and doing very well. She'll need physical & occupational therapy but improving every day. She's strong and beautiful.

mistellelyn

Posted by @mistellelyn, Tue, Jan 3 at 12:43pm CDT

Hi everyone! I was recently diagnosed with Chiari, and am in the process of finding out what Stage I am considered. My appointment with the neurologist is on January 31. I am so grateful to have found this blog, it's scary to look up the diagnosis online, yet there aren't a lot of options to find out about it either! One questions I have that I don't think I have seen anyone remark about is ear pain. I was diagnosed after having an MRI to try to figure out the cause of ear pressure and pain I have been experiencing over the past few months. The Chiari diagnosis was NOT what I expected to hear!! Has anyone else had similar symptoms? Oh, that and my hands, nose, and sometimes my feet are cold, even if the rest of me is warm. So odd! I'd appreciate any insight. And thanks again to each of you for sharing your story!!

nikoleprins

Posted by @nikoleprins, Tue, Jan 3 at 12:46pm CDT

I guess I didn't get that because of the Chiari diagnosis, however I do also have Raynauds which causes the cold hands feet and sometimes ears and nose.
I do have the ear pain, but I never chalked that up to Chiari and none of my doctors had mentioned it being apart of the condition.

valreno

Posted by @valreno, Mon, Jan 9 at 10:52am CDT

hello everyone-my daughter was diagnosed with chiari one malformation on dec.19,2016 and boy has it been a ride. There have been so many suggestions on where take her but when you start researching, these ultra chic hospitals and doctors all have some flaw or malpratice pending. We live in a rural area and have 2 local hospitals neither very impressive by any means. But after reading some case studies, going further away from home may not be a good option if there is any complications a few days or weeks after surgery. If any of you have had similiar reactions your experience will be appreciated

brendak

Posted by @brendak, Tue, Jan 10 at 4:41am CDT

Chiari really is a trip! I'm not sure how old your daughter is or where you're from, but consider if you plan to stay in the same location as her procedure takes place, or if you'll go back home. Visiting and bringing items she needs or wants need to be a consideration in addition to the obvious of where she'll get the best care. When I wrote my post about not being happy, I think I was mostly scared because nothing was as we expected. Now that she's home, I see things differently. Best of luck.

brendak

Posted by @brendak, Tue, Feb 21 at 3:12am CDT

My daughter goes for her post op check up today. She had her procedure on 12/23/16. The procedure was 7 hours long, She spent several days in ICU before being transferred to a regular room. She was discharged on 1/9/17, with PT/OT three times a week. She will now finish up one more month of PT, and finally be able to get on with her life. She's hoping to go back to work and be able to drive after her appointment today. She only takes one med, once a day now, and can laugh without a major headache. She's seeing signs of success in other areas too.

brendak

Posted by @brendak, Wed, Feb 22 at 12:21pm CDT

We claim success with my daughter's procedure.

deebee123

Posted by @deebee123, Tue, Jan 10 at 8:06am CDT

Hello everyone. I am new to this site. My daughter was diagnosed with Chiari 1 a couple of years ago. She is now 15. All along we have been told that her daily severe headaches, and waking up vomiting at night had nothing to do with the Chiari because she only has a 5mm herniation. A radiologist completed a CINE MRI that looks at the CSF flow in the brain. It was discovered that she has crowding in the foramen magnum area with turbulence and disruption of her CSF fluid. We are awaiting a call from her neurosurgeon as I type this. Not sure where we will go from here but hoping that this an answer to why she has been having these debilitating symptoms. Has anyone else on here had a CINE MRI that showed this? Any advice/suggestions would be appreciated.

tillman1966

Posted by @tillman1966, Mon, Feb 20 at 9:00pm CDT

I was diagnosed about 7 years ago.i was in my forties.And seem I am having more and more problems.

vienna

Posted by @vienna, Wed, Feb 22 at 10:51am CDT

Hi -- Has anyone (or anyone's child) had other issues related to the Chiari, such as significant loss of appetite? My son (13) has a 4 mm Chiari with some disruption of CSF flow, and has lived with an unrelenting headache for almost 2 years now (pain level without exacerbation is 3 on 1-10 scale). It's worsened by movement -- going up stairs, bending over, lifting, running, riding in the car. Spine MRI was fine, but he does have significant scoliosis. Neurosurgeon recommends putting surgery on the list of options but waiting because of the risks and possibility it won't work (and reading these comments, this seems quite possible). What else can we do? The appetite issue is disconcerting because he has not gained any weight in 2 years.

nikoleprins

Posted by @nikoleprins, Wed, Feb 22 at 10:55am CDT

After my second surgery I lost 20 pounds because of the infection and not wanting to eat. The best thing I could have done was start by eating whatever sounded good. And for me my go to thing was carrots. They were the only bland thing my stomach could handle at one point. From there we upped the protein in my meals and carbs to help me gain weight. I ate buttered noodles because they were bland.
Otherwise there are protein shakes out there. It took me 8 months to gain my weight back. Prayers to you and your boy.

brendak

Posted by @brendak, Wed, Feb 22 at 12:20pm CDT

Prayers to you and your son. My daughter's nurse commented that nausea could last for up to 18 months after her procedure, but it seems to be getting better. She didn't experience this prior to surgery. What I'm trying to say is that, even after a surgical procedure, it may not appear to help until a long while afterwards.

amnie

Posted by @amnie, Sun, Mar 5 at 3:22pm CDT

My husband is being diagnosed with chiari malformation 1 of 18mm after having these annoying headaches for few years constantly. He Showed to two neurosurgeons in Singapore, we are resided in Maldives. And one doctor recommended surgery (Posterior fossa decompression surgery), and other one didn't, and basically told that he should learn to live with the pain. I am very confused of doing the surgery or not. He is back in Maldives, but we are planning to go for surgery this month 15 again to Singapore. But i am not sure surgery would help him reduce the pain or it will cause more symptoms. He have so many symptoms now also, forgetfulness/memory loss, severe headaches, nausea, dizziness, and a lot more. Can anybody recommend me with a good way to deal with this ?

dink75

Posted by @dink75, 3 days ago

Nikki I am so thankful that Mayo clinic sent me your link. I also had to have 3 surgeries. My 1st was for the initial repair and the bovine patch was used. The day I went in to have my staples removed I started leaking while I was in the waiting room. So I was wheeled in over to the hospital for the 2nd surgery. Then 6 weeks later another leak. So then surgery # 3. That was 3 in a matter of 4 months. I must admit that many of my symptoms have subsided since surgery and others have lessened in severity. At the time of my surgeries a shunt was not needed I was told. Well as time goes in I am suffering from severe headaches and other symptoms from severe inmer cranial pressure. At times spinal fluid will drain from my nose or ear. I kniw that is not normal. I also have severe vomiting with it. The headache is close to when the doctor had to insert a drain in my back after my 1st surgery becauae the 1 he put in during surgery was nit draining correctly. Anyway as soon as he punctured my spinal column I got severely ill immediately. So if anyone has ever indured a similar headache from an experience with the spinal area may understand. Thats the best way I can explain how it feels when my pressure headaches occur. Now not every one is that severe, but even the severe ones are almost intolerable. So I was wondering if anyone may have an opinion on where to go to have this address. I can out it off much longer because I have no quality of life. I have Lupus on topnof the Chiari. I went to UT here in Knoxville and I feel I nees to check out better options. Also does it souls like I may need a shunt or some other possible surgery to help with this? Sorry if I "babbled" on and for writing a novel. I am just excited to know I am not alone. Also agian thank you Nikki for sharing your story. Your my hero girl!

Please login or become a member to post a comment.

Contact Us · Privacy Policy