Sharing Mayo Clinic

Stories from patients, family, friends and Mayo Clinic staff

October 17, 2016

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

By 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

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.

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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.

Liked by Sbenson

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Look into Idiopathic Intracranial Hypertension or other times called Pseudo tumor cerebri. This increased pressure can at times cause a Chiari Malformation, or visa versa Teally they haven’t discovered which comes first. Hugs Best of luck to you!

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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!!

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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.

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I went to an ENT for ear pain and hearing loss. He did a hearing test and ended up putting tubes in to release pressure and he is the one who ordered the MRI. He found the Chiari. So thankful that he was believing in me. As my family doctor had been telling me for a few years it was just allergies. I also have raynauds

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My fingers, face and nose are always colds. I have tons of pressure above my ears , like where my jaw muscles are .

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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

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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.

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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.

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We claim success with my daughter’s procedure.

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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.

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

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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.

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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.

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