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

REPLY

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

REPLY

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?

REPLY

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.

REPLY

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.

REPLY

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.

REPLY

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.

REPLY

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.

REPLY

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.

REPLY

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.

REPLY

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.

REPLY

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.

REPLY
Please sign in or register to post a reply.
Contact Us · Privacy Policy