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

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.

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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 ?

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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!

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Did anyone receive a diagnosis other than Chiari at first? I was recently diagnosed with Occipital Neuralgia and the treatments that I have had are not working. I am concerned that there is something else going on. I am waiting for Mayo to call back for scheduling.

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This article has helped me understand more about what I am looking at facing. I was diagnosed with Chiari Malformation 1 yesterday. My symptoms are headaches, numbness in my hands and feet, extreme back pain, issues with depth perception when driving and joint and muscle pain. Doctors are saying surgery. I guess my question is….at this stage am I better off living with the pain over having such a risky surgery. Also, doctors here in oklahoma are trying to get me in to see a doctor from Baylor or Mayo Clinic. Does anyone have a preference on best place to go?

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mnorthcutt …. we are located in Tulsa. After 2 1/2 years of misread MRI’s and a top rated neurosurgeon (connected with St Francis) telling my wife that her symptoms were female related, we finally found a local Mayo trained Neurologist that diagnosed her in less than 5 min. Options were Mayo, stay local, Cleveland Clinic or Baylor. We chose Mayo and were very pleased with her surgeon and the Mayo. Neurology follow up for some recurring symptoms has not met our expectations. But I think it is the exception and if we had to go through it again we would still pick Mayo. Jim

COMMENT

This is helpful. I have libed with pain so long to have a diagnosis in a day is what has me so worried. I have not read good thinga about the surgery. At all. I have rwad of many people not beimg able to walk after the surgery. Right now my worst symtoms are arm and joint pain. Numbness in my extremities and tiredness and weakness. My question is what stage of the game am I in amd hopefully whomever I see can answer that for me. I am young and I have two small children. I want to be as healthy as i can for them.

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Nikki I read in your post that you had some ear problems with your Chiari. I have horrible ear problems. I have a Chiari I. Could you explain to me what your ear problems were like? I appreciate your help so much! Also if there’s anybody on the site that can help me with that question I would appreciate that so much too!!!!!
Also I have many symptoms associated with the Chiari as all of you too but my most horrific symptoms have been with my ears

COMMENT

I was diagnosed with Arnold Chiari 1 in 2011. i seen a specialist after i got the MRI back . They said it wasn't bad enough for them to treat. I was having problems with Headaches, balance, tension headaches, and bad anxiety. So now several years later my headaches have progressed to the point of missing tons of work. In the meantime diagnosed with Fibromyalgia and Migraines . I got a Ct scan and it was all clear and i have an MRI scheduled tomorrow to see if that shows any progression of the Chiari. I am terrified I'm 46 years old i don't want a brain surgery with no guarantee of any results . Keep your fingers crossed for me .

COMMENT

After decades, I was finally diagnosed with Chiari 1. I have an HMO and they don’t recommend surgery because the excruciating daily headaches don’t seem to be brought in or worsen by activity. I live in the Los Angeles area and there don’t appear to be support groups available. Many of the Chiari support group have outdated information. Because if the distance and the type of insurance I have, I am wondering if the Mayo Clinic is out of the question for a 2nd opinion.

COMMENT

Thank you for writing. Unfortunately, we cannot diagnose conditions, provide second opinions or make specific treatment recommendations through this website. If you would like to seek help from Mayo Clinic, please call one of our appointment offices (Arizona: 480-301-1735 Florida: 904-953-0853 Minnesota: 507-284-2511) or visit https://www.mayoclinic.org/appointments.

You might also consider looking into our Mayo Clinic Connect website (http://connect.mayoclinic.org), where you can communicate with others who may have had similar experiences. You can also read Mayo Clinic expert blogs and take part in educational events.

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