[Shelton]

#11885

Hi doctor,

I had a decompression for Chiari malformation about a year ago and the surgery made me worse. I ended up getting a second opinion and being diagnosed with Klippel Fiel Syndrome and I am fused from skull to C2. KFS was causing clivo-axial instability and severe brain stem compression so I had another surgery fixing my alignment and fusing me from skull to C3 with my rib bone. I got a lot better but I’m becoming symptomatic again and my surgeon says the KFS is still causing some anterior brain stem compression and turbulent CSF flow. I have breathing difficulty and some heart rate issues. I also have a hemivertebrae as C4 causing leviscolosis, mild stenosis at C5-C6. I have just started diamox for head pressure and enlarged ventricles and am symptomatic for tethered cord. Do all these things go together? Is there anything else that can be done for the anterior brain stem compression? Do you accept MRIs without an appointment? I live in VA but I would like your opinion. I’m not sure what to do from here and none of my doctors have a clue except for my surgeon who I see next month.

Symptoms include:
High heart rate
Chest tightness
Back pain
Leg pain
Urinary frequency
Breathing difficulty
Headaches
Shoulder pain
Fatigue
Hyperreflexive in legs & knees buckle
Brain fog
Gait problems

Thanks in advance.

[Donald Corenman, MD, DC]

#11886

Chiari malformation is relative compression of the brain stem due to congestion at the foramen magnum. I assume the initial surgery you underwent was an opening of the posterior ring of the foramen magnum and a laminectomy of the C1 posterior arch. You probably did not improve due to instability (either present before the surgery or induced by the surgery).

Klippel Feil syndrome is a congenital fusion of at least two cervical vertebra. This would have been present (and obvious) before the first surgery and should by itself not have affected the results of the first surgery. You then underwent a fusion due probably to the instability. You have more than Klippel Feil syndrome if you have a hemivertebra at C4. This is a congenital malformation as half of the vertebral body did not form. There are commonly more malformations present and your CT scan needs to be studied further so no other surprises “pop-up”.

Breathing difficulties could be from the injury to your brain stem or from injury to your phrenic nerve (C3-5) which supplies your diaphragm.

Tethered cord is another problem. The end of the spinal cord is called the conus. Attached to the conus is the filum terminale, a thin structure that attaches to the sacrum and normally is of no consequence in life. In some individuals, this structure thickens and pulls the spinal cord lower (the end of the spinal cord normally terminates behind the body of L1). This is a tethered cord and contributes to the Chiari malformation.

You need a world expert to be able to understand the complete diagnosis and what is needed to help reduce your symptoms. I understand this disorder but do not generally treat it. Do some research on physicians who have written many papers on this subject as these individuals should have the most experience with this disorder.

Dr. Corenman

PLEASE REMEMBER, THIS FORUM IS MEANT TO PROVIDE GENERAL INFORMATION ON SPINE ANATOMY, CONDITIONS AND TREATMENTS. TO GET AN ACCURATE DIAGNOSIS, YOU MUST VISIT A QUALIFIED PROFESSIONAL IN PERSON.
 
Donald Corenman, MD, DC is a highly-regarded spine surgeon, considered an expert in the area of neck and back pain. Trained as both a Medical Doctor and Doctor of Chiropractic, Dr. Corenman earned academic appointments as Clinical Assistant Professor and Assistant Professor of Orthopaedic Surgery at the University of Colorado Health Sciences Center, and his research on spine surgery and rehabilitation has resulted in the publication of multiple peer-reviewed articles and two books.

[Shelton]

#11894

Thank you so much for providing your knowledgeable insight.

You are right about my surgical past. I had a 13 mm Chiari malformation, full CSF blockage, and instability from the beginning. The instability was not recognized by my first surgeon. I am now seeing a knowledge neurosurgeon at Georgetown University in DC on all things Chiari. I am concerned that I may need to see a more knowledgeable neurologist and/or surgeon to make sure I’m not missing anything. Do you have any recommendations of an expert you mentioned? I have done tons of research but I haven’t seen many who would fully understand. I have heard of Dr Lebl in New York and have thought of getting his opinion but I’m not sure where to go from here.

What could be the cause of the hemivertebrae at C4? I have researched that it can just go along with Klippel Feil Syndrome and haven’t thought much about it. Is the hemivertebrae a problem that needs to be fixed surgically? What do you mean by “you have more than just KFS because of the hemivertebrae?”

I am getting full spine MRIs soon. If the lumbar shows tethered cord, can the pulling cause autonomic issues since it can pull on spinal cord?

I have listed both post op and pre op results as the surgical fusion does not allow you to see the full results. This is what my MRIs of my neck show:

Surgical Fusion from occipital to C4 level with screws intact
Turbulent CSF flow below cerebellum & distortion of foraminal anatomy related to vertebral anomalies
Mutiple congenital segmentation anomalies
Right C1 is incorporated into the occipital of atlas
C1 : congenital arch
C2- C3 : levels are partially fused across the disc spaces and posterior elements with only rudimentary discs
C2 : level is quite hypoplastic
C4 : has a left hemivertebrae morphology with related levoscolosis
Mild end plate hypertrophic changes at C4-C5
C5-C6: uncovertebral osteophytic spurring with mild diffuse osteophyte complex; mild to moderate left foraminal stenosis (all developing after fusion surgery)
C6- C7: diffuse disc osteophyte complex with uncovertebral osteophytic spurring without significant stenosis; mild left foraminal narrowing is present (developing after fusion surgery)

Could any of this cause compression of the phrenic nerve like you mentioned? The breathing issues may be from the brainstem compression, I just wonder because they got better at first during recovery and now they have worsened again. Also, many of the problems are noted as being on the left side.

Thanks so much for your help.

Hannah

[Donald Corenman, MD, DC]

#11909

Congenital malformations of the spine (which include hemivertebra and Klippel Feil Syndrome) are generally a genetic predisposition although some can occur in utero if there is exposure to some toxic event.

You had a congenital fusion of C1 (“Right C1 is incorporated into the occipital of atlas”). This generally shortens the spine and can allow the brain stem to descend into the spine through the foramen magnum which is appears to be your disorder. The cerebellar tonsils (small orb shaped brain parts that sit on each the side of the cerebellum) then “jam” into the foramen magnum and “choke” the brain stem.

The phrenic nerve consists of nerve roots of C3 to C5. This means that there has to be foraminal stenosis at more than one level in the upper cervical spine which does not appear to be the case with you.

Dr. Corenman

PLEASE REMEMBER, THIS FORUM IS MEANT TO PROVIDE GENERAL INFORMATION ON SPINE ANATOMY, CONDITIONS AND TREATMENTS. TO GET AN ACCURATE DIAGNOSIS, YOU MUST VISIT A QUALIFIED PROFESSIONAL IN PERSON.
 
Donald Corenman, MD, DC is a highly-regarded spine surgeon, considered an expert in the area of neck and back pain. Trained as both a Medical Doctor and Doctor of Chiropractic, Dr. Corenman earned academic appointments as Clinical Assistant Professor and Assistant Professor of Orthopaedic Surgery at the University of Colorado Health Sciences Center, and his research on spine surgery and rehabilitation has resulted in the publication of multiple peer-reviewed articles and two books.

[Shelton]

#12099

Thank you very much for your opinion and insight.

I did end up getting a lumbar MRI to check for tethered cord and wondered if you could give an explanation of what the MRIs mean :

“There is transitional lumbosacral anatomy. The transitional segment is considered to represent S1 based on the presence of iliolumbar ligaments at the segment above the transitional segment. There is a complete L5-S1 disc & a rudimentary S1-S2 disc.

T12-L1: Small central disc herniation with cephaladad migration beneath the posterior longitudinal ligament, exerting mild mass effect on the ventral conus.

The tip of the conus lies at the inferior L2 level and there is lipomatous infiltration of the filum terminale. “

(Everything else is unremarkable.)

I am wondering what to do from here and what all of that means?

Thanks in advance.

[Donald Corenman, MD, DC]

#12100

You have “transitional anatomy which is to be expected based upon your cervical congenital fusion. It is difficult to know if you have a tethered cord. Typically the spinal cord ends behind the body of L1 and yours ends lower (behind the body of L2). This could be a sign of tethered cord but then you have transitional anatomy which could throw off the correct count (could L2 in your case really be L1)

You have filum lipomatosis (“lipomatous infiltration of the filum terminale”) which can occasionally go along with tethered cord. You have a disc herniation which is “exerting mild mass effect on the ventral conus”. The conus is responsible for bowel and bladder function. Is that normal?

The information may or may not indicate some cord abnormality. It is hard to say from this distance.

Dr. Corenman

PLEASE REMEMBER, THIS FORUM IS MEANT TO PROVIDE GENERAL INFORMATION ON SPINE ANATOMY, CONDITIONS AND TREATMENTS. TO GET AN ACCURATE DIAGNOSIS, YOU MUST VISIT A QUALIFIED PROFESSIONAL IN PERSON.
 
Donald Corenman, MD, DC is a highly-regarded spine surgeon, considered an expert in the area of neck and back pain. Trained as both a Medical Doctor and Doctor of Chiropractic, Dr. Corenman earned academic appointments as Clinical Assistant Professor and Assistant Professor of Orthopaedic Surgery at the University of Colorado Health Sciences Center, and his research on spine surgery and rehabilitation has resulted in the publication of multiple peer-reviewed articles and two books.

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