(This post contributed by Amy- a friend of Huntar and Barb)
This post is essentially a compilation of information received by the family from the medical professionals who have reviewed the case.
This first section is taken from a letter to Barb from a doctor at the Univeristy of Minnesota Medical Center- Fairview, dated March 4, 2010.
"I had the opportunity to review Huntar's MRI today. He has two conditions going on. He has a congenitally dislocated spine, where one of the vertebrae has slipped off the other one, and he has something called a tethered spinal cord. I have shown you on the MRI today where he has this tight string that is pulling down on his spinal cord. This makes the surgery probably a little more urgent to do sooner rather than later. We don't have to do this today or tomorrow, we don't have to do it next week, or next month, but we should probably do it before the summer time.
The surgery that he needs is several-fold. It involves going in and putting screws and rods and then taking out one of the dislocated vertebrae and realigning his spine. In addition, Dr........., from Neurosurgery will need to open up the spinal sac and release the tethered spinal cord. This surgery will probably take 6-8 hours. He will lose some blood and will need a blood transfusion. He will then be intubated overnight at least and in the pediatric ICU for somewhere in the range of one to three day range. He will need to be flat in bed for that time period, and if I am happy with the fixation we will not have to put him in a cast. We may or may not put him in a removeable brace. I would expect he would then go over to the Shriner's hospital somehwere between three and five days after the surgery and may need to stay perhaps as long as a week but perhaps as short as just a couple of days. "
The letter then goes on to discuss the risks that are involved with the surgery and what the follow up plan would be..... and then it states:
"Also, I want you to know that I have looked at this case and sent it around to one or two of my very trusted colleagues across the country. Everyone recognizes that this is a very unusual condition and everyone generally agrees with the concept that he does need it to be fixed. Everyone is sort of scratching their heads and saying 'well, I might do it this way' or 'well, I might do it that way', but everyone agrees with the general overall approach, and this was before we knew that he had the tethered spinal cord, which adds a little more impetus to the plan of going ahead and fixing it."
This next section comes from a letter dated March 3, 2010 that is correspondence between a doctor at Fairview and the Shriner's Hospital:
"I had the opportunity to see Huntar's MRI and to briefly discuss it with Dr.......... from Neurosurgery. Huntar has a tethered cord. It is interesting in several aspects of trying to figure out how to prove it is tethered because of teh congenital dislocation of his spine. Trying to determine if the conus medullaris is below the mid body of L2 is a little bit difficult, however, he has very tight- appearing fillum that appears to be dorsally stuck over approximately the S2 region consistent with a tethered cord.
I think this changes the already complex nature of the surgery. I think this will require a combined orthopedic and neurosurgical event. I would expect that what we would do would be to expose the posterior elements of his spine, place screws in a couple of levels above and below, perhaps even pelvic fixation, identify the vertebral body to be excised, probably cannulate those pedicle tracks with K-wires and then allow neurosurgery to do the de-tethering. Then, we would proceed with the resection of the dislocated posterior element which I will choose to call L4 and try to reduce L3 onto L5 with fixation above and below.
The biggest change in this game plan is going to be the issue of CSF leak which will be a potential challenge to deal with, and we will have to discuss with Dr......... as to whether or not this would merit a prophylactic upper lumbar drain which may be prudent to do although keeping the child flat in bed will also be challenging. I would also consider potentially a postoperative orthosis of some kind on him to just try to protect everything given the nature of this, perhaps a clamshell with a cutout over the incision by be prudent."
This next section is taken from a report generated by the Orthopedic -Riverside- University of MN Med Center from March 3, 2010:
"CHEIF COMPLANT/REASON FOR VISIT: Huntar Gustafson is a 16-month old male who presents for evaluation of a congenital spine anomaly.
HISTORY OF PRESENT ILLNESS: Huntar Gustafson is accompanied by his mother in clinic today to evaluate a congenital lumbar dislocation at the L4 to L5 level. They had already seen Dr. ...... at Shriner's hospital in St. Louis and have seen Dr........ at the University of Minnesota in consultation for this problem. Huntar is the product of a full term, 39-week gestation and was delivered by C-section. There were no reported complications of the pregnancy. The child has been meeting all of his normal milestones but routine screening of his pediatrician detected a lumbosacral anomaly which was evaluated with imaging studies. The child is still in diapers and does not have voluntary control of his bowel and bladder. He was also recently diagnosed with hemihypertrophy or hemihyperplasia, with a slightly longer right arm than leg, which also contributes to his problems in ambulation. The child is able to pull himself to his feet and to stand unsupported and bear weight. He holds his right leg in an externally rotated positon and is able to walk with some assistance. Otherwise he gets around by crawling or walking on his hands in a bent forward posture. There are no other known congenital anomalies.
PAST MEDICAL HISTORY:
1. Lumbosacral dislocation at L4-L5
2. Hemihyperplasia Syndrome
3. Undescended left testicle
4. Congenital left dislocation of hip."
"REVIEW OF SYSTEMS: As noted in the History of Present Illness, the patient has no voluntary bowel or bladder control. Otherwise he has met most milestones in terms of his cognitive and motor development. "
"IMAGING: Imaging studies are reviewed, and MRI of the lumbar spine as well as a CT of the lumbar spine. There is clearly a spinal dislocation at the L4-L5 level. The conus appears to be at the level of L2. There may be some distal tethering of the filum, although it is unclear if this is significant or not.
ASSESSMENT: Huntar Gustafson is a 16-month old with congenital dislocation of the lumbosacral spine, hemihypertrophy, and an undescended left testis. Otherwise, he is in good health. He presents for elective correction of his spinal deformity. As noted above, he appears to have met most of the expected milestones and has good control of his proximal as well as distal motor function. It is not possible to assess his bowel and bladder function at this time."