Breath-Holding Spells

Sat, 3 May 2008 17:11:29 -0600

Are neurally mediated syncope and breath-holding spells related? Do these spells have similar mechanisms as the cerebellar fits described in children with the Chiari I malformation?
To answer these questions, in a previous post we looked at NMS and in this post will look at breath-holding spells. Two types of breath-holding spells (BHS) have been described: cyanotic and pallid.
The cyanotic type, the more common of the two, is usually triggered by anger, frustration or, in some cases, a painful injury. The child may cry, overbreathe (hyperventilate), and then have a noiseless expiration. The skin becomes red or blue-purple and child stops breathing, looses consciousness, and becomes limp. Consciousness is regained in 1-2 minutes, and normal activity is resumed, although some will sleep for an hour or so.
The pallid type (also known as reflex anoxic seizure) is most commonly triggered by a painful event such as a blow to the head. There may be a single cry or none at all. The child becomes pale and looses consciousness. They may be drowsy or fatigued after the spell.
Both types of spells may be accompanied by brief muscle twitching (myoclonic jerks) or body stiffening. While often confused with a seizure, EEG’s performed after these spells have been normal.
In a review article (suggested in a comment by BJR), Dr. Robert Jeannette notes: “In some cases, a brief period of increased muscle tone, or opisthotonos, may be seen after or instead of limpness.” (Opisthotonos also occurs in cerebellar fits.)
Approximately 5% of children have breath-holding spells, usually between the ages of 6 months to 4 years. Boys and girls are equally affected.
Below is a description by a parent. Although the color of the skin is not mentioned, other features suggest a pallid type spell.

My three year old daughter stops breathing when she hurts herself badly.

The most recent episode was following a fall getting out of the bath when she caught her head on the shower screen. She did not cry, but just stared and held her breath.

I blew on her face, she gasped and then passed out. This lasted about 15 seconds (but felt longer) and then she recovered spontaneously.

She was a little nauseated and complaining of abdominal pain, having caught her tummy on the edge of the bath but she did not vomit.

Pathophysiology
Breath-holding spells have a physiological basis and are not due to emotional or behavior problems. A number of mechanisms have been proposed. Dr. Jeannette notes that:
...noxious stimuli may lead to centrally mediated cardiac inhibition through the vagus nerve. In turn, this mechanism may induce bradycardia or brief asystole and subsequent spells.
Similarly, cyanotic episodes may be caused by central inhibition of respiratory movements, again mediated through the vagus nerve. In both situations, cerebral hypoxia results.
Other suggested mechanisms include autonomic dysregulation, reduced sensitivity to hypoxia and hypercapnia, and abnormalities in pulmonary function. According to Dr. Jeannette, a “much less likely scenario” is the Arnold-Chiari malformation.
Recent Reports The recent study by Guilleminault et al. suggests abnormal nighttime breathing may be responsible for breath holding spells. Fourteen children with BHS were found to have sleep disordered breathing. Comprehensive evaluations revealed their upper-airways were narrow and their respiratory disturbance index was abnormal. The breath-holding spells disappeared following adenotonsillectomy.
An unusual case reported by Fujisawa et al. supports the brain stem as a site of origin. A 2-year-old girl underwent surgery for a tumor in the cervicomedullary junction (the area where the brain stem becomes the spinal cord):
Postoperatively, the function of the lower cranial nerves and cerebellum deteriorated and hemiparesis on the left became apparent, but she returned to the preoperative state in a few months. In addition, mild sleep apnea (Ondine curse) and severe cyanotic breath-holding spells occurred.

Treatment Dr. Jeannette recommends the following management:
It is most important to assure parents that although BHS are frightening to observe, they are benign and children will outgrow them.
Once an episode has resolved, the child should be reassured. However, drawing excessive attention to the event or expressing extreme worry to the child should be avoided.

A few cases may require treatment. Cases with iron deficiency anemia, though few, can be treated with iron supplements. Those with asystole or bradycardia can be treated with oral atropine. If severe and not responsive to atropine, a pacemaker may be required.
Di Pino et al. reported an infant with pallid breath-holding spells with frequent loss of consciousness. Cardiac monitoring revealed episodes of asystole lasting as long as 26 seconds. Implantation of a pacemaker resulted in complete resolution of symptoms. Wilson et al. reported a similar case.
The role of adenoidectomy and tonsillectomy, as mentioned above, is only recently being studied.
Conclusions BHS may be due to cardiac, airway, or pulmonary pathology. However, the most common mechanism appears to be nervous system dysfunction.
When trying to determine the frequency of CM-I in children with BHS, we need to consider that the spells frequently resolve as the child gets older, a finding that would not typically be expected if CM-I was responsible. As noted by Dr. Jeannette:
By the time patients are 4 years old, about half of breath-holding cases have spontaneously resolved; by age 6, about 90% have done so; and by age 7 or 8, virtually all have resolved.

However, apnea can occur in CM-I. Neurally mediated syncope, breath-holding spells, and cerebellar fits are likely to overlap to some degree. The question is, to what degree?

Next post in the series: Syncope in Chiari I.

John Oro’, MD
chiaritimes@mac.com

Copyright © 2007-2008 CyberMed, LLC

Neurally Mediated Syncope

Sun, 27 Apr 2008 18:57:37 -0600

In a comment on the Cerebellar Fits post, BJR raises the possibility of a relationship between breath holding spells, neurally mediated syncope, vasovagal syncope, reflex anoxic seizure and cerebellar fits. BJR asks an important question, are these “The same thing by another name?”

This post will review neurally mediated syncope. Subsequent posts will consider breath holding spells, reflex anoxic seizures and the possible overlap of these disorders with the Chiari I malformation.

Neurally Mediated Syncope
According to Dr. Zaqqa and Dr. Massumi of the Texas Heart Institute in Houston, Texas, neurally mediated syncope (NMS) “is a disorder of the autonomic regulation of postural tone, which results in hypotension, bradycardia, and loss of consciousness.” NMS is also know as neurocardiogenic syncope and vasovagal syncope.

The National Dysautonomia Research Foundation describes the physiological regulation of blood flow with standing as follows:

“The blood pressure control centers are located in the posterior parts of the brain (the brainstem or medulla). Every time a person stands, gravity pulls blood toward the lower extremities. The brain senses this change and compensates by increasing the heart rate and tightening (constricting) the blood vessels of the legs, forcing blood back upward to the brain. These centers in the brainstem (the autonomic centers), then work as a sort of thermostat to regulate blood pressure. “

If the thermostat is faulty, the blood pressure to falls “too low, causing the brain to lose its blood supply resulting in loss of consciousness (fainting).”

Zaqqa & Massumi explain it this way:

“The human body has a remarkable ability to maintain a stable blood pressure in the presence of ever-changing forces that constantly shift and redistribute the circulating blood volume. To achieve this steady control, reflex mechanisms continuously adjust the cardiac output and vascular tone. Even a simple change in posture, such as standing up, can result in a relatively “empty” ventricle owing to shifting of blood from the thorax to the abdomen and lower extremities. This shift in blood volume can markedly decrease the cardiac output.

While neurally mediated syncope can occur at any age, it is more frequent in adolescence. Knowing the triggers and warnings of NMS is important as we consider comparing it to CM-I. Zaqqa & Massumi note:

“The triggering factor for NMS varies widely and includes orthostatic stress, emotional stress, urination, coughing, swallowing, physical exercise, and stimulation of the carotid sinus in susceptible persons.”

“Typically, a patient with neurally mediated syncope experiences nausea, lightheadedness, a feeling of warmth, and pallor before abruptly losing consciousness.”

NMS may be prevented “by an increased fluid and salt intake and avoidance of triggering factors such as dehydration, extreme heat, alcohol consumption, and prolonged standing.” Persons with repeated spells are treated with beta blockers. Severe refractory cases may require a pacemaker.

Conclusions
The cause of neurally mediated syncope is dysfunction of the human body’s regulation of blood pressure. While orthostatic stress (assuming the upright position) is the most common trigger, coughing or emotional or physical stress can also trigger syncope.

However, a recently published MRI of a patient with Chiari I malformation shows increased herniation and brain stem compression in the upright position. Could some spells of neurally mediated syncope be due to the Chiari malformation? Cough can trigger syncope in patients with CM-I. Could some NMS patients with cough syncope have CM-I?

Next week, I will consider breath holding spells.

John Oro’, MD
chiaritimes@mac.com

Copyright © 2007-2008 CyberMed, LLC

Cerebellar Fits

Tue, 22 Apr 2008 19:30:11 -0600

There are a number of uncommon and rare presentations of the Chiari I malformation in children. Paroxysmal rage was discussed in the Jan 30 and Feb 2 posts. In this post, we will look at cerebellar fits.

In July 2001, Dr. Aditya Pandey and associates from Cleveland, Ohio published an article in Neurosurgical Focus reviewing 47 consecutive children with the Chiari I malformation that had surgical decompression from 1994 to 2000. As the authors note: “Patients with signs of acute brainstem dysfunction such as drop attacks, abnormal extensor posturing, and varying degrees of respiratory compromise were considered to have cerebellar fits.”

Thirteen of the 47 patients (28%), presented with cerebellar fits. Four children had a single episode while the remainder had 2 or more. Nine children had an EEG: 8 were normal and one showed some slowing. The mean tonsillar herniation in the 13 children with cerebellar fits was 8.7 mm. At surgery, “blanched discoloration of the tonsils or a deep indentation from herniation” was seen in 5 of the 13 children. The fits resolved in all 13 children after Chiari decompression.

The authors provide 3 illustrative cases:

A 2.5-year-old girl with spells of “tonic posturing, drop attacks, and apnea” also had complex partial seizures described as staring spells with lip smacking. Tonsils were pointed and herniated 10 mm; the tips of the tonsils were “extremely pale.” The drop attacks and apnea resolved after surgery although the complex partial seizures persisted. 
A 3-year-old boy “would stiffen and fall suddenly to the ground but not lose consciousness”. EEG, EKG, and Holter monitoring were normal. The drop attacks resolved after Chiari decompression. 
A 4-year-old boy presented with “episodes of extensor posturing with staring and apnea”. EEG was normal. The cerebellar fits resolved after surgical decompression.

The authors conclude their report as follows:

In Children with cerebellar fits and Chiari I malformation the presentation is an extreme manifestation of brainstem dysfunction. The most feared consequences of cerebellar fits in this population are respiratory arrest and sudden death. This outcome may be avoided by early diagnosis. Thus, it is important to remember that cerebellar fits in patients with Chiari I malformation can mimic other paroxysmal events such as epilepsy and cardiogenic syncope. The diagnosis of tonsillar herniation can be confirmed using MR imaging. The response of these patients to decompressive surgery is gratifying.

John Oro’, MD
chiaritimes@mac.com

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Full-Text Article
Pandey A, Robinson S, Cohen AR. Cerebellar fits in children with Chiari I malformation. Neurosurg Focus. 2001 Jul 15;11(1):E4.

Copyright © 2007-2008 CyberMed, LLC

Bobby Jones

Sun, 13 Apr 2008 17:05:27 -0600

Robert Tyre Jones was born in Atlanta, Georgia on March 17, 1902. He began playing golf at the age of 5 and at age 6 (photograph above) won the East Lake Children’s Tournament. By 14, he was the state amateur champion and at 15, the youngest-ever Southern Amateur champion, a tournament he would win for the next three years.

This was all warm up for a young man who would become the sports hero of his day. With his win at the US Open at age 21, he began a 7-year stretch of golfing dominance. His many titles culminated at the age of 29 in winning the Grand Slam of golf: the two leading titles in the US and Great Britain, an unequaled four major tournaments in one year. The fourth of these, the US Amateur, was played in Merion Cricket Club near Philadelphia:

For the 36-hole final, a large crowd (18,000) came to watch if Jones could make history against Gene Homans. Jones led by seven holes after the morning round, shooting a brilliant 33 on the back nine, and increased the lead to nine after four holes in the afternoon.
At the 11th hole (the 29th of the match), Jones closed out the match, 8-7. A contingent of U.S. Marines prevented America's latest hero from being swamped by hundreds of admiring fans. (ESPN)

Jones was active in other aspects of the sport. He advised golf course architect Dr. Alister Mackenzie on the design of the Augusta National golf course and established the Masters Tournament. With Spalding, he designed ”the first set of matched golf clubs to be mass produced”, the clubs were numbered instead of being referred to by the old Scottish names.

In 1948, at the age 46, Bobby Jones played his last Masters tournament. He was in “considerable pain” and had difficulty with his previously flawless swing due to weakness in his right arm and leg. Diagnosed with bone spurs at three cervical vertebrae, he underwent neck surgery later that year. However, the pain progressed to his left side and a second neck operation provided no benefit.

In 1956, he was diagnosed with syringomyelia at Columbia-Presbyterian Hospital in New York. With no treatment available, his neurological condition worsened and he had to use braces, crutches, and eventually a wheelchair.

In 1958 he was awarded the Freedom of the City award from St. Andrews. (The previous American recipient had been Benjamin Franklin just over 200 years previously.) Jones was honored as “...a man of outstanding character, courage, and accomplishment...”. When asked how he coped with his illness after being a singular sports figure, he simply said “Remember, we play the ball as it lies.”

Bobby Jones passed away in his sleep on December 18, 1971 at the age 69.

John Oro’, MD
chiaritimes@mac.com

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Learn More

Bobby Jones

Bobby Jones Society

Vocal Cord Paralysis

Sat, 5 Apr 2008 09:43:07 -0600

In response to a visitor’s comment on the 3/15/08 post regarding an episode of vocal cord paralysis in her 3-year-old daughter.

Vocal cord paralysis may be due to a variety of causes and is known to occur in persons with the Chiari I malformation. In 2002, Dr. Greenlee and colleagues at the University of Iowa published their experience in 31 children with the Chiari I malformation that were under the age of 6 (average age 3.3 years). As noted in their abstract:

“Chief presenting complaints included impaired oropharyngeal function (35%), scoliosis (23%), headache and neck pain (23%) sensory disturbance (6%), weakness (3%), and other (10%).”

On examination, vocal cord dysfunction was present in 26%, or 1 of 4 of the children. All of the children with vocal cord problems were under 3 years of age.

In 2003, Dr. Tubbs and colleagues at the University of Alabama and Duke University reported their findings on 130 pediatric patients with the Chiari I malformation. Hoarseness, a symptom of vocal cord dysfunction, was present 7 children, 5% of the group. This less frequent than in the Greenlee study. However, the patients in the Tubbs study were older: age ranged from 2 months to 20 years with an average age of 11 years.

In 2004, Wynn and Goldsmith from Downstate Medical Center at Brooklyn reported bilateral vocal cord paralysis in two adolescents with the Chiari I malformation. Other case studies in children are available in the literature through PubMed.

Summary
Children with the Chiari I malformation can present with vocal cord dysfunction and paralysis. While more common in children under 3 years of age, they occur in children of all ages.

Posted by John Oro’, MD
chiaritimes@mac.com

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References
Grundfast KM, Harley E.
Vocal cord paralysis.
Otolaryngol Clin North Am. 1989 Jun;22(3):569-97. Review.

Greenlee JD, Donovan KA, Hasan DM, Menezes AH.
Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years.
Pediatrics. 2002 Dec;110(6):1212-9.

Tubbs RS, McGirt MJ, Oakes WJ.
Surgical experience in 130 pediatric patients with Chiari I malformations.
J Neurosurg. 2003 Aug;99(2):291-6

Wynn R, Goldsmith AJ.
Chiari Type I malformation and upper airway obstruction in adolescents.
Int J Pediatr Otorhinolaryngol. 2004 May;68(5):607-11.

Copyright © 2007-2008 CyberMed, LLC

News & References

Sat, 29 Mar 2008 05:48:46 -0600

ASAP 2008 Annual Conference
The American Syringomyelia Alliance Project will hold its 2008 Annual Conference from July 23-26 in Arlington, VA. The meeting agenda has just been published.

CSF Announces Editorial Board
The new Chiari & Syringomyelia Foundation announces a distinguished Medical Research Board.

UK ChiariForums Launched
An new online Chiari community has been launched. The mission of Chiari Forums is to “offer forums for you to ask questions, chat with others with the condition and express your feelings about the condition and how it affects you and your life.” ChiariForums has been added to the Organizations section.

Miracle Wish for Chiari
Chiari family spearheads effort to launch camp for children with Chiari, syringomyelia, and other uncommon disorders. The goal is to provide a “fun-filled camp experience, but also a support system to help families cope with the many unexpected changes that chronic illness brings to the family unit.” MIracle Wish for Chiari has been added to the Organizations section.

February & March References
Lipson AC, Ellenbogen RG, Avellino AM.
Radiographic Formation and Progression of Cervical Syringomyelia in a Child with Untreated Chiari I Malformation.
Pediatr Neurosurg. 2008 Mar 20;44(3):221-223.

First case documenting an area of edema (hyperintensity) within the spinal cord developing into a syrinx in a child with CM-I.

Miralbés Terraza S, García Oguiza A, López Pisón J, Sáenz Moreno I, Jiménez Escobar V, Peña Segura JL, Marco Rived A.
[Central sleep apnea syndrome as the first sign of type I Chiari malformation.]
An Pediatr (Barc). 2008 Mar;68(3):277-81. Spanish.

Bertram CD, Bilston LE, Stoodley MA.
Tensile radial stress in the spinal cord related to arachnoiditis or tethering: a numerical model.
Med Biol Eng Comput. 2008 Mar 18.

Trauma or infection can cause scarring of the arachnoid membrane. The arachnoid scar can tug (cause traction) on spinal cord and lead to development of a syrinx.

Perrini P, Rawlinson A, Cowie RA, King AT.
Acute external hydrocephalus complicating craniocervical decompression for syringomyelia-Chiari I complex : Case report and review of the literature.
Neurosurg Rev. 2008 Mar 1.

The arachnoid membrane is frequently opened during a posterior fossa decompression for the Chiari I malformation - just as it is for any operation on the brain. Most often, spinal fluid stays in its space - inside the arachnoid. However, in a few rare cases, spinal fluid finds its way between the dura membrane and arachnoid membrane and becomes trapped. In this case, the collections were large enough to require treatment with a shunt.

Hayhurst C, Richards O, Zaki H, Findlay G, Pigott TJ.
Hindbrain decompression for Chiari - syringomyelia complex: an outcome analysis comparing surgical techniques.
Br J Neurosurg. 2008 Feb;22(1):86-91.

A retrospective study of patients treated from 1994 to 2005. While helpful, prospective studies are needed.

Wetjen NM, Heiss JD, Oldfield EH.
Time course of syringomyelia resolution following decompression of Chiari malformation Type I.
J Neurosurg Pediatrics. 2008 Feb;1(2):118-23.

Rusbridge C, Jeffery ND.
Pathophysiology and treatment of neuropathic pain associated with syringomyelia.
Vet J. 2008 Feb;175(2):164-72. Epub 2007 Feb 20.

Study in dogs with syringomyelia.

Ball JR, Little NS.
Chiari malformation, cervical disc prolapse and syringomyelia - always think twice.
J Clin Neurosci. 2008 Feb 5.

When significant spinal canal compression from a herniated disc is associated with syringomyelia, even in the presence of a Chiari I malformation, our approach has been to remove the disc first. Now, the first case report has been published showing resolution of the syrinx following spinal decompression without treatment of the Chiari malformation.

Bui CJ, Tubbs RS, Oakes WJ.
Tethered cord syndrome in children: a review.
Neurosurg Focus. 2007;23(2):1-9.

Posted by John Oro’, MD
chiaritimes@mac.com

Copyright © 2007-2008 CyberMed, LLC

Regarding Patient Inquiries

Mon, 24 Mar 2008 21:30:23 -0600

Chiari Times was created to increase awareness and understanding of the Chiari malformations and related disorders. Discussion is welcome. However, Chiari Times is not able to handle patient inquiries. For questions regarding someone affected with the Chiari malformation or syringomyelia, please contact one of the sites listed in the Organizations section.

Upright MRI; Pseudomeningocele

Fri, 21 Mar 2008 10:11:58 -0600

Upright MRI in Chiari & connective tissue disorder
You may have seen a recent new items on the use of upright MRI in the diagnosis of hypermobility and cranial settling in Chiari I patients with connective tissue disorders. While this is a very important observation, currently only one case using upright MRI has been published.

In the December 2007 issue of The Journal of Neurosurgery: Spine, Dr. Thomas Millhorat and colleagues performed detailed measurements of the cranio-cervical junction (CCJ) in patients with the Chiari I malformation. Of their database of 2813 CM-I patients, 357 patients had connective tissue disorders that can weaken the ligaments. These patients were taken to the operating room for cranial tong traction performed under sedation. Detailed measurements of the cranio-cervical junction were made before and after traction. The traction test revealed changes in the distances and angles at CCJ in patients with connective tissue disorders.

Toward the end of their study, the authors chose to use standing MRI to measure the CCJ in the supine (lying down) and sitting position. As noted in the images above, they found evidence of cranial settling and gliding of the joints between the skull and cervical 1 vertebrae. The scans show that the tonsils are up in the supine position and droop down into the upper spinal canal in the sitting position.

The findings in this study a very important and the authors are to be congratulated. We look forward to more experience in use of the upright MRI in certain CM-I cases.

Pseudomeningocele after decompression
In the February 10 post, I discussed the development of spinal fluid collection (pseudomeningocele) after surgery for CM-I. I consider a pseudomeningocele to be a clinically significant spinal-fluid collection developing outside of the dura membrane. It may cause pain, compression of the dural patch, crowding of the area of decompression, or spinal fluid leak through the skin.

We calculate the risks of surgery over the previous 100 cases. In the Feb. 10 post, I noted that our incidence of clinically significant fluid collection (pseudomenigocele) was 1% over the previous 100 cases. As of this writing, the risk is 2%.

One of our recent patients hit the top of her head on a shelf 5 weeks after surgery. There was no loss of consciousness, however, the blow was enough to “take her breath away”. Three to 4 days later she developed flu-like symptoms with coughing and gaging and noted return of headache and development of swelling of the wound.

An MRI revealed a pseudomeningocele. She was taken to surgery where spinal fluid was noted to be oozing through a small, few-millimeter, area at the superior suture line. The area was oversewn and a couple of days later she was discharged home doing well.

John Oro’, MD
chiaritimes@mac.com

Copyright © 2007-2008 CyberMed, LLC

The Chiari Book

Sat, 15 Mar 2008 15:42:58 -0600

Diane Mueller and I are pleased to announce the publication of The Chiari Book: A Guide for Patients, Families, and Health Care Providers. The book was written to increase awareness of the Chiari I malformation and syringomyelia and offer hope to those affected.

The Chiari Book is based on the experience gained in evaluating over 1,200 persons for the Chiari I malformation and syringomyelia. In 1998, Diane Mueller, ND, NP and I developed a specialized clinic at the University of Missouri Health Sciences Center in Columbia for the care of patients with these disorders. Since 2005, my experience continues at The Chiari Care Center in Aurora, Colorado.

Illustrated with drawings and photographs, the book is written in a manner that we hope is understandable. Its 96 pages are organized into 10 chapters and 3 appendices:

1. Introduction
2. A brief review of Neuroanatomy
3. The Chiari Malformations
4. Syringomyelia
5. Symptoms of Chiari malformation
6. Other illnesses that can mimic Chiari malformation
7. The diagnosis of Chiari malformation and syringomyelia
8. Treatment options for Chiari malformation and syringomyelia
9. Special considerations in Chiari (children and adolescents, pregnancy, elderly)
10. Summary
APPENDICES:
Chiari and Syringomyelia Articles
Glossary
Internet Resources

The book is available at Amazon.com at this link: The Chiari Book

Your comments are welcome. Let us know how we can improve a future edition.

John Oro’, MD
chiaritimes@mac.com

Chiari Related to Tight Filum Terminale?

Sun, 9 Mar 2008 22:19:01 -0600

The leading theory for the occurrence of the Chiari I malformation is underdevelopment of the posterior fossa: a small (hypoplastic) posterior fossa does not provide enough room for the lower part of the cerebellum, cerebellar tonsils, and brainstem. Treatment by posterior fossa decompression is effective for many.

However, could some cases of CM-I be due to a tethered spinal cord? What about syringomyelia and scoliosis?

In 2005, Dr. Miguel Royo Salvador, Director of the Barcelona Neurological Institute, and associates reported their results following treatment of scoliosis, syringomyelia, and Chiari I malformation by section of the filum terminale. The records of 20 patients treated between April 1993 and July 2003 were reviewed. The patients presented with scoliosis, syringomyelia, the Chiari I malformation, or “a combination of these conditions”.

The position of the conus medullaris was reported in 15 patients:

Level Patients
T12 1
L1 9
L1-L2 1
L2 1
L5-S1 3

Each patient was treated by sectioning (cutting) the filum terminale in the sacrum, with or without opening of the dura.

Outcome
The outcome is summarized as follows:

“...patients with syringomyelia showed an early clinical improvement of dysaesthesia, thermo-anaesthesia, hypo-aesthesia and walking difficulties. Rising of the medullary conus was also observed. In patients with scoliosis, back pain improved dramatically and a curve reduction was noticed, although progression of the curve was observed in one case. In patients with Chiari malformation, headache, dysaesthesia and paraparesis disappeared.”

A review of article’s Table 1 reveals:

Scoliosis: There were 8 patients with only scoliosis. The change in the curve was measured in three patients. It decreased in two (6 degrees, 8 degrees) and increased in one (4 degrees). Outcome in 6 patients ranged from 10-90% improvement. Outcome was not available in two.

Syringomyelia: There were 5 patients with only syringomyelia. Outcome was favorable: 40%, 90%, 95%, 100% improvement in 4 patients respectively. One was lost to follow-up. The syrinx cavity decreased in length and increased in width in 2 patients - an insignificant change - and was unchanged in the others.

Chiari I malformation: There were 4 patients with the Chiari I malformation. Of the two patients with only CM-I, one had “50% clinical improvement” and the other had “100% clinical improvement.” Of the two patients with CM-I, syringomyelia, and scoliosis, one had “40% clinical improvement” and the other was lost to follow-up.

Impressions
While the results suggest a possible new treatment approach, it is important to note the limitations of this pioneering study:

As a retrospective study based on review of patient records, some important information is not available.
The number of cases for each of these conditions is very small.
Change in spinal curve reported in only 3 patients.
Outcome information is available in only 3 patients with the Chiari I malformation.
The extent of tonsillar herniation in the CM-I patients is not reported.
Three patients were lost to follow-up.

Although the study is encouraging, caution is in order. As with any medical advance, the role of sectioning of the filum terminale in patients with scoliosis, syringomyelia, and the Chiari I malformation will depend on whether the results are confirmed by other investigators.

Comments and observations welcome.

Posted by John Oro’, MD
chiaritimes@mac.com

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Reference
Royo-Salvador MB, Sole-Llenas J, Domenech JM, Gonzalez-Adrio R. Results of the section of the filum terminale in 20 patients with syringomyelia, scoliosis and Chiari malformation. Acta Neurochir (Wien). 2005 May;147(5):515-23; discussion 523

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