I am currently in Arlington, Virginia at the 20th Annual Medical Conference of the American Syringomyelia Alliance Project. ASAP, established in 1988 by Don and Barbara White, has provided support to thousands of people with the Chiari malformations and/or syringomyelia.
The meeting is hosted by Dr. John Heiss, Head of the Surgical Neurology Branch at NINDS, NIH, Bethesda, Maryland. Among today’s presentations:
Chiari & Syringomyelia: Introduction and History of Treatment Methods
W. Jerry Oakes, MD
Professor of Pediatric Neurosurgery
University of Alabama at Birmingham Medical School
Dr. Oakes introduced the Chiari malformation and syringomyelia. He focused on the need to avoid treatment based solely on the findings seen on MRI. The important, and sometimes difficult part, is to determine whether the patients symptoms are due to cerebellar tonsil herniation sometimes found on MRI. Treat based on symptoms, not just on radiology.
Two out of three of the children Dr. Oakes treats for the Chiari malformation also have a syringomyelia. The syrinx is an “objective” finding, versus headache which is difficult to measure and is viewed as a “subjective” finding.
About 15% of the children had other, frequently unique, presentations. One example was a child who would not cry. The reason: if the child cried, the head and neck would arch back due to marked pain from a tight Chiari I malformation. Thus, the child learned not to cry. Another child with CM-I child would not have bowel movements since straining caused severe pain.
Dr. Oakes reported that 8-10% of children with CM-Ialso had hydrocephalus.
He then discussed the outcome following posterior fossa decompression. In children with syringomyelia, the syrinx resolved or improved in 96% within 8 months following surgery. This decreased to 84% at 7 years.
He does not believe the tethered cord causes syringomyelia and would not treat syringomyelia by section of the filum terminale. (More on this controversial issue in future posts. Also, look at Chiari related to tight filum terminale? )
Finally, it was Dr. Iskandar, Dr. Oakes, and colleagues who in 1998 defined Chiari 0. They reported 5 children with syringomyelia but without the classically described CM-I who were nevertheless treated with a posterior fossa decompression. All five children improved.
Dr. Oakes sticks to his definition of the Chiari O malformation: minimal tonsillar descent in a person with syringomyelia. (See Defining Chiari )
Pain Management in Patients with Chiari I and Syringomyelia
Dr. Ann M. Berger, MSN, MD
Head of the Pain Service
NIH Clinical Center, Bethesda, MD
Dr. Berger started with a description of the three types of pain. Somatic pain - pain from the extremities, trunk, and head - is due to activation of nociceptors (pain receptors). These receptors are activated by a stimulus such as inflammation or injury. Visceral pain, often vague and diffuse, is also due to activation of nociceptors. However, neuropathic pain is different. It is due to damage or destruction of the nerve fibers themselves and is usually more difficult to treat.
The pain team at the NIH Clinical Center manages pain using a broad multidisciplinary approach which includes pharmacological treatment, mind-body therapies, and a focus on psychosocial issues and the whole family unit.
Pharmacological treatment depends on the severity of pain. Mild pain, not frequently seen at the Center, is treated with aspirin, tylenol, ibuprofen, naproxen, or similar over-the-counter medications. Moderate pain is treated with opioid (narcotic) medications. Severe pain is treated with long-acting opioids together with short acting opioids for breakthrough pain.
(Dr. Berger reminds us not to forget treatment of constipation in patients on opioid medications.)
She then discussed tolerance, physical dependence, addiction, and pseudo-addiction. Tolerance is the physiological adaptation to opioids that results in a need to increase the dose in order to achieve the same effect over time. Physical dependence describes the changes in the nervous system that occur after prolonged opioid treatment. Sudden withdrawal call lead to withdrawal symptoms.
Dr. Berger notes that addiction, a psychological preoccupation with acquisition of pain medications, occurs in fewer than 0.1% of those on acute/chronic pain medications. She believes more often persons may show a pseudo-addiction: they appear to have addictive behavior but, in reality, are not be getting enough medication to treat their pain. (If anyone has any thoughts or experience on distinguishing these two groups, please add a comment).
Dr. Berger then spoke on the management of neuropathic pain, the type of pain most often suffered by those with syringomyelia. In addition to using analgesics, it is important to use adjuvant medications such as tricyclic antidepressants and selected anticonvulsants. A few patients with more refractory pain may benefit from Clonidine, corticosteroids, local anesthetics such as Mexilitine, or medications such as Ketamine or Baclofen.
In addition to pharmacological treatment, the NIH Pain Service focuses on the social, emotional, spiritual and psychological issues involved in pain. Their multidisciplinary team utilizes a broad range of mind-body therapies including:
Massage - particularly good for myofascial pain
Electro therapeutic point stimulators and TENS
Relaxation - meditation, guided imagery, breathing and others
Biofeedback - may be better for computer oriented people
Hypnosis - a specialized practitioner can train the patient in self-hypnosis
Yoga - effective for mind body and spirit
Acupuncture - “very helpful”
Reiki - “universal energy healing system” for its calming effect
Finger Labyrinth - finger traces path; calming effect
Mandala
Pet Therapy
Art Therapy
Music Therapy
Dr. Berger and her colleagues try to select the right mind-body therapy for each patient. Sometimes trying a series of therapies in order to find the most effective.
A final, and important point, is their emphasis on looking at the
whole family unit. They try to identify factors that could aggravate or maintain the pain and also resources that could be called upon to help alleviate pain and improve the quality of life.
I am currently in Arlington, Virginia at the 20th Annual Medical Conference of the American Syringomyelia Alliance Project. ASAP, established in 1988 by Don and Barbara White, has provided support to thousands of people with the Chiari malformations and/or syringomyelia.
The meeting is hosted by Dr. John Heiss, Head of the Surgical Neurology Branch at NINDS, NIH, Bethesda, Maryland. Among today’s presentations:
Chiari & Syringomyelia: Introduction and History of Treatment Methods
W. Jerry Oakes, MD
Professor of Pediatric Neurosurgery
University of Alabama at Birmingham Medical School
Dr. Oakes introduced the Chiari malformation and syringomyelia. He focused on the need to avoid treatment based solely on the findings seen on MRI. The important, and sometimes difficult part, is to determine whether the patients symptoms are due to cerebellar tonsil herniation sometimes found on MRI. Treat based on symptoms, not just on radiology.
Two out of three of the children Dr. Oakes treats for the Chiari malformation also have a syringomyelia. The syrinx is an “objective” finding, versus headache which is difficult to measure and is viewed as a “subjective” finding.
About 15% of the children had other, frequently unique, presentations. One example was a child who would not cry. The reason: if the child cried, the head and neck would arch back due to marked pain from a tight Chiari I malformation. Thus, the child learned not to cry. Another child with CM-I child would not have bowel movements since straining caused severe pain.
Dr. Oakes reported that 8-10% of children with CM-Ialso had hydrocephalus.
He then discussed the outcome following posterior fossa decompression. In children with syringomyelia, the syrinx resolved or improved in 96% within 8 months following surgery. This decreased to 84% at 7 years.
He does not believe the tethered cord causes syringomyelia and would not treat syringomyelia by section of the filum terminale. (More on this controversial issue in future posts. Also, look at Chiari related to tight filum terminale? )
Finally, it was Dr. Iskandar, Dr. Oakes, and colleagues who in 1998 defined Chiari 0. They reported 5 children with syringomyelia but without the classically described CM-I who were nevertheless treated with a posterior fossa decompression. All five children improved.
Dr. Oakes sticks to his definition of the Chiari O malformation: minimal tonsillar descent in a person with syringomyelia. (See Defining Chiari )
Pain Management in Patients with Chiari I and Syringomyelia
Dr. Ann M. Berger, MSN, MD
Head of the Pain Service
NIH Clinical Center, Bethesda, MD
Dr. Berger started with a description of the three types of pain. Somatic pain - pain from the extremities, trunk, and head - is due to activation of nociceptors (pain receptors). These receptors are activated by a stimulus such as inflammation or injury. Visceral pain, often vague and diffuse, is also due to activation of nociceptors. However, neuropathic pain is different. It is due to damage or destruction of the nerve fibers themselves and is usually more difficult to treat.
The pain team at the NIH Clinical Center manages pain using a broad multidisciplinary approach which includes pharmacological treatment, mind-body therapies, and a focus on psychosocial issues and the whole family unit.
Pharmacological treatment depends on the severity of pain. Mild pain, not frequently seen at the Center, is treated with aspirin, tylenol, ibuprofen, naproxen, or similar over-the-counter medications. Moderate pain is treated with opioid (narcotic) medications. Severe pain is treated with long-acting opioids together with short acting opioids for breakthrough pain.
(Dr. Berger reminds us not to forget treatment of constipation in patients on opioid medications.)
She then discussed tolerance, physical dependence, addiction, and pseudo-addiction. Tolerance is the physiological adaptation to opioids that results in a need to increase the dose in order to achieve the same effect over time. Physical dependence describes the changes in the nervous system that occur after prolonged opioid treatment. Sudden withdrawal call lead to withdrawal symptoms.
Dr. Berger notes that addiction, a psychological preoccupation with acquisition of pain medications, occurs in fewer than 0.1% of those on acute/chronic pain medications. She believes more often persons may show a pseudo-addiction: they appear to have addictive behavior but, in reality, are not be getting enough medication to treat their pain. (If anyone has any thoughts or experience on distinguishing these two groups, please add a comment).
Dr. Berger then spoke on the management of neuropathic pain, the type of pain most often suffered by those with syringomyelia. In addition to using analgesics, it is important to use adjuvant medications such as tricyclic antidepressants and selected anticonvulsants. A few patients with more refractory pain may benefit from Clonidine, corticosteroids, local anesthetics such as Mexilitine, or medications such as Ketamine or Baclofen.
In addition to pharmacological treatment, the NIH Pain Service focuses on the social, emotional, spiritual and psychological issues involved in pain. Their multidisciplinary team utilizes a broad range of mind-body therapies including:
Massage - particularly good for myofascial pain
Electro therapeutic point stimulators and TENS
Relaxation - meditation, guided imagery, breathing and others
Biofeedback - may be better for computer oriented people
Hypnosis - a specialized practitioner can train the patient in self-hypnosis
Yoga - effective for mind body and spirit
Acupuncture - “very helpful”
Reiki - “universal energy healing system” for its calming effect
Finger Labyrinth - finger traces path; calming effect
Mandala
Pet Therapy
Art Therapy
Music Therapy
Dr. Berger and her colleagues try to select the right mind-body therapy for each patient. Sometimes trying a series of therapies in order to find the most effective.
A final, and important point, is their emphasis on looking at the whole family unit. They try to identify factors that could aggravate or maintain the pain and also resources that could be called upon to help alleviate pain and improve the quality of life.