Ultrasonographic Assessment of Carpal Tunnel Syndrome
| Status: | Enrolling by invitation |
|---|---|
| Conditions: | Orthopedic |
| Therapuetic Areas: | Orthopedics / Podiatry |
| Healthy: | No |
| Age Range: | 3 - 12 |
| Updated: | 2/17/2019 |
| Start Date: | July 2013 |
| End Date: | July 2021 |
Ultrasonographic Assessment of Carpal Tunnel Syndrome in Children With Mucopolysaccharidosis and Comparison to Median Nerve Anatomy in Healthy Children.
Mucopolysaccharidosis are lysosomal storage disorders such as Hunter, Hurler, and Sanfilippo
syndromes. These patients have a genetic enzyme deficiency that results in the inability to
degrade glycosaminoglycans. The glycosaminoglycans accumulate in lysosomes causing cell
enlargement and subsequent dysfunction. The accumulation occurs in all tissues including
cartilage, joint capsule, and tendons and can lead to carpal tunnel syndrome, trigger digits,
and various other orthopaedic manifestations [Van Heest, White]. These children often suffer
from severe cognitive impairment and are often unable to communicate pain or numbness. Carpal
tunnel syndrome is almost always present, but may not become apparent until symptoms are
severe and loss of function has occurred. The current gold standard for diagnosis consists of
electromyographic (EMG) and nerve conduction velocity (NCV) studies under sedation or general
anesthetic [Khanna].
Primary Objective: The investigators plan to correlate EMG findings and median nerve
cross-sectional area in children with mucopolysaccharidosis. The investigators hypothesis is
that ultrasonography of the carpal tunnel in patients with mucopolysaccharidosis will prove
to be an effective, reliable, and safe method to evaluate the median nerve, thus avoiding the
need for EMG studies and anesthesia.
Secondary Objective: The investigators want to determine the cross-sectional area of the
median nerve using ultrasonography in a cohort of healthy children, ages 3-12. The
investigators plan to evaluate a cohort of healthy children to determine a normal
cross-sectional area of the median nerve.
syndromes. These patients have a genetic enzyme deficiency that results in the inability to
degrade glycosaminoglycans. The glycosaminoglycans accumulate in lysosomes causing cell
enlargement and subsequent dysfunction. The accumulation occurs in all tissues including
cartilage, joint capsule, and tendons and can lead to carpal tunnel syndrome, trigger digits,
and various other orthopaedic manifestations [Van Heest, White]. These children often suffer
from severe cognitive impairment and are often unable to communicate pain or numbness. Carpal
tunnel syndrome is almost always present, but may not become apparent until symptoms are
severe and loss of function has occurred. The current gold standard for diagnosis consists of
electromyographic (EMG) and nerve conduction velocity (NCV) studies under sedation or general
anesthetic [Khanna].
Primary Objective: The investigators plan to correlate EMG findings and median nerve
cross-sectional area in children with mucopolysaccharidosis. The investigators hypothesis is
that ultrasonography of the carpal tunnel in patients with mucopolysaccharidosis will prove
to be an effective, reliable, and safe method to evaluate the median nerve, thus avoiding the
need for EMG studies and anesthesia.
Secondary Objective: The investigators want to determine the cross-sectional area of the
median nerve using ultrasonography in a cohort of healthy children, ages 3-12. The
investigators plan to evaluate a cohort of healthy children to determine a normal
cross-sectional area of the median nerve.
Inclusion Criteria:
For the first arm of the study (25 participants):
- Children with mucopolysaccharidosis presenting to the senior author's clinic with
signs or symptoms of carpal tunnel syndrome will be enrolled to receive both an EMG
and carpal tunnel ultrasound of the upper extremities.
For the second arm of the study (100 participants):
- Healthy children ages 3-12 with no acute upper extremity trauma or history of
mucopolysaccharidosis.
- 25 additional participants requested in case of inconclusive ultrasound results,
patient removal, or other unusable results.
Exclusion Criteria:
First arm:
- Children with mucopolysaccharidosis who have undergone previous treatment for carpal
tunnel syndrome.
Second arm:
- Acute (<3month) wrist or hand surgery/injuries.
- Unable to obtain parental consent or patient assent.
- History of mucopolysaccharidosis.
- Non-english speaking.
- Cognitive impairment.
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