SCIENTIFIC ARTICLE

Idiopathic Carpal Tunnel Syndrome in Children and Adolescents Niles J. Batdorf, MD, Sean R. Cantwell, BS, Steven L. Moran, MD

Purpose A retrospective review of a single institution’s experience with idiopathic carpal tunnel syndrome (CTS) in children and adolescents was performed to evaluate management and outcomes in an effort to establish a treatment protocol. Methods All patients diagnosed with idiopathic CTS from ages 1 to 16 years of age between 1983 and 2013 were reviewed. The results of diagnostic testing and efficacy of therapeutic interventions were analyzed. The Boston Carpal Tunnel Questionnaire was sent to all patients following medical or surgical management. Results A total of 20 patients with 31 involved wrists met criteria for entrance into the study. The mean age at diagnosis was 14.4 years. Orthosis fabrication was used as the initial treatment in 30 of 31 wrists and was successful in completely alleviating symptoms in 9 of 30 wrists. A steroid injection was performed in 11 of 31 wrists, completely relieving symptoms in 5 of 11 wrists. Carpal tunnel release was performed in 10 of 31 wrists. Following surgery, patients had complete relief of symptoms in 5 of 10 wrists and partial relief of symptoms in 5 of 10 wrists. Questionnaire response incidence was 55% (11 of 20), with an average longterm follow-up of 17.6 years. Eight questionnaire respondents continued to have mild to moderate symptoms while performing activities of daily living. Conclusions Once metabolic, anatomical, and hereditary causes of pediatric CTS are ruled out, a reasonable treatment course should follow that of adults with orthosis fabrication, followed by injection, and then surgery for those that are refractory to nonsurgical treatment. (J Hand Surg Am. 2015;40(4):773e777. Copyright Ó 2015 by the American Society for Surgery of the Hand. All rights reserved.) Type of study/level of evidence Prognostic IV. Key words Carpal tunnel syndrome, adolescent, childhood, pediatric, idiopathic.

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with the diagnosis and treatment of carpal tunnel syndrome (CTS) in adults, but may be less confident about how to care for a child with CTS. AND SURGEONS ARE FAMILIAR

From the Division of Plastic Surgery; the Mayo Medical School; and the Division of Plastic Surgery and Orthopedic Surgery, Mayo Clinic, Rochester, MN. Received for publication November 11, 2014; accepted in revised form January 14, 2015. No benefits in any form have been received or will be received related directly or indirectly to the subject of this article. Corresponding author: Steven L. Moran, MD, Division of Plastic Surgery and Orthopedic Surgery, Mayo Clinic, 200 First St., SW, Rochester, MN 55905; e-mail: moran.steven@ mayo.edu. 0363-5023/15/4004-0021$36.00/0 http://dx.doi.org/10.1016/j.jhsa.2015.01.026

CTS in children and adolescents is rare and was described by Martin and Masse in 1958.1 Since then, it has become a well-recognized entity associated with metabolic causes and genetic syndromes that are not seen in adults. Etiologies for pediatric CTS can be divided into 3 broad categories (Table 1).2 Children with mucopolysaccharide metabolic disorders will commonly present with CTS, but the diagnosis is sometimes delayed because the association may not be recognized or the child may have difficulty communicating with their providers owing to developmental delay. Mechanical causes include genetically inherited conditions such as Klippel-Trenaunay syndrome or familial CTS.2 Other mechanical causes include trauma and space-occupying lesions such as

Ó 2015 ASSH

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Published by Elsevier, Inc. All rights reserved.

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TABLE 1.

IDIOPATHIC PEDIATRIC CARPAL TUNNEL SYNDROME

Reported Causes of Pediatric CTS Metabolic Causes

Mechanical Causes

Idiopathic/Overuse

Mucopolysaccharidosis

Primary familial CTS

Idiopathic

Mucopolysaccharidosis IH (Hurler)

Schwartz-Jampel

Sports related

Mucopolysaccharidosis IS (Scheie)

Hemophilia

Musical instrument

Mucopolysaccharidosis II (Hunter)

Massive hemangiomatosis

Mucopolysaccharidosis IV(Maroteux-Lamy)

Macrodactyly

Mucolipidosis

Melorheostosis

Mucolipidosis II—I cell disease

Aberrant anatomy

Mucolipidosis III—pseudo-Hurler polydystrophy

Hamartoma Acromicric dysplasia Juvenile idiopathic arthritis Pseudohypoparathyroidism

hamartomas, lipofibromatosis, or ganglion cysts. Early carpal tunnel release is indicated in a patient with CTS who has a metabolic or mechanical etiology.2 Idiopathic pediatric CTS does exist. Case reports and small case series have recognized otherwise healthy children or adolescents who present with classic symptoms of CTS.3e11 It is unclear from the scarce literature how to treat children with idiopathic CTS. It is also not known what the long-term outcomes are of children who have been treated for idiopathic CTS. Therefore, we performed a singleinstitution retrospective review of our 30-year experience and propose a treatment algorithm to help guide management.

anatomical abnormalities, familial CTS, mechanical causes such as a ganglion, intraneural hamartoma, or a history of trauma as the inciting cause of their initial CTS were excluded. Electromyography (EMG) or nerve conduction study (NCS) was not considered a criterion for the diagnosis of pediatric CTS, because this is invasive and/or uncomfortable and many parents declined an initial request for these tests. Study subjects were then mailed a questionnaire that included the validated Boston Carpal Tunnel Questionnaire to assess long-term outcomes.12 Subjects responded by mail or telephone and received no compensation for their participation. RESULTS Twenty patients with CTS due to idiopathic causes were diagnosed at our institution over a 30-year period. Patient sex, laterality of symptoms, age at diagnosis, body mass index (BMI), length of followup, handedness, and etiology are reported in Table 2. Two of the patients were obese, which we defined as a BMI greater than or equal to the ninety-fifth percentile of their age and sex. Presenting symptoms, examination findings, and electrodiagnostic testing results are reported in Table 3.13 Treatment results are reported in Table 4. The questionnaire response incidence was 55%. No patients who presented with unilateral symptoms developed contralateral CTS. A single patient originally treated with orthosis fabrication had recurrence of her symptoms 11 years later, which was also treated with orthosis fabrication. Results of the Boston Carpal Tunnel Questionnaire are summarized in Table 5 according to the classification proposed by Storey et al.14 One patient continued to have severe symptoms. This individual, initially treated with a

MATERIALS AND METHODS After obtaining institutional review board approval, an institutional database was used to collect records for all patients from 1 to 16 years of age diagnosed with idiopathic CTS between 1983 and 2013. A retrospective chart review was performed. Inclusion criteria included clinical symptoms of CTS (numbness within the median nerve distribution, paresthesias, pain, weakness, or nocturnal symptoms), diagnosis of CTS by a physician, treatment at our institution, and documented follow-up. Exclusion criteria included a diagnosis of CTS with no documented follow-up or diagnosis with treatment elsewhere. Patients were included in the study if they were diagnosed with CTS in at least one wrist before their seventeenth birthday. Treatment information for patients including subsequent development of CTS in the contralateral wrist was included even if it occurred after their seventeenth birthday. CTS due to metabolic causes, genetic overgrowth syndromes, J Hand Surg Am.

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TABLE 2. Patients)

Demographic Information (n [ 20

TABLE 3. Presenting Symptoms, Physical Examination Findings, and Electrodiagnostic Testing by Wrist (n [ 31)

Sex Female Male

15 (75%)

Number of Wrists (%)

5 (25%)

Bilateral

11 (55%)

Unilateral

9 (45%)

Presenting symptoms

14.4  2.8 (range, 4.2e16.9)

Numbness/paresthesias/nocturnal symptoms

29 (94)

Age at diagnosis (y  SD) BMI (kg/m2  SD)*

22.1  4.1 (range, 14.7e33.3)

Pain

24 (77)

Follow-up specific to CTS (y) Institutional follow-up (y)

3.0  3.5 (0.1e11.3)

Weakness/clumsiness

11.7  10.0 (0.2e30.5)

Handedness Right

19 (95%)

Ambidextrous

1 (5%)

Left

0

Phalen

20 (65)

Carpal compression

19 (61)

Tinel

22 (71)

No test documented Normal ( 4 mm)

10 (50%)

Abnormal ( 5 mm) Not recorded

Idiopathic

7 (35%)

Sports related

3 (15%)

1 (3)

2-Point discrimination

Etiology by patient history Overuse related to musical instrument

9 (29)

Provocative tests

17 (55) 4 (13) 10 (32)

Thenar atrophy Atrophy No atrophy

*Data missing in 2 patients.

Missing

2 (7) 20 (65) 9 (29)

Nerve conduction test

single steroid injection with complete relief of symptoms, related on her questionnaire that she needed to reduce the amount of work or housework that she performed 7 days a month. She did not have carpal tunnel release. Three patients who underwent bilateral carpal tunnel releases responded to the questionnaire: 1 remained asymptomatic, and the other 2 continued to have moderate symptoms and moderate functional impairment while performing activities of daily living.

Normal

2 (7)

Moderate (5e8 ms latency)

4 (13)

Severe (> 8 ms latency or nonrecordable)

3 (10)

Not performed

9 (29)

EMG Negative

10 (32)

Diagnostic

13 (42)

Not performed

DISCUSSION Experience with pediatric CTS is limited. Since its description by Martin and Masse in 1958,1 much more is now known about CTS in children and adolescents. However, the literature is limited to case reports and small case series, many of them heterogeneous in nature with many different contributing etiologies including metabolic causes and genetic syndromes.10,15e17 In these cases, there is little debate about management. CTS induced by trauma or space-occupying lesions requires alleviation of the compression through carpal tunnel release. Overgrowth syndromes such as KlippelTrenaunay syndrome, if causing CTS, require decompression. However, the largest experience with pediatric idiopathic CTS described 3 patients.3,5,8,9,11 It is J Hand Surg Am.

13 (42)

Mild (< 5 ms latency)

8 (26)

tempting to propose that the majority of cases of childhood and adolescent CTS is caused by repetitive stress injury or overuse. We hesitate to use this term when describing these patients’ symptoms, because the majority of children and adolescents are participating in many new activities. There are not enough patients in our series, nor do we have enough accurate time-specific information on how many hours each day our patients were engaged in an activity, to conclude that their symptoms were caused by overuse. Still, Gibson and Manske11 reported that sports was a predisposing etiology in their series of 3 patients, and 13/20 of our patients reported that playing a musical instrument or sports activity was r

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TABLE 4.

Treatment Results by Wrist (n [ 31)

TABLE 5. BCTQ*

Number of Wrists (%)

Survey response rate (%) Years of follow-up since diagnosis (mean)

Orthosis fabrication Yes

30 (97)

No

1 (3)

Results of orthosis fabrication Complete resolution of symptoms

9 (30)

Incomplete resolution of symptoms

18 (60)

No resolution of symptoms

Results of Follow-Up Survey Including 55 (n ¼ 11/20) 17.6 (range, 2.8e31.8)

Subsequent development of carpal tunnel in other wrist (%)

0

Recurrent carpal tunnel (%)

9 (1/11)

BCTQ symptom severity scale (%) Asymptomatic

3 (10)

Steroid injection

27 (3/11)

Mild

18 (2/11)

Moderate

45 (5/11)

Yes

11 (36)

Severe

9 (1/11)

No

20 (65)

Very severe

0

Results of steroid injection

BCTQ functional status scale (%)

Complete resolution of symptoms

5 (46)

Asymptomatic

Incomplete resolution of symptoms

5 (46)

Mild

45 (4/11)

No resolution of symptoms

1 (9)

Moderate

45 (4/11)

Carpal tunnel release Yes

10 (32)

No

21 (67) 5 (50)

Incomplete resolution of symptoms

5 (50)

No resolution of symptoms

0

the exacerbating factor that elicited their symptoms. Athletic activities inducing CTS7 and the development of CTS in instrumental musicians18 have been previously described. Both obese patients in our study underwent carpal tunnel release, so a high BMI may be a risk factor for the development of CTS in children. We believe that pediatric idiopathic CTS may be more common than previously reported, as Sainio et al3 proposed. When the majority of children and adolescents develop these symptoms, they are first seen by a family practitioner or pediatrician and are advised to proceed with activity modification and orthosis fabrication. In our medical chart review, we came across several children or adolescents who presented with classic symptoms, were diagnosed with CTS, given a wrist orthosis, and their symptoms presumably resolved because they were never mentioned again during subsequent visits. There are a number of limitations and weaknesses to our study. The retrospective analysis is over a long time period, with missing data and a short amount of follow-up in several patients. There could be a J Hand Surg Am.

Severe

0

Very severe

0

BCTQ, Boston Carpal Tunnel Questionnaire. *BCTQ symptom severity scale values are reported based on the following scores: asymptomatic (11), mild (12e22), moderate (23e33), severe (34e44), and very severe (45e55). Functional status scale values are reported based on the following scores: asymptomatic (8), mild (9e16), moderate (17e24), severe (25e32), and very severe (33e40).

Result of carpal tunnel release Complete resolution of symptoms

27 (3/11)

selection bias in that we are a tertiary referral center and are referred patients who do not improve with conservative therapy. Given the rarity of the condition, a prospective series would not be feasible. Not all patients received electrodiagnostic testing. We feel that this represents a realistic approach because most surgeons who are referred children and adolescents will not immediately recommend this uncomfortable investigation and instead will proceed in a stepwise fashion with conservative management. In addition, electrodiagnostic testing is not perfect, and many surgeons still feel that it is appropriate to proceed to surgery based on history and physical examination alone, even if electrodiagnostic testing is negative. This was the observation in one patient in our study who had negative electrodiagnostic testing but responded to a steroid injection and had complete alleviation of symptoms with bilateral carpal tunnel release. The nonspecific and subjective outcome measures of the study (complete, incomplete, or no resolution of symptoms) is another weakness of the study. However, we do feel that this represents the vast majority of surgeons’ determination of success r

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of therapy when treating CTS patients. Another weakness of our study is the modest response incidence from our validated questionnaire. With only 55% participation, it is difficult to make definitive conclusions. After examining our experience with this rare condition, we conclude that it is prudent to proceed in a stepwise fashion, starting with orthosis fabrication and proceeding with steroid injection if orthosis fabrication and activity modification do not alleviate symptoms. Lagos8 reported alleviation of CTS symptoms with good results in 2 children, 1 with a steroid injection and 1 with activity modification. At present, if orthosis fabrication fails to resolve symptoms, we would favor a single steroid injection. Such injections can be performed relatively painlessly in children with the use of topical lidocaine applied to the wrist 30 minutes prior to injection. If a single steroid injection proves unsuccessful, one could consider NCS/EMG prior to contemplating surgery. The NCS and EMG findings were diagnostic in less than 60% of patients. The reason for the low incidence of abnormal values with electrophysiological testing is difficult to interpret in light of the small patient numbers; however, these tests may be less reliable in ruling out CTS in children when compared with adults. Whereas carpal tunnel release was well tolerated, and no complications ensued, patients and their parents should be counseled that surgery can be expected to resolve symptoms completely in half of cases, with partial resolution of symptoms in the other half. The reason for the poor outcomes compared with adults is difficult to explain. Lettin4 described the lack of objective improvement in his patient was likely owing to the protracted compression that she had experienced in the time leading to the diagnosis of CTS. The reason for incomplete resolution of symptoms in our study could also be due to unrecognized intrinsic nerve abnormalities or genetic factors. Although the cause of ongoing

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symptoms is speculative, long-term questionnaire data showed that the majority of patients who are diagnosed and treated with CTS in childhood or adolescence can have enduring symptoms in adulthood. REFERENCES 1. Martin C, Masse P. Carpal tunnel syndrome in children. Arch Fr Pediatr. 1958;15(7):930e940. 2. Lamberti PM, Light TR. Carpal tunnel syndrome in children. Hand Clin. 2002;18(2):331e337. 3. Sainio K, Merikanto J, Larsen TA. Carpal tunnel syndrome in childhood. Dev Med Child Neurol. 1987;29(6):794e797. 4. Lettin AW. Carpal tunnel syndrome in childhood: report of a case. J Bone Joint Surg Br. 1965;47:556e559. 5. Lettin AW. Carpal tunnel syndrome in childhood. Proc R Soc Med. 1966;59(8):705. 6. Leslie BM, Ruby LK. Congenital carpal tunnel syndrome. A case report. Orthopedics. 1985;8(9):1165e1167. 7. Tropet Y, Brientini JM, Monnier G, Vichard P. Carpal tunnel syndrome before 20 years of age. A report of 7 cases. Ann Chir Main Memb Super. 1990;9(1):29e31. 8. Lagos JC. Compression neuropathy in childhood. Dev Med Child Neurol. 1971;13(4):531e532. 9. Feingold MH, Hidvegi E, Horwitz SJ. Bilateral carpal tunnel syndrome in an adolescent. Am J Dis Child. 1980;134(4):394e395. 10. Potulska-Chromik A, Lipowska M, Gawel M, Ryniewicz B, Maj E, Kostera-Pruszczyk A. Carpal tunnel syndrome in children. J Child Neurol. 2014;29(2):227e231. 11. Gibson CT, Manske PR. Carpal tunnel syndrome in the adolescent. J Hand Surg Am. 1987;12(2):279e281. 12. Levine DW, Simmons BP, Koris MJ, et al. A self-administered questionnaire for the assessment of severity of symptoms and functional status in carpal tunnel syndrome. J Bone Joint Surg Am. 1993;75(11):1585e1592. 13. Gellis M, Pool R. Two-point discrimination distances in the normal hand and forearm: application to various methods of fingertip reconstruction. Plast Reconstr Surg. 1977;59(1):57e63. 14. Storey PA, Fakis A, Hilliam R, Bradley MJ, Lindau T, Burke FD. Levine-Katz (Boston) Questionnaire analysis: means, medians or grouped totals? J Hand Surg Eur Vol. 2009;34(6):810e812. 15. Cruz Martinez A, Arpa J, Poilvache P, et al. Carpal tunnel syndrome in childhood: study of 6 cases. Electroencephalogr Clin Neurophysiol. 1998;109(4):304e308. 16. Davis L, Vedanarayanan VV. Carpal tunnel syndrome in children. Pediatr Neurol. 2014;50(1):57e59. 17. Van Meir N, De Smet L. Carpal tunnel syndrome in children. J Pediatr Orthop B. 2005;14(1):42e45. 18. Lederman RJ. Neuromuscular and musculoskeletal problems in instrumental musicians. Muscle Nerve. 2003;27(5):549e561.

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Idiopathic carpal tunnel syndrome in children and adolescents.

A retrospective review of a single institution's experience with idiopathic carpal tunnel syndrome (CTS) in children and adolescents was performed to ...
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