Arch Orthop Trauma Surg (2014) 134:139–144 DOI 10.1007/s00402-013-1901-8
HANDSURGERY
Tumors and tumor‑like lesions mimicking carpal tunnel syndrome Zoe H. Dailiana · Sofia Bougioukli · Socratis Varitimidis · Vasileios Kontogeorgakos · Euthimia Togia · Marianna Vlychou · Konstantinos N. Malizos
Received: 24 May 2013 / Published online: 11 December 2013 © Springer-Verlag Berlin Heidelberg 2013
Abstract Introduction Tumors and tumor-like lesions in or around the median nerve are uncommon causes of carpal tunnel syndrome (CTS). The purpose of the present study is to highlight the diagnostic approach and point out the profile of patients with CTS and potential underlying pathology. Materials and methods Twenty-eight patients with 32 affected hands had CTS correlated to a mass in or around the nerve. In 20 hands a palpable mass was present. Diagnostic workup included nerve conduction studies, ultrasound and/or MRI. Pre- and postoperative examination included two-point discrimination (2PD), grip strength, visual analogue scale (for pain) (VAS) and disabilities of the arm, shoulder and hand (DASH) scores. Results Twelve of 28 patients were young (range 9–38 years) and 10 were male. Nerve compression was due to 27 extraneural lesions (8 abnormal muscles, 5 lipomas, 7 tenosynovitis, 4 vascular tumors, 2 ganglia, 1 Dupuytren’s fibromatosis) and five intraneural tumors (three schwannomas, one neurofibroma, one sarcoma). Nerve decompression and excision of extraneural lesions were performed in all cases whereas in intraneural tumors, decompression was followed by excision in most cases and nerve grafting in one. Mean follow-up was 22 months (12–105 months). Extraneural masses were associated with a better outcome than nerve tumors. The mean postoperative VAS/DASH scores Z. H. Dailiana (*) · S. Bougioukli · S. Varitimidis · V. Kontogeorgakos · E. Togia · K. N. Malizos Department of Orthopaedic Surgery, Faculty of Medicine, University of Thessalia, Biopolis, 41110 Larissa, Greece e-mail: [email protected] M. Vlychou Department of Radiology, Faculty of Medicine, University of Thessalia, Biopolis, 41110 Larissa, Greece
were 0.3/16.2 in extraneural lesions and 2.5/22 in intraneural lesions. The 2PD improved gradually in all patients (mean pre- and postoperative 12 and 5 mm). The mean grip strength increased from 28 to 31.3 kg postoperatively. Conclusions Although rare, the surgeon should include in the differential diagnosis of CTS the unusual cause of tumors and tumor-like lesions, especially when the patients’ profile is not typical (young, male, no repetitive stress or manual labor). In addition, the presence of a palpable mass at the distal forearm or palm dictates the need for imaging studies. The extent, location and aggressiveness of the mass will determine the approach and type of procedure. Keywords Carpal tunnel syndrome · Lipoma · Median nerve compression · Neurofibroma · Schwannoma · Tenosynovitis · Tumor-like lesion
Introduction Carpal tunnel syndrome (CTS), or compressive neuropathy of the median nerve at the wrist, is a cause of pain, numbness and tingling in the upper extremity [1] and an increasingly recognized cause of work disability. Carpal tunnel syndrome is the most common peripheral entrapment neuropathy. The prevalence of clinically and electrophysiologically confirmed CTS is 2.7 % [2]. It affects mainly middle-aged women (5.8 % women and 0.6 % men) [3] and can be associated with any condition that causes median nerve pressure at the wrist. In the majority of patients the exact cause and pathogenesis of CTS is unclear (idiopathic CTS). However, in some cases the compression of the median nerve in the carpal tunnel is not idiopathic, but it is caused by other pathogenic factors, local or systemic [4]. Common systemic conditions that may cause CTS include diabetes, hypothyroidism,
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Arch Orthop Trauma Surg (2014) 134:139–144
Fig. 1 A 46-year-old female patient with CTS from a lipoma of the flexor digitorum profundus of the little finger. a Preoperative view of the palmar mass. b MRI of the palm depicting a lipoma between the flexor tendons of the ring and little fingers. c Intra-operative view after division of the transverse ligament, depicting a lipoma raising the palmar arch and compressing the median nerve at the point of exit the carpal tunnel. d Intra-operative view after dissection of the lipoma, depicting its origin from the flexor digitorum profundus of the little finger (little finger in flexed position)
pregnancy, menopause, amyloidosis, rheumatoid arthritis etc. [5] whereas local conditions include trauma, anatomical abnormalities and tumors. Work-tasks demanding forceful handgrip or use of vibrating tools are associated with CTS. The association is stronger if these tasks are accompanied by repetitive hand and wrist movements [6]. In idiopathic CTS, simple decompression of the median nerve by releasing the transverse carpal ligament is a highly successful treatment for the majority of patients [7]. However in cases with local underlying causes, simple release of the transverse carpal ligament may not relieve the symptoms and the causative factor must be also treated. We present the diagnostic and therapeutic approach for cases of carpal tunnel syndrome from space occupying lesions. The purpose of the present study is to highlight the diagnostic approach and point out the profile of patients with CTS and potential underlying pathology.
Materials and methods In a period of 9 years, over 1,100 CTS were operated in our department. In 28 patients with 32 affected hands and mean age of 36 years (range 9–74), symptoms were correlated to a mass arising from the median nerve or the surrounding tissues (Figs. 1a, 2a). Twenty-four patients had unilateral involvement and four had bilateral involvement. The main
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Fig. 2 A 17-year-old female patient with CTS from plexiform neurofibroma. a Preoperative view of a mass at the palmar aspect of the distal forearm. b Intra-operative view after division of the transverse ligament, depicting the enlarged nerve and the site of compression (arch) at the level of the transverse ligament
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Arch Orthop Trauma Surg (2014) 134:139–144 Fig. 3 CTS from flexor tenosynovitis in a 34-year-old female patient. a Ultrasonographic view. b Intra-operative view depicting the median nerve (pointed by the scissors) and a mass around the flexor tendons. c Intra-operative view, after dissection of the synovial tissue from the flexor tendons
symptoms were pain, numbness, tingling and weakness and in 20 hands a palpable mass was present. One patient presented with symptoms from compression not only of the median but also of the ulnar nerve. The mean duration of symptoms was 6 months. Physical examination included Phalen’s and Tinel’s tests, 2PD, grip strength and examination for palpable mass. Diagnostic workup involved nerve conduction studies, ultrasound (U/S) and/or MRI evaluation and the therapeutic approach included an open release of the carpal tunnel with excision of the space occupying lesion in the majority of cases. Postoperatively, the patients were evaluated subjectively and objectively. The subjective evaluation included the visual analogue scale (VAS) pain score, the disabilities of the arm, shoulder and hand (DASH) score, and the overall satisfaction 2 months after the surgical procedure. Patients were also asked to rate improvement of pain and numbness at the same visit. The objective evaluation included the measurement of grip strength with a Jamar dynamometer (Asimov Engineering Co., Los Angeles, CA) 2 months postoperatively and the evaluation of sensation by twopoint discrimination at different time intervals during the first postoperative year. Potential recurrence was assessed during the follow-up visits and in the long term (at the latest follow-up) through a telephone interview. Statistical calculation was performed using the Student’s t test.
IRB approval was not necessary for this retrospective study correlating the presence of tumors and tumor-like lesions of the distal radius-wrist and proximal palm with CTS as the patients underwent an ordinary (though extended) procedure for CTS release. However, all patients gave informed consent for participation in this retrospective study.
Results The patients were divided in 3 age groups: 12 patients were young (9–38 years), 11 were middle-aged (45–60) and 5 were old (65–74). According to the gender, 10 patients were male and 18 female. None of the patients had history of repetitive movements or manual labor. All patients had U/S before surgery that provided important features of the mass, such as size and echogenicity (Fig. 3a). MRI was performed in 24 cases to evaluate the exact dimensions, the tissue of origin, the potential compression of other structures, and the extent of the lesion (Fig. 1b). Twenty-seven lesions were extraneural (Figs. 1, 3, 4) and five intraneural (Fig. 2). The largest dimension of the masses ranged from 2.3 to 16 cm. In all patients abnormal nerve conduction velocities were found preoperatively. All patients underwent open CTS release through classic approach extending to the wrist-distal forearm and the
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Fig. 4 Intra-operative view of a 34-year-old female patient, depicting a muscle mass of the flexor digitorum superficialis of the middle finger (finger in flexed position) and contusion of the median nerve
palm. After release of the palmar fascia and the transverse carpal ligament, and identification and protection of the median nerve, the mass was carefully excised if extraneural (Figs. 1, 3, 4). In tumors arising from the nerve, the mass was excised in four cases (schwannomas and sarcoma) and sural nerve grafting was necessary in one case. In one case the extent of the giant plexiform neurofibroma prohibited excision of the lesion and an extended decompression and neurolysis were performed after the biopsy (Fig. 2). All lesions but one were benign. The extraneural masses included eight abnormal muscles or distally extending muscle bellies (concerning the flexor digitorum) (Fig. 4), five lipomas (Fig. 1), seven cases of flexor tenosynovitis (Fig. 3), four hemangiomas, two ganglion cysts, and one case of compression from abundant tissue in a patient suffering from Dupuytren’s disease. The five intraneural tumors were diagnosed as schwannoma in three cases (one as ancient schwannoma) [8], plexiform neurofibroma (Fig. 2), and sarcoma. In all cases alleviation of symptoms was noted in the immediate postoperative period without complications. The average follow-up period was 22 months (12–105 months). No recurrence was observed during this period. In the cases of extraneural lesions the mean VAS and DASH scores decreased from preoperative values of 8 and 37, respectively, to 0.3 and 16.2 postoperatively, whereas for the intraneural lesions the respective values decreased from 8.5 and 38.2 preoperatively to 2.5 and 22 two months after the procedure. The majority of patients denied having any pain, whereas three patients (three affected hands) rated their pain as mild. A significant difference was found between preoperative and postoperative values of DASH and VAS scores (p
HANDSURGERY
Tumors and tumor‑like lesions mimicking carpal tunnel syndrome Zoe H. Dailiana · Sofia Bougioukli · Socratis Varitimidis · Vasileios Kontogeorgakos · Euthimia Togia · Marianna Vlychou · Konstantinos N. Malizos
Received: 24 May 2013 / Published online: 11 December 2013 © Springer-Verlag Berlin Heidelberg 2013
Abstract Introduction Tumors and tumor-like lesions in or around the median nerve are uncommon causes of carpal tunnel syndrome (CTS). The purpose of the present study is to highlight the diagnostic approach and point out the profile of patients with CTS and potential underlying pathology. Materials and methods Twenty-eight patients with 32 affected hands had CTS correlated to a mass in or around the nerve. In 20 hands a palpable mass was present. Diagnostic workup included nerve conduction studies, ultrasound and/or MRI. Pre- and postoperative examination included two-point discrimination (2PD), grip strength, visual analogue scale (for pain) (VAS) and disabilities of the arm, shoulder and hand (DASH) scores. Results Twelve of 28 patients were young (range 9–38 years) and 10 were male. Nerve compression was due to 27 extraneural lesions (8 abnormal muscles, 5 lipomas, 7 tenosynovitis, 4 vascular tumors, 2 ganglia, 1 Dupuytren’s fibromatosis) and five intraneural tumors (three schwannomas, one neurofibroma, one sarcoma). Nerve decompression and excision of extraneural lesions were performed in all cases whereas in intraneural tumors, decompression was followed by excision in most cases and nerve grafting in one. Mean follow-up was 22 months (12–105 months). Extraneural masses were associated with a better outcome than nerve tumors. The mean postoperative VAS/DASH scores Z. H. Dailiana (*) · S. Bougioukli · S. Varitimidis · V. Kontogeorgakos · E. Togia · K. N. Malizos Department of Orthopaedic Surgery, Faculty of Medicine, University of Thessalia, Biopolis, 41110 Larissa, Greece e-mail: [email protected] M. Vlychou Department of Radiology, Faculty of Medicine, University of Thessalia, Biopolis, 41110 Larissa, Greece
were 0.3/16.2 in extraneural lesions and 2.5/22 in intraneural lesions. The 2PD improved gradually in all patients (mean pre- and postoperative 12 and 5 mm). The mean grip strength increased from 28 to 31.3 kg postoperatively. Conclusions Although rare, the surgeon should include in the differential diagnosis of CTS the unusual cause of tumors and tumor-like lesions, especially when the patients’ profile is not typical (young, male, no repetitive stress or manual labor). In addition, the presence of a palpable mass at the distal forearm or palm dictates the need for imaging studies. The extent, location and aggressiveness of the mass will determine the approach and type of procedure. Keywords Carpal tunnel syndrome · Lipoma · Median nerve compression · Neurofibroma · Schwannoma · Tenosynovitis · Tumor-like lesion
Introduction Carpal tunnel syndrome (CTS), or compressive neuropathy of the median nerve at the wrist, is a cause of pain, numbness and tingling in the upper extremity [1] and an increasingly recognized cause of work disability. Carpal tunnel syndrome is the most common peripheral entrapment neuropathy. The prevalence of clinically and electrophysiologically confirmed CTS is 2.7 % [2]. It affects mainly middle-aged women (5.8 % women and 0.6 % men) [3] and can be associated with any condition that causes median nerve pressure at the wrist. In the majority of patients the exact cause and pathogenesis of CTS is unclear (idiopathic CTS). However, in some cases the compression of the median nerve in the carpal tunnel is not idiopathic, but it is caused by other pathogenic factors, local or systemic [4]. Common systemic conditions that may cause CTS include diabetes, hypothyroidism,
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140
Arch Orthop Trauma Surg (2014) 134:139–144
Fig. 1 A 46-year-old female patient with CTS from a lipoma of the flexor digitorum profundus of the little finger. a Preoperative view of the palmar mass. b MRI of the palm depicting a lipoma between the flexor tendons of the ring and little fingers. c Intra-operative view after division of the transverse ligament, depicting a lipoma raising the palmar arch and compressing the median nerve at the point of exit the carpal tunnel. d Intra-operative view after dissection of the lipoma, depicting its origin from the flexor digitorum profundus of the little finger (little finger in flexed position)
pregnancy, menopause, amyloidosis, rheumatoid arthritis etc. [5] whereas local conditions include trauma, anatomical abnormalities and tumors. Work-tasks demanding forceful handgrip or use of vibrating tools are associated with CTS. The association is stronger if these tasks are accompanied by repetitive hand and wrist movements [6]. In idiopathic CTS, simple decompression of the median nerve by releasing the transverse carpal ligament is a highly successful treatment for the majority of patients [7]. However in cases with local underlying causes, simple release of the transverse carpal ligament may not relieve the symptoms and the causative factor must be also treated. We present the diagnostic and therapeutic approach for cases of carpal tunnel syndrome from space occupying lesions. The purpose of the present study is to highlight the diagnostic approach and point out the profile of patients with CTS and potential underlying pathology.
Materials and methods In a period of 9 years, over 1,100 CTS were operated in our department. In 28 patients with 32 affected hands and mean age of 36 years (range 9–74), symptoms were correlated to a mass arising from the median nerve or the surrounding tissues (Figs. 1a, 2a). Twenty-four patients had unilateral involvement and four had bilateral involvement. The main
13
Fig. 2 A 17-year-old female patient with CTS from plexiform neurofibroma. a Preoperative view of a mass at the palmar aspect of the distal forearm. b Intra-operative view after division of the transverse ligament, depicting the enlarged nerve and the site of compression (arch) at the level of the transverse ligament
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Arch Orthop Trauma Surg (2014) 134:139–144 Fig. 3 CTS from flexor tenosynovitis in a 34-year-old female patient. a Ultrasonographic view. b Intra-operative view depicting the median nerve (pointed by the scissors) and a mass around the flexor tendons. c Intra-operative view, after dissection of the synovial tissue from the flexor tendons
symptoms were pain, numbness, tingling and weakness and in 20 hands a palpable mass was present. One patient presented with symptoms from compression not only of the median but also of the ulnar nerve. The mean duration of symptoms was 6 months. Physical examination included Phalen’s and Tinel’s tests, 2PD, grip strength and examination for palpable mass. Diagnostic workup involved nerve conduction studies, ultrasound (U/S) and/or MRI evaluation and the therapeutic approach included an open release of the carpal tunnel with excision of the space occupying lesion in the majority of cases. Postoperatively, the patients were evaluated subjectively and objectively. The subjective evaluation included the visual analogue scale (VAS) pain score, the disabilities of the arm, shoulder and hand (DASH) score, and the overall satisfaction 2 months after the surgical procedure. Patients were also asked to rate improvement of pain and numbness at the same visit. The objective evaluation included the measurement of grip strength with a Jamar dynamometer (Asimov Engineering Co., Los Angeles, CA) 2 months postoperatively and the evaluation of sensation by twopoint discrimination at different time intervals during the first postoperative year. Potential recurrence was assessed during the follow-up visits and in the long term (at the latest follow-up) through a telephone interview. Statistical calculation was performed using the Student’s t test.
IRB approval was not necessary for this retrospective study correlating the presence of tumors and tumor-like lesions of the distal radius-wrist and proximal palm with CTS as the patients underwent an ordinary (though extended) procedure for CTS release. However, all patients gave informed consent for participation in this retrospective study.
Results The patients were divided in 3 age groups: 12 patients were young (9–38 years), 11 were middle-aged (45–60) and 5 were old (65–74). According to the gender, 10 patients were male and 18 female. None of the patients had history of repetitive movements or manual labor. All patients had U/S before surgery that provided important features of the mass, such as size and echogenicity (Fig. 3a). MRI was performed in 24 cases to evaluate the exact dimensions, the tissue of origin, the potential compression of other structures, and the extent of the lesion (Fig. 1b). Twenty-seven lesions were extraneural (Figs. 1, 3, 4) and five intraneural (Fig. 2). The largest dimension of the masses ranged from 2.3 to 16 cm. In all patients abnormal nerve conduction velocities were found preoperatively. All patients underwent open CTS release through classic approach extending to the wrist-distal forearm and the
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Fig. 4 Intra-operative view of a 34-year-old female patient, depicting a muscle mass of the flexor digitorum superficialis of the middle finger (finger in flexed position) and contusion of the median nerve
palm. After release of the palmar fascia and the transverse carpal ligament, and identification and protection of the median nerve, the mass was carefully excised if extraneural (Figs. 1, 3, 4). In tumors arising from the nerve, the mass was excised in four cases (schwannomas and sarcoma) and sural nerve grafting was necessary in one case. In one case the extent of the giant plexiform neurofibroma prohibited excision of the lesion and an extended decompression and neurolysis were performed after the biopsy (Fig. 2). All lesions but one were benign. The extraneural masses included eight abnormal muscles or distally extending muscle bellies (concerning the flexor digitorum) (Fig. 4), five lipomas (Fig. 1), seven cases of flexor tenosynovitis (Fig. 3), four hemangiomas, two ganglion cysts, and one case of compression from abundant tissue in a patient suffering from Dupuytren’s disease. The five intraneural tumors were diagnosed as schwannoma in three cases (one as ancient schwannoma) [8], plexiform neurofibroma (Fig. 2), and sarcoma. In all cases alleviation of symptoms was noted in the immediate postoperative period without complications. The average follow-up period was 22 months (12–105 months). No recurrence was observed during this period. In the cases of extraneural lesions the mean VAS and DASH scores decreased from preoperative values of 8 and 37, respectively, to 0.3 and 16.2 postoperatively, whereas for the intraneural lesions the respective values decreased from 8.5 and 38.2 preoperatively to 2.5 and 22 two months after the procedure. The majority of patients denied having any pain, whereas three patients (three affected hands) rated their pain as mild. A significant difference was found between preoperative and postoperative values of DASH and VAS scores (p
