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Ulnar nerve compression in Guyon's canal caused by calcinosis in scleroderma
Ulnar nerve compression in Guyon’s canal caused by calcinosis in scleroderma Calcium deposits in Guyon’s canal secondary to scleroderma in a 70-year-old
caused low ulnar nerve paralysis
white woman. Excision of calcium deposits resulted in rapid alleviation of pain.
After nerve regeneration,
the patient’s clawing disappeared,
and she was able to abduct and
adduct all digits. (J HAND SURC 1991;16A:739-41.)
Robert T. Thurman,
MD, Pankaj Jindal, MD, and Thomas W. Wolff, MD, Louisville,
Ky.
V
arious causes of ulnar nerve compression in Guyon’s canal have been described. ‘-I5We report a lesion caused by calcium deposits from scleroderma; no similar account. could be found in the English-language literature. The clinical manifestations of such a compression can vary depending on the exact location of the lesion. The present case history demonstrates this fact. Case report A 70-year-old white woman was seen initially with a 6month history of inability to manipulate the fingers of the left hand, of sharp pain on holding a skillet, and of dropping objects from the hand because of loss of control in the small and ring fingers. On examination she had an ulnar claw deformity with marked wasting of the interosseous muscles. This was not associated with any sensory loss. There was mild-to-moderate tenderness over the region of the hook of the hamate. Tinel’s sign was negative over the cubital tunnel and Guyon’s canal. She could not abduct her small and ring fingers. Plain radiographs showed an area of calcification over the hamate (Fig. 1). The patient’s symptoms dated back 28 years and initially included cold intolerance. The diagnosis of Raynaud’s disease was treated by bilateral cervical sympathectomy. This procedure helped her for almost 8 years, at which point painful ulceration of her fingertips developed. A few years later she
Fig. 1. Preoperative radiograph with arrows showing location of calcinosis in Guyon’s canal and circumscribing the hook of the hamate.
From the Christine M. Kleinert Institute for Hand and Micro Surgery, Louisville,
Ky.
Received for publication Sept. 15, 1990.
June 18, 1990; accepted
in revised form
No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Thomas W. Wolff, MD, One Medical Center Plaza, Suite 800, 225 Abraham Flexner Way, Louisville, KY 40202. 3/l/26502
had several episodes of drainage of pasty material from the region of both olecranons. These draining sinuses were excised after failure of conservative measures. When the patient was 67 years old, prepatellar calcification developed and was excised. In addition to multiple calcific deposits notably over the olecranon, prepatellar areas, and subcutaneous borders of the ulnas, the patient also had Raynaud’s phenomenon, dysphagia, sclerodermatous facies, ulceration of the tip of her small
THE
JOURNAL
OF HAND
SURGERY
739
740
The Journal of HAND SURGERY
Thurman et al.
B Fig. 2. A, Intraoperative view of calcinosis and stretched ulnar nerve. B, Diagrammatic tation of photograph, showing the sensory and motor branches of the ulnar nerve.
finger, and multiple telangiectatic spots over face and chest wall. The anterior aspect of her knee also showed calcification. This constituted a full-blown picture of CREST syndrome. The systemic manifestations of scleroderma are Calcinosis cutis. Raynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia which, when they occur together, are known as the CREST syndrome. During surgical treatment for the patient’s presenting condition, a well-circumscribed calcific deposit approximately 1 cm in diameter was found lying between the hamate and the pisiform (Fig. 2). This deposit was deep to the ulnar nerve and distal to its branches to the hypothenar muscles and to its sensory branches to the digits. As the ulnar nerve curved medially around the hook of the hamate, the mound of calcific deposits stretched its motor branch. There was a distinct demarcation at this point, with the proximal normal nerve appearing grayish-white and the distal stretched nerve appearing gelatinous. The nerve was carefully mobilized and the calcific deposits were excised without unnecessary handling of the nerve. This did not change the appearance of the nerve immediately. The operation resulted in rapid alleviation of pain. The surgical wound over the hand healed well. At follow-up 6 months after the operation the patient was noted to have regained good function of her hand. Her clawing had disappeared, and she was able to abduct and adduct all
represen-
digits. She also stated that she was recovering and that the pain had completely disappeared.
her strength
Discussion The spectrum of hand involvement in scleroderma ranges from Raynaud’s phenomenon, which is the most common presenting sign, to the more advanced stages of sclerodactyly, digital tip ulceration, and distal phalangeal resorption. Women are more frequently affected than men by a ratio of 4: 1. I6 Although sclerosis is the hallmark of the disease, calcinosis involving the subcutaneous tissue and major joints is noted in 40% of patients. 17.‘* Distal compression of the ulnar nerve was first predicted by Guyon in 1861 when he noted the space at the wrist through which it passes and its subsequent branching.” However, it was Hunt who first reported clinical cases of ulnar nerve compression in Guyon’s canal. ’ Ulnar nerve compression within Guyon’s canal has previously been associated with the following: ganglions’, 3; anomalous muscle, tendon, or ligamentous anatomy*. 4-6;neoplasia of neural elements, tendon
Vol. 16A, No. 4 July 1991
Ulnar nerve compression in Guyon’s canal
sheath, and adipose tissue7-lo; synovial and fascial inflammation2. ‘I; ulnar artery thrombosis and pseudoaneurysm formation”, ‘*; injuries resulting in forearm bone, carpal, and metacarpal fractures13. ‘O; and repetitive trauma related to occupational or sporting activities. II. 14. I5 Shea and McClain” correlated clinical findings of distal ulnar nerve compression with three distinct anatomic zones in the region of Guyon’s canal. Type I compression involves both motor and sensory fibers proximal to the bifurcating nerve. Type II lesions involve the deep branch and result in motor abnormalities with normal sensibility. Type III, compressing the superficial branch of the ulnar nerve, produces sensory disturbances without motor loss. Cadaver studies2’ have subsequently subdivided zone II into proximal compression, which involves all intrinsic muscles innervated by the deep branch of the ulnar nerve and distal compression as in our patient, which spares the proximally dividing hypothenar branches. Both studies demonstrated
that motor fibers to the palmaris
come from the superficial
(sensory)
branch
7
8.
9.
10.
11. 12.
13. 14.
brevis muscle of the ulnar
nerve. We would like to thank the Media Services department of the Christine M. Kleinert Institute for Hand and Micro Surgery for the photograph and illustration, Paul Adkins, RN, for follow-up examination of the patient, and Kell Julliard for editing.
15.
16.
17.
REFERENCES 1 Hunt JR. Occupation neuritis of the deep palmar branch of the ulnar nerve. A well defined clinical type of professional palsy of the hand. J Nervous Mental Dis 1908;35:673-89. 2. Kleinert HE, Hayes JE. The ulnar tunnel syndrome. Plast Reconstr Surg 1971;47:21-4. 3. Kuschner SH, Gelberman RH, Jennings C. Ulnar nerve compression at the wrist. J HAND SURG 1988;13A:57780. in nerve entrapment syn4. Howard FM. Controversies drome in the forearm and wrist. Orthop Clin North Am 1986;17:375-81. 5. O’Hara JJ, Stone JH. Ulnar neuropathy at the wrist associated with aberrant flexor carpi ulnaris insertion. J HAND SURG 1988;13A:370-1. 6. Robinson D, Aghasi MK, Halperin N. Ulnar tunnel syn-
18.
19.
20.
21.
22.
141
drome caused by an accessory palmaris muscle. Orthop Rev 1989;18:345-7. Bowers WH, Doppelt SH. Compression of the deep branch of the ulnar nerve by an intraneural cyst. J Bone Joint Surg 1979;61A:612-3. Sarangapani K, More AR. Neurilemmoma of deep branch of ulnar nerve presenting as ulnar tunnel syndrome. Hand 1983;15:216-7. Rafecas JC, Daube JR, Ehman RL. Deep branch ulnar neuropathy due to giant cell tumor: report of a case. Neurology 1988;38:327-9. McFarland GB, Hoffer MM. Paralysis of the intrinsic muscles of the hand secondary to lipoma in Guyon’s tunnel. J Bone Joint Surg 1971;53A:375-6. Grundberg AB. Ulnar tunnel syndrome. J HAND SURC 1984;98:72-4. Kalisman M, Laborde K, Wolff TW. Ulnar nerve compression secondary to ulnar artery false aneurysm at Guyon’s canal. J HAND SURG 1982;7: 137-9. Howard FM. Ulnar nerve palsy in wrist fractures. J Bone Joint Surg 1961;43A:1197-1201. Hankey GJ, Gubbay S. Compressive mononeuropathy of the deep branch of the ulnar nerve in cyclists. J Neural Psychiatry 1988;51: 1588-90. Stem PJ, Vice M. Compression of the deep branch of the ulnar nerve-a case report. J HAND SURG 1983;8: 72-4. Gilliland BC. Progressive systemic sclerosis (diffuse scleroderma). In: Braunwald E, Isselbach KJ, Petersdorf RG, Wilson JD, Martin JP, Fauci AT, eds. Harrison’s principles of internal medicine. 1 lth ed. New York: McGraw-Hill, 1987:1428-32. Bluefarb SM. Changes in connective tissue. In: Katz WA, ed. Diagnosis and management of rheumatic diseases. 2nd ed. Philadelphia: JB Lippincott, 1988: 171-82. Seibold JR. Scleroderma. In: Kelley WM, Harris ED, Shaun R, Sledge CB. eds. Textbook of rheumatology. 3rd ed. Philadelphia: WB Saunders, 1989:1215-44. Guyon F. Note sur une disposition anatomique prope a la face anterieure de la region du poignet et non encore decrite. Bull Sot Anat Paris Second Series 1861;6: 184-6. Vance R, Gelberman RH. Acute ulnar neuropathy with fractures at the wrist. J Bone Joint Surg 1978;60A: 962-5. Shea D, McClain EJ. Ulnar nerve compression syndromes at and below the wrist. J Bone Joint Surg 1969;51A:1095-1103. Gross MS, Gelberman RH. The anatomy of the distal ulnar tunnel. Clin Orthop 1985;196:238-47.
caused low ulnar nerve paralysis
white woman. Excision of calcium deposits resulted in rapid alleviation of pain.
After nerve regeneration,
the patient’s clawing disappeared,
and she was able to abduct and
adduct all digits. (J HAND SURC 1991;16A:739-41.)
Robert T. Thurman,
MD, Pankaj Jindal, MD, and Thomas W. Wolff, MD, Louisville,
Ky.
V
arious causes of ulnar nerve compression in Guyon’s canal have been described. ‘-I5We report a lesion caused by calcium deposits from scleroderma; no similar account. could be found in the English-language literature. The clinical manifestations of such a compression can vary depending on the exact location of the lesion. The present case history demonstrates this fact. Case report A 70-year-old white woman was seen initially with a 6month history of inability to manipulate the fingers of the left hand, of sharp pain on holding a skillet, and of dropping objects from the hand because of loss of control in the small and ring fingers. On examination she had an ulnar claw deformity with marked wasting of the interosseous muscles. This was not associated with any sensory loss. There was mild-to-moderate tenderness over the region of the hook of the hamate. Tinel’s sign was negative over the cubital tunnel and Guyon’s canal. She could not abduct her small and ring fingers. Plain radiographs showed an area of calcification over the hamate (Fig. 1). The patient’s symptoms dated back 28 years and initially included cold intolerance. The diagnosis of Raynaud’s disease was treated by bilateral cervical sympathectomy. This procedure helped her for almost 8 years, at which point painful ulceration of her fingertips developed. A few years later she
Fig. 1. Preoperative radiograph with arrows showing location of calcinosis in Guyon’s canal and circumscribing the hook of the hamate.
From the Christine M. Kleinert Institute for Hand and Micro Surgery, Louisville,
Ky.
Received for publication Sept. 15, 1990.
June 18, 1990; accepted
in revised form
No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Thomas W. Wolff, MD, One Medical Center Plaza, Suite 800, 225 Abraham Flexner Way, Louisville, KY 40202. 3/l/26502
had several episodes of drainage of pasty material from the region of both olecranons. These draining sinuses were excised after failure of conservative measures. When the patient was 67 years old, prepatellar calcification developed and was excised. In addition to multiple calcific deposits notably over the olecranon, prepatellar areas, and subcutaneous borders of the ulnas, the patient also had Raynaud’s phenomenon, dysphagia, sclerodermatous facies, ulceration of the tip of her small
THE
JOURNAL
OF HAND
SURGERY
739
740
The Journal of HAND SURGERY
Thurman et al.
B Fig. 2. A, Intraoperative view of calcinosis and stretched ulnar nerve. B, Diagrammatic tation of photograph, showing the sensory and motor branches of the ulnar nerve.
finger, and multiple telangiectatic spots over face and chest wall. The anterior aspect of her knee also showed calcification. This constituted a full-blown picture of CREST syndrome. The systemic manifestations of scleroderma are Calcinosis cutis. Raynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia which, when they occur together, are known as the CREST syndrome. During surgical treatment for the patient’s presenting condition, a well-circumscribed calcific deposit approximately 1 cm in diameter was found lying between the hamate and the pisiform (Fig. 2). This deposit was deep to the ulnar nerve and distal to its branches to the hypothenar muscles and to its sensory branches to the digits. As the ulnar nerve curved medially around the hook of the hamate, the mound of calcific deposits stretched its motor branch. There was a distinct demarcation at this point, with the proximal normal nerve appearing grayish-white and the distal stretched nerve appearing gelatinous. The nerve was carefully mobilized and the calcific deposits were excised without unnecessary handling of the nerve. This did not change the appearance of the nerve immediately. The operation resulted in rapid alleviation of pain. The surgical wound over the hand healed well. At follow-up 6 months after the operation the patient was noted to have regained good function of her hand. Her clawing had disappeared, and she was able to abduct and adduct all
represen-
digits. She also stated that she was recovering and that the pain had completely disappeared.
her strength
Discussion The spectrum of hand involvement in scleroderma ranges from Raynaud’s phenomenon, which is the most common presenting sign, to the more advanced stages of sclerodactyly, digital tip ulceration, and distal phalangeal resorption. Women are more frequently affected than men by a ratio of 4: 1. I6 Although sclerosis is the hallmark of the disease, calcinosis involving the subcutaneous tissue and major joints is noted in 40% of patients. 17.‘* Distal compression of the ulnar nerve was first predicted by Guyon in 1861 when he noted the space at the wrist through which it passes and its subsequent branching.” However, it was Hunt who first reported clinical cases of ulnar nerve compression in Guyon’s canal. ’ Ulnar nerve compression within Guyon’s canal has previously been associated with the following: ganglions’, 3; anomalous muscle, tendon, or ligamentous anatomy*. 4-6;neoplasia of neural elements, tendon
Vol. 16A, No. 4 July 1991
Ulnar nerve compression in Guyon’s canal
sheath, and adipose tissue7-lo; synovial and fascial inflammation2. ‘I; ulnar artery thrombosis and pseudoaneurysm formation”, ‘*; injuries resulting in forearm bone, carpal, and metacarpal fractures13. ‘O; and repetitive trauma related to occupational or sporting activities. II. 14. I5 Shea and McClain” correlated clinical findings of distal ulnar nerve compression with three distinct anatomic zones in the region of Guyon’s canal. Type I compression involves both motor and sensory fibers proximal to the bifurcating nerve. Type II lesions involve the deep branch and result in motor abnormalities with normal sensibility. Type III, compressing the superficial branch of the ulnar nerve, produces sensory disturbances without motor loss. Cadaver studies2’ have subsequently subdivided zone II into proximal compression, which involves all intrinsic muscles innervated by the deep branch of the ulnar nerve and distal compression as in our patient, which spares the proximally dividing hypothenar branches. Both studies demonstrated
that motor fibers to the palmaris
come from the superficial
(sensory)
branch
7
8.
9.
10.
11. 12.
13. 14.
brevis muscle of the ulnar
nerve. We would like to thank the Media Services department of the Christine M. Kleinert Institute for Hand and Micro Surgery for the photograph and illustration, Paul Adkins, RN, for follow-up examination of the patient, and Kell Julliard for editing.
15.
16.
17.
REFERENCES 1 Hunt JR. Occupation neuritis of the deep palmar branch of the ulnar nerve. A well defined clinical type of professional palsy of the hand. J Nervous Mental Dis 1908;35:673-89. 2. Kleinert HE, Hayes JE. The ulnar tunnel syndrome. Plast Reconstr Surg 1971;47:21-4. 3. Kuschner SH, Gelberman RH, Jennings C. Ulnar nerve compression at the wrist. J HAND SURG 1988;13A:57780. in nerve entrapment syn4. Howard FM. Controversies drome in the forearm and wrist. Orthop Clin North Am 1986;17:375-81. 5. O’Hara JJ, Stone JH. Ulnar neuropathy at the wrist associated with aberrant flexor carpi ulnaris insertion. J HAND SURG 1988;13A:370-1. 6. Robinson D, Aghasi MK, Halperin N. Ulnar tunnel syn-
18.
19.
20.
21.
22.
141
drome caused by an accessory palmaris muscle. Orthop Rev 1989;18:345-7. Bowers WH, Doppelt SH. Compression of the deep branch of the ulnar nerve by an intraneural cyst. J Bone Joint Surg 1979;61A:612-3. Sarangapani K, More AR. Neurilemmoma of deep branch of ulnar nerve presenting as ulnar tunnel syndrome. Hand 1983;15:216-7. Rafecas JC, Daube JR, Ehman RL. Deep branch ulnar neuropathy due to giant cell tumor: report of a case. Neurology 1988;38:327-9. McFarland GB, Hoffer MM. Paralysis of the intrinsic muscles of the hand secondary to lipoma in Guyon’s tunnel. J Bone Joint Surg 1971;53A:375-6. Grundberg AB. Ulnar tunnel syndrome. J HAND SURC 1984;98:72-4. Kalisman M, Laborde K, Wolff TW. Ulnar nerve compression secondary to ulnar artery false aneurysm at Guyon’s canal. J HAND SURG 1982;7: 137-9. Howard FM. Ulnar nerve palsy in wrist fractures. J Bone Joint Surg 1961;43A:1197-1201. Hankey GJ, Gubbay S. Compressive mononeuropathy of the deep branch of the ulnar nerve in cyclists. J Neural Psychiatry 1988;51: 1588-90. Stem PJ, Vice M. Compression of the deep branch of the ulnar nerve-a case report. J HAND SURG 1983;8: 72-4. Gilliland BC. Progressive systemic sclerosis (diffuse scleroderma). In: Braunwald E, Isselbach KJ, Petersdorf RG, Wilson JD, Martin JP, Fauci AT, eds. Harrison’s principles of internal medicine. 1 lth ed. New York: McGraw-Hill, 1987:1428-32. Bluefarb SM. Changes in connective tissue. In: Katz WA, ed. Diagnosis and management of rheumatic diseases. 2nd ed. Philadelphia: JB Lippincott, 1988: 171-82. Seibold JR. Scleroderma. In: Kelley WM, Harris ED, Shaun R, Sledge CB. eds. Textbook of rheumatology. 3rd ed. Philadelphia: WB Saunders, 1989:1215-44. Guyon F. Note sur une disposition anatomique prope a la face anterieure de la region du poignet et non encore decrite. Bull Sot Anat Paris Second Series 1861;6: 184-6. Vance R, Gelberman RH. Acute ulnar neuropathy with fractures at the wrist. J Bone Joint Surg 1978;60A: 962-5. Shea D, McClain EJ. Ulnar nerve compression syndromes at and below the wrist. J Bone Joint Surg 1969;51A:1095-1103. Gross MS, Gelberman RH. The anatomy of the distal ulnar tunnel. Clin Orthop 1985;196:238-47.