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Familial radial nerve entrapment syndrome: A case report and literature review
Familial radial nerve entrapment syndrome: A case report and literature review A case of familial peripheral nerve entrapment syndrome at the level of the lateral head of the triceps is presented. Treatment consisted of exploration of the radial nerve with release at the level of the fibrous arch in the lateral head of the triceps. Relief of symptoms and improvement in function occurred. (J HAND SURG 8:297-9, 1983.)
John D. Lubahn, M.D., and Graham D. Lister, F.R.C.S., Louisville, Ky.
Entrapment of the radial nerve with subsequent palsy of the posterior interosseous branch was first described in 1863 by Agnew. 1 This initial lesion was secondary to a "bursal" tumor arising medial to the biceps tendon. Surgical excision afforded the patient complete relief of her symptoms. Numerous articles have appeared in the literature since then describing various lesions of the radial nerve at different levels. Worthy of note are cases that describe compression of the posterior interosseous nerve at the arcade of Frohse. 2 Lesions at this level may be secondary to ganglion cysts,3 lipoma,4 or to rheumatoid arthritis.5 From the supinator proximally, lesions can be seen secondary to fibrous bands, the radial recurrent artery, and the tendinous edge of the extensor carpi radialis brevis muscle. 6 • 7 Lotem8 has described a fibrous arch at the level of the lateral head of the triceps that he associates with nerve compression secondary to swelling of this muscle following muscular effort, although he had no surgical proof of such a lesion. Finally, maladies such as lead poisoning, diabetes mellitus, alcoholism,9 as well as anatomical variation lO and injection trauma ll have been reported as causes of radial nerve palsy. Several recent publications in the medical literature have considered nerve compression syndromes on a familial or genetic basis. 12- 15 The following report deals with such a case, the surgical findings, and the final result.
Case report A 15-year-old, right-handed, white girl presented with a Ilh-month history of having awakened in the morning with a From the Department of Surgery (Hand), University of Louisville School of Medicine, Louisville, Ky. Received for publication Feb. 9, 1982. Reprint requests: Dr. John D. Lubahn, 406 Peach St., Erie, PA 16507.
right wrist drop. She denied any history of trauma and had no other ongoing medical problems. Her family history is relevant in that her father also has suffered from intermittent radial nerve palsy of the right upper extremity and her sister, who is 2 years her senior, had recently developed an identical lesion that was improving spontaneously. The remainder of the immediate family included a 17-year-old brother and a 41-year-old mother, both were asymptomatic. On physical examination, the patient had palsy of all radially innervated motor units distal to the triceps combined with decreased sensibility in the distribution of the radial nerve. Electromyographic (EMG) and nerve conduction study results were consistent with a lesion in the radial nerve at the level of the lateral head of the triceps. Because of the localized nature of the findings, coupled with the total lack of improvement in her symptoms following 6 weeks in an extension outrigger, an exploration of her radial nerve through a posterior incision as described by Henry was carried out. The fibrous arch at this level was noted to be causing compression on the nerve and it was released (Fig. 1). The nerve appeared hypervascular and there was no evidence of a neuroma. Postoperatively she has done well. Both motor and sensory return were gradual. Six months after the operation she was regaining motor strength in all her extensor musculature, with all the radially innervated motor units graded 4 to 5.
Discussion The concept of a nerve compression syndrome in the upper extremity existing purely on a familial basis presents a therapeutic challenge to the hand surgeon. This patient illustrates the presentation, evaluation, and follow-up surgery in such a case. The theory set forth by Mayer and Garcia-Mullin 12 could explain the process in this family. They describe a genetically determined defect in Schwann cellmyelin metabolism, probably autosomal dominant in nature. The disorder may be entirely asymptomatic. However, sites along the nerve that are subject to THE JOURNAL OF HAND SURGERY
297
298
The Journal of HAND SURGERY
Lubahn and Lister
Fig. 1. A, The inset shows the Henry approach and the asterisk shows the radial nerve prior to its passing through the lateral head of the triceps muscle . B, An operative photograph with the arrow showing the radial nerve at a point prior to its passing through the lateral head of the triceps. The photograph shows the radial nerve after the fibrous arch had been divided and the radial nerve had been released along its course through the lateral head of the triceps.
chronic or intennittent compression may undergo segmental demyelination with resultant nerve palsy. Recovery could then be expected with relief of the pressure and subsequent remyelination. The decision to explore the area surgically is thus justified, particularly in this case in which compression could be localized both clinically and by EMG to the lateral head of the triceps. It could be postulated that no genetic deficit in metabolism is necessary, but that a familial predisposition to an unduly constrictive arch in the triceps could be the cause. This postulate could only be proven by exploration of all the affected members of the family. This patient's father continues to have recurrent episodes of paralysis but refuses treatment, while her sister has completely recovered. Presumptive confinnation might be forthcoming if the patient remains trouble free while both of the unoperated, older members of the family have recurrent bouts of radial palsy. We would like to thank Grace Ascher for her assistance in the preparation of the illustrations for this article.
REFERENCES I . Agnew DH: Bursal tumor producing loss of power of forearm. Am J Med Sci 46:404-5, 1863
2. Spinner M: The arcade of Frohse and its relationship to 3.
4.
5.
6.
7.
posterior interosseous nerve paralysis. J Bone Joint Surg [Br] 50:809-12, 1968 Loser R, Schafer ER: Ein Ganglion der Ellenbeuge als Ursache einer dissoziierten radialparese von unterarmtyp Darstellung eines seltenen Falles. Neurochirurgia 5: 182-6, 1972 Wu KJ, Jordan FR, Eckert C: Lipoma, a cause of paralysis of deep radial (posterior interosseous) nerve-Report of a case and review of the literature. Surgery 75: 790-5, 1974 Marshall SC, Murray WR: Deep radial nerve palsy associated with rheumatoid arthritis . Clin Orthop Rei Res 103: 157-62, 1974 Roles NC, Maudsley RH: Radial tunnel syndrome: Resistant tennis elbow as a nerve entrapment. J Bone Joint Surg [Br] 54:499-508, 1972 Lister GD, Belsole RB , Kleinert HD: The radial tunnel syndrome. J HAND SURG 4:52-9, 1979
Vol. 8, NO.3 May 1983
8. Lotem M, Fried A, Levy M, Solze p, Najenson T, Nathan M: Radial palsy following muscular effort. J Bone Joint Surg [Br] 53:500-6, 1971 9. Barton NJ: Radial nerve lesions. Hand 5:200-8, 1973 10. Woltman HW, Learmonth JR: Progressive paralysis of the nervus interosseus dorsalis. Brain 57:25-31, 1934 11. Fischer W, Behlke M: Injektionsschadigung des Ramus profundus nervi radialis. Dtsch Ges Wesen 42: 19992000, 1969 12. Mayer FR, Garcia-Mullin R: Hereditary neuropathy manifested by pressure palsies-A Schwann cell disorder? Trans Am Neurol Assoc 93:238-40, 1968
Familial radial nerve entrapment syndrome
13. Karpati G, Carpenter S, Eisen AA, Geindel W: Familial multiple peripheral nerve entrapments-An unusual manifestation of a peripheral neuropathy. Trans Am Neurol Assoc 26A:267-9, 1973 14. Behse F, Buchthal F, Carlsen F, Knappeis GG: Hereditary neuropathy with liability to pressure palsies-Electrophysiological and histopathological aspects. Brain 95:777-94, 1972 15. Earl CJ, Fullerton PM, Wakefield GS, Schutta HS: Hereditary neuropathy, with liability to pressure palsies-A clinical and electrophysiological study of four families. Q J Med 33:481-98, 1964
The short-term effects of Dexon and nylon sutures in experimental microvascular surgery-A quantitative comparison Acute inflammatory responses to 10-0 Dexon and nylon were compared 2 weeks after experimental microvascular surgery in Sprague-Dawley rats. Statistical analysis revealed no significant differences, but the strength, long-term results, and minimal surrounding tissue reaction make Dexon a viable alternative to nylon. The main disadvantage of Dexon is its opaque blue-green color, which makes it nearly invisible in the operating field. (J HAND SURG 8:299-301, 1983.)
Allan F. Cook, B.Sc., Carlos A. Azar, M.D., and Melvyn I. Dinner, F.R.C.S., Cleveland, Ohio
During the first 2 weeks following microvascular repair the anastomosed artery is completely responsible for the survival of the tissue it supplies. After 2 weeks, collateral circulation becomes established to the point where occlusion of the main artery will not automatically result in necrosis of the tissue supplied.! Numerous studies show that, during this time period, significant thickening of the arterial wall occurs, resulting in a decrease in the size of the lumen. 2 - 5 This thickening has been directly related to the
From The Cleveland Clinic Foundation, Microsurgery Laboratory, Department of Plastic and Reconstructive Surgery Received for publication March 5, 1982; accepted in revised fonn July 13, 1982. Reprint requests: Allan F. Cook, B.Sc., Microsurgery Laboratory 2R52, 9500 Euclid Ave., Cleveland, OH 44106.
Table I. Mean percentage makeup of the total cross-sectional area of the vessels studied Material
Normal arteries Dexon Nylon
10 10
10
% Vessel wall
% Vessel lumen
31.3
68.7
56.7 60.8
43.3
39.4
acute inflammatory response, within the vessel wall, to the trauma of anastomosis. In 1979 and 1980 two separate studies were done comparing absorbable and nonabsorbable suture material in microvascular surgery. 6-7 Both studies pointed out that polyglycolic acid suture material has fewer long-term ill effects than nonabsorbable sutures. The short-term effects were the same regardless of the material used.
THE JOURNAL OF HAND SURGERY
299
John D. Lubahn, M.D., and Graham D. Lister, F.R.C.S., Louisville, Ky.
Entrapment of the radial nerve with subsequent palsy of the posterior interosseous branch was first described in 1863 by Agnew. 1 This initial lesion was secondary to a "bursal" tumor arising medial to the biceps tendon. Surgical excision afforded the patient complete relief of her symptoms. Numerous articles have appeared in the literature since then describing various lesions of the radial nerve at different levels. Worthy of note are cases that describe compression of the posterior interosseous nerve at the arcade of Frohse. 2 Lesions at this level may be secondary to ganglion cysts,3 lipoma,4 or to rheumatoid arthritis.5 From the supinator proximally, lesions can be seen secondary to fibrous bands, the radial recurrent artery, and the tendinous edge of the extensor carpi radialis brevis muscle. 6 • 7 Lotem8 has described a fibrous arch at the level of the lateral head of the triceps that he associates with nerve compression secondary to swelling of this muscle following muscular effort, although he had no surgical proof of such a lesion. Finally, maladies such as lead poisoning, diabetes mellitus, alcoholism,9 as well as anatomical variation lO and injection trauma ll have been reported as causes of radial nerve palsy. Several recent publications in the medical literature have considered nerve compression syndromes on a familial or genetic basis. 12- 15 The following report deals with such a case, the surgical findings, and the final result.
Case report A 15-year-old, right-handed, white girl presented with a Ilh-month history of having awakened in the morning with a From the Department of Surgery (Hand), University of Louisville School of Medicine, Louisville, Ky. Received for publication Feb. 9, 1982. Reprint requests: Dr. John D. Lubahn, 406 Peach St., Erie, PA 16507.
right wrist drop. She denied any history of trauma and had no other ongoing medical problems. Her family history is relevant in that her father also has suffered from intermittent radial nerve palsy of the right upper extremity and her sister, who is 2 years her senior, had recently developed an identical lesion that was improving spontaneously. The remainder of the immediate family included a 17-year-old brother and a 41-year-old mother, both were asymptomatic. On physical examination, the patient had palsy of all radially innervated motor units distal to the triceps combined with decreased sensibility in the distribution of the radial nerve. Electromyographic (EMG) and nerve conduction study results were consistent with a lesion in the radial nerve at the level of the lateral head of the triceps. Because of the localized nature of the findings, coupled with the total lack of improvement in her symptoms following 6 weeks in an extension outrigger, an exploration of her radial nerve through a posterior incision as described by Henry was carried out. The fibrous arch at this level was noted to be causing compression on the nerve and it was released (Fig. 1). The nerve appeared hypervascular and there was no evidence of a neuroma. Postoperatively she has done well. Both motor and sensory return were gradual. Six months after the operation she was regaining motor strength in all her extensor musculature, with all the radially innervated motor units graded 4 to 5.
Discussion The concept of a nerve compression syndrome in the upper extremity existing purely on a familial basis presents a therapeutic challenge to the hand surgeon. This patient illustrates the presentation, evaluation, and follow-up surgery in such a case. The theory set forth by Mayer and Garcia-Mullin 12 could explain the process in this family. They describe a genetically determined defect in Schwann cellmyelin metabolism, probably autosomal dominant in nature. The disorder may be entirely asymptomatic. However, sites along the nerve that are subject to THE JOURNAL OF HAND SURGERY
297
298
The Journal of HAND SURGERY
Lubahn and Lister
Fig. 1. A, The inset shows the Henry approach and the asterisk shows the radial nerve prior to its passing through the lateral head of the triceps muscle . B, An operative photograph with the arrow showing the radial nerve at a point prior to its passing through the lateral head of the triceps. The photograph shows the radial nerve after the fibrous arch had been divided and the radial nerve had been released along its course through the lateral head of the triceps.
chronic or intennittent compression may undergo segmental demyelination with resultant nerve palsy. Recovery could then be expected with relief of the pressure and subsequent remyelination. The decision to explore the area surgically is thus justified, particularly in this case in which compression could be localized both clinically and by EMG to the lateral head of the triceps. It could be postulated that no genetic deficit in metabolism is necessary, but that a familial predisposition to an unduly constrictive arch in the triceps could be the cause. This postulate could only be proven by exploration of all the affected members of the family. This patient's father continues to have recurrent episodes of paralysis but refuses treatment, while her sister has completely recovered. Presumptive confinnation might be forthcoming if the patient remains trouble free while both of the unoperated, older members of the family have recurrent bouts of radial palsy. We would like to thank Grace Ascher for her assistance in the preparation of the illustrations for this article.
REFERENCES I . Agnew DH: Bursal tumor producing loss of power of forearm. Am J Med Sci 46:404-5, 1863
2. Spinner M: The arcade of Frohse and its relationship to 3.
4.
5.
6.
7.
posterior interosseous nerve paralysis. J Bone Joint Surg [Br] 50:809-12, 1968 Loser R, Schafer ER: Ein Ganglion der Ellenbeuge als Ursache einer dissoziierten radialparese von unterarmtyp Darstellung eines seltenen Falles. Neurochirurgia 5: 182-6, 1972 Wu KJ, Jordan FR, Eckert C: Lipoma, a cause of paralysis of deep radial (posterior interosseous) nerve-Report of a case and review of the literature. Surgery 75: 790-5, 1974 Marshall SC, Murray WR: Deep radial nerve palsy associated with rheumatoid arthritis . Clin Orthop Rei Res 103: 157-62, 1974 Roles NC, Maudsley RH: Radial tunnel syndrome: Resistant tennis elbow as a nerve entrapment. J Bone Joint Surg [Br] 54:499-508, 1972 Lister GD, Belsole RB , Kleinert HD: The radial tunnel syndrome. J HAND SURG 4:52-9, 1979
Vol. 8, NO.3 May 1983
8. Lotem M, Fried A, Levy M, Solze p, Najenson T, Nathan M: Radial palsy following muscular effort. J Bone Joint Surg [Br] 53:500-6, 1971 9. Barton NJ: Radial nerve lesions. Hand 5:200-8, 1973 10. Woltman HW, Learmonth JR: Progressive paralysis of the nervus interosseus dorsalis. Brain 57:25-31, 1934 11. Fischer W, Behlke M: Injektionsschadigung des Ramus profundus nervi radialis. Dtsch Ges Wesen 42: 19992000, 1969 12. Mayer FR, Garcia-Mullin R: Hereditary neuropathy manifested by pressure palsies-A Schwann cell disorder? Trans Am Neurol Assoc 93:238-40, 1968
Familial radial nerve entrapment syndrome
13. Karpati G, Carpenter S, Eisen AA, Geindel W: Familial multiple peripheral nerve entrapments-An unusual manifestation of a peripheral neuropathy. Trans Am Neurol Assoc 26A:267-9, 1973 14. Behse F, Buchthal F, Carlsen F, Knappeis GG: Hereditary neuropathy with liability to pressure palsies-Electrophysiological and histopathological aspects. Brain 95:777-94, 1972 15. Earl CJ, Fullerton PM, Wakefield GS, Schutta HS: Hereditary neuropathy, with liability to pressure palsies-A clinical and electrophysiological study of four families. Q J Med 33:481-98, 1964
The short-term effects of Dexon and nylon sutures in experimental microvascular surgery-A quantitative comparison Acute inflammatory responses to 10-0 Dexon and nylon were compared 2 weeks after experimental microvascular surgery in Sprague-Dawley rats. Statistical analysis revealed no significant differences, but the strength, long-term results, and minimal surrounding tissue reaction make Dexon a viable alternative to nylon. The main disadvantage of Dexon is its opaque blue-green color, which makes it nearly invisible in the operating field. (J HAND SURG 8:299-301, 1983.)
Allan F. Cook, B.Sc., Carlos A. Azar, M.D., and Melvyn I. Dinner, F.R.C.S., Cleveland, Ohio
During the first 2 weeks following microvascular repair the anastomosed artery is completely responsible for the survival of the tissue it supplies. After 2 weeks, collateral circulation becomes established to the point where occlusion of the main artery will not automatically result in necrosis of the tissue supplied.! Numerous studies show that, during this time period, significant thickening of the arterial wall occurs, resulting in a decrease in the size of the lumen. 2 - 5 This thickening has been directly related to the
From The Cleveland Clinic Foundation, Microsurgery Laboratory, Department of Plastic and Reconstructive Surgery Received for publication March 5, 1982; accepted in revised fonn July 13, 1982. Reprint requests: Allan F. Cook, B.Sc., Microsurgery Laboratory 2R52, 9500 Euclid Ave., Cleveland, OH 44106.
Table I. Mean percentage makeup of the total cross-sectional area of the vessels studied Material
Normal arteries Dexon Nylon
10 10
10
% Vessel wall
% Vessel lumen
31.3
68.7
56.7 60.8
43.3
39.4
acute inflammatory response, within the vessel wall, to the trauma of anastomosis. In 1979 and 1980 two separate studies were done comparing absorbable and nonabsorbable suture material in microvascular surgery. 6-7 Both studies pointed out that polyglycolic acid suture material has fewer long-term ill effects than nonabsorbable sutures. The short-term effects were the same regardless of the material used.
THE JOURNAL OF HAND SURGERY
299