Skip to main content
SpringerLink
Log in

A Case of Congenital Bilateral Absence of Elbow Flexor Muscles: Review of Differential Diagnosis and Treatment

  • Original Article
  • Published:
HAND

Abstract

A 1-year-old boy presented to us with congenital inability to flex his elbow. He had bilaterally absent biceps brachii and brachialis muscles, a rare condition. We performed pedicle latissimus dorsi musculocutaneous flaps to the left and right volar upper arm at 21 and 24 months of age, respectively, to create elbow flexors. By 4 years of age, he had excellent elbow flexion bilaterally with strength grade in excess of 4.5. In addition to discussing our patient’s treatment options, we discuss other potential causes of weak elbow flexion when faced with this clinical dilemma.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Figure 1
Figure 2
Figure 3

Similar content being viewed by others

References

  1. Aicardi J. Arhtogyrposis multiplex congenital. Diseases of the Nervous System in Childhood. Oxford: Blackwood Scientific Publications; 1992. p. 1210–1.

    Google Scholar 

  2. Akasaka Y, Hara T, Takahashi M. Free muscle transplantation combined with intercostals nerve crossing for reconstruction for elbow flexion and wrist extension in brachial plexus injuries. Microsurg 1991;12:346–51.

    Article  CAS  Google Scholar 

  3. Azzarelli B, Roessmann U. Diffuse anoxic myelopathy. Neurology 1977;27:1049–52.

    PubMed  CAS  Google Scholar 

  4. Baliarsing AS, Doi K, Hattori Y. Bilateral elbow flexion reconstruction with functioning free muscle transfer for obstetric brachial plexus palsy. J Hand Surg 2002;27B:484–6.

    Google Scholar 

  5. Banker BQ, Victor M, Adams RD. Arthrogryposis multiplex due to congenital muscular dystrophy. Brain 1957;80:319–34.

    Article  PubMed  CAS  Google Scholar 

  6. Banker BQ. Neuropathological aspects of arthrogryposis multiplex congenital. Clin Orthop 1985;194:30–43.

    PubMed  Google Scholar 

  7. Bateman JE. Nerve injuries about the shoulder in sports. J Bone Joint Surg Am 1967;49:785–92.

    PubMed  CAS  Google Scholar 

  8. Braddom RL, Wolfe C. Musculocuteneous nerve injury after heavy exercise. Arch phys Med Rehabil 1978;58:290–3.

    Google Scholar 

  9. Brodtkorb E, Torbergsen T, Nakken KO, Andersen K, Gimse R, Sjaastad O. Epileptic seizures, arthrogryposis, and migrational brain disorders: a syndrome? Acta Neurol Scand 1994;90:232–40.

    Article  PubMed  CAS  Google Scholar 

  10. Brooke MH, Kaiser KK. The use and abuse of muscle histochemistry. Ann New York Acad Sci 1974;228:121.

    Article  CAS  Google Scholar 

  11. Brooke MH. A Clinician’s View of Neuromuscular Disease. 2nd ed. Baltimore, MD: Williams & Wilkins; 1986.

    Google Scholar 

  12. Carroll RE, Kleinman WB. Pectoralis major transplantation to restore elbow function to the paralytic limb. J Hand Surg 1979;4:501–7.

    CAS  Google Scholar 

  13. Carroll RE. Restoration of elbow function by transplantation of the triceps tendon. Surg Gynecol Obstet 1952;95:685–8.

    PubMed  CAS  Google Scholar 

  14. Chuang DC, Epstein MD, Yeh MC, Wei FC. Functional restoration of elbow function in brachial plexus injuries: results in 167 patients (excluding obstetrical brachial plexus injury). J Hand Surg 1993;18A:285–91.

    Google Scholar 

  15. Clarren SK, Hall JG. Neuropathological findings in the spinal cords of 10 infants with arthrogryposis. J Neurol Sci 1983;58:89–102.

    Article  PubMed  CAS  Google Scholar 

  16. David TJ. Nature and etiology of Poland anomaly. N England J Med 1972;287:487–9.

    Article  CAS  Google Scholar 

  17. Dundore DE, DeLisa JA. Musculocutaneous nerve palsy: an isolated complication of surgery. Arch Phys Med Rehabil 1979;60:130–3.

    PubMed  CAS  Google Scholar 

  18. Ethans KD, Leahy JL. Congenital bilateral absence of the elbow flexors. Am J Phys Med Rehabil 2001;80:759–61.

    Article  PubMed  CAS  Google Scholar 

  19. Ewing MR. Postoperative paralysis in the upper extremity: report of five cases. Lancet 1950;1:99–103.

    Article  PubMed  CAS  Google Scholar 

  20. Fowler M. Case of arthrogryposis multiplex congenital with lesions in the nervous system. Arch Dis Child 1957;34:505–10.

    Google Scholar 

  21. Gagnon E, Fogelson N, Seyfer AE. Use of latissimus dorsi muscle to restore elbow flexion in arthrogryposis. Plast Reconstr Surg 2000;106(7):1582–5.

    Article  PubMed  CAS  Google Scholar 

  22. Gherman RB, Ouzounian JG, Miller DA, Kwok L, Goodwin TM. Spontaneous vaginal delivery: a risk factor for Erb’s palsy? Am J Obstet Gynecol 1998;178:423–7.

    Article  PubMed  CAS  Google Scholar 

  23. Gordon N. Arthrogryposis multiplex congenital. Brain Dev 1998;20:507–11 (Review, Oct).

    Article  PubMed  CAS  Google Scholar 

  24. Hageman G, Ramaekers VT, Hilhorst BG, Rozeboom AR. Congenital cervical spinal muscular atrophy: a non-familial, non-progressive condition of the upper limbs. J Neurol Neosurg Psych 1993;56:365–8.

    Article  CAS  Google Scholar 

  25. Hall JG, Reed SD, Driscoll EP. Part 1. Amyoplasia: a common sporadic condition with congenital contractures. Am J Med Genet 1983;15:571–90.

    Article  PubMed  CAS  Google Scholar 

  26. Hardy AE. Birth injuries of the brachial plexus: incidence and prognosis. J Bone Joint Surg 1981;63B:98–101.

    Google Scholar 

  27. Hirayama T, Tada H, Katsuki M, Yoshida E. The pedicle latissimus dorsi transfer for reconstruction of the plexus brachialis and brachium. Clin Orthop Relat Re 1994;309:201–7.

    Google Scholar 

  28. Horoupian DS, Yoon JJ. Neuropathic arthrogryposis multiplex congenita and intrauterine ischemia of anterior horn cells: a hypothesis. Clin Neuropathol 1988;7:285–93.

    PubMed  CAS  Google Scholar 

  29. Ireland DCR, Takayama N, Flatt AE. Poland’s syndrome. A review of 43 cases. J Bone Joint Surg 1976;58A:52–8.

    Google Scholar 

  30. Jennett RJ, Tarby TJ, Kreinick CJ. Brachial plexus palsy: an old problem revisited. Am J Obstet Gynecol 1992;166:1673–6; discussion 1676–7.

    PubMed  CAS  Google Scholar 

  31. Kaiboriboon K, Hayat GR. Congenital cervical spinal atrophy: an intrauterine hypoxic insult. Neuropediatr 2001;32:330–4.

    CAS  Google Scholar 

  32. Kay SPJ. Arthrogryposis. In Hotchkiss RN, Pederson WC, editors, Green’s Operative Hand Surgery. 4th ed. Philadelphia: Churchill Livingstone; 1999. p. 473–80.

    Google Scholar 

  33. Kulhman KA, Batley RJ. Bilateral musculocuteneous nerve palsy: a case report. Am J Phys Med Rehabil 1996;75:227–31.

    Article  Google Scholar 

  34. Laubscher B, Janzer RC, Krahenbuhl S, Hirt L, Deonna T. Ragged red fibers and complex I deficiency in a neonate with arthrogryposis congenita. Pediatr Neurol 1997;17:249–51.

    Article  PubMed  CAS  Google Scholar 

  35. Mastaglia FL. Musculocutaneous neuropathy after strenuous physical activity. Med J Aust 1986;145:153–4.

    PubMed  CAS  Google Scholar 

  36. Mayerl, G. Experiences with the Steindler flexorplasty at the elbow. J Bone Joint Surg 1954;36A:775–89.

    Google Scholar 

  37. McMutry RY, Jochims JL. Congenital deficiency of the extrinsic extensor mechanism of the hand. Clin Orthop 1977;125:36–9.

    Google Scholar 

  38. Menelaus MB. Radial club hand with absence of the biceps muscle treated by centralization of the ulna and triceps transfer. J Bone Joint Surg 1976;58B:488–91.

    Google Scholar 

  39. Miles JM, Gilbert-Barness E. Pathological cases of the month. Type 1 spinal muscular atrophy (Werdnig–Hoffman disease). Am J Dis Child 1993;147:907–8.

    PubMed  CAS  Google Scholar 

  40. Muntoni F, Voit T. The congenital muscular dystrophies in 2004: a century of exciting progress. Neuromuscul Disord 2004;14:635–49.

    Article  PubMed  Google Scholar 

  41. Narbona J, Mestre M, Beguiristain JL, Martinez-Lage JM. Intramedullary telangiectasia causing congenital cervical spinal atrophy. Muscle Nerve 1982;5:256.

    PubMed  CAS  Google Scholar 

  42. O’Ceallaigh S, Mehboob Ali KS, O’Connor TP. Functional latissimus dorsi muscle transfer to restore elbow flexion in extensive electrical burns. Burns 2005;31:113–5.

    Article  PubMed  CAS  Google Scholar 

  43. Pearn J. Classification of spinal muscular atrophies. Lancet 1980;1:919–22.

    Article  PubMed  CAS  Google Scholar 

  44. Pearn JH. Fetal movements and Werdnig–Hoffman disease. J Neurol Sci 1973;18:373–9.

    Article  PubMed  CAS  Google Scholar 

  45. Pearn JH. The gene frequency of acute Werdnig–Hoffman disease (SMA type 1): a total population study of northeast England. J Med Genet 1973;10:260–5.

    PubMed  CAS  Google Scholar 

  46. Pena CE, Miller F, Budzilovich GN, Feigin I. Arthrogryposis multiplex congenital: report of two cases of a radicular type with familial incidence. Neurology 1968;13:926–30.

    Google Scholar 

  47. Raio L, Ghezzi F, Di Naro E, Buttarelli M, Franchi M, Durig P, et al. Perinatal outcome of fetuses with a birth weight greater than 4500 g: an analysis of 3356 cases. Eur J Obstet Gynecol Reprod Biol 2003;109(2):160–5.

    Article  PubMed  Google Scholar 

  48. Reimersma S, Vincent A, Beeson D, Newland C, Hawke S, Vernet-der Garabedian B, et al. Association of arthrogryposis multiplex congenital with maternal antibodies inhibiting fetal acetylcholine receptor function. J Clin Invest 1996;98:2358–63.

    Article  Google Scholar 

  49. Riordan DC. Congenital absence of the radius. J Bone Joint Surg 1955;37A:1129–40.

    Google Scholar 

  50. Rivet D, Boileau R, Saiveau M, Baudet J. Restoration of elbow flexion using the latissimus dorsi musculo-cutaneous flap. Ann Chir Main 1989;8:110–23.

    Article  PubMed  CAS  Google Scholar 

  51. Scott CI. Pterygium syndrome. The first conference on the clinical delineation of birth defects. Part II. Malformation syndromes. Birth Defects V, 1969;231–2.

  52. Smit LME, Barth PG. Arthrogryposis multiplex congenital due to congenital myasthenia. Develop Med Child Neurol 1980;22:371–4.

    Article  PubMed  CAS  Google Scholar 

  53. Sombekke BH, Molenaar WM, van Essen AJ, Schoots CJ. Lethal congenital muscular dystrophy with arthrogryposis multiplex congenital: three new cases and review of the literature. Pediatr Pathol 1994;14:277–85.

    Article  PubMed  CAS  Google Scholar 

  54. Stern PJ, Carey JP. The latissimus dorsi flap for reconstruction of the brachium and shoulder. J Bone Joint Surg Am 1988;70:526–35.

    PubMed  CAS  Google Scholar 

  55. Stern PJ, Caudle RJ. Tendon transfers for elbow flexion. Hand Clin 1988;4(2):297–307.

    PubMed  CAS  Google Scholar 

  56. Trojaborg W. Motor and sensory conduction in the musculocutaneous nerve. J Neurol Neurosurg Psych 1976;39:890–9.

    CAS  Google Scholar 

  57. Vajsar J, Sloane A, MacGregor DL, Ronen GM, Becker LE, Jay V. Arthrogryposis multiplex congenital due to congenital myasthenic syndrome. Pediatr Neurol 1995;12:237–41.

    Article  PubMed  CAS  Google Scholar 

  58. Van Heest A, Simmons B. Surgical treatment of arthrogryposis of the elbow. J Hand Surg 1998;23(6):1063–70.

    Article  Google Scholar 

  59. Vuopala K, Leisti J, Herva R. Lethal arthrogryposis with anterior horn cell disease. Neuropediatr 1994;25:308–15.

    CAS  Google Scholar 

  60. Yuill GM, Lynch PG. Congenital non-progressive peripheral neuropathy with arthrogryposis multiplex. J Neurol Neurosurg Psych 1974;37:316–23.

    CAS  Google Scholar 

  61. Zafeiriou DI, Pitt M, de Sousa C. Clinical and neurophysiological characteristics of congenital myasthenic syndromes presenting in early infancy. Brain Dev 2004;26:47–52.

    Article  PubMed  Google Scholar 

  62. Zancolli E, Mitre H. Latissimus dorsi transfer to restore elbow flexion. An appraisal of eight cases. J Bone Joint Surg Am 1973;55:1265–75.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Plastic Surgery, Baylor College of Medicine, 1 Baylor Plaza, Houston, TX, 77030, USA

    David T. Netscher & Eric Lewis

  2. Department of Orthopedic Surgery, Baylor College of Medicine, Houston, TX, 77030, USA

    David T. Netscher

  3. Hand Surgery Service, Texas Children’s Hospital, Houston, TX, 77030, USA

    David T. Netscher

  4. Plastic Surgery Section, Veterans Affairs Medical Center, 6624 Fannin St. #2730, Houston, TX, 77030, USA

    David T. Netscher

  5. Office of Student Affairs, Baylor College of Medicine, 1 Baylor Plaza, Houston, TX, 77030, USA

    Oluseyi Aliu & Saleh Samra

Authors
  1. David T. Netscher
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Oluseyi Aliu
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Saleh Samra
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Eric Lewis
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to David T. Netscher.

About this article

Cite this article

Netscher, D.T., Aliu, O., Samra, S. et al. A Case of Congenital Bilateral Absence of Elbow Flexor Muscles: Review of Differential Diagnosis and Treatment. HAND 3, 4–12 (2008). https://doi.org/10.1007/s11552-007-9056-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11552-007-9056-0

Keywords

Search

Navigation