eMedicine Specialties > Orthopedic Surgery > Hand & Upper Extremity

Ulnar Clubhand

Author: Minoo Patel, MBBS, MD, MS, FRACS, Senior Lecturer, Monash University, Melbourne, Australia; Consulting Adult/Pediatric Orthopedic Surgeon, Department of Orthopedic Surgery, Monash Medical Center, Melbourne, Australia
Coauthor(s): John Herzenberg, MD, FRCSC, Professor, Department of Orthopedic Surgery, Associate Professor, Department of Pediatrics, University of Maryland Medical School, Codirector of International Center for Limb Lengthening, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Contributor Information and Disclosures

Updated: Oct 19, 2007

Introduction

Ulnar clubhand is much less common than radial clubhand and is more appropriately referred to as ulnar deficiencies of the forearm. Most cases are sporadic in occurrence, although genetic syndromes are associated with ulnar dysplasia. Focal dermal hyperplasia, or Goltz syndrome (also known as Goltz-Gorlin syndrome), is an X-linked dominant condition that consists of ulnar dysplasia, long-bone defects, and split-hand or split-foot, with skin atrophy; anal, vulval, or lip papillomata; microphthalmia; iris coloboma; and mental retardation. Split-hand and split-foot with ulnar dysplasia have an autosomal dominant inheritance. An ulnar defect with mammary gland aplasia syndrome is associated with polydactyly or camptodactyly. (See also the eMedicine article Focal Dermal Hypoplasia Syndrome.)

Close to 70% of cases of ulnar clubhand are unilateral. A partial rather than complete absence of the ulna is common. Ulnar shortening with radial bowing and abnormal digits is the classic presentation of this condition, and digital anomalies can be found in close to 90% of cases with postaxial absence of ulnar rays.1 The fourth and fifth metacarpals, as well as the capitate, lunate, triquetrum, hamate, and pisiform, are absent or deformed.

Synostosis with the humerus is possible when the ulna is present; radiohumeral synostosis can also be present. The radial head is dislocated in close to 50% of cases.

Radial bowing is produced by the tethering effect of the fibrocartilaginous ulnar anlage, which can also tether the carpus, producing limitation of wrist movement. The radiocarpal joint has a fixed ulnar deviation in these cases. In most cases, however, function is very good, and the carpus can be actively centralized. The presence of a thumb makes for a functional hand, unlike the case for radial clubhands. (See also the eMedicine article Radial Clubhand.)

Frequency

Ulnar deficiency of the forearm is much less common than the occurrence of radial clubhand.

Etiology

Most cases of ulnar deficiency of the forearm are sporadic in occurrence, although genetic syndromes are associated with ulnar dysplasia (see Introduction).

Presentation

The patient's affected upper limb is examined in supination, pronation, extension, and flexion. Clinical considerations of this condition include the following:

  • Ulnar deviation of the hand
  • Absent ulnar digits
  • Syndactyly
  • Elbow stability
  • Elbow stiffness
  • Limited pronation, supination, or both
  • Radial head subluxation or dislocation
  • Deficient carpal bones
  • Stable wrist
  • Upper limb-length discrepancy

Classification

According to the traditional classification, there are 4 types of ulnar deficiencies of the forearm, which can have varying degrees of radial bow, with or without radial head dislocation. The classification is as follows:

  • Type 1 – Ulnar shortening (distally) with minor radial bow
    • Hypoplasia of the ulna
    • Proximal and distal epiphyses present
    • Hypoplastic or absent ulnar digits
    • Minimal radial bowing
  • Type 2 – Significant ulnar shortening with a fibrocartilaginous anlage attached to the ulnar carpus, with significant radial bowing
    • Partial aplasia of the ulna, distal third
    • Distal ulnar anlage
    • Bowed radius with anlage acting as a tether
    • Presence/absence of progressive radial head dislocation
  • Type 3 – Complete absence of the ulna
    • Unstable elbow
    • Straight radius
  • Type 4  – Complete absence of the ulna, with a fibrocartilaginous anlage attached to the ulnar carpus
    • Radiohumeral synostosis at the elbow
    • Bowed radius

Indications

See Treatment, Surgical therapy.

Contraindications

Nonspecific contraindications to ulnar clubhand surgery are as follows:

  • The presence of no or minimal functional deficit. In such cases, the surgeon is wary of making the condition functionally worse. (In most cases, the hand is reasonably functional, and correction of the forearm deformity is required to position the hand in a more favorable position in space.)
  • Any surgical risk factor due to associated syndromes
  • A severely affected limb and poor neurologic function

More on Ulnar Clubhand

Overview: Ulnar Clubhand
Workup: Ulnar Clubhand
Treatment: Ulnar Clubhand
References
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References

  1. Swanson AB, Tada K, Yonenobu K. Ulnar ray deficiency: its various manifestations. J Hand Surg [Am]. Sep 1984;9(5):658-64. [Medline].

  2. Tetsworth K, Krome J, Paley D. Lengthening and deformity correction of the upper extremity by the Ilizarov technique. Orthop Clin North Am. Oct 1991;22(4):689-713. [Medline].

  3. Paley D, Herzenberg JE. Distraction treatment of the forearm. In: Buck-Gramcko D, ed. Congenital Malformations of the Hand and Forearm. London, UK: Churchill Livingstone; 1998:90-2.

  4. El Hassan B, Biafora S, Light T. Clinical manifestations of type IV ulna longitudinal dysplasia. J Hand Surg [Am]. Sep 2007;32(7):1024-30. [Medline].

  5. Foucher G. [Toe transplantation in congenital malformations of the hand] [French]. Bull Acad Natl Med. Nov 1997;181(8):1737-44; discussion 1744-5. [Medline].

  6. Kakarala G, Kavarthapu V, Lahoti O. Distraction osteogenesis to improve limb function in congenital bilateral humeroradioulnar synostosis. Acta Orthop Belg. Dec 2006;72(6):765-8. [Medline].

  7. Malpas T, Anderson N, Langley S. Ulnar club-hand and constriction-ring syndrome. Pediatr Radiol. 1995;25(3):233-4. [Medline].

  8. Minguella-Solá J, Cabrera-González M, Escolá-Teixidó J. [Radial club-hand and contralateral pre-axial polydactyly and ulnar club-hand and contralateral post-axial polydactyly. Report of 2 unusual cases] [Spanish]. An Esp Pediatr. Jul 1999;51(1):68-70. [Medline].

  9. Saffar P. Ulna oblique osteotomy for radius and ulna length inequality: technique and applications. Tech Hand Up Extrem Surg. Mar 2006;10(1):47-53. [Medline].

  10. Wang AA, Hutchinson DT. Use of the elbow compass universal hinge in pediatric patients. J Pediatr Orthop. Jan-Feb 2006;26(1):58-60. [Medline].

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Further Reading

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Keywords

ulnar dysplasia, ulnar aplasia, ulnar deficiencies of the forearm, split hand, split foot, polydactyly, camptodactyly, focal dermal hyperplasia, Goltz syndrome, Goltz-Gorlin syndrome, skin atrophy, anal papillomata, vulval papillomata, lip papillomata, microphthalmia, iris coloboma, mental retardation, mammary gland aplasia syndrome

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Contributor Information and Disclosures

Author

Minoo Patel, MBBS, MD, MS, FRACS, Senior Lecturer, Monash University, Melbourne, Australia; Consulting Adult/Pediatric Orthopedic Surgeon, Department of Orthopedic Surgery, Monash Medical Center, Melbourne, Australia
Minoo Patel, MBBS, MD, MS, FRACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, AO Foundation, Australian Association of Surgeons, Australian Medical Association, Australian Orthopaedic Association, Orthopaedic Research Society, Orthopaedics Overseas, Pediatric Orthopaedic Society of North America, and Royal Australasian College of Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

John Herzenberg, MD, FRCSC, Professor, Department of Orthopedic Surgery, Associate Professor, Department of Pediatrics, University of Maryland Medical School, Codirector of International Center for Limb Lengthening, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
John Herzenberg, MD, FRCSC is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Orthopaedic Surgeons, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Medical Editor

Joseph E Sheppard, MD, Director of Hand and Upper Extremity, Associate Professor, Department of Orthopedic Surgery, University of Arizona
Joseph E Sheppard, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Medical Association, American Society for Surgery of the Hand, Southern Orthopaedic Association, and Western Orthopaedic Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

CME Editor

Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital
Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Association of Physicians of Indian Origin, American College of International Physicians, and American College of Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD, Consulting Surgeon, Broward Hand Center, Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami School of Medicine
Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society
Disclosure: Nothing to disclose.

 
 
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