eMedicine Specialties > Orthopedic Surgery > Hand & Upper Extremity

Ulnar Clubhand

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
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

Updated: Oct 19, 2007

Introduction

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

Workup

Imaging Studies

• Radiographic evaluation of the elbow, forearm, wrist, and hand: Serial radiographs can be useful in assessing the degree and course of the aplasia.
• Magnetic resonance imaging (MRI): MRI can be used to delineate noncalcified tissues such as the fibrocartilaginous anlage. This modality is also useful for studying the joint surfaces in the largely unossified cartilaginous articulations.

Other Tests

• All affected patients should undergo a detailed evaluation by a hand and upper limb therapist to assess hand function. Ongoing evaluation during treatment is important to avoid restriction of hand and elbow function (see Complications). In addition, appropriate genetic evaluation and counseling for individuals with syndromic ulnar aplasia is important in certain instances.

Treatment

Surgical Therapy

• Ulnar deviation – Casting from birth, long-arm cast, gradual stretching of the tight ulnar structures. (Mild cases will correct by age 6 mo.)
• Anlage
  • Early presentation – Excise the anlage before the occurrence of radial bowing or radial head dislocation, around age 6 months. The procedure is performed through an ulnar incision along the wrist. Care is taken to protect the ulnar artery and nerve. Excision of the entire anlage is not necessary; at least 50% should be excised. The wrist is placed in maximal radial deviation in a long-arm cast for a month. Night splints are worn for up to 6 months to maintain the correction.
  • Late presentation – Excise the entire anlage. Z-plasty of the skin and selective tenotomies may be necessary.
  • Radiohumeral synostosis – The anlage should be excised, as the anlage tether can cause progressive bowing.
• Bowing of the radius – Radial corrective osteotomy
• Forearm is in a less than useful rotation - Radial corrective osteotomy. (It is better to wait until the child is mature enough to make a decision about the appropriate position.)
• Radial head dislocation – Observe, resection only, or resection with the creation of a single-bone forearm. (Ulnar osteotomy has been performed with or without shortening, with open reduction and stabilization of the radial head and plus/minus early radial head prosthetic replacement.² A single-bone forearm is created through a posterior approach, protecting the posterior interosseous nerve. A sufficient amount of proximal radius is excised to achieve proper approximation and alignment with the ulna. An intramedullary nail or a plate is used for stabilizing the fusion.)
• Unstable elbow – Ulnohumeral or radiohumeral arthrodesis or elbow ligamentous reconstruction
• Radiohumeral synostosis
  • Young children: Excise the ulnar anlage.
  • Older children: Perform a distal humeral osteotomy to improve forearm alignment.

Distraction reconstruction treatment

Paley and Herzenberg combined the classifications of Kummel, Ogden, Swanson, and Johnson and Omer to create a classification that relates directly to the treatment strategies of ulnar deficiencies of the forearm as follows³ :

• Type 1 – Hypoplastic ulna with the distal epiphysis intact. This type is the most common with congenital dislocation of the radial head.
• Type 2 – Absence of the distal third of the ulna
• Type 3 – Absence of the distal two thirds of the ulna
• Type 4 – Complete absence of the ulna
• Type 5 – Radiohumeral synostosis

Treatment algorithms

Type 1 without radial head dislocation

This type is treated with ulnar lengthening, radius shortening, or both. Large amounts of radial shortening can result in radial head instability and dislocation. The radial bow is also corrected through the distraction osteotomy.³ The treatment is repeated throughout childhood as the limb grows and as the discrepancy increases.

Type 1 with radial head dislocation

The radial head dislocation probably represents a relatively longer radius in the presence of a short ulna. The radial head can be left in its dislocated position if the forearm rotation is well maintained along with elbow stability and mobility, and the radial head is not tender. Radial head reduction requires one of the following:

• Ulnar corrective osteotomy and radial shortening (similar to an untreated Monteggia fracture-dislocation)
• Ulnar corrective osteotomy and lengthening – The distal radioulnar relationship is preserved with a wire that transfixes the 2 bones. As the ulna lengthens, the radius is pulled distally, reducing the radial head. Following reduction, additional proximal radioulnar joint reconstruction may be needed. It may also be necessary to extend the external fixation to the humerus via a hinged elbow.

Types 2 and 3 with radial head dislocation

The ulnar clubhand appearance is pronounced with these 2 types. The primary goal of treatment is to correct the alignment of the hand and forearm. If only the distal third of the ulna is absent, the ulna can be transported distally to support the carpus. If the distal two thirds of the ulna is absent, creation of a single-bone forearm is a better alternative. If forearm rotation is good, all attempts should be made to transport the ulna distally.

The radius is osteotomized at 1 or 2 places, and the alignment is corrected. The ulna is osteotomized and distracted independently or with the radius.

Type 4

The ulnar clubhand deformity and elbow stability are the main issues to consider. Both can be improved with a corrective osteotomy to realign the hand and forearm. Lengthening requires extension of the external fixation to the humerus via a hinged elbow external fixator.

Type 5

An elbow-level osteotomy is performed to correct the elbow position. A radial osteotomy, ulnar osteotomy, or both can be used to realign forearm rotation and lengthen the forearm.

Complications

Most children learn to cope functionally with their condition.

One of the common complications that is seen in the lengthening reconstruction treatment of ulnar deficiencies of the forearm is the tightening of the flexor tendons to the digits, which restricts hand function.

Dislocation of an unstable elbow is also a potential complication. This can be avoided by protecting the elbow with a hinged fixator.

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].

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

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.

© 1994- by Medscape.
All Rights Reserved
(http://www.medscape.com/public/copyright)