National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Kienböck Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Related Disorders List
Information on the following diseases can be found in the Related Disorders section of this report:
- Carpal Tunnel Syndrome
- Sudeck's Atrophy
- Juvenile Osteoporosis
Kienböck Disease is an acquired bone disorder. Abnormalities of the lunate bone in the wrist develops following an injury or inflammation. Recurrent pain and stiffness occur in conjunction with thickening, swelling and tenderness in soft tissue overlying the lunate bone. The range of motion in the wrist may become limited.
Kienböck Disease is characterized by degenerative changes in the lunate bone of the wrist. Softening, deterioration, fragmentation or compression of the affected bone can occur. These changes may produce pain, swelling, tenderness, thickening and/or stiffness in the overlying tissues of the wrist. The range of motion may become restricted. Many individuals with Kienböck Disease have a shorter ulna than radius (negative ulnar variance). Healing occurs through formation of new bone in some cases.
The exact cause of Kienböck Disease is not known. However, it is believed to be caused by inflammation or injury of the wrist.
Kienböck Disease usually begins during childhood and seems to affect females more often than males.
Symptoms of the following disorders can be similar to those of Kienböck Disease. Comparisons may be useful for a differential diagnosis:
Carpal Tunnel Syndrome is a condition caused by compression of peripheral nerves in the wrist, affecting one or both hands. It is characterized by a sensation of numbness, tingling, burning and/or pain in the hand and wrist. Persons affected by this disorder may be awakened at night with the feeling that the hand has "gone to sleep". Various other diseases may occur in conjunction with this condition. With timely treatment, the prognosis in most cases is favorable. (For more information on this disorder, please choose "Carpal Tunnel" as your search term online.)
Sudeck's Atrophy, also known as post-traumatic osteoporosis, is marked by an acute atrophy of the bones. The wrist and ankle bones are most commonly affected, following a slight injury such as a sprain.
Juvenile Osteoporosis is marked by a porous condition or atrophy of bone tissue beginning before puberty. The exact cause has not been identified. This condition can lead to pain or fractures in many bones of the body including the wrist. Spontaneous remission may occur within several years.
Arthroscopic procedures, CT scan and/or x-ray imaging may be used for diagnosis.
The treatment of Kienböck's Disease may involve surgery of the lunate bone, shortening of the radial bone, lengthening of the ulna or the stiffening of the joints through an operation (arthrodesis). Cutting off the nerve supply of the wrist (wrist denervation) has been used in conjunction with some of the other procedures. Surgical options depend on how far the disease has progressed and the best option will differ for each individual.
If inflammation of the wrist has occurred, drug treatment may be recommended. Other treatment is symptomatic and supportive.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
Current Diagnosis & Treatment in Orthopedics, 1st Ed.: Harry B. Skinner, Editor; Appleton & Lange, 1995. P. 494.
Excision of the Lunate in Kienböck's Disease. Results After Long-Term Followup. H. Kawai et al.; J Bone Joint Surg [Br] (Mar 1988; 70(2)). Pp. 287-92.
Ulna-Minus Variance and Kienböck's Disease. P. A. Nathan et al.; J Hand Surg (Sept 1987; 12(5 pt 1)). Pp. 777-78.
Wrist Denervation Procedures in the Treatment of Kienböck's Disease. D. Buck-Gramcko; Hand Clin (Aug 1993; 9(3)). Pp. 517-20.
Review of Treatment Results in Kienböck's Disease. S. H. Kuschner et al.; Orthop Rev (Jun 1992; 21(6)). Pp. 717-28.
Osteomalacia of the Lunate Bone-Indications for and Limitations of Radial Shortening and Ulnar Lengthening. M. Fishcer et al.; Orthopade (Feb 1993; 22(1)). Pp. 52-56.
Long-Term Results of 9 Cases of Elongation of the Ulna in Treatment of Kienböck's Disease. P. Ducarmois et al.; Ann Chir Main Memb Supter (1997; 16(1)). Pp. 16-24.
Kienböck's Disease: Treatment by Implantation of Vascular Pedicle and Bone Grafting. M. S. Moneim et al.; Iowa Orthop J (1998; 18). Pp. 67-73.
Kienböck Disease and Negative Ulnar Variance. M. Bonzar et al.; J Bone Joint Surg Am (Aug 1998; 80(8)). Pp. 1154-57.
Arthroscopic Evaluation of Radial Osteotomy for Kienböck's Disease. K. Watanabe et al.; J Hand Surg (Sept 1998; 23(5)). Pp. 899-903.
Proximal Row Carpectomy Versus Limited Wrist Arthrodesis for Advanced Kienböck's Disease. R. Nakamura et al.; J Hand Surg (Dec 1998; 23(6)). Pp. 741-45.
FROM THE INTERNET
eMedicine - Kienböck Disease : Article by Brian J Divelbiss, MD
NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases
One AMS Circle
Bethesda, MD 20892-3675
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email firstname.lastname@example.org
Last Updated: 8/8/2007
Copyright 1989, 1995, 1999, 2007 National Organization for Rare Disorders, Inc.
Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.