Kienbock's disease

Title

Other Names:

Kienbock disease; Bilateral Kienbock's disease

Categories:

Musculoskeletal Diseases

Summary Summary

Kienbock's disease is a condition characterized by interruption of blood supply to one of the small bones of the hand near the wrist (the lunate). If blood supply to a bone stops, the bone can die; this is known as osteonecrosis. Affected people may first think they have a sprained wrist and may have experienced trauma to the wrist, which can disrupt the blood flow to the lunate. As the disease progresses, signs and symptoms may include a painful and/or swollen wrist; stiffness; decreased grip strength; tenderness directly over the bone; and pain or difficulty in turning the hand upward. The underlying cause of Kienbock's disease is unknown. Treatment aims to relieve the pressure on the bone and restore blood flow within the bone. Surgery may be recommended.^[1]

Last updated: 2/10/2015

Symptoms Symptoms

Kienbock's disease most commonly affects men between the ages of 20 and 40 years, but it affects women as well. Most affected people report a history of trauma to the wrist. Symptoms can vary depending on the stage of the condition, but usually include pain that is localized to the affected area, decreased motion, swelling, and weakness in the affected hand. Rarely, the condition may occur in both hands.^[2]

Last updated: 2/10/2015

The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.

Signs and Symptoms	Approximate number of patients (when available)
Abnormality of the wrist	Very frequent (present in 80%-99% of cases)
Arthralgia	Very frequent (present in 80%-99% of cases)
Bone pain	Very frequent (present in 80%-99% of cases)
Limitation of joint mobility	Very frequent (present in 80%-99% of cases)
Osteochondritis Dissecans	Very frequent (present in 80%-99% of cases)
Osteoarthritis	Frequent (present in 30%-79% of cases)

Last updated: 9/1/2017

Inheritance Inheritance

There is currently no evidence that Kienbock's disease is inherited. However, the cause of Kienbock's disease is poorly understood. It is possible that unidentified genetic factors contribute to the development of the condition.^[3]

Last updated: 2/10/2015

Treatment Treatment

There is no complete cure for Kienbock's disease, but there are several nonsurgical and surgical options for treatment.^[1] Treatment options depend on the stage of the disease (it progresses through four stages of severity), as well as the patient’s age, hand dominance, and ability to function.^[4]^[1] The goals of treatment are to relieve the pressure on the lunate and to try to restore blood flow within the bone.^[1]

Stage I of Kienbock's disease (with symptoms similar to those of a wrist sprain) is typically treated conservatively, with noninvasive bracing and activity modification. This aims to reduce mechanical stress on the lunate bone.^[4] Pain and swelling may be managed with anti-inflammatory medications.^[1]

Surgery is typically indicated for the later stages of the disease to reduce mechanical stress on the lunate, stop disease progression, and ideally facilitate restoration of blood flow.^[4] The choice of procedure depends mainly on how far the disease has progressed.^[1]

Surgical options may include:

Revascularization - In some cases, particularly in early stages, it may be possible to return the blood supply to the lunate. This involves removing a portion of bone with attached blood vessels from another bone (usually a forearm bone or another hand bone), and inserting it into the lunate.^[1]
Joint leveling - If the two bones of the lower arm (radius and ulna) are not the same length, a joint leveling procedure may be recommended. A bone can be made shorter by removing a section of bone (as in radial osteotomy); a bone can me made longer using bone grafts (as in ulnar lengthening).^[1]
Proximal row carpectomy - In severe cases, the lunate can be removed. In this procedure, the two bones surrounding the lunate are also removed. This procedure can relieve pain while maintaining partial wrist motion.^[1]
Fusion - Nearby wrist bones can be partially or completely fused together to make one, solid bone. If the disease has progressed to severe arthritis of the wrist, fusing all of the bones of the wrist to the radius will relieve pain and and improve hand function. However, all wrist motion would be eliminated.^[1]
Metaphyseal core decompression - In this procedure, the surgeon "scrapes off" part of the radius and ulna without actually removing any osseous tissue.^[4]
Capitate-shortening osteotomy - A central bony wedge of the capitate is removed, followed by fusing the two remaining capitate bone segments.^[4]
Total wrist joint replacement (arthroplasty)^[4]

More information about the surgical treatment options for Kienbock's disease is available from Medscape Reference.

Last updated: 1/24/2017

Prognosis Prognosis

The long-term outlook (prognosis) for people with Kienbock's disease is hard to predict and the natural history of the disease is not well understood.^[5] Kienbock's disease varies considerably in both severity and rate of progression.^[1] Some people with Kienbock's disease have no symptoms, while others have variable symptoms ranging from mild and intermittent, to severe and debilitating. Furthermore, the severity of the condition as seen on imaging studies does not always correlate with the degree of symptoms a person has.^[5]

Each person's response to treatment depends on the degree of damage to the lunate and surrounding wrist bones. Some people may need more than one procedure if the disease continues to progress. It has been suggested that people who are diagnosed and treated later in life tend not to do as well as those diagnosed and treated earlier. In general, people with Kienbock's disease should not expect to return to normal wrist function, even after treatment. However, treatment gives the greatest opportunity for long-term preservation of function and pain relief.^[1]^[6]

Last updated: 2/12/2015

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Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

National Osteonecrosis Foundation
Good Samaritan Professional Building
Suite 201
5601 Loch Raven Blvd.
Baltimore, MD 21239
Telephone: 410-532-5985
Fax: 410-532-5908
Website: http://www.nonf.org/index.html

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Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

The American Academy of Orthopaedic Surgeons has information on Kienbock's disease. Click on the link above to view this information page.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The Merck Manual provides information on this condition for patients and caregivers.
The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) support research into the causes, treatment, and prevention of arthritis and musculoskeletal and skin diseases, the training of basic and clinical scientists to carry out this research, and the dissemination of information on research progress in these diseases. Click on the link to view information on this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Kienbock's disease. Click on the link to view a sample search on this topic.

News & Events News & Events

News

Don’t Miss the NCATS Toolkit for Patient-Focused Therapy Development Event
August 31, 2017
Funding Opportunities Now Available for the Undiagnosed Diseases Network (UDN) Phase II
August 30, 2017
IRDiRC Goals 2017-2027: New Rare Disease Research Goals for the Next Decade
August 10, 2017
Young Investigator Initiative Career Development and Grant Mentoring Program
June 21, 2016

Related Diseases Related Diseases

The following diseases are related to Kienbock's disease. If you have a question about any of these diseases, you can contact GARD.

Osteochondrosis

GARD Answers GARD Answers

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How long does it take for this disease to develop? See answer
Is it hereditary? See answer
Is there a cure for Kienbock's disease that doesn't involve surgery? See answer

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References References

Charles D. Jennings. Kienbock's Disease. American Academy of Orthopaedic Surgeons. May, 2012; http://orthoinfo.aaos.org/topic.cfm?topic=a00017.
Cross D, Matullo KS. Kienböck disease. Orthop Clin North Am. January, 2014; 45(1):141-152. Accessed 2/10/2015.
Stahl S et al.,. An international opinion research survey of the etiology, diagnosis, therapy and outcome of Kienböck’s disease (KD). Chirurgie de la main. 2012; 31:128–137. http://www.ncbi.nlm.nih.gov/pubmed/22609038.
Carissa White, Prosper Benhaim, Benjamin Plotkin. Treatments for Kienböck disease: what the radiologist needs to know. Skeletal Radiology. April, 2016; 45(4):531-540.
Lutsky K, Beredjiklian PK.. Kienbock disease. J Hand Surg Am. September, 2012; 37(9):1942-1952.
Martin GR, Squire D. Long-term outcomes for Kienböck's disease. Hand (N Y). March, 2013; 8(1):23-26.
Takeshi Ogawa, Naoyuki Ochiai, Yasumasa Nishiura, Toshikazu Tanaka, and Yuki Hara. A new treatment strategy for Kienböck’s disease: combination of bone marrow transfusion, low-intensity pulsed ultrasound therapy, and external fixation. J Orthop Sci. March, 2013; 18(2):230-237.