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Disease Profile

Kienbock’s disease

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

-

ICD-10

M92.2 M93.1

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Kienbock disease; Bilateral Kienbock's disease

Categories

Musculoskeletal Diseases

Summary

Kienbock's disease causes one of the bones in the wrist (lunate bone) to slowly break down due to low blood supply. This process is called osteonecrosis. Symptoms include pain, swelling, limited movement, and decreased grip strength. The symptoms usually start in adulthood, but can occur at any age. Kienbock's disease usually slowly gets worse over time. The underlying cause of Kienbock's disease is unknown. Diagnosis is made by imaging studies such as an MRI or CT scan. Treatment aims to relieve pressure and restore blood flow within the bone, and includes both surgical and non-surgical methods.[1][2][3]

Symptoms

The following list includes the most common signs and symptoms in people with Kienbock's disease. These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition.

Symptoms may include:[4]

  • Wrist pain
  • Limitation of movement
  • Loss of grip strength
  • Joint swelling

It is not well understood how Kienbock's disease changes over time. Most people start to develop symptoms in early adulthood. Typically, only one wrist is affected. Over time, the wrist joint may break down leading to arthritis in the surrounding bones. Some people with Kienbock's disease have no symptoms and the condition is only discovered by chance.[1][5]

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Abnormality of the wrist
Abnormalities of the wrists
0003019
Arthralgia
Joint pain
0002829
Bone pain
0002653
Limitation of joint mobility
Decreased joint mobility
Decreased mobility of joints
Limited joint mobility
Limited joint motion

[ more ]

0001376
Osteochondritis Dissecans
0010886
30%-79% of people have these symptoms
Osteoarthritis
Degenerative joint disease
0002758

Cause

The cause of Kienbock's disease is unknown. It has been suggested that it is related to a combination of bone structure, blocked blood flow, and trauma.[1][3][4]

Diagnosis

Kienbock's disease is diagnosed based on a clinical exam and imaging studies, such as an MRI or CT scan.[2][4]

Treatment

There are several nonsurgical and surgical options for treating Kienbock's disease.[6] Nonsurgical options include keeping the wrist from moving and medications. Surgical options include restoring blood flow to the wrist and evening out the length of arm bones. The goals of treatment are to reduce pain, improve wrist movement, and stop the disease from getting worse.[2][4]

Specialists involved in the care of someone with Kienbock's disease include:

  • Orthopedist
  • Hand surgeon
  • Physical therapist

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 Merck Manual for health care professionals provides information on Kienbock's disease.
  • 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.

References

  1. Fontaine C. Kienböck's disease. Chir Main. 2015; 34(1):4-17. https://pubmed.ncbi.nlm.nih.gov/25600763.
  2. Nasr LA, Koay J. Kienbock Disease. StatPearls. Updated Apr 27, 2020; https://pubmed.ncbi.nlm.nih.gov/30725676.
  3. Lutsky K, Beredjiklian PK. Kienböck disease. J Hand Surg Am. 2012; 37(9):1942-1952. https://pubmed.ncbi.nlm.nih.gov/22916868.
  4. Lichtman DM, Pientka WF, Bain GI. Kienböck Disease: Moving Forward. J Hand Surg Am. 2016; 41(5):630-638. https://pubmed.ncbi.nlm.nih.gov/27055625.
  5. Cross D, Matullo KS. Kienböck disease. Orthop Clin North Am. January, 2014; 45(1):141-152. https://pubmed.ncbi.nlm.nih.gov/24267215.
  6. Yesiloz M, Louis M DeVerbizier J, et al. Kienböck's disease: Role of cross-sectional imaging in treatment choice and patient follow-up. Eur J Radiol. 2018; 105:269-282. https://pubmed.ncbi.nlm.nih.gov/30017293.
  7. Golay SK, Rust P, Ring D. The Radiological Prevalence of Incidental Kienböck Disease. Arch Bone Jt Surg. 2016; 4(3):220-223. https://pubmed.ncbi.nlm.nih.gov/27517065.

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