avascular necrosis

{{Short description|Death of bone tissue due to interruption of the blood supply}}

{{Use dmy dates|date=February 2020}}

{{Infobox medical condition (new)

| name = Avascular necrosis

| synonyms = Osteonecrosis, bone infarction, aseptic necrosis, ischemic bone necrosis

| image = Head of femur avascular necrosis.jpg

| caption = Femoral head showing a flap of cartilage due to avascular necrosis (osteochondritis dissecans). Specimen removed during total hip replacement surgery.

| field = Orthopedics

| symptoms = Joint pain, decreased ability to move

| complications = Osteoarthritis

| onset = Gradual

| duration =

| types =

| causes =

| risks = Bone fractures, joint dislocations, high dose steroids

| diagnosis = Medical imaging, biopsy

| differential = Osteopetrosis, rheumatoid arthritis, Legg–Calvé–Perthes syndrome, sickle cell disease

| prevention =

| treatment = Medication, not walking on the affected leg, stretching, surgery

| medication =

| prognosis =

| frequency = ~15,000 per year (US)

| deaths =

}}

Avascular necrosis (AVN), also called osteonecrosis or bone infarction, is death of bone tissue due to interruption of the blood supply.{{cite web|title=Questions and Answers about Osteonecrosis (Avascular Necrosis)|url=https://www.niams.nih.gov/health_info/Osteonecrosis/default.asp|website=NIAMS|language=en|date=October 2015|url-status=live|archive-url=https://web.archive.org/web/20170809040514/https://www.niams.nih.gov/health_info/Osteonecrosis/default.asp|archive-date=9 August 2017|df=dmy-all}}{{PD-notice}} Early on, there may be no symptoms. Gradually joint pain may develop, which may limit the person's ability to move. Complications may include collapse of the bone or nearby joint surface.

Risk factors include bone fractures, joint dislocations, alcoholism, and the use of high-dose steroids. The condition may also occur without any clear reason. The most commonly affected bone is the femur (thigh bone). Other relatively common sites include the upper arm bone, knee, shoulder, and ankle. Diagnosis is typically by medical imaging such as X-ray, CT scan, or MRI. Rarely biopsy may be used.

Treatments may include medication, not walking on the affected leg, stretching, and surgery. Most of the time surgery is eventually required and may include core decompression, osteotomy, bone grafts, or joint replacement.

About 15,000 cases occur per year in the United States. People 30 to 50 years old are most commonly affected.{{cite web|title=Osteonecrosis|url=https://rarediseases.org/rare-diseases/osteonecrosis/|website=NORD (National Organization for Rare Disorders)|access-date=8 August 2017|date=2009|url-status=live|archive-url=https://web.archive.org/web/20170219101821/https://rarediseases.org/rare-diseases/osteonecrosis/|archive-date=19 February 2017|df=dmy-all}} Males are more commonly affected than females.{{cite book|last1=Ferri|first1=Fred F.|title=Ferri's Clinical Advisor 2018 E-Book: 5 Books in 1|date=2017|publisher=Elsevier Health Sciences|isbn=9780323529570|page=166|url=https://books.google.com/books?id=wGclDwAAQBAJ&pg=PA166|language=en|url-status=live|archive-url=https://web.archive.org/web/20170809042055/https://books.google.ca/books?id=wGclDwAAQBAJ&pg=PA166|archive-date=9 August 2017|df=dmy-all}}

Signs and symptoms

In many cases, there is pain and discomfort in a joint which increases over time. It can affect any bone, and for in about half of affected people, multiple sites are damaged.{{cite web |url=http://www.niams.nih.gov/Health_Info/Osteonecrosis/default.asp |title=Osteonecrosis |date=March 2006 |author=National Institute of Arthritis and Musculoskeletal and Skin Diseases |publisher=Food and Drug Administration |access-date=25 May 2009| archive-url= https://web.archive.org/web/20090523032114/http://www.niams.nih.gov/Health_Info/Osteonecrosis/default.asp| archive-date= 23 May 2009| url-status= live|author-link=National Institute of Arthritis and Musculoskeletal and Skin Diseases }}

Avascular necrosis most commonly affects the ends of long bones, such as the femur. Other common sites include the humerus (upper arm),{{cite journal | vauthors = Chapman C, Mattern C, Levine WN | title = Arthroscopically assisted core decompression of the proximal humerus for avascular necrosis | journal = Arthroscopy | volume = 20 | issue = 9 | pages = 1003–6 | date = November 2004 | pmid = 15525936 | doi = 10.1016/j.arthro.2004.07.003 }}{{cite journal | vauthors = Mansat P, Huser L, Mansat M, Bellumore Y, Rongières M, Bonnevialle P | title = Shoulder arthroplasty for atraumatic avascular necrosis of the humeral head: nineteen shoulders followed up for a mean of seven years | journal = Journal of Shoulder and Elbow Surgery | volume = 14 | issue = 2 | pages = 114–20 | date = Mar 2005 | pmid = 15789002 | doi = 10.1016/j.jse.2004.06.019 }} knees,{{cite journal | vauthors = Jacobs MA, Loeb PE, Hungerford DS | title = Core decompression of the distal femur for avascular necrosis of the knee | journal = The Journal of Bone and Joint Surgery. British Volume | volume = 71 | issue = 4 | pages = 583–7 | date = August 1989 | pmid = 2768301 | doi=10.1302/0301-620X.71B4.2768301| s2cid = 16423679 | url = http://pdfs.semanticscholar.org/6aa4/90337662dc292fb3728ef89ea26c711802aa.pdf | archive-url = https://web.archive.org/web/20200726093740/http://pdfs.semanticscholar.org/6aa4/90337662dc292fb3728ef89ea26c711802aa.pdf | url-status = dead | archive-date = 2020-07-26 }}{{cite journal | vauthors = Bergman NR, Rand JA | title = Total knee arthroplasty in osteonecrosis | journal = Clinical Orthopaedics and Related Research | issue = 273 | pages = 77–82 | date = December 1991 | volume = 273 | pmid = 1959290 | doi = 10.1097/00003086-199112000-00011 | s2cid = 3235011 | url = https://dx.doi.org/10.1097/00003086-199112000-00011 | format = Free full text }} shoulders, ankles and the jaw.{{cite journal | vauthors = Baykul T, Aydin MA, Nasir S | title = Avascular necrosis of the mandibular condyle causing fibrous ankylosis of the temporomandibular joint in sickle cell anemia | journal = The Journal of Craniofacial Surgery | volume = 15 | issue = 6 | pages = 1052–6 | date = November 2004 | pmid = 15547404 | doi = 10.1097/00001665-200411000-00035 }}

Causes

The main risk factors are bone fractures, joint dislocations, alcoholism, and the use of high-dose steroids. Other risk factors include radiation therapy, chemotherapy, and organ transplantation. Osteonecrosis is also associated with cancer, lupus, sickle cell disease,{{Cite journal |last1=Martí-Carvajal |first1=Arturo J. |last2=Solà |first2=Ivan |last3=Agreda-Pérez |first3=Luis H. |date=2019-12-05 |title=Treatment for avascular necrosis of bone in people with sickle cell disease |journal=The Cochrane Database of Systematic Reviews |volume=2019 |issue=12 |pages=CD004344 |doi=10.1002/14651858.CD004344.pub7 |issn=1469-493X |pmc=6894369 |pmid=31803937}} HIV infection, Gaucher's disease, and Caisson disease (dysbaric osteonecrosis).{{Cite web|last=Campbell|first=Ernest S.|date=2019-04-04|title=Dysbaric Osteonecrosis and Diving|url=http://scuba-doc.com/dysbaric-osteonecrosis-and-diving/|access-date=2021-04-20|website=SCUBADOC - Diving Medicine Online|publisher=SCUBADOC|language=en-US}} Bisphosphonates are associated with osteonecrosis of the mandible (jawbone).{{cite journal |vauthors=Dannemann C, Grätz KW, Riener MO, Zwahlen RA |date=April 2007 |title=Jaw osteonecrosis related to bisphosphonate therapy: a severe secondary disorder |journal=Bone |volume=40 |issue=4 |pages=828–34 |doi=10.1016/j.bone.2006.11.023 |pmid=17236837}} The condition may also occur without any clear reason.

Prolonged, repeated exposure to high pressures (as experienced by commercial and military divers) has been linked to AVN, though the relationship is not well understood.{{Cite journal |last1=Uguen |first1=M. |last2=Pougnet |first2=R. |last3=Uguen |first3=A. |last4=Loddé |first4=B. |last5=Dewitte |first5=J. D. |date=2014 |title=Dysbaric osteonecrosis among professional divers: a literature review |url=https://pubmed.ncbi.nlm.nih.gov/25562949 |journal=Undersea & Hyperbaric Medicine |volume=41 |issue=6 |pages=579–587 |issn=1066-2936 |pmid=25562949}}{{Cite journal |last1=Sharareh |first1=Behnam |last2=Schwarzkopf |first2=Ran |date=March 2015 |title=Dysbaric osteonecrosis: a literature review of pathophysiology, clinical presentation, and management |url=https://pubmed.ncbi.nlm.nih.gov/24662571 |journal=Clinical Journal of Sport Medicine|volume=25 |issue=2 |pages=153–161 |doi=10.1097/JSM.0000000000000093 |issn=1536-3724 |pmid=24662571|s2cid=20119213 }}

In children, avascular osteonecrosis can have several causes. It can occur in the hip as part of Legg–Calvé–Perthes syndrome,{{cite journal|vauthors=Gross GW, Articolo GA, Bowen JR|year=1999|title=Legg-Calve-Perthes Disease: Imaging Evaluation and Management|journal=Seminars in Musculoskeletal Radiology|volume=3|issue=4|pages=379–391|doi=10.1055/s-2008-1080081|pmid=11388931|s2cid=260321190 }} and it can also occur as a result after malignancy treatment such as acute lymphoblastic leukemia and allotransplantation.{{Cite journal|last1=Kaste|first1=Sue C.|last2=Karimova|first2=Evguenia J.|last3=Neel|first3=Michael D.|date=May 2011|title=Osteonecrosis in Children After Therapy for Malignancy|journal=American Journal of Roentgenology|language=en|volume=196|issue=5|pages=1011–1018|doi=10.2214/AJR.10.6073|issn=0361-803X|pmc=4700933|pmid=21512065}}

Pathophysiology

The hematopoietic cells are most sensitive to low oxygen and are the first to die after reduction or removal of the blood supply, usually within 12 hours.{{cite web | url = http://emedicine.medscape.com/article/387545-overview | work = eMedicine Specialties | title = Bone Infarct | archive-url = https://web.archive.org/web/20100304130416/http://emedicine.medscape.com/article/387545-overview |archive-date=4 March 2010 | url-status = live | first1 = Ali Nawaz | last1 = Khan | first2 = Mohammed Jassim | last2 = Al-Salman | first3 = Muthusamy | last3 = Chandramohan | first4 = Sumaira | last4 = MacDonald | first5 = Charles Edward | last5 = Hutchinson | name-list-style = vanc }} Experimental evidence suggests that bone cells (osteocytes, osteoclasts, osteoblasts etc.) die within 12–48 hours, and that bone marrow fat cells die within 5 days.

Upon reperfusion, repair of bone occurs in two phases. First, there is angiogenesis and movement of undifferentiated mesenchymal cells from adjacent living bone tissue grow into the dead marrow spaces, as well as entry of macrophages that degrade dead cellular and fat debris. Second, there is cellular differentiation of mesenchymal cells into osteoblasts or fibroblasts. Under favorable conditions, the remaining inorganic mineral volume forms a framework for establishment of new, fully functional bone tissue.

Diagnosis

File:OCD Knee WalterReed-1.jpg (epiphyseal plates are open): arrows point to avascular necrosis and developing osteochondritis dissecans in the outer medial condyle of femur]]

In the early stages, bone scintigraphy and MRI are the preferred diagnostic tools.{{cite journal | vauthors = Maillefert JF, Toubeau M, Piroth C, Piroth L, Brunotte F, Tavernier C | title = Bone scintigraphy equipped with a pinhole collimator for diagnosis of avascular necrosis of the femoral head | journal = Clinical Rheumatology | volume = 16 | issue = 4 | pages = 372–7 | date = June 1997 | pmid = 9259251 | doi = 10.1007/BF02242454 | s2cid = 40304352 }}{{cite journal | vauthors = Bluemke DA, Zerhouni EA | title = MRI of avascular necrosis of bone | journal = Topics in Magnetic Resonance Imaging | volume = 8 | issue = 4 | pages = 231–46 | date = August 1996 | pmid = 8870181 | doi = 10.1097/00002142-199608000-00003 | s2cid = 2554184 }}

X-ray images of avascular necrosis in the early stages usually appear normal. In later stages it appears relatively more radio-opaque due to the nearby living bone becoming resorbed secondary to reactive hyperemia. The necrotic bone itself does not show increased radiographic opacity, as dead bone cannot undergo bone resorption which is carried out by living osteoclasts. Late radiographic signs also include a radiolucency area following the collapse of subchondral bone (crescent sign) and ringed regions of radiodensity resulting from saponification and calcification of marrow fat following medullary infarcts.{{citation needed|date=October 2020}}

{{anchor|Kümmel}}

File:Osteonecrosis humerus 1.jpg|Radiography of total avascular necrosis of right humeral head. Woman of 81 years with diabetes of long evolution.

File:Osteonecrosis femur 1.jpg|Radiography of avascular necrosis of left femoral head. Man of 45 years with AIDS.

File:Osteonecrosis femur 2img.jpg|Nuclear magnetic resonance of avascular necrosis of left femoral head. Man of 45 years with AIDS.

File:Intravertebral vacuum cleft sign.jpg|The intravertebral vacuum cleft sign (at white arrow) is a sign of avascular necrosis. Avascular necrosis of a vertebral body after a vertebral compression fracture is called Kümmel's disease.{{cite journal | vauthors = Freedman BA, Heller JG | title = Kummel disease: a not-so-rare complication of osteoporotic vertebral compression fractures | journal = Journal of the American Board of Family Medicine | volume = 22 | issue = 1 | pages = 75–8 | year = 2009 | pmid = 19124637 | doi = 10.3122/jabfm.2009.01.080100 | s2cid = 15539206 }}

File:Pathology of avascular necrosis.jpg|Pathology of avascular necrosis, with a photograph of a cross-section of the involved bone at top left. The reactive zone shows irregular trebaculae with empty lacunae, and fibrosis of the marrow space.

=Types=

When AVN affects the scaphoid bone, it is known as Preiser disease. Another named form of AVN is Köhler disease, which affects the navicular bone of the foot, primarily in children. Yet another form of AVN is Kienböck's disease, which affects the lunate bone in the wrist.{{Cite journal |last1=Cross |first1=Danielle |last2=Matullo |first2=Kristofer S. |date=2014-01-01 |title=Kienböck Disease |url=https://www.sciencedirect.com/science/article/pii/S0030589813001132 |journal=Orthopedic Clinics of North America |volume=45 |issue=1 |pages=141–152 |doi=10.1016/j.ocl.2013.09.004 |pmid=24267215 |issn=0030-5898}}

Treatment

A variety of methods may be used to treat the disease, with the most common being total hip replacement (THR). However, THRs have a number of downsides, including long recovery times and the lifespans of the hip joints (often around 20 to 30 years). THRs are an effective means of treatment in the older population; however, in younger people, they may wear out before the end of a person's life.{{Cite journal|last1=Evans|first1=Jonathan T|last2=Evans|first2=Jonathan P|last3=Walker|first3=Robert W|last4=Blom|first4=Ashley W|last5=Whitehouse|first5=Michael R|last6=Sayers|first6=Adrian|date=2019-02-16|title=How long does a hip replacement last? A systematic review and meta-analysis of case series and national registry reports with more than 15 years of follow-up|journal=The Lancet|language=en|volume=393|issue=10172|pages=647–654|doi=10.1016/S0140-6736(18)31665-9|pmid=30782340 |pmc=6376618 |issn=0140-6736}}

Other techniques, such as metal-on-metal resurfacing, may not be suitable in all cases of avascular necrosis; its suitability depends on how much damage has occurred to the femoral head.{{cite book | first1 = Benedetto | last1 = de Bernard | editor-last1=Hall| editor-first1= Brian K. | name-list-style = vanc | chapter = Calcium Metabolism and Bone Mineralization |title=Bone|date=15 November 1989|publisher=CRC Press|chapter-url=https://books.google.com/books?id=ZI9gkQa7DzYC&pg=PA74 |isbn=978-0-936923-24-6|pages=74– }} Bisphosphonates, which reduce the rate of bone breakdown, may prevent collapse (specifically of the hip) due to AVN.{{cite journal | vauthors = Agarwala S, Jain D, Joshi VR, Sule A | title = Efficacy of alendronate, a bisphosphonate, in the treatment of AVN of the hip. A prospective open-label study | journal = Rheumatology | volume = 44 | issue = 3 | pages = 352–9 | date = March 2005 | pmid = 15572396 | doi = 10.1093/rheumatology/keh481 | doi-access = }}

=Core decompression=

Other treatments include core decompression, whereby internal bone pressure is relieved by drilling a hole into the bone, and a living bone chip and an electrical device to stimulate new vascular growth are implanted; and the free vascular fibular graft (FVFG), in which a portion of the fibula, along with its blood supply, is removed and transplanted into the femoral head.{{cite journal | vauthors = Judet H, Gilbert A | title = Long-term results of free vascularized fibular grafting for femoral head necrosis | journal = Clinical Orthopaedics and Related Research | volume = 386 | issue = 386 | pages = 114–9 | date = May 2001 | pmid = 11347824 | doi = 10.1097/00003086-200105000-00015 | s2cid = 25970488 }} A 2016 Cochrane review found no clear improvement between people who have had hip core decompression and participate in physical therapy, versus physical therapy alone. There is additionally no strong research on the effectiveness of hip core decompression for people with sickle cell disease.

The disease's progression may be halted by transplanting nucleated cells from the bone marrow into avascular necrosis lesions after core decompression. However, much further research is needed to establish this technique.{{cite journal | vauthors = Gangji V, Hauzeur JP | title = Treatment of osteonecrosis of the femoral head with implantation of autologous bone-marrow cells. Surgical technique | journal = The Journal of Bone and Joint Surgery. American Volume | volume = 87 Suppl 1 | issue = Pt 1 | pages = 106–12 | date = March 2005 | pmid = 15743852 | doi = 10.2106/JBJS.D.02662 | url = http://www.ejbjs.org/cgi/content/full/87/1_suppl_1/106 | archive-url = https://web.archive.org/web/20090214034512/http://www.ejbjs.org/cgi/content/full/87/1_suppl_1/106 | url-status = dead | archive-date = 14 February 2009 | access-date = 27 April 2010 }}{{cite journal | vauthors = Lieberman JR, Conduah A, Urist MR | title = Treatment of osteonecrosis of the femoral head with core decompression and human bone morphogenetic protein | journal = Clinical Orthopaedics and Related Research | volume = 429 | issue = 429 | pages = 139–45 | date = December 2004 | pmid = 15577478 | doi = 10.1097/01.blo.0000150312.53937.6f | s2cid = 25883407 }}

Prognosis

The amount of disability that results from avascular necrosis depends on what part of the bone is affected, how large an area is involved, and how effectively the bone rebuilds itself. The process of bone rebuilding takes place after an injury as well as during normal growth. Normally, bone continuously breaks down and rebuilds—old bone is resorbed and replaced with new bone. The process keeps the skeleton strong and helps it to maintain a balance of minerals. In the course of avascular necrosis, however, the healing process is usually ineffective and the bone tissues break down faster than the body can repair them. If left untreated, the disease progresses, the bone collapses,{{cite journal | vauthors = DiGiovanni CW, Patel A, Calfee R, Nickisch F | title = Osteonecrosis in the foot | journal = The Journal of the American Academy of Orthopaedic Surgeons | volume = 15 | issue = 4 | pages = 208–217 | date = April 2007 | pmid = 17426292 | doi = 10.5435/00124635-200704000-00004 | s2cid = 31296534 }} and the joint surface breaks down, leading to pain and arthritis.

Epidemiology

Avascular necrosis usually affects people between 30 and 50 years of age; about 10,000 to 20,000 people develop avascular necrosis of the head of the femur in the US each year.{{citation needed|date=September 2022}}

Society and culture

Cases of avascular necrosis have been identified in a few high-profile athletes. It abruptly ended the career of American football running-back Bo Jackson in 1991. Doctors discovered Jackson to have lost all of the cartilage supporting his hip while he was undergoing tests following a hip injury he had on the field during a 1991 NFL Playoff game.{{cite news|url=https://www.nytimes.com/1991/03/20/sports/jackson-s-case-is-dividing-the-doctors.html|title=Jackson's Case Is Dividing The Doctors|first=Lawrence K.|last=Altman | name-list-style = vanc |work = The New York Times |date=20 March 1991}} Avascular necrosis of the hip was also identified in a routine medical check-up on quarterback Brett Favre following his trade to the Green Bay Packers in 1992.{{cite web | url = http://www.jsonline.com/story/index.aspx?id=348858 |archive-url= https://web.archive.org/web/20060927180333/http://www.jsonline.com/story/index.aspx?id=348858 | archive-date = 27 September 2006 | url-status = dead | work = JS Online | title = What, his hip? Favre reveals he has avascular necrosis|date=27 September 2006}} However, Favre would go on to have a long career at the Packers.{{citation needed|date=October 2020}}

Another high-profile athlete was American road racing cyclist Floyd Landis,{{cite web | url = https://www.nytimes.com/2006/07/16/magazine/16landis.html | title = What He's Been Pedaling | work = The New York Times | date = 16 July 2006 }} winner of the 2006 Tour de France, the title being subsequently stripped from his record by cycling's governing bodies after his blood samples tested positive for banned substances.{{Cite news| title= Landis Tests Positive; Title is a total complete loss| newspaper=Chicago Tribune| date= 5 August 2006}} During that tour, Landis was allowed cortisone shots to help manage his ailment despite cortisone also being a banned substance in professional cycling at the time.{{Cite news |url = http://sport.independent.co.uk/general/article1193126.ece |title = Cycling: Landis the Tour king celebrates a triumph of survival |first = Alasdair |last = Fotheringham | name-list-style = vanc |date = 24 July 2006|access-date = 2006-07-28|newspaper = The Independent|location = London|url-status = dead |archive-url = https://web.archive.org/web/20060806030336/http://sport.independent.co.uk/general/article1193126.ece |archive-date = 2006-08-06 }} (subscription required)

Rafael Nadal successfully continued his tennis career after having surgery for Mueller–Weiss syndrome (osteonecrosis of the navicular bone in the foot).{{Cite web |last=Roy |first=Neelabhra |title=What is Mueller-Weiss Syndrome, the foot injury Rafael Nadal suffers from? |url=https://www.sportskeeda.com/tennis/news-what-mueller-weiss-syndrome-what-foot-injury-rafael-nadal-suffers-from |access-date=2022-06-05 |website=www.sportskeeda.com |date=13 May 2022 |language=en-us}}

Youtuber Steve Wallis has revealed that he has the condition in his hip.{{where|date=October 2024}}

See also

  • Sperm whale skeletons can show damage from avascular necrosis caused by decompression.{{cite journal|vauthors=Moore MJ, Early GA|title=Cumulative sperm whale bone damage and the bends|journal=Science|year=2004|volume=306|issue=5705|page=2215|doi=10.1126/science.1105452|pmid=15618509}}

References

{{Reflist}}

Steve Wallis Step 2 livestream 19 December 2020

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