Presentation
Figure 3 Figure 3: AP Radiograph of the pelvis of F.W. on presentation with right hip pain. Following the presentation of A.B.' [ispub.com]
Symptoms present at birth, and continuous. [102] Symptoms may be present at birth or early infancy. [102] Purpuric rash presenting at birth or in infancy. Vasculitis noted on skin biopsy. [autoinflammatory-search.org]
Avascular necrosis (AVN) is a very rare presenting feature of endogenous hypercortisolaemia. [edmcasereports.com]
Fractures to the talar neck with fragmentation were present. In addition, there appeared to be a fracture to the talar body with slight plantar compression. [podiatrytoday.com]
Palpation for splenic size to ensure early presentation of splenic sequestration can significantly reduce deaths. [patient.info]
Immune System
- Splenomegaly
Some with hepatomegaly, splenomegaly or hepatosplenomegaly. [autoinflammatory-search.org]
[…] transient red cell aplasia, and hyperhaemolysis in patients with severe infection. [ 3 ] Increased susceptibility to infections by encapsulated bacteria such as pneumococcus; the risk of overwhelming infection is highest before the age of 3 years. [ 3 ] Splenomegaly [patient.info]
Entire Body System
- Pain
Symptoms The primary symptom of osteonecrosis is pain. Other symptoms of osteonecrosis include limitation of motion joint stiffness and muscle spasms. Pain Pain can start out slowly as mild pain especially when walking standing or lifting. [orthop.washington.edu]
The pain test typically involves placing a local anesthetic inside the hip joint with the contrast dye. The pain test may help assess whether the pain is coming from inside the hip joint. Can back pain be a sign of FAI? Yes. [hipfai.com]
The articular cartilage becomes damaged and, over time, causes hip pain and stiffness. [bonesmart.org]
Acute pain: assess all patients with sickle cell disease who present with acute pain to determine whether their pain is being caused by an acute painful sickle cell episode or whether an alternative diagnosis is possible, particularly if pain is reported [patient.info]
As the disease progresses, however, most experience joint pain. At first, the pain occurs only when putting weight on the affected joint. Later, it occurs even at rest. Pain usually develops gradually, and may be mild or severe. [multiple-sclerosis-research.blogspot.com]
- Collapse
Post collapse Late-Collapse - symptomatic treatment till resurfacing or THR necessary Guide-lines for management 36. [slideshare.net]
and; Severe and painful osteoarthritis in the affected joint once the bone collapses. [healthand.com]
These include the possibility of subtalar joint arthrodesis, ankle joint arthrodesis or tibiocalcaneal arthrodesis. 7 Note that surgical intervention is not always necessary following collapse of the talus. [podiatrytoday.com]
We can also state that most radiographically evident lesions progress until the femoral head collapses [1]. [physio-pedia.com]
Most patients demonstrated no femoral head collapse preoperatively, but more than 50% had progressed to radiographic collapse at a mean follow-up of 3 years. [ijoonline.com]
- Gaucher Disease
This can be caused by disease, or a severe trauma, such as a break or dislocation, that affects the blood supply to the bone. Causes of avascular necrosis can include long-term steroid use, sickle cell disease, Gaucher disease, gout or diabetes. [bonesmart.org]
To date, we know that you may be at an increased risk for developing ON if you’ve dislocated or fractured a hip, suffer with alcoholism, use corticosteroids, or have any number of glandular diseases, including rheumatoid arthritis, Gaucher’s disease, [orthosportsmed.com]
Risk factors are many, such as hip trauma, alcohol abuse, excessive use of corticosteroids, hemoglobinopathy, Gaucher’s disease, pregnancy, coagulopathies, Caisson disease, inflammatory or autoimmune disease, antiretroviral therapy, hyperbaric exposure [link.springer.com]
[…] the mandible),[9] sickle cell anaemia,[10] and Gaucher's Disease.[11] In some cases it is idiopathic (no cause is found).[12] Rheumatoid arthritis and lupus are also common causes of AVN. [hipresurfacingsite.com]
Risk factors There are several risk factors that can result in avascular necrosis: Corticosteroid medication that is used in high doses or long-term; Chemotherapy or radiotherapy ; Organ transplants; HIV, Gaucher disease and cancer ; Lupus, gout and decompression [healthand.com]
- Enuresis
Nocturnal enuresis. Infection: patients are prone to infection, especially pneumococcus, typhoid osteomyelitis and haemophilus because of hyposplenism resulting from sickling and consequent autosplenectomy. [patient.info]
Respiratoric
- Aspiration
A small trochar (MarrowStim, Biomet Biologics, Warsaw, IN, USA) is inserted into the iliac crest and bone marrow is aspirated. [dovepress.com]
HUMAN STUDIES Use of concentrated bone marrow aspirate and platelet rich plasma during minimally invasive decompression of the femoral head in the treatment of osteonecrosis. Martin JR, Houdek MT, Sierra RJ. Croat Med J. 2013 Jun;54(3):219-24. [kumc.edu]
In a prolonged episode, aspiration and irrigation of the corpora cavernosa with adrenaline (epinephrine) or etilefrine is now the treatment of choice. [patient.info]
Bone Marrow Aspirate Concentrate in Combination With Intravenous Iloprost Increases Bone Healing in Patients With Avascular Necrosis of the Femoral Head: A Matched Pair Analysis. Orthop Rev (Pavia). 2016 Nov 17. 8 (4):6902. [Medline]. [Full Text]. [emedicine.medscape.com]
- Sputum
Acute chest syndrome (vaso-occlusive crisis affecting the lungs): Defined as a new pulmonary infiltrate on the chest radiograph combined with one or more manifestations such as fever, cough, sputum production, tachypnoea, dyspnoea, or new-onset hypoxia [patient.info]
In some cases, sputum samples stained with Oil Red O will show fat-laden macrophages. Exchange transfusion has been used with success in some patients with acute bone marrow necrosis. [sickle.bwh.harvard.edu]
Course
- Severe Clinical Course
All had severe clinical courses marked by varying degrees of splenic sequestration crises, acute chest syndrome, vaso-occlusive crises, osteomyelitis and avascular necrosis of the femoral head. [karger.com]
Bone marrow transplantation: [ 6 ] Haematopoietic stem cell transplantation is potentially curative but is currently used only in patients with a severe clinical course and a matched sibling donor. [patient.info]
The patterns of severity apply only to populations. Broad overlap in the clinical patterns prevents the use of haplotypes to predict the clinical course in a particular person. [sickle.bwh.harvard.edu]
Gastrointestinal
- Abdominal Distension
May present with swollen painful joints, tachypnoea or other signs of lung involvement, neurological signs, acute abdominal distension and pain (mesenteric sickling and bowel ischaemia), loin pain (renal papillary necrosis may cause renal colic or severe [patient.info]
Musculoskeletal
- Joint Stiffness
Other symptoms of osteonecrosis include limitation of motion joint stiffness and muscle spasms. Pain Pain can start out slowly as mild pain especially when walking standing or lifting. [orthop.washington.edu]
Range of motion exercises It is important that your child keep all joints flexible. Often joint stiffness can occur before you even notice. [stjude.org]
If osteonecrosis progresses and the bone and surrounding joint surface collapse, pain may develop or increase dramatically. Pain may be severe enough to cause joint stiffness by limiting the range of motion in the affected joint. [rarediseases.org]
Skin
- Skin Disease
NIAMS: National Institute of Arthritis and Musculoskeletal and Skin Diseases. Osteonecrosis. National Institutes of Health, Department of Health and Human Services. Available at: http://www.niams.nih.gov/Health_Info/Osteonecrosis/default.asp. [orthosportsmed.com]
Bethesda, MD: National Institute of Arthritis and Musculoskeletal and Skin Diseases; 2008 [cited 8 April 2008]. Available from: URL link Avascular necrosis [online]. Philadelphia, PA: Rothman Institute Orthopaedics; 2008 [cited 8 April 2008]. [myvmc.com]
Archived from the original on 9 August 2017. ^ a b National Institute of Arthritis and Musculoskeletal and Skin Diseases (March 2006). "Osteonecrosis". Food and Drug Administration. Archived from the original on 23 May 2009. [en.wikipedia.org]
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). Last Reviewed: 10/30/2015. Available at: http://www.niams.nih.gov/health_info/osteonecrosis/osteonecrosis_ff.asp Accessed April 9, 2018. Jones LC, Mont MA. [rarediseases.org]
Brooks, MD, PhD APPLE was supported by the NIH (National Institute of Arthritis and Musculoskeletal and Skin Diseases contract N01-AR-2-2265), the Edna and Fred L. Mandel Jr. [ped-rheum.biomedcentral.com]
- Erythema
Periorbital erythema, and testicular swelling in one case. [102] Unknown [102] Unknown [102] Periorbital erythema. [102] It is unknown, or not noted if there are any symptoms affecting the heart or lungs. [autoinflammatory-search.org]
Psychiatrical
- Suggestibility
Thrombophilia and hypofibrinolysis have been suggested as genetic causes of osteonecrosis in both adults and children 4. [ispub.com]
"I suggest reading the NIH health information sheet for more information on AVN. The treatment of AVN is really symptomatic, but if the AVN is unilateral a core decompression of the opposite hip may prevent it becoming bilateral. [multiple-sclerosis-research.blogspot.com]
Aldrige et al, [72] (2008) suggested modifications in the FVFG surgical technique, where they suggested confirmation of patency of channels by an intra operative arteriogram after the fibula has been placed within the femoral head but before the anastomosis [ijoonline.com]
Garvin observed that morbidity apparently increased in men; further, sex-related differences in some acute and chronic complications of this disorder were suggested [ 9 ]. [annexpublishers.co]
Clinical findings were not suggestive of infection. She was diagnosed with AVN of the left femoral head. Management consisted of NSAIDS, restricted wt. bearing. [gait.aidi.udel.edu]
Urogenital
- Hematuria
Microscopic hematuria is a common problem in patients with sickle cell disease (as well as some patients with sickle cell trait). Hematuria per se requires no intervention unless blood loss is massive. [sickle.bwh.harvard.edu]
Hematuria, proteinuria, glomerulonephritis. [97] Splenomegaly. Some with lymphadenopathy. [97] Arthralgia, arthritis. Some with relapsing polychondritis. [autoinflammatory-search.org]
- Nocturnal Enuresis
Nocturnal enuresis. Infection: patients are prone to infection, especially pneumococcus, typhoid osteomyelitis and haemophilus because of hyposplenism resulting from sickling and consequent autosplenectomy. [patient.info]
- Painful Erection
Stroke : clinical evidence of stroke occurs by age 20 years in 11% of patients with sickle cell disease. [ 3 ] Priapism: males with sickle cell disease may experience painful erections, which may be brief but recurrent or may last six hours or more and [patient.info]
Workup
He sent me the following coagulation work up to be performed: COAGULATION WORKUP A COAGULATON WORKUP B The above tests have LabCorp codes. [osteonecrosis.me]
See Workup. Early diagnosis and appropriate intervention can delay the need for joint replacement. However, most patients present late in the disease course. [emedicine.medscape.com]
Microbiology
- Streptococcus Pneumoniae
Routine childhood vaccinations include protection against Haemophilus influenzae type B and conjugated vaccines against Streptococcus pneumoniae in most high-income countries. [patient.info]
The trend indicated clearly that penicillin protected patients from infection or death due to overwhelming infection by Streptococcus pneumoniae. [sickle.bwh.harvard.edu]
Treatment
No single treatment method will work for all patients, and no single treatment consistently prevents the development of femoral head deformity. [scottishritehospital.org]
To reach these goals, the doctor may use one or more of the following treatments. Non-operative Treatment There is no known pharmaceutical cure for osteonecrosis. [rarediseases.org]
This can sometimes develop following radiotherapy to the head and neck area, bisphosphonates treatment, or after a tooth extraction while having either of the two treatments. [healthand.com]
Osteonecrosis: etiology, diagnosis and treatment. American Academy of Orthopaedic Surgeons, Rosemont, IL:391–396 Google Scholar Hungerford DS (2007) Treatment of osteonecrosis of the femoral head. [link.springer.com]
Prognosis
[…] contributing authors—twenty of them new to this edition—provide comprehensive coverage of every aspect of cutaneous and systemic lupus erythematosus, including definitions, pathogenesis, autoantibodies, clinical and laboratory features, management, prognosis [books.google.com]
Osteochondroses differ in their anatomic distribution, course, and prognosis; they typically cause pain and have important orthopedic implications. [msdmanuals.com]
: overall prevalence of 9.7% Reports vary: Bishop etal. 19% - 31% Many have bilateral hip involvement Prognosis: Young children have a reasonable potential for healing avascular necrosis, while the older adolescent with total head involvement will likely [gait.aidi.udel.edu]
There is a high success rate and the prognosis is quite good provided that rehabilitation is done after surgery. [pugvillage.com]
Etiology
The etiology is controversial. Few reports describe the association with trauma, steroid use and systemic lupus erythematosus. [radiopaedia.org]
Etiology of osteochondroses is typically unknown; some of the disorders have a familial component, but inheritance is complex. [msdmanuals.com]
It is now understood that LCPD is caused by an ischemic injury, yet the exact etiology remains unclear. [ped-rheum.biomedcentral.com]
Osteonecrosis: etiology, diagnosis and treatment. American Academy of Orthopaedic Surgeons, Rosemont, IL:391–396 Google Scholar Hungerford DS (2007) Treatment of osteonecrosis of the femoral head. [link.springer.com]
Epidemiology
Epidemiology Frequency United States The frequency of AVN depends on the site involved. The most common site is the hip; other locations include the carpals, talus, femur, metatarsal, mandible, and humerus. [emedicine.medscape.com]
Epidemiology ethiology and diagnosis of osteoporosis. Am J Obstet Gynecol. 2006;194:S3–S11. Leon A. Assael. [jpmh.org]
If left untreated, the disease progresses, the bone collapses, [21] and the joint surface breaks down, leading to pain and arthritis. [1] Epidemiology [ edit ] Avascular necrosis usually affects people between 30 and 50 years of age; about 10,000 to 20,000 [en.wikipedia.org]
Support for this concept comes from epidemiological studies in malaria-endemic regions of Africa. [sickle.bwh.harvard.edu]
The epidemiology of slipped capital femoral epiphysis: an update. J Pediatr Orthop. 2006;26(3):286–90. doi:10.1097/01.bpo.0000217718.10728.70. View Article PubMed Google Scholar Steiner C, Elixhauser A, Schnaier J. [ped-rheum.biomedcentral.com]
Pathophysiology
The pathophysiology of avascular necrosis of the femoral head has not been completely accounted for. In some patients there has clearly been a direct cause (trauma, radiation,..), while in others the pathophysiology is still uncertain. [physio-pedia.com]
Risk factors for AVN Although the pathophysiology of AVN is not yet well-understood, it is thought to be a multifactorial disease, with patients reporting a history of exposure to one or more risk factors, including trauma to the hip, alcohol abuse, corticosteroid [mayoclinic.org]
Pathophysiology Although the pathophysiology of AVN is not fully understood, the final common pathway is interruption of blood flow to the bone. [emedicine.medscape.com]
American Volume 88 1117 – 1132. ( doi:10.2106/JBJS.E.01041 ) Search Google Scholar Export Citation Glueck CJ Freiberg R Tracy T Stroop D Wang P 1997 Thrombophilia and hypofibrinolysis: pathophysiologies of osteonecrosis. [edmcasereports.com]
Avascular necrosis of the femoral head: etiology, pathophysiology, classification, and current treatment guidelines. Am J Orthop (Belle Mead NJ). 2004;33:327–332. 13. Zhao D, Cui D, Wang B, et al. [dovepress.com]
Prevention
Prevention of this course is paramount to preventing joint replacement surgery. [drlox.com]
The primary goals of treatment for Perthes disease are to alleviate pain and to prevent lasting deformity of the femoral head. [scottishritehospital.org]
Prevention As the saying goes "prevention is the best medicine." If you are taking high doses of steroids for any reason it is important for you to continue to see your doctor on a regular basis. [orthop.washington.edu]
The best way to prevent AVN is to maintain strength and flexibility. Be sure to warm up before any physical activity and include strength training in your workouts. [upmc.com]
Avascular necrosis is presently not preventable but research has shown that lipid lowering drugs such as lipitor can reduce or prevent avascular necrosis in animals. [clinicaltrials.gov]