Revision as of 02:32, 4 April 2016

Welcome to <a href="Pathophysiology of Complex Patient Problems">PT 635 Pathophysiology of Complex Patient Problems</a> This is a wiki created by and for the students in the School of Physical Therapy at Bellarmine University in Louisville KY. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!

Original Editors - <a href="Pathophysiology of Complex Patient Problems">Carly McArtor and Krista Polanin from Bellarmine University's Pathophysiology of Complex Patient Problems project.</a>

Top Contributors - <img _fck_mw_template="true" _fckrealelement="1" _fckfakelement="true" src="http://www.physio-pedia.com/extensions/FCKeditor/fckeditor/editor/images/spacer.gif" class="FCK__MWTemplate">

Definition/Description[edit | edit source]

Fibrodysplasia Ossificans Progressiva is a rare, genetic disorder that transforms ligaments, muscles and tendons into bone outside the skeleton that impairs movement.^[1] It was first described in 1692 and is characterized by progressive heterotropic ossification in anatomic structures. ^[2]At first it was given the name Myositis Ossificans Progressiva which means "muscle that turns to bone." ^[2]The name was later changed to Fibrodysplasia Ossificans Progressiva indicating "soft tissue that turns to bone." ^[2]The name Fibrodysplasia Ossificans Progressiva means that ligaments and tendons are also turning to bone not just muscle which differentiates it from Myositis Ossificans Progressiva.^[2]This difference was discovered by Dr. Victor McKusick of Johns Hopkins University in the 1970's. ^[2]

Prevalence[edit | edit source]

1 in every 2 million people are diagnosed with Fibrodysplasia Ossificans Porgressiva^[1]
Nearly 90% of the time it is misdiagnosed and mismanaged.^[1]
67% undergo invasive procedures for diagnosis and treatment^[1]
Mostly begins in childhood, usually diagnosed before age 10^[2]
It has not been shown to be linked with any specific gender, ethnicity or race^[2]
There have been 800 confirmed cases in the world and 285 in the United States^[2]

Characteristics/Clinical Presentation[edit | edit source]

Congenital hallux valgus with microdactyly and monophalangeal great toe are early signs that are often visible at birth.^[1]
FOP primarily starts at the neck and shoulders then progressively moves to the trunk, back and extremities once the extra bone begins to form.^[1]
Ossification of intercostal muscles^[3]
Kyphoscoliosis and lordosis^[4]
Severe weight loss^[1]
Torticollis^[5]
TMJ complications^[4]

Flare-ups are usually sporadic and unpredictable. It is impossible to predict duration and severity of the flare-ups even though there has been some characteritic patterns described in some research. It is also possible that flare-ups can occur from trauma.^[3]

Acute flare-ups due to: intramuscular immunizations, mandibular blocks for dental work, muscle fatigue, blunt muscle trauma from bumps, bruises, falls, influenza-like viral illnesses^[3]

Prognosis
[edit | edit source]

The average lifespan of these patients is approximately 40 years. Death is usually caused by respiratory infections.^[2]
Most individuals with FOP are confined to a wheelchair by the second decade of life.^[6]
More than 50% will experience lifelong diabilities.^[6]

Medications[edit | edit source]

For acute flare-ups:

short term high does corticosteroids^[1]
NSAIDS^[1]
Biophosphonates^[1]

Radiotherapy^[1]

For chronic discomfort and ongoing flare-ups:

Cyclo-oxygenase-2 inhibitors^[3]
Leukotreine inhibitors^[3]
Mast Cell Stabilizers^[3]

Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]

Blood Samples

Positive for heterozygous R206H mutation of the ACVR1 gene.^[1]

Computed Tomography^[1]

Magnetic Resonance Imaging^[1]

Bone Scans^[1]

ESR elevated during acute flare-ups^[1]

Etiology/Causes[edit | edit source]

Genetic R206H mutation of the ACVR1 gene^[1]
ACVR1 gene is a bone morphogenetic protein (BMP) type1 receptor signaling endochondral ossification^[1]
R206H mutation leads to an increase in enhanced BMP signaling^[1]
Confirmation of a heterozygous gene mutation of the ACVR1 gene^[1]

Systemic Involvement[edit | edit source]

Cardiopulmonary system

Respiratoy problems caused by thoracic insufficiencies (i.e. decreased chest wall expansion)^[4]
Restrictive pulmonary diseases (i.e. pneumonia)^[4]

Nervous system

Middle ear ossifications^[4]
Hearing impairments^[4]

Immune system

Flare-ups following viral infections can occur^[4]
Inflammation^[4]

Renal system

Individuals with FOP are 2 times more likely to get kidney stones^[3]

Integumentary system^[5]

These individuals are at risk for pressure sores due to the decrease in soft tissue and the increase in bone.

Medical Management (current best evidence)[edit | edit source]

Medications^[3]

Reduces the pain and severity of flare-ups

Surgical release of joint contractures^[3]

Usually unsuccessful

Osteotomy of heterotropic bone^[3]

Mobilizes joints
Usually counterproductive because new heterotrophic ossificans can form at the site

Repositioned surgically^[3]

Improves the patients overall functional status
Rare

Ultimately, there is not much that can be done to cure this disease.

Physical Therapy Management (current best evidence)[edit | edit source]

Physical therapy has been shown in some cases to make the condition worse in individuals with FOP because it tends to cause flare-ups which eventually leads to further ossification of the ligaments, tendons and muscle.^[2] Aquatic therapy, however has been shown to be beneficial in maintaining ROM, pain relief, and aerobic endurance.^[2] Aquatic therapy provides low impact and resistance created by the water which ultimately, enhances the ease of activities of daily living, makes functional activities as easy as possible, and implements fall prevention precautions.^[2]

Differential Diagnosis
[edit | edit source]

Myositis Ossficians Progressiva
Juvenile Fibromatosis connective tissues cells called fibroblasts that invade ligaments, tendons and muscles causing swelling.^[2]
Lymphoedema
Soft tissue sarcomas/neoplasms there are many doctors that misdiagnose FOP for neoplasms due to the recurrence of soft tissue flare-ups.^[2]

Case Reports/ Case Studies[edit | edit source]

4 year old boy with FOP

Clinical and Genetic Analysis of Fibrodysplasia Ossificans Progressiva: A Case Report and Literature Review^[1]

20 year follow-up of a 23 year old female

A case of fibrodysplasia ossificans progressiva: 20 years of follow‑up^[6]

29 year old man with onset of sypmtoms at age 9

Fibrodysplasia ossificans progressiva without characteristic Skeletal anomalies^[5]

Resources
[edit | edit source]

International Fibrodysplasia Ossificans Progressiva Association

There is a support group offered to individuals diagnosed with this disorder called Internation Fibrodysplasia Ossificans Progressiva Association. It discusses the latest research, what it is like to live with FOP, and clinical trials. It was founded by Jeannie Peeper who has FOP. She wanted to end social isolation.^[2]
IFOPA's "Mission of Hope" http://www.ifopa.org/component/content/article/33/105.html

http://www.ifopa.org/

Henry Eastlack Skeleton
Henry Eastlack was a gentlemen who suffered and died from FOP. His skeleton is now on display at The Mutter Museum of The College of Physicians in Philadelphia. When individuals die their skeleton falls apart because the connective tissue, ligaments and tendons are no longer holding their skeleton together. When a skeleton is put on display at a museum it has to meticulously be rearticulated with wires and glue. Henry's skeleton never had to be rearticulated becuase of the extra bone created by FOP. His skeleton truly shows the harsh reality of FOP and the dysfunctions it can create.^[2]

Recent Related Research [edit | edit source]

<?xml version="1.0" encoding="utf-8"?>

Extension:RSS -- Error:

Not a valid URL: <channel> <title>pubmed: quality of life of p...</title> <link>http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=Search&db=PubMed&term=Quality%20of%20life%20of%20patients%20with%20fibrodysplasia%20ossificans%20progressiva.</link> <description>NCBI: db=pubmed; Term=Quality of life of patients with fibrodysplasia ossificans progressiva.</description> <language>en-us</language> <docs>http://blogs.law.harvard.edu/tech/rss</docs> <ttl>1440</ttl> <image> <title>NCBI pubmed</title> <url>http://www.ncbi.nlm.nih.gov/entrez/query/static/gifs/iconsml.gif</url> <link>http://www.ncbi.nlm.nih.gov/sites/entrez</link> <description>PubMed comprises more than 24 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.</description> </image> <item>

   <title>Quality of life of patients with fibrodysplasia ossificans progressiva.</title>         
   <link>http://www.ncbi.nlm.nih.gov/pubmed/26564023?dopt=Abstract</link>    
   <description>

<![CDATA[<table border="0" width="100%"><tr><td align="left"><a href="http://dx.doi.org/10.1007/s11832-015-0704-6%22><img src="//www.ncbi.nlm.nih.gov/corehtml/query/egifs/http:--production.springer.de-OnlineResources-Logos-springerlink.gif" border="0"/></a> <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/pmid/26564023/%22><img src="//www.ncbi.nlm.nih.gov/corehtml/query/egifs/http:--www.ncbi.nlm.nih.gov-corehtml-pmc-pmcgifs-pubmed-pmc.gif" border="0"/></a> </td><td align="right"><a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=Link&LinkName=pubmed_pubmed&from_uid=26564023%22>Related Articles</a></td></tr></table>

       <p><b>Quality of life of patients with fibrodysplasia ossificans progressiva.</b></p>          
       <p>J Child Orthop. 2015 Dec;9(6):489-93</p>
       <p>Authors:  Ortiz-Agapito F, Colmenares-Bonilla D</p>
       <p>Abstract<br/>
       INTRODUCTION: Fibrodysplasia ossificans progressiva (FOP) is a rare disorder characterized by episodes of acute pain and heterotopic ossification of soft tissue, and progressively limited physical function and social participation.<br/>
       OBJECTIVE: We aimed to determine the impact of FOP on quality of life, specifying areas or dimensions most affected.<br/>
       MATERIALS AND METHODS: This was a transverse observational study; patients with FOP were assessed using the Short Form 36. Questionnaire results were obtained using Quality Metric software and analyzed using frequency distribution, percentages and measures of central tendency.<br/>
       RESULTS: Eight patients, mean age 30.2 years, were included. The physical dimension was the most affected, with an average of 25.5 points. The most representative items were impaired function and physical role. Physical pain was found with an average of 44.5 points. The best scores were reported in the areas of emotional role and mental health, with an average of 79 and 76 respectively.<br/>
       CONCLUSIONS: FOP is a severely disabling disease, generating a significant deterioration in quality of life secondary to progressive deterioration in physical abilities. The findings of this study demonstrate good self-rated health of participants.<br/>
       </p><p>PMID: 26564023 [PubMed]</p>
   ]]></description>
   <author> Ortiz-Agapito F, Colmenares-Bonilla D</author>
   <category>J Child Orthop</category>
   <guid isPermaLink="false">PubMed:26564023</guid>

</item> <item>

   <title>Myositis ossificans traumatica of the medial pterygoid muscle following a third molar extraction.</title>         
   <link>http://www.ncbi.nlm.nih.gov/pubmed/25487562?dopt=Abstract</link>    
   <description>

<![CDATA[<table border="0" width="100%"><tr><td align="left"><a href="http://linkinghub.elsevier.com/retrieve/pii/S0901-5027(14)00433-0%22><img src="//www.ncbi.nlm.nih.gov/corehtml/query/egifs/http:--linkinghub.elsevier.com-ihub-images-PubMedLink.gif" border="0"/></a> </td><td align="right"><a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=Link&LinkName=pubmed_pubmed&from_uid=25487562%22>Related Articles</a></td></tr></table>

       <p><b>Myositis ossificans traumatica of the medial pterygoid muscle following a third molar extraction.</b></p>          
       <p>Int J Oral Maxillofac Surg. 2015 Apr;44(4):488-90</p>
       <p>Authors:  Torres AM, Nardis AC, da Silva RA, Savioli C</p>
       <p>Abstract<br/>
       Myositis ossificans (MO) is a rare disease involving heterotopic ossification in the muscle or soft tissue. Myositis ossificans traumatica (MOT) disease presents as a calcification within the injured muscle, resulting from a single or repetitive injury. There are few reports of MOT in the masticatory muscles. The case of a patient with MOT in the medial pterygoid muscle caused by a complication related to the extraction of an erupted upper third molar is reported. The major symptom was severe trismus. Despite surgical treatment, the disease relapsed. MOT can lead to serious consequences for the patient. Its aetiopathogenesis needs to be better understood, so that the most appropriate treatment is established and relapses are minimized. This will improve the quality of life of these patients. <br/>
       </p><p>PMID: 25487562 [PubMed - indexed for MEDLINE]</p>
   ]]></description>
   <author> Torres AM, Nardis AC, da Silva RA, Savioli C</author>
   <category>Int J Oral Maxillofac Surg</category>
   <guid isPermaLink="false">PubMed:25487562</guid>

</item> <item>

   <title>Fibrodysplasia ossificans progressiva: a case report.</title>         
   <link>http://www.ncbi.nlm.nih.gov/pubmed/24366806?dopt=Abstract</link>    
   <description>

<![CDATA[<table border="0" width="100%"><tr><td align="left"/><td align="right"><a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=Link&LinkName=pubmed_pubmed&from_uid=24366806%22>Related Articles</a></td></tr></table>

       <p><b>Fibrodysplasia ossificans progressiva: a case report.</b></p>          
       <p>J Orthop Surg (Hong Kong). 2013 Dec;21(3):383-6</p>
       <p>Authors:  Dhamangaonkar AC, Tawari AA, Goregaonkar AB</p>
       <p>Abstract<br/>
       Fibrodysplasia ossificans progressiva or myositis ossificans is a rare disease characterised by bony deposits or the ossification of soft tissues. It transforms skeletal muscles, tendons, ligaments, fascia, and aponeuroses into heterotopic bony deposits through an endochondral process. This leads to progressive immobility; patients are usually wheelchair-bound by the second decade of life and die of thoracic insufficiency by the fourth decade of life. There is no treatment apart from symptomatic treatment with steroids during flare-ups. Excision of heterotopic ossification is not recommended as it can provoke extensive and painful new growths. It is important to detect the disease early from characteristic signs of great toe abnormalities and heterotopic ossifications to improve quality of life through early physiotherapy.<br/>
       </p><p>PMID: 24366806 [PubMed - indexed for MEDLINE]</p>
   ]]></description>
   <author> Dhamangaonkar AC, Tawari AA, Goregaonkar AB</author>
   <category>J Orthop Surg (Hong Kong)</category>
   <guid isPermaLink="false">PubMed:24366806</guid>

</item> <item>

   <title>Fibrodysplasia ossificans progressiva: diagnosis in primary care.</title>         
   <link>http://www.ncbi.nlm.nih.gov/pubmed/23703055?dopt=Abstract</link>    
   <description>

<![CDATA[<table border="0" width="100%"><tr><td align="left"/><td align="right"><a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=Link&LinkName=pubmed_pubmed&from_uid=23703055%22>Related Articles</a></td></tr></table>

       <p><b>Fibrodysplasia ossificans progressiva: diagnosis in primary care.</b></p>          
       <p>Rev Paul Pediatr. 2013 Jan-Mar;31(1):124-8</p>
       <p>Authors:  Garcia-Pinzas J, Wong JE, Fernández MA, Rojas-Espinoza MA</p>
       <p>Abstract<br/>
       OBJECTIVE: To show that it is possible to diagnose fibrodysplasia ossificans progressiva in the primary health care.<br/>
       CASE DESCRIPTION: A 10-year-old female patient that has developed, since the age of 4, progressive stiffness of the joints and spine and ossification of soft tissues, often associated with trauma. The hallux valgus deviation of both toes was present from birth. X-ray showed the presence of heterotopic ossification.<br/>
       COMMENTS: This disease is likely to be diagnosed with the resources available in primary health care, since it is based on clinical findings. Currently, there is no cure for this disease, but high doses of corticosteroids and the use of nonsteroidal anti-inflammatory drugs, which are available in the primary care level, may limit the development of new calcifications and mitigate the pain, improving the quality of life of these patients.<br/>
       </p><p>PMID: 23703055 [PubMed - indexed for MEDLINE]</p>
   ]]></description>
   <author> Garcia-Pinzas J, Wong JE, Fernández MA, Rojas-Espinoza MA</author>
   <category>Rev Paul Pediatr</category>
   <guid isPermaLink="false">PubMed:23703055</guid>

</item> <item>

   <title>Evaluation of 20 years experience of fibrodysplasia ossificans progressiva in Iran: lessons for early diagnosis and prevention.</title>         
   <link>http://www.ncbi.nlm.nih.gov/pubmed/22526474?dopt=Abstract</link>    
   <description>

<![CDATA[<table border="0" width="100%"><tr><td align="left"><a href="http://dx.doi.org/10.1007/s10067-012-1968-6%22><img src="//www.ncbi.nlm.nih.gov/corehtml/query/egifs/http:--production.springer.de-OnlineResources-Logos-springerlink.gif" border="0"/></a> </td><td align="right"><a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=Link&LinkName=pubmed_pubmed&from_uid=22526474%22>Related Articles</a></td></tr></table>

       <p><b>Evaluation of 20 years experience of fibrodysplasia ossificans progressiva in Iran: lessons for early diagnosis and prevention.</b></p>          
       <p>Clin Rheumatol. 2012 Jul;31(7):1133-7</p>
       <p>Authors:  Raees-Karami SR, Jafarieh H, Ziyayi V, Shekarriz Foumani R, Aghighi Y</p>
       <p>Abstract<br/>
       Fibrodysplasia ossificans progressiva (FOP) is a rare genetic inflammatory disorder characterized by progressive heterotopic ossification presenting as recurrent soft tissue masses and swelling which may cause disabling, restricted joint mobility. Congenital malformations of the hallux are characteristic features of classic FOP, predating the appearance of disabling features. As no definite treatment is available, the early diagnosis and prevention of exacerbating factors may lead to significant benefits in terms of the life quality of patients. A retrospective study of 12 consecutive FOP patients referred to and admitted in the rheumatology unit at an urban tertiary care academic center between 1991 and 2011. Data, such as age, gender, and past medical history, were collected from the medical history, physical examination, and skeletal survey in order to characterize the clinical presentations. All 12 children (six boys and six girls; ages 2.0-13.5 years) had congenital malformations of the great toes (microdactyly and hallux valgus deformity), in addition to heterotopic ossification presenting as multiple soft tissue tumor-like swellings. Spinal involvement, most notably in the cervical region, suggestive of an early FOP, was present in 83.3 %. Eleven patients (91.6 %) had a prior history of direct physical trauma, while 7 of 11 (63.6 %) had undergone invasive diagnostic procedures, both correlating with the exacerbations of their condition. Clinical awareness of fibrodysplasia ossificans progressiva and its early diagnostic features, particularly congenital malformations of the hallux, during a thorough neonatal examination may lead to an early diagnosis preventing the development of disabling, practically irreversible lesions of heterotopic ossification. Genetic and molecular studies can play a considerable role in the diagnosis of FOP in suspected cases. Early institution of prophylactic and precautionary measures, such as categorical avoidance of trauma and invasive procedures, can significantly reduce the debilitating acute exacerbations of the condition.<br/>
       </p><p>PMID: 22526474 [PubMed - indexed for MEDLINE]</p>
   ]]></description>
   <author> Raees-Karami SR, Jafarieh H, Ziyayi V, Shekarriz Foumani R, Aghighi Y</author>
   <category>Clin Rheumatol</category>
   <guid isPermaLink="false">PubMed:22526474</guid>

</item> <item>

   <title>[Imaging features of neurologic and orthopedic complications from severe trauma].</title>         
   <link>http://www.ncbi.nlm.nih.gov/pubmed/21242935?dopt=Abstract</link>    
   <description>

<![CDATA[<table border="0" width="100%"><tr><td align="left"><a href="http://www.masson.fr/masson/MDOI-JR-12-2010-91-12-C2-0221-0363-101019-201004873%22><img src="//www.ncbi.nlm.nih.gov/corehtml/query/egifs/http:--www.masson.fr-images-Medline-goto3.gif" border="0"/></a> <a href="http://www.masson.fr/masson/MDOI-JR-12-2010-91-12-C2-0221-0363-101019-201004873%22><img src="//www.ncbi.nlm.nih.gov/corehtml/query/egifs/http:--www.masson.fr-images-Medline-goto.gif" border="0"/></a> </td><td align="right"><a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=Link&LinkName=pubmed_pubmed&from_uid=21242935%22>Related Articles</a></td></tr></table>

       <p><b>[Imaging features of neurologic and orthopedic complications from severe trauma].</b></p>          
       <p>J Radiol. 2010 Dec;91(12 Pt 2):1371-86</p>
       <p>Authors:  Ezra J, Roffi F, Eichwald F, Colas F, Mokhtari S, Le Breton C, Safa D, Genet F, Mompoint D, Vallée C, Carlier RY</p>
       <p>Abstract<br/>
       Cranial and spinal trauma are a frequent cause of disability in the general population. Post-traumatic paraplegia or quadriplegia or hemiplegia from vascular injury (CVA) can lead to early complications (respiratory, cardiovascular, urinary, cutaneous, infectious...) that may have an impact on the immediate prognosis. Neurologic and orthopedic complications occur later and further impair the quality of life of patients. Orthopedic complications include: neurogenic paraosteoarthropathy (NPOA) or neurogenic osteoma or myositis ossificans (NMO). The nomenclature currently in use is NMO; Osseous complications: osteoporosis and secondary insufficiency fractures; Joint complications: degenerative arthropathy and stiffness; Overuse mechanical complications; Muscular complications; Infectious complications: arthritis and myositis complicating skin ulcers and bed sores. The purpose of this paper is to describe these neuro-orthopedic complications and review their imaging features.<br/>
       </p><p>PMID: 21242935 [PubMed - indexed for MEDLINE]</p>
   ]]></description>
   <author> Ezra J, Roffi F, Eichwald F, Colas F, Mokhtari S, Le Breton C, Safa D, Genet F, Mompoint D, Vallée C, Carlier RY</author>
   <category>J Radiol</category>
   <guid isPermaLink="false">PubMed:21242935</guid>

</item> <item>

   <title>[Fibrodysplasia ossificans progressiva. Anesthetic management of a 2-year-old child].</title>         
   <link>http://www.ncbi.nlm.nih.gov/pubmed/20238093?dopt=Abstract</link>    
   <description>

<![CDATA[<table border="0" width="100%"><tr><td align="left"><a href="http://dx.doi.org/10.1007/s00101-010-1707-9%22><img src="//www.ncbi.nlm.nih.gov/corehtml/query/egifs/http:--production.springer.de-OnlineResources-Logos-springerlink.gif" border="0"/></a> </td><td align="right"><a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=Link&LinkName=pubmed_pubmed&from_uid=20238093%22>Related Articles</a></td></tr></table>

       <p><b>[Fibrodysplasia ossificans progressiva. Anesthetic management of a 2-year-old child].</b></p>          
       <p>Anaesthesist. 2010 Jun;59(6):535-8</p>
       <p>Authors:  Iber T, Klösel S, Schoenes B, Zacharowski K</p>
       <p>Abstract<br/>
       Fibromyalgia ossificans progressiva (FOP) is a severely disabling disorder of connective tissue characterized by congenital malformation of the toes, fingers and vertebrae associated with progressive ossification of striated muscles. Anesthetic management of these patients involves preferably general anesthesia as local or regional anesthesia should be avoided due to possible heterotopic ossification. Airway management is determined by the age of the patient and the progression of the disease. Only a few cases in the literature have reported the anesthetic management of FOP patients and to our knowledge only one case has been published on pediatric patients. In adult, cooperative patients awake fiberoptic intubation is recommended, as ankylosis of the temporo-mandibular joint is the most important clinical feature for anesthesia. As demonstrated and discussed in this case report of a 2-year-old boy, fiberoptic intubation after induction of general anesthesia should be preferred in pediatric patients. Puncture of a vein should be non-traumatic, i.m. injections strictly avoided and careful positioning and padding are needed. Every effort should be made to avoid situations stimulating new heterotopic ossification due to its substantial effect on the quality of life of FOP patients.<br/>
       </p><p>PMID: 20238093 [PubMed - indexed for MEDLINE]</p>
   ]]></description>
   <author> Iber T, Klösel S, Schoenes B, Zacharowski K</author>
   <category>Anaesthesist</category>
   <guid isPermaLink="false">PubMed:20238093</guid>

</item> <item>

   <title>Surgical outcome in fibrodysplasia ossificans progressiva: A case study.</title>         
   <link>http://www.ncbi.nlm.nih.gov/pubmed/17618217?dopt=Abstract</link>    
   <description>

<![CDATA[<table border="0" width="100%"><tr><td align="left"/><td align="right"><a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=Link&LinkName=pubmed_pubmed&from_uid=17618217%22>Related Articles</a></td></tr></table>

       <p><b>Surgical outcome in fibrodysplasia ossificans progressiva: A case study.</b></p>          
       <p>Ortop Traumatol Rehabil. 2004 Oct 30;6(5):658-64</p>
       <p>Authors:  Wzietek B, Frańczuk B</p>
       <p>Abstract<br/>
       Background. Fibrodysplasia ossificans progressiva (FOP), also known as Munchmeyer's Disease, is a rare inherited disease. By 1996 there had been no more than 200 cases described in world literature. The disease is characterized by progressive ectopic ossification and congenital deformity of the hallux. Patients with FOP require full-time nursing care. Case history. This article presents the case of a female patient, initials LP, age 42, who has been suffering from FOP since early childhood. The authors describe the course of the disease, the patient's current clinical status, the results of surgical treatment for a decubitus ulcer on the right thigh, and a review of the literature on this rare disease. At present the patient is almost completely immobilized. The spine is completely stiffened in a slightly kyphotic position. The ribs are not mobile. The decubitus ulcer has been excised. After surgery on the knee, the range of motion in the operated knee joint increased to 20 degrees . The improvement in mobility is stable and has remained unchanged for 16 months after surgery. Conclusions. In this patient with FOP it turned out to be possible to operate on the knee, provided that a delicate surgical technique was used, with the appropriate use of non-steroid analgesic drugs, along with biphosphonians and glycosteroids. The range of motion in the knee was increased by releasing the tendon of the femoral biceps, which improved the patient's quality of life.<br/>
       </p><p>PMID: 17618217 [PubMed]</p>
   ]]></description>
   <author> Wzietek B, Frańczuk B</author>
   <category>Ortop Traumatol Rehabil</category>
   <guid isPermaLink="false">PubMed:17618217</guid>

</item> <item>

   <title>Management decisions for unusual periampullary tumors.</title>         
   <link>http://www.ncbi.nlm.nih.gov/pubmed/9322675?dopt=Abstract</link>    
   <description>

<![CDATA[<table border="0" width="100%"><tr><td align="left"/><td align="right"><a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=Link&LinkName=pubmed_pubmed&from_uid=9322675%22>Related Articles</a></td></tr></table>

       <p><b>Management decisions for unusual periampullary tumors.</b></p>          
       <p>Am Surg. 1997 Oct;63(10):927-32</p>
       <p>Authors:  Todd KE, Lewis MP, Gloor B, Kusske AM, Ashley SW, Reber HA</p>
       <p>Abstract<br/>
       The pessimism associated with the treatment of pancreatic cancer may result in inappropriate management in certain patients thought to have that disease. We analyzed the recent UCLA experience with a variety of periampullary tumors in which various issues concerning management were unusual. The records of nine patients (age 15-75 years) with pancreatic or periampullary tumors were reviewed retrospectively. The tumor was evident on CT scan in all patients. The diameter of the mass was greater than 5 cm in five cases. Eight of the tumors appeared to arise from the pancreas, but at exploration, two were found to originate from other structures (duodenum and retroperitoneum). One patient with an apparent gastric lesion on CT scan was found to have a mass of pancreatic origin at operation. Operative procedures included: pancreaticoduodenectomy (four), distal pancreatectomy (three), total pancreatectomy (one), and retroperitoneal tumor resection (one). Pathological diagnoses included: solid and papillary epithelial neoplasm (two), mucinous cystic neoplasm (two), serous microcystic adenoma (two), myositis ossificans (one), degenerative neurilemoma (one), spindle cell tumor (one), and intraductal papillary carcinoma (one). We conclude that patients with large or unusual-appearing pancreatic or periampullary tumors should be managed aggressively. Major resections can be done safely with the achievement of an excellent quality of life in individuals at the extremes of age. Unlike the usual pancreatic ductal adenocarcinoma, the prognosis for many of these neoplasms is excellent.<br/>
       </p><p>PMID: 9322675 [PubMed - indexed for MEDLINE]</p>
   ]]></description>
   <author> Todd KE, Lewis MP, Gloor B, Kusske AM, Ashley SW, Reber HA</author>
   <category>Am Surg</category>
   <guid isPermaLink="false">PubMed:9322675</guid>

</item> <item>

   <title>Outcome of persistent vegetative state following hypoxic or traumatic brain injury in children and adolescents.</title>         
   <link>http://www.ncbi.nlm.nih.gov/pubmed/8737825?dopt=Abstract</link>    
   <description>

<![CDATA[<table border="0" width="100%"><tr><td align="left"><a href="http://www.thieme-connect.com/DOI/DOI?10.1055/s-2007-973756%22><img src="//www.ncbi.nlm.nih.gov/corehtml/query/egifs/http:--www.thieme.de-classic-images-tc-logo_tc.jpg" border="0"/></a> </td><td align="right"><a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=Link&LinkName=pubmed_pubmed&from_uid=8737825%22>Related Articles</a></td></tr></table>

       <p><b>Outcome of persistent vegetative state following hypoxic or traumatic brain injury in children and adolescents.</b></p>          
       <p>Neuropediatrics. 1996 Apr;27(2):94-100</p>
       <p>Authors:  Heindl UT, Laub MC</p>
       <p>Abstract<br/>
       Persistent vegetative state (PVS, apallic syndrome) has become a significant medical and social problem. The outcome of young people with PVS is a matter of great interest. Therefore, we analysed the outcome of 127 children and adolescents who were in PVS for at least 30 days following traumatic (n = 82) or hypoxic (n = 45) brain injury. After 19 months of follow-up, 84% of the patients of the traumatic brain injury (TBI) group, but only 55% of the hypoxic brain injury (HBI) group had left PVS (p &lt; 0.001). The TBI patients regained consciousness earlier. Later than 9 months post trauma less than 5% of the patients of both groups left PVS. Hypoxic brain injury patients had a higher incidence of seizures (p = 0.01) and a higher seizure frequency. They had significantly more complications like pneumonia, gastrointestinal disturbance or myositis ossificans (= heterotopic ossification). Posttraumatic hyperthermia and autonomic dysfunctions were correlated with worse outcome in the TBI group, but not in the HBI group. Thirteen patients (16%) with TBI became independent in everyday life versus only two (4%) with HBI. These results underline the important contribution of hypoxia in severe and permanent brain impairment. They also may help to establish the prognosis of children in PVS.<br/>
       </p><p>PMID: 8737825 [PubMed - indexed for MEDLINE]</p>
   ]]></description>
   <author> Heindl UT, Laub MC</author>
   <category>Neuropediatrics</category>
   <guid isPermaLink="false">PubMed:8737825</guid>

</item>

</channel>

References[edit | edit source]

↑ ^1.00 ^1.01 ^1.02 ^1.03 ^1.04 ^1.05 ^1.06 ^1.07 ^1.08 ^1.09 ^1.10 ^1.11 ^1.12 ^1.13 ^1.14 ^1.15 ^1.16 ^1.17 ^1.18 ^1.19 ^1.20 Lakkireddy M, Chilakamarri V, Ranganath P, Arora A, Vanaja M. Clinical and Genetic Analysis of Fibrodysplasia Ossificans Progressiva: A Case Report and Literature Review. Journal Of Clinical & Diagnostic Research [serial online]. August 2015;9(8):1-3. Available from: Academic Search Complete, Ipswich, MA. Accessed March 19, 2016.
↑ ^2.00 ^2.01 ^2.02 ^2.03 ^2.04 ^2.05 ^2.06 ^2.07 ^2.08 ^2.09 ^2.10 ^2.11 ^2.12 ^2.13 ^2.14 ^2.15 International Fibrodysplasia Ossificans Progressiva Association. 2009. Available at: http://www.ifopa.org/. Accessed March 22, 2016.
↑ ^3.00 ^3.01 ^3.02 ^3.03 ^3.04 ^3.05 ^3.06 ^3.07 ^3.08 ^3.09 ^3.10 Cite error: Invalid <ref> tag; no text was provided for refs named Pig
↑ ^4.0 ^4.1 ^4.2 ^4.3 ^4.4 ^4.5 ^4.6 ^4.7 Kaplan FS, Le merrer M, Glaser DL, et al. Fibrodysplasia ossificans progressiva. Best Pract Res Clin Rheumatol. 2008;22(1):191-205.
↑ ^5.0 ^5.1 ^5.2 Ulusoy H. Fibrodysplasia ossificans progressiva without characteristic skeletal anomalies. Rheumatol Int. 2012;32(5):1379-82.
↑ ^6.0 ^6.1 ^6.2 Abhishek K, Jain S, Khadgawat R. A case of fibrodysplasia ossificans progressiva: 20 years of follow-up. Neurology India [serial online]. March 2016;64(2):354-356. Available from: Academic Search Complete, Ipswich, MA. Accessed March 22, 2016.

[Lak-1] 1.00 ^1.01 ^1.02 ^1.03 ^1.04 ^1.05 ^1.06 ^1.07 ^1.08 ^1.09 ^1.10 ^1.11 ^1.12 ^1.13 ^1.14 ^1.15 ^1.16 ^1.17 ^1.18 ^1.19 ^1.20 Lakkireddy M, Chilakamarri V, Ranganath P, Arora A, Vanaja M. Clinical and Genetic Analysis of Fibrodysplasia Ossificans Progressiva: A Case Report and Literature Review. Journal Of Clinical & Diagnostic Research [serial online]. August 2015;9(8):1-3. Available from: Academic Search Complete, Ipswich, MA. Accessed March 19, 2016.

[IFOP-2] 2.00 ^2.01 ^2.02 ^2.03 ^2.04 ^2.05 ^2.06 ^2.07 ^2.08 ^2.09 ^2.10 ^2.11 ^2.12 ^2.13 ^2.14 ^2.15 International Fibrodysplasia Ossificans Progressiva Association. 2009. Available at: http://www.ifopa.org/. Accessed March 22, 2016.

[Pig-3] 3.00 ^3.01 ^3.02 ^3.03 ^3.04 ^3.05 ^3.06 ^3.07 ^3.08 ^3.09 ^3.10 Cite error: Invalid <ref> tag; no text was provided for refs named Pig

[Kap-4] 4.0 ^4.1 ^4.2 ^4.3 ^4.4 ^4.5 ^4.6 ^4.7 Kaplan FS, Le merrer M, Glaser DL, et al. Fibrodysplasia ossificans progressiva. Best Pract Res Clin Rheumatol. 2008;22(1):191-205.

[Ulusoy-5] 5.0 ^5.1 ^5.2 Ulusoy H. Fibrodysplasia ossificans progressiva without characteristic skeletal anomalies. Rheumatol Int. 2012;32(5):1379-82.

[Abhishek-6] 6.0 ^6.1 ^6.2 Abhishek K, Jain S, Khadgawat R. A case of fibrodysplasia ossificans progressiva: 20 years of follow-up. Neurology India [serial online]. March 2016;64(2):354-356. Available from: Academic Search Complete, Ipswich, MA. Accessed March 22, 2016.

[1]

[2]

[3]

[4]

[5]

[6]

Fibrodysplasia Ossificans Progressiva: Difference between revisions

Revision as of 02:32, 4 April 2016

Contents

Definition/Description[edit | edit source]

Prevalence[edit | edit source]

Characteristics/Clinical Presentation[edit | edit source]

Prognosis
[edit | edit source]

Medications[edit | edit source]

Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]

Etiology/Causes[edit | edit source]

Systemic Involvement[edit | edit source]

Medical Management (current best evidence)[edit | edit source]

Physical Therapy Management (current best evidence)[edit | edit source]

Differential Diagnosis
[edit | edit source]

Case Reports/ Case Studies[edit | edit source]

Resources
[edit | edit source]

Recent Related Research [edit | edit source]

References[edit | edit source]

Physiopedia

Content

Legal

Fibrodysplasia Ossificans Progressiva: Difference between revisions

Revision as of 02:32, 4 April 2016

Definition/Description[edit | edit source]

Prevalence[edit | edit source]

Characteristics/Clinical Presentation[edit | edit source]

Prognosis [edit | edit source]

Medications[edit | edit source]

Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]

Etiology/Causes[edit | edit source]

Systemic Involvement[edit | edit source]

Medical Management (current best evidence)[edit | edit source]

Physical Therapy Management (current best evidence)[edit | edit source]

Differential Diagnosis[edit | edit source]

Case Reports/ Case Studies[edit | edit source]

Resources [edit | edit source]

Recent Related Research [edit | edit source]

References[edit | edit source]

Our Partners

Physiopedia

Content

Legal

Prognosis
[edit | edit source]

Differential Diagnosis
[edit | edit source]

Resources
[edit | edit source]