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Osteitis Fibrosa Disseminata
Bone Fibrous Dysplasia

Presentation

Three cases of particular interest are presented in detail. In one, both pituitary gigantism (in addition to severe deformity of facial bones) and hyperthyroidism were present. [amjmed.com]

Degree of confidence Plain radiographs are highly specific when characteristic features are present in a lesion. [emedicine.medscape.com]

The patient to be presented has been under constant medical scrutiny since the onset of symptoms at the age of three, and [annals.org]

It is clinically characterized by a triad of Pigmentation ( café-au-lait macule), polyostotic/monostotic fibrous dysplasia (FD) and precocious puberty (PP). [1], [2] A full presentation is rare, and it is more common in females [3], [4], [5] who present [ijpd.in]

If Albright's syndrome is present, an operation on the parathyroids can be avoided, since these glands are not involved in... Bibliography 1. [acpjournals.org]

Entire Body System

  • Goiter

    The thyroid gland, a butterfly-shaped organ at the base of the neck, may become enlarged (a condition called a goiter) or develop masses called nodules. [ghr.nlm.nih.gov]

    These include generalized enlargement called goiter and irregular masses called nodules and cysts. Some patients have subtle structural changes detected only by ultrasonography. [rxlist.com]

    Joseph: Osteodystrophia fibrosa combined with precocious puberty and exophthalmic goiter. Pathologic Report of a case. Amer. J. Dis. Childr. 63, 748–783 (1942). Google Scholar Thannhauser, S. [link.springer.com]

    Acromegaly, multinodular goiter and silent polyostotic fibrous dysplasia: a variant of the McCune-Albright syndrome. J Endocrinol Invest. 1990; 13: 671-675 PubMed Google Scholar Albright F. Polyostotic fibrous dyplasia: a defense of the entity. [cell.com]

    This is always associated with low or undetectable TSH levels and most patients have multinodular goiters rather than the typical findings of Grave's disease. [7] Rare association includes the Cushing's syndrome, [3] hyperparathyroidism [2], [4], [5] [jpgmonline.com]

  • Accelerated Growth

    In a previous study, the median age at the diagnosis/manifestation of acromegaly was 24 years of age, and GH excess was associated with accelerated growth in 85% of pediatric patients (9). [frontiersin.org]

    GH excess is associated with growth acceleration and/or facial dysmorphism. However, growth acceleration may be obscured in MAS patients with PP, and facial dysmorphism is often difficult to assess due to craniofacial FD. [eje.bioscientifica.com]

Skin

  • Cafe-Au-Lait Spots

    When melanotic pigmentation (cafe-au-lait spots) and multiple endocrine hyperfunction are additionally associated it is referred to as albright syndrome ICD-9-CM Volume 2 Index entries containing back-references to 756.54 : [icd9data.com]

    Cafe-au-lait spots-light brown hyperpigmented macular skin lesions with irregular borders-"Coast of Maine" cafe-au-lait spots. Remain constant in size and number. [gait.aidi.udel.edu]

    Some children have no cafe-au-lait pigment at all; in a few, it is confined to small areas, such as the nape of the neck or crease of the buttocks. There are seldom any medical problems associated with the areas of cafe-au-lait pigment. [rxlist.com]

    SKIN Some individuals with MAS have abnormal skin coloring (pigmentation) known as café-au-lait spots. Café-au-lait spots are abnormal patches of light-brown skin that have irregularly-shaped, jagged borders. [rarediseases.org]

    Abstract Objective McCune–Albright syndrome (MAS) is a sporadic, postzygotic disease presenting with fibrous dysplasia, cafe-au-lait spots and multiple endocrinopathies. [eje.bioscientifica.com]

Neurologic

  • Trigeminal Neuralgia

    neuralgia, anomia, and epiphora. [17] [18] Clinical diagnosis Clinical diagnosis relies on both facial changes and imaging characteristics. [eyewiki.aao.org]

  • Hyperactivity

    Complications: (1) bone deformity, which may result in facial asymmetry (2) bone fractures (3) chondroblastic sarcoma (4) short adult height (following precocious puberty) (5) cranial nerve compression with blindness and deafness (6) life threatening endocrine hyperactivity [meducator3.net]

Workup

"Parathyroid Carcinoma: Differential Diagnoses & Workup". eMedicine. Archived from the original on 2009-05-27. Retrieved 2009-01-28. ^ a b Arabi, A. (2006). "Regression of Skeletal Manifestations of Hyperparathyroidism with Oral Vitamin D". [en.wikipedia.org]

Treatment

We analyze the difficulties associated with treating a patient with poor bone quality over several years, discuss our treatment options, review the literature for similar cases and look at what we could have done differently. [scirp.org]

Make informed clinical choices for each patient, from diagnosis and treatment selection through post-treatment strategies and management of complications, with new evidence-based criteria throughout. [books.google.com]

Generally, no treatment is required in asymptomatic cases. [dovemed.com]

Our patient received external irradiation treatment and long-acting octreotide therapy (Sandostatin LAR). With this treatment, serum growth hormone and serum IGF-1 levels decreased markedly but not to normal range. [turkjem.org]

Treatment with medication is also of vast value. [eje.bioscientifica.com]

Prognosis

The prognosis is dependent upon the severity of the disorder Currently, there are no known methods to prevent Fibrous Dysplasia The types of Fibrous Dysplasia include: Monostotic Fibrous Dysplasia: When only a single bone is involved Polyostotic Fibrous [dovemed.com]

Diagnosis - McCune Albright syndrome Prognosis - McCune Albright syndrome The long-term outlook (prognosis) for people with McCune Albright syndrome (MAS) varies depending on the nature and severity of features in each affected person. [checkorphan.org]

Part I explores basic techniques to researching mccune-albright syndrome (e.g. finding guidelines on diagnosis, treatments, and prognosis), followed by a number of topics, including information on how to get in touch with organizations, associations, [amazon.in]

Prognosis Fibrous dysplasia has a good prognosis with low rates of malignant transformation. [eyewiki.aao.org]

A report showed that in 8 out of 11 instances where cavities caused by OFC were filled with transplanted bone, the lesion healed and the transplanted bone blended rapidly and seamlessly with the original bone.[37] Prognosis[edit] Almost all who undergo [en.wikipedia.org]

Etiology

It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology. Fibrous dysplasia represents about 2, 5% of all bone tumors and over 7% of all benign tumours. [journal-imab-bg.org]

Another author in 1938 Lichtenstein coined the term 'Fibrous Dysplasia of bone' and described it as a different entity of unknown etiology. [omicsonline.org]

Etiology of fibrous dysplasia and McCune-Albright syndrome. Int J Oral Maxillofac Surg 1999;28:366–371. 20. Zimmerman DC, Dahlin DC, Stafne EC. Fibrous dysplasia of the maxilla and mandible. Oral Surg Oral Med Oral Pathol 1958;11:55–68. 21. [synapse.koreamed.org]

Etiology The etiology of hyperparathyroidism in sporadic cases is unknown; however, hereditary aspects of the disease have been identified. Details of the disorders, related conditions, and treatment considerations are discussed below. [emedicine.medscape.com]

[…] possibly hypogonadotrophic hypogonadism [8] and soft tissue myxomas. [9] Association of multiple endocrinopathies is a rare occurrence, although gigantism and hyperthyroidism in one case of Albright syndrome was recorded by Firat and Stutzman. [5] The etiology [jpgmonline.com]

Epidemiology

Metyrapone may also be used for treatment. [12] Epidemiology [ edit ] McCune–Albright syndrome is estimated to occur at a frequency between 1 person in 100,000 to 1 person in 1,000,000 individuals worldwide. [15] Notable cases [ edit ] Mauricio Saravia [en.wikipedia.org]

Pathophysiology

Pathophysiology, evaluation, and treatment. J Bone Joint Surg [Am]. 2005;87:1848-64. Han I, Choi ES, Kim HS. Monostotic fibrous dysplasia of the proximal femur: natural history and predisposing factors for disease progression. [ijoro.org]

Surgical treatment of hyperparathyroidism requires a thorough understanding of the pathophysiology of the disease and the embryologic basis of parathyroid gland surgical anatomy. [emedicine.medscape.com]

"The Role of Type 1 and Type 2 5′-Deiodinase in the Pathophysiology of the 3,5,3′-Triiodothyronine Toxicosis of McCune-Albright Syndrome". The Journal of Clinical Endocrinology and Metabolism. 93 (6): 2383–2389. doi : 10.1210/jc.2007-2237. [en.wikipedia.org]

[…] of all orbital tumors (0.6-2%). [4] Fibrous dysplasia represents approximately 18% of all primary orbital bone tumors. [4] The majority of cases with orbital involvement have monostotic fibrous dysplasia of the frontal, sphenoid or ethmoid bones. [4] Pathophysiology [eyewiki.aao.org]

Prevention

The prognosis is dependent upon the severity of the disorder Currently, there are no known methods to prevent Fibrous Dysplasia The types of Fibrous Dysplasia include: Monostotic Fibrous Dysplasia: When only a single bone is involved Polyostotic Fibrous [dovemed.com]

Alendronate is at present indicated for use in prevention and treatment of osteoporosis and Paget’s disease. It should be used in conjunction with adequate amounts of vitamin D and calcium in the prevention of progressive loss of bone mass. [indianpediatrics.net]

Bisphosphonates are helpful in relieving bone pain, but it is no longer believed that they prevent progression of the disease. [en.wikipedia.org]

Albright like syndrome, Osteitis fibrosa disseminata, Polyostotic, fibrous dysplasia, Precocious puberty with polyostotic fibrosis and abnormal pigmentation, MAS, PFD, POFD Symptoms - McCune Albright syndrome Causes - McCune Albright syndrome Prevention [checkorphan.org]

No known prevention exists for fibrous dysplasia. [eyewiki.aao.org]

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