icd-10 code for gorham stout

by Shania Jacobi 5 min read

ICD-10:M89. 5 (- NTBT (ORPHA code's Narrower Term maps to a Broader Term). - Attributed (The ICD code is attributed by Orphanet).)

Full Answer

What is the latest version of the ICD-10 for gout?

Gout, unspecified. The 2019 edition of ICD-10-CM M10.9 became effective on October 1, 2018. This is the American ICD-10-CM version of M10.9 - other international versions of ICD-10 M10.9 may differ.

Is Gorham-Stout syndrome a monocentric bone disease?

In 1999, Möller and colleagues concluded, "The Gorham-Stout syndrome may be, essentially, a monocentric bone disease with a focally increased bone resorption due to an increased number of paracrine – or autocrine – stimulated hyperactive osteoclasts. The resorbed bone is replaced by a markedly vascularized fibrous tissue.

How is Gorham's disease (Gorham disease) diagnosed?

Often, Gorham's disease is not recognized until a fracture occurs, with subsequent improper bone healing. The diagnosis essentially is one of exclusion and must be based on combined clinical, radiological, and histopathological findings.

What does M10 gout mean?

M10 Gout. Gout is a common, painful form of arthritis. It causes swollen, red, hot and stiff joints. Gout occurs when uric acid builds up in your blood. This happens if your body produces extra acid or does not eliminate enough, or if you eat too many foods with purines, such as liver and dried beans.

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What is Gorham-Stout disease?

Gorham-Stout disease (GSD), which is also known as vanishing bone disease, disappearing bone disease, massive osteolysis, and more than a half-dozen other terms in the medical literature, is a rare bone disorder characterized by progressive bone loss (osteolysis) and the overgrowth (proliferation) of lymphatic vessels.

How common is Gorham-Stout disease?

Gorham's disease is extremely rare and may occur at any age, though it is most often recognized in children and young adults. It strikes males and females of all races and exhibits no inheritance pattern. The medical literature contains case reports from every continent.

What is Gorham's?

Gorham's disease is a rare bone disorder characterized by bone loss (osteolysis), often associated abnormal blood vessel growth (angiomatous proliferation). Bone loss can occur in just one bone, or spread to soft tissue and adjacent bones. Symtoms may include pain, swelling, and increased risk of fracture.

Is Gorham disease painful?

Gorham's disease is a rare bone disorder characterized by bone loss (osteolysis), often associated abnormal blood vessel growth (angiomatous proliferation). Bone loss can occur in just one bone, or spread to soft tissue and adjacent bones. Symtoms may include pain, swelling, and increased risk of fracture.

Is Gorham-Stout disease fatal?

Diagnosis of Gorham-Stout disease is made by exclusion, and its clinical presentation varies widely, from spontaneous remission to a fatal outcome.

What does Gorham-Stout disease feel like?

The clinical presentation of a patient suffering from vanishing bone disease includes, most frequently, pain, functional impairment and swelling of the affected region, although asymptomatic cases have been reported, as well as cases in which the diagnosis was made after a pathologic fracture[20].

When was the first case of Gorham-Stout disease?

The first case of Gorham-Stout disease was already reported in 1838 and described a patient with a vanishing of the complete humerus bone over 11 years (19). After more than a century, in 1955, Gorham and Stout investigated the characteristic histopathological findings of the massive osteolysis based on 8 cases.

What is Boneitis?

Boneitis is a deadly disease that cripples the bones, ultimately resulting in death. Steve Castle, who was from the 20th century, was stricken with the disease. Symptoms include bone twisting, bone breaking and bone loss.

What are the 3 major bone diseases?

Low bone density and osteoporosis, which make your bones weak and more likely to break. Osteogenesis imperfecta makes your bones brittle. Paget's disease of bone makes them weak. Bones can also develop cancer and infections.

What is the treatment for Gorham's disease?

The medical treatment for Gorham's disease includes radiation therapy, anti-osteoclastic medications (bisphosphonates), and alpha-2b interferon. Surgical treatment options include resection of the lesion and reconstruction using bone grafts and/or prostheses.

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Epidemiology

To date around 300 cases have been reported in the literature. GSD does not display a clear race, sex predilection (1.6:1; male: female ratio) or geographic distribution.

Clinical description

GSD can present at any age, but is commonly diagnosed in children and young adults (average 13 years). GSD may affect any bone in the body but most commonly affects the ribs, followed by the cranium, clavicle and cervical spine. Additional affected areas include maxillofacial bones (mostly the mandible), sternum, humerus, hand, femur, and foot.

Etiology

Etiology of GSD is still elusive. The pathological process is the benign vascular proliferation of endothelial channels adjacent to or within bone, leading to extreme thinning of bony trabecula, osteoclast-mediated resorption, and replacement of bone with fibrous tissue.

Diagnostic methods

Diagnosis relies on radiographic findings revealing progressive osteolysis and cortical destruction. Magnetic resonance imaging shows complete resorption of bone and replacement with infiltrative soft tissue that is of low signal intensity on T1-weighted imaging and high signal intensity on T2, with intense enhancement on contrast imaging.

Differential diagnosis

Differential diagnosis includes generalized lymphatic anomaly (the major distinguishing characteristic is the progressive osteolysis seen in GSD), acroosteolysis dominant type, multicentric carpo-tarsal osteolysis with or without nephropathy, autosomal recessive carpotarsal osteolysis, hereditary sensory and autonomic neuropathy type 2, Farber lipogranulomatosis, Torg-Winchester syndrome, and idiopathic phalangeal acro-osteolysis (see these terms).

Management and treatment

Treatment of GSD includes drugs (bisphosphonates and/or interferon alpha 2b, sirolimus is also being studied) to stabilize progressive disease, and supportive procedures that may reduce or halt chylothorax (pleurectomy, pleurodesis, thoracentesis, and thoracic duct embolization or ligation), or may stabilize affected regions of the skeleton.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

Convert M89.8X9 to ICD-9 Code

The General Equivalency Mapping (GEM) crosswalk indicates an approximate mapping between the ICD-10 code M89.8X9 its ICD-9 equivalent. The approximate mapping means there is not an exact match between the ICD-10 code and the ICD-9 code and the mapped code is not a precise representation of the original code.

Information for Patients

Your bones help you move, give you shape and support your body. They are living tissues that rebuild constantly throughout your life. During childhood and your teens, your body adds new bone faster than it removes old bone. After about age 20, you can lose bone faster than you make bone.

What is the ICD10 code for Gorham Stout disease? And the ICD9 code for Gorham Stout disease?

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What are the most common sites of Gorham's disease?

It may affect any part of the skeleton, but the most common sites of disease are the shoulder, skull, pelvic girdle, jaw, ribs, and spine. In some cases, no symptoms are seen until a fracture occurs either spontaneously or following minor trauma, such as a fall.

What is the end result of Gorham's disease?

In many cases, the end result of Gorham's disease is severe deformity and functional disability. Symptoms such as difficulty breathing and chest pain may be present if the disease is present in the ribs, scapula, or thoracic vertebrae. These may indicate that the disease has spread from the bone into the chest cavity.

How does Gorham's disease affect bone mass?

The specific cause of Gorham's disease remains unknown. Bone mass and strength are obtained and maintained through a process of bone destruction and replacement that occurs at the cellular level throughout a person's life. Cells called osteoclasts secrete enzymes that dissolve old bone, allowing another type of cells called osteoblasts to form new bone. Except in growing bone, the rate of breakdown equals the rate of building, thereby maintaining bone mass. In Gorham's disease, that process is disrupted.

Is Gorham's disease a differential diagnosis?

Recognition of the disease requires a high index of suspicion and an extensive workup. Because of its serious morbidity, Gorham's must always be considered in the differential diagnosis of osteolytic lesions.

Is Gorham's disease experimental?

To date, no known interventions are consistently effective for Gorham's, and all reported interventions are considered experimental treatments, though many are routine for other conditions. Some people may require a combination of these approaches. Unfortunately, some people will not respond to any intervention.

Is Gorham's disease palliative or progressive?

Treatment of Gorham's disease is for the most part palliative and limited to symptom management. Sometimes, the bone destruction spontaneously ceases and no treatment is required, but when the disease is progressive, aggressive intervention may be necessary.

Can Gorham's disease be fatal?

The disease may stabilize after a number of years , go into spontaneous re mission, or in cases involving the chest and upper spine, prove fatal. Recurrence of the disease following remission can also occur. Involvement of the spine and skull base may cause a poor outcome from neurological complications. In many cases, the end result of Gorham's ...

What is gouty tophus?

Gouty tophus of right olecranon bursa. Clinical Information. A condition marked by increased levels of uric acid in the blood, joints, and tissue. The buildup of uric acid in the joints and tissues causes arthritis and inflammation. Gout is a common, painful form of arthritis.

Why does gout make my joints swell?

It causes swollen, red, hot and stiff joints. Gout occurs when uric acid builds up in your blood. This happens if your body produces extra acid or does not eliminate enough, or if you eat too many foods with purines, such as liver and dried beans. Pseudogout has similar symptoms and is sometimes confused with gout.

What is Gorham Stout disease?

Disease. Gorham-Stout disease (GSD) is a rare, progressive angiomatous disorder characterized by osteolysis and vascular anomalies . GSD, also known as “vanishing bone disease,” frequently involves a bone of the axial skeleton . The disorder is characterized by resorption, cortical loss, and progressive osteolysis of bone with an angiomatosis ...

When was GSD first diagnosed?

Epidemiology. GSD, first characterized in the 1950s, has both congenital and acquired forms . Although GSD primarily affects children and young adults, there is a bimodal distribution pattern and some patients present after the age of 50 years . GSD shows no predilection for gender or race .

What is GSD in ophthalmology?

GSD is a rare disorder characterized by progressive osteolysis and angiomatosis of blood vessels and lymphatic vessels. Although ocular manifestations of GSD are rare, the ophthalmologist can play an important role in the diagnosis of GSD if a patient presents with proptosis with cortical resorption, massive osteolysis, and angiogenic malformations. Medication, surgery, and radiation are available treatment options, but appropriate management on a case-by-case basis would lead to favorable results in GSD patients.

What are the symptoms of GSD?

Signs/Symptoms. Localized pain is the most common symptom, while other symptoms include weakness, swelling, and functional impairment of affected limbs . GSD patients may be asymptomatic until they experience a minor trauma or a fracture .

Is GSD pathophysiology?

The pathophysiology of GSD is not entirely known . Angiogenic and osteoclastogenic cytokine production, including vascular endothelial growth factor (VEGF) and interleukin-6 (IL-6), are hypothesized to drive the disease processes . VEGF and VEGF-C are overexpressed in GSD patients, indicating their implications in the pathologic endothelial cell proliferation . Furthermore, VEGF and VEGF-C are also involved in disease activity and response to interferon-α (IFN-α) therapy in some patients . Amounts of IL-6, VEGF-A, and tumor necrosis factor-α (TNF-α) are elevated in ex vivo cells from a patient’s soft-tissue lesion .

Is GSD a clinical diagnosis?

GSD may be a likely diagnosis if a patient presents an early asymptomatic clinical course, positive biopsy of angiomatous tissue, progressive osteolysis of a bone in the axial skeleton, and osteolytic radiographic pattern with negative hereditary, metabolic, immunologic, infectious, or neoplastic etiology .

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