1. C31 Gorham-Stout Disease (GSD)

2. Outline What is Gorham-Stout Disease What does it looks like
Who can get it
Imaging 
Prognosis
Case
Treatment
Resources

3. What is Gorham-Stout Disease
Characterized by the pathologic process Osteolysis—Destruction of bone
Types I-IV: Gorham-Stout is IV
Idiopathic
Affects multiple body systems
Skeletal, Lymphatic, Nervous
Diagnosis Criteria
"Progressive osteolysis with resorption and cortical loss'
Typically regional, but can affect more than one area
Extremely rare only 300 cases are recorded in medical literature 

4. What does it look like Patient presented with chronic pelvic pain
Patients first radiograph taken in 1985 
Second taken a year later

5. What does it look like 
Distortion of the left clavicle and scapula in a 12 year old 

6. Who can get it Usually found in young and adults up to the age of 40
also found in infants less than a month old and adults older than 70
Affects more males than females
GSD is so rare it goes undiagnosed or misdiagnosed
No connection to ethnicity or heredity 

7. Imaging
" Plain X-rays, initially, show radiolucent foci in the intramedullary or subcortical regions and, later, slowly progressive atrophy, dissolution, fracture, fragmentation and disappearance of a part of a bone, with tapering or “pointing” of the remaining osseous tissue and atrophy of soft tissues"
X-ray
Bone Density
MRI

8. Prognosis Generally positive prognosis
Better prognosis if the disease affects extremities rather than the spine or ribs
Can be fatal if the disease creates neurological or lymphatic problems

9. Case
9 year old girl presents with trouble breathing and pain in lower extremity
Modalities used for diagnosis
X-rays CT
MRI
Nuclear medicine: lymphoscintigraphy
Findings
Multiple osteolytic lesions in the left tibia and fibula
Lytic mass on the right clavicle
Pleural effusions
Chylothorax
Multiple fractures

10. Citation: Davalos EA, Gandhi NM, Barank D. Varma RK
Citation: Davalos EA, Gandhi NM, Barank D. Varma RK. Gorham-Stout disease presenting with dyspnea and bone pain in a 9-year-old girl. Radiology Case Reports. (Online) 2015;10(2);1110

11. Treatment Each treatment is specific to the patients symptoms
Surgery to remove the affected areas
Bone grafts have been used to support new bone growth in conjunction with surgery
Interferon for lymphatic system
Bisphosphonates for slowing bone destruction
Radiation therapy had been proven affective. 
Positive results have been seen with a total dose of Gy
For upper extremity positive results were seen with a total dose of 15 Gy

12. Resources
Davalos EA, Gandhi NM, Barank D. Varma RK. Gorham-Stout disease presenting with dyspnea and bone pain in a 9-year-old girl. Radiology Case Reports. (Online) 2015;10(2);1110
El-Kouba G, de Araújo Santos R, Pilluski P, Severo A, Lech O. GORHAM-STOUT SYNDROME: PHANTOM BONE DISEASE. Revista Brasileira de Ortopedia (English Edition) ;45(6): doi: /s (15)30313-x.
Gorham-Stout Disease - NORD (National Organization for Rare Disorders). NORD (National Organization for Rare Disorders) Available at: diseases/gorham-stout-disease. Accessed March 11, 2018.
Kulenkampff, HA., Richter, G.M., Hasse, W.E. et al. International Orthopaedics (1990) 14: 361. 
Lala, S., Mulliken, J.B., Alomari, A.I. et al. Skeletal Radiology (2013) 42:
Liu, Y., Zhong, DR., Zhou, PR. et al. Clin Rheumatol (2016) 35:
Stöve, J. & Reichelt, A. Arch Orthop Trauma Surg (1995) 114: