Gorham-Stout Disease (Gorham’s Disease)

Epidemiology

  • xxx

Physiology

  • Generalized Lymphatic Anomaly with Lymphatic Proliferation
    • Lymphatic Proliferation in Bones Results in Cortical Bone Loss and Progressive Bone Resorption
    • Sporadic, Non-Hereditary

Diagnosis

  • xxx

Clinical Manifestations

General Comments

  • Onset: usually before age 20
  • Multi-Organ Involvement: 75% of cases have involvement of more than one organ

Cardiovascular Manifestations

Chylopericardium (see Chylopericardium, [[Chylopericardium]])

  • Epidemiology: may occur

Gastrointestinal Manifestations

Chylous Ascites (see Chylous Ascites, [[Chylous Ascites]])

  • Epidemiology: may occur

Neurologic Manifestations

Weakness (see xxxx, [[xxxx]])

  • Epidemiology: may occur

Pulmonary Manifestations

Chylothorax (see Pleural Effusion-Chylothorax, [[Pleural Effusion-Chylothorax]])

  • Epidemiology: may occur
  • Diagnosis
    • Pleural Fluid (see Thoracentesis, [[Thoracentesis]])
      • Triglyceride: >110 mg/dL
      • Chylomicrons: detected
  • Clinical
    • Chylothorax May Be Unilateral or Bilateral

Rheumatologic Manifestations

Bone Pain

  • Main Bones Affected
    • Pelvic Girdle
    • Shoulders
    • Skull

Pathologic Fractures

  • Main Bones Affected
    • Pelvic Girdle
    • Shoulders
    • Skull

Treatment

Management of Chylothorax

  • Ligation of Thoracic Duct: may be required

References

  • Diffuse pulmonary lymphangiomatosis. Hum Pathol. 1993 Dec;24(12):1313-22 [MEDLINE]
  • Pulmonary lymphangiomatosis. Lymphat Res Biol. 2011;9(4):191-3. doi: 10.1089/lrb.2011.0023 [MEDLINE]
  • Gorham’s disease and diffuse lymphangiomatosis in children and adolescents. Pediatr Blood Cancer. 2011 Apr;56(4):667-70. doi: 10.1002/pbc.22948. Epub 2010 Dec 22 [MEDLINE]
  • Gorham-Stout disease and generalized lymphatic anomaly–clinical, radiologic, and histologic differentiation. Skeletal Radiol. 2013 Jul;42(7):917-24. doi: 10.1007/s00256-012-1565-4. Epub 2013 Jan 31 [MEDLINE]