Epidemiology

• xxx

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Physiology

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

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Diagnosis

• xxx

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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

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Treatment

Management of Chylothorax

• Ligation of Thoracic Duct: may be required

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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]