However, the root causes of these conditions remains unknown and further research is necessary. Diagnosis
Because it is rare and has a wide spectrum of clinical, histological, and imaging features, diagnosing lymphangiomatosis can be challenging. Plain x-rays reveal the presence of lytic lesions in bones, pathological fractures, interstitial infiltrates in the lungs, and chylous effusions that may be present even when there are no outward symptoms.The most common locations of lymphangiomatosis are the lungs and bones and one important diagnostic clue is the coexistence of lytic bone lesions and chylous effusion. An isolated presentation usually carries a better prognosis than does multi-organ involvement; the combination of pleural and peritoneal involvement with chylous effusions and lytic bone lesions carries the least favorable prognosis.When lung involvement is suspected, high resolution computed tomography (HRCT) scans may reveal a diffuse liquid-like infiltration in the mediastinal and hilar soft tissue, resulting from diffuse proliferation of lymphatic channels and accumulation of lymphatic fluid; diffuse peribronchovascular and interlobular septal thickening; ground-glass opacities; and pleural effusion. Pulmonary function testing reveals either restrictive pattern or a mixed obstructive/restrictive pattern. While x-rays, HRCT scan, MRI, ultrasound, lymphangiography, bone scan, and bronchoscopy all can have a role in identifying lymphangiomatosis, biopsy remains the definitive diagnostic tool.Microscopic examination of biopsy specimens reveals an increase in both the size and number of thin walled lymphatic channels along with lymphatic spaces that are interconnecting and dilated, lined by a single attenuated layer of endothelial cells involving the dermis, subcutis, and possibly underlying fascia and skeletal muscle.
Signs and symptoms are typically present before the age of 20 and the condition is often under-recognized in adults.It affects males and females of all races and exhibits no inheritance pattern. The medical literature contains case reports from every continent.Because it is so rare, and commonly misdiagnosed, it is not known exactly how many people are affected by this disease. References
Further reading
Dellinger M. T., Garg N, Olsen B.R. (2014). "Viewpoints on vessels and vanishing bones in Gorham-Stout disease". Bone. 63C: 47–52. doi:10.1016/j.bone.2014.02.011. PMID 24583233. {{cite journal}}:  CS1 maint: multiple names: authors list (link)
Trenor C, Chaudry G (Aug 2014). "Complex lymphatic anomalies". Semin Pediatr Surg. 23 (4): 186–90. doi:10.1053/j.sempedsurg.2014.07.006. PMID 25241096. Lala S, Mulliken JB, Alomari AI, Fishman SJ, Kozakewich HP, Chaudry G (Jul 2013). "Gorham-Stout disease and generalized lymphatic anomaly—clinical, radiologic, and histologic differentiation". Skeletal Radiol. 42 (7): 917–24. doi:10.1007/s00256-012-1565-4. PMID 23371338. S2CID 22640055. {{cite journal}}:  CS1 maint: multiple names: authors list (link)