This rare congenital disorder can affect the bones or the visceral organs. Skeletal involvement leads to pathological fracture, joint deformity and may cause pain. The bones are filled with a network of interconnected lymph channels containing fluid.

The pattern of skeletal involvement varies, with multiple contiguous bones heavily involved and others completely normal. Other patients present with multiple cystic lesions in non-contiguous bones combined with lymph fluid collections in the chest and abdominal cavities (chylothorax and chyloperitoneum). Generalized lymphedema of the extremity may occur.

There have been isolated cases of association with other mesenchymal dysplasias, such as Mafucci syndrome and osteochondromas. Gorham's disease (idiopathic massive osteolysis, dissapearing bone disease) may be an aggressive form of lymphangiomatosis. A familial cluster of cases with an autosomal dominant pattern of inheritance has been reported.

Individuals without visceral involvement have a better prognosis. The skeletal lesions may involute or inprove after skeletal maturity. The author is not aware of any report of sarcomatous degeneration in these lesions.

Treatment is centered on minimizing skeletal complications and the associated deformity. Bisphosphonate medications may limit the bone loss and the risk of pathologic fracture associated with expansion of the lesions. The optimum dosage and pharmacokinetics of the oral bisphosphonate medicines available for these conditions have not yet been fully established in children, but there use is under active investigation.

Surgical intervention is necessary for certain pathological fractures and to correct symptomatic deformity. Fractures treated with non-surgical techniques can be expected to heal. Other minimally invasive interventions that have been used to treat related lesions, such as percutaneous osteoplasty as used for hemangioma of bone, may hold some promise. One report of the use of radiation to control these lesions in an inoperable location has been published.

Since the condition is very rare, each case must be evaluated and treated as a unique entity.

In the case illustrated here, severe involvement of the pelvis and the resulting pathological collapse of the articular surface of the femoral head lead to disabling pain. This was treated by Girdlestone-type resection arthroplasty of the hip.

At surgery, the lymph fluid seeps and runs from the cut bone surface, with the fluid welling up and drawing back into the bone in response to slight changes of the central venous pressure.

Ref:

Mirra, Bone Tumors Lea and Febinbger, pp1426

11/2002 HD

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