Lymphangiomatosis with Extensive Skeletal Involvement

Abstract

Aim: To review a case presentation of systemic lymphangiomatosis- an uncommon medical condition that can be easily misdiagnosed in the clinical setting

Abstract: Generalized lymphangiomatosis is a rare condition with characteristic multi-centric angiomatous malformations of the skeletal system, with involvement of surrounding soft tissues and skin.  It results from a congenital anomaly and presents predominantly in the paediatric age group.  Systemic involvement includes not only the skeletal system, but also the pulmonary and gastrointestinal systems. Its diagnosis can be difficult to ascertain but is hinged on clinical presentation, imaging and histological confirmation.  The condition carries a variable prognosis as treatment options are very limited.  Pulmonary involvement is usually terminal, however.  A case is presented to highlight the difficulties in diagnosing and managing this condition.

Conclusion: The diagnosis of lymphangiomatosis involving the skeletal system is highly reliant on imaging studies; that depict well defined osteolytic changes of the bone. Management is supportive and no cure currently exists.

Keywords: lymphangiomatosis, systemic, skeletal involvement