Summary

Lymphangiomatosis is a rare developmental disorder characterised by diffuse proliferation of anastomosing lymphatic channels (lymphangiomas). It is believed to result from anomalous lymphatic development and usually presents in childhood. It typically occurs as a part of systemic lymphangiomatosis and isolated organ involvement is rare. Only nine cases of isolated cystic lymphangiomatosis of spleen have been reported between 1990 and 2010. Tuberculosis is a significant health problem in India and varied forms of this disease are seen in clinical practice. Isolated splenic tuberculosis, though a rare entity, has been described in the Indian population. We present a case of isolated splenic lymphangiomatosis in a 42-year-old woman that was initially misdiagnosed as splenic tuberculosis and was treated accordingly. Unresponsive to medical treatment, the patient underwent splenectomy and on histopathological examination, cystic lymphangiomatosis was diagnosed. The patient’s symptoms resolved after surgery and she is doing well at a follow-up of 3 months.