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Lymphology 16 (1983) 181 --:184 ©Georg Thieme Verlag Stuttgart · New York

Lymphangiomatosis of Bone and Soft Tissue (Results of lymphangiographic examinations)

A.F. Tsyb, I. Kh. Mukhamedzhanov, L.l. Guseva

Research Institute of Medical , Academy of Medical Sciences of the USSR , Obninsk, USSR

Summary monstrates thinning and protrusion. Histologic The results of clinical roentgenologic examination examination shows lymphatic sinuses limited of 2 patients with lymphangiomatosis are discussed. to epithelium. The diagnosis is more often The analysis of angiographic findings has shown based on soft tissue histology. Lymphography that arteries and veins remained unchanged in affect· is of great value as it can reveal considerable ed area. Lymphography revealed fully changed lym· phatics in affected areas. Numerous markedly dilat· changes in lymphatics (4, 5, 6, 7). ed lymph vessels and cystic cavities of different The purpose of this study was to demonstrate shape and size were noted. In bony spongiosus sub· stance the fissural spaces were filled with oily con· diagnostic significance of lymphography during trast medium. Roentgenologically, they appeared as examination of patients with lymphangiomato· numerous small conglomerates limited to bone con· sis of bone and soft tissue . tours. Case Reports Lymphangiomatosis of bone has been report· Case 1. Female patient, N., 31 years. At 12 ed under various names: massive osteolysis, years of age, pains and swelling in the right essential osteolysis, acute spontaneous bone knee joint had arised without apparent cause, absorption, primary lymphangioma of bone temperature was found to be elevated. The and others. In 1838 Jackson described first patient received antiphlogistic therapy. One lymphangiomatosis of bone. More recently year after onset of disease a fracture of the 39 cases were reported (2). neck of the right femur happened. An open of soft tissues and bones in the upper The etiology of this disease is unknown, but third of the right femur was performed. Histo· all the authors consider lymphangiomatosis logically, proliferation of epithelized lymph to be a basis of the process. Lymphangioma· vessels was noted. Cystic angiomatosis of tosis occurs at the young age. The first symp· bones and soft tissues of the right lower ex· toms include pathologic bone fracture and tremity was diagnosed. The patient was seen deformity. The process progresses as a rule at 29 years of age in the clinic of Research and involves adjacent bones and soft tissues. Institute of Medical Radiology. The right Lymphangiomatosis is the subject of some lower extremity was shorter by 18 em than case reports which suggest that all the bones the left. The hip and knee joints showed lim· of the skeleton can be involved. Bone lesions ited motion. Soft tissues of this limb were of the hand and the foot in this disease are swollen. The skin of the lower leg showed rare. Specific roentgenologic signs are as fol· numerous blue patches and areas of thinning lows: lytic osteopathy, sones of decreased from which lymph exuded. Survey roentgeno· density and cysts, the walls of which have grams of the right lower extremity, the shell-like contours or are eaten. Areas of and the vertebral column demonstrated a sclerosis are always absent. The corticalis de·

Permission granted for single print for individual use. Reproduction not permitted without permission of Journal LYMPHOLOGY. 182 A.F. Tsyb, l.Kh. Mukhamedzhanov, L.l. Guseva marked deformity of the femur, the ischial and pubic bones on the right. The femoral head and a part of the femoral neck were absent and the 5th lumbar vertebra was some what shorter. A deformity of the knee joint and massive marginal bone proliferations were observed. On the background of com­ mon osteoporosis the structure of these bones was found to. be cellular with cystic changes in some areas. Follow-up roentgeno­ graphy during 7 years demonstrated marked progression of the earlier changes and involve· ment of adjacent bones. Selective of right half of the pel­ vis and the lower limb was performed. Major arteries and veins appeared normal. Then, di­ rect lymphography was performed using Myo· dil, which was injected in 7 ml into the lympl vessels on the dorsum of the feet. Lympho­ grams of the right lower limb showed marked changes in the lymphatics (Fig. 1). ln soft tis· sues, numerous markedly dilated vessels and cystic cavities of different shape and size, where contrast medium persisted, were seen. Inguinal, iliac and lumbar lymphatics and lymph nodes on the right were not opacified Fig. 1 Lymphogram of the leg shows numerous because of increased capacity of the leg lym­ markedly dilated lymphatics and lymph cysts of different shape and size. Note persistence of con­ phatics. The lymph vessels of the left limb trast material. Capacity of lymphatic bed of the remained unchanged. Unusual lymphatics de­ extremity is increased viated from the left inguinal plexus towards the ischial bone. Analogous lymphatics de­ viated from the iliac plexus towards the up­ Roentgenologically, an acute osteomyelitis per flaring portion of the ilium and the 4th was diagnosed. The boy underwent conserva­ and 5th lumbar vertetirae. The most part of tive treatment. On examination in the clinic contrast material has filled fissural spaces, of our Institute a slight protrusion of the formed in spongy substance of these bones. right iliac bone backwards, swelling in the Radiologically, numerous small conglomerates right inguinal region without clear lirrtits were limited to bone contours were noted (Fig. 2). noted. The skin appeared normal. There was In contrast to lymphatics the lymph nodes no pain on joint motion. Radiologic findings showed milder changes. Their sinusal system showed deformity of the pelvic girdle, struc­ was filled irregularly and stained less intensive. tural cystic changes in the iliac, ischial and Diagnosis on the basis of lymphangiographic pubic bones on the right. Cysts were irregu­ findings: lymphangiomatosis of soft tissues lar in shape and different in size with clear and bones of the right lower extremity, the contours. Angiography did not demonstrate pelvis and lumbar part of the vertebral col­ any alteration of arteries and veins. Bilateral umn. foot lymphography with the use of Lipiodol Case II. Male patientS., 12 years. Two years UF revealed no changes in limb lymphatics. ago a strong pain in the right half of the pel­ In the upper inguinal region and the iliac one vis appeared and temperature was higher. on the right, numerous deformed varicose

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Fig. 2A Lymphogram obtained on filling phase. Fig. 28 Lymphogram obtained on storage phase . Contrast material is entering spongy substance of Note persistence of oily contrast material in the the 5th lumbar vertebra pelvic bones and especially, in the body of the 5th lumbar vertebra

lymphatics and lymph cysts of different size Discussion were seen . In dilated lymphatics and cystic Lymphangiomatosis is a rare, insufficient:\y cavities oily contrast medium broke down explored disease . Little is known about nor­ into separate drops {Fig. 3A). After 24 h mal and pathologic state of the osteal lymph contrast medium was on the whole removed system. Lymphographic findings suggest deep from the dilated lymphatics of the right up­ morphologic and functional changes in the per inguinal and iliac regions. It was accumu­ lymph vessels of bones and adjacent soft tis­ lated by the acetabular roof, in rami of the sues. Angiography excludes angiomatosis of ischial and pubic bones as well as in the up­ blood vessels. Follow-up roentgenography for per flaring portion of the ilium on the right. a few years shows progression of the process In cystic cavities of changed bones storage and involvement of new regions. of contrast medium was noted (Fig. 3B). 4 years later repeat lymphography revealed pro­ At the present time it is discussed, whether gression of lymphangiomatous changes: The the process originates in bone or in soft tis­ right upper plexus iliaci communis and the sues. plexus lumbalis were found to be affected.

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Fig. 3A Lymphogram obtained on filling phase . In Fig. 38 Lymphogram obtained on storage phase . the inguinal and iliac region on the righ numerous Note accumulation of oily contrast material by the varicose lymphatics and lymph cysts of different acetabular roof, in rami of the ischial and pubic bones, size are seen as well as in the upper flaring portion of the ilium on the right

Bickel and Broders consider lymphangiomatosis 2 Halliday, D.R., D.C. Dahlin, D.C. Pugh, H.H. as a primary lymphangioma of bone. These Joung: Massive osteolysis and angiomatosis. Radio logy 82: 4 (1964) 637 - 644 authors inclicate that histologically, the perio· 3 Jackson, J.B.S.: A singular case of absorption of steum of a normal bone contains a dense net­ bone (a bone-less ). S.J. 11: 18 (1838) 368- work of lymphatic capillaries. Therefore, dis· 369 ease originates perhaps from the periosteum. 4 Menzhinskaya, G. V., A.F. Tsyb: Lymphangiogra· But taking into account the fact that the lym­ phy in diagnostics of combined lymphangiomata­ sis of bone and soft tissue. Ortopediya, travmato· phatics in Haversian canals accompany the logiya i protesirovaniye 1 (1971) 35 - 38 blood vessels we suppose that bones are pri· 5 Nixon, G.: Lymphangiomatosis of bone demon­ marily affected. strated by lymphangiography. Am. J. Roentgenol. 10: 3 (1970) 582- 586 6 Reilly, B.J. , J. W. Dawidson, H. Bain: Lymphangi· References ectasis of the skeleton. A case report. Radiology 103 (1972) 385 - 386 1 Bickel, W.H., A.C. Broders.· Primary lymphangio· 7 Winterberger, A.R.: The roentgenologic diagnosis: rna of the ilium: report of a case. Joint. Surg. 29 lymphangiomatosis of bone. Radiology 162: 2 (1947) 517 - 522 (1972) 321 - 324

A.F. Tsyb,Research Institute of Medical Radiology, Academy of Medical Sciences of the USSR, Obninsk, USSR

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