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CASE REPORT  
Year : 2013  |  Volume : 47  |  Issue : 5  |  Page : 515-518
Percutaneous osteoplasty in treatment of bone lymphangiomatosis


Department of Trauma and Orthopaedic, University Clinic Hospital of Valencia, Valencia, Spain

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Date of Web Publication17-Sep-2013
 

   Abstract 

Primary bone lymphangiomatosis is a disease of unknown etiology that can cause lytic lesions in long bones, the pelvis, the spinal column and the cranium. We are presenting the case of a woman with localized bone lymphangiomatosis in the left knee. The authors believe this is the first case in which percutaneous osteoplasty was used in long bones for the treatment of bone lesions resulting from this disease showing good clinical results.

Keywords: Bone, knee, lymphangiomatosis, osteoplasty, percutaneous

How to cite this article:
Mifsut D, Renovell P, Gomar F, Saravia M. Percutaneous osteoplasty in treatment of bone lymphangiomatosis. Indian J Orthop 2013;47:515-8

How to cite this URL:
Mifsut D, Renovell P, Gomar F, Saravia M. Percutaneous osteoplasty in treatment of bone lymphangiomatosis. Indian J Orthop [serial online] 2013 [cited 2019 Dec 10];47:515-8. Available from: http://www.ijoonline.com/text.asp?2013/47/5/515/118210

   Introduction Top


Lymphangioma is a rare, primary, benign, bone disease that was first described by Bickel and Broders in 1947. [1] The lesion is composed of noncommunicating, sequestered, lymphoid tissue that is surrounded by lymphatic endothelium. [2] Its etiology is unknown; however, it has been associated with a congenital blockage of lymphatic drainage, inflammatory processes, toxins, etc. The purpose of this work is to present a case of bone lymphangiomatosis with involvement of distal femoral and proximal tibial epiphysis and metaphysis, with no visceral involvement.


   Case Report Top


We present a case of a 34 year old woman who was diagnosed as bone lymphangiomatosis in the femur and tibia. The patient had undergone surgery for the lower limb discrepancy. Epiphysiodesis distal of the right femur was done at the age of 12 years and left femoral lengthening at the age of 14 years. Since, the age of 21 years the patient has had symptoms of lymphangitis, with an acute inflammatory process in the left thigh and leg as well as signs and symptoms of phlogosis, pain, edema, functional impairment and a fever of 39.5°C. Laboratory tests showed leukocytosis with left shift. Throughout the years, she suffered an additional thirteen episodes of lymphangitis, which was bacterial in some cases.

At the age of 29, the patient underwent curettage and filling of the femoral and tibial cavities with freeze-dried bone and autogenous cancellous bone from the iliac crest. As a result of these procedures, it was observed that this was a particular case of dysplasia as there was an accumulation of lymph in the interior of the cavity as well, which suggested lymphatic dysplasia or bone lymphangiomatosis. Reabsorption of the bone grafts was later observed, which coincided with two additional episodes of lymphangitis.

The differential diagnosis included neoplastic and proliferative bone marrow disorders, especially histiocytosis; as well as benign lesions such as solitary bone cysts, fibrous dysplasia, fibrous bone defects, cystic fibrous osteitis resulting from hyperparathyroidism and congenital generalized fibromatosis. She was diagnosed by histopathological study and she was treated by percutaneous osteoplasty and was followed for 2 years and 10 months in the femur and 2 years and 3 months in tibia.

The radiographic imaging preosteoplasty showed large areas of medullary resorption and cortical thinning of distal femoral and proximal tibial epiphysio metaphysial region [Figure 1], CT scans revealed decreased trabeculation and endosteal scalloping with cortical thinning [Figure 2]. Magnetic resonance imaging (MRI) scans revealed diffuse lymphangiomatosis of bones and soft-tissues [Figure 3].
Figure 1: Anteroposterior (a) and lateral (b) radiographs of knee joint (preosteoplasty) showing large areas of medullary resorption (large arrow) and cortical thinning (small arrow)

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Figure 2: CT scan of knee joint femoral level cut right (a) and left side (b); tibial level cut right (c) and left side (d) showing decreased trabeculation and endosteal erosion with cortical thinning

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Figure 4: Histopathological section showing abundant lymphatic vascular spaces, distinguished from arterial and venous spaces (H and E, ×20)

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Histological sections stained with H and E, which revealed dense fibro-connective tissue with an abundance of large-caliber vascular channels [Figure 4]. Said channels were lined by flat monostratified epithelium, which tested positive for D2-40 (antibody that specifically stains lymphatic endothelial cells) through a specific immunohistochemical technique [Figure 5]. The postoperative images show cavities filled with acrylic cement in tibia (December 2010) [Figure 6] and femur (May 2010) [Figure 7]. After over 2 years of followup (2 years and 10 months in the femur and 2 years and 3 months in tibia) there are no signs of disease progression at this level in the radiographic images [Figure 8].
Figure 5: Immunohistochemical section showing positive D2-40 antibody in lymphatic endothelial cells and negative D2-40 antibody in the vascular endothelium

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Figure 6: Postoperative radiographic image of knee joint anteroposterior (a) and lateral (b) views showing femoral osteoplasty with the filling of the bone cavity

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Figure 7: Postoperative radiographic image of knee joint anteroposterior (a) and lateral (b) views showing osteoplasty tibia with good cement filling

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Figure 8: Radiographic image anteroposterior (a) and lateral (b) views (more than 2 years of followup) showing no signs of disease progression and good intramedullary support

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   Discussion Top


Lymphangioma or bone lymphangiomatosis is a rare disease, with equal gender distribution and no hereditary tendency, that usually onsets during the first 20 years of life. [3] It is most frequently localized in the diaphysis or metaphysis of the tibia and humerus as well as the iliac bone, skull, jaw and vertebrae. Lesions are usually discovered at birth (50-65% of cases) or at least during the first 20 years of life (90%). Lesions can be solitary or multifocal, but its disseminated form, known as Gorham-Stout disease has a worst prognosis because of visceral involvement. [2]

Lymphangiomas, similar to hemangiomas, are classified and sub-classified according to the size of the vessels, such as capillary, cavernous, cystic, or mixed. [4] The most common variant is cystic. Radiographic study revealed well defined lytic bone images of geographic features in the bones. Radiographic differential diagnosis includes neoplastic and proliferative bone marrow disorders, especially histiocytosis; as well as benign lesions such as solitary bone cysts, fibrous dysplasia, fibrous bone defects, cystic fibrous osteitis resulting from hyperparathyroidism and congenital generalized fibromatosis. [3],[5]

CT scans reveal the presence of bone gaps while MRI scans reveal hypointense signal on T1-weighted images and hyperintense on T2-weighted images, with no postgadolinium injection uptake. In the histopathological study, the D2-40 monoclonal antibody is a marker of the vascular lymphatic endothelium that allows distinguishing between the endothelium of arterial and venous vessels of the lymphatic vessels in tissue sections. [6] With regard to treatment, excision is recommended for isolated lesions; however, in lymphangiomatosis, especially when associated with osteolysis, treatment usually involves multiple curettage, bone grafting and radiotherapy. [7] Other treatments that have been used include bisphosphonates [8],[9] and interferon-alpha. [2],[6]

In our case, which has been the only one described at the knee, curettage and bone grafting was not successful as there was reabsorption of the bone graft. In 2005, Wallace and Ross, [10] used acrylic cement in a case of lymphangiomatosis affecting the sacrum with good pain relief results; therefore, we decided to perform this technique in our case, in order to manage pain and provide stability to the bony architecture of the lesion and were able to verify the effectiveness of this technique in the achievement of our objectives.

To conclude, percutaneous osteoplasty can be considered as a therapeutic option in cases of long bone marrow lymphangiomatosis in which, in addition to achieving pain management, it provides the structural support of the bone cavities caused by lymphangiomatosis. However, it is not a biological solution, hence further long term followup is warranted.

 
   References Top

1.Bickel WH, Broders AC. Primary lymphangioma of the ilium. Report of a case. J Bone Joint Surg 1961;43A: 107-17.  Back to cited text no. 1
    
2.Schultz K, Rosenberg AE, Ebb DH, Mankin HJ. Lower-Extremity Lymphangiomatosis. A case report with a seventeen-year followup. J Bone Joint Surg 2005;87A: 162-7.  Back to cited text no. 2
    
3.Steiner GM, Farman J, Lawson JP. Lymphangiomatosis of bone. Radiology 1969;93:1093-8.  Back to cited text no. 3
[PUBMED]    
4.Sökmensüer C, Sungur A, Tokgözoglu M, Ruacan S. Lymphangiomatosis of bone. A case report. Int Orthop 1995;19:63-4.  Back to cited text no. 4
    
5.Nixon GW. Lymphangiomatosis of bone demonstrated by lymphangiography. Am J Roentgenol Radium Ther Nucl Med 1970;110:582-6.  Back to cited text no. 5
[PUBMED]    
6.Fukunaga M. Expression of D2-40 in lymphatic endothelium of normal tissues and in vascular tumours. Histopathology 2005;46:396-402.  Back to cited text no. 6
[PUBMED]    
7.Khan AQ, Sherwani MK, Gupta K, Agarwal S. Lymphangiomatosis of tibia. Saudi Med J 2008;29:923, 924.  Back to cited text no. 7
[PUBMED]    
8.Avelar RL, Martins VB, Antunes AA, de Oliveira Neto PJ, Andrade ES. Use of zoledronic acid in the treatment of Gorham's disease. Int J Pediatr Otorhinolaryngol 2010;74:319-22.  Back to cited text no. 8
[PUBMED]    
9.Sun S, Liu X, Ma B, Zhou Y, Sun H. Could local deliver of bisphosphonates be a new therapeutic choice for Gorham-Stout syndrome? Med Hypotheses 2011;76:237-8.  Back to cited text no. 9
[PUBMED]    
10.Wallace MJ, Ross M. Bone lymphangiomatosis: Treatment with percutaneous cementoplasty. Spine (Phila Pa 1976) 2005;30:E336-9.  Back to cited text no. 10
    

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Correspondence Address:
Damián Mifsut
Blasco Ibanez Av. 18, 46010 Valencia
Spain
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5413.118210

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]

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