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Cavernous lymphangioma
I NSIGHTS
Cavernous lymphangioma
A A male infant was born with swelling and deformity of soft tissues of the left leg from the upper thigh to the toes, with visible vascular channels (Figure, A). Normal bony architecture of the lower limb was palpable. Plain radiographs showed no osteolytic lesion. Magnetic resonance imaging (MRI) revealed a massive lymphatic malformation with macrocystic spaces in the foot and lower leg (Figure, B); significant arterial or venous components of the lesion were not demonstrated. Needle aspiration of serosanguineous fluid from the foot, followed by sclerotherapy into the subcutaneous space, resulted in complete decompression of the cystic mass and adhesion of the tissues to the thigh and upper leg. At 3 weeks of age, excision of the redundant tissues of the foot resulted in decompression of the residual cystic lesions on the ankle and lower leg and significant contour J Pediatr 2001;138:146. *References available from authors. Copyright © 2001 by Mosby, Inc. 0022-3476/2001/$35.00 + 0 9/45/110998 doi:10.1067/mpd.2001.110998
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B improvement. Histopathologic examination of the excised skin revealed diffuse lymphatic elements of intermediate and large caliber in the subepidermal and deeper tissues. Recurrent swelling of the left lower leg was noted at 2 years of age. Repeat MRI showed a venous component to the malformation. Subsequent sclerotherapy yielded good results (Figure, C). The child is currently 3 years old and is fully ambulatory. Lymphangiomatosis is a rare developmental malformation of lymphoid tissue characterized by aberrant lymphatic proliferation in soft tissues, bones, or parenchymal organs with obliteration of draining lymphatics causing obstruction.* There are 4 major forms: lymphangioma simplex, lymphangioma circumscriptum, cavernous lymphangioma, and cystic hygroma. Most cases are present at birth or develop in the first 2 years of life. Cavernous lymphangioma presents as a large, diffuse, poorly defined, fluctuant soft tissue mass with cystic dilation of the lymphatics in the dermis, subcutaneous tissue, and intermuscular septa.* Skin changes such
C as hyperplasia, vesicle formation, and hyperpigmentation may also be seen. Histologically, cavernous lymphangioma is characterized by dilated lymph channels lined by a flattened layer of endothelial cells with separation of the dermal collagen bundles.* Imaging studies such as computed tomography* or MRI* may help define the extent of tissue involvement and in guiding treatment. Treatment options include surgical excision and imageguided sclerotherapy. Sclerotherapy can obliterate the cystic spaces within the malformation, thus abrogating the need for debulking surgery. Eugene H. Ng, MD, Vibhuti S. Shah, MD, MRCP, Derek C. Armstrong, MD, FRCPC, and Howard M. Clarke, MD, PhD, FRCSC, FAAP, FACS, NeonatalPerinatal Medicine, The Hospital for Sick Children, University of Toronto; Department of Newborn and Developmental Paediatrics, Sunnybrook and Women’s College Health Sciences Centre, University of Toronto; Division of Neuroradiology, Department of Radiology, The Hospital for Sick Children, University of Toronto; Division of Plastic Surgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Cavernous lymphangioma
A A male infant was born with swelling and deformity of soft tissues of the left leg from the upper thigh to the toes, with visible vascular channels (Figure, A). Normal bony architecture of the lower limb was palpable. Plain radiographs showed no osteolytic lesion. Magnetic resonance imaging (MRI) revealed a massive lymphatic malformation with macrocystic spaces in the foot and lower leg (Figure, B); significant arterial or venous components of the lesion were not demonstrated. Needle aspiration of serosanguineous fluid from the foot, followed by sclerotherapy into the subcutaneous space, resulted in complete decompression of the cystic mass and adhesion of the tissues to the thigh and upper leg. At 3 weeks of age, excision of the redundant tissues of the foot resulted in decompression of the residual cystic lesions on the ankle and lower leg and significant contour J Pediatr 2001;138:146. *References available from authors. Copyright © 2001 by Mosby, Inc. 0022-3476/2001/$35.00 + 0 9/45/110998 doi:10.1067/mpd.2001.110998
146
B improvement. Histopathologic examination of the excised skin revealed diffuse lymphatic elements of intermediate and large caliber in the subepidermal and deeper tissues. Recurrent swelling of the left lower leg was noted at 2 years of age. Repeat MRI showed a venous component to the malformation. Subsequent sclerotherapy yielded good results (Figure, C). The child is currently 3 years old and is fully ambulatory. Lymphangiomatosis is a rare developmental malformation of lymphoid tissue characterized by aberrant lymphatic proliferation in soft tissues, bones, or parenchymal organs with obliteration of draining lymphatics causing obstruction.* There are 4 major forms: lymphangioma simplex, lymphangioma circumscriptum, cavernous lymphangioma, and cystic hygroma. Most cases are present at birth or develop in the first 2 years of life. Cavernous lymphangioma presents as a large, diffuse, poorly defined, fluctuant soft tissue mass with cystic dilation of the lymphatics in the dermis, subcutaneous tissue, and intermuscular septa.* Skin changes such
C as hyperplasia, vesicle formation, and hyperpigmentation may also be seen. Histologically, cavernous lymphangioma is characterized by dilated lymph channels lined by a flattened layer of endothelial cells with separation of the dermal collagen bundles.* Imaging studies such as computed tomography* or MRI* may help define the extent of tissue involvement and in guiding treatment. Treatment options include surgical excision and imageguided sclerotherapy. Sclerotherapy can obliterate the cystic spaces within the malformation, thus abrogating the need for debulking surgery. Eugene H. Ng, MD, Vibhuti S. Shah, MD, MRCP, Derek C. Armstrong, MD, FRCPC, and Howard M. Clarke, MD, PhD, FRCSC, FAAP, FACS, NeonatalPerinatal Medicine, The Hospital for Sick Children, University of Toronto; Department of Newborn and Developmental Paediatrics, Sunnybrook and Women’s College Health Sciences Centre, University of Toronto; Division of Neuroradiology, Department of Radiology, The Hospital for Sick Children, University of Toronto; Division of Plastic Surgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada