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Lymphedema of the lower extremity as a complication of local burns
Burns 24 (1998) 767±769
Lymphedema of the lower extremity as a complication of local burns Sanjeev Anand *, Hitesh Lal, B.K. Dhaon Department of Orthopaedics, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India Accepted 7 May 1998
Abstract Lymphedema of an extremity is a rare complication of local burns, due to intact deep lymphatics. Here we present a case of delayed lymphedema of the foot, developing due to deep scarring after local burns. # 1998 Elsevier Science Ltd for ISBI. All rights reserved.
1. Introduction Lymphedema of the lower extremities is usually due to the low output failure of the lymph circulatory system, that is, the overall lymphatic transport is reduced. This derangement may be primary (congenital lymphatic dysplasia) or secondary to local anatomical obstruction or functional de®ciency to the lymph ¯ow. High output failure of lymph circulation on the other hand occurs when transport capacity of intact lymphatics is overwhelmed by excessive capillary in®ltrate [1]. Although lymphedema has been reported after debridement and skin grafting for burns [2], it has not been reported to occur as a direct complication of local burns. Here, we are presenting a case of lymphedema of the foot developing after burns of the lower limb.
2. Case report A twenty ®ve year old patient suered from deep dermal burns of his left lower limb, extending below midthigh region. Besides initial acute management of the burns the patient did not receive any long term treatment. The burned area healed by scarring of skin * Corresponding author. 40/180, Chittaranjan Park, New Delhi, PIN 110019, India. Tel.: +91-11-6219652/6462155; Fax: +91-116219651.
and deeper tissues. Gradually he developed ¯exion contractures at the knee. One year after the initial insult, the patient noticed swelling of the aected foot which progressively worsened. This was associated with gradually worsening paraesthesia over the sole of the foot. When the patient was presented to us, his whole limb was scarred from mid-thigh to the lower leg. The foot was grossly swollen with non-pitting edema (Fig. 1). Distal pulses could not be felt due to the swelling, but capillary circulation was good. There was loss of sensation on the sole of the foot. The knee had a ¯exion contracture of thirty degrees, with further possible ¯exion till one hundred twenty degrees. We looked for other possible causes of the gigantism of the foot. On general examination, there were no stigmata of neuro®bromatosis. No enlarged group of lymph nodes or skin pigmentation spots were seen. There was no palpable mass in the abdomen. Routine blood investigations including eosinophil count was normal and the peripheral smear for micro®laria was negative. Indirect ¯uorescence and ELISA tests for antibodies to Micro®laria were negative. Ultrasonography of the abdomen and the pelvis showed a normal study. Arteriography of the limb showed normal ¯ow till trifurcation of the popliteal artery into anterior tibial, posterior tibial and peroneal arteries. Both posterior tibial and peroneal arteries were attenuated but could be traced till the foot. Anterior tibial artery was visualized only in its proximal part beyond which there was a sudden cut-o due to external compression (Fig. 2). Biopsy from the foot
0305-4179/98/$19.00 # 1998 Elsevier Science Ltd for ISBI. All rights reserved. PII: S 0 3 0 5 - 4 1 7 9 ( 9 8 ) 0 0 0 7 8 - 3
768
S. Anand et al. / Burns 24 (1998) 767±769
Fig. 1. Photograph showing scari®ed lower limb with lymphedema of the foot.
showed collagenization of the dermis with myxoid areas.^There was no increase in acid mucopolysaccharide content, the overall impression being of lymphedema. After considering all features, that is, the insensate foot with recalcitrant ®brosclerotic edema, the associated knee ¯exion deformity and the long term morbidity associated with the treatment of the lymphedema; the patient was given the surgical option of below knee amputation with prosthesis. At present, the patient is walking with a below knee prosthesis with no complaints. 3. Discussion Lymphedema is a condition characterised by swelling of one or more extremities caused by lymphatic insuciency. Clinical lymphedema may be idiopathic (primary lymphedema) or may be caused by acquired insuciency of the lymphatic pathways. Primary lymphedema is of three types, distinguished by the age of the onset, viz: Milroy's disease at birth or infancy, Lymphedema praecox at puberty, Lymphedema tarda
after thirty years. Secondary lymphedema is due to obstruction from various causes including infection, parasites (®lariasis being the commonest cause in the developing world), iatrogenic due to surgery or radiation and obstruction by neoplasm. Functional de®ciencies like lymphangiospasm, paralysis and valvular insuciency also lead to lymphedema. Other causes like cirrhosis, nephrotic syndrome, venous insuciency lead to high output failure of the lymphatic circulation [1]. Diagnosis of lymphedema is usually evident clinically. It usually begins at the ankle and progresses proximally. Progression is slow and passes through three grades of varying severity. Grade I lymphedema pits on pressure and is reduced on elevation. In grade II, edema is non-pitting, swelling becomes larger and harder and in late cases patient presents with skin changes. In grade III or elephantiasis, skin gets very thick with folds [3]. Unlike edema secondary to the venous disease, the lymphedematous extremity has no dark brawny edema or ulceration of the skin [2]. Diagnosis can be proven by lymphangiography or lymphoscintigraphy [1]. Other investigations like ultrasonography may reveal
S. Anand et al. / Burns 24 (1998) 767±769
Fig. 2. Arteriogram of the limb showing cut-o of anterior tibial artery (marked with arrow), with attenuated posterior tibial and peroneal arteries.
volumetric increase of both subcutaneous and subfascial compartments [4]. MRI may also help in dierentiating lymphedema from phleboedema or lipedema of the lower limb [5]. Therapy of lymphedema is divided into conservative and operative methods. Non-operative methods form the ®rst line of treatment. They include combination of physical therapy involving skin care, manual lymphedema treatment, exercises, elevation, compression applied with multilayered bandages and intermittent pneumatic compression. Drugs such as diuretics, benzopyrones, antimicrobials, intraarterial injections of lymphocytes, etcetera, have also been used. Operations like resection/debulking and microsurgical procedures are at best used as an adjunct to physical therapy or when it is unsuccessful [1]. Skin lymphatics are distributed in three layers: the super®cial valveless plexus in upper third of the dermis, intermediate valveless plexus in the middle third and the deep dermal valved plexus. Then there is a subcutaneous layer having valved collecting vessels and deep lymphatics running along blood vessels deep to deep fascia in the intermuscular plane [6]. Lymphedema is rarely seen as a complication of burns. This is because in burns involving skin and subcu-
769
taneous tissues, when scarring obliterates the super®cial lymphatics, new paths of lymphatic drainage are created. First, there is establishment of collaterals through the residual bridge of healthy skin. Second, the communicating channels through the deep fascia between super®cial and deep lymphatics open, rerouting the ¯ow of lymph through deep lymphatics [6]. This prevents any lymphedema from developing. While, in burns severe enough to damage the deep group of lymphatics (that is, fourth degree burns), the adjoining vessels and nerves are also liable to be damaged, thus rendering the limb nonviable. In this case, our patient suered from partial thickness deep dermal burns, which healed by extensive scarring. The circumstantial nature of the scarring prevented development of any super®cial collaterals, as no healthy bridge of skin remained. The progressive relentless scarring ultimately aected the deeper tissues as well, causing compression of the deeper lymphatics and vessels. The thin walled lymphatics may have been preferentially aected over larger arterial and venous channels of the limb, which were only attenuated. Thus, this out¯ow obstruction to the lymph would have resulted in the lymphedema of the foot. By strangulating deep nerves this deep scarring may also have caused delayed loss of sensations on the sole of the foot.
References Consensus document of the International Society of Lymphology Executive Committee. The diagnosis and treatment of peripheral lymphedema. Lymphology 1995, 28, 113. Balakrishnan C, Webber JD, Prasad JK. Lymphedema of lower extremities following debridement of extensive full skin thickness burns. Burns 1994;20:365. Casley-Smith JR, Casley-Smith JR. Modern treatment of lymphedema I. Complex physical therapy: The ®rst 200 Australian limbs. Australas. J. Dermatol. 1992;33:61. Doldi SB, Lattuada E, Zappa MA et al. Ultrasonography of extremity lymphedema. Lymphology 1992;25:129. Duewell S, Hagspiel KD, Zuber J et al. Swollen lower extremity: Role of MR imaging. Radiology 1992;184:227. Crockett DJ. Lymphatic anatomy and Lymphedema. Br. J. Plast. Surg. 1965;18:12.
Lymphedema of the lower extremity as a complication of local burns Sanjeev Anand *, Hitesh Lal, B.K. Dhaon Department of Orthopaedics, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India Accepted 7 May 1998
Abstract Lymphedema of an extremity is a rare complication of local burns, due to intact deep lymphatics. Here we present a case of delayed lymphedema of the foot, developing due to deep scarring after local burns. # 1998 Elsevier Science Ltd for ISBI. All rights reserved.
1. Introduction Lymphedema of the lower extremities is usually due to the low output failure of the lymph circulatory system, that is, the overall lymphatic transport is reduced. This derangement may be primary (congenital lymphatic dysplasia) or secondary to local anatomical obstruction or functional de®ciency to the lymph ¯ow. High output failure of lymph circulation on the other hand occurs when transport capacity of intact lymphatics is overwhelmed by excessive capillary in®ltrate [1]. Although lymphedema has been reported after debridement and skin grafting for burns [2], it has not been reported to occur as a direct complication of local burns. Here, we are presenting a case of lymphedema of the foot developing after burns of the lower limb.
2. Case report A twenty ®ve year old patient suered from deep dermal burns of his left lower limb, extending below midthigh region. Besides initial acute management of the burns the patient did not receive any long term treatment. The burned area healed by scarring of skin * Corresponding author. 40/180, Chittaranjan Park, New Delhi, PIN 110019, India. Tel.: +91-11-6219652/6462155; Fax: +91-116219651.
and deeper tissues. Gradually he developed ¯exion contractures at the knee. One year after the initial insult, the patient noticed swelling of the aected foot which progressively worsened. This was associated with gradually worsening paraesthesia over the sole of the foot. When the patient was presented to us, his whole limb was scarred from mid-thigh to the lower leg. The foot was grossly swollen with non-pitting edema (Fig. 1). Distal pulses could not be felt due to the swelling, but capillary circulation was good. There was loss of sensation on the sole of the foot. The knee had a ¯exion contracture of thirty degrees, with further possible ¯exion till one hundred twenty degrees. We looked for other possible causes of the gigantism of the foot. On general examination, there were no stigmata of neuro®bromatosis. No enlarged group of lymph nodes or skin pigmentation spots were seen. There was no palpable mass in the abdomen. Routine blood investigations including eosinophil count was normal and the peripheral smear for micro®laria was negative. Indirect ¯uorescence and ELISA tests for antibodies to Micro®laria were negative. Ultrasonography of the abdomen and the pelvis showed a normal study. Arteriography of the limb showed normal ¯ow till trifurcation of the popliteal artery into anterior tibial, posterior tibial and peroneal arteries. Both posterior tibial and peroneal arteries were attenuated but could be traced till the foot. Anterior tibial artery was visualized only in its proximal part beyond which there was a sudden cut-o due to external compression (Fig. 2). Biopsy from the foot
0305-4179/98/$19.00 # 1998 Elsevier Science Ltd for ISBI. All rights reserved. PII: S 0 3 0 5 - 4 1 7 9 ( 9 8 ) 0 0 0 7 8 - 3
768
S. Anand et al. / Burns 24 (1998) 767±769
Fig. 1. Photograph showing scari®ed lower limb with lymphedema of the foot.
showed collagenization of the dermis with myxoid areas.^There was no increase in acid mucopolysaccharide content, the overall impression being of lymphedema. After considering all features, that is, the insensate foot with recalcitrant ®brosclerotic edema, the associated knee ¯exion deformity and the long term morbidity associated with the treatment of the lymphedema; the patient was given the surgical option of below knee amputation with prosthesis. At present, the patient is walking with a below knee prosthesis with no complaints. 3. Discussion Lymphedema is a condition characterised by swelling of one or more extremities caused by lymphatic insuciency. Clinical lymphedema may be idiopathic (primary lymphedema) or may be caused by acquired insuciency of the lymphatic pathways. Primary lymphedema is of three types, distinguished by the age of the onset, viz: Milroy's disease at birth or infancy, Lymphedema praecox at puberty, Lymphedema tarda
after thirty years. Secondary lymphedema is due to obstruction from various causes including infection, parasites (®lariasis being the commonest cause in the developing world), iatrogenic due to surgery or radiation and obstruction by neoplasm. Functional de®ciencies like lymphangiospasm, paralysis and valvular insuciency also lead to lymphedema. Other causes like cirrhosis, nephrotic syndrome, venous insuciency lead to high output failure of the lymphatic circulation [1]. Diagnosis of lymphedema is usually evident clinically. It usually begins at the ankle and progresses proximally. Progression is slow and passes through three grades of varying severity. Grade I lymphedema pits on pressure and is reduced on elevation. In grade II, edema is non-pitting, swelling becomes larger and harder and in late cases patient presents with skin changes. In grade III or elephantiasis, skin gets very thick with folds [3]. Unlike edema secondary to the venous disease, the lymphedematous extremity has no dark brawny edema or ulceration of the skin [2]. Diagnosis can be proven by lymphangiography or lymphoscintigraphy [1]. Other investigations like ultrasonography may reveal
S. Anand et al. / Burns 24 (1998) 767±769
Fig. 2. Arteriogram of the limb showing cut-o of anterior tibial artery (marked with arrow), with attenuated posterior tibial and peroneal arteries.
volumetric increase of both subcutaneous and subfascial compartments [4]. MRI may also help in dierentiating lymphedema from phleboedema or lipedema of the lower limb [5]. Therapy of lymphedema is divided into conservative and operative methods. Non-operative methods form the ®rst line of treatment. They include combination of physical therapy involving skin care, manual lymphedema treatment, exercises, elevation, compression applied with multilayered bandages and intermittent pneumatic compression. Drugs such as diuretics, benzopyrones, antimicrobials, intraarterial injections of lymphocytes, etcetera, have also been used. Operations like resection/debulking and microsurgical procedures are at best used as an adjunct to physical therapy or when it is unsuccessful [1]. Skin lymphatics are distributed in three layers: the super®cial valveless plexus in upper third of the dermis, intermediate valveless plexus in the middle third and the deep dermal valved plexus. Then there is a subcutaneous layer having valved collecting vessels and deep lymphatics running along blood vessels deep to deep fascia in the intermuscular plane [6]. Lymphedema is rarely seen as a complication of burns. This is because in burns involving skin and subcu-
769
taneous tissues, when scarring obliterates the super®cial lymphatics, new paths of lymphatic drainage are created. First, there is establishment of collaterals through the residual bridge of healthy skin. Second, the communicating channels through the deep fascia between super®cial and deep lymphatics open, rerouting the ¯ow of lymph through deep lymphatics [6]. This prevents any lymphedema from developing. While, in burns severe enough to damage the deep group of lymphatics (that is, fourth degree burns), the adjoining vessels and nerves are also liable to be damaged, thus rendering the limb nonviable. In this case, our patient suered from partial thickness deep dermal burns, which healed by extensive scarring. The circumstantial nature of the scarring prevented development of any super®cial collaterals, as no healthy bridge of skin remained. The progressive relentless scarring ultimately aected the deeper tissues as well, causing compression of the deeper lymphatics and vessels. The thin walled lymphatics may have been preferentially aected over larger arterial and venous channels of the limb, which were only attenuated. Thus, this out¯ow obstruction to the lymph would have resulted in the lymphedema of the foot. By strangulating deep nerves this deep scarring may also have caused delayed loss of sensations on the sole of the foot.
References Consensus document of the International Society of Lymphology Executive Committee. The diagnosis and treatment of peripheral lymphedema. Lymphology 1995, 28, 113. Balakrishnan C, Webber JD, Prasad JK. Lymphedema of lower extremities following debridement of extensive full skin thickness burns. Burns 1994;20:365. Casley-Smith JR, Casley-Smith JR. Modern treatment of lymphedema I. Complex physical therapy: The ®rst 200 Australian limbs. Australas. J. Dermatol. 1992;33:61. Doldi SB, Lattuada E, Zappa MA et al. Ultrasonography of extremity lymphedema. Lymphology 1992;25:129. Duewell S, Hagspiel KD, Zuber J et al. Swollen lower extremity: Role of MR imaging. Radiology 1992;184:227. Crockett DJ. Lymphatic anatomy and Lymphedema. Br. J. Plast. Surg. 1965;18:12.