Painful fat syndrome in a male patient q

Transcription

1 The British Association of Plastic Surgeons (2004) 57, CASE REPORT Painful fat syndrome in a male patient q Shyi-Gen Chen a, *, Shih-Dar Hsu b, Tim-Mo Chen a, Hsian-Jenn Wang a a Division of Plastic and Reconstructive Surgery, Department of Surgery, Tri-Service General Hospital, National Defense Medical Center, No. 325, Section 2, Cheng-Gung Road, Taipei 100, Taiwan, ROC b Department of Surgery, Armed Forces Keelung Hospital, Keelung, Taiwan, ROC Received 2 June 2003; accepted 17 December 2003 KEYWORDS Painful fat syndrome; Lipoedema; Lymphoedema; Phleboedema; Tumescent liposuction Summary Painful fat syndrome or lipoedema is a distinct clinical condition, characterised by bilateral and symmetrical enlargement of the upper and lower leg with tenderness, but excluding the feet. Lipoedema occurs almost exclusively in females, and no male patient has been reported in the literature published in English. We report on an extremely rare presentation of lipoedema in a male patient. A thorough study based on the case history, physical manifestations, and magnetic resonance imaging (MRI) provided valuable clues for a differential diagnosis. Conservative treatment using weight reduction, compression-stocking application, and diuretic therapy was not effective. Tumescent liposuction with postoperative pressure garments provided a satisfactory treatment. Q 2004 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved. Swelling of the leg sometimes poses problems for diagnosis and management. Painful fat syndrome or lipoedema is a distinct clinical condition, characterised by bilateral and symmetrical enlargement of the upper and lower leg, with tenderness caused by the abnormal deposition of subcutaneous fat. 1 The feet are usually free of swelling. The aetiology and pathogenesis of lipoedema are still unknown. It occurs almost exclusively in females, and no male patient has been reported in the literature published in English. For the majority of patients, diagnosis is based on case history and typical clinical presentation. Magnetic resonance imaging q This paper was presented at the 59th General Scientific Meeting of the Taiwan Surgical Association, Taipei, Taiwan, March 25 26, *Corresponding author. Tel.: þ ; fax: þ address: shyigen@ms26.hinet.net (MRI) offers strong qualitative and quantitative parameters for differential diagnosis of lipoedema, phleboedema and lymphoedema. 2 Treatment is often difficult, and conservative treatment using weight reduction, compression-stocking application, and/or diuretic therapy is of little value. 1, 3 Dermolipectomies are not advisable because of the large scars which result, and the risk of damaging the lymphatic system. 4 We report on an extremely rare presentation of lipoedema in a male patient and describe successful treatment using tumescent liposuction. Case report A 20-year-old male presented with painful symmetrical enlargement of both legs that began above S /$ - see front matter Q 2004 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved. doi: /j.bjps

2 Painful fat syndrome in a male patient 283

3 284 S.-G. Chen et al. the ankle and extended proximally to the upper thighs. The symptoms had developed insidiously since puberty. Physicians had told the patient that his condition was lymphoedema and he had tried various diuretic therapy regimens without improvement. Treatment with a compression stocking was of little help in reducing the swelling in the leg, which always returned after the stocking was removed. The distressing symptoms of symmetrical swelling and aching in the legs did not affect the patient s feet. The discomfort was aggravated by standing for prolonged periods of time and by exercise. Because of the persistent and progressive symptoms, he was admitted for further investigation. The patient s medical and family history were unremarkable. Physical examination revealed a young male, 167 cm tall, weighing 65 kg with a body mass index (BMI) of 23.3 (normal range 20 25). His general condition was good; his lungs, his heart and his chest and abdomen were normal. Although a neurological examination was negative, the patient complained of a dull painful sensation and tenderness in both lower limbs. These were swollen symmetrically, although his feet were not affected (Fig. 1(A)). The skin was soft and the subcutaneous tissue contained increased amounts of soft fatty tissue. There was no trace of the dermal thickening or induration seen so commonly in patients with lymphoedema. There was no pitting oedema, and Stemmer s sign in which the skin at the base of the second toe cannot be pinched up was negative. His routine laboratory blood values for haematology, biochemistry and clotting studies were normal. A chest radiograph revealed mild scoliosis of the thoracic spine, with convexity to the right, and a hip radiograph showed deformity of the left femoral head, with dysplasia and steep acetabulum, also known as developing dysplasia. Sonography of the abdomen and hepatitis markers were negative. A series of diagnostic studies was performed for various tumour (AFP, CEA, CA-125, PSA, CA-153), autoimmune (ANA, ANCA, HLA-B27, Anti-dsDNA, CRP, C3,C4, RF), and HIV markers; for immunoglobulins (IgG, IgA, IgM, IgD, IdE, cryoglobulin), together with thyroid function tests; all proved negative. No significant abnormalities were demonstrated from three-phase bone, liver, and spleen computer tomography (CT) scans, or a brain CT Figure 2 MRI study. (A) A coronal proton-density image (TR/TE: 3500/20) of the thighs through the femurs shows generalised homogeneous thickening of the subcutaneous fat. (B) The high signal intensity of this fatty tissue was suppressed using a subtraction technique, excluding the likelihood of lymphoedema. scan. MRI of the lower extremities revealed generalised and homogenous thickening of subcutaneous fat without evidence of soft tissue mass (Fig. 2(A) and (B)). A muscle biopsy was performed, which only showed lymphocyte infiltration. Figure 1 (A) Preoperative photography illustrated bilateral and symmetrical swelling of the lower legs without the feet being affected. (B) Lateral view. (C) Postoperative appearance showed a significant improvement both cosmetically and symptomatically. (D) Lateral view.

4 Painful fat syndrome in a male patient 285 Figure 3 Histology of the aspirate showed normal, mature, but oedematous fatty cells (200, Haematoxylin and eosin staining). Lymphoscintigraphy showed his lymphatic function to be normal. Based on his case history, typical clinical presentation and MRI appearance, lipoedema was diagnosed. Conservative treatment using a weight reduction program, compression pressure garments, and diuretics was not effective. Tumescent liposuction was carried out on each thigh and calf area using as the tumescent agent 30 cc of 1% lidocaine and 1 ampoule of epinephrine added to 1 L Ringer s lactate. No skin or soft tissue was excised. In total, 1500 and 1600 cc of fat were aspirated from the right and left lower limb, respectively. Histology of the aspirate showed mature oedematous fatty cells (Fig. 3). Postoperatively, his left calf developed a seroma, which was treated successfully using repeated aspiration. Significant improvement was achieved both cosmetically and symptomatically after this procedure. The patient was happy with the result and was discharged uneventfully. Postoperative photographs were obtained 6 months after the operation (Fig. 1(B)). The respective preoperative vs postoperative leg circumferences were 57 cm vs 48 cm for both thighs, 39 cm vs 34 cm for the right calf and 41 cm vs 35 cm for the left calf. The patient continues to demonstrate good leg contour after three-and-a-half-years follow-up. Discussion Leg swelling is a common clinical problem, and the differentiation and treatment is still a challenge for physicians. Systemic causes of leg oedema include idiopathic cyclic oedema, heart failure, cirrhosis, renal insufficiency, and other hypoproteinaemic states, 5 which had not occurred in this patient. Causes of localised swelling of the lower extremity, such as infiltrating lipomas, lymphangiomas, haemangiomas, plexiform neurofibromas, and reflex sympathetic dystrophy, are easily excluded on the basis of their morphology and clinical criteria. 2 The most important differential diagnosis in this case lay in the distinction between lipoedema, lymphoedema and phleboedema. Painful fat syndrome or lipoedema was first described by Allen and Hines in 1940, 6 who described it as lipodystrophy with subcutaneous fat deposition in the buttocks and lower extremities, and fluid accumulation in the legs (orthostatic oedema). In 1949, Wold et al., 7 after analysis of 119 cases, defined the diagnostic criteria for lipoedema: (1) almost exclusive occurrence in women; (2) bilateral and symmetrical manifestation with minimal involvement of the feet; (3) minimal pitting oedema; (4) pain, tenderness and easy bruising; and (5) persistent enlargement after elevation of the extremities or weight loss. A review of the later literature 1 3,8 reveals that lipoedema typically involves bilateral and symmetrical fat deposition of the legs in women; it is painful to pressure, nonpitting, not discoloured or inflamed, and does not affect the foot. The affected tissue in the lower extremities is described as being soft and pliable, unlike that seen in the latter stages of lymphoedema. The precise mechanisms responsible for lipoedema have not been fully established. Stallworth 3 found that 12 out of 14 patients had normal lymphangiograms and no patients had clinical lymphoedema or venostasis. Harwood et al. 8 performed photoplethysmography and quantitative lymphoscintigraphy to investigate the possible role of venous and lymphatic function in the pathogenesis of lipoedema. They concluded that lipoedema is a distinct entity rather than a direct consequence of any primary venous or lymphatic insufficiency. Diagnosis must, therefore, be based on the history and clinical manifestation. Lymphoscintigraphy may have some benefits for the differential diagnosis of lymphoedema, but it is not conclusive in diagnosing lipoedema. MRI plays an important role in the diagnosis of lipoedema, which shows homogeneously enlarged subcutaneous layers, with no increase in signal intensity in T2- weighted images or after contrast administration. 2 The patient in this study showed normal lymphoscintigraphy, and the typical MRI images confirmed the diagnosis of lipoedema. Total body fat content is approximately 15 percent in young adults according to Katch s statement. 9 This can be influenced by individual differences, gender, ethnic and genetic background, and increases with age. 10

5 286 S.-G. Chen et al. Males typically develop adipose deposition in the abdomen and upper body, the android adipose distribution. 11 This young patient s weight (65 Kg) and BMI (23.3) were within ideal categories according to the WHO definition. 12 Lymphoedema is the abnormal accumulation of protein-rich interstitial fluid within the skin and subcutaneous tissue caused by lymphatic dysfunction, 13 and may manifest in either a primary or secondary form. Primary lymphoedema represents a developmental abnormality of the lymphatic system. Secondary lymphoedema commonly results from damage or removal of regional lymph nodes through surgery, radiation, infection, tumour invasion or compression and is usually unilateral. The swelling is characteristically hard, and does not yield to impression. The foot and toes are affected when the Stemmer s sign is positive. The overlying skin thickens and displays the typical orange colour of congested dermal lymphatics. Cracks and furrows often develop and accommodate debris and bacteria, leading to lymphorrhoea; the leakage of lymph onto the surface of the skin. In the majority of patients, a diagnosis can be made on the basis of history and physical examination. A typical honeycomb pattern above the fascia between muscle and subcutis was observed in MRI study. 2 Phleboedema occurs as a consequence of chronic venous incompetence, swelling of the lower leg, varicosities, and indurations. It can be diagnosed radiologically using phlebography and MRI, which typically show areas with increased amounts of intramuscular fluid and subcutaneous fat. 2 Treatment of lipoedema is difficult, since conservative treatment using a weight reduction program, compression pressure garments and diuretics are not effective. 1,3 Dermolipectomies are not advisable because of the resulting large scars and the risk of damaging the lymphatic system. 4 Postoperative swelling and exacerbation of burning sensations in the heel have also been reported. 1 Suction-assisted lipectomy offers a successful alternative. 1 Liposuction was developed for body contouring, 14 and has been modified and extended to lymphedema. 15 The tumescent liposuction technique is a modification that has been shown to reduce dramatically bleeding, improve postoperative comfort, and accelerate recovery time. 16 Although there was a minor complication involving seroma in this patient, it was solved by repeated aspiration, and the patient eventually recovered. This case demonstrates an unusual lipoedema in a male patient. A thorough history, physical examination, and MRI study provided valuable clues in making the differential diagnosis. Tumescent liposuction with postoperative pressure garments provided a satisfactory treatment. Acknowledgements We thank Chun-Jung Juan, MD, Instructor in the Department of Radiology, Tri-Service General Hospital, for reading the MRI images. References 1. Rudkin GH, Miller TA. Lipedema: a clinical entity distinct from lymphedema. Plas Reconstr Surg 1994;94: Duewell S, Hagspiel KD, Zuber J, et al. Swollen lower extremity: role of MR imaging. Radiology 1992;184: Stallworth JM, Hennigar GR, Jonsson HT, Rodriguez O. The chronically swollen painful extremity a detailed study for possible etiological factors. JAMA 1974;228: Ketterings C. Lipodystrophy and its treatment. Ann Plast Surg 1988;21: Young JR. The swollen leg. Am Fam Physician 1977;15: Allen EV, Hines Jr EV. Lipedema of the legs: a syndrome characterized by fat legs and orthostatic edema. Proc Staff Meet Mayo Clin 1940;15: Wold LE, Hines EA, Allen EV. Lipedema of the legs. Ann Intern Med 1949;34: Harwood CA, Bull RH, Evans J, et al. Lymphatic and venous function in lipoedema. Br J Dermat 1996;134: Katch F, McCadle WD. Nutrition, weight control, and exercise, 2nd ed. Philadelphia: Lea and Febiger; Chumlea WC, Roche AF, Siervogel RM, et al. Adipocytes and adiposity in adults. Am J Clin Nutr 1981;34: Markman B. Anatomy and physiology of adipose tissue. Clin Plast Surg 1989;16: World Health Organization, The Asia-Pacific perspective: redefining obesity and its treatment. Geneva: WHO; Mortimer PS. The pathophysiology of lymphedema. Cancer 1998;83(Suppl. 12): Illouz YG. Body contouring by lipolysis: a 5-year experience with over 3000 cases. Plast Reconstr Surg 1983;72: O Brien BM, Khzanchi RK, Kumar PAV, et al. Liposuction in the treatment of lymphoedema; a preliminary report. Br J Plast Surg 1989;42: Klein JA. Tumescent liposuction and improved postoperative care using tumescent liposuction garments. Dermat Clin 1995;13: