Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 6  |  Issue : 2  |  Page : 132-134

A case report of lipedema with acute bilateral lower limb ischemia


1 Department of Critical Care, Columbia Asia Referral Hospital, Yeshwantpur, Bengaluru, Karnataka, India
2 Department of Vascular Surgery, Columbia Asia Referral Hospital, Yeshwantpur, Bengaluru, Karnataka, India

Date of Web Publication6-Jun-2019

Correspondence Address:
Dr. Pradeep Rangappa
Department of Critical Care, Columbia Asia Referral Hospital, Yeshwantpur, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_73_18

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  Abstract 


Lipedema is a poorly recognized disorder of subcutaneous adipose tissue that commonly presents as lower limb enlargement due to large deposits of subcutaneous fat and is seen mainly in women. It requires to be differentiated from lymphedema and venous insufficiency that may present similarly. Early recognition is important as the condition carries the risk of venous and arterial thrombosis and associated complications such as pain and ischemia.

Keywords: Arterial thrombosis, bilateral lower limb ischemia, lipedema


How to cite this article:
Mutnal S, Rangappa P, Jacob I, Chaitanya K, Rao K. A case report of lipedema with acute bilateral lower limb ischemia. Indian J Vasc Endovasc Surg 2019;6:132-4

How to cite this URL:
Mutnal S, Rangappa P, Jacob I, Chaitanya K, Rao K. A case report of lipedema with acute bilateral lower limb ischemia. Indian J Vasc Endovasc Surg [serial online] 2019 [cited 2019 Jun 25];6:132-4. Available from: http://www.indjvascsurg.org/text.asp?2019/6/2/132/259658




  Introduction Top


Lipedema is an uncommon disorder characterized by localized adiposity of the lower extremities, often occurring in females with a family history of this condition. It is described as “adiposis dolorosa” or painful fat and is related to the more extreme adipose tissue disorder, Dercum's disease. The underlying pathology involves a symmetrical, localized, subcutaneous deposition of fat which leads to a chronic progressive enlargement of the lower extremities.[1] The condition may be difficult to differentiate clinically from vascular diseases that affect the lower extremities such as lymphedema, deep vein thrombosis, and venous insufficiency. There should be a low threshold to investigate such patients with risk factors for intravascular thrombosis, as early diagnosis and management can prevent complications. We present here a case of lipedema with bilateral lower limb arterial thrombosis.


  Case Report Top


A 53-year-old female patient, diabetic and hypertensive, operated about 10 years earlier for left femur fracture presented with sudden onset of right lower limb weakness, tingling sensation, and pain since few hours. She had no previous history of pain or paresthesia or DVT. She was conscious, oriented, and hemodynamically stable with power 5/5 in the upper limbs, 1/5 on the right lower limb, and 4/5 on left lower limb. Examination showed a grossly abnormal deposition of fat from the ankle joint to the waist [Figure 1]. Both lower limbs were cold and femoral and popliteal and dorsalis pedis pulsations were absent bilaterally. Handheld Doppler showed absent signals in the right lower leg and low-velocity bi- to tri-phasic signals in the left leg. Routine blood investigations, echocardiogram, and magnetic resonance imaging spine were normal. Computed tomography angiography (CTA) was normal up to the thoracic aorta with extensive thrombosis below aortic bifurcation and complete nonopacification of bilateral popliteal, right anterior tibial, and left posterior tibial arteries [Figure 2]. The patient underwent emergency right transfemoral thrombectomy. A right groin incision was made, femoral arteries isolated, and transverse arteriotomy was done near the bifurcation. Embolectomy was done using 4-Fr and 5-Fr Fogarty catheters. CTA had shown a 1-cm stump of the patent right common iliac artery; hence, contralateral balloon occlusion was not considered in this case of unilateral common iliac thrombotic occlusion. The embolectomy specimen appeared as an organized thrombus/plaque and was sent for histopathology. Postoperatively, she was initially started on enoxaparin injection 1 mg/kg. Right leg fasciotomy was done in view of compartment syndrome. She had improved sensations in the right leg with improved limb power of 4/5. Dorsalis pedis artery pulse was palpable, as confirmed by improved handheld Doppler signals. Bedside duplex ultrasound showed the thrombus in the left lower limb remaining confined to popliteal artery. However, after 3 days, she developed pain and weakness in the left lower limb as well, with power dropping to 0/5 and Doppler showing poor signals. Enoxaparin was switched to a heparin infusion in view of the expected further surgery, and activated partial thromboplastin time was maintained at 2 times normal, up to 120 s. She underwent left-sided transfemoral thrombectomy. Postoperatively, pulsations were felt and power improved up to 4/5 in the left leg. Tests for thrombophilic disorders, including anticardiolipin, beta2 glycoprotein, MPL gene mutation and BCRABL gene, were negative. There was an improvement in symptoms and in bilateral triphasic Doppler signals. She was shifted to the wards, mobilized, and discharged after 5 days.
Figure 1: Presentation of lipedema with gross deformities on thighs

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Figure 2: Computed tomography angiogram showing extensive thrombosis below the aortic bifurcation; presence of at least 1-cm stump of the right common iliac artery and arrow pointing to left popliteal artery occlusion

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  Discussion Top


The term “lipedema” was first used in 1940 to describe a syndrome of “fat legs and orthostatic edema.”[2] Its incidence remains unknown because epidemiological studies have been inconclusive. Most case series show prevalence rate of 10%–23%.[3]

Evaluation of patients presenting with enlarged or swollen lower extremities starts by eliciting a complete history. It usually starts insidiously around puberty, although age of onset may vary greatly and progresses gradually.[4] The adiposity is typically unresponsive to weight loss. Its etiology is little understood, and few physicians recognize the various signs and symptoms for a correct diagnosis. In this patient, history showed that the lipedema had developed over the last 8–10 years, following the femur fracture surgery.

The diagnosis of lipedema is usually clinical; however, imaging studies may be required to distinguish lipedema from other causes of leg swelling such as lymphedema or venous insufficiency.[5] The Stemmer sign (the ability to pinch and lift a fold of skin at the base of the second toe) was elicited on both feet [Figure 1] and [Figure 3]. This was negative, thus excluding lymphedema.[6] Features of lymphedema on CT scan such as soft-tissue edema, skin thickening, and interstitial fibrosis were absent,[7] reaching a diagnosis of lipedema by exclusion. Lipedema may be distinguished from lifestyle induced fat by palpating for spheroids in the fat as well as pain or tenderness on palpation.[8]
Figure 3: Stemmer sign elicitation

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Lipedema has 3 stages: Stage 1 with normal skin surface and enlarged hypodermal fat, Stage 2 with skin indentations, and Stage 3, as in this patient, with large extrusions of subcutaneous fat causing gross deformities, especially on the thighs.[8] Lipedema fat is usually distributed from ankle up to the lower abdomen and on the upper arms, with the greater size of body being noted below the waist.

Although venous disease may accompany lipedema,[1] the instances of unprovoked acute bilateral lower limb arterial thrombosis are rare. The etiology of acute limb ischemia is broadly categorized as traumatic (10%) and nontraumatic (90%), with the latter occurring secondary to either embolism (30%) and thrombosis (60%).[9] In the present case, embolic and thrombotic causes were ruled out, suggesting lipedema with associated morbid obesity as a possible risk factor for arterial thrombosis. This association has been attributed to the role of adipokines, mainly leptin, in promoting platelet adhesion, activation, and aggregation, leading to both arterial and venous thrombosis.[10] There are no similar cases reported in literature, and further research may throw some light on this rare entity. Thus, early recognition of lipedema by its clinical manifestations is necessary to prevent complications such as ischemia.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Wold LE, Hines EA Jr., Allen EV. Lipedema of the legs; a syndrome characterized by fat legs and edema. Ann Intern Med 1951;34:1243-50.  Back to cited text no. 1
    
2.
Allen EU, Hines EA Jr. Lipedema of the legs. A syndrome characterized by fat legs and orthostatic edema. Proc Staff Meet Mayo Clin 1940;15:184-7.  Back to cited text no. 2
    
3.
Forner-Cordero I, Szolnoky G, Forner-Cordero A, Kemény L. Lipedema: An overview of its clinical manifestations, diagnosis and treatment of the disproportional fatty deposition syndrome – Systematic review. Clin Obes 2012;2:86-95.  Back to cited text no. 3
    
4.
Rudkin GH, Miller TA. Lipedema: A clinical entity distinct from lymphedema. Plast Reconstr Surg 1994;94:841-7.  Back to cited text no. 4
    
5.
Tiwari A, Cheng KS, Button M, Myint F, Hamilton G. Differential diagnosis, investigation, and current treatment of lower limb lymphedema. Arch Surg 2003;138:152-61.  Back to cited text no. 5
    
6.
Stemmer R. A clinical symptom for the early and differential diagnosis of lymphedema. Vasa 1976;5:261-2.  Back to cited text no. 6
    
7.
Monnin-Delhom ED, Gallix BP, Achard C, Bruel JM, Janbon C. High resolution unenhanced computed tomography in patients with swollen legs. Lymphology 2002;35:121-8.  Back to cited text no. 7
    
8.
Herbst KL, Mirkovskaya L, Bharhagava A, ChavaY, Te CH. Lipedema Fat and Signs and Symptoms of Illness, Increase with Advancing Stage. Available from: http://www.archivesofmedicine.com/medicine/lipedema-fat-and-signs-and-symptoms- of-illness-increase-with-advancing-stage.php?aid=6744.[Last accessed on 2018 Sep 17].  Back to cited text no. 8
    
9.
Gunawansa N, Atraumatic acute limb ischemia: Clinical presentation, classification, assessment and management – A review. Int J Vasc Surg Med 2017;3:46-52.  Back to cited text no. 9
    
10.
Schäfer K, Konstantinides S. Adipokines and thrombosis. Clin Exp Pharmacol Physiol 2011;38:864-71.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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