• Vol. 38 No. 7, 636–638
  • 15 July 2009

Primary Lymphoedema at an Unusual Location Triggered by Nephrotic Syndrome

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ABSTRACT

Introduction: Lymphoedema results from impaired lymphatic transport leading to the pathologic accumulation of protein-rich lymphatic fl uid in the interstitial space, most commonly in the extremities. Primary lymphoedema, a developmental abnormality of the lymphatic system, may become evident later in life when a triggering event exceeds the capacity of normal lymphatic fl ow. Clinical Picture: We present a 3-year-old nephrotic syndrome patient with an unusual localisation for primary lymphoedema. Treatment and Outcome: The patient was treated with conservative approach and she was cured. Conclusion: In this particular case, lymphoedema developed at an unusual localisation, which has not been recorded before.


Lymphatic system returns proteins, lipids and accompanying water from the interstitium to the venous circulation near the subclavian vein-internal jugular vein junction, bilaterally. Lymphoedema results from impaired lymphatic transport leading to the pathologic accumulation of protein-rich lymphatic fluid in the interstitium, most commonly in the extremities.1 Lymphoedema may be classified as primary or secondary, based on underlying aetiology. Primary lymphoedema, a developmental abnormality of the lymphatic system, not always clinically evident at birth, may become evident later in life when a triggering event or worsening of the condition causes the lymphatic transport capacity to exceed the volume of interstitial fluid formation, causing the patient to be unable to maintain normal lymphatic flow.2,3

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