Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins)

Abstract

Primary lymphedema is a rare chronic pathology associated with constitutional abnormalities of the lymphatic system. The objective of this French National Diagnosis and Care Protocol (Protocole National de Diagnostic et de Soins; PNDS), based on a critical literature review and multidisciplinary expert consensus, is to provide health professionals with an explanation of the optimal management and care of patients with primary lymphedema. This PNDS, written by consultants at the French National Referral Center for Primary Lymphedema, was published in 2019 (https://has-sante.fr/upload/docs/application/pdf/2019-02/pnds_lymphoedeme_primaire_final_has.pdf). Primary lymphedema can be isolated or syndromic (whose manifestations are more complex with a group of symptoms) and mainly affects the lower limbs, or, much more rarely, upper limbs or external genitalia. Women are more frequently affected than men, preferentially young. The diagnosis is clinical, associating mild or non-pitting edema and skin thickening, as confirmed by the Stemmer’s sign (impossibility to pinch the skin on the dorsal side or the base of the second toe), which is pathognomonic of lymphedema. Limb lymphoscintigraphy is useful to confirm the diagnosis. Other causes of swelling or edema of the lower limbs must be ruled out, such as lipedema. The main acute lymphedema complication is cellulitis (erysipelas). Functional and psychological repercussions can be major, deteriorating the patient’s quality of life. Treatment aims to prevent those complications, reduce the volume with low-stretch bandages, then stabilize it over the long term by exercises and wearing a compression garment. Patient education (or parents of a child) is essential to improve observance.

PIEZO1‐gene gain‐of‐function mutations with lower limb lymphedema onset in an adult: Clinical, scintigraphic, and noncontrast magnetic resonance lymphography findings

“…Lymphedema treatment is symptomatic, based on complete decongestive therapy (low‐stretch bandages, manual lymph drainages, and skin care) and long‐term compression (Vignes et al, 2021). Although it is difficult to predict therapeutic efficacy in primary lymphedema patients, having visualized dermal backflow may help manage lymphedema‐volume reduction (Vignes et al, 2020).…”

“…Lymphedema treatment is symptomatic, based on complete decongestive therapy (low-stretch bandages, manual lymph drainages, and skin care) and long-term compression (Vignes et al, 2021).…”

Primary lymphedema of the left lower limb in a new-born boy: a case report

“…Active involvement of parents is most important in the care of children diagnosed with primary/congenital lymphedema. 5,9,11,13,14 The present case was clinically diagnosed as a case of primary congenital lymphedema of the left lower limb and was subjected to needle aspiration of lymphatic fluid and compressing dressing. Unfortunately, he died prior to completion of the therapy offered to him, and therefore the result of the same could not be evaluated.…”

“…There may be aplasia, hypoplasia, or dysplasia of the lymphatic channels/system, and results in the accumulation of the lymphatic fluid at the affected parts. [1][2][3][4][5] Secondary lymphedema is acquired in nature, and it is due to a consequence of destruction or obstruction of the lymphatic channels by other pathological conditions such as infection, trauma, post-operative, malignancy, or postradiation. Secondary lymphedema is more common than primary lymphedema.…”

“…2,4 Lymphoscintigraphy, Single-photon emission computed tomography (SPECT), computed tomography (CT), and Magnetic resonance imaging (MRI) lymphangiography are the radiological investigation offered for the diagnosis and confirmation of lymphedema. [5][6][7][8] The options for the management of lymphedema are compression therapy, physiotherapy, manual lymphatic drainage, and operative therapy. [9][10][11] Surgical CAse REport (SCARE) 2020 guidelines published by Agha et al are followed for reporting the present case.…”