Paediatric primary lymphoedema: a cohort of 155 children and newborns

Vidal, Florence; Arrault, M.; Vignes, S.

doi:10.1111/bjd.14556

Cited by 14 publications

(19 citation statements)

References 11 publications

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“…Primary lymphedemas develop more frequently in young females, sometimes with onset in children or newborns. 1 They can be isolated, with congenital and familial (Milroy disease), or syndromic forms accompanied by other clinical malformations associated with genetic mutations or chromosomal abnormalities. 2,3 Secondary lymphedemas are the most frequent in industrialized countries, mostly occurring after cancer treatment, including lymph-node excision, sometimes associated with radiotherapy (breast cancer for the upper extremity, pelvic cancer or melanoma for the lower limb), while filariasis is the first cause worldwide.…”

Section: Introductionmentioning

confidence: 99%

Specialized consultations in a hospital-based referral center for patients suspected of having limb lymphedema: Impact on diagnosis

Vignes¹,

Vidal²,

Arrault³

2017

Vasc Med

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Lymphedema, a chronic debilitating disease, is not always easily diagnosed. A total of 254 new patients ((217 women, 37 men), median (Q1-Q3) age 61 (46-72) years) were referred for suspected limb lymphedema to an exclusively lymphedema-dedicated department for a first consultation (January - March 2015) were included; among 118 with upper limb involvement, 100 (84.7%) were diagnosed with post-breast cancer therapy and four with primary lymphedemas; among 136 with lower limb involvement, 31 (22.8%) were diagnosed with primary lymphedemas and 35 (25.7%) with post-cancer lymphedemas. The main alternative diagnoses were: 32 (45.7%) lipedemas/lipo-lymphedemas and 21 (30%) chronic venous insufficiencies. Age at symptom onset, body mass index, referral origins and first-symptom-to-specialized-consultation intervals differed between primary, post-cancer lymphedema and alternative diagnosis patients. Among the entire cohort, 57 (22.4%) had cellulitis. For all 135 (53.1%) upper or lower limb post-cancer lymphedemas and the 119 (46.9%) others, the median (Q1-Q3) first-symptom-to-specialized-consultation intervals were 1.4 (0.7-3.8) and 4 (1.1-18.8) years, respectively ( p < 0.0001). Specialized consultations confirmed primary and post-cancer lymphedema diagnoses and identified others, especially for patients with suspected lower limb lymphedema.

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Section: Introductionmentioning

confidence: 99%

Specialized consultations in a hospital-based referral center for patients suspected of having limb lymphedema: Impact on diagnosis

Vignes¹,

Vidal²,

Arrault³

2017

Vasc Med

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“…In our patient group, we found no patients with Milroy disease, although that is the most common gene defect ( FLT4 ) known in primary lymphedema at this time [ 24 ]. Perhaps this is because Milroy disease only affects the lower limbs and often has a mild course.…”

Section: Discussionmentioning

confidence: 99%

Shaving Technique and Compression Therapy for Elephantiasis Nostras Verrucosa (Lymphostatic Verrucosis) of Forefeet and Toes in End-Stage Primary Lymphedema: A 5 Year Follow-Up Study in 28 Patients and a Review of the Literature

Damstra

Dickinson-Blok

Voesten

2020

JCM

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Background. Longstanding lymphedema can lead, especially when there is recurrence of erysipelas, to irreversible elephantiasis nostras verrucosa (ENV). This predisposes to new episodes of erysipelas, leading to further damage of the lymphatics and deterioration of the lymphedema as a whole. We report the results of 28 patients with primary lymphedema and surgical removal ENV of the forefoot and toes treated between 2006 and 2014. Method: Retrospective descriptive 5 year follow-up study of 28 patients with various diagnosis of primary lymphedema. Wound healing time, number of erysipelas, body mass index (BMI), recurrence of EVN and types of compression were documented during follow-up. Results: After preoperative multidisciplinary work up, operation of the toes with shaving and excision was performed within a conservative treatment program. During the follow up, the number of erysipelas attacks decreased dramatically (mean 17.6 vs. 0.6). Before treatment, no toecaps were used; and in follow up, it was a part of treatment. Recurrence of ENV was not observed. Compared to the literature with often BMI > 35, the mean BMI in our group was 30.0 (overweight). In 12 patients, we concurrently performed circumferential suction-assisted lipectomy for end-stage lymphedema of the leg. Conclusion: Although lymphedema patients are treated with garments during the maintenance phase, compression of the toes is often too challenging. Surgical removal of the verrucosis of toes is an effective therapeutic modality as part of an integrated lymphedema treatment program to restore the shape of the toes and enable the wearing of toecaps. This technique can also be effective for ENV of origins other than primary lymphedema. Although ENV is a generally accepted term, it can have undesirable connotations. We suggest using a more inclusive name such as lymphostatic verrucosis, because long-lasting lymphatic impairment is involved in all ENV and the term verrucosis is above discussion.

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“…Dear Editor , Primary childhood lymphoedema is a rare disorder, attributed to constitutional lymphatic system abnormality(ies), with familial, syndromic and nonsyndromic forms, associated or not with known gene mutation(s) . Lymphoedema treatment for adults is based on complete decongestive physiotherapy, comprising an initial intensive phase to reduce volume, which includes low‐stretch bandaging, manual lymph drainage, exercises and skin care, followed by a maintenance phase, essentially based on wearing an elastic garment .…”

Section: Main Characteristics Of 48 Children With Lower Limb Primary mentioning

confidence: 99%

“…Recommendations addressing upper limb lymphoedema after breast cancer, exclusively for adult women, were extrapolated to primary lymphoedema. For children, conservative nonsurgical management requiring parents' commitment is not consensual and differs according to the team; some teams have developed new surgical techniques . Low‐stretch bandages are the cornerstone of therapy to reduce lymphoedema volume in adults but no specific data on children are available.…”

Section: Main Characteristics Of 48 Children With Lower Limb Primary mentioning

confidence: 99%

Low-stretch bandages to treat primary lower limb lymphoedema: a cohort of 48 children

Benoughidane¹,

Simon²,

Fourgeaud³

et al. 2018

Br J Dermatol

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Paediatric primary lymphoedema: a cohort of 155 children and newborns

Cited by 14 publications

References 11 publications

Specialized consultations in a hospital-based referral center for patients suspected of having limb lymphedema: Impact on diagnosis

Specialized consultations in a hospital-based referral center for patients suspected of having limb lymphedema: Impact on diagnosis

Shaving Technique and Compression Therapy for Elephantiasis Nostras Verrucosa (Lymphostatic Verrucosis) of Forefeet and Toes in End-Stage Primary Lymphedema: A 5 Year Follow-Up Study in 28 Patients and a Review of the Literature

Low-stretch bandages to treat primary lower limb lymphoedema: a cohort of 48 children

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