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What is difference in management of primary lymphedema between adults and children, and how much?
Published in Byung-Boong Lee, Peter Gloviczki, Francine Blei, Jovan N. Markovic, Vascular Malformations, 2019
Cristobal M. Papendieck, Miguel A. Amore
Lymphedema in pediatrics deserves special consideration due to its etiopathogenesis, which is completely different from lymphedema in adults. In pediatrics, 80% of lymphedema cases are primary. Based on data from genetic studies, lymphedema is considered to be a part of 144 different syndromes—life-conditioning syndromes, or syndromes for the whole life. For this reason, only part of primary lymphedema is expressed in adults. Terminology used to diagnose this disorder as congenital lymphedema (early and late) is semantic and not etiopathogenesis based1 (Figures 63.1 through 63.3).
Conservative Treatment of Lymphedema
Published in Waldemar L. Olszewski, Lymph Stasis: Pathophysiology, Diagnosis and Treatment, 2019
Dysplasias of the lymphatics may or may not result in congenital lymphedema, depending on the relationship between lymphatic load and transport capacity. Before lymphedema praecox or tardum, respectively, arises, “lymphangiopathy with a sufficient lymph vascular system” prevails (see Figure 3). Concerning its functional and clinical consequences, this situation is identical with the latent phase of lymphedema.
Application of Microcirculation Research to Clinical Disease
Published in John H. Barker, Gary L. Anderson, Michael D. Menger, Clinically Applied Microcirculation Research, 2019
Bengt Fagrell, Alfred Bollinger
A distinction between primary and secondary lymphedema is not possible. However, the technique provides a means for classifying primary lymphedema into a form with normal superficial microlymphatics, aplasia and hyperplasia. Aplasia or hyperplasia are found in primary lymphedema manifesting at birth (Nonne-Milroy’s disease), normal microlymphatics in primary lymphedema with onset after puberty.3 Enlargement of lymphatic capillaries is detected in some patients with congenital lymphedema. These differences in microvascular morphology may have a prognostic and therapeutic impact in the future.
Novel variant of KIF11 associated with MCLMR syndrome
Published in Ophthalmic Genetics, 2023
Ghaida Alahmadi, Abdulaziz A. Alshamrani, Amani Albakri
Microcephaly in MLCRD syndrome ranges from mild to severe and is often associated with a variable degree of developmental delay and facial dysmorphic features. Chorioretinopathy is the most common ocular feature. Typically, the Congenital lymphedema is confined to the dorsal of the feet, and lymphoscintigraphy demonstrated the absence of radioactive isotope uptake from the web spaces between the toes (3). Jones et al. (3) reviewed in their Cohort study 37 individuals with a heterozygous mutation in KIF11 and found that the major clinical findings were microcephaly followed by ocular abnormality and lymphedema. Chorioretinopathy was the most common ocular abnormalities followed by hypermetropia and myopic astigmatism. In our patient, a few areas of lacunar chorioretinal atrophy with compound myopic astigmatism was observed. The congenital lymphedema associated with KIF11 pathogenetic variant is described as bilateral, and confined to the dorsal of the feet. In our patient, the lymphedema was subtle with the same characteristic findings of congenital lymphedema in KIF11 were observed. The dysmorphic facial features reported in the Jones et al. study (3) were upslanting palpebral fissures, broad nose with rounded tip, long philtrum with thin upper lip, and prominent, large ears, and in our patient prominent ears, up slanting palpebral fissures, a broad nose, a thin upper lip, and micro-retrognathia were observed.
Giant condylomata acuminata of Buschke–Lowenstein
Published in Journal of Obstetrics and Gynaecology, 2020
Ither Sandoval, Ronald Hernández, Esperanza Torres, Omar Yanque
A 23-year-old female was referred to our unit for a vulvar mass. The tumour had been growing in size for six months since the patient first noticed it. She had a history of congenital lymphedema of the right arm and leg of the body. Her menarche had started when she was at the age of 13 years old, she had initiated sexual intercourse at 19 years old and had been with two different partners, had no pregnancies, regular menses nor a history of allergies or surgeries.