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Hereditary late-onset lymphedema.

Two different types of hereditary late-onset lymphedema are presented. In one family the father and one son had recurrent streptococcal lymphangitis beginning in childhood. In the son there was lymphatic hypoplasia in both legs with the infection having only occurred in one. Prophylaxis with penicillin prevented the recurrent lymphangitis. Because of 30 years of untreated lymphangitis, the father has chronic severe lymphedema. The second type, lymphedema associated with extra eyelashes (distichiasis) and a wide spinal canal, occurred in a woman whose lymphedema began at age 12 but in whom the hereditary nature of the disorder was not recognized until she was 29. Both of these types of late-onset lymphedema, lymphedema with lymphangitis and lymphedema with distichiasis, are due to autosomal dominant genes. Both families would have benefited from early diagnosis of the cause of the lymphedema.

Adult

Stage IB carcinoma of the cervix, the Norwegian Radium Hospital, 1968-1970: results of treatment and major complications. I. Lymphedema.

Different methods of treatment have been debated over the years for Stage IB carcinoma of the cervix, especially in view of the results and complications. We have reviewed the experience of the Norwegian Radium Hospital for carcinoma of the cervix, Stage IB, for the years 1968, 1969, and 1970; most of the patients receive preoperative intrauterine and intravaginal radium, followed by radical hysterectomy and pelvic lymphadenectomy; if the nodes are involved, they also receive external pelvic irradiation. During this period, 437 patients had Stage IB; the 5 year survival was 80.2% (not corrected). We focused our attention on lymphedema following such a method of treatment; of 402 patients operated upon, 23.4% developed lymphedema ranging from mild to moderate to severe; 20 patients (5%) had severe lymphedema; factors involved are analyzed.

Adenocarcinoma

The Charles procedure for primary lymphedema. Long-term clinical results.

The best treatment for primary lymphedema has been a controversial point, because of the lack of clinical documentation of really long-term successful results after any therapy. We report the long-term results in 10 patients (12 extremities) after the Charles procedure. These patients were evaluated at an average period of 10 1/2 years following the surgery (4 of these extremities were examined 20 or more years following the surgery). All of them demonstrated excellent functional results; none demonstrated a recurrence of the lymphedema. We consider the Charles procedure to be the operation of choice in patients with primary lymphedema which is not responsive to conservative therapy.

Adolescent

Fabry's disease with familial lymphedema of the lower limbs. Case report and family study.

The case of a 49-year-old man with Fabry's disease (FD), confirmed by histopathological findings of kidney and skin biopsies and enzymatic studies, is reported. Clinical symptoms mainly consisted in severe neurological involvement, and in conspicuous lymphedema of the lower limbs. Two decreased brothers of the patient were also affected with symptons strongly suggesting FD, as well as the lymphedema of the lower limbs. On the basis of these data, the association of FD with familial lymphedema of the lower limbs is discussed: a lipid accumulation in the lymphatic as well as the blood vessel wall is proposed as a possible explanation; the hypothesis of an inborn error in the development of the lymphatic system, controlled by a gene closedly associated with the FD gene on the same chromosome can also be advanced.

Cerebroside-Sulfatase

Pleural effusion associated with primary lymphedema: a perspective on the yellow nail syndrome.

A 28-year-old woman with bilateral pleural effusions and generalized, primary lymphedema beginning with facial erysipelas at 6 years of age is presented. The pleural effusions were exudates with 250 cells per mm3, 92 per cent of which were lymphocytes. Lymphatic stasis was demonstrated by persistence of the blue dye in the dorsa of her feet 3 months after a lymphangiogram of both lower extremities, pelvis, and abdomen. Her nails were not remarkable. Our patient represents the twentieth recorded case of pleural effusion in association with primary lymphedema. Women have been afflicted more than twice as often as men, and the age of onset has varied from birth to the eighth decade. Yellow dystrophic nails may precede or follow lymphedema or the pleural effusion and have occurred in only 11 of the 20 patients.

Adult

Surgical management of congenital lymphedema in infants and children.

Of 67 children and infants with lymphedema, 28 had the congenital type. Congenital lymphedema appears during the first few weeks of life, frequently involves more than one extremity, and enlarges at a slower rate than general body growth. The swelling usually becomes less pronounced with age, and no specific therapy is required in two thirds of the patients. Seven of the 28 children had swelling of the upper extremities and a generalized lymphangiopathy syndrome. Subcutaneous lymphangiectomy was performed on ten of 28 patients who had moderate to severe swelling. Those with hand and arm involvement were particularly benefited; however, operations on the dorsum of the foot produced hypertrophic scars in one third of the cases. The operation is deferred until after age 2 years to permit optimal technical repair and to identify those patients whose conditions will improve spontaneously.

Adolescent

Nerve entrapments associated with postmastectomy lymphedema.

Ninety females underwent mastectomy for breast cancer and were thereafter investigated to determine whether nerve entrapments were responsible for some of the disabling symptoms in their arms. The majority of these patients suffered from fullness (edema), numbness, paraesthesia, weakness and pain of the arm on the mastecotmized side. Lymphedema of varying degrees found in 50% of these patients was associated with brachial plexus entrapment and carpal tunnel syndrome (CTS). 28% of the patients has CTS, and 28% suffered from brachial plexus entrapment of the arm on the mastecotmized side, as compared with 8% and 5%, respectively, on the nonoperated side. 12% of the patients suffered from both types of entrapment. Thus we consider that brachial plexus entrapment and carpal tunnel syndrome should be added to the list of complications following mastectomy, with lymphedema playing an active part in their development.

Adult

Noonan syndrome in an adult family presenting with chronic lymphedema.

A 27 year old man with multiple findings of the Noonan syndrome ("male Turner" phenotype) presented with chronic lymphedema which was also present in his mother. Noonan syndrome should be considered in the differential diagnosis of chronic or familial lymphedema.

Abnormalities, Multiple

Insights into KIF11 pathogenesis in microcephaly-lymphedema-chorioretinopathy syndrome from a lymphatic perspective.

Pathogenic variants in kinesin KIF11 underlie microcephaly-lymphedema-chorioretinopathy (MLC) syndrome. Although well known for regulating spindle dynamics ensuring successful cell division, the association of KIF11 (encoding EG5) with development of the lymphatic system and how KIF11 pathogenic variants lead to lymphatic dysfunction and lymphedema remain unknown. Using patient-derived lymphoblastoid cells, we demonstrated that patients with MLC carrying pathogenic stop-gain variants in KIF11 have reduced mRNA and protein levels. Lymphoscintigraphy showed reduced tracer absorption, and intestinal lymphangiectasia was detected in one patient, pointing to impairment of lymphatic function caused by KIF11 haploinsufficiency. We revealed that KIF11 is expressed in early human and mouse development with the lymphatic markers VEGFR3, podoplanin, and PROX1. In zebrafish, single-cell RNA-Seq identified kif11 specifically expressed in endothelial precursors. In human lymphatic endothelial cells, EG5 inhibition with ispinesib reduced VEGFC-driven AKT phosphorylation, migration, and spheroid sprouting. KIF11 knockdown reduced PROX1 and VEGFR3 expression, providing for the first time to our knowledge a link between KIF11 and drivers of lymphangiogenesis and lymphatic identity.

Humans

Surgical treatment of lymphedema--a reappraisal.

The clinical picture of lymphedema and its classification have been briefly reviewed with a discussion of surgical treatment both historical and current. It has been pointed out that the primary mode of treatment of congenital or secondary lymphedema of the extremities is intensive medical therapy and that surgical intervention is warranted in only rare and unusual circumstances. The gamut of surgical procedures attempted have been reviewed and it is this author's contention that the only procedure of limited, worthwhile value is a combination of the Kondoleon and the Thompson procedures. The Kondoleon phase of the procedure debulks the extremity in stages and removes the deep fascia. The Thompson form of the procedure advances a deepithelialized dermal flap into the deep lymphatical venous system. It has not been conclusively proven whether this dermal flap is of significant physiologic benefit, but I feel it is worth adding this to the Kondoleon procedure so that in time, we will have an opportunity to evaluate the value of this phase of the procedure.

Humans

[Significance of iontophoresis in dermatology. With special reference to the management of lymphedemas].

The development of modern medicine has very often pushed older methods of treatment to the background. It will be tried to represent the importance of iontophoresis as a special form of electrotherapy for dermatology according to studies of literature and own observations. Iontophoresis is for most of the 40 to 50 dermatologic indications named in literature without any importance for the present. This is also to be said for their diagnostic and experimental application. Because of the synergistic effect of the galvanic current and the medicament sluiced in, iontophoresis is said to be recommended following literature, for the treatment of the rest of therapeutically only with difficulty to be influenced idiopathic hyperhidrosis manuum et pedum, constitutional hypertrichosis, the lymphedema and also the induratio penis plastica and of thrombophlebitis. It will be reported about own experiences in the treatment of lymphedema.

Follow-Up Studies

[Physiopathology of lymphedema (author's transl)].

On the vast and still unclear problem of lymphedema, the author stresses the major role played by the permeability of the blood vessel wall to proteins, with accumulation of these in the interstitial space. The walls of capillaries are not semi-permeable. There is a passage of proteins even under normal conditions. Increased during inflammatory process, it determines the accumulation of protein molecules in the interstitial space. The increased passage of protein into the interstitial space during a temporary process is often the causative factor in lymphedema. All situations which interfere with the drainage of these substances by the lymphatic system maintains the edema and leads to a passage to chronicity.

Capillary Permeability

[Lymphedema after modified radical mastectomy as performed at the Bordet Institute. A report of 60 cases (author's transl)].

Three hundred and fifteen patients who underwent a radical mastectomy between 1969 and 1976 were studied. Nineteen percent developed lymphedema of the upper limb. Ten percent were resistant to all forms of therapy while the pathology gradually subsided in 9%. The causes of lymphedema as well as the preventive and curative methods were statistically studied and compared with those in the literature. Several preventive measures are proposed, in particular, the modified radical mastectomy.

Female

Lymphedema of the hand following a fracture of the distal radius.

A case of chronic lymphedema of the hand and forearm following a fracture of the distal radius is reported. None of the classic causes of secondary lymphedema appeared to be the cause. A strong psychogenic component appeared to be the underlying mechanism. This rare phenomenon was thought to be self-induced and not genetic in nature.

Adult

Surgical treatment of male genital lymphedema.

The etiologic classification and history of the surgical treatment of male genital lymphedema are presented. Two case reports and their results illustrate the surgical technique of skin flaps and split thickness skin grafting.

Adult

Safety and efficacy of Meridian sinew tuina (MST) for post-surgical upper limb lymphedema: a systematic review and meta-analysis.

BACKGROUND: Complex Decongestive Therapy (CDT) is the non-operative standard for breast cancer-related lymphedema (BCRL), but many patients experience persistent subcutaneous stiffness, pain, and restricted mobility. This study systematically reviews the safety and clinical efficacy of Meridian Sinew Tuina (MST) protocols for BCRL. METHODS: Global and regional databases (PubMed, Cochrane Library, Embase, Web of Science, CNKI, Wanfang, VIP) were searched from inception to January 15, 2026, with alerts monitored through April 30, 2026. Randomised controlled trials (RCTs) evaluating MST (deep tissue mobilisation along the six-hand meridian sinew [Jingjin] lines via plucking, kneading, and pressing) were included. Two reviewers independently extracted data, evaluated risk of bias using Cochrane RoB 2, and assessed evidence certainty via GRADE using a random-effects model. RESULTS: Fifteen RCTs were included. For the primary anthropometric outcome, MST significantly reduced upper limb circumference compared to controls (SMD = 1.59; 95% CI: 1.44 to 1.74; Z&#x2009;=&#x2009;20.81; p&#x2009;<&#x2009;0.0001; I2=0.0%; N&#x2009;=&#x2009;924; GRADE: Moderate certainty). The Clinical Response Efficacy Rate (&#x2265; 30% swelling reduction and symptom relief) favoured MST (RR = 1.69; 95% CI: 1.54 to 1.87; Z&#x2009;=&#x2009;10.62; p&#x2009;<&#x2009;0.0001; I2=0.0%; N&#x2009;=&#x2009;1,114; GRADE: Moderate certainty). Trial Sequential Analysis confirmed sample size sufficiency. For secondary outcomes (N&#x2009;=&#x2009;924; GRADE: Low to Very Low certainty due to performance bias and clinical heterogeneity), MST showed favourable 3-month improvements in DASH functional scores (SMD&#x2009;=&#x2009;-1.81; 95% CI: -2.11 to -1.51; I2=45.1%), pain intensity (SMD&#x2009;=&#x2009;-2.44; 95% CI: -2.93 to -1.95; I2=50.4%), and quality of life (SMD = 1.04; 95% CI: 0.79 to 1.29; I2=0.0%). No serious adverse events occurred. CONCLUSIONS: MST protocols are associated with favourable short- and mid-term reductions in upper limb swelling. However, confidence is tempered by unblinded performance bias and control group variations. MST cannot be unconditionally recommended for standalone implementation but represents a promising, optional supportive adjunctive intervention within oncological rehabilitation.

Humans

Lymphedema of the penis.

Lymphangiectomy with primary closure or skin grafting appears to be the treatment of choice for lymphedema of the penis. The method offers good cosmetic and functional results.

Adolescent

Lymphedema of the eyelids.

Lymphedema of the eyelids is a relatively rare condition whose main effect is to obstruct vision, and it is unsightly. We present 3 cases in which the edema was treated by excision and skin grafting of the eyelid, with satisfactory functional and cosmetic results. It is suggested that a split-skin graft be used for the upper eyelid and a full-thickenss graft for the lower eyelid. The lower eyelid skin can be "de-fatted" and used as a graft, but in the more severe cases we suggest a postauricular skin graft be used.

Adult