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FEBRUARY 2008 LYMPHEDEMA ABSTRASTS                 
Arch Gynecol Obstet. 2008 Feb 28 [Epub ahead of print]
Recurrence of secondary angiosarcoma in a patient with post-radiated breast for breast cancer.Kunkel T, Mylonas I, Mayr D, Friese K, Sommer HL.
First Department of Obstetrics and Gynecology, Ludwig–Maximilians-University Munich, Maistrasse 11, 80337, Munich, Germany.

INTRODUCTION: Angiosarcoma of the breast is a rare finding. Two different subtypes of angiosarcomas have been described: (a) the Stewart-Treves syndrome and (b) the cutaneous post-radiation angiosarcoma. We report a case where both types of angiosarcoma occurred. CASE REPORT: At first, an angiosarcoma affecting parenchyma of the breast was observed after radiotherapy following breast conserving therapy and a history of lymphoedema of the radiated area. Additionally, a subsequent local recurrence of the angiosarcoma of the skin after mastectomy and complete resection of the primary angiosarcoma was diagnosed. DISCUSSION: This case is distinguished by a short latency period after primary therapy (less than 4 years) and a rapid recurrence after complete resection (14 weeks). Patients should be pointed to this possible complication of radiotherapy and transferred to seek medical advice immediately in case of skin lesion in the irradiated area: even many years after radiotherapy. Additionally, every oncologist should be aware of this rare complication as quick diagnosis and prompt surgical treatment is indispensable due to the aggressive entity of angiosarcoma.

PMID: 18305948 [PubMed - as supplied by publisher]
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Breast Cancer Res Treat. 2008 Feb 24 [Epub ahead of print]
Risk factors for arm lymphedema following breast cancer diagnosis in Black women and White women.Meeske KA, Sullivan-Halley J, Smith AW, McTiernan A, Baumgartner KB, Harlan LC, Bernstein L.
Department of Preventive Medicine, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA.

Purpose Lymphedema of the arm is a potential complication of breast cancer therapy. This study examines pre-disposing factors that may operate in conjunction with treatment-related factors in the development of arm lymphedema in a large cohort of White and Black breast cancer survivors. Methods 494 women (271 White and 223 Black) with in situ to Stage III-A primary breast cancer completed a baseline interview within 18 months of diagnosis. Information on lymphedema was collected during a follow-up interview, conducted on average 50 months after diagnosis. Self-reported data were used to classify women with or without lymphedema. Multivariable logistic regression models were developed to identify risk factors for arm lymphedema. Results Arm lymphedema was associated with younger age at diagnosis (odds ratio, OR per year of age = 0.96; 95% confidence interval, CI = 0.93-0.99), positive history of hypertension (OR = 2.31; 95% CI = 1.38-3.88), obesity (OR for body mass index, BMI>/=30 = 2.48; 95% CI = 1.05-5.84) and having had surgery where 10 or more lymph nodes were excised (OR = 2.16; 95% CI = 1.12-4.17). While Black women had higher prevalence of arm lymphedema than White women (28% vs. 21%), race was not associated with lymphedema risk in models adjusted for multiple factors (adjusted OR = 1.01; 95% CI = 0.63-1.63). Conclusion Risk of arm lymphedema did not differ significantly for Black and White women. Risk factors identified in this study offer opportunities for interventions (weight loss, control of blood pressure, use of sentinel node biopsy where possible) for reducing incidence of lymphedema or controlling the symptoms associated with this condition.

PMID: 18297429 [PubMed - as supplied by publisher]
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Orphanet J Rare Dis. 2008 Feb 22;3(1):5 [Epub ahead of print]
Primary intestinal lymphangiectasia (Waldmann's disease).Vignes S, Bellanger J.
ABSTRACT: Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool alpha-1 antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.

PMID: 18294365 [PubMed - as supplied by publisher]

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J Clin Oncol. 2008 Feb 20;26(6):884-9.
Comment in:
J Clin Oncol. 2008 Feb 20;26(6):828-9.
Sentinel node dissection is safe in the treatment of early-stage vulvar cancer.Van der Zee AG, Oonk MH, De Hullu JA, Ansink AC, Vergote I, Verheijen RH, Maggioni A, Gaarenstroom KN, Baldwin PJ, Van Dorst EB, Van der Velden J, Hermans RH, van der Putten H, Drouin P, Schneider A, Sluiter WJ.
Department of Obstetrics and Gynecology, University Medical Center Groningen, PO Box 30.001, 9700 RB Groningen, the Netherlands. a.g.j.van.der.zee@og.umcg.nl

PURPOSE: To investigate the safety and clinical utility of the sentinel node procedure in early-stage vulvar cancer patients. PATIENTS AND METHODS: A multicenter observational study on sentinel node detection using radioactive tracer and blue dye was performed in patients with T1/2 (< 4 cm) squamous cell cancer of the vulva. When the sentinel node was found to be negative at pathologic ultrastaging, inguinofemoral lymphadenectomy was omitted, and the patient was observed with follow-up for 2 years at intervals of every 2 months. Stopping rules were defined for the occurrence of groin recurrences. RESULTS: From March 2000 until June 2006, a sentinel node procedure was performed in 623 groins of 403 assessable patients. In 259 patients with unifocal vulvar disease and a negative sentinel node (median follow-up time, 35 months), six groin recurrences were diagnosed (2.3%; 95% CI, 0.6% to 5%), and 3-year survival rate was 97% (95% CI, 91% to 99%). Short-term morbidity was decreased in patients after sentinel node dissection only when compared with patients with a positive sentinel node who underwent inguinofemoral lymphadenectomy (wound breakdown in groin: 11.7% v 34.0%, respectively; P < .0001; and cellulitis: 4.5% v 21.3%, respectively; P < .0001). Long-term morbidity also was less frequently observed after removal of only the sentinel node compared with sentinel node removal and inguinofemoral lymphadenectomy (recurrent erysipelas: 0.4% v 16.2%, respectively; P < .0001; and lymphedema of the legs: 1.9% v 25.2%, respectively; P < .0001). CONCLUSION: In early-stage vulvar cancer patients with a negative sentinel node, the groin recurrence rate is low, survival is excellent, and treatment-related morbidity is minimal. We suggest that sentinel node dissection, performed by a quality-controlled multidisciplinary team, should be part of the standard treatment in selected patients with early-stage vulvar cancer.

PMID: 18281661 [PubMed - indexed for MEDLINE]
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Cancer. 2008 Feb 15;112(4):950-4.
Manipulative therapy of secondary lymphedema in the presence of locoregional tumors.Pinell XA, Kirkpatrick SH, Hawkins K, Mondry TE, Johnstone PA.
Radiation Oncology Department, Emory University School of Medicine, Atlanta, Georgia, USA.

BACKGROUND: Complete decongestive therapy (CDT), including manual lymphatic drainage (MLD) is a manipulative intervention of documented benefit to patients with lymphedema (LE). Although the role of CDT for LE is well described, to the authors' knowledge there are no data regarding its efficacy for patients with LE due to tumor masses in the draining anatomic bed. Traditionally, LE therapists are wary of providing therapy to such patients with 'malignant' LE for fear of exacerbating the underlying cancer, and that the obstruction will render therapy less effective. In the current study, the authors' experience providing CDT for such patients is discussed. METHODS: Cancer survivors with LE were referred to therapists at 2 Atlanta-area clinics. CDT consists of treatment (Phase 1) and maintenance phases (Phase 2). During Phase 1, the patient undergoes manipulative therapy and bandaging daily until the LE reduction plateaus; at that point, Phase 2 (self-care) begins. At the beginning and end of Phase 1, LE is quantified and differences in girth volume calculated. The results for patients completing Phase 1 therapy for LE in the presence of locoregional masses were compared with results for patients with LE in the absence of such disease. Both volume reduction of the affected limb and number of treatments to plateau were analyzed. RESULTS: Between January 2004, and March 2007, LE of 82 limbs in 72 patients was treated with CDT and Phase 1 was completed. The median number of treatments to plateau was 12 (range, 4-23 treatments); the median limb volume reduction was 22% (range, -23 to 164%). Nineteen limbs (16 patients) with associated chest wall/axillary or pelvic/inguinal tumors had nonsignificant difference in LE reduction (P = .75) in the presence of significantly more sessions to attain plateau (P = .0016) compared with 63 limbs in 56 patients without such masses. CONCLUSIONS: Patients with LE may obtain relief with CDT regardless of whether they have locoregional disease contributing to their symptoms. However, it will likely take longer to achieve that effect. Manipulative therapy of LE should not be withheld because of persistent or recurrent disease in the draining anatomic bed. Cancer 2008. (c) 2007 American Cancer Society.

PMID: 18085587 [PubMed - in process]
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Breast Cancer Res Treat. 2008 Feb 13 [Epub ahead of print]
Lymphatic venous anastomosis (LVA) for treatment of secondary arm lymphedema. A prospective study of 11 LVA procedures in 10 patients with breast cancer related lymphedema and a critical review of the literature.Damstra RJ, Voesten HG, van Schelven WD, van der Lei B.
Department of Dermatology, Phlebology and Lymphology, Nij Smellinghe Hospital, Compagnonsplein 1, 9202 NN, Drachten, The Netherlands, r.damstra@nijsmellinghe.nl.

Objective The incidence of breast cancer related lymphedema (BCRL) varies between 7-35% depending on the combination of treatment modalities. Early detection of BCRL is crucial in order to start an effective non-operative treatment program. Because of the lack of prospective research on this topic, this study was undertaken to prospectively determine the effect of Lympho Venous Anastomosis (LVA) on BCRL and to review the current literature. Study design and methods Ten patients who were previously treated for breast cancer by surgery, radiotherapy, and chemotherapy, and were unresponsive to 12-weeks of non-operative treatment, underwent an LVA procedure (Degni-Cordeiro). Objective measurements were gathered for circumferential measurement and water volumetry, and quality of life. Various types of lymphoscintigraphy were carried out pre-operatively and post-operatively at 3 and 12 months. Treatment was embedded in a multidisciplinary setting. Results Post-operative volume measurements initially showed a 4.8% reduction of lymphedema at 3 months and a 2% reduction after one year. Various scintigraphic parameters showed some improvement. Quality of life questionnaires reported minimal improvement. Reviewing the literature, only retrospective studies were found; these reported varying results for LVA procedures. The selection of patients, classification of lymphedema, indications and types of LVA, and additional therapeutic options were heterogeneous, not comparable, and lacked a validated method of effect-assessment. Conclusions Our results showed a minimal reduction in volume of lymphedema following LVA; in the literature, there was no convincing evidence of the success of LVA. Non-operative treatment and elastic stockings are still preferred by most patients with lymphedema, especially in early stages with few irreversible changes.

PMID: 18270813 [PubMed - as supplied by publisher]
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JAMA. 2008 Feb 13;299(6):632-3; author reply 633.
Comment on:
JAMA. 2007 Oct 24;298(16):1911-24.
Treatment for lymphatic filariasis and elephantiasis.Fox L, Beach MJ, Lammie PJ.
PMID: 18270350 [PubMed - indexed for MEDLINE]
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J Clin Oncol. 2008 Feb 10;26(5):759-67.
Implementing a survivorship care plan for patients with breast cancer.Ganz PA, Hahn EE.
Division of Cancer Prevention and Control Research, Jonsson Comprehensive Cancer Center, 650 Charles Young Dr South, Room A2-125 CHS, Los Angeles, CA 90095-6900, USA. pganz@ucla.edu

Breast cancer survivors account for 23% of the more than 10 million cancer survivors in the United States today. The treatments for breast cancer are complex and extend over a long period of time. The post-treatment period is characterized by gradual recovery from many adverse effects from treatment; however, many symptoms and problems persist as late effects (eg, infertility, menopausal symptoms, fatigue), and there may be less frequent long-term effects (eg, second cancers, lymphedema, osteoporosis). There is increasing recognition of the need to summarize the patient's course of treatment into a formal document, called the cancer treatment summary, that also includes recommendations for subsequent cancer surveillance, management of late effects, and strategies for health promotion. This article provides guidance on how oncologists can implement a cancer treatment summary and survivorship care plan for breast cancer survivors, with examples and linkage to useful resources. Providing the breast cancer treatment summary and survivorship care plan is being recognized as a key component of coordination of care that will foster the delivery
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Clin Appl Thromb Hemost. 2008 Feb 8 [Epub ahead of print]
Gastric Signet-Ring Cell Adenocarcinoma Presenting With Left Arm Deep-Vein Thrombosis and Bilateral Chylothorax.Kayacan O, Karnak D, Can BA, Sak SD, Beder S.
Ankara University School of Medicine.

A 28-year-old housewife, a life-long nonsmoker, presented with 3 weeks of pleuritic chest pain along with swollen right leg, left arm, and left breast. Six months previously she had left subclavian vein thrombosis. On admission, bilateral supraclavicular lymphedema on right leg and left arm and breast was observed and bilateral pleural fluid, chylous exudates, was detected. Abdomen computed tomography revealed abundant ascites and right ovarian enlargement. Whole body bone scintigraphy showed bone metastases on left humerus, right femur, and pelvis. Bronchial biopsy, obtained from edematous, hyperemic-irregular mucosa, revealed a carcinoma composed of signet-ring cells with intracytoplasmic mucin. Breast biopsy also showed signet-ring cells within the lymphatics. Pleural fluid cytology showed similar malignant cells. The patient was diagnosed as gastric signet-ring cell adenocarcinoma with endobronchial, mammary, ovarian, pleural, pericardial, peritoneal, and osteal metastases. The authors recommend that deep-vein thrombosis in unusual sites deserves further evaluation for an occult malignancy.

PMID: 18263634 [PubMed - as supplied by publisher]
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Gynecol Oncol. 2008 Feb 4 [Epub ahead of print]

Isolated sentinel lymph node dissection with conservative management in patients with squamous cell carcinoma of the vulva: A prospective trial.Moore RG, Robison K, Brown AK, Disilvestro P, Steinhoff M, Noto R, Brard L, Granai CO.
Program in Women's Oncology, Women and Infants' Hospital, Brown University, Providence, RI, USA.

OBJECTIVES: Sentinel lymph node (SLN) dissections have a high sensitivity and negative predictive value for the detection of metastatic disease. The objective of this study was to examine the inguinal recurrence rate along with complication rates for patients undergoing inguinal SLN dissection alone for vulvar carcinoma. METHODS: An IRB approved prospective study enrolled patients with biopsy proven squamous cell carcinoma of the vulva. Peritumoral injection of Tc-99 sulfur colloid and methylene blue dye was used to identify SLNs intraoperatively. Patients with SLNs negative for metastatic disease were followed clinically. Patients with metastasis detected in a SLN subsequently underwent a full groin node dissection followed by standard treatment protocols. RESULTS: Thirty-six patients were enrolled onto study with 35 undergoing a SLN dissection. All SNL dissections were successful with a mean of 2 SLN obtained per groin. There were 24 patients with stage I disease, 8 stage II, 3 stage III and 1 stage IV. A total of 56 SLN dissections were performed with 4 patients found to have inguinal metastasis by SLN dissection. There were 31 patients with a total of 46 SLN dissections found to be negative for metastatic disease. The median follow-up has been 29 months (range 8 to 51) with 2 groin recurrences for a groin recurrence rate of 4.3% and a recurrence rate per patient of 6.4%. There have been no reports of groin breakdown, extremity cellulitis or lymphedema. CONCLUSIONS: The recurrence rate for patients undergoing inguinal sentinel node dissection alone is low. These patients did not experience any complications as seen with complete groin node dissections. Sentinel lymph node dissection should be considered as an option for evaluation of inguinal nodes for metastatic disease.

PMID: 18255128 [PubMed - as supplied by publisher]
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Am J Dermatopathol. 2008 Feb;30(1):67-72.
Otophyma: a case report and review of the literature of lymphedema (elephantiasis) of the ear.Carlson JA, Mazza J, Kircher K, Tran TA.
Division of Dermatology, Albany Medical College, 47 New Scotland Ave., Albany, NY 12208, USA. carlsoa@mail.amc.edu

Phymas (swellings, masses, or bulbs) are considered the end-stage of rosacea and mostly affect the nose (rhinophyma), and rarely involve the chin (gnatophyma), the cheek (metophyma), eyelids (blepharophyma), or ears (otophyma). Herein, we report the case of a 57-year-old man who developed unilateral enlargement of his left ear over 2 years. Biopsy revealed changes of rosaceous lymphedema associated with Demodex infestation. Corticosteroid and minocycline therapies resulted in partial reduction of the ear enlargement. Literature review examining for cases of lymphedema (elephantiasis) of the ear revealed that chronic inflammatory disorders (rosacea (most frequent), psoriasis, eczema), bacterial cellulitis (erysipelas), pediculosis, trauma, and primary (congenital) lymphedema can all lead to localized, lymphedematous enlargement of the ear. Depending on the severity, medical treatment directed at the inflammatory condition for mild, diffuse enlargement to surgical debulking for extensive diffuse enlargement or tumor formation can improve the signs and symptoms of otophyma. Decreased immune surveillance secondary to rosaceous lymphedema may explain why Demodex infestation is common in rosacea and support the suspicion that phymatous skin is predisposed to skin cancer development.

PMID: 18212550 [PubMed - in process]
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Ann Plast Surg. 2008 Feb;60(2):228.
Lymphedema: a comprehensive review.Shimony A, Tidhar D.
PMID: 18216521 [PubMed - in process
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Int J Dermatol. 2008 Feb;47(2):174-7.
Cutaneous diffuse large B-cell lymphoma of the leg associated with chronic lymphedema.González-Vela MC, González-López MA, Val-Bernal JF, Fernández-Llaca H.
Department of Anatomical Pathology, Marqués de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain.

Development of malignant tumors is a rare but well known complication in chronic lymphedema (CL). We report herein a cutaneous diffuse large B-cell lymphoma of the leg associated with CL. An 89-year-old man presented with multiple cutaneous lesions on his right limb that showed a CL. Dermatological examination disclosed multiple violaceous, firm, slightly infiltrated nodules on the anterior aspect of the leg and the dorsum and sole of the foot. A biopsy of one nodule of the leg disclosed a diffuse large B-cell lymphoma, type of the legs. There was no evidence of lymphadenopathy on computed tomography (CT) scans of the chest, abdomen, and pelvis. A bone marrow aspiration and biopsy showed normal results. The patient was treated with local radiotherapy at a dose of 40 Gy, obtaining a highly significant, almost complete, clinical remission. A literature search identified 11 additional cases of primary cutaneous lymphoma associated with CL. An inadequate lymphatic drainage may make the lymphedematous region an immunologically vulnerable area, predisposing to neoplasia.

PMID: 18211492 [PubMed - in process]
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Int J Dermatol. 2008 Feb;47(2):154-9.
Cutaneous manifestations of chikungunya fever: observations made during a recent outbreak in south India.Inamadar AC, Palit A, Sampagavi VV, Raghunath S, Deshmukh NS.
Department of Dermatology, Venereology and Leprosy, BLDEA's SBMP Medical College, Hospital and Research Center, Bijapur, Karnataka, India. aruninamadar@rediffmail.com

BACKGROUND; Chikungunya fever is an Aedes mosquito-borne Arbo viral illness with significant morbidity. METHODS: In a recent outbreak of the disease in south India, the dermatologic manifestations of 145 patients attending a tertiary care hospital were recorded. RESULTS: All age groups were affected, including newborns. Some of the cutaneous features were observed during the acute stage of the illness, and others during convalescence or thereafter. Pigmentary changes were found to be the most common cutaneous finding (42%), followed by maculopapular eruption (33%) and intertriginous aphthous-like ulcers (21.37%). Lesions with significant morbidity were generalized vesiculobullous eruptions (2.75%), found only in infants, lymphedema, and intertriginous aphthous-like ulcers. Exacerbation of existing dermatoses, such as psoriasis, and unmasking of undiagnosed Hansen's disease were observed. A perivascular lymphocytic infiltrate was a consistent histopathologic finding in all types of skin lesions. All patients responded well to symptomatic, conservative treatment. CONCLUSIONS: The cutaneous findings hitherto not reported may be the result of the African genotype of the virus detected during this outbreak in India.

PMID: 18211486 [PubMed - in process]
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Plast Reconstr Surg. 2008 Feb;121(2):521-8.
Learning from a lymphedema clinic: an algorithm for the management of localized swelling.Garfein ES, Borud LJ, Warren AG, Slavin SA.
Boston, Mass. From the Harvard Plastic Surgery Program, Harvard Medical School, and the Division of Plastic Surgery, Beth Israel Deaconess Medical Center.

BACKGROUND:: Lymphedema is a chronic disease causing significant morbidity for affected patients. It can be difficult to diagnose, and patients are often frustrated by multiple referrals and inadequate therapies. Centralized, comprehensive care for the patient presenting with lymphedema or other causes of localized swelling allows for appropriate evaluation and provides improved management and treatment. METHODS:: A 4-year review of the first 100 patients seen at the Beth Israel Deaconess Medical Center Lymphedema Clinic was performed. On the basis of the clinical experience from these patients, an algorithm for diagnosing and managing patients with localized swelling was developed. RESULTS:: The mean age of the patients was 50 years, and 81 percent of the patients were women. On average, patients had experienced their symptoms for 11.6 years (range, 3 months to 60 years). Lymphoscintigraphy was performed on 43 patients, 81 percent of whom showed lymphatic obstruction or dysfunction. In total, 75 percent of patients were diagnosed with lymphedema based on clinical presentation or additional testing. Fourteen of these patients underwent subsequent excisional procedures, whereas the rest were managed conservatively with compression garments. CONCLUSIONS:: Patients presenting with swollen extremities can frequently be diagnosed through history and physical examination alone, but many patients require more extensive diagnostic workup. An algorithm for the management of these patients can facilitate evaluation and treatment.

PMID: 18300971 [PubMed - in process]
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Prenat Diagn. 2008 Feb;28(2):167.
Perinatal diagnosis of a lymphoedema-distichiasis syndrome.Brice G.
St Georges University of London, Clinical Genetics, UK for the Lymphoedema Research Consortium.

PMID: 18236429 [PubMed - in process