• Title/Summary/Keyword: congenital lymphedema

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Surgical Treatment for Primary Lymphedema: A Systematic Review of the Literature

  • Miguel Angel Gaxiola-Garcia;Joseph M. Escandon;Oscar J. Manrique;Kristin A. Skinner;Beatriz Hatsue Kushida-Contreras
    • Archives of Plastic Surgery
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    • v.51 no.2
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    • pp.212-233
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    • 2024
  • This is a retrospective review of surgical management for primary lymphedema. Data were extracted from 55 articles from PubMed MEDLINE, Web of Science, SCOPUS, and Cochrane Central Register of Controlled Trials between the database inception and December 2022 to evaluate the outcomes of lymphaticovenous anastomosis (LVA) and vascularized lymph node transfer (VLNT), and outcomes of soft tissue extirpative procedures such as suction-assisted lipectomy (SAL) and extensive soft tissue excision. Data from 485 patients were compiled; these were treated with LVA (n = 177), VLNT (n = 82), SAL (n = 102), and excisional procedures (n = 124). Improvement of the lower extremity lymphedema index, the quality of life (QoL), and lymphedema symptoms were reported in most studies. LVA and VLNT led to symptomatic relief and improved QoL, reaching up to 90 and 61% average circumference reduction, respectively. Cellulitis reduction was reported in 25 and 40% of LVA and VLNT papers, respectively. The extirpative procedures, used mainly in patients with advanced disease, also led to clinical improvement from the volume reduction, as well as reduced incidence of cellulitis, although with poor cosmetic results; 87.5% of these reports recommended postoperative compression garments. The overall complication rates were 1% for LVA, 13% for VLNT, 11% for SAL, and 46% for extirpative procedures. Altogether, only one paper lacked some kind of improvement. Primary lymphedema is amenable to surgical treatment; the currently performed procedures have effectively improved symptoms and QoL in this population. Complication rates are related to the invasiveness of the chosen procedure.

Effects of Manual Lymph Drainage on Patients with Secondary Lymphedema of Legs After Gynecologic Cancer (부인과 암 이후 이차적인 다리 림프 부종 환자에게 적용한 림프흡수 마사지의 효과)

  • Jeong, Seong-gwan;Lee, Seung-byung
    • The Journal of Korean Academy of Orthopedic Manual Physical Therapy
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    • v.22 no.2
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    • pp.35-39
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    • 2016
  • Background: The superficial lymphatic system is divided into areas called lymphatic territories which are separated by watersheds. When the lymphatic system fails to remove its load either due to surgery, radiotherapy or some congenital malformation of it then the fluid and the proteins and wastes contained within it accumulates in that territory. Anastomotic connections exist across the watersheds and while they can work unaided manual lymph drainage (MLD) can significantly help drainage across them into unaffected lymphatic territories. The purpose of the study is to examine the effectiveness of a manual technique in moving fluids and softening hardened tissues using a tape measure and Patient-Specific Functional Scale. Methods: We examined the movement of fluids from the affected limbs of lymphedema patients who underwent a standardized 30-min treatment using the Dr. Vodder method of MLD. We chose a typical cross section of patients with secondary leg or secondary arm lymphedema. The lymphedema patient was also measured after the conclusion of treatment and underwent a follow-up control measurement, within 8 weeks. Both evaluation tools indicated a movement of fluid to different and unblocked lymphatic territories as well as a softening of tissues in some of the affected limbs. Results: MLD is an effective means of fluid clearance when it accumulates as a consequence of a failure of the lymphatic system. It seems likely that MLD has a systemic effect on the lymphatic system and that it can improve flow from otherwise normal tissues. Conclusions: It is hypothesized that a series of treatments would result in even more significant improvements.

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Congenital Constriction Band Syndrome of Hand ; The Correction Under The Microscope (선천성 수부 윤상 수축대 증후군에 대한 미세현미경을 이용한 수술적 교정)

  • Hwang, Ho;Kim, Yong-Kyu;Whang, Jong-Ick
    • Archives of Reconstructive Microsurgery
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    • v.14 no.1
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    • pp.70-76
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    • 2005
  • The Z-plasty has been known as a most common surgical treatment of congenital circumferential constriction band syndrome of the hand. There were thirteen patients of congenital circumferential constriction band syndrome of the hand. All patients underwent Z-plasty under microscope. we did microsurgical dissection to minimize vascular, neural and lymphatic injury, and then considering secondary correction and scar contracture, tried to preserve as much subcutaneous fat and skin flap as possible without any excision. There were less skin necrosis and lymphedema as a result of vascular compromise. Using microscope offers several advantages. First, preventing vascular, neural and lymphatic injury. Second, getting an accurate suture approximation. finally, preserving as much subcutaneous fat and skin flap as possible without any excision. The outcome of digit growth and contour can be excellent than we expected. As a results, we believe that correction of congenital circumferential constriction band syndrome of the hand under the microscope have better results.

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Management for Gait Disturbance and Foot Pain in a Patient with Klippel-Trenaunay-Weber Syndrome : A case report

  • Choi, Yoon-Hee
    • Journal of The Korean Society of Integrative Medicine
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    • v.9 no.4
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    • pp.85-89
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    • 2021
  • Background : Klippel-Trenaunay-Weber syndrome (KTS) is a rare congenital medical condition characterized by complex vascular malformation. KTS consists of a classic triad of capillary malformation (hemangioma), venous malformations and bone or soft tissue hypertrophy causing limb asymmetry. The aim of this report is to describe management for gait disturbance and foot pain in a Patient with KTS using custom-made total contact insole. Case presentation : A 32-year-old man with KTS presented with a 3-year history of gait disturbance on hard surface due to right first toe pain and Achilles tendon tightness. The patient had soft tissue hypertrophy, varicose veins and port-wine stains over the right lower limb associated with KTS. True leg length discrepancy was 2 cm. We prescribed custom-made total contact insole to protect his deformed foot and correct leg length discrepancy. The insole of right side included wedge shaped heel lift and the insole of left side included full length lift to add extra support on unaffected side. Also, we provided compression stocking and physiotherapy including manual lymphatic drainage for lymphedema and stretching exercise for tightness in right lower extremity. At 3 years follow-up, postural alignment including pelvic obliquity was improved using a custom-made total contact insole. The degree of scoliosis and foot pain were also reduced. Conclusion : An individualized and multidisciplinary approach is essential regarding the complexity of comorbidities in patients with KTS. For patients with KTS, orthotic management should be considered to prevent and correct deformities related to KTS. Active orthotic management, compression stocking and physiotherapy can enhance the quality of life and function in patients.