Cases reported "Lymphedema"

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11/158. Congenital balloon digits in two neonates caused by constriction rings.

    Balloon digits were found in two neonates with congenital constriction ring syndrome. The affected digits were the right long finger and right great toe. They were surgically treated at the age of 10 and 9 days, respectively. Morphologic improvement was dramatic after surgery. In cases with extensive enlargement, severe cyanosis, redness, and no subsidence of edema within several days after birth, early operative treatment may be necessary to maintain digit viability and prevent autoamputation due to circulatory embarrassment. It can also be helpful to prevent fibrosis of the subcutaneous tissue. Pathologic examination revealed marked proliferation of fibrous tissue and lymphatic vessels.
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12/158. Vulvar hypertrophy with lymphedema. A mimicker of aggressive angiomyxoma.

    We report the case of a 43-year-old quadriplegic woman with bilateral vulvar enlargement. The clinical impression was labial hypertrophy, but the microscopic features mimicked aggressive angiomyxoma because of the location, hypocellular proliferation of fibroblastic cells in an edematous-myxoid stroma, and vessels with perivascular collagen deposition, which simulated the thick-walled vessels of aggressive angiomyxoma. Since the lesion lacked true thick-walled vessels and contained ectatic tortuous lymphatics, the pathologic interpretation was lymphedema. This vulvar lesion should be recognized to prevent the misdiagnosis of aggressive angiomyxoma.
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13/158. Cutaneous oozing of lymphatic fluid after interventional cardiac catheterization in a patient with noonan syndrome.

    A 15-year-old girl with noonan syndrome, intestinal lymphangiectasia and severe valvar pulmonary stenosis had an abnormal lymphangioscintigram that showed intense activity in the inguinal regions bilaterally. Cutaneous oozing of lymphatic fluid from the groin wound complicated percutaneous balloon pulmonary valvoplasty. This previously unreported complication highlights the risk of damage to abnormal lymphatic channels in patients with noonan syndrome who undergo interventional catheterization.
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keywords = lymphatic
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14/158. Bilateral upper limb lymphoedema associated with psoriatic arthritis: a case report and review of the literature.

    Lymphoedema is an unusual extra-articular feature of rheumatoid arthritis and has rarely been described in psoriatic arthritis. We report a 41-year-old man with psoriasis and psoriatic arthritis who developed bilateral lymphoedema of the upper extremities. lymphoscintigraphy showed absent lymphatic drainage in the right arm and a subnormal increase in lymphatic flow under manual exertion in both arms. Colour Doppler ultrasound studies did not reveal venous or arterial abnormalities. Conservative management and therapy with cyclosporin (for worsening arthritis) resulted in partial resolution of the lymphoedema and improvement of flow parameters on the right side upon repeat lymphoscintigraphy.
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keywords = lymphatic
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15/158. Congenital penoscrotal lymphedema complicated by sepsis associated with a streptococcal infection.

    Congenital lymphedema is a relatively rare disease caused by congenital abnormality of the lymphatic system. Although bacterial infection frequently causes complications with lymphedema, severe sepsis in congenital lymphedema of the genitalia has not yet been reported. We describe a patient with congenital penoscrotal lymphedema complicated by cellulitis, lymphangitis, and severe sepsis associated with a streptococcal infection. This case represents the importance of obtaining a detailed clinical history and physical findings.
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keywords = lymphatic
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16/158. yellow nail syndrome: does protein leakage play a role?

    yellow nail syndrome is characterized by primary lymphoedema, recurrent pleural effusion and yellow discoloration of the nails. Although mechanical lymphatic obstruction is assumed to be the underlying pathology, it cannot explain the common finding of high albumin concentration in the pleural space. This paper describes a case of yellow nail syndrome presenting with the classical triad of lymphoedema, recurrent pleural effusion and yellow discoloration of the nails, associated with persistent hypoalbuminaemia and increased enteric loss of albumin. Based on the findings in this case and those in the literature, it is speculated that increased microvascular permeability may contribute to the pathogenesis of this syndrome.
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17/158. Severe lower limbs lymphedema of late onset revealing polysplenia syndrome--a case report.

    Polysplenia syndrome includes a group of congenital abnormalities involving the spleen, gastrointestinal tract, liver, and cardiovascular system. A case of severe lower limbs lymphedema occurring in a young woman with polysplenia, azygous continuation of the inferior vena cava, short pancreas, and preduodenal portal vein is reported. Lower limb lymphedema could represent a new clinical manifestation associated with polysplenia syndrome. lymphedema could be the result of a primary anomaly of the lymphatic system or be induced by high pressure in the venous system or by compression of the lymphatic circulation by the hypertrophic azygous vessels in the posterior mediastinum.
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18/158. Granulomatous lymphangitis of the scrotum and penis. Report of a case and review of the literature of genital swelling with sarcoidal granulomatous inflammation.

    BACKGROUND: Acquired lymphedema of the genitalia is a rare childhood presentation and is more common in elderly individuals secondary to pelvic/abdomenal malignancy or its therapy or worldwide due to filariasis. OBJECTIVE: Herein, we report a case of a healthy 11-year-old boy who presented with a 1-year history of chronic, asymptomatic scrotal and penile swelling. biopsy revealed edema, lymphangiectases and peri- and intralymphatic sarcoidal type granulomas. This histologic pattern of granulomatous lymphangitis is most commonly associated with orofacial granulomatosis (granulomatous cheilitis and melkersson-rosenthal syndrome) and Crohn's disease. Treatment with topical steroids and physical support has resulted in marked improvement. No systemic disease (Crohn's disease) is evident 1 year later. literature review revealed 44 cases of genital lymphedema with non-infectious granulomas. The majority of these young patients had Crohn's disease, frequently with anal involvement and a minority, both with and without Crohn's disease, had orofacial granulomatosis. CONCLUSIONS: Granulomatous lymphangitis should be considered in the differential diagnosis of chronic idiopathic swelling of the genitalia, particularly in younger individuals. Further clinical examination, additional laboratory studies and close follow-up for co-existing or subsequent development of Crohn's disease should be performed. The overlap between granulomatous lymphangitis of the genitalia, Crohn's disease and orofacial granulomatosis suggest that granulomatous lymphangitis of the genitalia may represent a forme fruste of Crohn's disease.
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keywords = lymphatic
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19/158. The use of manual edema mobilization for the reduction of persistent edema in the upper limb.

    Management of persistent edema with the common treatment methods reported in the literature is not always successful. Manual edema mobilization (MEM) is a relatively new treatment regimen derived from established European and Australian lymphedema reduction regimens. It includes the use of exercises, light skin-tractioning massage techniques following the lymphatic pathways, and the use of low-compression garments. The typical patient who may benefit from the use of MEM has a presumed healthy lymphatic system, is an active participant, and performs some of the techniques independently between therapy sessions. This case report describes the use of MEM on a patient with multiple trauma, which resulted in a significant reduction--78%--of the persistent edema in the affected upper limb. A theoretic rationale is offered for each MEM technique.
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ranking = 2
keywords = lymphatic
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20/158. Congenital lymphedema presenting with increased nuchal translucency at 13 weeks of gestation.

    Congenital lymphedema is an autosomal dominant condition characterized by chronic tissue swelling caused by deficient lymphatic drainage due to hypoplastic/aplastic lymphatic vessels and usually affecting the lower limbs. The locus of the gene has been identified in the long arm of chromosome 15. We report one case of congenital lymphedema presenting with increased nuchal translucency at 13 weeks of gestation.
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