Developmental Disorders of the Lymphatics

An information blog for disorders of the lymphatics. For all articles, please click on "Archives" - Due to spammers, I will no longer allow comments, sorry.

Wednesday, September 26, 2007

Complex lymphatic malformations: diagnostic and therapeutical implications

Complex lymphatic malformations: diagnostic and therapeutical implications
Cir Pediatr. 2007 Apr
Luis AL, López JC, Encinas JL, Suárez O, Burgos L, Diaz M, Soto C, Ros Z.
Hospital Universitario La Paz, Departamento de Cirugía Pediátrica, Madrid.

BACKGROUND: Complex lymphatic malformations (CLM) consist of disturbances of lymphatic system development, most often with a genetic origin and with mixed vascular system involvement: lymphatic, venous and capillary. They affect a large corporal area or are associated to other syndromes or systemic diseases.

METHODS: We reviewed 21 patients with CLM treated in our hospital during the last 15 years. We used D2-40 monoclonal antibody (by immunohistochemistry) as lymphatic marker to evaluate the level of lymphatic involvement. Furthermore we analysed surgical implications in this group of patients.

RESULTS: Twelve children had only lymphatic involvement and nine mixed lymphatic-capillary or lymphatic-venous one. Two died of: respiratory insufficiency (in the neonatal period) and refractory hypoproteinemia (at 8 years of age). The skin was affected between 10 and 35% of total body surface. Three patients suffered from visceral involvement (lungs and mediastinum) and eighteen musculoskeletal. Severe deformity (20), lymphorhagia (15), repeated lymphangitis and chronic pain (5) were the most common symptoms reported. The immunoreaction intensity with monoclonal antibody D2-40 was related to the severity of the local and systemic involvement as well as to the presence of associated malformations. Fifteen cases underwent sequential surgical treatment, seven were treated with sclerotherapy (OK-432) and four with CO2 laser vaporization. Residual lymphorhagia in patients with total extirpation of the lymphatic malformation stopped after repeated evacuator punctures and healing took place.

CONCLUSIONS: (1) D2-40 monoclonal antibody is a marker of bad prognosis in CLM. (2) The complete excision of the lymphatic malformation lead to healing and the associated lymphorragia should not be considered as a recurrence, which will stop with evacuator punctures in all cases. (3) A multidisciplinary team approach is essential for the proper care of CLM in order to minimize postoperative sequelae and late complications.

PMID: 17650723 [PubMed - in process]

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Wednesday, September 12, 2007

Radiotherapy in congenital vulvar lymphangioma circumscriptum.

Radiotherapy in congenital vulvar lymphangioma circumscriptum.

Int J Gynecol Cancer. 2007 Aug

Yildiz F, Atahan IL, Ozyar E, Karcaaltincaba M, Cengiz M, Ozyigit G, Aydin A, Usubütün A, Ayhan A.

Ferah Yildiz, MD, Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, 06100 Ankara, Turkey. Email:

Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Congenital lymphangioma circumscriptum (LC) of the vulva is a rare disorder with unknown etiology. Treatment options include ablative approaches such as laser therapy, sclerotherapy, and surgery. Radiotherapy has been shown to be effective in the management of congenital lymphangioma especially in the thoracic and abdominal lesions. In this report, we describe a patient with persistent vulvar LC despite sclerosing therapy and several surgical excisions. She was treated with a course of external radiotherapy and showed a dramatic objective response with relief of all symptoms.

Blackwell Synergy

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