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      Extensive Fetal Congenital Subcutaneous Mixed Venous Lymphatic Lesion: Prenatal Diagnosis and Postnatal Management

      case-report

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          Abstract

          Vascular lesions may be categorized as proliferative tumors, such as hemangiomas, or nonproliferative malformations that include capillary, lymphatic, venous, arterial, or mixed lesions. Lymphatic malformations are benign localized congenital malformations of the lymphatic system. They may be microcystic or macrocystic lesions or a combination of both. The lesions may also be uniseptate or multiseptate, and are more commonly located in the head and neck or axillary region. Prenatal diagnosis is based on ultrasound and magnetic resonance imaging. Postnatal management largely depends on the size and location of the lesion. This is the first case report of prenatally diagnosed extensive subcutaneous macrocystic venous lymphatic malformation involving the fetal thorax, back, pelvis, and lower extremities. Prenatal course and postnatal management are described. This report will aid other specialists in the field of prenatal diagnosis and postnatal surgery in the evaluation and management of these patients.

          Most cited references20

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          Percutaneous sclerotherapy for lymphatic malformations: a retrospective analysis of patient-evaluated improvement.

          To evaluate the midterm outcomes of percutaneous sclerotherapy of lymphatic malformations (LMs) as judged by patients.
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            Vascular low-flow malformations in children: current concepts for classification, diagnosis and therapy.

            Congenital vascular malformations (CVM) are made of dysplastic vessels with no cellular proliferation. Low- or slow-flow malformations (LFM) consist predominantly of venous and/or lymphatic vessels. Correct terminology is necessary for differentiating vascular malformations from tumours such as haemangiomas, in order to prevent ineffective or even adverse therapy. The role of the radiologist in the management of patients is two-fold: making the diagnosis with the use of ultrasound and magnetic resonance imaging, and performing sclerotherapy, which is the treatment of choice. Prior to sclerotherapy, percutaneous phlebography is necessary to visualize the dynamic situation inside the lesion and the flow into the adjacent vascular system. The double-needle technique is a useful therapy option reducing the risk of embolisation of the sclerosing agent. Large lesions might need subsequent surgical treatment. A multidisciplinary approach is substantial for optimal patient management.
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              Percutaneous treatment of low flow vascular malformations.

              Low flow vascular malformations, especially venous and macrocystic lymphatic malformations, are effectively treated by percutaneous intralesional injection of sclerosant drugs, such as ethanol and detergent sclerosant drugs. Good to excellent results are possible in 75%-90% of patients who undergo serial sclerotherapy. Most adverse effects are manageable, but severe complications can result from the intravascular administration of ethanol. It is generally recommended that the treatment of vascular malformations be performed in a multidisciplinary setting by practitioners with appropriate training and support.
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                Author and article information

                Journal
                AJP Rep
                AJP Rep
                10.1055/s-00000169
                AJP Reports
                Thieme Medical Publishers (333 Seventh Avenue, New York, NY 10001, USA. )
                2157-6998
                2157-7005
                25 February 2015
                April 2015
                : 5
                : 1
                : e37-e42
                Affiliations
                [1 ]Department of Obstetrics and Gynecology, University of Arkansas for Medical Sciences, Arkansas
                [2 ]Department of Radiology, Arkansas Children's Hospital, Arkansas
                [3 ]Department of Otolaryngology, Arkansas Children's Hospital, Arkansas
                Author notes
                Address for correspondence Imelda N. Odibo, MD 4301 W. Markham St., #518 Little Rock, AR 72205 inodibo@ 123456uams.edu
                Article
                140040
                10.1055/s-0034-1544107
                4502633
                4bf972e9-0987-44a6-b5db-ff9028c6a06b
                © Thieme Medical Publishers
                History
                : 24 July 2014
                : 04 December 2014
                Categories
                Article

                congenital disorders,pediatrics,pregnancy,obstetrics and gynecology,maternal fetal medicine,venous lymphatic lesions

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