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

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      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.

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      Most cited references 22

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      Newly delineated syndrome of congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE syndrome) in seven patients.

      We present a series of seven patients who were previously diagnosed with Proteus syndrome, but who do not meet published diagnostic criteria for this disorder and whose natural history is distinct from that of Proteus syndrome. This newly recognized phenotype comprises progressive, complex, and mixed truncal vascular malformations, dysregulated adipose tissue, varying degrees of scoliosis, and enlarged bony structures without progressive bony overgrowth. We have named this condition congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE syndrome) on a heuristic basis. In contrast to the bony distortion so characteristic of Proteus syndrome, distortion in CLOVE syndrome occurs only following major or radical surgery. Here, we contrast differences and similarities of CLOVE syndrome to Proteus syndrome.
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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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          Characterization of a distinct syndrome that associates complex truncal overgrowth, vascular, and acral anomalies: a descriptive study of 18 cases of CLOVES syndrome.

           Ahmad Alomari (2009)
          Overgrowth syndromes with complex vascular anomalies pose a challenge for diagnosis and management. The purpose of this descriptive study is to present a cohort of patients with congenital lipomatous overgrowth, vascular malformations, and epidermal nevi syndrome, a distinct clinical phenotype characterized by a complex truncal-lipomatous mass, vascular, acral, and other anomalies. This cohort was ascertained following review of patient data entered into the Vascular Anomalies Center database of the Children's Hospital, Boston over a period of 7 years. Clinical data, imaging findings, and the photographic archive were reviewed. The search identified 18 unrelated patients with a distinct phenotype. Variable portions of the truncal masses observed in these patients were composed of a lymphatic malformation. Capillary malformations and high-flow lesions were commonly encountered. The lipomatous mass infiltrated the adjacent anatomic spaces and was associated with capillary, lymphatic, venous, and arteriovenous vascular malformations. Paraspinal-intraspinal extension was associated with morbid sequelae. Acral deformities included large, wide feet and hands, macrodactyly, and a wide sandal gap. Scoliosis and other musculoskeletal, neurologic, renal, and cutaneous malformations were also encountered. The uniform and highly characteristic features of the truncal lipomatous mass, in combination with vascular, acral, and other anomalies, provide evidence of a distinct nosologic and clinical entity. Morbid sequelae of the truncal involvement in this condition can be deforming and disabling; hence, prompt diagnosis and multidisciplinary care are necessary.

            Author and article information

            [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@
            AJP Rep
            AJP Rep
            AJP Reports
            Thieme Medical Publishers (333 Seventh Avenue, New York, NY 10001, USA. )
            25 February 2015
            April 2015
            : 5
            : 1
            : e37-e42
            4502633 10.1055/s-0034-1544107 140040
            © Thieme Medical Publishers


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