Cutaneous Vascular Malformations Research Articles

Obstetric Management of Klippel-Trenaunay Syndrome

Klippel-Trenaunay syndrome is a rare congenital disease characterized by extensive cutaneous vascular malformations, venous varicosities, focal abnormalities of the deep venous system, and underlying soft tissue or bony hypertrophy. Given the rarity of the disease, there is little information available to counsel patients with Klippel-Trenaunay syndrome regarding obstetric outcome. We report our experience with 3 patients in whom Klippel-Trenaunay syndrome complicated 4 pregnancies. Successful delivery of a healthy infant at or beyond 36 weeks of gestation was achieved in all pregnancies. One of the 4 pregnancies was complicated by pulmonary embolism. Klippel-Trenaunay syndrome was once thought to be a contraindication to pregnancy. With careful management, successful pregnancies can be achieved.

Cutaneous vascular malformation associated with osteolysis or Gorham's sign

International Journal of DermatologyVolume 43, Issue 12 p. 958-961 Cutaneous vascular malformation associated with osteolysis or Gorham's sign M. Stefanidou, Corresponding Author M. Stefanidou From the Departments of Dermatology and Pathology, Heraklion University General Hospital, Heraklion, Crete, Greece M. Stefanidou Department of Dermatology Heraklion University Hospital Heraklion 71110 Crete Greece E-mail: [email protected]Search for more papers by this authorD. Ioannidou, D. Ioannidou From the Departments of Dermatology and Pathology, Heraklion University General Hospital, Heraklion, Crete, GreeceSearch for more papers by this authorJ. Panayotides, J. Panayotides From the Departments of Dermatology and Pathology, Heraklion University General Hospital, Heraklion, Crete, GreeceSearch for more papers by this authorA. Tosca, A. Tosca From the Departments of Dermatology and Pathology, Heraklion University General Hospital, Heraklion, Crete, GreeceSearch for more papers by this author M. Stefanidou, Corresponding Author M. Stefanidou From the Departments of Dermatology and Pathology, Heraklion University General Hospital, Heraklion, Crete, Greece M. Stefanidou Department of Dermatology Heraklion University Hospital Heraklion 71110 Crete Greece E-mail: [email protected]Search for more papers by this authorD. Ioannidou, D. Ioannidou From the Departments of Dermatology and Pathology, Heraklion University General Hospital, Heraklion, Crete, GreeceSearch for more papers by this authorJ. Panayotides, J. Panayotides From the Departments of Dermatology and Pathology, Heraklion University General Hospital, Heraklion, Crete, GreeceSearch for more papers by this authorA. Tosca, A. Tosca From the Departments of Dermatology and Pathology, Heraklion University General Hospital, Heraklion, Crete, GreeceSearch for more papers by this author First published: 09 August 2004 https://doi.org/10.1111/j.1365-4632.2004.01985.xCitations: 9 Fosamax is the trade name of alendronate sodium. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume43, Issue12December 2004Pages 958-961 RelatedInformation

Skeletal vascular lesions in childhood and adolescence

Currently, vascular lesions are being classified either as tumors with inherent proliferative potential or as vascular malformations with early manifestation and growth commensurate with the growth of the child. This new classification is also applicable to skeletal vascular lesions. 30% of all skeletal vascular lesions manifest in the first two decades of life, with a preponderance of malformations. They are attributed to disturbances in the complex cascade of angiogenesis and a minority may be hereditary. Multiple cutaneous vascular malformations are associated with mutations of the TIE2- and Glomulin-Genes. Vascular malformations with involvement of multiple skeletal elements may pursue an aggressive course and manifest as massive osteolysis. Among the epithelioid vascular tumours of bone, also in young patients, benign epithelioid hemangioma can be distinguished on a morphological basis from epithelioid hemangioendothelioma as an entity with low malignant potential.

Estudios de imagen en el diagnóstico de los hemangiomas y malformaciones vasculares

The diagnosis of haemangiomata and cutaneous vascular malformations is based on clinical history and physical exploration. Imaging studies might be necessary to clarify and confirm the diagnosis, and in order to analyse the extent of the lesions by permitting an evaluation of their non-visible component as well as the affection of neighbouring structures. Finally, they also play an important role when it comes to planning and directing treatment, whether surgical or endovascular. The imaging techniques employed for haemangiomas and vascular malformations include: plain films ultrasound (Doppler), computerised tomography (Angio-CT), magnetic resonance (Angio-MR) and the angiographic techniques (arteriography, phlebography).

Cutis marmorata telangiectatica congenita with hemangiomatous histopathologic features

Cutis marmorata telangiectatica congenita (CMTC) is a congenital cutaneous vascular malformation that is diagnosed on the typical clinical findings of persistent cutis marmorata, telangiectasia, phlebectasia, occasional ulceration and atrophy, and tendency for clinical improvement. Histopathologic findings seldom have been reported. We report a case of CMTC with atypical histopathologic findings of proliferation of vasculature in addition to dilated capillaries in the dermis. We have reviewed all cases of CMTC with histopathologic findings published in the English-language literature. The most common and prominent finding was dilation of capillaries and veins in the dermis (13/15 cases). Two cases, including our own, demonstrated proliferation of vascular channels. The implications of vascular proliferation on the clinical course of CMTC are unclear. (J Am Acad Dermatol 2003;48:950-4.)

Hemangiomas y malformaciones vasculares cutáneas y patología asociada. Un nuevo síndrome neurocutáneo

Cutaneous hemangiomas and vascular malformations constitute a systemic disease that is the most frequent of the neurocutaneous syndromes reported to date. It appears more frequently in women than in men. The aim of this study was to describe the characteristics of this new neurocutaneous syndrome. We studied a large number of patients who presented cutaneous hemangiomas and/or vascular malformations located anywhere on the body. The patients underwent periodic clinical follow-up and imaging studies due to their aesthetic, functional and/or neurological problems. Clinical evaluation revealed a wide range in the size and severity of cutaneous vascular lesions and in their relationship with various internal anomalies, which were almost always located in the areas underlying the cutaneous vascular lesions. Those situated in the head, face, neck and chest were first discovered and are the best known. The most frequent findings were cerebellar malformations, persistence of the trigeminal artery, absence of the carotid or vertebral arteries and congenital heart disease. Many of the patients with this syndrome had mental retardation or borderline mental retardation. This disorder appears more frequently in females than in males. It is the most recently described neurocutaneous syndrome and is also the most prevalent. This syndrome is associated with numerous disorders that can be located in the areas underlying the cutaneous vascular lesions anywhere on the body. Cutaneous hemangiomas and subjacent hemangiomatous tumors, like other associated vascular abnormalities, can show a parallel, spontaneous increase or decrease in size without treatment. Complete occlusion of the vessels can occur while vascular malformations show no progression or involution. This new neurocutaneous syndrome is also known as Pascual-Castroviejo type II syndrome and PHACE.

KLIPPEL-TRÉNAUNAY SYNDROME: 2 CASE REPORTS AND A REVIEW OF GENITOURINARY MANIFESTATIONS

We summarize the literature and present our experience with genitourinary manifestations of the Klippel-Trénaunay syndrome, which can lead to challenging management problems. We report on 2 patients with genitourinary manifestations of the Klippel-Trénaunay syndrome and performed a MEDLINE review of the literature using the key words "Klippel-Trénaunay," "vascular malformation" and "genitourinary." Genitourinary manifestations were cited in 18 articles, including 1,174 cases of the Klippel-Trénaunay syndrome, detailing the presentation and management of bladder, external genitalia and retroperitoneal involvement in the Klippel-Trénaunay syndrome. The overall genitourinary symptoms in patients with the Klippel-Trénaunay syndrome seem to occur in the more severe cases and usually involve cutaneous vascular malformations of the trunk, pelvis and genitalia. Intra-abdominal and intrapelvic extension of the vascular malformations of the Klippel-Trénaunay syndrome frequently occurs concurrently with the lower abdominal, pelvic cutaneous involvement of the external genitalia, as in our 2 cases and in our review of the literature. These data provide a better understanding of the spectrum of genitourinary manifestations in the Klippel-Trénaunay syndrome and provide insight for the clinician to formulate individual therapies for these patients.

Acquired tufted angioma in association with a complex cutaneous vascular malformation

Acquired tufted angioma in association with a complex cutaneous vascular malformation

Blue rubber bleb nevus (Bean syndrome): evolution of four cases and clinical response to pharmacologic agents.

Blue rubber bleb nevus (BRBN) syndrome is a rare disorder characterized by distinctive cutaneous and gastrointestinal vascular malformations. The latter may lead to bleeding complications. We followed four affected children for at least 5 years. The evolution of their disease and the value of pharmacologic agents (steroids, interferon) in the management of some of these patients are discussed.

An association between autosomal dominant cerebral cavernomas and a distinctive hyperkeratotic cutaneous vascular malformation in 4 families.

Cerebral cavernomas (CCMs) are vascular malformations that may be inherited as an autosomal dominant condition for which a gene, CCM1, was mapped to chromosome 7. Poorly defined cutaneous malformations were sometimes described in association with CCMs. During a national survey, 57 French CCM families were studied. Co-occurrence of CCMs and a distinctive cutaneous vascular malformation was observed in 4 families. Ten individuals belonging to these families showed similar hyperkeratotic cutaneous capillary venous malformations (HCCVMs). In 3 families, the histology showed orthokeratosis and hyperkeratosis as well as dilated capillaries in the dermis extending to the hypodermis and confirmed the diagnosis of HCCVM. Genetic analysis strongly supports linkage of these families to the CCM1 locus on chromosome 7. The HCCVM seems to be a peculiar cutaneous vascular malformation associated with CCMs. These data strongly suggest that HCCVMs and CCMs in these families are due to the same genetic abnormality.

Cutaneous vascular anomalies. Part I. Hamartomas, malformations, and dilatation of preexisting vessels

Classification of cutaneous vascular anomalies is difficult because conceptual confusion persists between vascular neoplasms and malformations. However, hemangiomas of the infancy fulfill criteria both for hyperplasia and neoplasm because they result from proliferation of endothelial cells, but often undergo complete regression. Despite these pitfalls we have classified cutaneous vascular anomalies into the following categories: hamartomas, malformations, dilatations of preexisting vessels, hyperplasias, benign neoplasms, and malignant neoplasms. In this first part of our clinicopathologic review of vascular anomalies, hamartomas, malformations, and dilatation of preexisting vessels are covered. Hamartomas include several combined vascular and melanocytic proliferations grouped as phakomatosis pigmentovascularis and the so-called eccrine angiomatous hamartoma that consists of proliferations of both eccrine glands and blood vessels. Vascular malformations result from anomalies of embryologic development, and in some of them the abnormalities of the involved vessels are more functional than anatomic, as is the case of nevus anemicus. In contrast, other cutaneous vascular malformations show striking morphologic abnormalities of the vascular structures. These anatomic vascular malformations are subdivided into the following groups: capillary, venous, arterial, lymphatic, and combined anomalies. Spider angioma, capillary aneurysm–venous lake, and telangiectases are not vascular proliferations at all, but dilations of preexisting vessels. In our opinion, most of the lesions described with the generic term of “angiokeratoma” are not authentic vascular neoplasms, but hyperkeratotic malformations of capillaries and venules or acquired telangiectases of preexisting blood vessels of the papillary dermis. Therefore the first group of these “angiokeratomas” are included in the vascular malformations section, and the second group are covered in the section of dilation of preexisting vessels. Lymphangiectases are considered the lymphatic counterpart of angiokeratomas because they result from ectasia of preexisting lymphatic vessels of the papillary dermis. (J Am Acad Dermatol 1997;37:523-49.) Learning objective: At the completion of this learning activity participants should be familiar with vascular abnormalities in terms of classification, clinical characteristics, histopathologic features, differential diagnosis, and treatment. This article provides a comprehensive and updated review of the vascular hamartomas, malformations, and dilatation of preexisting vascular structures.

Options for laser selection in the treatment of cutaneous vascular and pigmented disorders and tattoos

A confusing array of new lasers is now available in the treatment of cutaneous vascular and pigmented disorders and tattoos. This article describes the physiology and clinical usage of currently available cutaneous lasers (excluding the C0 2 laser). Clinical indications for each of these lasers are summarized. Cutaneous vascular malformations such as port wine stains, hemangiomas, and telangiectasia may be treated using argon, neodymium:yttrium-aluminum-garnet (Nd:YAG) krypton, argon-pumped tunable dye, copper vapor, or flash lamp pulsed-dye (585 nm) lasers. Automated scanners have extended the use and safety of continuous-wave lasers. Melanocyte disorders such as epidermal lentigo, cafe au lait spots, age spots, and dermal melanocytosis (nevus of Ota) may be treated by argon, flash lamp pulsed-dye (510 nm), argon-pumped tunable dye, copper vapor, or Q-switched Nd:YAG, ruby, or alexandrite lasers. Professional, amateur, and some traumatic tattoos can be treated with Q-switched ruby, Nd:YAG, and alexandrite lasers, resulting in marked lightening of most colors after a series of treatments.

Inflammatory linear verrucous epidermal naevus with auto-immune thyroiditis: coexistence of two auto-immune epithelial inflammations?

The copper vapour laser has in theory qualities which make it interesting in the treatment of cutaneous vascular malformations. In order to study the clinical effect of the copper vapour laser and to compare its effect with the effect of the argon laser, we treated 38 patients with port-wine stain or facial telangiectasia with the copper vapour laser. Thirty-one patients were evaluated and of these 24 were improved or much improved. Nineteen patients had previously been treated with the argon laser, and in this group the copper vapour laser had better effect than the argon laser in 8 patients and equal effect in 9 patients. In 7 patients side-effects like scarring or hyperpigmentation were noted after treatment with copper vapour laser.

Infantile Hemorrhagic Angiodysplasia

We report a female infant who presented at birth with an unusual syndrome of disseminated cutaneous and gastrointestinal vascular malformations associated with severe thrombocytopenia and chronic gastrointestinal hemorrhage. The infant required extensive blood-product support and expired at 7 months of age. Postmortem examination confirmed the presence of numerous flat vascular lesions, descriptively classified as angiodysplastic, and composed of congeries of dilated capillaries, arterioles, and postcapillary venules. No visceral space-occupying hemangiomas were found. The case is discussed in relation to a spectrum of congenital vascular malformation syndromes including disseminated neonatal hemangiomatosis and hereditary hemorrhagic telangiectasia (HHT). Some pathologic characteristics appear to link it to the latter entity. However, other clinical and pathologic features distinguish it from the reported spectrum of congenital HHT, prompting its essentially descriptive designation.

Preservation of sensation in a cutaneous vascular malformation in lepromatous leprosy.

THE remarkably stereotyped distribution of motor and sensory deficits in lepromatous leprosy has been attributed to a wide variety of factors, including the entrapment of the enlarged nerves, the density of nerve endings in different skin areas and the degree of exposure of involved nerves to chronic trauma. The case reported below indicates that variations in surface temperature may affect the pattern of sensory loss in lepromatous leprosy. The superficial tissues of the body have been observed to bear the brant of the pathologic changes in lepromatous leprosy. Thus, clinically, abnormalities are most frequently noted in the skin, anterior third . . .

Value of cutaneous angiomas in the arteriographic localization of spinal-cord arteriovenous malformations.

Abstract A careful search for segmentally related cutaneous hemangiomas in a series of 28 patients with spinal-cord arteriovenous malformations revealed six with such an association, a much higher prevalence than previously reported. Close scrutiny of the back is required to detect these often minor skin changes. Selective arteriography demonstrated opacification of both the spinal and the cutaneous vascular malformation from the same intercostal or lumbar artery, confirming the similar metameric origin of both lesions. Recognition of the skin lesion in a patient with obscure spinal-cord symptoms is important both as a clue to the etiology of the myelopathy and as an aid in the localization of arterial feeders to the spinal malformation before surgery.