1/21. Naevus varicosus osteohypertrophicus. An early diagnostic approach.Naevus varicosus osteohypertrophicus (synonym Klippel-Trenaunay Syndrome KTS) is relatively rare circumscribed, usually quadrant-related gigantism with vascular hyperplasia or malformations based on the embryonic development. The authors observed an 18- and a 30-year-old female with a triad of symptoms: cutaneous nevus flammeus, varicose and dilated veins, and bony and soft tissue hypertrophy of the low limb. The second patient also had two venous ulcers as a dominant clinical feature--a rare manifestation of Klippel-Trenaunay syndrome. A diagnosis of Klippel-Trenaunay syndrome was made by clinical observations, laboratory findings, dermoscan, radiological examination of the bones of the limb, Doppler ultrasonography, photopletismography and venoscan. A bone isotope scan was also done to the first patient. Making an early diagnosis of this sporadic congenital disease with unknown aetiology is important in order to be able to provide early prophylactic and therapeutic measures. Klippel and Trenaunay in 1900 were the first to describe a patient with the simultaneous appearance of osteohypertrophy, hemangiomas and varicose veins involving one extremity [1]. In 1907 Parkes and Weber reported a similar syndrome--they described a patient who had dilated and pulsatile arteries in the affected region including the presence of arterio-venous communications. In 1918 they used the compromise term "haemangiectatic hypertrophy" to embrace all conditions which were associated with congenital vascular malformations including A-V anastomoses associated with bone and soft tissue hypertrophy. Most authors are agreed that Klippel-Trenaunay syndrome and the syndrome of multiple congenital arterio-venous fistulae are two separate features of the Parkes Weber hypertrophy. KTS is manifesting with a triad of symptoms: cutaneous vascular nevus (more frequently nevus flammeus type), superficial venous varicosities and hypertrophy of the affected limb. Usually one quadrant of the body is involved: quite often a leg, an arm, lateral side of the trunk, very rarely the face. More than one quadrant and bilateral involvement are rarely affected. Naevus flammeus appeared at birth. It is extremely variable both in extent and in color--the latter ranging from pale pink to deep purple. Veinous varicosities appear in childhood and adolescence. They are painful and may be complicated by superficial or deep venous thrombosis and rarely, ulceration. hypertrophy of the affected extremities is due to bone and soft tissue hypertrophy. KTS can be associated with other developmental anomalies such as: polydactyly, syndactyly, oligodactyly [2] macrocephaly, blue nevus, epidermal naevus, venous malformations.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
2/21. Treatment of vascular lesions in pigmented skin with the pulsed dye laser.BACKGROUND: Vascular lesions occurring in African-American patients are often not treated because of the risk of local side effects. OBJECTIVE: The study was to determine the efficacy of the flashlamp-pumped dye (FLPD) laser in the treatment of vascular malformations in African-American patients. methods: All lesions in three patients were treated with the FLPD laser using a 585 nm wavelength, 5 mm spot size, 450 pulse width, and fluences ranging from 6.5 to 8.5j per cm2. CONCLUSION: The FLPD was effective in treating vascular malformations. Transient changes in colour and skin texture occurred at the treated sites.- - - - - - - - - - ranking = 0.66666666666667keywords = malformation (Clic here for more details about this article) |
3/21. Mixed capillary/lymphatic malformation with coexisting port-wine stain: treatment utilizing 3D MRI and CT-guided sclerotherapy.BACKGROUND: Lymphatic malformation, a benign malformation of the skin and the subcutaneous tissues, is divided into two major groups: the classical and the localized forms. Pathologically lymphatic malformation often consists of sequestered lymphatic cisterns with thick muscle walls lying deeply in the subcutaneous tissue. Communicating via dermal lymphatic channels with superficial pseudovesicles, they can vary in size depending on the pressure transmitted by the cisterns beneath. methods: We present a patient with mixed capillary/lymphatic malformation and coexisting port-wine stain since birth. To demonstrate the anatomic extent and the subcutaneous involvement we performed a 3D reconstruction of a magnetic resonance imaging (MRI). The diagnostic procedures, therapeutic possibilities, and complications regarding this rare appearance are reviewed. RESULTS: Good results could be obtained with CO2 laser vaporization of the superficial lesions and computed tomography (CT)-guided transcutaneous sclerotherapy for the deeper cisterns with doxycycline. CONCLUSION: The combination of CO2 laser treatment and sclerotherapy with doxycycline seems to present a treatment option for cutaneous and subcutaneous lymphangioma circumscriptum with rare side effects.- - - - - - - - - - ranking = 2.6666666666667keywords = malformation (Clic here for more details about this article) |
4/21. Partial re-emergence of a port-wine stain following successful treatment with flashlamp-pumped dye laser.We present a 49-year-old patient with a congenital superficial vascular malformation of port-wine stain (PWS) type which has partially re-emerged in the 2.5 years since it was almost completely obliterated with the flashlamp-pumped short pulse pulsed-dye laser (FPDL). This observation is discussed with respect to the possible pathogenesis of PWS, with particular reference to the underlying autonomic nerve supply. The latter would not be expected to respond to FPDL and may explain re-emergence of the lesion.- - - - - - - - - - ranking = 0.33333333333333keywords = malformation (Clic here for more details about this article) |
5/21. Treatment of port wine stains using the pulsed-dye laser at 585 nm with the dynamic cooling device.Port wine stains (PWS) are common vascular malformations appearing more frequently on the face and neck. One of the most prevalent treatment modalities for PWS is the pulsed-dye laser (PDL). The first generation PDL was limited to a 450 microseconds pulse width which was inadequate for the treatment of larger caliber vessels. Second generation PDLs have pulsed widths approximately three times longer (1.5 ms). This, along with the dynamic cooling device (DCD), which allows the safe use of higher fluences, should result in more clinical improvement in the treatment of PWS that were previously resistant or minimally responsive to first generation PDL treatment. We report a case of a 29-year-old white male with extensive PWS on the left face, left neck, and back, which displayed only mild changes with the first generation PDL. However, the use of the 1.5 ms PDL at 585 nm at high fluences in conjunction with the DCD resulted in marked improvement of the patient's PWS.- - - - - - - - - - ranking = 0.33333333333333keywords = malformation (Clic here for more details about this article) |
6/21. hydranencephaly in an infant with vascular malformations.hydranencephaly is a condition in which cerebral hemispheres are absent and reduced to fluid-filled sacs in a normal skull. Numerous causes have been proposed. We report a male infant with hydranencephaly and congenital vascular malformations (port wine stains, generalized nevus flammeus, anomalous retinal vessels, and absent internal carotid flow). magnetic resonance imaging of the brain showed absence of most of the cerebrum except for small portions of the occipital cortex and thalami. magnetic resonance angiography showed flow within the vertebral and basilar arteries without internal carotid intracranial flow above the internal carotid petrous and cavernous portion. This is a report of cutaneous and retinal malformations associated with hydranencephaly. vascular malformations of larger vessels (e.g., webbing of the carotid arteries and an absent internal carotid arterial system) have been observed in other infants with hydranencephaly, and are proposed to lead to brain destruction. The case reported herein supports the role of primary vascular malformations in the development of some cases of hydranencephaly.- - - - - - - - - - ranking = 2.6666666666667keywords = malformation (Clic here for more details about this article) |
7/21. Meyerson phenomenon within a nevus flammeus. The different eczematous reactions within port-wine stains.Only few reports about eczematous reactions overlying nevi flammei exist. All of them were observed in children. The description of an eczematous reaction within a congenital nevus flammeus on the left lower leg of a male adult gives reason to discuss this rare phenomenon. eczema or inflammatory changes within a port-wine stain may mostly be a collision dermatosis with an atopic dermatitis, especially when they arise in children and are localized to the neck and face. When they are observed within a grossly visible vascular malformation, as for example in the Klippel-Trenaunay syndrome, they may have a pathogenesis similar to stasis dermatitis. In rare cases, an eczematous reaction within a nevus flammeus may be the result of genetic mosaicism and is interpreted as a variant of the so-called Meyerson phenomenon.- - - - - - - - - - ranking = 0.33333333333333keywords = malformation (Clic here for more details about this article) |
8/21. Treatment of phacomatosis pigmentovascularis: a combined multiple laser approach.BACKGROUND: Phacomatosis pigmentovascularis (PPV) consists of a capillary malformation with a variety of melanocytic lesions, which involve various regions of the body and are difficult to treat with conventional therapeutic tools. OBJECTIVE: We described two cases with PPV (type IIa and IIb) that were successfully treated with different lasers. methods: The areas involved by both melanocytic lesions and port-wine stains were treated using the Q-switched ruby laser, the Q-switched Alexandrite laser, and the flashlamp pumped pulsed-dye laser. RESULTS: Removal of a good portion of cutaneous and vascular lesions using combined multiple laser approach was achieved after 6 sessions in the first case under general anesthesia and after 31 sessions under local anesthesia in the second case. CONCLUSION: PPV type II can be treated successfully by laser treatment. We prefer to start combined multiple laser treatment of PPV in childhood period under general anesthesia because it will reduce the number of treatment, improve the patient's quality of life, and increase the cost-effectiveness of the treatment.- - - - - - - - - - ranking = 0.33333333333333keywords = malformation (Clic here for more details about this article) |
9/21. Acral arteriovenous tumor developed within a nevus flammeus in a patient with sturge-weber syndrome.The sturge-weber syndrome consists of a large facial nevus flammeus in the distribution of the ophthalmologic division of the trigeminal nerve accompanied by ipsilateral leptomeningeal angiomatosis. Usually, when angiomatous nodules develop in a nevus flammeus of a patient with sturge-weber syndrome they are pyogenic granulomas. We describe an acral arteriovenous tumor developed within the nevus flammeus of a patient with sturge-weber syndrome. To our knowledge, acral arteriovenous tumor has not been previously described in the cutaneous vascular malformation of patients with sturge-weber syndrome. The development of acral arteriovenous tumor within the vascular malformation of a nevus flammeus in this patient with sturge-weber syndrome probably results from a vascular proliferation secondary to underlying arteriovenous shunts.- - - - - - - - - - ranking = 0.66666666666667keywords = malformation (Clic here for more details about this article) |
10/21. Lower lip hypertrophy secondary to port-wine stain: combined surgical and carbon dioxide laser treatment.BACKGROUND: Port-wine stains (PWSs) are capillary malformations that usually show progressive stasis of the vascular channels and cause slow hyperplasia of the soft and hard tissues. When these lesions involve the lower lip, macrocheilia may be developed along the time. Vascular-specific lasers are not adequate to correct these three-dimensional tissue deformities, and surgical management becomes necessary, resulting in considerable morbidity and aesthetic disturbances. OBJECTIVE: To report a case of macrocheilia secondary to PWS treated by combination of surgery and carbon dioxide laser. methods: A 51-year-old man with macrocheilia of the lower lip and severe functional impairment, secondary to long evolution PWS, received treatment with carbon dioxide laser vaporization and minimal surgical correction, resulting in significant improvement of the lower lip hypertrophy, good aesthetic and functional status, and preservation of the muscular function. CONCLUSIONS: Combined carbon dioxide laser and surgery treatment may constitute a valuable alternative in treatment of macrocheilia secondary to PWS because bleeding risk is minimized and improves the preservation of muscular function and aesthetic results in relationship to conventional surgical approaches.- - - - - - - - - - ranking = 0.33333333333333keywords = malformation (Clic here for more details about this article) |
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