A two-stage antiglaucoma operation was planned for the left eye of an 11-year-old boy with bilateral Sturge-Weber syndrome to avoid intraoperative and postoperative choroidal and retinal effusion as with previous trabeculectomy. The Ex-Press miniature glaucoma implant was placed 10 days before trabeculectomy with mitomycin C. There were no complications with ...

Parkes Weber syndrome is a disorder characterized by cutaneous blush, arteriovenous fistula, and overgrowth of the affected limb. It has been differentiated from Klippel-Trenaunay syndrome on the basis of the presence of arteriovenous fistula that are always absent in the latter. We report a case of Parkes Weber syndrome with ...

This report describes the case of a 21-year-old man with a history of Sturge-Weber syndrome and varicose veins. Examination of the left lower limb revealed the presence of significant port-wine staining, and hypertrophy. Duplex examination revealed a severely hypoplastic deep venous system with a tortuous, dilated, long saphenous vein, which ...

Sturge-Weber syndrome is a rare neurocutaneous disorder characterized by a facial nevus flammeus and extensive angiomatous changes involving the leptomeninges, the dura, and vessels of the gray and white matter. Oculodermal melanocytosis is characterized by hyperpigmentation of the facial skin in the distribution of the ophthalmic, maxillary, and occasionally mandibular ...

The concept of segmental vascular syndromes with different, seemingly unrelated, diseases is based on the embryology of the neural crest and the mesoderm migration of cells that share the same metameric origin. Migrating patterns of these cells link the brain, the cranial bones, and the face on the same side. ...

The authors describe a girl with Costello syndrome who showed cerebral palsy and neurosensorial deafness. Brain computer tomography and magnetic resonance findings were normal. Multivoxel proton magnetic resonance spectroscopy showed a lowering of the peak of choline with a reduced choline/creatine ratio at the level of the centrum semiovale. These ...

Klippel-Trénaunay-Weber syndrome (KTWS) is a rare congenital disease characterized by cutaneous hemangiomata, venous varicosities and osseous soft tissue hypertrophy of the affected limb. We report a case of a patient with KTWS who had developed severe chronic, non-healing cutaneous ulcers resulting from several angiography procedures with embolization by various agents. ...

BACKGROUND: Mirror syndrome is associated with both nonimmune and immune hydrops fetalis. The clinical manifestations are quite varied, and the pathophysiology is poorly understood. We describe a case of mirror syndrome associated with afetus that had a rapidly growing sacrococcygeal teratoma (SCT) without overt hydrops. CASE: At 30 weeks' gestational ...

Avellis syndrome is a rare form of alternating hemiparesis that is usually because of atherosclerosis. We report a 67-year-old man who developed paresthesiae of the left arm, dysphagia and dysphonia. The clinical picture, magnetic resonance imaging and cerebrospinal fluid findings were consistent with Avellis syndrome caused by brain stem arteritis ...

BACKGROUND: Klippel-Trenaunay syndrome is defined by a coexistence of nevus flammeus and overgrowth of one or more limbs. Remarkably, however, deficient growth of an affected limb may likewise be noted. OBSERVATIONS: We collected from the literature a number of cases of Klippel-Trenaunay syndrome associated with deficient growth such as shortening ...

BACKGROUND AND PURPOSE: Head and neck infantile hemangiomas are common lesions that are rarely observed in an intracranial location. We report 4 patients with orbital infantile hemangiomas and ipsilateral enhancing intracranial lesions, presumed to be infantile hemangiomas. METHODS: Imaging studies and medical records of 4 infants with orbital hemangiomas and ...

Sturge-Weber syndrome (SWS) is defined by the association of a facial capillary malformation (port-wine stain), with a vascular malformation of the eye, and/or vascular malformation of the brain (leptomeningeal angioma). Variants exist where only one of these three structures is involved with the vascular malformation. SWS occurs sporadically and is ...

A 48-year-old slender woman presented with a rare case of spontaneous intracranial hypotension syndrome manifesting as patulous Eustachian tube. The patient complained of sudden onset of ear fullness and nasal voice as well as typical orthostatic headache. Patulous Eustachian tube was identified by observation of synchronous movement of the tympanic ...

Klippel-Trénaunay syndrome is a congenital malformation characterized by the triad of flat red hemangiomas, soft tissue and bony hypertrophy and varicose veins. In addition, venous malformations or hemangiomas may also be found in the lung, gastrointestinal tract, liver, kidney and bladder, which may result in recurrent hemor-rhage and compromise the ...

Holmes tremor has a characteristic rest, intention, and postural component. The syndrome arises as a consequence of a lesion in the upper brainstem and cerebral peduncles, which, it is postulated, interrupts the cerebello-rubrothalamic pathway. Ataxia, ophthalmoplegia, and bradykinesia are associated features. We present a case of Holmes tremor secondary to ...

Parry-Romberg syndrome is a rare pathologic process, characterized by progressive hemifacial atrophy. A case of Parry-Romberg syndrome with ocular involvement is reported. A 27-year-old male patient with Parry-Romberg syndrome was interdisciplinary investigated and treated. For reconstruction of hemifacial soft tissues a free vascular parascapular graft was performed. Opthalmological findings included ...

Eisenmenger's syndrome occurs when a large congenital or surgically created shunt between the left and right sides of the heart causes an increase in pulmonary vascular resistance that equals or surpasses systemic resistance, resulting in a reversal of the shunt from a left-to-right shunt to a right-to-left or bi-directional shunt. ...

This vascular review is organized under the following headings: vasculogenesis and angiogenesis; vascular endothelial growth factors, their receptors, TIE receptors, and angiopoietins; other factors in blood vessel formation; parallel patterning in blood vessels and nerves; physiological and pathological neovascularization; the role of VEGF receptors in metastasis; anti-angiogenic therapy for tumors; ...

Phacomatosis pigmentokeratotica (PPK) represents a specific "twin nevus" syndrome in which a speckled lentiginous nevus (SLN) is associated with an organoid nevus with sebaceous differentiation. A boy with a large nevus sebaceus on the left face and upper part of the trunk, a giant segmental SLN extending from the abdomen ...

Klippel-Trenaunay syndrome is characterized by a triad of varicose veins, cutaneous capillary malformation, and hypertrophy of bone and soft tissue. Appropriate evaluation and treatment of children displaying features of the disease may minimize morbidity. The clinical appearance, etiology, genetics, diagnostics, and treatment of Klippel-Trenaunay syndrome are herein explored.

OBJECTIVE: Hemihyperplasia is a heterogenous group of disorders characterized by asymmetric limb growth. There is considerable confusion regarding their classification and ascertainment into various syndromes. We tried to look into the various aspects of hemihyperplasia syndromes. METHODS: Records of 17 consecutive cases of hemihyperplasia were reviewed and were ascertained into ...

Sturge-Weber syndrome (SWS), Klippel-Trenaunay syndrome (KTS), and Parkes-Weber syndrome (PWS) represent a wide range of congenital vascular abnormality syndromes. Although many of the diagnostic criteria overlap for these syndromes, there are important differences among these diseases that carry important prognostic and therapeutic implications. This review attempts to clearly distinguish among ...

In comparison with the lateral (Wallenberg), medial (Dejerine) and hemimedullary (Reinhold) medulla oblongata syndromes, the Babinski-Nageotte and Cestan-Chenais syndromes are much less familiar cerebrovascular disorders. While the Babinski-Nageotte syndrome is usually confused with the hemimedullary syndrome, reports of the extremely rare Cestan-Chenais syndrome are missing from the modern neurological literature. ...

A 35-year-old woman with Klippel-Trénaunay syndrome developed a popliteal artery aneurysm in the affected extremity. The aneurysm was successfully treated by aneurysmectomy and bypass grafting with autologous saphenous vein. In Klippel-Trénaunay syndrome, angiodysplasia in the venous system is common. However, reports of an arterial aneurysm in a patient with Klippel-Trénaunay ...

A middle-aged woman was admitted to hospital after a motor vehicle accident. A large acute intracranial haematoma was initially thought to be traumatic in origin, but its unusual position and the lack of external features of head trauma prompted investigation for other causes of intracerebral haemorrhage. Moyamoya intracranial vascular abnormalities ...

Extracellular matrix mineralization or calcification occurs in many pathologic conditions, including atherosclerosis, medial wall calcification, diffuse idiopathic skeletal hyperostosis, and chondrocalcinosis. Vascular wall calcification is the most common and involves two mechanisms: passive calcification resulting from breakdown of the protection system and active calcification resulting from transdifferentiation of mesenchymal cells ...

Sturge-Weber syndrome (SWS) is a rare, sporadic, progressive, congenital syndrome. In its complete trisymptomatic form, SWS is physically characterized by port-wine stains over the trigeminal area, leptomeningeal angiomas usually over the parieto-occipital region, and eye abnormalities. Clinical manifestation for infants with SWS depends on the affected organs, but can include ...

Sturge-Weber syndrome is characterized by a facial port-wine nevus, leptomeningeal angiomatosis, and glaucoma; it is commonly complicated by epilepsy and hemiparesis. We present a patient with a head and neck port-wine nevus, glaucoma, abnormalities of the intracranial deep veins, and untreated communicating hydrocephalus. The patient lacks any radiologic or clinical ...

Klippel Trenauney syndrome is a rare congenital abnormality that includes extremity hypertrophy. We report an unusual case of breast asymmetry due to unilateral chest and limb hypertrophy and describe successful unilateral breast reduction. We highlight the potential problems of surgery within this group and a management approach to minimise complications.

Sturge-Weber syndrome is a neurocutaneous syndrome that manifests with vascular malformations involving the brain, eye, and skin. We report the case of an elderly patient suffering from Sturge-Weber syndrome who presented with episodic "angry" slapping symptoms to a psychiatric facility. A detailed history, physical and mental-state examination, and elaborate neuropsychological, ...

Klippel-Trenaunay syndrome is a rare congenital disorder characterized by combined vascular malformations, varicosities, and limb enlargement. A child case of Klippel-Trenaunay syndrome with a large cervicomediastinal cystic lymphatic malformation and a vascular mass on the chest wall has been presented to demonstrate combined use of conservative and surgical approaches in ...

The Klippel-Trenaunay Syndrome (KTS) is defined as a triad of cutaneous capillary malformations, venous varicosities, bone and soft tissue hemi-hypertrophy. The urinary tract is involved in up to 10%. We report the clinical presentation and surgical management of a 9-year-old boy with extensive lympho-venous malformations of the bladder which led ...

CASE REPORT: We document a previously unreported association of Vogt-Koyanagi-Harada (VKH) syndrome with intracranial meningioma. A female patient with diminished vision, exudative retinal detachment, and headache was diagnosed with VKH syndrome, more precisely a Harada form of disease with intracranial meningioma, on the basis of exudative retinal detachment, typical fundus ...

Sturge-Weber syndrome can be associated with facial port-wine stains and intracranial calcification, and concurrent Klippel-Trenaunay-Weber syndrome has been reported. Klippel-Trenaunay-Weber syndrome is a rare congenital mesodermal phakomatosis characterized by cutaneous hemangiomas, venous varicosities and soft tissue or bone hypertrophy of the affected extremities. This report is presented a rare case ...

A patient with Klippel-Feil syndrome reported with a pneumatocyst of the proximal epiphysis of a right cervical rib. There was no additional skeletal abnormality except for fusion of the C3 and C4 vertebral bodies. To the best of our knowledge, this is the first reported instance of this particular association.

The patient presented here has extensive hemangiomata plana, especially on the right forehead, right upper eyelid, and right leg, as well as right leg hypertrophy and macrocephaly. Cerebral magnetic resonance imaging (MRI) showed abnormalities in only one occipital lobe consisting of focal cortical atrophy, leptomeningeal enhancement, and ipsilateral choroid plexus ...

A 9-year-old cyanosed child suffering from Osler-Weber-Rendu syndrome with bilateral pulmonary arteriovenous malformations (PAVMs) was posted for cerebral angiography under general anaesthesia. Careful preanaesthetic evaluation led to the diagnosis of coexisting congenital methaemoglobinaemia. There is no previous report of Osler-Weber-Rendu syndrome coexisting with congenital methaemoglobinaemia. This report emphasizes that a ...

We present here the case of a 12-year-old boy who had Klippel-Feil syndrome with renal, cardiac and multiple skeletal anomalies, and we show the relevent three-dimensional computed tomography images. Our patient had a triple renal pelvis, mitral valve prolapsus, multiple cervical vertebrae fusions, cervical ribs, hypoplasia of the right thumb, ...

Klippel-Trenaunay syndrome (KTS) is a rare congenital anomaly characterized by unilateral limb overgrowth, venous varicosities, and capillary malformations (port wine stains) of the affected limb or limbs. Large venous malformations such as those observed in KTS are rare, and many physicians are unfamiliar with the potential complications, which include hypercoagulability, ...

In two patients, one with Klippel-Feil syndrome and one with fibrous dysplasia of the maxilla, no part of the larynx could be visualized by direct laryngoscopy. In both cases, the use of an intubation device equipped with a charge-coupled device camera, the endotracheal intubation device (EID), allowed smooth tracheal intubation.

Cortical lesion might elicit restricted acral sensory deficit but a disparity of topographies in cheiro-oral-pedal syndrome is very rare. We report the first case of cheiro-oral-pedal syndrome due to a contralateral parasagittal hemangioma involving the supplement sensory area in parietal lobe. This unusual link between neuroanatomy and neurological feature is ...

A girl (aged 8.5 years) with Klippel-Trénaunay-Weber syndrome received orthodontic treatment that included rapid palatal expansion. The patient had early and complete intraoral dental eruption with a posterior crossbite and an expressive transverse atresic maxilla. No carious lesions were found, but hemangioma-like changes of the mucosa were observed. A Haas ...

Narrow duplicated internal auditory canal (IAC) is a rare malformation of the temporal bone that is associated with ipsilateral congenital sensorineural hearing loss. This may be an isolated finding or a part of a syndrome. Radiological examination should demonstrate aplasia or hypoplasia of the neural components of the narrow IAC, ...

Sturge-Weber syndrome is associated with leptomeningeal angioma, trigeminal port-wine stain, epilepsy, and glaucoma. Clinically, many patients with Sturge-Weber syndrome are observed to have both seizures and headaches, but this has never been described in the literature. A questionnaire was mailed to 190 patients with reported comorbid epilepsy and headache as ...

An 83-year-old woman was admitted for investigation of sudden loss of vision. Extensive plum-purple papules and nodules involved her skin and lips but not mucosae. A cutaneous biopsy demonstrated irregular vascular cavernous channels in the dermis and subcutis; deeper vessels displayed smooth muscle. Blue rubber bleb naevus syndrome was diagnosed. ...

BACKGROUND: The term 'glomeruloid haemangioma' was coined by Chan et al. for a histologically distinctive cutaneous haemangioma, which they considered a specific cutaneous marker for POEMS syndrome. Glomeruloid haemangiomas appear to be specific to POEMS syndrome, because they have not been reported in patients without this syndrome. METHODS: We report ...

Sturge-Weber syndrome presents with vascular malformations of the brain, skin, and eye. Fibronectin has potent effects on angiogenesis, vessel remodeling, and vessel innervation density. To determine fibronectin expression in the blood vessels of Sturge-Weber syndrome brain and skin tissue and to quantify the density and circumference of Sturge-Weber syndrome blood ...

Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by triad of vascular nevi, venous varicosities and hyperplasia of soft and hard tissues in the affected area. This syndrome usually affects the extremities but occasionally can manifest in the craniofacial region, including the oral cavity. We report a case of KTS ...

Sturge-Weber syndrome is an uncommon condition characterized by presence of Portwine stains on the face along with ocular disorders, mental retardation, oral involvement and leptomeningeal angiomas. A report of a case with atypical manifestations of this syndrome along with a step-by-step protocol oral rehabilitation of such patients is described.

Pleomorphic xanthoastrocytoma is a rare, cortically based, partially cystic astroglial tumour with a strongly enhancing solid mural nodule. We report an 11-year-old boy with a pleomorphic xanthoastrocytoma in the contralateral hemisphere to that affected by Sturge-Weber syndrome. This unique case supports the association between Sturge-Weber syndrome and astrocytomas. Patients with ...