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Paulus Yannis M - - 2009
PURPOSE: Report the resolution of a persistent exudative retinal detachment in a patient with Sturge-Weber syndrome following intravitreal pegaptanib injection. DESIGN: Case report. METHODS: A 13-year-old male with Sturge-Weber syndrome presented with a choroidal hemangioma associated with an exudative retinal detachment that failed to resolve 6 months after external beam ...
Fakir Ebrahim - - 2009
Klippel-Trenaunay-Weber syndrome is characterized by a triad of features, namely, vascular nevi, venous varicosities, and hyperplasia of hard and soft tissues in the affected area. Involvement of the orofacial region is uncommon, but nevertheless, a wide range of orofacial abnormalities may necessitate specialized dental and anesthesia management. We have documented ...
Abdul-Rahman N R - - 2009
The Klippel-Trenaunay syndrome is a combination of venous and capillary malformations associated with soft tissue and/or bony limb hypertrophy, with or without lymphatic malformations. Although persistent foetal veins are rare, the persistence of the lateral marginal vein is a common association in this syndrome. It results in venous hypertension, which ...
Vurucu S - - 2009
Klippel-Trenaunay syndrome (KTS) is a rare disorder characterised by congenital vascular hamartomas, limb hypertrophy, lymphangiomas and atresia of lymph vessels with non-pitting oedema. A 6-year-old girl with KTS was referred to our hospital for evaluation of intractable seizures. In addition to findings consistent with KTS, we also found hemimegalencephaly, retroperitoneal ...
Değerliyurt Aydan - - 2009
Sturge-Weber syndrome without facial nevus is rare. Twenty-four cases were previously reported. Although hypomelanosis of Ito is a relatively common disorder, there was only one previous case in association with Sturge-Weber syndrome. We describe an 11-year-old boy with Sturge-Weber syndrome without facial nevus, coexistent with hypomelanosis of Ito.
Gober-Wilcox Julie K - - 2009
Klippel-Trenaunay syndrome (KTS) is a rare disorder involving a triad of cutaneous capillary malformations (port-wine stain), varicose veins or venous malformations, and bony or soft tissue hyperplasia of an extremity. It is one of many heterogeneous disorders known as overgrowth syndromes that are characterized by either generalized or localized somatic ...
Zhou Qin - - 2009
Sturge-Weber syndrome (SWS) is a rare, congenital neurocutaneous disorder with a leptomeningeal, facial trigeminal nerve dominative area and choroidal angioma. The cause of this disease remains unclear. Due to the occurrence of localized abnormality of blood vessel formation, somatic mutation has been put forward. Studies have indicated that fibronectin gene ...
Khawaja Omar M - - 2009
Klippel-Feil syndrome is a visually arresting deformity wherein severe restriction of cervical motion predicts a difficult airway. Even minor distraction of the neck risks cervical spine or neurologic injury, so regional techniques, awake fiberoptic intubation, or awake tracheostomy are recommended anesthetic approaches. We present a case of aortic dissection in ...
Agrawal Amit - - 2009
Klippel-Feil syndrome (KFS) is defined as congenital fusion of two or more cervical vertebrae and patients with KFS are frequently asymptomatic. However, these patients are especially prone to cervical cord injury after a minor fall or a major traumatic episode. We report an unusual case of KFS where the patient ...
Onesti Maria Giuseppina - - 2009
Sturge-Weber syndrome (also called encephalofacial or encephalotrigeminal angiomatosis) is a rare neurocutaneous syndrome characterized by facial port-wine stains in the trigeminal nerve distribution area. An innovative treatment based on surgical and laser techniques of a patient affected with Sturge-Weber syndrome is reported. The aesthetic and functional results were satisfactory.
Gandhi Manju - - 2009
The Sturge-Weber Syndrome (SWS) is a neurocutaneous disorder characterized by leptomeningeal and facial angiomas, neurologic and ocular manifestations. We report a case of oophorectomy for ovarian dermoid in a 14 year-old girl who was a diagnosed case of Sturge-Weber Syndrome. General anaesthesia was given for the procedure. The perioperative anaesthestic ...
Charles V - - 2009
Lyme disease is a multisystemic disorder caused by an epizootic organism of the spirochete group, called Borrelia burgdorferi, which is transmitted to humans by ticks of the genus Ixodes. Lyme neuroborreliosis may occur during the early dissemination phase, most often as a painful meningo-radiculitis and very rarely as a radiculo-myelitis, ...
Clarke Raymond A - - 2009
This is the first case description of the association of Klippel-Feil Syndrome (KFS), Tourette Syndrome (TS), Motor Stereotypies, and Obsessive Compulsive Behavior, with chromosome 22q11.2 Duplication Syndrome (22q11DupS). Neuropsychiatric symptoms in persons with 22q11.2 deletion, including obsessive compulsiveness, anxiety, hyperactivity, and one prior case report of TS, have been attributed ...
Al-Holou Wajd N - - 2009
Myelomeningocele is associated with other neurological abnormalities, including hydrocephalus, Chiari II malformations, syringomyelia, and secondary tethered cord syndrome. Tethered cord syndrome occurs because of abnormal attachment of the spinal cord to the caudal dural sac, causing cord ischemia. Occasionally, symptoms of progressive neurological deterioration may occur and can significantly affect ...
Toker Serdar - - 2009
Osteopoikilosis is a rare, usually asymptomatic, autosomal dominant bone disorder, which is usually found incidentally on X-ray. Klippel-Feil syndrome is a rare disorder characterized by the congenital fusion of any two of the seven cervical (neck) vertebrae. It is caused by a failure in the normal segmentation or division of ...
Grant Lorna W - - 2008
Sturge-Weber Syndrome is a nonheritable congenital syndrome characterized by a "port-wine stain" on the face and angioma of the meninges. Ocular findings include diffuse choroidal hemangioma, retinal detachment, and various types of glaucoma. Management of diffuse choroidal hemangioma is aimed at preserving the affected eye and preventing glaucoma. In the ...
Gambrelle J - - 2008
PURPOSE: To report a case of uveal effusion with subtotal exudative retinal detachment induced by topical administration of travoprost. CASE REPORT: A 20-year-old woman with a medical history of right-sided Sturge-Weber-Krabbe syndrome and bilateral aphakia secondary to congenital cataract extraction was referred to our department for retinal detachment associated with ...
Lozano Francisco Javier Rodríguez - - 2008
We present a case of gingival overgrowth, with aesthetic repercussions, associated with Rendu-Osler-Weber syndrome. Treatment consisted of electric gingivectomy. We noted that it is necessary to carefully monitor hemorrhaging during surgery as well as to provide a correct differential diagnosis to distinguish this syndrome from other illnesses that involve gingival ...
Willis-Owen C A - - 2008
Klippel-Trenaunay syndrome patients often suffer degenerative joint disease at an early age. Performing arthroplasty in these patients is particularly difficult for a number of reasons. In this case report, we describe the second reported case of total hip replacement in Klippel-Trenaunay syndrome, using novel techniques to surmount the problems faced ...
Valverde Ricardo - - 2008
Alpha-1-antitrypsin deficiency is a congenital error of metabolism linked to pulmonary (emphysema) and liver (cirrhosis) disease. Since 1972, panniculitis has been associated with this deficiency, initially related to Weber-Christian syndrome and finally as a differentiated entity. Clinical manifestations typically consist of wide nodular lesions on the trunk and proximal extremities ...
Dirik Eray - - 2008
Vertebral artery dissection as a cause of stroke is rarely reported in children. The association between vertebral artery dissection and Klippel-Feil syndrome is also very rare. We report on a case of vertebral artery dissection with posterior circulation involvement in a child with Klippel-Feil syndrome after a hard physical-training lesson. ...
Smith Pearl M - - 2009
Sturge-Weber syndrome is a rare neurocutaneous disorder that typically presents with angiomas involving the face, ocular choroid and ipsilateral supratentorial leptomeninges. Posterior fossa involvement is extremely rare. We present two patients with simultaneous supra- and infratentorial involvement. Magnetic resonance imaging (MRI) and digital subtracted angiography (DSA) findings are discussed.
Akcali C - - 2008
The case of a 14-year-old girl suffering from Klippel-Trenaunay syndrome associated with multiple port-wine stain type vascular anomalies and varicose veins involving upper limbs is described. Finger deformations are common in Klippel-Trenaunay syndrome. In our patient, striking hypertrophy of soft tissues and overgrowth of bones were observed on both forearms ...
Kanaheswari Y - - 2008
Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized predominantly by cutaneous vascular malformations and pigmentary naevi. The most frequently reported form, Type II b, is associated with systemic involvement. Sturge-Weber Syndrome (SWS) with concomitant glaucoma, Klippel-Trenaunay Syndrome (KTS) and naevus of Ota have been frequently described, but there have ...
Krishnamoorthy Thamburaj; Penn State ...
A 10-year-old girl with features of Klippel Trenaunay Syndrome developed a large left frontoparietal subdural hemorrhage. CT angiography and cerebral angiography identified prominent subependymal veins and deep venous system predominantly in the left cerebral hemisphere in association with a dilated left vein of Labbe and hypoplastic superior sagittal sinus. No ...
Curatolo P - - 2009
MRI appearance of Sturge-Weber Syndrome (SWS) in patients with Tuberous Sclerosis (TSC) has been rarely reported. We describe a new patient with confirmed diagnosis of TSC and MRI appearance of SWS and review the pertinent literature. We discuss these findings on the basis of the new classifications of brain malformations, ...
Comi Anne M - - 2008
Sturge-Weber syndrome is a rare disorder manifesting with a facial port-wine birthmark and a vascular malformation of the brain. Infants and children present with seizures and stroke-like episodes with focal neurologic deficits. Our previous investigations revealed that growth-hormone deficiency occurs with an increased prevalence in Sturge-Weber syndrome, presumably secondary to ...
Vlasveld L T - - 2008
BACKGROUND: Myomatous erythrocytosis syndrome is defined by the combination of erythrocytosis, myomatous uterus and persistent restoration of normal haematological values after hysterectomy. A pathogenic role of erythropoietin is suggested by clinical and experimental data. CASE REPORT: A postmenopausal patient is described with the classical clinical signs of the myomatous erythrocytosis ...
Gajinov Z - - 2008
A patient with extensive facial and intracranial features of Sturge-Weber syndrome was found to have a persistent primitive hepatic venous plexus, characterized by three additional vessels arising from the inferior vena cava, circulating between liver segments, which formed a common trunk in the supra-hepatic region that flowed into the right ...
Iizuka Y - - 2008
Summary: We describe a 24-year-old woman who presented with twice previously unverified subarachnoid hemorrhages from the conus medullaris spinal arteriovenous malformation with Parkes-Weber-syndrome. Spinal MRI examination is considered to be necessary for the diagnosis of Klippel-Trenaunay-Weber syndrome. For diagnosis of the spinal cord arteriovenous malformation, it is indispensable to search ...
Elsayes Khaled M - - 2008
The purpose of this review is to describe the role of imaging and associated findings in the diagnosis of blue rubber bleb nevus syndrome, Proteus syndrome, Klippel-Trénaunay syndrome, and Kasabach-Merritt syndrome. Blue rubber bleb nevus, Proteus, Klippel-Trénaunay, and Kasabach-Merritt syndromes are a diverse group of vascular malformation and hemangiomatosis syndromes. ...
Hennedige Anusha A - - 2008
Laser therapy is the optimal approach for treating port-wine stains, but whether it is effective for patients with facial dermatomal port-wine stains and Sturge-Weber syndrome is undetermined. This project aimed to verify the incidence of Sturge-Weber syndrome and glaucoma within the population, compare the response in syndromic and nonsyndromic patients ...
Menezes Arnold H - - 2008
INTRODUCTION: The craniocervical junction is a vital component in understanding the function of the human central nervous system. It is the threshold for major pathways affecting both brain and spinal cord function, and these structures are intricately housed in a network of bone, ligaments, and soft tissues. Abnormal development of ...
Bhansali Rahul S - - 2008
BACKGROUND: Sturge-Weber syndrome (encephalotrigeminal angiomatosis) is a rather uncommon congenital condition that is characterized by a combination of venous angioma of leptomeninges over the cerebral cortex and ipsilateral angiomatous lesions of the face and sometimes of the skull, jaws, and oral soft tissues. It is commonly referred to as Sturge-Weber ...
Mathews M S - - 2008
Klippel-Trenaunay syndrome (KTS) a rare mesodermal phakomatosis consisting of capillary malformations, varicose veins, and limb hypertrophy, often associated with vascular malformations and benign tumours. A 33-year-old male presented with headaches secondary to a previously diagnosed intracranial tumour. He had a large blanching port-wine stain and hypertrophy of the left side ...
Oduber Charlène E U - - 2008
Klippel-Trenaunay syndrome (KTS) is a congenital malformation syndrome involving blood and lymph vessels and disturbed growth of bone and soft tissues. The clinical presentation can be extremely variable. An extensive literature search showed that various authors used many different diagnostic criteria. Uniform diagnostic criteria are an absolute prerequisite for successful ...
O'Donnel Daniel P - - 2008
A healthy 19-year-old man presented with signs and symptoms of central cord syndrome after a moderate fall. He had congenital fusion of multiple cervical vertebrae consistent with Klippel-Feil syndrome. This disorder, although rare, is important for emergency medicine physicians to recognize as a risk factor for cervical cord syndromes.
Ahmed Shakeel - - 2008
A girl with Klippel-Trenaunay-Weber syndrome with partial motor seizures is reported. She had hemimegalencephaly and band heterotopia on MRI of the brain.
Lane Tameka K - - 2008
We present a 14-year-old girl with a nine-year history of progressive subcutaneous atrophy on the right side of the face, which is consistent with a diagnosis of Parry-Romberg syndrome. The onset of the atrophy was heralded by the development of a morpheaform plaque on the right temple. This case highlights ...
Albertini Giorgio - - 2008
PURPOSE: The Klippel-Trenaunay-Weber syndrome is a rare, congenital disorder characterized by benign excessive growth of blood vessels on the skin. Little is still known about its cognitive development and adaptive behaviour functioning. CASE STUDY: This case study describes the clinical history of a female child suffering from this rare syndrome ...
Lowman Erik - - 2008
OBJECTIVE: To describe a case of Klippel-Trénaunay syndrome in an adult patient with symmetric macromelia suggestive of an acromegaloid syndrome. METHODS: We report clinical and laboratory data that were extracted from the medical records of the study patient. We also survey the relevant reports identified through a MEDLINE search of ...
Yazaki Masahide - - 2008
We report a unique male patient presenting with portosystemic encephalopathy (PSE) due to intrahepatic portohepatic venous (PHV) shunts. He was diagnosed as having Klippel-Trénaunay-Weber syndrome (KTWS) based on the findings of a hemitruncal port-wine stain with subcutaneous arteriovenous fistulae and varicose veins in the legs. However, limb-hypertrophy, which is one ...
Parsa Cameron F - - 2008
According to a new, unifying view of the pathogenesis of Sturge-Weber syndrome and related syndromes, signs and symptoms all arise from localized primary venous dysplasia, with effects of venous hypertension transmitted to nearby areas via persisting communicating venous passageways and compensatory collateral venous channels. Port-wine stains result from a vascular ...
Rahman Mm - - 2008
Sturge-Weber Syndrome (SWS) occurs sporadically with a frequency of approximately 1 in 50,000. SWS is a mesodermal phakomatosis. Klippel-Trenaunay Weber syndrome (KTWS) is another very rare phakomatosis. Overlap between SWS & KTWS is very rarely encountered. We report a 19 months old boy with features of both SWS and KTWS. ...
Matysik-Woźniak Anna - - 2007
The paper presents the coexistence of a nevus flammeus and basal cell carcinoma affecting the left upper lid of 61-year-old farmer with Sturge-Weber syndrome. The occurrence of basal cell carcinoma in nevus flammeus is extremely rare. The etiology of the malignancy in this situation is unknown. It is difficult to ...
Latessa Victoria - - 2007
Klippel-Trenaunay syndrome (KTS) is a congenital developmental disorder characterized by port wine stain, venous abnormalities, soft tissue, and bony deformities of the affected extremity. It is usually diagnosed in early childhood and has many long-term sequelae. Patients not only have physical health problems but also must learn to cope with ...
Biglioli Federico - - 2007
INTRODUCTION: Total parotidectomy has aesthetic and functional sequelae, including Frey' syndrome and soft tissue depression of the region. The aim of repair is to reduce these problems, importing tissue into the surgical field to fill the region and to avoid Frey's syndrome. The soft tissues to use are those which ...
Leung Clarence H S - - 2007
Technological advances have made more options available for surgical intervention in spinal disorders. From spinal fusion to artificial disc implantation, these advancements have brought great benefits, allowing preservation of spinal motion and flexibility after intervertebral discectomy. Yet the use of artificial discs as a treatment for congenital spinal disorders has ...
Tovar-Moll Fernanda - - 2007
Experimental studies in nonhuman primates have questioned the selectivity of pyramidal tract damage in giving rise to the classical pyramidal syndrome in humans, characterized by permanent spastic hemiplegia (PSH). According to this view, concomitant injury of extrapyramidal pathways is necessary for the development of both hemiplegia and spasticity. In this ...
Fernandes Teresa - - 2007
Klippel-Feil syndrome, or synostosis of the cervical spine, is the result of an abnormal division of somites during embryonic development. This report analyses an adult male (exhumed from a Portuguese graveyard dating from the 13th to the 15th century) with malformations in the cranium and vertebral column. Besides the lesions ...
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