scholarly journals Coexistence of Neonatal Lupus Erythematous and Sturge–Weber Syndrome

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Zahra Nikyar ◽  
Parvaneh Hatami ◽  
Zeinab Aryanian ◽  
Kambiz Kamyab Hesari ◽  
Azadeh Goodarzi ◽  
...  
Keyword(s):  
Sun Exposure ◽  
Rare Condition ◽  
Neonatal Lupus ◽  
Diagnostic Challenge ◽  
Neurologic Involvement ◽  
Capillary Malformation ◽  
First Case ◽  
Weber Syndrome ◽  
Congenital Condition ◽  
Sturge Weber Syndrome

Neonatal lupus erythematous (NLE) is a rare condition presented by lupus dermatitis shortly after birth or later following sun exposure. Sturge–Weber syndrome (SWS) is also an uncommon congenital condition characterized by extensive capillary malformation and ophthalmic and/or neurologic involvement. Here, we describe the first case of coexistence of NLE and SWS which posed a significant diagnostic challenge to clinicians.

scholarly journals Extent of leptomeningeal capillary malformation is associated with severity of epilepsy in Sturge-Weber syndrome

2020 ◽  
Author(s):  
Hidenori Sugano ◽  
Yasushi Iimura ◽  
Ayuko Igarashi ◽  
Mika Nakazawa ◽  
Hiroharu Suzuki ◽  
...  
Keyword(s):  
Capillary Malformation ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

scholarly journals Periodontal Manifestations and Unusual Radiographic Features in a Patient with Sturge-Weber Syndrome: A Case Report

2018 ◽  
Vol 6 (1) ◽  
pp. 28-34
Author(s):  
Mohammad Taghi Chitsazi ◽  
Adileh Shirmohammadi ◽  
Nasrin Rahmanpour ◽  
Monir Moradzadeh Khiyavi
Keyword(s):  
Congenital Glaucoma ◽  
Port Wine ◽  
Capillary Malformation ◽  
Gingival Enlargement ◽  
Weber Syndrome ◽  
Vascular Proliferation ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
Hands And Feet ◽  
Developmental Condition

The Sturge-Weber syndrome or encephalotrigeminal angiomatosis is a rare neurological and congenital disorder with a frequency of 1 in 50,000 births. This syndrome is a nonhereditary developmental condition and is characterized by the presence of congenital capillary malformation and a hamartomatous vascular proliferation involving the face (port-wine stain or facial birthmark), sometimes skull and the tissues of brain, jaws, oral soft and hard tissues and rarely other body organs. Seizures, mental retardation, and cortical calcification (tram-tracks) and congenital glaucoma may be seen in this syndrome. We report here a 40-year-old female with Sturge-Weber syndrome associated with bilateral cutaneous capillary malformation on her face, neck, hands and feet and also gingival enlargement.

scholarly journals Dyke Davidoff Masson Syndrome – A Case Report

2013 ◽  
Vol 3 (2) ◽  
pp. 106-109
Author(s):  
Md. Abu Taher ◽  
Fattah Ahma ◽  
Md. Anwar Pasha ◽  
Md. Mofazzal Sharif ◽  
Md. Mohit Ul Alam ◽  
...  
Keyword(s):  
Cerebral Hemisphere ◽  
Facial Asymmetry ◽  
Rare Condition ◽  
Clinical History ◽  
Final Diagnosis ◽  
Convulsive Therapy ◽  
Right Cerebral Hemisphere ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Control Seizure

A 10 year old male child reported to paediatric neurology OPD of BIRDEM General Hospital with the complaints of weakness of left side of body, seizure and facial asymmetry for 2 years. He was on regular anti convulsive therapy and failed to control seizure. General examination revealed no significant abnormality neither delayed mile stones of development happened. Neurological examination revealed left sided spastic hemiparesis, brisk tendon reflexes and extensor planter on left side. With detailed history and examination he was diagnosed as a case of infantile seizure and undergone CT scan of brain followed by MRI scan. Both the reports revealed severe atrophy of right cerebral hemisphere, thinning of cortical gyri, widening of sulci and dilatation of right lateral ventricle with ipsilateral midline shift and was concluded as hemiatrophy of right cerebral hemisphere with suspicion of Dyke Davidoff Masson Syndrome (DDMS), Hemimegalencephaly and Sturge-Weber syndrome. Dyke-Davidoff-Masson syndrome (DDMS) is a rare condition characterized by asymmetric cerebral hemispheric growth with unilateral atrophy, ipsilateral compensatory osseous hypertrophy, hyperpneumatization of the paranasal sinuses and mastoid cells, and contralateral paresis. Varying degrees of hemiparesis, hemiplegia, seizures, mental retardation, and facial asymmetry can be associated with DDMS. Considering clinical history and imaging findings, final Diagnosis was Dyke Davidoff Masson Syndrome. Birdem Med J 2013; 3(2): 196-109 DOI: http://dx.doi.org/10.3329/birdem.v3i2.17215

scholarly journals Desafios do tratamento odontológico na síndrome de Sturge-Weber: relato de caso

2020 ◽  
Vol 19 (4) ◽  
pp. 642
Author(s):  
Tila Fortuna ◽  
Caroline Argolo Brito De Oliveira ◽  
Alena Ribeiro Alves Peixoto Medrado ◽  
Viviane Maia Barreto De Oliveira ◽  
Roberta Santos Tunes ◽  
...  
Keyword(s):  
Dental Treatment ◽  
Treatment Plan ◽  
Rare Condition ◽  
Health Maintenance ◽  
Health Team ◽  
Gingival Bleeding ◽  
Weber Syndrome ◽  
Oral Environment ◽  
Sturge Weber Syndrome ◽  
Bad Breath

<p><strong>Introduction</strong>: Sturge-Weber syndrome (SWS) is a rare condition characterized by facial capillary malformation, involves ocular, neurological, and cutaneous alterations. Associated with unilateral characteristic port-wine stains, gingival growth and purple-red coloration. <strong>Aim</strong>: his case aims to report dental treatment challenges in patients with SWS and importance of oral health maintenance in these individuals. <strong>Case report</strong>: a 20-year-old woman with an established diagnosis of SWS, presented bad breath and spontaneous gingival bleeding, with gingival growth and reddish-purple spots spread to labial and alveolar mucosa, tongue, and palate. Conditioning of the patient’s oral environment by supra and subgingival scraping, dental unit extraction was performed. A conservative treatment plan was adopted for management adequacy of oral environment owing to possible complications inherent to the condition. <strong>Conclusion</strong>: it is important to emphasize the importance of dental surgeon’s performance in relation to a multidisciplinary health team, as well as cooperation of patient, to obtain better results from the proposed therapy</p>

Sturge-Weber syndrome Case report

2018 ◽  
Vol 14 (2) ◽  
pp. 51-57
Author(s):  
Joanna Witanowska ◽  
Małgorzata Laskowska ◽  
Małgorzata Zadurska
Keyword(s):  
Case Report ◽  
Oral Cavity ◽  
Neurological Disorders ◽  
Orthodontic Treatment ◽  
Type I ◽  
Cutis Marmorata Telangiectatica Congenita ◽  
Weber Syndrome ◽  
Congenital Condition ◽  
Sturge Weber Syndrome ◽  
The Brain

Sturge-Weber Syndrome (SWS – encephalotrigeminal angiomatosis) is a rare non-hereditary congenital condition. It develops in the early foetal period, at the stage when the embryo head is being formed, as a result of a random somatic mutation of the GNAQ gene due to unknown reasons. The main symptom is the formation of congenital angiomas, especially in the area of capillaries, in the leptomeninges in the brain, and along the course of the trigeminal nerve. Such angiomas may also be present on lips, and in the whole oral cavity: on its bottom, palate and gingivae. It may lead to serious malocclusions and breathing problems. <b>Aim</b>. This article aims to present problems regarding orthodontic treatment of patients with Sturge-Weber syndrome, using an example of a 7-year-old female patient.<b> Case report</b>. A 7-year-old girl with all signs of this syndrome, namely glaucoma, epilepsy, neurological disorders, cutis marmorata telangiectatica congenita and after sanitation of the oral cavity, had hygiene procedures and exercises with a vestibul plate introduced. Two attempts to take impressions for appliances failed due to patient’s anxiety reactions. <b>Conclusions</b>. This case shows difficulties that may be faced by an orthodontist when treating patients with Sturge-Weber syndrome. In this case, namely type I in the Roach scale, due to neurological disorders it was necessary to take special precautions in relation to procedures introduced and to avoid stress in a patient. It makes orthodontic treatment difficult.<b> (Witanowska J, Laskowska M, Zadurska M. Sturge-Weber syndrome. Case report. Orthod Forum 2018; 14: 143-9)</b>.

scholarly journals Sturge-Weber Syndrome Associated with Monolateral Ocular Melanocytosis, Iris Mammillations, and Diffuse Choroidal Haemangioma

2017 ◽  
Vol 8 (2) ◽  
pp. 375-384 ◽  
Author(s):  
Andrea Maria Plateroti ◽  
Rocco Plateroti ◽  
Roberto Mollo ◽  
Aloisa Librando ◽  
Maria Teresa Contestabile ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Anterior Segment ◽  
Rare Condition ◽  
Retinal Nerve Fibre Layer ◽  
Enhanced Depth Imaging ◽  
Optical Coherence ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
The Right ◽  
Ocular Melanocytosis

We present the case of a 12-year-old boy with Sturge-Weber syndrome and ocular melanocytosis who presented with bilateral naevus flammeus of the face and hyperpigmentation of the right iris associated with ipsilateral iris mammillations. The patient had glaucoma and a diffuse choroidal haemangioma of the right eye. Optical coherence tomography of the anterior segment confirmed iris hyper-pigmentation and did not show abnormalities of the chamber angle structures. B-scan ultrasonography and enhanced depth imaging optical coherence tomography were performed and showed a marked difference in thickness and reflectance between the right and left choroid. Visual field examinations with perimetry showed early defects in the right eye. Peripapillary optical coherence imaging showed borderline values of retinal nerve fibre layer thickness reduction in the right eye. Sturge-Weber syndrome associated with ocular melanocytosis and iris mammillations is an extremely rare condition. This paper highlights the role of multi-imaging methods in the enhanced evaluation of rare diseases towards choosing the most appropriate management strategies and improving the follow-up of patients over time.

Outcome of infants with unilateral Sturge-Weber syndrome and early onset seizures

2000 ◽  
Vol 42 (11) ◽  
pp. 756-759 ◽  
Author(s):  
Uri Kramer ◽  
Esther Kahana ◽  
Zamir Shorer ◽  
Bruria Ben-Zeev
Keyword(s):  
Early Onset ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome
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