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 Sturge-Weber Syndrome with Osteohypertrophy of Maxilla

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Prashant Babaji ◽  
Anju Bansal ◽  
Gopal Krishna Choudhury ◽  
Rashmita Nayak ◽  
Ashok Kodangala Prabhakar ◽  
...  
Keyword(s):  
Skin Disorder ◽  
Unusual Case ◽  
Oral Manifestations ◽  
Port Wine Stain ◽  
Port Wine ◽  
Weber Syndrome ◽  
Ocular Disorders ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
Developmental Condition

Sturge-Weber syndrome is a rare nonhereditary developmental condition with neurological and skin disorder, characterized by presence of port wine stain on the face along with ocular disorders, oral manifestations and leptomeningeal angiomas. Here we present an unusual case of Sturge-Weber syndrome with osseous hypertrophy of maxilla.

scholarly journals Periodontal Management of Sturge-Weber Syndrome

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Butchibabu Kalakonda ◽  
Koppolu Pradeep ◽  
Ashank Mishra ◽  
Krishnanjaneya Reddy ◽  
Tupili Muralikrishna ◽  
...  
Keyword(s):  
Oral Manifestations ◽  
Port Wine Stain ◽  
Scaling And Root Planing ◽  
Port Wine ◽  
Ocular Abnormalities ◽  
Parietal Lobes ◽  
Gingival Enlargement ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Neurocutaneous Syndromes

Sturge-Weber syndrome (SWS) is a sporadic disorder and is frequent among the neurocutaneous syndromes specifically with vascular predominance. This syndrome consists of constellation of clinical features like facial nevus, seizures, hemiparesis, intracranial calcifications, and mental retardation. It is characterized by focal port-wine stain, ocular abnormalities (glaucoma), and choroidal hemangioma and leptomeningeal angioma most often involving occipital and parietal lobes. The present paper reports three cases of SWS with oral manifestations and periodontal management, which included thorough scaling and root planing followed by gingivectomy with scalpel and laser in cases 1 and 3 consecutively to treat the gingival enlargement. However, the treatment in case 2 was deferred as the patient was not a candidate for periodontal surgery.

STURGE WEBER SYNDROME- A RARE CASE REPORT ORIGINAL

2021 ◽  
pp. 4-5
Author(s):  
Om Prakash Singh ◽  
Vikas Kumar ◽  
Pushp Kant Tiwari
Keyword(s):  
Rare Case ◽  
Signs And Symptoms ◽  
Port Wine ◽  
Occipital Area ◽  
Weber Syndrome ◽  
Rare Case Report ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
The Brain ◽  
Embryonic Vessels

Sturge-Weber Syndrome (SWS) is one of the encephalotrigeminal angiomatosis and one of the important segmental vascular neurocutaneous disorders .The occurrence is not very uncommon and the prevalence is 1:20000 to 1:50000.(1) SWS occurs due to the presence of residual embryonic vessels . The various signs and symptoms include capillary malformation in the face a port wine birthmark and similar malformation in the brain involving leptomeniges as well as blood vessels of the eye causing glaucoma. The patient presents with seizures , hemiparesis and stroke like symptoms, headaches and developmental delay.(2) The imaging nding in SWS children is the calcication in the parietal and occipital area of the brain. The EEG ndings in SWS are the attenuation and the excess of slow activities.We are presenting here a rare case of , a fourteen year old male child who presented to our emergency department with status epilepticus. The aim of presenting this case is to share the classical presentation and the challenges involved in the management

scholarly journals Use of Intralesional Bleomycin for Oral Hemangioma of Sturge-Weber Syndrome

2015 ◽  
Vol 6 (1) ◽  
pp. 17-19
Author(s):  
Devayani Shinde ◽  
Yogesh G Dabholkar ◽  
Akanksha A Saberwal ◽  
Haritosh Kamalakar Velankar ◽  
Adip K Shetty
Keyword(s):  
Vascular Malformations ◽  
Accurate Diagnosis ◽  
Port Wine Stain ◽  
Port Wine ◽  
Weber Syndrome ◽  
Neurocutaneous Disorder ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
Venous Angiomas ◽  
Head Neck

ABSTRACT Sturge-Weber syndrome (SWS) or encephalotrigeminal angiomatosis is a rare neurocutaneous disorder characterized with vascular malformations and capillary venous angiomas involving the face, choroid of the eye and leptomeninges with port wine stain, seizures activity and mental retardation. In this paper we report a case of a patient affected by Sturge-Weber syndrome presenting with tongue hemangioma and emphasize the importance of an accurate diagnosis and management in the clinical practice. How to cite this article Shinde D, Dabholkar YG, Saberwal AA, Velankar HK, Shetty AK. Use of Intralesional Bleomycin for Oral Hemangioma of Sturge-Weber Syndrome. Int J Head Neck Surg 2015;6(1):17-19.

scholarly journals Sturge Weber Syndrome: A Case Report

1970 ◽  
Vol 30 (3) ◽  
pp. 164-165
Author(s):  
S Devkota ◽  
S Upadhyay
Keyword(s):  
Venous Drainage ◽  
Congenital Glaucoma ◽  
Inherited Disease ◽  
Port Wine ◽  
Venous Plexus ◽  
The Family ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Neurocutaneous Syndrome ◽  
Cortical Venous Drainage

Sturge-Weber syndrome is a neurocutaneous syndrome characterized by port wine stain, congenital glaucoma, and underlying anomalous leptomeningeal venous plexus and the lack of normal cortical venous drainage. It is a congenital but not an inherited disease and it occurs sporadically and is very rare, incidence being approximately 1 on 50000. It occurs with rare exception but occasionally the other members of the family may have hemangiomata of a lesser degree.  DOI: 10.3126/jnps.v30i3.3920J Nep Paedtr Soc 2010;30(3):164-165

scholarly journals Anesthetic Challenges of a Child with Sturge–Weber Syndrome for Epilepsy Surgery: A Case Report

2019 ◽  
Vol 06 (01) ◽  
pp. 037-039
Author(s):  
Roshan Kurian ◽  
Karen R. Lionel ◽  
Ramamani Mariappan
Keyword(s):  
Vascular Malformations ◽  
Port Wine ◽  
Congenital Disease ◽  
Anesthesia Management ◽  
Massive Blood Loss ◽  
Weber Syndrome ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
Venous Angiomas ◽  
Intracranial Vasculature

AbstractSturge–Weber syndrome is a rare congenital disease, also called encephalotrigeminal angiomatosis, caused by persistence of transitory primordial arteriovenous connections of the fetal intracranial vasculature. It is characterized by vascular malformations with capillary venous angiomas that involve the face, choroid of the eye, and leptomeninges. The main clinical features of this syndrome are port-wine stains, glaucoma, convulsions, and angiomas of the airway. Anesthesia management is directed toward anticipating a difficult airway, avoiding trauma to the hemangioma during airway manipulation, preventing the rise in the intracranial and intraocular pressures, anticipating and managing massive blood loss and the complications associated with massive blood transfusion, and avoiding factors that might trigger a seizure such as hypoxia, hypercarbia, hypotension, hypoglycemia, and hyperthermia.

scholarly journals Sturge-Weber Syndrome with Port Wine Stain and Bilateral Choroid Plexus Angiomas: A Case Report

2014 ◽  
Vol 15 (1) ◽  
pp. 68-70
Author(s):  
Kaleem Ahmad ◽  
Sajid Ansari ◽  
Kanchan Dhungel ◽  
Mukesh Kumar Gupta ◽  
Ashok Raj Pant ◽  
...  
Keyword(s):  
Case Report ◽  
Choroid Plexus ◽  
Clinical Features ◽  
Port Wine Stain ◽  
Port Wine ◽  
Bilateral Involvement ◽  
Weber Syndrome ◽  
Vascular Proliferation ◽  
Sturge Weber Syndrome ◽  
Hereditary Condition

Sturge-Weber syndrome is a rarenon-hereditary condition characterized by a hamartomatous vascular proliferation. It is usually unilateral; bilateral involvement is seen only in 15% cases. We report a case of a 6 month old male child with Sturge-Weber syndrome with its inherent clinical features and typical imaging findings.DOI: http://dx.doi.org/10.3329/jom.v15i1.19877 J Medicine 2014; 15: 68-70

scholarly journals Sturge Weber Syndrome

2016 ◽  
Vol 8 (1) ◽  
pp. 68
Author(s):  
Kazi Nilufar Moly ◽  
S.M. Abu Ahsan ◽  
Md. Shafiqul Lslam
Keyword(s):  
Trigeminal Nerve ◽  
Male Child ◽  
Port Wine Stain ◽  
Port Wine ◽  
Weber Syndrome ◽  
Neurocutaneous Disorder ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
Nevus Flammeus

<p>Sturge weber syndrome is a rare sporadic condition of mesodermal phacomatosis, also called encephalotrigeminal angio­matosis (synonyms : fourth phacomatosis or mother spot), is a neurocutaneous disorder with angiomas that involve the leptomeninges (leptomeningeal angiomas) and the skin of the face (purple colored flat cutaneous haemangiomas ), typically in the ophthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve. The hallmark of sturge weber syndrome is a facial cutaneous venous dilation, also referred to as a nevus flammeus or port wine stain (PWS). Because of the rarity, we report here a one &amp; half year old male child who presented with features of the Sturge Weber Syndrome on both side of face.</p>

Skin problems of the face, nose, eyelids, and ears

2020 ◽  
pp. 123-136
Keyword(s):  
Lupus Erythematosus ◽  
Skin Diseases ◽  
Port Wine ◽  
Clinical Images ◽  
Skin Changes ◽  
Port Wine Stains ◽  
Eyelid Swelling ◽  
Weber Syndrome ◽  
The Face ◽  
Sturge Weber Syndrome

This chapter attempts to provide a comprehensive list of most common and some rare skin diseases affecting the face, nose, eyelids, and ears, excluding the oral mucosa and tongue dealt with in Chapter 10. Illustrative clinical images are used throughout. It lists causes of ‘red facial rashes’ and ‘recognizing rashes and tumours by facial distribution’, although fuller descriptions of many diseases are found in other chapters. Particular emphasis is given to recognizing diseases with a particular predilection for certain sites such as the butterfly distribution of photosensitivity seen in systemic lupus erythematosus (SLE) or the periorbital rash of neonatal LE. Attention is drawn to those skin changes (rashes or tumours) that might indicate an underlying developmental problem for example segmental port-wine stains associated with the Sturge–Weber syndrome or the perinasal and chin angiofibromas of tuberose sclerosis complex. There is also a helpful box on causes of eyelid swelling.

Dr. Jacobs Replies

PEDIATRICS ◽  
1977 ◽  
Vol 60 (5) ◽  
pp. 785-786
Author(s):  
Alvin H. Jacobs
Keyword(s):  
Skin Involvement ◽  
Neurological Manifestations ◽  
Port Wine ◽  
Weber Syndrome ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
Nevus Flammeus

Drs. Gorman and Snead bring up a purely semantic question: "What is the Sturge-Weber syndrome?" According to McKusick,1 Sturge-Weber syndrome, sometimes called the fourth phacomatosis, is characterized by nevus flammeus of the face and angioma of the meninges. Warkany2 states that it "consists of a facial port wine nevus, convulsions and other neurological manifestations." In fact, the latter author, in discussing reported cases, rules out as Sturge-Weber syndrome a case with the CNS changes but no skin involvement.

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