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 Bilateral Sturge Weber Syndrome- a rare case report

2013 ◽  
Vol 5 (1) ◽  
pp. 129-132 ◽  
Author(s):  
P Singh ◽  
S Singh
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Unusual Case ◽  
The Other ◽  
Advanced Stage ◽  
Weber Syndrome ◽  
Rare Case Report ◽  
The Face ◽  
Bilateral Condition ◽  
Sturge Weber Syndrome

Background: Sturge-Weber syndrome is a rare congenital neuro- oculo- cutaneous disorder. Objective: To report a very rare unusual case of bilateral manifestation of Sturge Weber syndrome. Case: We report an unusual case of a 17-year-old female with advanced stage of bilateral glaucoma associated with facial nevus extending to the other half of the face as well and bilateral intracranial calcification. Conclusion: Sturge -Weber syndrome can manifest as a bilateral condition. Nepal J Ophthalmol 2013; 5(9):129-132 DOI: http://dx.doi.org/10.3126/nepjoph.v5i1.7841

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 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 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 Cerebrofacial Venous Metameric Syndrome 2 Plus 3: Facial and Cerebral Manifestations

2007 ◽  
Vol 13 (1) ◽  
pp. 55-58 ◽  
Author(s):  
R. Agid ◽  
K.G. TerBrugge
Keyword(s):  
Wide Spectrum ◽  
Venous Malformations ◽  
Mri Imaging ◽  
Unique Case ◽  
Intracranial Involvement ◽  
Weber Syndrome ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
The Brain

A unique case of Cerebrofacial Venous Metameric Syndrome (CVMS) in a 25- year-old women is described with venous malformations involving simultaneously the brain and the face. This case represents CVMS 2 plus 3 according to the classification described by Lasjaunias et Al. The metameric distribution of the cerebrofacial venous syndromes is well illustrated in this case. The extracranial and intracranial involvement is described and the appearance on MRI imaging is detailed. In our opinion this case demonstrates that cerebrofacial venous metameric syndromes include a wide spectrum of possible phenotypes including Sturge Weber syndrome and cases such as we describe here.

scholarly journals Sturge-Weber Syndrome– A Rare Case Report

2014 ◽  
Vol 13 (5) ◽  
pp. 39-41
Author(s):  
Divyank Pathak ◽  
◽  
Dhananjay.Y Shrikhande ◽  
Nilesh Maniya ◽  
Somdeepak Reddy ◽  
...  
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Weber Syndrome ◽  
Rare Case Report ◽  
Sturge Weber Syndrome

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.

Sturge-Weber Syndrome

2000 ◽  
Vol 3 (3) ◽  
pp. 301-305 ◽  
Author(s):  
Venita Jay
Keyword(s):  
Nerve Tissue ◽  
Port Wine ◽  
Difficult Question ◽  
Probable Relationship ◽  
Weber Syndrome ◽  
Mucous Membranes ◽  
Sturge Weber Syndrome ◽  
The Right ◽  
The Brain

I have no intention of going into the difficult question of the pathology of port-wine mark. The point to which I wish to call particular attention is the probable relationship between the mark and the fits …. From the nature of the fits, and from their mode of onset, I think there can be no doubt that they are due to some cause external to the nerve-tissue … and this external cause is in all probability to be found in the presence of a “port-wine mark” on the surface of the right side of the brain, just as we have found it in the skin, mucous membranes, and retina of that side. William Allen Sturge, 1879

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