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.

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.

Port-Wine Stain (Nevus Flammeus) in Sturge-Weber Syndrome

2018 ◽  
pp. 1406-1408
Author(s):  
Jason E. Hale ◽  
Sumayya J. Almarzouqi ◽  
Michael L. Morgan ◽  
Andrew G. Lee
Keyword(s):  
Port Wine Stain ◽  
Port Wine ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Nevus Flammeus

scholarly journals The Pathogenesis of Port Wine Stain and Sturge Weber Syndrome: Complex Interactions between Genetic Alterations and Aberrant MAPK and PI3K Activation

2019 ◽  
Vol 20 (9) ◽  
pp. 2243 ◽  
Author(s):  
Vi Nguyen ◽  
Marcelo Hochman ◽  
Martin C. Mihm ◽  
J. Stuart Nelson ◽  
Wenbin Tan
Keyword(s):  
Endothelial Cells ◽  
Human Skin ◽  
Current Knowledge ◽  
Vascular Malformations ◽  
Genetic Alterations ◽  
Future Research ◽  
Port Wine Stain ◽  
Port Wine ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

Port wine stain (PWS) is a congenital vascular malformation involving human skin. Approximately 15–20% of children a facial PWS involving the ophthalmic (V1) trigeminal dermatome are at risk for Sturge Weber syndrome (SWS), a neurocutaneous disorder with vascular malformations in the cerebral cortex on the same side of the facial PWS lesions. Recently, evidence has surfaced that advanced our understanding of the pathogenesis of PWS/SWS, including discoveries of somatic genetic mutations (GNAQ, PI3K), MAPK and PI3K aberrant activations, and molecular phenotypes of PWS endothelial cells. In this review, we summarize current knowledge on the etiology and pathology of PWS/SWS based on evidence that the activation of MAPK and/or PI3K contributes to the malformations, as well as potential futuristic treatment approaches targeting these aberrantly dysregulated signaling pathways. Current data support that: (1) PWS is a multifactorial malformation involving the entire physiological structure of human skin; (2) PWS should be pathoanatomically re-defined as “a malformation resulting from differentiation-impaired endothelial cells with a progressive dilatation of immature venule-like vasculatures”; (3) dysregulation of vascular MAPK and/or PI3K signaling during human embryonic development plays a part in the pathogenesis and progression of PWS/SWS; and (4) sporadic low frequency somatic mutations, such as GNAQ, PI3K, work as team players but not as a lone wolf, contributing to the development of vascular phenotypes. We also address many crucial questions yet to be answered in the future research investigations.

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 Choroidal Alterations of Sturge-weber Syndrome Secondary Glaucoma and Non-glaucoma Port-wine Stain Patients Distinguished by Enhanced Depth Imaging Optical Coherence Tomography

2020 ◽  
Author(s):  
Yue Wu ◽  
Lulu Huang ◽  
Yixin Liu ◽  
Li Xu ◽  
Wenyi Guo
Keyword(s):  
Optical Coherence Tomography ◽  
Secondary Glaucoma ◽  
Posterior Segment ◽  
Enhanced Depth Imaging ◽  
Port Wine Stain ◽  
Port Wine ◽  
Depth Imaging ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Imaging Optical Coherence Tomography

Abstract Background: To evaluate the choroidal changes in Sturge-Weber syndrome (SWS) secondary glaucoma and non-glaucoma port-wine stain (PWS) patients by enhanced depth imaging optical coherence tomography (EDI-OCT).Methods: SWS and PWS patients who were over 3 years old and treated or screened at our ophthalmology department were included in the study. Baseline demographics, EDI-OCT and fundus photography data were collected from all patients.Results: Forty-six non-glaucoma PWS (NGPWS) patients and 35 SWS secondary glaucoma（SG）patients were included, with mean ages of 16.52±13.63 and 13.94±8.27 years, respectively (p>0.05). Among these patients, 2 exhibit bilateral PWS and unilateral glaucoma. Thus the two eyes of each person were divided into NGPWS group and SG group respectively. Twenty-one eyes had choroidal hemangiomas and 7 eyes had excessive thickening of the choroid without choroidal hemangiomas. Choroidal hemangiomas were only observed in ipsilateral eyes of SG patients. The choroidal thicknesses of the ipsilateral and contralateral eyes of NGPWS patients were 358.10±117.40 μm (45 eyes) and 288.20±79.04 μm (41 eyes), respectively (p<0.05). The choroidal thicknesses of the ipsilateral and contralateral eyes of SG patients were 511.40±242.10 μm (15 eyes) and 283.90±92.27 μm (29 eyes), respectively (p<0.05). Significant differences were found between the ipsilateral eyes of SWS and PWS patients (p<0.05). Six of 12 eyes (50%) with choroidal hemangiomas exhibited post-operative posterior segment complications. Conclusions: Non-glaucoma PWS and SWS secondary glaucoma patients had a thicker choroid in the ipsilateral eye. The trend was even more pronounced in SWS secondary glaucoma patients. Choroidal hemangiomas were only found in the ipsilateral eyes of SG. In addition, choroidal hemangioma was a risk factor for post-operative posterior segment complications in SWS patients.

scholarly journals Choroidal Alterations of Sturge-Weber Syndrome Secondary Glaucoma and Non-glaucoma Port-wine Stain Patients Distinguished by Enhanced Depth Imaging Optical Coherence Tomography

2020 ◽  
Author(s):  
Yue Wu ◽  
Lulu Huang ◽  
Yixin Liu ◽  
Li Xu ◽  
Wenyi Guo
Keyword(s):  
Optical Coherence Tomography ◽  
Secondary Glaucoma ◽  
Posterior Segment ◽  
Enhanced Depth Imaging ◽  
Port Wine Stain ◽  
Port Wine ◽  
Depth Imaging ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Imaging Optical Coherence Tomography

Abstract Background: To evaluate the choroidal changes in Sturge-Weber syndrome (SWS) secondary glaucoma and non-glaucoma port-wine stain (PWS) patients by enhanced depth imaging optical coherence tomography (EDI-OCT).Methods: SWS and PWS patients who were over 3 years old and treated or screened at our ophthalmology department were included in the study. Baseline demographics, EDI-OCT and fundus photography data were collected from all patients.Results: Forty-six non-glaucoma PWS (NGPWS) patients and 35 SWS secondary glaucoma（SG）patients were included, with mean ages of 16.52±13.63 and 13.94±8.27 years, respectively (p>0.05). Among these patients, 2 exhibit bilateral PWS and unilateral glaucoma. Thus the two eyes of each person were divided into NGPWS group and SG group respectively. Twenty-one eyes had choroidal hemangiomas and 7 eyes had excessive thickening of the choroid without choroidal hemangiomas. Choroidal hemangiomas were only observed in ipsilateral eyes of SG patients. The choroidal thicknesses of the ipsilateral and contralateral eyes of NGPWS patients were 358.10±117.40 μm (45 eyes) and 288.20±79.04 μm (41 eyes), respectively (p<0.05). The choroidal thicknesses of the ipsilateral and contralateral eyes of SG patients were 511.40±242.10 μm (15 eyes) and 283.90±92.27 μm (29 eyes), respectively (p<0.05). Significant differences were found between the ipsilateral eyes of SWS and PWS patients (p<0.05). Six of 13 eyes (46%) with choroidal hemangiomas exhibited post-operative posterior segment complications. Conclusions: Non-glaucoma PWS and SWS secondary glaucoma patients had a thicker choroid in the ipsilateral eye. The trend was even more pronounced in SWS secondary glaucoma patients. Choroidal hemangiomas were only found in the ipsilateral eyes of SG. In addition, choroidal hemangioma was a risk factor for post-operative posterior segment complications in SWS patients.

scholarly journals A Rare Presentation of Acquired Port Wine Stain in an Elderly Male

2021 ◽  
Vol 4 (1) ◽  
pp. 457-458
Author(s):  
Prasanna Kumar Jha ◽  
Satyendra Kumar Singh
Keyword(s):  
Blood Vessels ◽  
Vascular Lesions ◽  
Port Wine Stain ◽  
Port Wine ◽  
Elderly Male ◽  
Rare Presentation ◽  
Vascular Plexus ◽  
Port Wine Stains ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

Acquired port wine stain though an uncommon entity that develops later in life, resembles congenital port wine stain morphologically and histologically. Congenital port wine stains are vascular lesions caused by progressive ectasia of blood vessels which is located in the vascular plexus of the dermis. Congenital port-wine stains may be associated with Sturge Weber syndrome causing neurological and eye abnormalities such as glaucoma. Here we report a 60-year-old male presenting with a complaint of asymptomatic reddish patches over the nose for 15 years.

scholarly journals Choroidal Alterations of Sturge-Weber Syndrome Secondary Glaucoma and Non-glaucoma Port-wine Stain Patients Distinguished by Enhanced Depth Imaging Optical Coherence Tomography

2020 ◽  
Author(s):  
Yue Wu ◽  
Lulu Huang ◽  
Yixin Liu ◽  
Li Xu ◽  
Wenyi Guo
Keyword(s):  
Secondary Glaucoma ◽  
Posterior Segment ◽  
Enhanced Depth Imaging ◽  
Port Wine Stain ◽  
Port Wine ◽  
Depth Imaging ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Imaging Optical Coherence Tomography ◽  
Fellow Eyes

Abstract Background: To evaluate the choroidal changes in Sturge-Weber syndrome (SWS) secondary glaucoma and non-glaucoma port-wine stain (PWS) patients by enhanced depth imaging optical coherence tomography (EDI-OCT).Methods: SWS and PWS patients who were over 3 years old and treated or screened at our ophthalmology department were included in the study. Baseline demographics, EDI-OCT and fundus photography data were collected from all patients.Results: Overall, 46 non-glaucoma PWS (NGPWS) patients and 35 SWS secondary glaucoma（SG）patients were included, with mean ages of 16.52±13.63 and 13.94±8.27 years, respectively (p>0.05). Among these patients 2 exhibited bilateral PWS and unilateral glaucoma. Thus, the two eyes of each patient were divided into NGPWS and SG group, respectively. Twenty-one eyes had choroidal hemangiomas and 7 eyes had excessive thickening of the choroid without choroidal hemangiomas. Choroidal hemangiomas were only observed in ipsilateral eyes of SG patients. The choroidal thicknesses of the ipsilateral and fellow eyes of NGPWS patients were 358.10±117.40 μm (45 eyes) and 288.20±79.04 μm (41 eyes), respectively (p<0.05). The choroidal thicknesses of the ipsilateral and fellow eyes of SG patients were 511.40±242.10 μm (15 eyes) and 283.90±92.27 μm (29 eyes), respectively (p<0.05). Significant differences were found between the ipsilateral eyes of SWS and PWS patients (p<0.05). Six of 13 eyes (46%) with choroidal hemangiomas exhibited post-operative posterior segment complications. Conclusions: NGPWS and SG patients had a thicker choroid in the ipsilateral eye. The trend was even more pronounced in SG patients. Choroidal hemangiomas were only found in the ipsilateral eyes of SG. In addition, choroidal hemangioma was a risk factor for post-operative posterior segment complications in SWS patients.

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