Sturge- Weber syndrome (SWS), is a rare neuro-cutaneous angiomatosis which affects male and females alike. The clinical manifestations include angiomas, haemangiomas of the lips, tongue and palatine region. The oral manifestations are usually unilateral and are susceptible to bleed. Patients can also present with macroglossia and maxillary bone hypertrophy which can lead to malocclusion of the oral cavity. Food accumulation due to occlusion can cause growth of bacteria which can intensify infections and can cause gingival hyperplasia. A case of a middle-aged 39 year old female was reported in the Ziauddin Hospital, Karachi on 2nd of February,2022 with the presenting complaints of intermittent fever and drowsiness for 10 days. On examination she had massive tongue enlargement, drooling, malocclusion, difficulty in eating and breathing. She was a known case of Sturgeweber syndrome. Based on the clinical and radiological findings, she was managed along the lines of prelaryngeal soft tissue and submandibular infection.
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http://dx.doi.org/10.47391/JPMA.9033 | DOI Listing |
Semin Ophthalmol
January 2025
Department of Ophthalmology, Medical College of Wisconsin, Milwaukee, WI, USA.
Purpose: To examine propranolol efficacy in treating diffuse (DCH) and circumscribed choroidal hemangiomas (CCH) and controlling intraocular pressure (IOP) in patients with Sturge-Weber syndrome (SWS).
Methods: A SWS patient case treated with propranolol for DCH is presented. Following PRISMA guidelines, we also performed a systematic review using PubMed/Web of Science, analyzing 14 studies detailing propranolol treatment outcomes for DCH, CCH, and IOP control in SWS.
Curr Med Imaging
January 2025
Department of Radiology, Sengkang General Hospital, Singapore.
Background: Leptomeningeal enhancement, visible on MRI, can indicate a variety of diseases, both neoplastic and non-neoplastic.
Objective: This comprehensive pictorial review aims to equip radiologists and trainees with a thorough understanding of the diverse imaging presentations of leptomeningeal disease.
Methods: Drawing from a retrospective analysis of MRI scans conducted between 1 January 2008 and 30 September 2022, at two tertiary teaching hospitals in Singapore, this review covers a wide range of conditions.
Jpn J Radiol
December 2024
Department of Radiology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, Mie, 514-8507, Japan.
Brush sign (BS) was first reported as prominent hypointensity of deep medullary veins and subependymal veins on T2*-weighted images at 3 T MRI in patients with acute stroke in the territory of the middle cerebral artery. Subsequently, BS in central nervous system (CNS) diseases such as moyamoya disease, cerebral venous thrombosis, and Sturge-Weber syndrome was also described on susceptibility-weighted imaging (SWI), and the clinical implications of BS were discussed. The purpose of this review is to demonstrate BS on SWI in various CNS diseases and its mechanisms in the above-mentioned diseases.
View Article and Find Full Text PDFInt J Surg Case Rep
December 2024
King Khaled Eye Specialist Hospital, KKESH, Riyadh, Saudi Arabia.
Introduction And Importance: Sturge Weber Syndrome (SWS) is a congenital neurocutaneous disorder that affects several organs. Abnormal ocular findings are typically on the same side as the SWS. These changes can affect various parts of the eye, including the eyelid, front chamber, cornea, choroid, and retina.
View Article and Find Full Text PDFFront Neurol
December 2024
Neurology and Developmental Medicine, Kennedy Krieger Institute, Baltimore, MD, United States.
Sturge-Weber syndrome (SWS) is a rare congenital neurovascular disorder that initially presents with a facial port-wine birthmark (PWB) and most commonly associated with a R183Q somatic mosaic mutation in the gene . This mutation is enriched in endothelial cells. Contrast-enhanced magnetic resonance imaging (MRI) diagnoses brain abnormalities including leptomeningeal vascular malformation, an enlarged choroid plexus, and abnormal cortical and subcortical blood vessels.
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