scholarly journals Klipplel-Trénaunay-Weber syndrome: A case report

2009 ◽  
Vol 56 (2) ◽  
pp. 97-101
Author(s):  
de Melo ◽  
do Boechat ◽  
Silva Coutinho ◽  
Cardoso Silveira ◽  
Cavalcanti Leite
Keyword(s):  
Clinical Examination ◽  
Complete Blood Count ◽  
Port Wine ◽  
Normal Range ◽  
Anterior Maxilla ◽  
Lymphatic Abnormalities ◽  
Gingival Enlargement ◽  
Weber Syndrome ◽  
Orofacial Region ◽  
Medical Examinations

Klippel-Tr?naunay-Weber Syndrome (KTWS) is a rare congenital malformation that may include port-wine stain, soft tissue and bony hypertrophy, and venous malformations and lymphatic abnormalities. The oral findings include an enlarged maxilla, gingival enlargement, malocclusion and premature tooth eruption. This report describes a case of gingival enlargement in an 8-year-old male child with KTWS. The extraoral clinical examination revealed discrete hemifacial hypertrophy on the left side and hyperpigmented spots on the anterior region of the neck, left arm and left leg. The intraoral clinical examination showed a deep palate and enlarged buccal and palatal gingiva in the anterior maxilla. Complete blood count and all general medical examinations showed values within normal range while the psychological evaluation indicated a mild learning delay. Although it rarely involves the orofacial region, KTWS should be included in the differential diagnosis of severe gingival enlargement. In view of the potentially dangerous complications that can arise, it is imperative the dental surgeon has a thorough knowledge of this condition and its management.

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.

Rare Instance of Gingival Enlargement in Klippel-Trenaunay Syndrome: A Case Report

2006 ◽  
Vol 7 (3) ◽  
pp. 92-98 ◽  
Author(s):  
Pradeep S. Anand ◽  
T. Roshna
Keyword(s):  
Case Report ◽  
Radiographic Examination ◽  
Port Wine ◽  
Imaging Studies ◽  
Patient History ◽  
Lymphatic Abnormalities ◽  
Gingival Enlargement ◽  
Klippel Trenaunay Syndrome ◽  
Head Neck ◽  
Rare Instance

Abstract Klippel-Trenaunay Syndrome is a rare congenital malformation that may include port-wine stain, soft tissue and bony hypertrophy, and venous malformations and lymphatic abnormalities. Although it usually involves the limbs, it may also rarely involve the head, neck, and orofacial regions. Despite its rarity, Klippel-Trenaunay Syndrome should be considered in the differential diagnosis of gingival enlargement. The condition can be easily recognized clinically, but further investigations including imaging studies have to be carried out in order to better understand the nature of the lesion. This report describes a case of gingival enlargement in Klippel- Trenaunay Syndrome in a 16-year-old female patient. The diagnosis of the condition was made based on the patient history, clinical and radiographic examination, computed tomography (CT), and angiogram. Citation Anand PS, Roshna T. Rare Instance of Gingival Enlargement in Klippel-Trenaunay Syndrome: A Case Report. J Contemp Dent Pract 2006 July;(7)3:092-098.

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.

Results of testing of an automated system for clinical examination and preventive medical examinations

2016 ◽  
Vol 18 (12) ◽  
pp. 27-30 ◽  
Author(s):  
V.A. Egorov ◽  
◽  
L.Yu. Drozdova ◽  
A.M. Kalinina ◽  
◽  
...  
Keyword(s):  
Clinical Examination ◽  
Automated System ◽  
Medical Examinations

scholarly journals Giant Cell Tumour of Clivus: A Rare Case Report

2021 ◽  
Author(s):  
Swapan Shantiram Goswami ◽  
Sonal Jain ◽  
Shalin Nagori
Keyword(s):  
Giant Cell ◽  
Complete Blood Count ◽  
Bone Cyst ◽  
Middle Cranial Fossa ◽  
Lytic Lesion ◽  
Left Internal Carotid Artery ◽  
Normal Range ◽  
Prepontine Cistern ◽  
Rare Case Report ◽  
Mild Anaemia

Giant Cell Tumours (GCT) of the skull is rare, being less than 0.05% of all skeletal tumours. They are usually located in the middle cranial fossa affecting the temporal, sphenoid, petrosal and occipital bone. Clival GCT is rarer and 15 cases are described in the literature. Authors report a case of a female patient aged 20 years, who complained of headache with decrease in vision of left eye. Neurological examination revealed left optic nerve palsy. Laboratory test of Vitamin B12, blood urea, Serum Creatinine, Serum Electrolytes were within normal range. Complete blood count showed mild anaemia. The serum calcium and parathyroid hormones were within normal range. Computed Tomography (CT) scan and Magnetic Resonance Imaging (MRI) were done, which demonstrated expansile destructive lytic lesion involving sphenoid bone and clivus, reaching upto sellar, parasellar regions, sphenoid sinus and left posterior ethmoid sinus, effacing left optic foramina and posteriorly effacing prepontine cistern. The left internal carotid artery was partially encased by the mass. The tumour was partially removed by endoscopic trans-nasal trans-sphenoidal approach. Histopathology confirmed it as a benign GCT. Surgical treatment of clival GCTs are fraught with complication because of its close proximity to vital structures. High vascularity, potential malignant behaviour, inaccessibility and very few published cases preclude a definite outcome of this lesion. Histopathology is necessary to differentiate various lytic lesion like chordoma, aneurysmal bone cyst, invasive pituitary adenoma, chondrosarcoma and brown tumour (parathyroidism).

scholarly journals Gingival enlargement in thalassemia patient – A Conjectural Association

2020 ◽  
Vol 6 (3) ◽  
pp. 74-77
Author(s):  
CS BAIJU ◽  
Gunjan Gupta ◽  
Karuna Joshi ◽  
Shagufta ◽  
N.D Gupta
Keyword(s):  
Single Gene ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Thalassemia Patient ◽  
Anterior Maxilla ◽  
Facial Region ◽  
Periodontal Tissues ◽  
Beta Thalassemia Major ◽  
Gingival Enlargement ◽  
Maxilla And Mandible

Thalassemia is a single gene inherited blood disease. Beta thalassemia major is life threating. It causes abnormality in various organs and oral-facial region. Thalassemia patients are immune-deficient because of iron-overload. Immune system abnormality includes neutrophilic dysfunction and impairment of phagocytosis by the monocyte-macrophage system. Iron accumulation also affects periodontal tissues, which seems to increase the level of cytokines and thus have an enhancing effect on gingival inflammation. This article highlights a peculiar case of gingival enlargement in anterior maxilla and mandible. The patient was known case of a beta-thalassemia major. Blood investigation revealed a lower level of hemoglobin. The patient underwent non-surgical periodontal therapy. Proper periodontal care improves the quality of life in these patients. This case report reinforces the significance of proper history taking with all minor details and the role of patient education in phase I therapy

Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome

2010 ◽  
Vol 14 (1) ◽  
pp. 105 ◽  
Author(s):  
Debajit Ray ◽  
Anil K. Mandal ◽  
G. Chandrasekhar ◽  
Milind Naik ◽  
Niteen Dhepe
Keyword(s):  
Vascular Malformations ◽  
Port Wine ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

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

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

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