Phacomatosis pigmentovascularis with sturge-weber syndrome and congenital glaucoma: A rare case report

2021 ◽  
pp. 112067212110678
Author(s):  
Vijaikrishnan Manavalan ◽  
Subashini Kaliaperumal ◽  
Swathi Subramanian ◽  
Malavika Mani
Keyword(s):  
Seizure Control ◽  
Treatment Initiation ◽  
Congenital Glaucoma ◽  
Early Screening ◽  
Systemic Complications ◽  
Systemic Involvement ◽  
Phacomatosis Pigmentovascularis ◽  
Weber Syndrome ◽  
Rare Case Report ◽  
Sturge Weber Syndrome

Phacomatosis pigmentovascularis (PPV) is a rare congenital disease characterized by the co-existence of cutaneous vascular malformation and pigmentary nevi with or without extracutaneous systemic involvement. Here, we present a 2-month old child diagnosed with phacomatosis cesioflammea type of PPV with Sturge-Weber syndrome and secondary congenital glaucoma of the left eye. She underwent combined trabeculotomy and trabeculectomy in the left eye for glaucoma and was started on anti-epileptics for seizure control following pediatric evaluation. Early screening and treatment initiation can prevent blindness and other systemic complications associated with PPV.

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

Two Reports of Phacomatosis Pigmentovascularis Type Iib, One in Association with Sturge-Weber Syndrome and Klippel-Trenaunay Syndrome

2010 ◽  
Vol 27 (3) ◽  
pp. 303-305 ◽  
Author(s):  
Lindsey B. Finklea ◽  
Melinda R. Mohr ◽  
Molly M. Warthan ◽  
David H. Darrow ◽  
Judith V. Williams
Keyword(s):  
Phacomatosis Pigmentovascularis ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Klippel Trenaunay Syndrome

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 Severe Rhabdomyolysis in Phacomatosis Pigmentovascularis Type IIb associated with Sturge-Weber Syndrome

2015 ◽  
Vol 30 (4) ◽  
pp. 329-335 ◽  
Author(s):  
Bongjin Lee ◽  
Hyung Joo Jeong ◽  
Yu Hyeon Choi ◽  
Chong Won Choi ◽  
June Dong Park
Keyword(s):  
Phacomatosis Pigmentovascularis ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

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 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.

Two Cases of the Sturge-Weber Syndrome

1966 ◽  
Vol 112 (488) ◽  
pp. 709-711
Author(s):  
Ranan Rimon ◽  
Olavi Katila
Keyword(s):  
Young Girl ◽  
Congenital Glaucoma ◽  
X Ray ◽  
Naevus Flammeus ◽  
Weber Syndrome ◽  
Calcium Deposits ◽  
Sturge Weber Syndrome ◽  
The Brain

The Sturge-Weber syndrome belongs to the relatively uncommon group of ailments known as heredodegenerative disorders. In 1879 Sturge described a case of epilepsy in a young girl who exhibited a congenital glaucoma and a facial angioma or naevus flammeus. Sturge speculated that the patient's convulsions were caused by a similar naevus formation in the brain. Kalischer (1897, 1901) discovered angiomatotic changes in the meninges, and later the typical calcium deposits corresponding to these angio-mata were found in X-ray examinations by Weber (1922), Dimitri (1923), and Krabbe (1934). The appellation of the Sturge-Weber syndrome varies, owing to the number of its investigators. Levison (1953) calls attention to this question of nomenclature.

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
Sign in / Sign up

Export Citation Format

Share Document