To report a clinical case of sturge weber syndrome in a 29yeralold male patient who presented oral manifestations related to the syndrome. Sturgeweber syndrome sws is a sporadic congenital neurooculocutaneous disorder that may present with glaucoma and vascular malformations of the conjunctiva, episclera, choroid and retina. Case report photodynamic therapy for diffuse choroidal. Portwine birthmarks are caused by enlarged blood vessels right underneath the skin. Cases journal management of patient with sturge weber syndrome. Case report sturgeweber syndrome with osteohypertrophy of.
A case report on sturge weber syndrome seizures with stain. We speculate that all the main symptoms observed in our patient could be the expression of a functional imbalance between the atrophic right hemisphere and the hyperexcitable left cortex. Paraskev stoyanov, varna journal of imab annual proceeding scientific papers 2008, book 1 abstract sturge weber syndrome sws is a sporadic. The color can range from dark red to light pink and it is usually found on one side of the face. Sturgeweber syndrome is a rare, congenital, neurooculocutaneous disorder which is characterised extraorally by unilateral port wine stains on the face, glaucoma, seizures and mental retardation, and intraorally by ipsilateral gingival haemangioma which frequently affects the maxilla or mandible. Seizures occur in 83% of individuals with sturge weber syndrome and may be extremely difficult to control. Idiopathic inflammatory myopathy comorbid with sturge. This is case report of a 18yearold mentally disabled boy, with longstanding seizures, with a portwine nevi on the left side of the face along the distribution of trigeminal nerve. Pdf localized choroidal haemangioma associated with sturge. This case report presents a typical case of sturge weber syndrome. Pdf roachs type ii variant of sturge weber syndrome.
A 7 yearold boy, with a long history of ocular symptoms and an unspecified ocular surgery, presents with a painful blind left. Case report open access sturgeweber syndrome coexisting with episodes of rhabdomyolysis min zhu, xiaobin li, meihong zhou, hui wan, yuchen wu and daojun hong abstract background. The sturge weber syndrome sws is a rare congenital disorder that occurs sporadically and features lesions of both skin and nervous system. Sturgeweber syndrome genetic and rare diseases information. Bilateral sturge weber syndrome a rare case report singh p, singh s department of glaucoma lumbini eye institute, bhairahawa, nepal abstract background. Babies with sws are born with a birthmark on their face known as a portwine stain. Gnaq mutation r183q as a potential cause of familial. Kamble 8 department of pedodontics, sppgidms, lucknow, india department of pedodontics, buddha institute of dental sciences, patna. A case report case report in the present study, a 4 generation family with a history of sws was analyzed and demonstrated dominant inheritance of sws fig. It gives the radiological and ct scan findings and the important role played by them in the diagnosis of this syndrome. We report a case of a 17monthold child affected by sturgeweber syndrome who had unusually rapid overgrowth of the left frontal, temporal, orbital, and maxillary regions. Sturge weber syndrome is a rare developmental neuroectodermical disorder.
Seizures and other neurologic complications are the result of leptomeningeal angioma vascular malformations in the lining of the brain. To report an untypical presentation of a presumed sturge weber syndrome sws, and to highlight the indispensable value of thorough clinical examination as primary means for proper diagnosis and management. We report a case of localized choroidal haemangioma. Case report open access idiopathic inflammatory myopathy comorbid with sturgeweber syndrome. Role of the cerebral ultrasound in a case of sturgeweber.
We report a case of a 17monthold child affected by sturge weber syndrome who had unusually rapid overgrowth of the left frontal, temporal, orbital, and maxillary regions. We report an apparently classical sturgeweber syndrome and hypothesize a shared pathophysiologic mechanism for clinical symptoms. Manipal journal of nursing and health sciences, 41. Journal of indian society of pedodontics and preventive dentistry 2008. The sturgeweber syndrome sws is a rare congenital disorder that occurs sporadically and features lesions of both skin and nervous system. Cerebral ultrasound cus can be a valuable noninvasive diagnostic tool for brain involvement in sturgeweber syndrome sws. A rare case of incidentally detected sturgeweber syndrome. Sturge weber syndrome with intraoral manifestations. Case report open access sturge weber syndrome coexisting with episodes of rhabdomyolysis min zhu, xiaobin li, meihong zhou, hui wan, yuchen wu and daojun hong abstract background. Pradeep nigam1, shivaji thakare2, umesh pratap singh3, manoj indurkar4.
Page number not for citation purposes 1 case report sturgeweber syndrome. Jan 18, 2019 sturge weber syndrome sws is a rare disorder affecting the skin and nervous system. Sturgeweber syndrome is a rare sporadic neurocutaneous syndrome the hallmark of which is a facial portwine stain involving the first division of the trigeminal nerve, ipsilateral leptomeningeal. Babies with sturgeweber syndrome sws are born with a facial birthmark known as a portwine stain. Literature discussing the relevance of ultrasound in sws is, however, scarce. In addition, people with sws have abnormal growth of blood vessels within the tissue that covers the brain and spinal cord leptomeningeal angioma. Paraskev stoyanov, varna journal of imab annual proceeding scientific papers 2008, book 1 abstract sturgeweber syndrome sws is a sporadic. Cerebral ultrasound cus can be a valuable noninvasive diagnostic tool for brain involvement in sturge weber syndrome sws. Sturgeweber syndrome sws is a rare congenital disease that affects the brain, skin, and eyes, and is a sporadically occurring neurocutaneous syndrome that affects intracerebral veins, which is associated with venous thrombosis. Sturgeweber syndrome sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder.
Intraoral examination revealed hypervascular changes in the ipsilateral mucosa, including a large soft expan sion of the hard palate. A report of a case with facial port wine stains, gingival overgrowth, and dilated ocular vessels is described. We report a case of 7 yearold girl with sturgeweber syndrome and discuss its clinicopathological features, differential diagnosis and. Pdf the sturgeweber syndrome sws or encephalotrigeminal angiomatosis is specifically congenital, nonhereditary, rare condition of unknown. Sturgeweber syndrome is a rare congenital neuro oculo cutaneous disorder. Cases journal management of patient with sturgeweber syndrome. Sturge weber angiomatosis is a rare, nonhereditary developmental condition characterized by a hamartomatous vascular proliferation involving the tissues of brain and face. Sturgeweber syndrome sws is a rare congenital disorder characterized by a facial vascular nevus associated with an ipsilateral. A 7 yearold boy, with a long history of ocular symptoms and an unspecified ocular surgery, presents with a painful blind left eye. The goal of the present paper is to report a case of a 34yearold white man with sturge weber syndrome presenting its inherent clinical features and to underline the importance of its diagnosis. Case study sturgeweber syndrome with intracerebral hemorrhage. Introduction the sturgeweber syndrome sws or encephalotrigeminal angiomatosis, or even craniofacial angiomatosis, is specifically congenital, nonhereditary condition of rare development 1,2, although the literature presents case reports inherited in an autosomal recessive and dominant manner 3, namely of unknown etiology, marked by angiomas involving the leptomeninges and the facial.
Sturge weber syndrome is a neurocutaneous disorder caused by persistence of transitory primordial arteriovenous connection of the foetal intracranial vasculature. Sturgeweber syndrome, portwine stains, gingival enlargement. Case report sturgeweber syndrome with osteohypertrophy. We report a case of 7 yearold girl with sturgeweber syndrome and discuss its clinicopathological features, differential diagnosis and also emphasize the importance of its diagnosis in the clinical oral practice. Case presentation this is case report of a 18yearold mentally disabled boy, with longstanding seizures, with a portwine nevi on the left side of the face along the distribution of trigeminal nerve. Intracranially, hemangiomatosis often calcified occurs over the cerebral cortex. Journal of indian society of pedodontics and preventive dentistry2008. An interventional case report of a yearold girl with sws who developed an exudative rd visual acuity hand motions that was treated with pdt. Sturgeweber syndrome sws is a rare and sporadic congenital neurocutaneous disorder, that is. A modified surgical approach nancy shi yin yuen1, ian yat hin wong2 1the hong kong ophthalmic associates, central, hong kong 2the eye institute, university of hong kong, pokfulam, hong kong pissn. Sturgeweber angiomatosis is a rare, nonhereditary developmental condition characterized by a hamartomatous vascular proliferation involving the tissues of brain and face. The patient reported that he had presented a cutaneous vascular nevus on the. The male patient had portwine tumoration on the left side of the face. It manifests with vascular malformations involving the brain, eye and skin with resulting neurological and orbital manifestations.
Sturgeweber syndrome with intracerebral hemorrhage. Encephalotrigeminal angiomatosis, sturge weber syndrome, port wine stain, neurocutaneous disorder. Case report oromaxillofacial osseous abnormality in sturgeweber syndrome. Interictal encephalogram showed bilateral slow activity, pronounced in the.
Our study reports 14 cases of sturgeweber disease, 6 male and 8 female patients, studied in follow up. Sturgeweber syndrome sws, also called as encephalotrigeminal angiomatosis, is a rare nonhereditary condition characterized by a facial cutaneous vascular nevus nevus flammeus or portwine stain in association with leptomeningeal angiomatosis. Leptomeningeal angioma are present in 100% of individuals with sturge weber syndrome. We herein report a rare case of sws with intracerebral hemorrhage derived from sinus thrombosis. Karthikeya patil 1, mahima v guledgud 1, ankita sahni 1. Pdf on aug 12, 2014, nihar ranjan mishra and others published sturge weber syndromea rare case report find, read and. Idiopathic inflammatory myopathy comorbid with sturgeweber. Sturgeweber syndrome is a congenital neurocutaneous disorder characterized by facial portwine stain, leptomeningeal angioma, and neurological disorders.
Case report open access sturgeweber syndrome coexisting with. A case report 1saleem shaikh, 2abdur rahman alatram, 3sachdeva harleen abstract sturge weber syndrome is a neurocutaneous disorder caused by persistence of. We report a case of a newborn with sws and serious brain abnormalities diagnosed on the first day of life with a cus. Kulkarni s, akhade sn, chandak r, shaikh f 2015 sturgeweber syndrome. It is characterized by a facial portwine stain and a leptomeningeal angiomata, frequently localized ipsilateral to the facial portwine stain. An apparently classical case report of sturgeweber. In 5 of 10 cases, the seizures started within the first year of life.
We report a case of 7 yearold girl with sturge weber syndrome and discuss its clinicopathological features, differential diagnosis and also emphasize the importance of its diagnosis in the clinical oral practice. Sturge weber syndrome is a rare non hereditary congenital sporadic disorder of elusive etiology. We report a case of sws with oral, ocular and neurological features. To report a clinical case of sturgeweber syndrome in a 29yeralold male patient who presented oral manifestations related to the syndrome. Sturgeweber syndrome sws is a rare congenital developmental, disorder manifesting with a facial portwine birthmark, and a vascular. This is case report of a 18yearold mentally disabled boy, with longstanding seizures, with a.
Hsj institute of dental sciences, panjab university, chandigarh, india 3 department of radiology, aarhus hospital, denmark. The patient reported that he had presented a cutaneous vascular nevus on the face during childhood as well as epileptic crisis episodes. Sturgeweber angiomatosis is a rare, nonhereditary developmental condition characterized by a hamartomatous vascular proliferation involving the tissues of. Sturge weber syndrome sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. Sws was diagnosed in 3 of the family members ii1, iii11 and iv6. Diagnosis, treatment, quantitative measures, and controversies. Oromaxillofacial osseous abnormality in sturgeweber syndrome.
We observed 10 cases since they presented seizures, and 4 cases for the presence of a severe picture of psychomotorial retardation. Sturge weber syndrome is a rare, congenital, neurooculocutaneous disorder which is characterised extraorally by unilateral port wine stains on the face, glaucoma, seizures and mental retardation, and intraorally by ipsilateral gingival haemangioma which frequently affects the maxilla or mandible. Localized choroidal haemangioma associated with sturge. Case report sturgeweber syndrome with osteohypertrophy of maxilla prashantbabaji, 1 anjubansal, 2 gopalkrishnachoudhury, 3 rashmitanayak, 4 ashokkodangalaprabhakar, 5 nagarathnasuratkal, 6 veenaraju, 7 andsureshs. Sturge weber syndromea case report rujuta joshi,bhagyashri bora, jayashri jadhav,priti mhatre abstract sturge weber angiomatosis is a rare, nonhereditary developmental condition characterized by a hamartomatous vascular proliferation involving the tissues of brain and face, port wine stains, dilated ocular vessels and glaucoma1. Gnaq mutation r183q as a potential cause of familial sturge. Sturge weber syndrome a case report rujuta joshi,bhagyashri bora, jayashri jadhav,priti mhatre abstract sturge weber angiomatosis is a rare, nonhereditary developmental condition characterized by a hamartomatous vascular proliferation involving the tissues of brain and face, port wine stains, dilated ocular vessels and glaucoma1. She was treated with a rst session of multispot pdt. It has a vast continuum of cutaneous, neurologic, ophthalmic and oral manifestations. Sturge weber syndrome is a congenital neurocutaneous disorder characterized by facial portwine stain, leptomeningeal angioma, and neurological disorders.
The pathologic process involved both the soft and osseous tissues of both the maxilla and mandible of the affected. Sturgeweber syndrome and discuss its clinicopathological features, differential diagnosis and also emphasize the. Case report bilateral sturge weber syndrome a rare case report. A case report theresa l mendonca, sunseamol t thomas email. The purpose of this study is to perform a case report of sturge weber angiomatosis, attended at a stomatology ser vice. Hsj institute of dental sciences, panjab university, chandigarh, india 2 department of pedodontia, dr. Sws affects male and female newborns at an incidence of approximately 1 in 20,00050,000 births. Port wine stain, glaucoma and seizures are some of the commonly seen symptoms, depending on the. Ct angiography illustrated osteohypertrophy with periostitis and associated soft tissue hypertrophy directly corresponding to the distribution of the cutaneous portwine stain. A case of sturge weber syndrome, uncommon in its intraoral extent, is presented.
In the present case, a 15yearold female patient presented with a port wine stain on the. To report a very rare unusual case of bilateral manifestation of sturge weber syndrome. The purpose of this study is to perform a case report of sturgeweber angiomatosis, attended at a stomatology ser vice. Sturgeweber syndrome sws is a rare congenital disease that affects the brain, skin, and eyes, and. Sturge weber syndrome is a rare sporadic neurocutaneous syndrome the hallmark of which is a facial portwine stain involving the first division of the trigeminal nerve, ipsilateral leptomeningeal. Case report bilateral sturge weber syndrome a rare case. Case report sturge weber syndrome with osteohypertrophy of maxilla prashantbabaji, 1 anjubansal, 2 gopalkrishnachoudhury, 3 rashmitanayak, 4 ashokkodangalaprabhakar, 5 nagarathnasuratkal, 6 veenaraju, 7 andsureshs. However, intracranial hemorrhage in patients with sws is rare. We report a case of 7 yearold girl with sturge weber syndrome and discuss its clinicopathological features, differential diagnosis and. Kaprelyan department of neurology, medical university prof.