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 Page number not for citation purposes 1 case report sturgeweber syndrome.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