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We present the case of a pregnant young woman with symptomatic diffuse choroidal hemangioma (CH), diagnosed with Sturge-Weber syndrome (SWS). Three months postpartum, there was full spontaneous resolution of the subretinal fluid and improvement in the visual acuity (VA). A 29-year-old, 31-week pregnant female with a coagulation disorder and enoxaparin (clexane) treatment, complained of a left visual disturbance of 2 weeks duration. On examination, the right eye was normal; left eye VA was 6/12 (20/40) with a left eyelid port-wine stain and on funduscopic examination there was a serous retinal detachment. On ultrasound, there was diffuse thickening of the choroid with a focus superior and nasal to the disc with active vascularization - a diagnosis of diffuse CH was made. Magnetic resonance imaging (MRI) and MR venography studies of the brain and orbit revealed engorged orbital vasculature, and the diagnosis of SWS was made. Three months after Cesarean section with birth of a healthy baby boy, VA returned to 6/9 with full resorption of subretinal fluid. On fundoscopic exam, there were no retinal or vein occlutions but there were tortuous blood vessels with A-V crossing. This is a rare case of newly diagnosed SWS with symptomatic diffuse CH in pregnancy. Postpartum, there was full resolution of the subretinal fluid. A watchful waiting approach can be considered in such cases. [ABSTRACT FROM AUTHOR]

Sturge–Weber syndrome (SWS) is a neurocutaneous syndrome characterized by capillary venous malformations in the skin, eye, and brain with the occurrence of angiomas of the face, choroid, and leptomeninges. The characteristics signs include facial port-wine birth mark, glaucoma, choroidal hemangioma with brain malformations, and cognitive abnormalities. In this article, we have documented the case report of a 24-year-old male with SWS presenting with glaucoma. [ABSTRACT FROM AUTHOR]

Pediatric cerebral hemiatrophy is a rare entity with widespread etiology. Imaging in form of computed tomography and magnetic resonance imaging is helpful in differentiating between the etiologies of hemiatrophy. We describe imaging findings in three different cases of hemiatrophy due to Sturge-Weber syndrome, Dyke-Davidoff-Masson syndrome, and Rasmussen encephalitis. [ABSTRACT FROM AUTHOR]

A case study of 13-year-old female child born of non-consanguineous marriage presented to the dermatology outpatient services with dark-red discolouration over the face. Topics include Cutaneous examination revealed reddish-blue hyperpigmentation over the bilateral cheek and periocular region; and multiple linear and dotted vessels with an additional detection of homogenous greyish-black pigmentary area.

Nevus flammeus is the most common benign congenital capillary malformation, often known as a port-wine stain. Sturge–Weber syndrome (SWS) is a congenital, sporadic, nonfamilial disease characterized by intracranial and ophthalmic vascular anomalies and nevus flammeus. It usually manifests as developmental delay, learning problems, paralysis, seizures, glaucoma and attention deficit, and hyperactivity disorder. A 29-year-old male patient presented with a reddish patch over the face since birth. He was found to have hemihypertrophy of face, hemiparesis of right limbs, and low intelligence quotient. On ophthalmic examination, the patient was found to have glaucoma and only perception of light in the left eye. Computed tomography brain showed atrophy of the left cerebral hemisphere and calcifications in the left frontal, parietal and occipital regions. With these findings, he was diagnosed as SWS type I. A multidisciplinary approach was followed for patient evaluation and management. This case also highlights the irreversible sequelae of this rare phacomatosis. [ABSTRACT FROM AUTHOR]

Asymptomatic erythematous patches are mostly congenital vascular malformations that may be a part of syndromes like Sturge-Weber or Klippel Trenaunay syndrome. Acquired erythematous lesions are difficult to diagnose with differentials ranging from acquired port-wine stain to early morphea. We are reporting a case of a middle aged woman suffering from early morphea hardly distinguishable from Port-wine stain that was further complicated by her acquiring lymphatic filariasis in the same leg. [ABSTRACT FROM AUTHOR]

Encephalotrigeminal Angiomatosis is a rare developmental phakomatoses characterized by the occurrence of nevus flammeus (port-wine stain) along the distribution of branches of trigeminal nerve, vascular angiomas in the eye, and leptomeningeal angiomas affecting 1 in 1,00,000 South Asian population. Herewith, such a rare case of such encephalotrigeminal angiomatosis in a 24-year-old male is described. [ABSTRACT FROM AUTHOR]

Sturge-Weber syndrome (SWS) includes facial, leptomeningeal and choroidal hemangioma. The retinal vasculature is essentially normal. Rare cases of retinal vascular tortuosity and arterio-venous malformations have been reported. We report two cases with rare concomitant retinal vascular abnormalities along with SWS. Both the patients had nevus flammeus, hemifacial hypertrophy, and choroidal hemangioma. In one case, retinal cavernous hemangioma was seen in the affected eye. The other case revealed retinal neovascularization secondary to proliferative diabetic retinopathy in the eye with choroidal hemangioma. [ABSTRACT FROM AUTHOR]

Encephalotrigeminal angiomatosis, also called Sturge–Weber syndrome (SWS), is a syndrome of etiology which is not yet clear. It is a nonhereditary condition. The clinical features include pigmentation over the facial skin known as port-wine stain, abnormalities of ocular region, and central nervous system involvement as leptomeningeal angioma. In this manuscript, we present a rare case report with an unusual combination of SWS with facial lobular capillary hemangioma. [ABSTRACT FROM AUTHOR]

Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder which was known to occur in 1 in 50,000 births with a triad of features including the port-wine stain on the face, glaucoma, and leptomeningeal angiomas. It is further divided into three types based on the regions involved. Here, we present the case of an 18-year-old male, Type II SWS with facial involvement since childhood who was presented with affective symptoms. The psychiatric manifestations ranging from symptomatic to a diagnostic presentation to be kept in mind which are often missed in the patients presented with SWS. [ABSTRACT FROM AUTHOR]

Abstract A 9-year-old boy with the diagnosis of Sturge-Weber Syndrome, and port-wine stain involving the right side of the face, presented with acute visual loss of the right eye. Examination revealed a high intraocular pressure and a combined cilioretinal artery and inferior hemi-retinal vein occlusion in the right eye. Systemic work-up was negative. After controlling the intraocular pressure, gradual improvement of vision was noticed with simultaneous disappearance of signs of vascular occlusion. [ABSTRACT FROM AUTHOR]

Klippel–Trenaunay–Weber syndrome (KTWS) is a rare congenital malformation, characterized by constellation of arteriovenous malformation, cutaneous capillary malformation, and skeletal or soft-tissue hypertrophy. The exact cause of KTWS is unknown. We present a case of a 1 ½-year-old boy with multiple swellings over the right lower extremity since birth which increased in size in proportion to his general growth. He was diagnosed clinically as Klippel–Trenaunay syndrome (KTS) which on Doppler imaging and on histopathology showed the presence of a multichanneled slow-flowing arteriovenous malformation. KTWS should be distinguished from KTS since clinical features, management, and prognosis of these two entities are distinctly different. Although the diagnosis is primarily clinical, confirmation requires laboratory and imaging studies. The present case is being reported as an interesting case which highlights the fact that distinction between KTWS and KTS may sometimes be difficult. [ABSTRACT FROM AUTHOR]

The article presents a case of a 19-year-old male who was rushed to a hospital due to multiple painless bluish-red skin lesions on the dorsum of his right foot since early childhood and profuse bleeding following local trauma to discuss Stewart-Bluefarb syndrome (SBS). Also cited are the differential diagnosis of his condition like Parkes Weber syndrome (PWS), Klippel-Trenaunay syndrome (KTS), and verrucous hemangioma.

The article presents a case study of a 44-year-old man with complaints of raised skin lesion in front of and above the right ear. Topics include ipsilateral leptomeningeal angiomatosis, glaucoma, seizures, stroke and intellectual disability; and histopathology of nodule from port‑wine stain usually showing well‑circumscribed proliferation of thin and thick‑walled blood vessels.

This report demonstrates the blood flow pattern in a case of choroidal hemangioma (CH) using swept-source-optical coherence tomography angiography (SS-OCTA). Fluorescein angiography, SS-OCT, and SS-OCTA images of a patient with CH were obtained using a standard protocol. The internal vascular pattern of the tumor was identified on both OCT and OCTA. Dark areas were identified in the CH. These were interspersed between areas of visible blood flow, as imaged on SS-OCTA. Peripheral vascular arcades were also identified within the tumor. SS-OCTA should be evaluated as an imaging tool to study the blood flow within choroidal tumors. [ABSTRACT FROM AUTHOR]

A 6-year-old girl presented with blurred vision and was found to have elevated intraocular pressure (IOP) and glaucomatous optic disc damage in both eyes. She also displayed capillary malformations on the face (port-wine stain), upper back and all four limbs, angiomatosis in the brain and had hypertrophy of the left upper and lower limbs typical of overlapping Sturge-Weber syndrome and Klippel-Trenaunay syndromes. She was initially managed with IOP lowering topical medications but required trabeculectomy in the right eye followed by Ahmed valve implantation in both eyes. Despite multiple measures over a 7-year period, her IOP still remained uncontrolled with gradual progression of the glaucomatous damage. This case exhibits a very rare occurrence of overlapping syndromes reported only a handful of times in literature. Most cases with Sturge-Weber syndrome have ipsilateral glaucoma affecting the eye on the same side as the port-wine stain. This case presented with bilateral refractory childhood glaucomas, which is exceedingly rare. [ABSTRACT FROM AUTHOR]

Sturge.Weber syndrome (SWS), also known as encephalotrigeminal angiomatosis, is a rare vascular neurocutaneous alteration. It is characterized by facial nevus . usually unilateral, seizures, hemiparesis, intracranial calcification, mental retardation and ocular involvement. Nevertheless each case of SWS is unique and exhibits the characteristic features to a varying degree. We report here a case of SWS who presented with features suggestive of this syndrome, and having bilateral facial nevus as well. [ABSTRACT FROM AUTHOR]

The Sturge-Weber syndrome is a rare uncommon neurocutaneous disorders with angiomas involving the leptomeninges (Leptomeningeal Angiomas) and skin of the face, typically in the ophthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve. The cutaneous angioma is called a Port-Wine Stain. It is commonly referred to as "Sturge-Weber syndrome after Sturge and Weber who first described this affliction in 1879. This article present a case of Sturge-Weber syndrome associated with gingival enlargement, its management and follow-up results. [ABSTRACT FROM AUTHOR]

Encephalotrigeminal angiomatosis (Sturge-Weber syndrome) is a rather uncommon congenital condition characterized by the combination of venous angioma of the leptomeninges over the cerebral cortex with ipsilateral angiomatous lesions of the face, and sometimes the skull, jaws, and oral soft tissues. A case of portwine stain with intraoral gingival hemangioma is presented. There were no other systemic manifestations. Patient reported with a complaint of localized tumor-like swelling in gums. Based on the presence of sharply demarcated vascular lesion unilaterally on the face and with ipsilateral intraoral vascular hyperplasia in the lip and gingiva, a variant of encephalotrigeminal angiomatosis was diagnosed. Ultrasound Doppler flowmetry was used to determine the blood flow. Dental management included plaque control instructions, scaling, root planning, and excision of the lesion done under general anesthesia. Close follow-up and meticulous plaque control have kept the oral condition under fairly good control. [ABSTRACT FROM AUTHOR]

We report a case of a suspected Sturge–Weber syndrome variant diagnosed at the age of 58 with the help of enhanced depth imaging spectral‑domain ocular coherence tomography (EDI‑SDOCT). A 58‑year‑old female with unilateral glaucoma was suspected to have choroidal vascular lesion, conjunctival angioma, and no facial port‑wine stain who presented to the clinic with bleb dysesthesia many years after trabeculectomy. EDI‑SDOCT was performed and revealed markedly increased choroidal thickness in the affected eye. EDI‑SDOCT may be helpful in diagnosing Sturge–Weber variants without facial involvement and may aid in the investigation of the pathogenesis of this disease. [ABSTRACT FROM AUTHOR]

Sturge-Weber syndrome (SWS) is characterized by a facial capillary hemangioma (port wine stain) involving the periorbital area, forehead or scalp, a venous angioma of the leptomeninges and a choroidal angioma. Following normal early development, children with SWS often experience neurological and developmental deterioration in association with the onset of seizures and consequently suffer a high rate of disability. The classical imaging finding in these children is the evidence of calcification in the occipital area. The striking features of the EEG in SWS are the attenuation and the excess of slow activities. Recent advances in the diagnosis and treatment, particularly the surgical options are discussed. [ABSTRACT FROM AUTHOR]

Sturge-Weber syndrome is an uncommon condition characterized by presence of Portwine stains on the face along with ocular disorders, mental retardation, oral involvement and leptomeningeal angiomas. A report of a case with atypical manifestations of this syndrome along with a step-by-step protocol oral rehabilitation of such patients is described. [ABSTRACT FROM AUTHOR]

A letter to the editor focusing on Sturge-Weber syndrome, a sporadic-congenital syndrome characterized by capillary-venous angiomas is presented.

Phacomatosis pigmentovascularis (PPV) is a rare cutaneous congenital malformation syndrome, characterized by the simultaneous occurrence of cutaneous vascular and pigmentary anomalies. Only one case of phacomatosis cesioflemmea has been reported so far from India. We hereby report a second case of the disorder in a two year old boy, who presented with Mongolian spot and nevus flemmus. Our case had systemic involvement in the form of Sturge Weber Syndrome (SWS) with glaucoma, buphthalmos and MRI findings of cerebral cortical atrophy and enlarged choroid plexus. [ABSTRACT FROM AUTHOR]

Angiomatosis is a diffuse vascular lesion which clinically mimics hemangioma or vascular malformation. It usually involves multiple tissues and is histopathologically characterised by proliferation of vessels of varying calibre intimately admixed with large amount of adipose tissue. Its surgical removal is very difficult because of its infiltrative nature. Therefore, a precise histopathological diagnosis is of utmost importance. It is usually seen in females in the first two decades and commonly involves lower extremities. Angiomatosis of head and neck region is very rare. Here we present a rare case of angiomatosis of the lower face involving right cheek and lip in a 4-year-old boy clinically diagnosed as hemangioma. Histopathological differential diagnosis of angiomatosis is also discussed. [ABSTRACT FROM AUTHOR]

Phakomatosis pigmentovascularis (PPV) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi. Four types and two subtypes have been described where subtype ‘a’ present only with cutaneous form and subtype ‘b' also with systemic association like in Sturge‑Weber syndrome or Klippel‑Trenaunay syndrome. Hereby, we report a case where our patient presented with port‑wine stain, Nevus of Ota, Sturge‑Weber syndrome, and Klippel‑Trenaunay syndrome; which has made it a rare combination. [ABSTRACT FROM AUTHOR]

The article presents a case study of 36‑year‑old male presented with new onset recurrent left focal Seizure with secondary generalization for 48 hours. It mentions diagnosis of Sturge‑Weber syndrome (SWS) Type 3 which can present in adults for the first time, usually with seizures or migraine; and also mentions volume loss in the right parieto-occipital lobe with gyriform cortical hypointensity in T2‑WI.

The article presents a case study of a 22-year-old female reported with progressive visual diminution in both of her eyes and diagnosed with bilateral sturge-weber syndrome (SWS). Clinical examination of the patient indicates facial port-wine staining leptomeningeal angioma ipsilateral as SWS' most striking neurological feature. Also noted is the fact that impairment in ocular, dermal and central nervous system does not preclude SWS diagnosis.

Sturge--Weber syndrome (SWS) is a rare, sporadically occurring neurocutaneous disorder with a frequency of approximately 1 per 50,000. The hallmark is an intracranial leptomeningeal vascular angioma in association with a port wine nevus, usually involving ophthalmic or maxillary distribution of trigeminal nerve. Other clinical findings associated with SWS are seizures, glaucoma, hemiparesis and mental retardation. The radiological hallmark is "Tram-line" or "Gyri-form" calcification. 25 to 56% of patients experience recurrent episodes of paroxysmal focal neurological deficits in form of transient hemiparesis, which may be due to vascular ischemia or postictal in origin. EEG helps to differentiate the exact etiology, as it is normal in former. Aspirin prophylaxis in those, due to ischemia decreases their recurrences and improves overall neurological prognosis. We report a 25-month-old child of SWS with recurrent episodes of transient hemiparesis and atypical midline location of facial vascular nevus. [ABSTRACT FROM AUTHOR]

A case study of a 45‑year‑old female who is diagnosed with Sturge–Weber syndrome is presented. Topics discussed include provisional diagnosis of SWS with postpartum psychosis was made; examination revealed left‑sided capillary hemangioma with proptosis, band‑shaped keratopathy; and need for special psychiatric care along with comprehensive interdisciplinary management.

Vascular lesions represent one of the rare disorders affecting overall quality of life of a child. A wide variety of these conditions are known, ranging from a simple nevus to life-threatening hemangiomas. These conditions make the treatment options more complex due to the fear of uncontrollable bleeding. The present case is one of the rare combinations of Sturge-Weber syndrome and pyogenic granuloma. Conditions of importance and treatment options keeping hemangioma in mind are discussed. [ABSTRACT FROM AUTHOR]

Sturge-Weber syndrome (SWS) is a rare, sporadic neurocutaneous syndrome characterized by a classical triad of facial port wine nevus, ipsilateral leptomeningeal angiomatosis (LAM) and glaucoma. The incidence of SWS is 1/50,000 live births, although it is more often underreported. The incidence of SWS without facial nevus is not known, although very few patients without facial nevus have been reported. In these patients, the diagnosis of SWS is made by the findings of computed tomography, magnetic resonance imaging, and histopathology. Here, we report three patients with SWS from our cohort of 28 patients with SWS without facial nevus and discuss their clinical profile and outcome. [ABSTRACT FROM AUTHOR]

Cutis marmorata telangiectatica congenita (CMTC) is a congenital cutaneous vascular anomaly evident as persistent cutis marmorata, telangiectasia and phlebectesia. Klippel-Trenaunay-Weber syndrome (KTW) consists of port wine stains, varicose veins and limb hypertrophy. A term baby was delivered shocked with an extensive cutaneous malformation resembling a port wine stain with hypertrophied forearm and fingers. Deep substance hemangiomas were seen in the liver raising the possibility of KTW syndrome. With resuscitation over the next few days, the malformation evolved into a more marbled appearance with axillary phlebectesia. This along with the presence of syndactyly raised the potential for CMTC but did not explain the liver hemangioma or bony hypertrophy. In this case, there some features of CMTC and some of KTW syndrome raising the possibility of their coexistence in the same case. Sturge-Weber syndrome, KTW syndrome and CMTC may be included in a spectrum of vascular diseases that are associated with other developmental defects of the mesoderm during embryonic life. The variability in presentation with overlap, which is seen in these conditions, may have a common pathophysiology explained by the Happle gene hypothesis. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 21-year-old male suffering with encephalotrigeminal angiomatosis or the Sturge-Weber (SWS), was posted for open reduction and internal fixation of fracture left ankle. The clinical features of patient were port wine stains with hemiparesis. The patient was premedicated with midazolam intravenously (IV) and anaesthesia was maintained with N2O:O2 ratio of 60:40 and one to two percent sevoflurane, with intermittent doses of vecuronium.

Sturge-Weber syndrome is a rare sporadic condition of mesodermal phakomatosis, characterized by purple-colored flat cutaneous cranial (face) hemangiomas (most commonly along the trigeminal nerve), glaucoma and vascular lesions in the ipsilateral brain and meninges. Klippel-Trenaunay syndrome is also an uncommon mesodermal phakomatosis characterized by a triad of cutaneous and visceral hemangiomas, venous varicosities and soft tissue or bone hypertrophy. Sturge-Weber syndrome in combination with Klippel-Trenaunay syndrome is unusual. Because of the rarity, we report here a 3-year-old boy who presented with overlapping features of both the syndromes. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented regarding the transient stroke-like episodes that are an uncommon presentation in Sturge-Weber syndrome, which is a rare sporadic neurocutaneous syndrome.

Klippel—Trenaunay syndrome and Sturge—Weber syndrome are rare disorders with neurologic and cutaneous signs of vascular origin. Phakomatosis pigmentovascularis represents the association of widespread, aberrant, and persistent nevus flammeus and pigmentary abnormalities. We describe a case with features suggestive of overlap between them. A ten-month-old boy presented with seizures, developmental delay, skin lesions on face, trunk and legs, buphthalmos and right lower limb hypertrophy. CT scan of head showed atrophy of brain and calcification. Our case had overlap of Klippel—Trenaunay syndrome and Sturge—Weber syndrome with phakomatosis pigmentovascularis [ABSTRACT FROM AUTHOR]

Sturge-Weber angiomatosis is a rare, nonhereditary developmental condition characterized by a hamartomatous vascular proliferation involving the tissues of brain and face. A report of a case with facial port wine stains, gingival overgrowth, and dilated ocular vessels is described. [ABSTRACT FROM AUTHOR]

A 6½ year-old female child has been described. The condition is characterized by port wine stains in the body, massive gingival overgrowth, history of convulsive disorder, and dilated ocular vessels. [ABSTRACT FROM AUTHOR]

A 15-year-old boy with Sturge-Weber syndrome underwent strabismus surgery (oculus sinister [OS]) for the treatment of exotropia. The patient's visual acuity (OS) decreased to hand motion 10 days after the surgery. One month after the surgery, the patient's visual acuity decreased to light perception, and a fundus examination showed total exudative retinal detachment (OS). [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "A rare presentation of bilateral Sturge-Weber syndrome," by N. Nema, J. Jain, and V. Porwal in the 2014 issue, which cites the case of a four-month-old infant with bilateral Sturge-Weber syndrome, myopia, and early onset convulsion.

A letter to the editor is presented which discusses a rare paediatric case of Klippel-Trenaunay-Weber syndrome.

The Sturge Weber syndrome is characterized by developmental delay, seizures in infancy, unilateral cutaneous lesions with ipsilateral leptomeningeal enhancement. We report an unusual presentation of Sturge Weber syndrome with bilateral port wine nevus on the trunk and face along with bilateral cortical involvement in a developmentally normal child with progressive megalencephaly. [ABSTRACT FROM AUTHOR]

The article presents a study which shows the neuroimage of a five-month-old female infant with accelerated myelination in the affected hemisphere which is a diagnostic feature in sturge weber syndrome (SWS). The infant was presented with three episodes of right focal motor seizures and cerebral atrophy involving the left fronto-temporal region was observed with the use of magnetic resonance imaging (MRI). Early detection of SWS help initiate appropriate AED or definitive surgical treatment.