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When a syndrome forms the background of a systemic involvement of periodontal disease, it is necessary to fully exploit the resources outside, which soon reaches its limits in private practice. In the patient's environment, it must be checked whether support for the patient can be guaranteed. Without support, as in this presented case, the patient's oral hygiene could hardly be maintained. This article reports on a female patient who was referred to the Center for Dental Medicine at the University of Zurich. In addition to various secondary carious lesions, an apical whitening, two carious wisdom teeth and two extremely mobile molars in the third quadrant, the patient had chronic, localized advanced (stage III, grade B) periodontitis associated with systemic disease (deafness and Sturge-Weber syndrome). For two years, the patient was treated at the Department of Periodontology. Due to the strong bleeding tendency on the left side, facial localization of the naevus flammeus, the patient was partially referred to the Polyclinic of Oral Surgery and treated there. Numerous oral hygiene sessions, scaling and root planing, restoration with composite fillings, a root filling, removal of wisdom teeth and finally removal of hypermobile molars 36 and 37 during corona lockdown were performed. In the meantime, the patient has been orally rehabilitated. Home oral hygiene was reorganized with the patient's family and the patient was discharged to a close supportive periodontal recall for the time being.

Phakomatoses refer to a heterogeneous and inconsistently defined group of neurocutaneous disorders. Tuberous sclerosis, neurofibromatosis types 1 and 2, Sturge-Weber syndrome and von Hippel-Lindau disease are entities with typical findings in neuroimaging studies STANDARD RADIOLOGICAL METHODS: These findings are usually easily depicted on magnetic resonance imaging (MRI) studies, thus, making diagnosis easier.

Das Sturge-Weber-Syndrom (SWS) ist eine seltene, kongenitale Erkrankung, die das Gehirn, die Haut und die Augen betrifft. Es handelt sich um ein sporadisch auftretendes neurokutanes Syndrom mit Beteiligung intrazerebraler Venen (venose Angiomatose). Die Haufigkeit wird auf 1:50.000 Geburten geschatzt [1]. Leitsymptom ist eine intrakranielle leptomeningeale Angiomatose, die meist den Okzipital- und posterioren Parietallappen betrifft und ein- aber auch beidseitig vorkommen kann. Ipsilateral treten hierzu faziale kutane vaskulare Veranderungen in Form eines Portweinnavus auf, die ublicherweise im Versorgungsgebiet des Trigeminusastes V1 zu finden sind. Andere mit dem SWS assoziierte klinische Erscheinungen sind Anfalle, Glaukom, Kopfschmerzen, transiente neurologische „schlaganfallahnliche“ Episoden und kognitive Beeintrachtigungen. Neurologisch finden sich oft eine Hemiparese, Hemiatrophie und Hemianopsie kontralateral zu den kortikalen Veranderungen.

Sturge-Weber syndrome is a rare congenital disorder consisting of cutaneous, leptomeningeal and ocular vascular malformations. Glaucoma occurs in 30-70 % of patients. Treatment is challenging due to a high risk of severe complications such as expulsive haemorrhage, massive choroidal effusion and serous retinal detachment. Ocular findings and the occurrence of complications under different treatment modalities have been reviewed.A retrospective analysis of a case series of 5 children (5 eyes/mean age 5.6 years) with secondary glaucoma associated with Sturge-Weber syndrome was undertaken. Ocular findings, treatment modalities, intraocular pressure and complications were assessed.Facial port-wine nevus, i.e., nevus flammeus and dilated episcleral vessels were present in all cases. Diffuse choroidal haemangiomas were seen in four eyes. Glaucoma surgery was performed in 4 children, one child was treated with antiglaucomatous medication alone. Reversible uveal effusion and subluxation of the lens appeared postoperatively in one eye, persistent serous retinal detachment occurred 3 years after surgery in another eye (with an intraocular pressure of 10 mmHg). Both complications were found in eyes with diffuse choroidal haemangioma.Management of glaucoma associated with Sturge-Weber syndrome is difficult and controversial. Medical treatment often does not decrease intraocular pressure sufficiently. When planning surgical intervention an increased risk of severe complications has to be considered, especially in the presence of diffuse choroidal haemangioma.

The purpose of this study was to evaluate ultrasound, MRI, and CT investigations in children with Sturge-Weber syndrome.From 1996 to 2005, a total of 15 investigations of the brain performed in 6 children with Sturge-Weber syndrome were reviewed. We evaluated four ultrasound, five CT, and six MRI exams.With ultrasound an increase of the echogenicity of the periventricular white matter was depicted in the first days of life. MRI is the best modality to demonstrate the vascular malformation, the impaired cerebral venous drainage, and the atrophy of one hemisphere.One-sided periventricular hyperechogenicity on ultrasound can be an early sign of Sturge-Weber syndrome in children with a nevus flammeus. MRI is the method of choice to diagnose Sturge-Weber syndrome and to follow up these children if the neurological status of the patients changes.

Part 2 mainly presents intrinsic lesions as the most common brain tumors (astrocytoma of all grades, ependymoma), arising in the region of the visual pathway. A second focus is on vascular pathology, not only infarction, but cavernoma and venous malformation in Sturge-Weber syndrome. The third topic deals with inflammatory processes such as multiple sclerosis or sarcoidosis.

The classification of vascular anomalies is controversial since the same terms are applied for different lesions. The biological classification according to Mulliken and Glowacki is based on the correlation of cellular features of vascular lesions during infancy and childhood with the physical examination and natural history. On this basis, two main categories of vascular anomalies can be distinguished: 1. Hemangiomas with endothelial proliferation and clinical course of rapid postnatal growth followed by slow involution. 2. Vascular malformations as developmental anomalies with normal endothelial cell cycles. These are subdivided according to the part of the vascular system involved.

The considerable variety of diseases involving both the skin and the peripheral or central nervous system is due to the developmental relationship between skin and nervous system. Diseases of the nervous system resulting in skin changes comprise disorders of nails, hair, sweat and sebaceous glands as well as neuropathic ulcers in various manifestations. Neurocutaneous syndromes (phacomatoses) are nevoid systemic diseases comprising neurofibromatoses, tuberose sclerosis, ataxia telangiectasia, nevoid angiomatoses and other neurocutaneous disorders. Polytopic disorders of the skin and nervous system may be due to infectious diseases, diseases of the connective tissue, fat, inflammatory vascular and granulomatous disorders.

The authors report on a case where the Klippel-Trenaunay symptom complex was combined with a Sturge-Weber-Krabbe syndrome, which, however, was only noticeable during pregnancy by fetal macrosomy and hydramnios. The child was delivered on term by Caesarean section and presented no paediatric problems; however, during the first few years of its life mental retardation became clearly evident.

A patient with obstructive sleep-apnea-syndrome and congenital temporal and cervical hemangiomas developed severe headache under nasal BiPAP ventilation. Internal hydrocephalus was diagnosed by CCT and MRI and rise of intracranial pressure (ICP) under artificial ventilation was supposed. Polysomnography, ICP-measurement with a ventricular catheter and resistance to outflow-measurement were used to clarify differential diagnosis. During periods of apnea ICP was raised severely with a maximum in REM-sleep. With nasal ventilation no apnea was observed and ICP was normalized. Resistance to outflow was normal. A disturbance of CSF-dynamics or CSF-absorption leading to the patient's headache could be excluded. Duplex-sonography and cerebral angiography did not provide further information. After exclusion of other causes artificial respiration dependent swelling of the subcutaneous temporal and cervical hemangiomas was the most likely cause triggering the headache. A sufficient therapy however was not yet available. Probably the patient suffers from an abortive form of a Sturge-Weber-Krabbe-Dimitri-Syndrome.

Clinical presentation of a child with port-wine stain and seizures leads to the suspicion of Sturge-Weber disease (SWD). This diagnosis can be confirmed by the detection of a meningeal angiomatosis. In rare cases, early detection of meningeal pathology by ultrasound has been reported. Key findings are brain atrophy, gyriform cortical calcifications demonstrated by skull radiographs after the first year of life or earlier by cranial CT, and dys- or aplasia of the deep cerebral veins on angiography. Radionuclide imaging shows focal or diffuse tracer accumulation over the affected brain regions. MR demonstrates an abnormal appearance of the affected meninges, especially thickening and pathologically increased signal intensity after Gd-DTPA application. This, in association with the demonstration of abnormal enhancement in deep medullary veins, is the most characteristic finding. Contrast-enhanced MR allows early and non-invasive diagnosis of SWD, mainly by revealing leptomeningeal angiomatosis and abnormal venous vessels.

We report on symptoms, clinical course and prognostic factors of eight children (6 girls and 2 boys) with Sturge-Weber syndrome. The period of observation was 1 to 17 years (mean 8.8 years). All patients had the classical symptoms with facial port-wine stain nevus and intracranial calcifications. In two cases a bilateral distribution of the nevus was found. All patients but one had epileptic seizures which were followed by Todd's palsies in five. Four children additionally suffered from hydrophthalmia. Another patient had an angiomatosis of the retina and cataract. Neurological findings were normal in three patients, minimal signs were found in three, one patient had a spastic hemiplegia and another one hypotone tetraplegia. The mental development was normal in three patients, however severe dyscalculia occurred in one of them. Five patients were mentally retarded, three of them severely. The prognosis was the worse the earlier the cerebral seizures appeared.

1. Congenital angiodysplasias of the extremities are often associated with skeletal disorders, i.e., overgrowth (local giantism, hyperplasia) or reduced growth (hypoplasia). From a clinical point of view three types of combined lesions may be differentiated: (a) a secondary local giantism induced by hemodynamically active congenital a.v. fistulas, usually of intra- and extraosseous location (Weber-type); - (b) local giantism in combination with venous and/or lymphatic angiodysplasias; in this group skeletal overgrowth seems not to be caused by the vascular anomalies but represents more probably a coordinated "inborn error" of tissue composition and distribution (Klippel-Trenaunay-type); - (c) hemangiomatosis usually of venous (cavernous) type, affecting soft tissues as well as bones in association with skeletal hypoplasia. There is a retardation of bone growth by substitution and destruction of the epiphysial cartilages by the intraosseous hemangioma (Servelle-Martorelltype). In most cases (a-c) the use of special angiological investigations allows a clear diagnosis particularly concerning the pathogenesis of skeletal disorders. 2. In looking for an unequivocal terminology the clinical classification of these anomalies should be based on the leading clinical symptome, i.e., the giantism or the angiodysplasia. In this respect the vascular findings take on a predominant position. Paying regard to historical aspects the classification may be completed by the additional term "Klippel-Trenaunay" type, "Weber" type, "Servelle-Martorell" type. 3. An exact angiologic diagnosis gives further informations about prognosis and choice of treatment (group I: Early operative elimination or reduction of the a-v shuntvolume; group II and III: "wait and see!"; usually conservative treatment with compression bandages).

Childhood epilepsies (not including the first 2 years of life) are outlined and discussed; particular emphasis is laid upon the variety of certain forms of epileptic conditions and their clinical course. These forms are divided as follows: a) The Lennox-Gastaut syndrome: a poly-etiological condition with distinct clinical-ictal and electroencephalographic characteristics, mostly associated with mental defects and prognostically unfavorable. b) "Common generalized epilepsy" (also called "centrencephalic" epilepsy), characterized by petit mal absences or a combination of petit mal and grand mal and with a predominantly favorable prognosis. c)Childhood epilepsies with focal spikes in the EEG, in most cases a very benign form with an excellent prognosis. These 3 forms of seizure disorders may be divided in subgroups. The distinction of fine diagnostic nuances is quite helpful but requires well integrated epileptological and EEG experience. The special role of temporal lobe epilepsy is briefly discussed. Furthermore, several etiologies of childhood etiologies are singled out such as inborn errors of metabolism (lipidoses, amino-acidurias), essential hereditary myoclonus epilepsy, tuberous sclerosis, Sturg-Weber's disease, encephalitis, brain tumor and brain abscess. The fringe of the seizure ("borderland of epilepsy") is briefly delineated.

The vascular system of the head is of great importance in most types of headache because of the sympathetic efferents supplying the vessels. From neuroanatomic data it is to be assumed that vascular diseases of different genesis and cardiovascular diseases may give rise to headaches. The connection between headache and hypertensin is discussed at length with reference to pathophysiology. Further, headache is presented as a symptom with reference to observations of our own cases of inflammatory diseases of the vessels, sinus thrombosis, vessel deformities, accidents in anticoagulant therapy with subdural and intraspinal haemorrhages, cerebrospinal lues and endocrinopathies. Tables and illustrations supplement the text.

A rare form of angiomatous dysplasia with hypotrophy of the left arm is described. Numerous haemodynamically significant arterio-venous fistulae could be demonstrated in the soft tissues and in bone. The venous and lymphatic systems were normal. The lacunar reticular-cystic changes in the spongiosa of the phalanges are pathognomonic of arterial angiodysplasia of the Weber type. In most cases this is associated with bone hypertrophy.

An 18 year old patient is reported who suffered from cerebral convulsions, originating in the right hemisphere, beginning at age fourteen. Explorative trepanation of the scull was carried out after confirmation of (1) discreet neurologic disturbances on neurologic examination in the right hemisphere, (2) focal sign on the right side in the EEG (focal slowing and focal sharp wave), and (3) a right-parietal increase of radioactive activity in the scintigram. Cerebral angiographia, insufflation encephalographia, skull x-rays and the ophthalmologic examinations were without pathologic findings. During trepanation a macroscopically typical finding of Sturge-Weber-syndrome could be demonstrated (angioma capillare et venosum) covering almost the entire right posterior hemisphere.

Thin-layer computed tomography of the orbit together with contrast representation by computed tomography in form of circulation studies is the only non-invasive method to represent intraocular hemangiomatous tumors hidden under an exsudative detachment of the retina. This method allows to establish exact tumor parameters which are necessary for an efficient therapy. In this case, radiotherapy with Cs137 is applied.

A rare case of episcleral and orbital varix in a 16-year-old female is reported. The diagnosis was confirmed by CT scan and ultrasonography, and showed during Valsalva's maneuver a typical expansion of the orbital tumor. CT scan showed the tumor to be composed of a plexus of dilated vessels. During Valsalva's maneuver a 10 mm Hg rise in the venous pressure in the episcleral varix was noted, which suggests a direct communication between the episcleral and the orbital varices.

A 12 year old patient was treated for Sturge-Weber syndrome with choroidal haemangioma and total exsudative retinal detachment. The diagnosis was supported by echography and thin-layer computed tomography. Because of the exsudative retinal detachment photocoagulation could not be performed. Treatment by percutaneous radiotherapy with Caesium 137 was performed. The retina was reattached by application of 30 Gray. A flat pigmented scar developed in the area of the haemangioma at the posterior pole of the eye. No side effects appeared. The follow-up time is 26 months.

Up until now 39 cases of combined Klippel-Trénaunay syndrome and Sturge-Weber syndrome have been described. Here follows the report of a girl, now 4 years of age, displaying a full combination of these syndromes. Only a small part of the body surface is not covered with naevi teleangiectatici laterales. The patient has clear hypertrophy of the left cheek and of the left lower extremity, less noticeable on the left upper extremity. For therapeutic reasons the left side of the head and the left lower extremity were thoroughly angiographically examined--this revealed typical abnormalities. The vessel-alteration of the lower extremity are not extremely far developed and arteriovenous fistulas on a large scale are also absent. This allows us to dismiss the F.P. Weber syndrome on the one hand, while it explains the absence of complications of the Klippel-Trénaunay syndrome, as described in literature, on the other. The significance of the alterations of lymph nodes in this disease, which we are the first to describe, is at present not fully clear. The cerebral attacks have until now showed only a temporary response to medication.

The CT criteria of the Sturge-Weber syndrome are described on the basis of 5 cases observed by the authors. Computerized tomography makes it possible to identify earlier - and in some cases more clearly - than by conventional roentgenography, the classical signs of the disease (calcification of the cerebral cortex, unilateral shrinking of the skullcap, distension of the frontal and sphenoidal sinus, thickening of the skullcap as a whole). Anomalies of vascularization, which had so far been demonstrable only by invasive methods, can also be demonstrated direct by means of computerized tomography.

For the first time, a report is given on the percutaneous radiotherapy of hemangiomas of the choroid which could not be treated by photocoagulation. Diagnosis and control examinations of these tumors were performed by ophthalmoscopy, fluorescence angiography, and ultrasonography. Ten eyes (nine patients, three out of them with Sturge-Weber's disease) were treated between 1967 and 1982. A first group (five eyes, five patients) treated until 1974 received a target volume dose of 1,65 to 6,5 Gy combined with subsequent photocoagulation. All these eyes could be preserved. In addition, the second group (five eyes, four patients) treated since 1975 with unique radiotherapy showed better results with respect to visual acuity. The average follow-up period was four years (seven months up to nine years). Late effects were not observed. Even in case of advanced disease with retinal ablation and secondary glaucoma, an inactivation of the hemangioma of the choroid and a simultaneous regression of the secondary alterations could be achieved. According to our experiences, enucleations can be prevented by an irradiation with a target volume dose of 20 to 30 Gy.

Following a case presentation clinical and radiological signs of the Sturge-Weber-Krabbe syndrome as well as its differentiation into three clinical entities according to Poser and Taveras are discussed. Calcification within the atrophic cerebral cortex is a predominant sign. Angiomatous lesions of the external ascending and descending cerebral veins, and occasionally of the internal cerebral veins may be demonstrated by angiography. Calcifications of the cerebral cortex demonstrable on plain skull radiograms diminish the diagnostic value of angiography because of their pathognomic significance. Hyperplasia of the frontal and ethmoid sinuses and thickening of the calcaria are probably directly related to the cerebral atrophy.

From the broad spectrum of contrast-free radiological diagnosis of the skull, diseases with asymmetrical appearance of the face were selected and the syndromes similar to them are presented from the differential diagnostic point of view. Asymmetry of the head very frequently indicates a disease of the skull area and should be subjected to appropriate diagnostic investigations.