|Year : 2008 | Volume
| Issue : 5 | Page : 29-31
Sturge-Weber syndrome: A case report
Clinical Tutor, North Bengal Dental College, Sushurtnagar, Darjeeling, West Bengal, India
North Bengal Dental College, Sushurtnagar, Darjeeling, West Bengal
Source of Support: None, Conflict of Interest: None
| Abstract|| |
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.
Keywords: Angiomatosis, convulsion ocular, port wine stain
|How to cite this article:|
Mukhopadhyay S. Sturge-Weber syndrome: A case report. J Indian Soc Pedod Prev Dent 2008;26, Suppl S1:29-31
| Introduction|| |
Sturge- Weber syndrome More Details More Details or encephalotrigeminal angiomatosis is a rare nonhereditary developmental condition. It is characterized by angiomatosis of face (nevus flammeus) with a variable distribution sometimes matching the dermatomes of one or more divisions of trigeminal nerve. , The port wine stain is a congenital malformation of the dermis that involves venules, capillaries, and possibly perivenular nerves  It occurs in an estimated three per thousand births.  Most lesions appear on the face and approximately 5% of patients have associated ocular involvement, mental retardation, and seizures due to the involvement of the vasculature of eye and the central nervous system. A constellation of findings is called Sturge-Weber syndrome.  Oral changes occur in 40% cases of this syndrome and may include massive growth of the gingiva and asymmetric jaw growth.  Intracranial convolutional calcification is discernible on skull radiograph  Most potent vascular lesions will blanch under pressure. When intraluminal clots are formed, they become palpable and the lesion will not blanch usually. Thrombi in angioma may eventually calcify and such lesion will feel hard on palpation. The calcified nodules or phlebolith may be radio graphically evident. When located on the surface of the skin or oral mucosa port wine stains are readily identified. Large lesions are warm and may even be pulsatile if associated with a large vessel. The blood vessels have simple endothelial lining and little connective tissue stroma. Such lesions characteristically bleed profusely when traumatized. This study illustrates a case of Sturge-Weber syndrome in a 6½-year-old girl.
| Case Report|| |
A 6½-year-old female child was brought to the Department of Pedodontis, North Bengal Dental College, Siliguri with chief complaint of gum bleeding and a bluish red swelling on the right side of the face. The lesion was gradually enlarging. The bluish red lesion (port wine stain) was seen in other parts of the body also. Her parents and brother accompanied the patient. The patient was elder of the two siblings born of a non-consanguineous marriage at full term by cesarean delivery. Her parents and brother appeared normal. Family history did not reveal any similar complaint from her immediate or distant relatives. The patient had a history of convulsion. The last episode took place one month ago. She did not take any medicine for this disorder. The Patient was moderately built; she was a class one student in a nearby rural school.
Extraoral examination revealed the port wine stain or nevus flammeus had a bilateral distribution along multiple segments of trigeminal nerve [Figure 1]. Additional port wine lesions were found elsewhere in the body [Figure 2]. Hands, chest, abdomen, back, ear, leg, and even skin of the head were also involved. Ipsilateral (right) side of the body was more affected. The lesion was present since birth and gradual enlargement was noticed. When digital pressure was applied on the port wine stain blanching was readily identified. No bruit or thrill was heard on auscultation in the face. But pulsation was present in the other port wine stains of the body. Examination of eyes revealed that the blood vessels in the right eye were dilated [Figure 3]. Left eye appeared normal. Parents were advised to visit ophthalmology department.
Intraoral examination revealed massive gingival over growth on the right side of the oral cavity. The oral hygiene was poor, but the amount of gingival over growth was in consistent with the amount of plaque present. The gingiva in the left side appeared normal [Figure 4]. Unilateral vascular involvement of soft palate and buccal mucosa were noticed. There was history of spontaneous exfoliation of primary teeth. Anterior teeth were mal aligned [Figure 5]. Restorative treatment was carried out on carious teeth. On the both upper and lower lips appeared normal. History of spontaneous gum bleeding and bleeding on brushing was present. Lateral skull radiograph showed evidence of calcification in temporal lobe [Figure 6].
| Discussion|| |
Hemangiomas appear predominantly at or around birth. They are the most common tumor of infancy and are more common among females.  Vascular malformations are frequently called hemangionas, but their pathogenesis is distinct.  They are true developmental anomalies and although they are always present at birth they may not be apparent. They do not proliferate nor do they involute, rather undergoes a slow steady hypertrophy. Progressive dilatation of preformed vascular channels results in hypertrophy. It is primarily the size of the vascular channels and not the number that causes hypertrophy. 
Many investigators believe that hemangioma and vascular malformations are different. Hemangioma appears weeks after birth, results from increase in number of capillaries, shows rapid growth, often circumscribed, involutes spontaneously, shows less recurrence, no bruit on auscultation. Whereas vascular malformation results from dilatation of arteries, veins and capillaries, usually present at birth, grows with patients, poorly circumscribed, difficult to resects, recurs, produces bruit. 
Port wine stains in childhood are classically faint, pink macules, tend to darken progressively to red purple; may be isolated with well-delineated border or may be very diffuse. It has usually unilateral distribution along one or more segments of trigeminal nerve. Occasionally bilateral involvement or additional port wine lesions are found elsewhere in the body.  In the present study, bilateral involvement of the face and other parts of the body was seen. The lesion was extensive on the right side along the branches of trigeminal nerve. Right eye was also involved. Vascular malformation of eye is seen when ophthalmic branch of trigeminal nerve is involved. Sullivan et al . found that patients with involvement of ophthalmic branch of trigeminal nerve were at risk for full development of the condition.  Affected individuals also have leptomeningeal angioma that overlie the ipsilateral cerebral cortex. Meningeal angiomatosis is usually associated with a convulsive disorder and sometimes contralateral hemiplegia and mental retardation.  In the present study, patient had a history of convulsion.
Intraoral involvement is common, resulting in hypervascular changes to the ipsilateral mucosa. The gingiva in the present study showed a massive hemangiomatous proliferation that felt soft on palpation. Such gingival overgrowth might be attributable to the increased vascular component. 
Heller et al , stated that chief morbidity produced by the port wine stain was psychological. Personality development is adversely affected in virtually all patients by negative reaction of others to a "marked person".  Differential diagnosis includes angio-osteodystrophy syndrome, characterized by port wine stains in face, varices and hypertrophy of bone. Usually long bones are involved. Rendu-Oslar-Weber syndrome is another rare hereditary condition characterized by abnormal dilatation of terminal vessels of skin, mucosa, and occasionally viscera. Epistaxis is most common. Bleeding from oral cavity is also common .Diagnosis is based on clinical findings, hemorrhagic history, and family history. Other rarer angiomatous syndromes are Maffuci syndromes (multiple angiomas of skin and chondromas of bone) and Von Hippel Lindan disease (afamilial syndrome involving hemangioblastoma in the retina and cerebellum, and renal cysts).
The treatment for port wine stains depends on the site and extant of the lesion. The choices are laser photo coagulation, sclerotherapy, surgical resection, and a combination of these. Some lesions may not be curable and should be treated for symptom relief. Surgery should be considered especially if magnetic resonance imaging (MRI) shows the lesion to have a defined border and to be accessible. For lesions that cannot be operated patient is advised to sleep with the head elevated at an in incline of at least 45° for the rest of their lives. Significant venous distention of these vessels occurs over the years as a result of supine positioning.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
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