Benign Tumor of Jaws Question And Answers

Benign Tumor Of Jaws Question And Answers

Question 1. Write short note on Sturge-Weber syndrome.
Answer. It is also known as encephalotrigeminal angiomatosis.

Classification Sturge-Weber syndrome

• Complete trisymptomatic: Here all three organ systems, i.e. eye, skin and central nervous system are involved.
• Incomplete bisymptomatic: In this, involvement is either oculocutaneous or neurocutaneous.
• Incomplete monosymptomatic: In this, there is neural or cutaneous involvement.

Sturge-Weber syndrome Etiology

• Due to dysplasia of embryonal vascular system which leads to hemangiomatosis. This occurs in 6th week of intrauterine life.
• The syndrome affects the precursor of tissue which develops in promesencephalic and mesencephalic neural crest. This leads to vascular malformation.
• It can arise from aberrant migration of mesodermal and ectodermal elements inside the brain and the meninges in the fetal growth.

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Sturge-Weber syndrome Clinical features

• Port-wine stain: This is seen at birth. Its color ranges from light pink to deep purple.
• Neurological features: Angiomas are seen at the time of birth. Patient is also suffering from seizures. Many of the patients are suffering from epilepsy. In few cases, patient feel weakness over opposite side to port-wine stain. Headache may also occur.
• Eyes: Glaucoma can be present. This occurs due to mechanical obstruction of angle of eyes. If glaucoma is not treated for longer time there is reduction in vision and blindness may occur.

Sturge-Weber syndrome Oral Manifestations 

• Vascular hyperplasia occurs in oral mucosa.
• enlargement may also occur.

Sturge-Weber syndrome Radiological Features

• Conventional radiography: This shows presence of classical tramlines or tram-track or trolley-tract calcification of skull.
• Computed tomography: This shows brain atrophy, enlargement of choroid plexus and abnormal draining veins.
• Angiography: This shows lack of superficial cortical veins, abnormal tortuous vessels and non-filling of dural sinuses.

Sturge-Weber syndrome Management 

• Port-wine stain should undergo cosmetic treatment. Lesion is covered by camouflage.
• LASER: It is done for removing port-wine stain.
• Anticonvulsants are given for treating seizures.
• Antiglaucoma treatment should be given.
• Because of the syndrome, hemorrhage may occur in oral cavity while doing dental treatment. So it should be carefully managed.