Year : 2005 | Volume
: 23 | Issue : 2 | Page : 99--102
Total oral rehabilitation in a patient with portwine stains
BS Suprabha1, M Baliga2,
1 Department of Pedodontics and Preventive Dentistry, Manipal College of Dental Sciences, Mangalore, India
2 Department of Oral and Maxillofacial Surgery, Manipal College of Dental Sciences, Mangalore, India
B S Suprabha
«DQ»Shreyas«DQ», 5th Cross Road, Shivabagh, Kadri, Mangalore - 2, Karnataka
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.
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Suprabha B S, Baliga M. Total oral rehabilitation in a patient with portwine stains.J Indian Soc Pedod Prev Dent 2005;23:99-102
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Suprabha B S, Baliga M. Total oral rehabilitation in a patient with portwine stains. J Indian Soc Pedod Prev Dent [serial online] 2005 [cited 2020 Feb 16 ];23:99-102
Available from: http://www.jisppd.com/text.asp?2005/23/2/99/16452
Portwine stains represent hamartomatous capillary malformations and are named so due to the deep red hue that they leave on the skin or mucosa.  Their presence can signify a developmental anamoly involving central neural axis. Sturge-Weber syndrome is one such hamartoneoplastic syndrome characterized by presence of Portwine stains. It was first described by Schirmer and later more specifically by Sturge in 1879 and is also known as Sturge-Weber disease, Encephalotrigeminal angiomatosis, meningofacial angiomatosis, Sturge-Weber-Dimitri Syndrome, etc.  It is a congenital disorder due to dysfunction of embryonal vascular system resulting in hemangiomatosis. During the sixth week of i.u. life, a vascular plexus develops around the cephalic portion of the neural tube and under the ectoderm destined to become facial skin. Normally, it regresses at ninth week of intrauterine life. Failure of regression results in manifestations of Sturge-Weber disease. Variation in the degree of regression results in variation in clinical manifestation. 
The classic feature of this disease is angioma of leptomeninges. The other common clinical features are epilepsy (80%), dermal angiomas resulting in Portwine stains (76%), abnormal findings in skull radiographs (63%) mental retardation (54%), ocular involvement (37%) and hemiplegia (37%). , Characteristically, angioma of the leptomeninges occurs as a unilateral lesion, overlying the posterior temporal, parietal and occipital areas. Abnormalities of cerebrovascular system such as AV malformations, arterial thrombosis, subdural hematoma and cerebral atrophy can occur secondary to the angioma. The presence of angioma results in alteration of vascular dynamics causing precipitation of calcium deposits in cerebral cortex underlying the angioma. These are seen as gyriform calcifications in skull radiographs. Seizures, mental retardation, hemiplegia or hemiparesis develop secondary to this process and their severity depends on the extent of lesion. , Dermal angiomas commonly called Portwine stains occur as sharply demarcated vascular lesions and occur unilaterally along the dermatomes supplied by first two divisions of trigeminal nerve. A large variation has been reported in pattern of occurrence of these facial nevi; often they are bilateral or totally absent or may extend to neck, limbs and other parts of the body. Involvement of the area supplied by ophthalmic division of trigeminal nerve is pathognomonic. , Ocular involvement can result in glaucoma, choroidal haemangioma, bupthalmos or hemianopsis. 
Intra orally angiomatosis can involve lips, causing macrochelia, resulting in hemihypertophy of buccal mucosa, palate and floor of the mouth. Gingival enlargement can vary from light vascular hyperplasia to monstrous over growth making closure almost impossible. Pyogenic granulomas, unilateral hypertrophy of alveolus, ipsilateral premature eruption or delayed eruption, malocclusion are other abnormalities reported. , This syndrome is of rare occurrence and management becomes complicated due to risk of haemorrhage.
A 13-year old female patient reported to the dental clinic with a complaint of pain in lower right posterior region and bleeding gums.
History revealed that at 2 years of age, she developed tonic clonic convulsions and was treated with phenobarbitone. For the past 3 years, she was free of epileptic symptoms and was not on medication either. There was no sign of mental retardation. The patient was communicative but apprehensive. The family history was negative.
Extra oral examination revealed a limping gait, presence of Portwine stains on the face on the left-hand side extending to the right-hand side [Figure 1]. Portwine stains were also present on the neck, back, right arm and left leg [Figure 2]. Reddish discolouration of sclera of left eye was noted and was diagnosed to have choroidal haemangioma by the ophtalmologist. Facial asymmetry and macrochelia was noted with enlargement on the left side.
Intra oral examination revealed reddish discolouration of buccal mucosa, floor of the mouth, tongue and gingiva bilaterally. Enlargement of tongue was noted causing posterior open bite on the left side. Gingival enlargement on the upper anterior region and posteriorly on left side was prominent. Blanching on pressure was noted in the enlarged gingiva suggesting angiomatous enlargement. Teeth on the upper arch were malpositioned due to enlarged gingival, i.e. 12 and 22 were labially placed; 12, 23 and 24 were rotated. The DMFT was high with almost all posterior teeth either filled or decayed. Both lower first molars had deep caries. Oral hygiene was poor with extensive amounts of plaque [Figure 3], [Figure 4], [Figure 5].
A CT Scan was taken in early childhood and had revealed bilateral cortical atrophy. Radiographs of limbs and chest X-ray showed no abnormality. Blood picture was normal. A PA view revealed the classical gyriform intracranial calcifications and enlargement of soft tissue on the left side [Figure 6]. The IOPAs showed evidence of periapical pathology in relation to the lower molars. Based on clinical features and radiographic features of PA view, a diagnosis of Sturge-Weber syndrome was made.
Physician's consent was obtained prior to treatment. Root canal therapy was completed on lower molars and stainless steel crowns were cemented. Normal bleeding was noted during pulp extirpation. The decayed teeth were restored. A thorough plaque control regimen was started from day one to minimize gingival enlargement. It included oral prophylaxis carried out every week for the first month, use of chlorhexidine mouth rinse, oral hygiene instructions, taking plaque index and motivating the patient at each visit. After 1 month, the patient reported that no bleeding occurred during brushing and the degree of the gingival enlargement had also decreased marginally. It was decided to follow up the patient every month and continue with plaque control regimen [Figure 7] and [Figure 8].
Although the syndrome appears with a number of manifestations, they are highly variable. For diagnosis, evidence of angioma of the leptomeninges constitutes the minimum criterion for the disorder.  Differential diagnosis of this syndrome includes: Klippel Trenauny-Weber syndrome, Hereditary hemorrhagic telengectesia (Render Osler-Weber disease), Maffucci's syndrome, Von Hippel Lindau disease. , Portwine stains should be differentiated from nevus flammeus neonatorum which occurs as a isolated lesion, pyogenic granuloma and venous varicosities. 
The general management of the patient includes control of epilepsy and ocular lesions. Portwine stains on the face can be a cosmetic problem and should be treated by a plastic surgeon. A high dose of hydrocortisone given orally in infancy results in regression of well-localized small lesions.  . Other methods of treatment include dermabrasion, tattooing, lash lamp pulse tunable dye laser therapy. They can result in partial or complete clearing of the Portwine stains. Cryosurgery may be used to correct lip and other soft tissue deformities. ,
The dental rehabilitation of patients with Sturge-Weber disease is a complex process requiring initial conservative management and later surgery.  Behavioural problems may be encountered either due to previous exposure to hospital set up or due to mental retardation which demand the use of behaviour management techniques. 
Poor oral hygiene leading to secondary inflammatory gingival enlargement and high DMFT are often encountered. Patient and parent education and implementation of preventive procedures should be done.  A thorough plaque control regimen can go a long way in avoiding gingivectomy which is risky in these patients. Hancock et al.  achieved regression of gingival enlargement secondary to nifedipine therapy by vigorous plaque control measures only.
Endodontic treatment in these patients does not carry the risk of excessive haemorrhage as angioma may not involve the pulpal tissue. Bleeding can be controlled by cotton pellets and vasoconstrictors. However, precaution should be taken not to cause undue trauma to periapical tissue by over instrumentation to avoid haemorrhage, as occurrence of angiomas in the periapical region has been reported. 
Gingival enlargement is reported to be often super imposed by phenytoin induced gingival hyperplasia. A distinction should be made with the help of angiography.  Although phenobarbitone can also cause gingival hyperplasia,  in the present case, it should be noted that patient had discontinued the drug and the blanching effect seen on examination confirmed the angiomatous nature of gingival enlargement.
In spite of strict oral hygiene measures, angiomatous gingival enlargement poses a threat to gingival health and may at some point require gingivectomy.  Whenever a surgical procedure is planned, achieving hemostasis can be a significant problem making hospitalization mandatory.  The various methods used to manage risk of haemorrhage are:
• patient's blood typed and cross matched,
• provision for blood transfusion,
• use of haemostatic agents - topical bovine thrombin,
• use of postoperative splints,
• injecting sclerosing solutions,
• percutaneous transcatheter vascular embolization using gelfoam or polyvinyl alcohol. ,
Hylton  has described the use of CO2 LASER to perform gingivectomy in a patient with Sturge-Weber disease. With advent of NdYAG LASER which can be used in a contact mode with fibre optic hand piece, surgical procedures with lasers seems to be the best option in these patients. The advantages are immediate hemostasis, little damage to surrounding tissues leading to no postoperative pain, obtaining a sterile field and hence, no risk of secondary infection and no need for postoperative periodontal dressing. A combination of surgical gingivectomy and LASERs can be used if large bulk is to be removed.  Recurrence of the hemangiomatous growth following gingivectomy can occur. Wilson et al.  recommended the use of positive pressure splint to prevent recurrence. Maintaining good oral hygiene is vital even after gingivectomy.
The alveolar overgrowth, gingival enlargement and poor oral hygiene in these patients can affect the final outcome of orthodontic treatment. Orthognathic surgeries may be required once growth is completed in case of severe enlargement of the jaw bones.  Macroglossia can also contribute to malocclusion as in this case. Problems in speech, trauma during mastication are other secondary problems. Partial glossectomy combined with LASERs is the method of choice to manage macroglossia. 
Management of a patient with Sturge-Weber syndrome may be challenging due to risk of hemorrhage. Suitable armamentarium should be at disposal in case complication ensues. The dentist should focus on comprehensive therapy, starting with behaviour management and stress on preventive measures as with any patient with developmental disabilities.
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