|Year : 2014 | Volume
| Issue : 4 | Page : 287-290
Gingival overgrowth associated with port-wine stains: A case report of Sturge-Weber syndrome
Panthula Veerendranath Reddy, Koduganti Rekha Rani, Pinnamaneni Indumathy, Dasari Rajashree
Department of Periodontics, Panineeya Mahavidyala Institute of Dental Sciences, Dilsukh Nagar, Hyderabad, Andhra Pradesh, India
|Date of Web Publication||10-Dec-2014|
Department of Periodontics, Panineeya Mahavidyala Institute of Dental Sciences, Road No 5, Kamala Nagar, Dilsukh Nagar, Hyderabad - 500 060, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Sturge-Weber syndrome is a rare neurocutaneous disorder, also known as encephalotrigeminal angiomatosis. It has varied systemic and oral manifestations, and the management of oral condition in such a case is a dental practitioner's challenge. In this case report, we describe a rare clinical presentation of Sturger-Weber syndrome with dermatological and intraoral manifestations, but without any neurological involvement.
Keywords: Gingival overgrowth, periodontal, Sturge-Weber
|How to cite this article:|
Reddy PV, Rani KR, Indumathy P, Rajashree D. Gingival overgrowth associated with port-wine stains: A case report of Sturge-Weber syndrome. J NTR Univ Health Sci 2014;3:287-90
|How to cite this URL:|
Reddy PV, Rani KR, Indumathy P, Rajashree D. Gingival overgrowth associated with port-wine stains: A case report of Sturge-Weber syndrome. J NTR Univ Health Sci [serial online] 2014 [cited 2020 Apr 4];3:287-90. Available from: http://www.jdrntruhs.org/text.asp?2014/3/4/287/146662
| Introduction|| |
Sturge-Weber syndrome is a neuro-cutaneous disordercharacterized by the presence of a facial port-wine stain, a leptomeningeal angioma, and occular abnormalities, most commonly gaucoma.  The angiomatosis and skin changes follow the maxillary and ophthalmic distribution of the trigeminal nerve.  Sturge-Weber syndrome, first described by Schirmer and later on by Sturge in 1979  is classified according to Roach scale  as:
- Type I: Both facial and leptomeningeal angiomas (LA); may have glaucoma.
- Type II: Facial angioma alone; may have glaucoma.
- Type III: Isolated LA; usually no glaucoma.
Its prevalence is approximately 1/50,000 live births; there are equal chances of males and females being affected, and there is no racial bias.  The classic feature of this disease is angioma of leptomeninges, and other common clinical characteristics are epilepsy, dermal angiomas resulting in port-wine stains, abnormal findings in skull radiographs, mental retardation, ocular involvement and hemiplegia. 
Facial lesions, more specifically, rosy-purple nevus flammeus lesions are seen, and ocular involvement present in the form of choroidal angioma, glaucoma, hemianopsia, or buphthalmos.  Oral manifestations seen in about 38% of cases, may involve hemangiomatous lesion.  The most common oral manifestation is gingival overgrowth restricted to the ipsilateral maxilla or mandible. 
This paper presents a case report of a patient with Sturge-Weber syndrome characterised by port-wine stains, glaucoma, and ipsilateral manifestation of gingival enlargement.
| Case report|| |
A 24-year-old female patient reported to our outpatient department, with a chief complaint of food impaction, swollen and bleeding gums, for the past 2 years. Patient gave a history of discoloration of the face since birth. The patient was apparently healthy. Patient confirmed that there is no relevant family history. Queries related to the neurological symptoms proved negative, patient did not give previous history of convulsions. This was her first dental visit. Patient was educated and motivated for dental therapy.
On extra oral examination, facial discoloration was noted covering the entire right half of the face [Figure 1]. Ocular involvement was seen bilaterally [Figure 2]. Marked facial asymmetry manifesting as right facial hemihypertrophy was seen. Macrochelia was prominent with edematous, swollen lips deviated towards right side of the face and incompetent.
|Figure 1: Right-sided hemihypertrophy of the face with port-wine stains; deviation of the mouth toward the right side of the face; swollen, edematous, and incompetent upper and lower lips|
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|Figure 2: Ocular lesions present more prominent on the right side of the face|
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On intra-oral examination, patient manifested poor oral hygiene. There was gingival overgrowth on the right upper and lower quadrants, more pronounced in relation to maxillary canine (12, 13) and mandibular posteriors (35, 36, 37) [Figure 3]. Gingival overgrowth was soft, sessile, edematous and tender. There was no angiomatous gingival overgrowth on the left upper and lower quadrants [Figure 4]. No intra oral reddish discoloration was observed. The patient was provisionally diagnosed with Sturge-Weber syndrome.
|Figure 3: Preoperative intraoral view manifesting gingival overgrowth on the right side|
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|Figure 4: Preoperative intraoral view showing normal gingiva on the left side of the oral cavity|
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Investigations carried out, included orthopantamogram, computed tomography (CT) scan and complete blood picture. The orthopantamogram showed severe alveolar bone loss in relation to the right maxillary and mandibular quadrants (missing 16, bone loss in relation to 17, 15, 36, 37 >50%) [Figure 5]. CT scan however exhibited a normal picture with no intracranial manifestations [Figure 6]. Blood picture was conclusive of microcytic hyporchromic anemia.
As the patient was found to be anemic, she was referred to the general physician for the same. She was also advised to seek consultation with an ophthalmologist and dermatologist. The patient initially underwent nonsurgical periodontal therapy, which included scaling, root planning and stringent plaque control regimen. Patient was recalled after a month, for surgical procedure, by this time, her hemoglobin levels had risen to normal levels, and patient was considered fit to undergo surgical therapy.
A combination of gingivectomy and flap surgery was performed for a patient under local anesthesia (lignocaine with adrenaline 1:80,000) [Figure 7]. There was excessive intra operative bleeding due to the angiomatous nature of the lesion, that was managed using pressure packs, and flap was sutured back with 3-0 mersilk nonresorbable sutures. Flap surgery was complimented with systemic antibiotic and analgesic therapy. Patient was asked to abstain from brushing on the operated side for a week, and put on chemical plaque control (chlorhexidine 0.2% mouth rinse for a week). Suture removal was done after a week. Healing was uneventful. Patient was recalled after a month, and henceforth every 3 months for maintenance therapy. Patient was followed-up for 2 years and showed no signs of recurrence of gingival overgrowth [Figure 8].
Histopathologic examination revealed numerous proliferating capillaries in the connective tissue, suggestive of Sturge-Weber angiomatosis, confirming provisional diagnosis [Figure 9].
|Figure 9: H and E, ×10 photomicrograph showing numerous capillaries with extravasated red blood cells|
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| Discussion|| |
Sturge-Weber syndrome is a rare, congenital neurocutaneous syndrome characterized by unilateral facial cutaneous vascular malformation (nevus flammeus or port-wine stain) in association with ipsilateral leptomeningeal angiomatosis.  According to Roach Scale classification,  this patient could be classified under Scale 2 category. The patient was concerned about her facial port-wine stains. She was asked to consult a dermatologist for the same, and was advised laser therapy. Furthermore, patient was referred to an ophthalmologist for treatment of ocular manifestations.
Intra-oral involvement in this particular case, was restricted to ipsilateral gingival enlargement extending to the upper and lower right quadrants, without any intraoral port-wine stains. The gingival overgrowth associated with Sturge-Weber syndrome could be as a result of angiomatous proliferation of the involved gingival tissue, antiepileptic medication or a combination.  Since this patient had no neurological involvement or history of antiepileptic medication, the gingival overgrowth presented could be purely attributed to angiomatous proliferation.
Periodontal management includes proper mechanical debridement, motivating the patient to follow a stringent plaque control regimen and surgical management. For the present case, gingival overgrowth was managed by a combination of gingivectomy and flap surgery. Although excessive bleeding is an impending risk in these patients, it is of utmost importance to treat the gingival overgrowth so that the patient would be able to maintain oral hygiene, thus preventing further periodontal deterioration.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]