Periodontal management of gingival enlargement associated with Sturge-Weber syndrome
A forty three year old female patient reported to the Department of Periodontics, JKKN Dental College and hospitals, complaining of swollen and bleeding gums in the maxillary right back tooth region for the last two years. The patient did not complain of any pain and difficulty during mastication. The patient was noticed the swelling before two years and gradually attain to present size. The swelling in the associated region had been gradually increasing since that time.\nThe patient apparently was healthy. She was mentally normal. There was no history of seizures. She was born full term and delivered normally.\nThe patient was observed to have a normal intelligence, a moderate build, and normal temperature, pulse, respiration and blood pressure. On extra oral examination, a hemihypertrophy was observed on the right side of the face with Port-Wine Stain []. Her mouth was deviated towards the right side of the face. There was increased malar prominence with increased nasal bridge width. Intra oral examination revealed soft, non-tender gingival enlargement involving right upper quadrant of the oral cavity []. Supra gingival calculus and plaque were noticed in both of the arches. Purplish – red discoloration was observed over the buccal mucosa on the right side and also present in the marginal gingiva, attached gingiva, and interdental papilla of 14 to 17 [].\nInvestigation that were carried out in this case included an orthopantomograph (OPG), Intra oral peri-apical radiographs (IOPA), complete hemogram, and a spiral computed tomography (CT) scan. The OPG revealed hemi facial hypertrophy in the right side of face []. The complete hemogram showed normal haemoglobin levels with no other abnormality in the total or differential white blood cell counts. Bleeding and clotting time were within normal limits. Spiral CT scan showed no abnormalities like calcification, atrophy with dilatation of ventricle and enlargement of sinuses []. Ophthalmologist investigated for any ocular disturbances like choroidal angioma, glaucoma, hemianopsia, or buphthalmos (enlargement of the coating of the eye), but no abnormalities were detected.\nThe finding of the various investigations and the clinical examination confirmed the diagnosis of SWS. However, Klippel-Trenaunay-Weber syndrome[] has a striking resemblance to SWS. Transitory nevus flammeus lesions during the neonatal period are common; however, they may not be as intense in colour as the Sturge-Weber lesion, which tends to be darker and most frequently is unilateral in distribution. The association of angiomatosis with other lesions may occur in disseminated hemangiomatosis, neurofibromatosis, Bannyan-Rily-Ruvalcaba syndrome[] and Coats disease.[]\nHistopathological examination of the excised tissue revealed normal epithelium with underlying connective tissue composed mainly of dilated and engorged blood vessels and a patchy distribution of chronic inflammatory cell infiltrate [].\nThe complete treatment procedures were explained to the patient, a written informed consent was obtained from the patient, and a medical consent was obtained from the general physician.\nInitially, a thorough scaling and root planing were done carefully to remove any local irritating factors that may have been responsible for the superimposed gingival inflammation. The patient was educated regarding good oral hygiene maintenance practices. The excisional biopsy was taken in relation to buccal side of 16, 17 by internal bevel incision. This biopsy was done under strict aseptic conditions using local anaesthesia (Lignocaine and adrenaline 1:80,000). The excised tissue was kept in 10% formalin and sent for histopathological examination. The patient was kept under observation for 24 hrs to watch for any bleeding from the biopsy site. However no post biopsy complications were observed.\nAfter one week, as the initial healing took place in the biopsy site, the patient was appointed for surgical intervention under antibiotic coverage (Amoxicillin 500 mg, three times a day) one day prior to the surgery. An internal bevel gingivectomy was performed in the region of teeth # 13 to 17 under strict aseptic conditions and local anaesthesia. There was profuse intraoperative bleeding that was controlled with the help of pressure packs. She was advised to continue the antibiotic-analgesic regimen (Amoxicillin 500 mg, three times a day; Ibuprofen 250 mg + Paracetamol 250 mg, three times a day) for seven days and to use 0.2% chlorhexidine mouth rinse for two weeks post operatively. The healing was satisfactory without any overt complications. The patient was advised to return for a check-up every three months thereafter to look for any sign of recurrence of the gingival enlargement up to 12 months []. No recurrence was noticed after 12 months of follow-up [].