Journal of Pharmacy And Bioallied Sciences
Journal of Pharmacy And Bioallied Sciences Login  | Users Online: 132  Print this pageEmail this pageSmall font sizeDefault font sizeIncrease font size 
    Home | About us | Editorial board | Search | Ahead of print | Current Issue | Past Issues | Instructions | Online submission




 
 Table of Contents  
CYTOLOGY SECTION - CASE REPORT
Year : 2012  |  Volume : 4  |  Issue : 6  |  Page : 349-352  

Sturge-Weber syndrome


1 Department of Oral and Maxillofacial Surgery, Vivekanandha Dental College for Women, Tiruchengode, India
2 Department of Periodontology and Implantology, Vivekanandha Dental College for Women, Tiruchengode, India
3 Department of Periodontology and Implantology, Meenakshi Ammal Dental College, Chennai, Tamil Nadu, India

Date of Submission01-Dec-2011
Date of Decision02-Jan-2012
Date of Acceptance26-Jan-2012
Date of Web Publication28-Aug-2012

Correspondence Address:
Natarajan Manivannan
Department of Oral and Maxillofacial Surgery, Vivekanandha Dental College for Women, Tiruchengode
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-7406.100304

Rights and Permissions
   Abstract 

Encephalotrigeminal angiomatosis (Sturge-Weber syndrome) is a rather uncommon congenital condition characterized by the combination of venous angioma of the leptomeninges over the cerebral cortex with ipsilateral angiomatous lesions of the face, and sometimes the skull, jaws, and oral soft tissues. A case of portwine stain with intraoral gingival hemangioma is presented. There were no other systemic manifestations. Patient reported with a complaint of localized tumor-like swelling in gums. Based on the presence of sharply demarcated vascular lesion unilaterally on the face and with ipsilateral intraoral vascular hyperplasia in the lip and gingiva, a variant of encephalotrigeminal angiomatosis was diagnosed. Ultrasound Doppler flowmetry was used to determine the blood flow. Dental management included plaque control instructions, scaling, root planning, and excision of the lesion done under general anesthesia. Close follow-up and meticulous plaque control have kept the oral condition under fairly good control.

Keywords: Hemangioma, portwine stain, Sturge-Weber syndrome, ultrasound doppler


How to cite this article:
Manivannan N, Gokulanathan S, Ahathya RS, Gubernath, Daniel R, Shanmugasundaram. Sturge-Weber syndrome. J Pharm Bioall Sci 2012;4, Suppl S2:349-52

How to cite this URL:
Manivannan N, Gokulanathan S, Ahathya RS, Gubernath, Daniel R, Shanmugasundaram. Sturge-Weber syndrome. J Pharm Bioall Sci [serial online] 2012 [cited 2020 Oct 20];4, Suppl S2:349-52. Available from: https://www.jpbsonline.org/text.asp?2012/4/6/349/100304

Sturge-Weber syndrome is a rare congenital disorder characterized by the presence of leptomeningeal angiomas, portwine stains (sharply demarcated vascular lesions that occur unilaterally along the dermatomes supplied by first two divisions of trigeminal nerve), along with ocular disorders, mental retardation, and oral involvement. It was first described by Schirmer and later more specifically by Sturge in 1879. It is also known as Sturge-Weber disease, encephalotrigeminal angiomatosis, meningofacial angiomatosis, and Sturge-Weber-Dimitri syndrome. [1]

It is a congenital disorder occurring due to dysfunction of embryonal vascular system, resulting in hemangiomatosis. The classic feature of this disease is angioma of the 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%). [1],[2]

Portwine stains or nevus flammeus represent hamartomatous capillary malformations and are named so due to the deep purple hue that they leave on the skin or mucosa. [3] Intraorally, angiomatosis can involve lips, causing macrochelia, and also buccal mucosa, palate, and floor of the mouth. Gingival enlargement can vary from light vascular hyperplasia to monstrous overgrowth, making closure almost impossible. Pyogenic granulomas, unilateral hypertrophy of alveolus, ipsilateral premature eruption, or delayed eruption and malocclusion are the other abnormalities reported. [1],[4] This syndrome is of rare occurrence and management becomes complicated due to the risk of hemorrhage.

This paper presents a case report of a patient with portwine stain and intraoral hemangioma.


   Case Report Top


A 20-year-old female patient reported with a chief complaint of pain and bleeding gums while brushing in left upper posterior region and for replacement of a poorly constructed bridge in the upper anterior region. She was unable to follow proper oral hygiene because of recurrent bleeding from the swelling in the left upper posterior region.

Patient's history revealed that the lesion started 3 years back and slowly attained the present stage. She was free of any epileptic symptoms and there was no sign of mental retardation. The patient was communicative but apprehensive. The family history revealed no relevant findings.

Extraoral examination revealed the presence of portwine stains on the left-hand side of the face, extending along the second division of the trigeminal nerve unilaterally [Figure 1]. Facial asymmetry and macrochelia was noted with enlargement on the left side.
Figure 1: Extraoral view

Click here to view


Intraoral examination revealed prominent reddish purple gingival enlargement posteriorly on the left side [Figure 2] and [Figure 3]. Blanching on pressure was noted in the enlarged gingiva, suggesting angiomatous enlargement. Upper anterior teeth had a broken fixed partial denture. Teeth on the lower arch were malpositioned. Oral hygiene was poor with extensive amounts of plaque and calculus.
Figure 2: Intraoral view of gingival hemangioma

Click here to view
Figure 3: Intraoral occlusal view (mirror image)

Click here to view


Chest X-ray and PA view of the skull showed no abnormality. Blood picture was normal. Ultrasound Doppler flowmetric study [Figure 4] and [Figure 5] was done to determine the blood flow in the growth. Treatment plan consisted of oral prophylaxis, surgical excision of the lesion under general anesthesia (GA), and replacement of the worn out bridge in the upper anterior region.
Figure 4: Color Doppler of the intraoral lesion

Click here to view
Figure 5: Spectral Doppler flowmetry of the intraoral lesion

Click here to view


The scope of the treatment and the goals were explained and informed consent was obtained from the patient. A thorough plaque control regimen was started from day 1 to minimize the inflammatory component of the gingival enlargement. It included oral prophylaxis, use of chlorhexidine mouth rinse, oral hygiene instructions, and motivating the patient at each visit.

Intralesional injection with sodium tetradecyl sulfate was given once a week for 2 weeks as a pretreatment to the excision of the growth. [5],[6] Because of the risk of bleeding, surgical excision was done under GA following hospitalization. The excision of the growth was done using electrosurgery [Figure 6]. Periodontal pack was placed after the patient regained consciousness. Patient was given postoperative instructions and discharged after 24 hours.

Histopathologic examination of the excised tissue revealed excessive number of dilated blood vessels lined by endothelial cells in the connective tissue matrix, confirming the lesion to be a hemangiomatous lesion [Figure 7].
Figure 6: Electrosurgical excision under GA

Click here to view
Figure 7: Histopathologic view showing numerous dilated capillaries

Click here to view


Patient was reviewed on the 7 th postoperative day. One-month follow-up showed satisfactory healing of the gingiva at the operated site [Figure 8]. A newly constructed fixed partial denture was given to replace the old one in the region of the upper anterior teeth. Oral hygiene instructions were reinforced and the patient was made to understand its importance. Sixth month follow-up showed complete regression of the lesion and her oral hygiene maintenance was satisfactory [Figure 9] and [Figure 10].
Figure 8: One month postoperative view showing satisfactory healing

Click here to view
Figure 9: Sixth month postoperative view showing remission of the lesion

Click here to view
Figure 10: Sixth month postoperative view showing remission of the lesion

Click here to view



   Discussion Top


Although Sturge-Weber syndrome appears with a number of manifestations, they are highly variable. In the case presented here, there were no systemic manifestations except for portwine stains on the face and intraoral hemangioma. Unless the vascular lesions include the region innervated by the ophthalmic branch of the trigeminal nerve, usually the patient does not have central nervous system involvement.

Portwine stains on the face can be a cosmetic problem and if needed should be treated by a plastic surgeon. A high dose of hydrocortisone given orally in infancy results in regression of well-localized small lesions. [7] Other methods of treatment include dermabrasion, tattooing, and 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. [8],[9] Although all the treatment modalities were explained to the patient, she was not interested in correcting the extraoral lesion.

The dental rehabilitation of patients with Sturge-Weber disease is a complex process requiring initial conservative management and later surgery. [10] Poor oral hygiene leading to secondary inflammatory gingival enlargement is often encountered. Patient education and implementation of preventive procedures should be done. [11] A thorough plaque control regimen can go a long way in avoiding gingivectomy which is risky in these patients. Hancock et al. [12] achieved regression of gingival enlargement by vigorous plaque control measures alone.

Drug-induced gingival enlargement is reported to be often superimposed on the existing hemangioma when the patient is under epileptic medication, as epilepsy is one of the common clinical features in these patients. [1],[2] A distinction should be made between hemangioma and drug-induced gingival enlargement with the help of angiography. [13] Although phenobarbitone can also cause gingival hyperplasia, [14] in the present case, it should be noted that the patient had no history of epilepsy 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. [9] Whenever a surgical procedure is planned, achieving hemostasis can be a significant problem making hospitalization mandatory. [2] The various methods used to manage the risk of perioperative hemorrhage are: [15]

  1. Patient's blood typed and cross matched
  2. Provision for blood transfusion
  3. Use of hemostatic agents-topical bovine thrombin
  4. Use of postoperative splints
  5. Injecting sclerosing solutions
  6. Percutaneous transcatheter vascular embolization using gelfoam or polyvinyl alcohol.


Electrosurgery was preferred in this case since electrosurgical procedures have several advantages which include the following: [16]

  1. Clean tissue separation, with little or no bleeding
  2. Clear view of the surgical site
  3. Planing of soft tissue is possible
  4. Access to difficult-to-reach areas is increased
  5. Time and operator fatigue are reduced
  6. The technique is pressureless and precise.



   Conclusion Top


Despite their benign origins and behavior, hemangiomas in the region of oral cavity are always of clinical importance to the dental profession and require appropriate clinical management. Dental practitioners and oral surgeons need to be aware of these lesions because they may pose serious bleeding risks. Suitable armamentarium should be at disposal to manage any complications that may arise.

 
   References Top

1.Gorlin RJ, Pindborg JJ, editors. Syndromes of head and neck. New York: McGraw-Hill; 1964. p. 406-9.  Back to cited text no. 1
    
2.Caiazzo A, Mehra P, Papagearge MB. The use of preoperative percutaneous transatheter vascular occlusive therapy in the management of sturge weber syndrome-Report of a case. J Oral Maxillofac Surg 1998;56:775-8.  Back to cited text no. 2
    
3.Fishman SJ, Muliken JB. Hemangiomas and vascular malformations of infancy and childhood. Pediatr Clin North Am 1993;40:1177-200.  Back to cited text no. 3
    
4.Ahluwalia TPS, Lata J, Kanwa P. Sturge-Weber Syndrome with intra oral manifestations: A case report. Indian J Dent Res 1998;9:140-5.  Back to cited text no. 4
[PUBMED]    
5.Cek DI. Sclerosing agent therapy for venous malformations of the lip and perioral region. Eur J Plast Surg 1992;15:276-8.  Back to cited text no. 5
    
6.Woods JE. Extended use of sodium tetradecyl sulfate in treatment of hemangiomas and other related conditions. Plast Reconstr Surg 1987;79:542-9.  Back to cited text no. 6
    
7.Saddan NS, Wolach B. Treatment of hemangiomas of infants with high doses of prednisone. J Pediatr 1996;128:141-6.  Back to cited text no. 7
    
8.Stewart RE, Barber TK, Trontman KC, editors. Pediatric dentistry, scientific foundations and clinical practice. St. Louis: CV Mosby; 1982. p. 502-3.  Back to cited text no. 8
    
9.Laskin MD, editors. Oral and maxillofacial surgery. Vol. 2. St. Louis: CV Mosby Co.; 1985. p. 528-9.  Back to cited text no. 9
    
10.Perkins TM, Duncan WK, Hill WJ, Krolls SO. The sturge weber syndrome. A case involving a 13 year old black male. Ann Dent 1992;51:40-3.  Back to cited text no. 10
    
11.Yukna RA, Cassingham RJ, Carr RF. Periodontal manifestations and treatment in case of sturge weber syndrome. Oral Surg Oral Med Oral Pathol 1979;47:408-15.  Back to cited text no. 11
    
12.Hancock RH, Swan RH. Nifedipine induced gingival overgrowth. Report of a case treated by controlling plaque. J Clin Periodontol 1992;19:12-4.  Back to cited text no. 12
    
13.Wilson S, Venzwel JM, Miller R. Angiography, gingival hyperplasia and sturge weber syndrome: Report of a case. J Dent Child 1986;53:283-6.  Back to cited text no. 13
    
14.Gregarion AP, Schneider PE, Shaw PR. Phenobarbitone induced gingival over growth report of two cases and complications in management. J Dent Child 1996;63:408-13.  Back to cited text no. 14
    
15.Suprabha BS, Baliga M. Total oral rehabilitation in a patient with portwine stains. J Indian Soc Pedod Prev Dent 2005;23:99-102.  Back to cited text no. 15
[PUBMED]  Medknow Journal  
16.Gnanasekhar, Al-Duwairi. Electrosurgery in dentistry. Quintessence Int 1998;29:649-54.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]


This article has been cited by
1 Tooth extraction with Sturge-Weber syndrome
Shunsuke Hino,Yosuke Iijima,Masayuki Takahashi,Takahiro Kaneko,Norio Horie,Tetsuo Shimoyama
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology. 2017;
[Pubmed] | [DOI]
2 Analysis of Sturge–Weber syndrome: A retrospective study of multiple associated variables
A.I. Maraña Pérez,M.L. Ruiz-Falcó Rojas,V. Puertas Martín,J. Domínguez Carral,I. Carreras Sáez,A. Duat Rodríguez,V. Sánchez González
Neurología (English Edition). 2017;
[Pubmed] | [DOI]
3 Análisis del síndrome de Sturge-Weber: estudio retrospectivo de múltiples variables asociadas
A.I. Maraña Pérez,M.L. Ruiz-Falcó Rojas,V. Puertas Martín,J. Domínguez Carral,I. Carreras Sáez,A. Duat Rodríguez,V. Sánchez González
Neurología. 2016;
[Pubmed] | [DOI]
4 A Case Report of Sturge-Weber Syndrome With Gelastic Seizures
Fei Wang,Songbai Gui
Neurosurgery Quarterly. 2015; 25(2): 197
[Pubmed] | [DOI]
5 Periodontal Growth in Areas of Vascular Malformation in Patients With Sturge-Weber Syndrome
Flávia Sirotheau Corrêa Pontes,Nicolau Conte Neto,Rodrigo Moreira Bringel da Costa,Arlison Miranda Loureiro,Liliane Silva do Nascimento,Hélder Antônio Rebelo Pontes
Journal of Craniofacial Surgery. 2014; 25(1): e1
[Pubmed] | [DOI]
6 Prednisolone as an Effective Treatment for Epilepsy
F. Ilik,A.C. Pazarli,A. Dogan
European Journal of Inflammation. 2014; 12(2): 395
[Pubmed] | [DOI]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
   Case Report
   Discussion
   Conclusion
    References
    Article Figures

 Article Access Statistics
    Viewed2246    
    Printed109    
    Emailed2    
    PDF Downloaded76    
    Comments [Add]    
    Cited by others 6    

Recommend this journal