Sturge–Weber syndrome

Kalpana Verma; Seema Thakur; Ashish Justa

doi:10.4103/ijohr.ijohr_25_20

CASE REPORT

Year : 2021 | Volume : 7 | Issue : 2 | Page : 74-77

Sturge–Weber syndrome

Kalpana Verma, Seema Thakur, Ashish Justa
Department of Pediatric and Preventive Dentistry, H. P. Government Dental College and Hospital, Shimla, Himachal Pradesh, India

Date of Submission	27-Sep-2020
Date of Acceptance	24-Feb-2021
Date of Web Publication	23-Dec-2021

Correspondence Address:
Kalpana Verma
Room No-310, Department of Pediatric and Preventive Dentistry, H. P. Government Dental College and Hospital, Shimla - 171 001, Himachal Pradesh
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ijohr.ijohr_25_20

Abstract

The Sturge–Weber syndrome (SWS) or encephalotrigeminal angiomatosis is specifically congenital, nonhereditary, and rare condition of unknown etiology. It belongs to a group of disorders collectively known as the phakomatoses (“motherspot” diseases). In its complete tri-symptomatic form, SWS is physically characterized by port-wine stains over the trigeminal area, leptomeningeal angiomas usually over the parieto-occipital region, and eye abnormalities. Clinical manifestation of SWS depends on the affected organs and can include seizures, mental retardation, and glaucoma.

Keywords: Dentistry, pediatric, Sturge–Weber syndrome

How to cite this article:
Verma K, Thakur S, Justa A. Sturge–Weber syndrome. Indian J Oral Health Res 2021;7:74-7

How to cite this URL:
Verma K, Thakur S, Justa A. Sturge–Weber syndrome. Indian J Oral Health Res [serial online] 2021 [cited 2023 Mar 30];7:74-7. Available from: https://www.ijohr.org/text.asp?2021/7/2/74/333379

Introduction

Sturge-Weber syndrome (SWS) was first described by Schirmer in 1860 and later more specifically by William Allen Sturge in 1879, who associated dermatological and ophthalmic changes of the disease to neurologic manifestations. Frederick Parkes Weber in 1929 complemented it with the documentation of radiologic alterations seen in these patients.^[1],[2] It is a rare disorder occurring with a frequency of 1:50,000 live births.^[3] It is believed to be caused by the persistence of vascular plexus around the cephalic portion of the neural tube. This plexus develops during the 6^th week of intrauterine life of embryo development but normally undergoes regression during the 9^th week.^[4] Both sexes are affected equally and no racial predilection is seen.^[5]

The classic feature of this disorder is the angioma of leptomeninges and other features are epilepsy, port-wine stain and dermal angiomas, abnormal findings in skull radiographs, mental retardation, ocular involvement and hemiplegia. Oral manifestations of the disease may vary considerably and changes in morphology and histology of gingiva, periodontium, and pulp have been reported. However, the most common feature is a gingival hemangiomatous lesion usually restricted to the ipsilateral maxilla, mandible, the floor of mouth, lips, cheeks, palate, and tongue.^[2]

Case Report

A 13-year-old male patient reported to the department of pediatric and preventive dentistry with a chief complaint of pain in the upper left back region for the past 1 month and bleeding gums on brushing. Past medical history revealed that the patient had developed convulsive disorder at the age of 6 months, for which he was under medication. He is elder of the two siblings at full-term baby by normal delivery. His parents also gave a history of delayed developmental milestones and learning disabilities. The patient was diagnosed with moderate mental abnormality and hemiplegia of the right side of the body.

Extraoral examination revealed a reddish discoloration (port-wine stain) on the face since birth which was gradually darkening with age. The stain had bilateral distribution. It extended from the forehead and involved the eye, nose, cheek, philtrum, and upper lip till the angle of the mouth from where it extended to the ear. The upper and lower lips were not involved. A port-wine stain was also seen bilaterally involving the left half of the neck. Both eyes appeared normal [Figure 1].

Figure 1: Showing bilateral port-wine stain

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On intraoral examination, caries was present with respect to the upper right first molar and upper left first molar. Intraoral periapical radiograph revealed reversible and irreversible pulpitis of upper right first molar and upper left first molar respectively. Generalized gingivitis with calculus index score of 1 and debris index score of 2 was present. The patient had poor oral hygiene and bleeding on probing was present. Gingiva was bright red in color and showed blanching on applying pressure suggesting angiomatosis enlargement [Figure 2]. Retained 71 and congenital missing 41 were also reported [Figure 3]. The clinical picture and examination pointed to SWS and further investigations were done to confirm the same.

Figure 2: Showing positive blanch test

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Figure 3: Showing congenital missing 41 and retained 71 deciduous dentition

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A root canal treatment was done on 26 and indirect pulp capping with respect to 16. Restoration was done on 36 and 46. A thorough plaque control regimen was started, which included oral prophylaxis at regular intervals, oral hygiene instruction, and motivation of the patient at each visit [Figure 4]a and [Figure 4]b.

Figure 4: Showing oral hygiene status a) Pre-Operative and b) Post-Operative

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After 1 month, the patient reported no bleeding on tooth brushing as well as on probing. Hence, it was decided to follow-up the patient every month to evaluate plaque control. Following root canal crown was planned.

X-ray of the skull shows tramline calcifications [Figure 5].

Figure 5: X-rays of the skull shows tramline calcifications. (a) Lat Ceph. (b) After oral prophylaxis done

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On computed tomography (CT) frontal lobe atropy of left hemisphere is detected. Tramline calcification of left frontal lobe enlarged the left choroid plexus.

Discussion

SWS consists of a group of disorders that may present with neurological, cutaneous, ocular, and oral manifestations. The most common clinical characteristic is a port-wine stain on the face which normally follows the course of the trigeminal nerve, especially the ophthalmic division.^[6] Port-wine stains are reported in about 87%–90% of cases and generally occur on the right side, although these lesions present bilaterally in about 33% of cases.^[7] In the present case, the port-wine stain was present bilaterally on the face. Leptomeningeal angiomatosis is another important clinical feature of this syndrome, progressive cerebral calcification, epileptic convulsions, and mental retardation.^[6] In this case, the patient had a history of epileptic convulsions and the skull radiograph showed intracranial calcification in the left frontal lobe. According to the Roach scale, SWS is classified as follows:^[8]

Type I: Both facial and leptomeningeal angiomas; may have glaucoma
Type II: Facial angiomas only (no central nervous system involvement); may have glaucoma
Type III: Isolated leptomeningeal angioma; usually no glaucoma.

According to the above criteria, our case was type I SWS as the patient had bilaterally port-wine stain and no glaucoma with epileptic convulsions and calcification of the left frontal lobe. The most common oral manifestations in SWS are mainly seen in the unilateral side of the gingiva and finish abruptly at the midline. Angiomatous gingival lesions range from slight vascular hyperplasia to severe hemangiomatous proliferation due to abnormal increases in the vascular component. As a result of these changes, gingiva bleeds on slight provocation or minor trauma.^[1]

Various manifestations of SWS are tabulated in [Table 1].

Table 1: Sturge Weber syndrome

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Differential diagnosis

Cerebral arteriovenous malformation
Infection
TORCH infection
PHACE syndrome
Healed cortical infarct
Gobbi syndrome.

Conclusion

In SWS, the challenge for the oral physician is to be aware of its other manifestations and institute treatment and referral accordingly. The key in the management of SWS is to prevent or reduce the intensity of complications as the underlying pathology cannot be treated. Early identification and early institution of treatment is imperative for the patient to have a better quality of life.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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