According to the Medical dictionary a disorder is defined as: A disturbance of function, structure, or both, resulting from a genetic or embryonic failure in development or from exogenous factors such as poison, trauma, or disease.
Sturge-Weber syndrome (SWS) is a rare, congenital, neuro-oculo-cutaneous disorder which is characterized extra-orally by unilateral port wine stains on the face, glaucoma, seizures and mental retardation, and intra-orally by ipsilateral gingival hemangioma which frequently affects the maxilla or mandible (1). This disorder occurs in 1:50 000 live birth (2) The main characteristic features of this disorder are port wine stains on the forehead and/or scalp, seizures and mental retardation due to angioma formation in the brain, and glaucoma.(1)
SWS presents with oral conditions that require
I was recently asked to see a 2 year old boy with gingival inflammation. His primary care dentist felt the inflamed gums were due to his poor oral hygiene, and lack of regular dental visits. His mom felt the inflammation was due to his port wine stain. The Pediatric Doctor asked North Carolina Hospital Dentistry for our expert opinion. SWS is associated with hyperplastic tissue, and potential to form excess tissue. Management depends on several factors including severity, and the age of the patient. In a two year old most likely you will see hyperplastic tissue, which can be inflamed due to normal irritants ex. plaque and oral hygiene.
This is hyperplastic tissue which can be managed with good home care, routine dental visits, and adult supervision.
Oral manifestations include unilateral hemangiomatous lesions in the maxillary or mandibular gingiva, lips, tongue, palatine region, etc. In gingiva, these lesion present as unilateral hyperplasia due to an increase vascular component, and demonstrate bleeding following minimal trauma. This requires surgical removal, and a biopsy.
Gingival Hypertrophy could be attributed to Seizure medication in conjunction with the underlying SWS. This requires surgical intervention, and can recur.
In the case the North Carolina Hospital Dentistry was consulted about the 2 year old boy that presented with hyperplastic tissue. The hyperplastic tissue was from his SWS, and the tissue inflammation is due to local irritants ex. plaque and lack of home care. The mom and dentist were both correct about the cause of his inflamed gums. He did not present with hypertrophy or gingival enlargement. This case was referred back to his primary care dentist for routine dental cleaning.
In conclusion Sturge-Weber syndrome is disorder due to abnormal development of the primordial vascular bed, which occurs during the early stages of cerebral vascularization, when the blood supply to the brain, meninges and face is undergoing re-organization. As a result of these abnormal changes, the overlying leptomeninges are richly vascularized and the brain beneath becomes atrophic and calcified, mainly in the molecular layer of the cortex.
Its important to note that SWS isn't a condition ex. pregnancy, nor a disease ex. diabetes, as there are different treatment modalities in each scenario. SWS is a disorder that requires meticulous oral hygiene, and a dentist that can teach the parents what to look for, and how to manage their child unique oral health concerns.
If you are looking a hospital dentist with extensive experience managing oral surgery and general dental needs contact North Carolina Hospital Dentistry.
(1) Sturge-Weber syndrome: oral and extra-oral manifestations
(2) Neto FXP, Junior MAV, Ximenes LS et al. . Clinical features of Sturge-Weber syndrome. Intl Arch Otorhinolaryngol 2008;12:565–70
(3) Miller-keane Encyclopedia and Dictionary of Medicine, Nursing, and AlliedHealth, SeventhEdition.