Blue rubber bleb nevus syndrome with oral hemangiomas

A case of blue rubber bleb nevus syndrome with oral hemangiomas is reported. Attention is directed to this syndrome because, although rare, it is usually associated with oral lesions. The blue rubber bleb nevus syndrome should always be considered a possibility in patients with oral hemangiomas and bluish skin nodules.     

Rett syndrome--a rare and often misdiagnosed syndrome: case report

Rett syndrome is a recently discovered, progressive neurological disorder that occurs exclusively in females early in life. Females with this rare and often misdiagnosed clinical entity have unusual but distinctive oral/digital habits. Dentists aware of Rett syndrome and its distinct manifestations will be able to aid in early diagnosis and treatment of those afflicted. A case report of a 4-year, 3-month-old white female with Rett syndrome and review of pertinent literature are presented. The previously reported oral/digital habits and oral manifestations of Rett syndrome are listed along with other unreported oral findings. Many of these oral manifestations are not unique to Rett syndrome. There is a likelihood that they may be related to trauma secondary to the excessive oral/digital habits, poor gait, and seizure activity.     

'Emily goes to the dentist'. Oral care for individuals with Down syndrome

This article is primarily based on an editorial letter in the Journal of Oral Health and Disability that describes the visits of a patient with Down syndrome named Emily. Oral health care for individuals with Down's syndrome and other people with learning disabilities in The Netherlands is discussed. Due to the syndrome related oral aspects and specificity, the authors argue strongly in favour of working multidisciplinary within oral health care centres. The dependency of persons with Down's syndrome necessitates an appeal to parents, relatives and carers to maintain oral health. Client-centred care is mandatory for an optimal oral health condition of this vulnerable group.     

Neonatal permanent jaw constriction because of oral synechiae and Pierre Robin sequence in a child with van der Woude syndrome.

OBJECTIVE: To report a newborn with van der Woude syndrome, Pierre Robin sequence, and oral synechiae. Pierre Robin sequence is a rare manifestation of van der Woude syndrome as are oral synechiae. We speculate that the oral synechiae may be causally related to the development of Pierre Robin sequence in this patient.     

Use of dental implants in patients with Down syndrome: a case report

Down syndrome is caused by trisomy of the 21st chromosome and is associated with well-described physical and systemic problems. Most people with Down syndrome have some degree of mental retardation as well as malformation of head and neck. Oral structures that are commonly affected include the tongue (microglossia), abnormalities in the number and shape of teeth, and poor quality (osteoporotic-like) of alveolar bone and jaw. These oral malformations as well as a tendency toward poor cooperation in the dental office contribute to the belief among dentists that people with Down syndrome are not good candidates for oral rehabilitation with dental implants. This article describes the use of dental implants in the oral rehabilitation of a 16-year-old boy with Down syndrome. Although more experience is needed before dental implants can be considered a suitable option during oral rehabilitation in people with Down syndrome, this case report shows a promising beginning.     

Mucocutaneous candidiasis as first manifestation of autoimmune polyglandular syndrome type I

Autoimmune polyglandular syndrome type 1 exhibits very specific oral manifestations in the form of enamel hypoplasia and oral candidiasis. The authors present the case of a 10-year-old girl with autoimmune polyglandular syndrome characterized by chronic mucocutaneous candidiasis infections, hypoparathyroidism, and enamel hypoplasia. The importance of this entity is stressed, with special attention to the observed oral anomalies.     

Kabuki syndrome: a case report

Abstract

This article reports the case of an 8-year-old female with Kabuki syndrome and the oral/dental implications of this syndrome, namely hypodontia with interdental spacing, abnormal tooth morphology, malocclusion and a defect in the anterior midline of the palate. The oral findings will aid the clinician in diagnosing this syndrome, which was once thought to be seen exclusively in the Japanese population.     

New dental findings in the median cleft facial syndrome

BACKGROUND: The main features of median cleft facial syndrome are hypertelorism, cranium bifidum occultum, widow's peak, and midine clefting of the nose, upper lip and palate. Since this pathology was first described in 1967, many cases have been reported in the literature, but none of these reports has addressed oral anomalies in depth. CASE DESCRIPTION: The authors present the case of a female patient aged 4 years and 7 months who was diagnosed with median cleft facial syndrome. In this case, the patient had an abnormal number of teeth, owing to fusion or agenesis of teeth, supernumerary teeth and ectopic eruption of some teeth. These oral anomalies had not been described before in the literature as being associated with this syndrome. CLINICAL IMPLICATIONS: Median cleft facial syndrome is a rare pathology; however, dentists should know its possible alterations at an oral level, because children with this syndrome need dental treatment to achieve good esthetics and correct occlusion.     

Oral diseases in a patient affected with Prader-Willi syndrome.

BACKGROUND: A case of Prader-Willi syndrome (PWS) in a 13-year-old girl is reported. The patient presents with systemic and oral manifestations of the disease. Case report The patient shows: obesity, hypotonia, mental retardation, small hands, prominent forehead, strabismus, hypoplastic teeth, poor oral hygiene, caries, oral candidiasis and thick, sticky saliva. This case is reported to underline the importance of the oral and dental problems of these patients. Caries and oral candidiasis are correlated with the reduced secretion of saliva and with poor oral hygiene. The role of paediatric dentistry is considered to be necessary for the prevention of oral complications of this syndrome.     

Total oral rehabilitation in a patient with portwine stains

Sturge-Weber syndrome is an uncommon condition characterized by presence of Portwine stains on the face along with ocular disorders, mental retardation, oral involvement and leptomeningeal angiomas. A report of a case with atypical manifestations of this syndrome along with a step-by-step protocol oral rehabilitation of such patients is described.     

Oral,radiographical, and clinical findings in Weaver syndrome: a case report

Weaver syndrome is a rare condition characterized by accelerated growth, advanced osseous maturation, and distinct craniofacial features that can occur in both males and females. Etiology is unknown and no definitive oral characteristics have been established. The objective of this case report is to describe the oral and radiographic findings of a 14‐year‐old male with Weaver syndrome. A comprehensive dental examination was performed under general anesthesia. The clinical findings were: severe generalized heavy plaque with subgingival calculus, gingival edema, malocclusion and maxillary atresia, mild micrognathia of the mandible, enlarged tongue, and bifid uvula. Clinical and radiographic caries were not found. Out of over 40 possible cases of Weaver syndrome previously reported in the literature, only two were related to the oral cavity. Bifida uvula was not mentioned until now as clinical findings associated with the syndrome. Autism, macroglossia, and cleft palate were seldom mentioned. The oral manifestations in Weaver syndrome are not yet well known. Future studies will be required to improve the subject matter.     

Moebius syndrome with oral involvement

Summary. The oral findings of 12 patients with Moebius syndrome are described. Facial weakness, hypoplastic upper lip, microstomia, mouth-angle drooping, hypoplasia of mandible, gothic palate, tongue weakness, fissured tongue, tongue atrophy and open bite were found. In addition to describing the oral findings, this report discusses some implications for dental treatment of patients with this syndrome.     

Prevalence of oral mucosal disorders in institutionalized and non-institutionalized psychiatric patients: a study from AVBR Hospital in central India

Dental treatment is reported to be the greatest unattended health need of people with a disability. The aim of the present study was therefore to quantify the prevalence of oral diseases with a psychosomatic component (recurrent aphthous stomatitis, burning mouth syndrome, and oral lichen planus) in psychiatric patients and to screen these patients for any other oral disorders, so that better care could be provided. In this cross-sectional, single-assessment study, 150 psychiatric patients were evaluated for presence of oral disorders. They were screened based on their socio-demographic profiles, clinical profile, and standardized psychiatric scales. The prevalence of recurrent aphthous stomatitis (RAS), burning mouth syndrome (BMS), and oral lichen planus (OLP) was 19.33%(29 patients), 20.66% (31 patients) and 5.33% (8 patients), respectively, amongst all psychiatric patients. The prevalence of burning mouth syndrome was much higher in patients taking psychiatric medications (25%) than in drug-na?ve patients. On screening for other oral disorders, 35.33% of psychiatric patients had at least one other such disorder. We concluded that this patient group experiences a considerable burden of occult oral disorders necessitating thorough oral care. We also described the possible causes of the higher prevalence of oral disorders in psychiatric patients.     

Current concepts of the adult respiratory distress syndrome.

The adult respiratory distress syndrome (ARDS) is a sequel to pulmonary injury that may be direct, closed chest trauma or indirect, through air or vascular passages, aspiration, or fat embolization. An understanding of this syndrome is essential for the oral surgeon who not only manages severe maxillofacial injuries but is also a member of a trauma team that manages multisystem injuries. Emphasis on pathophysiologic pathways resulting in ARDS is presented with a discussion on oxygenation and ventilation abnormalities. Application of these guidelines will assist the oral surgeon in understanding the management of patients with this acute progressive syndrome.     

Multiple radiopaque masses in the jaws

In this report, the less common oral findings occurring in Gardner's syndrome are described, which occurred concurrently with colorectal polyposis. In addition, what clinically appeared as small intestinal polyps, microscopically represented nodular lymphoid hyperplasia, as reported in the literature. Review of the literature showed the oral findings reported here are not common and that enzyme assays can help detect polyps at an early stage of this complex syndrome.     

Oral aphthous-like lesions, PFAPA syndrome: a review

Aphthous ulcers are the most common oral mucosal lesions in the general population. Several precipitating factors for aphthous ulcers are suggested to operate on subjects with genetic predisposition. Sometimes aphthous ulcers can be the sign of systemic diseases. Therefore, it is essential to establish a correct diagnosis to determine suitable therapy. There are several diseases potentially responsible for oral ulcers. Sometimes appearance of periodic oral ulcers coincides with periodic fever and other symptoms leading to the diagnosis of a rare childhood disease: PFAPA (periodic fever, aphthous stomatitis, pharyngitis and adenopathy) syndrome. PFAPA or Marshall's syndrome is characterized by abrupt onset of periodic episodes of high fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis, often associated with headache and / or abdominal or joint pain. Owing to the periodic onset of oral symptoms, often an oral physician or pediatric dentist may be the first healthcare worker to evaluate a child with clinical signs compatible with PFAPA syndrome. Children diagnosed with this condition require systematic oral follow-up to monitor for signs of ulceration.     

Idiopathic lenticular mucocutaneous pigmentation (Laugier-Hunziker syndrome): a report of a case

Laugier-Hunziker syndrome (LHS) is an acquired, benign, macular hyperpigmentation of the lips and oral mucosa, often associated with pigmentation of the nails. It is a rare disorder thought to be more common than the number of reported cases would suggest. It is important to include this condition in the differential diagnosis of diffuse oral pigmentation. Here we report the first case of the Laugier-Hunziker syndrome in Scandinavia. Other conditions causing diffuse or multifocal pigmented oral lesions are discussed.     

Oral manifestations in Cowden's syndrome. Report of four cases

Cowden's syndrome or multiple hamartoma and neoplasia syndrome is a hereditary disorder combining multiple hamartomas of many organs. These hamartomas are derived from ectodermal, mesodermal, and endodermal tissues and may undergo spontaneous malignant changes. Small papular cutaneous lesions and papillomatous outgrowth and papular lesions of the oral mucosa are the most predominant features of the syndrome. Since the orocutaneous lesions often precede the associated malignant degenerations, they may serve as a diagnostic signal for the probably developing neoplasia in other organs. The presence of extensive papillomatous and papular lesions in the oral mucosa combined with skin lesions should therefore alert the clinician to suspect the presence of Cowden's syndrome, the early diagnosis of which is important in order that the concomitant malignant changes might be discovered at the earliest possible moment. Four cases of Cowden's disease are presented, with emphasis on the oral manifestations of this syndrome.     

Multiple hamartoma syndrome (Cowden's syndrome): case report and literature review

Multiple hamartoma syndrome (also known as Cowden's syndrome) is a genetic disorder. The signs and symptoms of this disorder often do not appear concurrently or with the same degree of severity. While much of the literature regarding Cowden's syndrome (CS) focuses on the dermatologic and gastro-enterologic features of the disease, there also are unique oral and facial symptoms that manifest early in this condition. This disorder might be overlooked and underdiagnosed by the dental community. There is a strong link between CS and female breast cancer, thyroid cancer, gastrological polyps, and rare forms of brain cancer; as a result, it is essential for the dental community to gain a greater understanding of this syndrome and its unique oral manifestations.     

Congenital analgia syndrome

 Hereditary sensory and autonomic neuropathy is a rare syndrome which is seen in early childhood. Five different types have been described. Absence of pain and self-mutilation are characteristic findings of this syndrome. This report describes one female and two male children with the syndrome. The most severe oral consequence of their disorder was damage to the oral tissues and tongue. The primary aim in management was to monitor the eruption of the permanent teeth. Received: June 12, 2001 / Accepted: December 20, 2001