Orofacial manifestations of Sturge-Weber syndrome. Review with a case report.

Sturge-Weber syndrome is reported in a 19 year old male. Intraoral manifestations of SWS are uncommon. Hence, the present case is reported for the presence of gingival capillary hemangioma which was surgically treated. A review from the literature pertaining to orofacial manifestations of SWS is given.     

Orofacial aspects in Noonan syndrome: 2 case report

Noonan syndrome (NS) is an autosomal dominant disorder characterized by dysmorphic facial features in association with short stature and heart disease. A webbed neck, chest deformities, mental retardation, and bleeding disorders are also frequently associated with this pathology. NS is relatively common, with an estimated incidence of 1 per 1,000 to 1 per 2,500 live births. Many cases have been reported in the dental literature, yet only a few of them describe some details of the oral features. The aim of this paper is to describe 2 cases of Noonan syndrome, emphasizing oral and facial aspects and particularities of the dental treatment in subjects affected by this genetic disease.     

Orofacial manifestations in a child with Fanconi's syndrome

Orofacial and dental findings in a child with Fanconi's syndrome and accompanying glycogen storage disease are presented. Remarkable retardation of skeletal growth, resulting in dwarfism, was noted. Cephalometric analyses revealed that the size of the patient's craniofacial bones at 10 years of age was as small as that of the average 4-year-old child. Development of the permanent dentition was retarded for 3 to 4 years. Histologic examination of an exfoliated primary canine showed hypomineralization of the dentin structure.     

Self-inflicted gingival injury in a pediatric patient: a case report

Self-inflicted oral mutilation can result in oral lesions that are difficult for a pediatric dentist to diagnose. Sometimes its execution is premeditated, but it can also happen accidentally or as the result of an unconscious, deleterious habit. The purpose of this case report was to describe: (1) the diagnosis of a gingival lesion caused by self-inflicted oral mutilation in a 9-year-old patient; and (2) the proposed treatment.     

The dentofacial manifestations of XXXXY syndrome: a case report

This paper presents a six-year-old patient with XXXXY syndrome, whose oral findings included a cleft soft palate, hyper- or meso-taurodontism in eight primary molars and in the mandibular permanent first molars, five congenitally missing premolars, and delayed development of the permanent tooth germs. The maxillary and mandibular primary central incisors were in a cross-bite relationship. Cephalometric findings showed a short ramus of the mandible and a short maxilla in the anterio-posterior plane. The anteroposterior jaw relationship was in harmony. The cross-bite was considered to be due to the retroinclination of the maxillary primary incisors. This case emphasises the importance of regular dental care, and monitoring of facial growth and dental development in children with XXXXY syndrome.     

Hemangiopericytoma of the maxilla in a pediatric patient: a case report

The hemangiopericytoma is a malignant vascular tumor arising from mesenchymal cells with pericytic differentiation. Hemangiopericytoma is most commonly seen in adults, and only 5% to 10% of cases occur in children. The tumor is extremely rare in the head and neck region (16%). Cytogenic abnormalities have been present in some hemangiopericytoma cases. Surgical resection remains the mainstay treatment. Adjuvant chemotherapy and radiotherapy is appropriate for cases of incomplete resections and life-threatening tumors particularly in children. Late relapses may occur and require long-term follow-up. A 4-year-old child patient with hemangiopericytoma of the maxilla presented with firm, recurrent, but painless jaw mass. Radiographic investigations revealed a poorly circumscribed radiolucency. The lesion biopsy showed wellcircumscribed multiple lobules of tumor mass consisting of tightly packed, spindleshaped cells. Chemotherapy and radiotherapy of the lesion was conducted. The role of the pediatric dental team is extensive in children with hemangiopericytoma, who require a regular dental review. The patient's oncologist should be immediately contacted if there is any suspicion of recurrence.     

Orofacial pain of cardiac origin: a case report

It is the responsibility of the dentist to make the differential diagnosis of pain in the region of the oral cavity. Cardiac pain most commonly radiates to the left arm, shoulder, neck and face. In rare instances the pain may present as dental pain. In this case report, the patient presented with complaints of intense bilateral pain in the jaws which was diagnosed as pain of cardiac origin.     

Odontodysplasia in a pediatric patient: literature review and case report

A case of a three-year-old Caucasian male with odontodysplasia is presented. Although the etiology of this condition is unknown, this anomaly involves both the mesodermal and ectodermal dental components and results in deficient and abnormal formation of dentin and enamel. The orofacial characteristics and dental findings of the condition are presented in this case report.     

Primary hyperoxaluria in a pediatric dental patient: case report.

A case is presented in which primary hyperoxaluria and oxalosis in a 14-year-old Caucasian female were diagnosed. Generalized root resorption resulted in a remarkable mobility of her maxillary central and lateral incisors, although no bone loss was noted. The management of the patient's dental concerns in this rare heritable metabolic disorder consisted of removing the maxillary incisor teeth and placing two sequential prostheses, which the patient tolerated well. A history of trauma to the maxillary incisors was ruled out, so this case adds previously unreported information to our knowledge about the effect of oxaluria on teeth and oral tissues.     

Peripheral complex odontoma in a pediatric dental patient: a case report

Odontomas are the most commonly occurring benign odontogenic tumors of the jaws. Although a majority of odontomas are intraosseous, there are case reports of odontomas that erupted into the oral cavity. Even less common are peripheral or soft-tissue odontomas, only a few of which have been reported to date. We report a peripheral odontoma that arose in the alveolar mucosa of the posterior maxilla in a young child. The diagnosis, complications, treatment, and prognosis of this entity will be discussed.     

Surgical treatment of tongue lymphangioma in a pediatric patient: a case report

Lymphangiomas are benign tumors that occur predominantly in the head and neck regions. They are very common in children and are usually diagnosed at birth or during the first years of life. Understanding their clinical features is crucial to an early diagnosis. The purpose of this paper was to report the case of a 3-year-old patient with a nodular lesion that evolved for 2 years on the tongue's dorsal surface, highlight the surgical treatment and characteristics of this kind of pathology, and explore the differential diagnosis.     

Dental management of a pediatric patient with mastocytosis: a case report

Mastocytosis is a heterogeneous group of clinical disorders characterized by an excessive number of normal mast cells in a variety of tissues (skin, bone marrow, liver, spleen and lymph nodes). It is most often seen in the skin in pediatric-onset mastocytosis presenting as urticaria pigmentosa. Children with this disorder are on a strict avoidance protocol of triggering factors to decrease the likelihood of life-threatening anaphylactic reactions. Close monitoring and the avoidance of known histamine-releasing drugs is necessary in the pediatric dental office, as is a readiness to use resuscitative measures. A case of a 4-year, 6-month-old pediatric dental patient with mastocytosis is presented. Dental treatment was provided in an ambulatory setting utilizing nitrous oxide, oxygen analgesia and H1 and H2 antihistamines to prevent mast cell degranulation and to provide sedation.     

Oral manifestations of Ehlers-Danlos syndrome: report of case

First described in the beginning of this century, Ehlers-Danlos syndrome is a hereditary connective tissue disorder. This case report describes an 11-year-old girl who had frequent unexplainable bruises. During an initial orthodontic evaluation, characteristic symptoms were spotted, and a subsequent diagnosis made.     

Oral and craniofacial manifestations in a Hunter syndrome patient with hematopoietic stem cell transplantation: A case report

We described herein the oral and craniofacial features of a 7‐year‐old boy, diagnosed in utero with mucopolysaccharidosis II (MPS II), who was treated with hematopoietic stem cell transplantation (HSCT) at 70 days of age. The main oral clinical findings were the following: macroglossia, posterior cross‐bite, crowding, pointed cuspid teeth, delayed tooth eruption, retained primary teeth, and enamel hypoplasia. The image examination showed: retention eruption, posterior primary teeth with short roots, absence of some permanent teeth, and stretching of the stylohyoid processes bilaterally. This patient showed the importance of early diagnosis and HSCT therapy in attenuating the clinical and radiographic oral and craniofacial manifestations of the MPS II patient.     

Periodontal disease in a Rubinstein-Taybi syndrome patient: case report

INTRODUCTION: Rubinstein-Taybi syndrome (RTS) is a rare disorder affecting 1 of 300,000 people, characterized by growth, mental and motor retardation, small stature, broad thumbs and toes, characteristic face, high-arched palate, and recurrent respiratory infections. CASE REPORT: The present report describes the periodontal and immunological status of a 14-year-old female patient with RTS. Probing depth, clinical attachment level, bleeding on probing, and radiographic evaluation were performed. Periodontal examination revealed severe attachment loss in incisors and molars and generalized bleeding on probing. Periodontal treatment consisted of scaling and root planing and oral hygiene instructions. Periodontal treatment resulted in resolution of gingival inflammation and pocket depth reductions. The association of periodontal disease and RTS is previously undescribed. CONCLUSION: This case report underscores the importance of periodontal clinical diagnosis and the possibility of successful periodontal treatment in RTS patients.