RESUMEN
BACKGROUND: Woolly Hair Syndrome (WHS) is a rare birth condition that affects the structure of hair in non-black people. The pathogenesis is not yet defined. It is postulated that the hair follicle's desmosomes (specifically desmoplaquine, placoglobin and placofilin-1, which are cell structural proteins that keep the adhesion among close cells) would be altered in this pathology, leading to fragility in the cellular union. It is subdivided into two large groups: the localized or circumscribed variant and the generalized variant. From birth or first months of life, patients with WHS are clinically characterized by the presence of a portion or entire scalp area of very short frizzy hair, usually of a smaller diameter, brittle and lighter color. The most frequent skin manifestations are pilar keratosis and palmo-plantar keratodermia. The diagnosis is based on the clinical findings, and it is facilitated by trichotoscopic examination. However, a definitive diagnosis of WHS requires genetic testing. Oral agenesis, enamel defects (such as hypomineralization), atypical caries, dental inclusion, and malformed pin-shaped teeth may occur. OBJECTIVE: To describe an aesthetic alternative of oral rehabilitation using the mock-up technique in a patient with WHS. CLINICAL CASE: A 5-year-old female patient diagnosed with WHS from the National Institute of Child Health NIHCH: Breña, Lima, who was referred from the Genetic service to the Pediatric Dentistry service in order to screen outbreaks of infection associated with odontogenic origin and dental anomalies. At the ectoscopy, a patient with short capillary length, brittle and curly hair, dry skin, and nail dystrophy was observed. At the intraoral clinical examination, anterior pieces of 52, 51, 61, 62, and enamel hypomineralization were observed in all teeth. The radiographic examination showed agenesis of parts 41, 34, and 45. Integral dental treatment was performed in the operating room under general anesthesia due to the complexity of the case. Pulpectomy in pieces 52, 51, 61, 62, post of composite resin, and rehabilitation with supra-nanow filling resin using the mock-up technique were proposed as alternative treatments. CONCLUSION: The making of supra-nano filling resin-based crowns using the mock-up technique is an alternative treatment for aesthetic oral rehabilitation in deciduous dentition of patients with WHS. The aesthetic treatment was achieved using supra nano-filling resins. After 12 months of dental treatment, a favorable response was observed, improving the chewing, phonation and aesthetics of the patient.
RESUMEN
BACKGROUND: Idiopathic neuropathy is a peripheral nervous system disorder of unknown etiology. Patients with this disorder often suffer from bruxism problems. Bruxism is defined as an oral parafunction characterized by occlusal contacts resulting from non-physiological, repetitive, and involuntary masticatory motor activities. These events can trigger accentuated wear of the tooth structure leading to sensitivity. Tooth sensitivity is the pain arising from the exposed dentin in response to chemical, thermal, tactile, or osmotic stimuli that cannot be explained as the result of any other dental defect or disease. Sensitivity treatment using adhesive systems has a long-term or permanent effect. Therefore, this article is aimed at describing the management of dental sensitivity, through dentin sealing and resin coating techniques, during the dental restoration process of a child with idiopathic neuropathy and dental attrition in the anterior sector. All of this, with the consideration of using the minimal intervention philosophy. CASE PRESENTATION: A 6-year-old male patient diagnosed with idiopathic neuropathy and suffering from multiple dental attritions with dental sensitivity in the upper anterior teeth. With the clinical-radiographic evaluation, the family history, and the interconsultations with the neuropaediatrics service, the definitive diagnosis of dental sensitivity due to stage-3 moderate attrition was obtained, for which rehabilitation of the upper anterior sector was planned through the use of immediate dentin sealing (IDS) and resin coating (RC). CONCLUSION: The use of IDS and RC techniques shows acceptable sensitivity, which is why these techniques are recommended in cases of moderate attrition in early mixed dentition. The immediate rehabilitation along with the stamping technique improved the aesthetic and functional aspects. Therefore, it is advisable to use them immediately after dentin sealing.
