ABSTRACT
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.
ABSTRACT
BACKGROUND: Wolff-Parkinson-White syndrome is a congenital abnormality of the heart. Patients with this syndrome and multiple dental caries with pulp exposure may require comprehensive dental treatment under general anesthesia. Pulpectomy is performed in primary teeth with pulp expose. This treatment in anterior teeth requires palatal access and the majority there is no good visibility, requiring more time to perform. Therefore, this case aimed to demonstrate a pulp chamber opening alternative in pulpectomies in a child with Wolff-Parkinson-White syndrome. CASE PRESENTATION: A 3-year-old female patient with Wolff-Parkinson-White syndrome and multiple dental caries with reversible pulpitis in anterior upper teeth. The dental treatment was performed under general anesthesia. Due to abundant hemorrhage in anterior upper teeth, pulpectomies were performed, which pulp chamber openings were modified by labial and the treatment itself was completed. After, these teeth were rehabilitated with direct restorative veneers using light-curing composite resin. The follow-up at 12 months showed an absence of evidence clinic and radiographic. CONCLUSION: This case demonstrated the pulp chamber opening performed by labial in pulpectomy is an effective and viable alternative to reduce operative time and improve visibility in pulpotomies in a child with Wolff-Parkinson-White syndrome.
