title: Amelogenesis imperfecta associated with dental follicular-like hamartomas and generalised gingival enlargement.
abstract: BACKGROUND
  Amelogenesis imperfecta (AI) is an inherited disorder characterised by generalised defects of dental enamel, but has been associated with other dental and medical conditions. It affects the appearance and structure of teeth, both in the primary and secondary dentition. AI in the presence of dental follicular hamartomas and gingival hyperplasia is rare and the management presents several challenges to the clinician.
  
  
  METHODS
  This article describes a case of a girl who presented to the paediatric department at the age of 7 years complaining of discomfort when eating and that she was unhappy with the appearance of her anterior teeth. The patient was born in the UK but she and her family were African and of Kenyan origin. She was otherwise fit and well. Investigations included clinical, radiographic and pathological examination as well as cone beam computed tomography imaging and X-ray Microtomography of extracted primary teeth. A diagnosis of AI in the presence of dental follicular hamartomas and generalised gingival hyperplasia was made, which had resulted in the delayed eruption of permanent teeth and an associated anterior open bite. There was no family history of dental defects.
  
  
  METHODS
  Initial treatment included preventative advice and the application of preformed metal crowns on all primary molars. Extraction of all remaining primary incisors was carried out followed by gingivectomy around the maxillary permanent incisors, mandibular central incisors and maxillary left second primary molar. Composite resin reconstruction of all permanent incisors and mandibular primary canines was complicated by the poor quality of enamel. Orthodontic extrusion of the anterior incisors was carried out to improve surface area for bonding with some success. A multidisciplinary team managed this case and decided that no surgical intervention of the dental follicular hamartomas was warranted.
  
  
  RESULTS
  The patient coped well with treatment and attended for regular review over an 8-year period. She was reviewed at 6 monthly intervals clinically by a paediatric dentist and radiographs were taken as required to monitor the hamartomas, maintain the restorations and monitor her dental development. She was also seen on the multidisciplinary clinic once a year.
  
  
  CONCLUSIONS
  This is the first case of its kind to be reported in the UK and the 8th worldwide. While this condition is rare, it is important that paediatric dentists are made aware of it.
authors.affiliation: Guys Hospital, London, SE1 9RT, UK.
authors.forename: S
authors.initials: S
authors.lastname: O'Connell
authors.forename: J
authors.initials: J
authors.lastname: Davies
authors.forename: J
authors.initials: J
authors.lastname: Smallridge
authors.forename: M
authors.initials: M
authors.lastname: Vaidyanathan
mesh_terms: D000567:Amelogenesis Imperfecta / Q000175:diagnosis*; D002648:Child; D003481:Cuspid / Q000473:pathology; D003818:Dentition, Mixed; D005260:Female; D005500:Follow-Up Studies; D005882:Gingival Diseases / Q000175:diagnosis*; D005885:Gingival Hyperplasia / Q000175:diagnosis; D006222:Hamartoma / Q000175:diagnosis*; D006801:Humans; D007180:Incisor / Q000473:pathology; D024343:Open Bite / Q000175:diagnosis; D014078:Tooth Eruption / Q000502:physiology; D014094:Tooth, Deciduous / Q000473:pathology