A 21-year-old male patient presented with a chief complaint of small upper and lower permanent teeth. According to the history reported by the patient, the size of his teeth never changed with age. No contributing family or medical history was identified. The patient did not report any abnormal oral habits. The findings of his general physical examination were normal.\nAn intraoral soft tissue examination showed the presence of brown pigmented gingiva with a normal contour (). A hard tissue examination revealed retained deciduous teeth and permanent teeth with atypical findings, as well as multiple decayed teeth (). Many of the permanent teeth were missing, and the erupted teeth were widely spaced, with a yellowish brown discoloration (). The enamel layer was absent clinically and post-eruption wearing of the teeth was evident (). The upper central incisors exhibited a semi-lunar shape and the molars had flat occlusal surfaces (). Severe attrition resulted in a collapsed bite and reduced vertical dimensions during occlusion ().\nThe panoramic radiography and cone-beam computed tomography (CBCT) images revealed multiple findings. All of the teeth were affected, especially the posterior teeth (). Multiple calcification nodules were seen in the pulp chambers of the primary and permanent teeth; these nodules were needle-shaped in the incisors and round in the posterior teeth (). The differential radiodensity expected between the dentine and the enamel was absent (). Pericoronal radiolucencies delineated by sclerotic margins around non-erupted teeth were significant (). The impacted teeth presented with complete root formation, exhibiting dilacerations and curvature (). The dental follicles around the unerupted teeth appeared to be hyperplastic (). Extensive crown resorption with partial replacement of the resorbed dentin by globular calcified structures was observed ().\nThe impacted permanent canines, second molars, and third molars were observed to have fully formed roots (). An accessory mental foramen was seen on the right side of the mandible and extensive bone loss was observed around the mesiobuccal root of the left maxillary first molar in the volume-rendered CBCT images (). A provisional diagnosis of amelogenesis imperfecta was made based on the clinical and radiographic findings. The histopathological examination of an extracted deciduous canine using light microscopy confirmed the absence of enamel and the absence of scalloping of the dentinoenamel junction. (). Hence, based on the Witkop classification, the final diagnosis was the enamel agenesis (type 1G) subtype of hypoplastic-type amelogenesis imperfecta, which is an autosomal recessive disorder. The histopathological analysis of specimens obtained during treatment also confirmed the presence of calcified pulpal tissue () and dense collagen bundles with abundant vasculature in the gingival tissue ().\nAs this type of amelogenesis imperfecta can be associated with other organ and metabolic abnormalities, further investigations were carried out. An ultrasound examination of his kidneys demonstrated multiple hyperechoic foci in the medulla of both the kidneys, suggesting bilateral nephrolithiasis (). A 24-hour urine spectrophotometric analysis revealed very low citrate levels (18.9 mg/24 hours; reference range, 116-924 mg/24 hours). Other biochemical and haematological findings were normal (alkaline phosphatase, 128 U/L; urea, 12.84 mg/dL, blood urea nitrogen, 6 mg/dL; creatinine, 1.33 mg/dL, blood urea nitrogen to creatinine ratio, 4.51; estimated glomerular filtration rate, >60 mL/min/1.73 m; sodium, 136 mmol/L, potassium, 4.0 mmol/L; chloride, 98 mmol/L; bicarbonate, 19 mmol/L; calcium, 9.4 mg/dL; vitamin D3, 34.63 ng/mL; urine albumin, 32.1 mg/L). A physical, microscopic, and chemical examination of a urine sample did not reveal any abnormalities (urine creatinine, 17.51 mg/dL; urine albumin-creatinine ratio, 183.32 mg/g; 24 hours calculated citrate in urine, 18.9 mg/24 hours; urine oxalate, 14.27 mg/24 hours, urinary calcium, 98.7 mg/24 hours; parathyroid hormone, 19.70 pg/mL).\nThus, based on previous findings, the diagnosis was modified to enamel renal syndrome with associated amelogenesis imperfecta, nephrolithiasis, and hypocitraturia.
A 4-year-old male patient reported to the Department of Pedodontics and Preventive Dentistry with a chief complaint of decayed upper front teeth. Patient's medical history was noncontributory. Patient's mother gave a history of breast feeding for 1 year after which the child was bottle fed for 3 years. Intraoral examination revealed a complete set of deciduous dentition. It was observed that 55, 52, 51, 61, 62, 65, 71, 72, 73, 74, 75, 81, 82, 83, 84, and 85 were affected by dental caries (). Intraoral periapical radiographs revealed pulp involvement with 52, 51, 61, and 62 (). Diet analysis, counseling, and oral prophylaxis were done. 74 and 75 were grossly carious and were indicated for pulpectomy followed by a stainless steel crown (). 52, 51, 61, and 62 were indicated for pulpectomy, followed by glass fibre-reinforced composite resin posts and composite buildups. 55 and 65 presented with pit and fissure caries and were indicated for composite resin restorations.\nThe treatment plan was divided into 2 steps for 51, 52, 61, and 62.\nAn infraorbital block was administered for 61 and 62, and labial and palatal infiltration was carried out for 51 and 52. Gross carious lesions were removed with a no. 330 round carbide steel bur. Unsupported enamel was not removed so as to preserve as possible. The pulp chamber was opened and working length determination IOPA was taken with a no. 10 K-file.The pulp tissue was extirpated using no. 10–no. 35 K-files. After irrigation with copious amounts of 2.5% NaOCl and normal saline, the root canal was dried using paper points. A thick mix of Endofloss paste was then condensed with lentulo spiral into the canal. The obturated material was allowed to set for 10 minutes ().\nThe post space was prepared 1 week after the endodontic treatment was completed. The post space was created by removing approximately 4 mm of Endofloss material using a thin straight fissure bur. All visible Endofloss cement on the walls of the post space was removed.\nThe prepared post space was then cleaned with saline, air dried, and acid etched with 37% phosphoric acid for 15 seconds. This space was rinsed and air dried with oil-free compressed air. A light-cured bonding agent was brushed on the etched surface and uniformly dispersed by a compressed air blast. It was then light cured with for 20 seconds. The GFRC post was then cured for 20 seconds in order to gain rigidity, before insertion into the post space. Light cured flowable composite resin was then inserted into the canal chamber after which the GFRC post was inserted (). The fiber post and composite were then cured with together for 60 seconds. The coronal portion of the glass fibre-reinforced composite post was splayed to increase the surface area for the retention of the core.\nThe coronal enamel was then etched for 20 seconds, rinsed with water and air dried followed by application of bonding agent—which was then light cured. The coronal post was then covered with the flowable composite for core build up, followed by light curing it for 60 seconds, and finally teeth were restored with hybrid composite. The final finishing and polishing was done with finishing burs. Occlusal interferences in normal and paranormal mandibular movements were removed ().