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 7-year-old boy reported to our clinic with the chief complaint of decayed teeth. Clinical and radiographic examination revealed multiple carious lesions in all teeth with retained roots of 61, 62. Complete preventive care was provided, retained roots were extracted and all carious teeth were restored. Primary mandibular first molar (84) was grossly carious [] and a decision to place biologic shell crown was made.\nThe operative procedure is described below:\nStep 1: Biologic shell crown preparation: Primary mandibular right first molar (possibly extracted for serial extraction) was selected based on mesiodistal dimensions of the tooth to be restored. The selected tooth was sectioned approximately 1 mm occlusal to cemento-enamel junction. Any remaining pulp tissue was removed using sharp spoon excavator. All undercuts were removed and dentinal surfaces were prepared to diverge cervically. The cervical margin was prepared flat with rounded internal line angles so that it can be seated on prepared margin of primary mandibular first molar. Sterilization was performed as described previously.\nStep 2: Complete caries excavation was performed under local anesthesia with area isolated using rubber dam. After caries removal, a layer of GIC (GC Fuji II, Glass Ionomer Restorative, GC, USA) was placed. Circumferential shoulder margin with rounded internal line angles was placed using round end tapered diamond parallel to long axis of tooth []. Withdrawal form was established by rounding of all line and point angles and removing all undercuts.\nStep 3: Rubber dam was removed and occlusal clearance was checked using modeling wax sheet.\nStep 4: Trial fit of biologic shell crown was done and adjustments were done if needed [].\nStep 5: Etching of dentinal surfaces of prepared biologic shell crown and prepared tooth surfaces was done with 35% phosphoric acid (3M™/ESPE™ Dental Products, USA) for 15 s. Final cementation was performed using dual cure resin cement (RelyX™ U100 Self-Adhesive Resin Cement, 3M™/ESPE™ Dental Products, USA) following manufacturer's instructions.\nStep 6: Final check for occlusion in centric as well as eccentric positions to check for any functional interferences. Thin carbon paper was used and pre-maturities, if any were removed.\nStep 7: Finishing and polishing of margins was done with composite finishing and polishing tips (CompoSite™ Finishing Kit, CompoSite™ Polishing Kit, Shofu, USA).\nClinical examination [] at 1, 6, and 9 months showed the cemented biologic crown to be functioning well in the oral cavity.