IADR Abstract Archives
Clinical, biological and therapeutic features of a patient with Acro Dermato Ungual Lacrimal Tooth syndrome
Objectives: The aim of this report was to present a 22-year-old Tunisian female with Acro Dermato Ungual Lacrimal Tooth syndrome and discuss the clinical, biological and therapeutic features of the early loss of permanent teeth.
Methods: A 22-year-old female, born to clinically healthy and consanguineous parents, was referred for a full mouth rehabilitation. The patient’s medical history included chronic dacryocystitis and post-operative clinodactyly.
Physical, oral, biological and radiological examinations were carried out. Minor salivary glands, mucosa, temporary and decayed permanent teeth were taken under local anesthesia. Polarized light and scanning electron microcopies were then performed.
Results: Physical examination revealed that the patient was mentally retarded, underweight and photosensitive. She presented a fine hair, hypoplastic mammary glands, bilateral nasolacrimal duct obstruction and onychodysplasia.
Oral examination showed the early loss of several permanent teeth. Those present were peg shaped and decayed. Enamel was poorly formed.
Biological examination revealed eosinophilia, positive Shirmer test, xerostomia and acid pH saliva.
The radiological examination showed fingers camptodactyly, toes syndactyly, squeletal maturity, hypodontia, enamel hypoplasia, the loss of permanent teeth and a skeletal class III relationship.
The patient underwent full mouth rehabilitation with a complete fixed maxillary prosthesis and a combined mandibular prosthesis.
Polarized light microscopy revealed that the epithelium’s basal membrane of the oral masticatory mucous presented erased epithelial ridges. It also showed globular and dystrophic enamel and inflammatory infiltration in accessory salivary glands. Scanning electron microscopy showed absent acellular extrinsic fiber cementum and vertical periodontal fibers. The cellular intrinsic fiber cementum was present on the root layer and on a great part of the enamel surface.
Conclusions: To our knowledge, this is the first report discussing the histological and therapeutic features of the premature loss of permanent teeth reported in ADULT syndrome patients.
Methods: A 22-year-old female, born to clinically healthy and consanguineous parents, was referred for a full mouth rehabilitation. The patient’s medical history included chronic dacryocystitis and post-operative clinodactyly.
Physical, oral, biological and radiological examinations were carried out. Minor salivary glands, mucosa, temporary and decayed permanent teeth were taken under local anesthesia. Polarized light and scanning electron microcopies were then performed.
Results: Physical examination revealed that the patient was mentally retarded, underweight and photosensitive. She presented a fine hair, hypoplastic mammary glands, bilateral nasolacrimal duct obstruction and onychodysplasia.
Oral examination showed the early loss of several permanent teeth. Those present were peg shaped and decayed. Enamel was poorly formed.
Biological examination revealed eosinophilia, positive Shirmer test, xerostomia and acid pH saliva.
The radiological examination showed fingers camptodactyly, toes syndactyly, squeletal maturity, hypodontia, enamel hypoplasia, the loss of permanent teeth and a skeletal class III relationship.
The patient underwent full mouth rehabilitation with a complete fixed maxillary prosthesis and a combined mandibular prosthesis.
Polarized light microscopy revealed that the epithelium’s basal membrane of the oral masticatory mucous presented erased epithelial ridges. It also showed globular and dystrophic enamel and inflammatory infiltration in accessory salivary glands. Scanning electron microscopy showed absent acellular extrinsic fiber cementum and vertical periodontal fibers. The cellular intrinsic fiber cementum was present on the root layer and on a great part of the enamel surface.
Conclusions: To our knowledge, this is the first report discussing the histological and therapeutic features of the premature loss of permanent teeth reported in ADULT syndrome patients.