A rare condition affecting the primary and permanent by dr ibrahim


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  • Boulder=a very large rock , lava =hot liquid rock which comes out of the Earth through a volcano, or the solid rock formed when it cools molten lava ; amorphous-having no fixed form or shape an amorphous mass of jelly
  • Logan et al. _suggested that the dentinal papilla(dentine and pulp) is responsible for the abnormalities in root development.They suggested that multiple degenerative foci within the papilla become calcified, leading to reduced growth and final obliteration of the pulp space.
  • A rare condition affecting the primary and permanent by dr ibrahim

    1. 1. JOMR;2013:1(1) 13-16 Sekerci A.E., Etoz.M, Sahman.H, Sisman.Y, Nazlim.S DR IBRAHIM
    2. 2.    Dentin dysplasia (DD) is a rare hereditary disturbance is inherited as an autosomal dominant trait. unknown etiology that affects approximately 1 :100,000. In 1972, Witkop classified it into type I and type II which affect both dentitions. Shafer WG, Hine MK, Levy BM. Developmental disturbances of oral and paraoral structures. In A text book of oral pathology; 4 thed, Philadelphia: Elsevier Science, Saunders Co.; 2003. p. 2-85
    3. 3. Radicular dentin dysplasia  Characterized by:1.Both dentitions are affected. 2.Normal appearing crowns 3.No or only rudimentary root development (rootless teeth) 4.Incomplete or total obliteration of the pulp chamber. 5.Teeth may exhibit extreme mobility and exfoliate prematurely.
    4. 4. coronal dentin dysplasia  Characterized by:1.partial pulpal obliteration. 2.Thistle-tube-or flame-shaped coronal pulp chambers 3. Thread-like root canals 4. Usually the absence of periapical radiolucencies. 5. In this type of anomaly, teeth roots are of normal shape and contour.
    5. 5.    The enamel and the immediately subjacent dentin appear normal. Deeper layers of dentin show an atypical tubular pattern with an amorphous, atubular area, and irregular organization. Normal dentinal tubule formationn appears to have been blocked so that new dentine forms around obstacles and takes on the characteristic appearances described as “lava flowing around boulders”
    6. 6.    A 7-year-old girl was referred to the Department complaining mobile teeth and a swelling in the maxillary deciduous right incisor area. The patient had lost her mandibular central incisors teeth by the age of 3 and mandibular left primary second molar by the age of 5. Her older brother had a similar condition. The patient's mother became edentulous at an early age and had required full maxillary and mandibular dentures
    7. 7.   The grandfather of the girl stated that he too had a history of delayed eruption. He further stated that he had lost most of his teeth at an early age because of spontaneous exfoliation, which had necessitated a full denture in the maxilla and a partial denture in the mandible by the end of his adolescence. Dental examination of the patient's father revealed no evidence similar condition.
    8. 8.     The patient's medical history revealed no evidence of disturbance in general health. The patient was having class III malocclusion with spacing between maxillary and mandibular anteriors. Anterior open bite and mandibular prognatie. All the teeth were normal in shape and size. There was a painful fistule on the buccal region of the maxillary right primary central incisor Periapical radiolucencies were present at the permanent upper central incisors.
    9. 9. a. The intraoral examination revealed normal size, morphology and color of teeth
    10. 10. The radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development
    11. 11.  The maxillary primary central incisors, right lateral incisor, mandibular primary right canine, first and second molars mandibular left second premolar was extracted owing to extensive mobility
    12. 12.  The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin.
    13. 13.     In the present case:The calcified pulp chambers, Rootless teeth, Periapical radiolucent areas, and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I.
    14. 14.      The etiology of DD is still unknown; however, several theories have been proposed in the dental literature. Logan et al. _suggested that the dentinal papilla is responsible for the abnormalities in root development. Sauk et al. -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation. Wesley et al. disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation and/or function of these odontoblasts. The cause of periapical radiolucencies in DD type I is not understood.
    15. 15.     Steidler et al.-suggest that they are the result of pulpal necrosis, occurring either secondary to caries or spontaneously. DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation. When multiple family members have a similar pattern of pathosis, this supports a diagnosis of a hereditary condition. An attempt to identify a familial history in our case, the patient's brother, mother, and grandfather had similar condition. Although from the patient's description.
    16. 16.    Histologically-the immediately subjacent dentin and the enamel appear normal. Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous, atubular globular, or nodular masses of abnormal dentin are seen. The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts; however, in some cases, a diagnosis of periapical granuloma has been reported.
    17. 17.       The management of patients with DD is difficult. Extraction Follow-up and routine conservative treatment is another choice of treatment plan in DD Maintenance of periodontal health. Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers. Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling, which is recommended in teeth with long roots.
    18. 18. •Since these patients usually have early exfoliation of the teeth and consequently, maxilla-mandibular bony atrophy, treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfully. •Though a malalignment of the arch is one of the most common characteristics of this disorder, orthodontic correction should not be applied in a routine fashion. •In the majority of cases, despite early diagnosis and the provision of regular dental care, teeth are lost because of spontaneous abscess formation. •There is no treatment for severe cases of DD type I other than extraction of symptomatic teeth. Even for special cases, onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
    19. 19. •Therapy, including extraction of all teeth curettage of cystic alteration, and functional rehabilitation by the insertion of a conventional complete denture, has been presented previously by Neumann et al. •Munoz-Guerra et al. _ reported successful treatment of a 24-year old female after onlay bone grafting and sinus augmentation.
    20. 20. •Though many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain. •Early diagnosis of the condition is important for the initiation of effective preventive treatment. Follow-up studies on patients with DD type I might provide insights into treatment strategies. •Future molecular genetic research may determine precise information about the gene locus responsible for DD type I.
    21. 21. Dentin dysplasia 􀂄 Type II 􀂄 Coronal type 􀂄 Root length normal in both dentitions 􀂄 Primary teeth 􀂄 Clinically resemble dentinogenesis imperfecta 􀂄 Radiographically have similar appearance to Type I 􀂄 Permanent teeth 􀂄 Normal coloration 􀂄 Pulp chambers enlarged with apical extensionthistletubeshaped or flame-shaped
    22. 22. Dd type 11
    23. 23. Autosomal dominant Type I: Radicular The teeth have normal crowns and abnormal roots The teeth are generally exfoliated prematurely Type II: Coronal Primary teeth are translucent with an amber color Adult teeth appear normal
    24. 24. -autosomal dominant disturbance rare (1:100,000) Type I (radicular) normal color & shaped in both dentition malaligned arch, drifting and exfoliate with little or no trauma short or abnormal root shaped, pulp chamber & root canals completely fill in before eruption 20 % of teeth with type I disease have apical radiolucencies  
    25. 25. TypeII (coronal) primary dentition appears as D.I., but permanent dentition is normal obliterated of the pulp chamber & reduced root canals after eruption roots are normal in shape & proportion