Unusual Tooth Dysplasia in Child with Dandy-Walker Variant

DOI
  • MASUDA Keiji
    Department of Pediatric Dentistry and Special Needs Dentistry, Kyushu University Hospital
  • NAKAMURA Shiho
    Section of Pediatric Dentistry, Division of Oral Health, Growth and Development, Kyushu University Faculty of Dental Science
  • YAMAGUCHI Noboru
    Medical Juridical person PLATANUS, IZAKI Dental Clinic
  • OGASAWARA Takako
    Department of Pediatric Dentistry and Special Needs Dentistry, Kyushu University Hospital
  • YAMAZA Haruyoshi
    Section of Pediatric Dentistry, Division of Oral Health, Growth and Development, Kyushu University Faculty of Dental Science
  • NISHIGAKI Soichiro
    Section of Pediatric Dentistry, Division of Oral Health, Growth and Development, Kyushu University Faculty of Dental Science
  • YANAGITA Ken-ichi
    Department of Pediatric Dentistry and Special Needs Dentistry, Kyushu University Hospital
  • HIROFUJI Yuta
    Section of Pediatric Dentistry, Division of Oral Health, Growth and Development, Kyushu University Faculty of Dental Science
  • NONAKA Kazuaki
    Section of Pediatric Dentistry, Division of Oral Health, Growth and Development, Kyushu University Faculty of Dental Science

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Other Title
  • Dandy-Walker variantの患児に歯の形成不全症の合併が疑われた1例
  • Case Report

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Abstract

Dandy-Walker malformation and Dandy-Walker variant are congenital cerebellar malformations characterized by cystic dilation of the fourth ventricle and enlarged posterior fossa associated with hypoplasia of the vermis. In Dandy-Walker variant, there is no substantial enlargement of the posterior fossa because of a relatively small cystic dilation of the fourth ventricle. We report a 10-year-6-month-old boy with Dandy-Walker variant complicated by hypoplasia in several of his permanent teeth. The patient was referred to the Pediatric and Special Needs Dental Clinic at Kyushu University Hospital for diagnosis and treatment of an unusual infected right mandibular lesion, which was possibly caused by a periapical infection of the right mandibular first molar. Due to severe mental retardation associated with Dandy-Walker variant, he was unable to cooperate for a further dental examination. In order to perform comprehensive dental assessment and treatment, intra-oral and dental X-ray examinations, and treatment were performed under general anesthesia. X-ray findings revealed a thin root or wide pulp chamber in many of the erupted permanent teeth, including the right mandibular first molar and permanent tooth germs. Also in the right mandibular first molar, a periapical cystic radiolucent lesion was noted without pulp exposure. These findings were most suggestive of general tooth hypoplasia along with fragile enamel or dentin. The periapical lesion in the right mandibular first molar was considered likely to be caused by a continuous bacterial infection in the root canals through fragile crowns. Amelogenesis imperfecta, dentinogenesis imperfecta, and odontodysplasia were also considered as possible existing conditions. However, none could be definitively diagnosed because no discoloration, attrition, enamel fracture, or severe dental caries were apparent in any of the erupted teeth. Further examinations are required to establish an accurate diagnosis. For treatment of the right mandibular first molar, mechanical root canal preparation, filling, and restoration with a steel crown were performed, followed by periapical curettage to remove pathologic tissue, and the postoperative course was satisfactory and uncomplicated. After 2 months, a periapical infectious lesion was found in the left mandibular first molar without any pulp exposure, which was very similar to the previous right mandible condition. We performed the same treatment under general anesthesia. In addition, prophylactic steel crowns were placed on the maxillary first molars, because general tooth hypoplasia with fragile crowns was strongly suggested. We found that long-term follow-up examinations were essential for early diagnosis and management of pulp infection without pulp exposure in other affected teeth.

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