Treatment of Natal Tooth in Hallerman-Streiff Syndrome

DOI
  • MASUDA Keiji
    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
  • OGASAWARA Takako
    Department of Pediatric Dentistry and Special Needs Dentistry, Kyushu University Hospital
  • OOKUMA Yukiko
    Department of Pediatric Dentistry and Special Needs Dentistry, Kyushu University Hospital
  • YANAGITA Ken-ichi
    Department of Pediatric Dentistry and Special Needs Dentistry, Kyushu University Hospital
  • 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
  • Hallermann・Streiff 症候群に歯肉腫瘤を伴う先天歯を認めた1例
  • Case Report

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Abstract

Hallerman-Streiff Syndrome is a rare congenital disease characterized by dyscephaly, hypotrichosis,microphthalmia, cataracts, proportionate short stature, skin atrophy, and dental anomalies. Airway management is usually difficult in affected patients, because the dyscephaly in this syndrome includes micrognathia and microstomia. We treated a 13-day-old male with Hallerman-Streiff Syndrome who was presented with a natal tooth in the anterior region of the upper jaw. The natal tooth was apparently unstable because of a small tumor-like lesion in the gingiva and X-ray images revealed no root formation. In addition, micrognathia, microstomia, and thoracocyllosis were found in association with Hallerman-Streiff Syndrome. These findings raised the possibility that the natal tooth might easily become dislodged, and then be difficult to find and remove from the oral cavity. Under local anesthesia we excised the tumorous gingiva along with the natal tooth and occluded the bleeding point with an absorbable suture. The postoperative course was satisfactory. Herein, we discuss risks and treatment of a natal tooth in patients with craniofacial anomalies, particularly micrognathia and microstomia.

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