Hallermann-Streiff syndrome: A case report

• Published in: International dental journal 2024-10, Vol.74, p.S42-S43
• Main Authors: Kesim, Yaprak Öyküm, Karabaş, Hülya Çakır
• Format: Article
• Language: English
• ISSN: 0020-6539
• DOI: 10.1016/j.identj.2024.07.699

Hallermann-Streiff Syndrome (HSS) is a rare developmental disorder. The main diagnostic features of HSS include dyscephaly, bird-like facial structures, congenital cataracts, abnormal dentition, hypotrichosis, skin atrophy, bilateral microphthalmia, and proportionate dwarfism. There have been fewer than 300 reported cases worldwide. The aim of this case report is to describe the clinical and radiological features of HSS, which is a rare condition. The case involves an 18-year-old female patient. Her apperance is consistent with HSS. Intraoral examination indicates small tongue and high arched palate. Many premolar and molar teeth exhibit large restorations, except for teeth 17 and 37. Class 2 malocclusion is present. Maxilla and mandible are hipoplastic. Radiologic examination shows three wisdom teeth are present but preerupted, and tooth 48 is absent. Teeth 12, 22, 32, 33, 34, 42, 43, and 44 are missing, while primary teeth 74, 83, and 84 persist. Both condyl heads are resorped. Frontal sinuses are absent. Sphenoid and maxillary sinuses are immature. Orodental abnormalities are reported to be 50-85% of cases. In this case, the patient shows most of the deformities reported before. Due to the narrower upper airways in these patients, surgical interventions requiring general anesthesia may precipitate respiratory distress. Therefore, it is beneficial to have a CBCT image before treatment. Case reporting plays a pevotal role in enhancing our understanding of abnormalities, even more crucial when it comes to rare diseases. Detecting radiologic features of these abnormalities has a significant role to being able to increase life quality of these patients in need.