Hallermann-Streiff Syndrome: A Rare Case Report

Hallermann Streiff Syndrome-The Oral Manifestations in a Child

“…coronoid processes, anterior displacement of the condyles, and anterior disk displacements [10]. The hypoplasia of condyles and coronoid processes also was found to be present in our case.…”

“…The case reported here was found to be mute who has not earlier been reported in this syndrome although no correlation to this syndrome could be established. Orodental anomalies reported in the literature (50-85% of the cases) are microstomia, a small and retracted tongue, mandibular hypoplasia, a high arched palate, class II malocclusion, open bite, hypoplasia of deciduous and permanent teeth, absence of teeth, persistence of deciduous teeth, supernumerary teeth, natal teeth, malformed teeth, and severe and premature caries [10,11]. The orodental abnormalities in the present case (confirmed by radiographic examination) were in the form of severely carious hypoplastic teeth, missing permanent teeth, persistent deciduous teeth, bilateral hypoplastic condyles and coronoid processes.…”

A familial study of Hallermann–Streiff–François syndrome

“…There may be mandible hypoplasia, lower jaw movement restriction, high-arched palate (palatine vault is curved), microdontia (narrow teeth root), presence of neonatal teeth (teeth present from birth), supernumerary teeth,18 dental growth restriction,26 and at times hypodontia (loss of a few teeth), or partial anodontia (absence of a series of teeth). Enamel hypoplasia may also be frequent, causing dental cavities; there may also be an abnormal dental alignment in the majority of cases27 or some ghost teeth 13,28. Stomatologic abnormalities in our patients were principally: an arched palate with a palatine depression and mandible hypoplasia, microdontia, macrodontia, and oligodontia in the father’s case with abnormal teeth alignment.…”

“…Differential diagnoses are oculo-dento-digital dysplasia (also called oculo-dento-bone dysplasia or Meyer-Schwickerath syndrome or Hallermann–Streiff pseudo-progeroid syndrome (PHS),13 mandibulo-facial dysostosis (Franceschetti syndrome or its less severe form, Treacher Collins syndrome), cleidocranial dysostosis or dysplasia, progeria and other progeroid syndromes 17. In Hallermann–Streiff PHS, or oculo-dento-digital dysplasia, we observe a severe spastic quadriplegia with apparently normal appearance at birth except for the absence of eyelashes and eyebrows 13.…”

“…It is said that 100 cases have been described worldwide15,16 with a prevalence of less than 1/1,000,000 people 16. Despite the fact that many hypotheses on the genetic transmission have been raised,17–19 the etiology of this syndrome remains unknown1 and there is no predilection of gender 1,13…”