Hallermann Streiff syndrome: Cranio-facial manifestations systematic review and report of two cases

Preudhomme, Renaud; Veyssière, A.; Ambroise, Bruno; Benateau, Hervé

doi:10.1016/j.jormas.2021.11.002

Cited by 6 publications

(2 citation statements)

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“…There was complete bilateral syndactyly between her fourth and fifth fingers (syndactyly type III) (Figure 1). Furthermore, she suffered from mild intellectual disability, which is an uncommon manifestation of the syndrome (5). Despite suffering from recurrent respiratory infections and breathing problems as an infant, the patient did not show nor report any signs of breathing difficulties at the time of examination.…”

Section: Case Reportmentioning

confidence: 96%

“…There is no reported sex predilection, nor is its genetic basis elucidated; it is widely believed to be caused by dominant sporadic mutations (4). Almost 85% of patients show dental anomalies such as enamel hypoplasia, natal teeth, high and narrow palatal vault, microstomia, and micrognathia (5). The etiology of this syndrome remains unknown and is widely believed to be caused by a de novo dominant mutation.…”

Section: Introductionmentioning

confidence: 99%

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Hallermann–Streiff syndrome: Case report with abnormal pulp calcifications

Ammar

El-Tekeya

2022

Front. Dent. Med

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Hallermann–Streiff syndrome (HSS) is a disorder of rare occurrence affecting the craniofacial complex, with approximately 200 cases reported in the literature. Nonetheless, its distinctive facial features render it highly recognizable. We present the case of a 5-year-old girl with this syndrome and review the dental manifestations and management in this patient. In addition to the diagnostic facial features of brachycephaly with frontal bossing, beak-shaped nose, microphthalmia, and mandibular retrusion, multiple dental manifestations were noted, including the absence of the mandibular condyle, ghost teeth, and unusual pulpal calcifications in both the primary and the permanent teeth, which have not been previously reported in a case of HSS. There is no consensus on the suitable treatment plan to be given for HSS patients from a young age due to an underreporting of these cases in the literature. In this report, we discuss pediatric dental management options for a patient with HSS and share her perspective of the treatment.

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Section: Case Reportmentioning

confidence: 96%

Section: Introductionmentioning

confidence: 99%