NAGER SYNDROME: A RARE CASE REPORT

Turan Ö. N., Gümrü Çelikel A. D., Tufan Yücesoy M., Çakır Karabaş H., Hocaoğlu E.

FDI World Dental Congress 2024, İstanbul, Türkiye, 12 - 15 Eylül 2024, ss.1

  • Yayın Türü: Bildiri / Özet Bildiri
  • Basıldığı Şehir: İstanbul
  • Basıldığı Ülke: Türkiye
  • Sayfa Sayıları: ss.1
  • İstanbul Üniversitesi Adresli: Evet

Özet

                           NAGER SYNDROME: A RARE CASE REPORT

INTRODUCTION:
Nager syndrome represents a subset of acrofacial dysostoses, marked by craniofacial and limb abnormalities.Facial features include downward-slanting eye openings, a retruded midface, and micrognathia. Limb anomalies primarily affect radial components, resulting in underdeveloped thumbs, radial hypoplasia, and fusion of radius and ulna bones.Its prevalence is extremely rare, with approximately a hundred reported cases.This case report aims to evaluate medical and orthodontic considerations in a 24-year-old female patient diagnosed with Nager Syndrome.

 CASE:

It is the fourth pregnancy of a healthy young,non-consanguineous couple.The first three pregnancies ended in spontaneous abortion.There is no similar anomaly in the family.Dysmorphism and cleft including the soft palate were detected.The typical features of Nager syndrome are present in our patient.Bilateral sphenoid sinus aplasia,coronoid process hyperplasia and mandibular micrognathia were observed on Cone Beam Computed Tomography.In addition to typical Nager syndrome findings,an image compatible with Type III ankylosis was observed in both condyle heads.Ultrasonography showed a decrease in joint space.

 DISCUSSION:

In the clinical examination of the patient,who applied to our clinic with the complaint of toothache,it was observed that the mouth opening was very restricted due to ankylosis.Primarily, tooth extractions and dental fillings were performed in accessible areas to relieve the patient's pain.As a result of the consultation with the plastic surgeon,it was decided to perform mandibular distraction osteogenesis and ear,hand and cheekbone reconstruction.

 CONCLUSION:

Management of patients with Nager syndrome requires a multidisciplinary treatment approach,including genetics, speech therapy,orthodontics,plastic surgery,and orthopedics. Comprehensive rehabilitation addressing functional,psychosocial,and aesthetic aspects requires recognition of orofacial symptoms by dentists and orthodontists.

  Keywords: Nager Syndrome, multidisciplinary treatment