A congenital cranial dysinnervation disorder: Möbius syndrome

MUTLU ALBAYRAK H., TARAKÇI N., ALTUNHAN H., ÖRS R., ÇAKSEN H.

TURK PEDIATRI ARSIVI, cilt.52, 2017 (ESCI) identifier identifier identifier

  • Yayın Türü: Makale / Vaka Takdimi
  • Cilt numarası: 52
  • Basım Tarihi: 2017
  • Doi Numarası: 10.5152/turkpediatriars.2017.2931
  • Dergi Adı: TURK PEDIATRI ARSIVI
  • Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), Scopus, TR DİZİN (ULAKBİM)
  • Anahtar Kelimeler: Artificial reproductive technologies, congenital facial paralysis, Mbius' syndrome, INTRACYTOPLASMIC SPERM INJECTION
  • Ankara Üniversitesi Adresli: Evet

Özet

Mbius' syndrome, also known as Mbius' sequence, is a nonprogressive cranial dysinnervation disorder characterized by congenital facial and abducens nerve paralysis. Here, we report a 5-day-old girl who was conceived after in vitro fertilization with poor suck and facial paralysis. She had bilaterally ptosis and lateral gaze limitation, left-sided deviation of the tongue, dysmorphic face, hypoplastic fingers and finger nails on the left hand, and was diagnosed as having Mbius' syndrome. Involvement of other cranial nerves such as three, four, five, nine, 9 and 12, and limb malformations may accompany this syndrome. However, several factors have been proposed for the etiology, some rare cases have also been reported with artificial reproductive technologies. Feeding difficulties and aspiration are the main problems encountered in infancy. The other cranial nerves should be examined further in newborns who present with congenital facial palsy, and other cranial dysinnervation disorders should be considered in the differential diagnosis.