Plastik ve Rekonstirüktif Cerrahi / Plastic and Reconstructive Surgery OLGU SUNUMU / CASE REPORT Management of a Neonate with a Rare Congenital Palatosubglossal Synechia in Pierre Robin Sequence

Acıbadem Üniversitesi, Sağlık Dergisi, Kahraman Berkhan Yılmaz, Halil İbrahim Canter, Mustafa Çakmakçı
2015 Cilt: 6 • Sayı: 4 • Ekim   unpublished
Development of the human face begins in the fourth week of gestation, as a series of several complicated and well-organized sequential intrauterine events, disruption of which causes some sort of facial dismorhogenesis. Oral synechiae is a rare congenital anomaly usually recognized at birth secondary to airway or nutritional compromise. They help to understand the intrauterine developmental steps of the facial region. In this particular case with congenital palatosubglossal synechia, anterior
more » ... ynechia, anterior soft palate of the patient with Pierre Robin sequence was repaired with the oral mucosal flap elevated from the floor of the mouth by using the syn-echial band as the pedicle of the flap. Keywords: congenital palatosubglossal synechia, Pierre Robin Sequence, surgical repair PİERRE ROBİN SENDROMLU BİR YENİDOĞANDA NADİR GÖRÜLEN PALATOSUBGLOSAL YAPIŞIKLIĞIN TEDAVİSİ ÖZET Yüzün gelişimi hamileliğin 4. haftasında başlar. İyi organize, bazı komplike intrauterin olayların ardısıra cerayan etmesi sonucu yüzün gelişimi tamam-lanır. Bu süreçleri bozacak olaylar ise çeşitli fasiyal dismorfogenetik olaylara neden olabilir. Ağız içinde sineşi görülmesi nadir bir konjenital durumdur. Genelde doğum sonrasında nefes problemleri ve beslenme problemlerine neden olabileceği için tanı konulur. Bu gelişimsel bozukluklar bize yüzün embriyoljik olarak nasıl geliştiği konusunda da ip ucu verir. Bu vaka sunumumuzda Pierre Robin Sekanslı bir hastada olan palatosubg-lossal sineşi bandının pedikül olarak kullanılarak, dilin alt tarafından kal-dırılan mukoza flebi ile onarılan anterior palatal yarığın tedavisini sunduk. Anahtar sözcükler: konjenital palatoglosal sineşi, Pierre Robin Sekansı, cerrahi tedavi Gönderilme Tarihi: 07 Ocak 2015 • Revizyon Tarihi: 09 Nisan 2015 • Kabul Tarihi: 21 Nisan 2015 İletişim: Kahraman Berkhan Yılmaz • E-Posta: berkhany@isbank.net.tr P ierre Robin sequence is characterized by the sequence of clinical events associated with a small mandible (micrognathia). The tongue is enlarged and retracted (glossoptosis), obstructing the airway, which results respiratory symptomatology, that can make oral feeding difficult or sometimes impossible in severe cases. Patients with Pierre Robin sequence may also present with a cleft palate, which is not a diagnostic criteiron. It is thought that a relatively prominent tongue in such mi-crognatic patients leads to mechanical interference of the lateral palatine processes during palatal fusion. Therefore cleft palate might result from a delay in the transition of the palatine shelves from a vertical to horizontal orientation (1). Development of the human face begins during the fourth week of gestation when migrating neural crest cells from the dorsal region of the anterior neural tube (cranial neural crest, [CNC]) combine with mesodermal cells to form the facial primordia. Then the maxillary prominences enlarge and grow towards each other and the nasal prominences.
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