Author/Authors :
Didinen, Serhan Department of Pediatric Dentistry - Faculty of Dentistry - Istanbul Medipol University, Turkey , Atabek, Didem Department of Pediatric Dentistry - Faculty of Dentistry - Gazi University, Turkey , Kip, Gülay Department of Pediatric Dentistry - Faculty of Dentistry - Gazi University, Turkey , MünevveroLlu, AslJ PatJr Department of Pediatric Dentistry - Faculty of Dentistry - Istanbul Medipol University, Turkey , TulunoLlu, Özlem Department of Pediatric Dentistry - School of Dental Medicine - Case Western Reserve University, Cleveland, OH, USA
Abstract :
The Pallister-Killian syndrome (PKS) is an extremely rare genetic disorder with an incidence estimated around 1/25000. PKS is a
multiple congenital anomaly deficit syndrome caused by mosaic tissue limited tetrasomy for chromosome 12p.The presented report
is the first confirmed case with PKS in Turkey. This report focuses on the orofacial clinical manifestations of an 6-year-old boy with
PKS who was referred to the Department of Paediatric Dentistry clinic, Gazi University. It has been learned that the PKS was
diagnosed 1 year after birth. Due to intellectual disability, it was decided to make the dental treatments under moderate sedation.
Although significant tongue thrust and anterior open bite were determined, any oral appliances could not be applied because of the
2 epilepsy seizures in the last 2 years. The aim was to treat decayed teeth and set good oral hygiene in the patient’s mouth. Still, there
is a probability for epilepsy seizures. If epileptic seizures stop permanently, we can apply an oral appliance to block tongue thrust.
The patient is now under control. In cases of systemic and oral findings such as PKS, conducting medical and dental approaches
together will increase the life quality of patients.