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Dental Treatment of a Child with Pallister-Killian Syndrome


Affiliations
1 Department of Pediatric Dentistry, Istanbul Medipol University, Turkey
2 Department of Pediatric Dentistry, Gazi University, Turkey
3 Department of Pediatric Dentistry, School of Dental Medicine, CaseWestern Reserve University, Cleveland, OH, United States
 

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 bymosaic 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'smouth. 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.
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  • Dental Treatment of a Child with Pallister-Killian Syndrome

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Authors

Serhan Didinen
Department of Pediatric Dentistry, Istanbul Medipol University, Turkey
Didem Atabek
Department of Pediatric Dentistry, Gazi University, Turkey
Gulay Kip
Department of Pediatric Dentistry, Gazi University, Turkey
Asli Patir Munevveroglu
Department of Pediatric Dentistry, Istanbul Medipol University, Turkey
Ozlem Tulunoglu
Department of Pediatric Dentistry, School of Dental Medicine, CaseWestern Reserve University, Cleveland, OH, United States

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 bymosaic 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'smouth. 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.