MAJEWSKI OSTEODYSPLASTIC PRIMORDIAL DWARFISM TYPE II: CLINICAL FINDINGS AND DENTAL MANAGEMENT OF A CHILD PATIENT

Abstract

Majewski osteodysplastic primordial dwarfism type II (MOPD II) is an unusual autosomal recessive inherited form of primordial dwarfism, which is characterized by a small head diameter at birth, but which also progresses to severe microcephaly, progressive bony dysplasia, and characteristic facies and personality. This report presents a case of a five-year-old girl with MOPD II syndrome. The patient was referred to our clinic with the complaint of severe tooth pain at the left mandibular primary molar teeth. Clinical examination revealed that most of the primary teeth had been decayed and all primary teeth were hypoplastic. Patient’s history revealed delayed development in the primary dentition and radiographic examination showed rootless primary molar teeth and short-rooted incisors. The treatment was not possible due to the lack of root of the left mandibular primary molars; so the teeth were extracted. Thorough and timely dental evaluation is crucial for the prevention of dental problems and the maintenance of oral health in patients with MOPD II syndrome is of utmost importance.

 

 

Keywords

• Majewski; Osteodysplastic; Primordial Dwarfism Type II

Majewski Osteodisplastik Primordial Cücelik Tip II: Bir Çocuk Hastanın Klinik Bulguları ve Dental Rehabilitasyonu

Abstract

Mikrosefalik osteodisplastik primordial cücelik tip II (MOPCII), küçük kafa ölçüleri ile doğum ve ilerleyen ciddi mikrosefali, ilerleyen kemik displazisi ve tipik yüz ve kişilik farklılıkları ile karakterize, otozomal çekinik olarak aktarılan ve nadir görülen bir cücelik tipidir. Bu olgu sunumunda, MOPC II sendromlu beş yaşında bir kız bildirilmektedir. Hasta, kliniğimize sol mandibular molar süt dişlerde şiddetli diş ağrısı ile başvurdu. Klinik incelemede birçok süt dişinin çürük olduğu ve dişlerin hipoplastik olduğu görüldü. Sol alt süt molar dişler köksüz olduğu için tedavi edilmesi mümkün değildi ve bu dişler çekildi. MOPC II sendromlu hastaların dişlerinin doğru ve zamanında değerlendirilmesi, bu hastalarda dişsel problemlerin önlenebilmesi ve ağız sağlığının sürdürülebilmesi için büyük önem taşımaktadır

Keywords

• Majewski, Osteodisplastik, Primordial cücelik Tip II

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