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.

Renal Dysplasia and Precocious Diabetes Onset in Microcephalic Osteodysplastic Primordial Dwarfism Type II Syndrome: A Case Report

“…Microcephalic osteodysplastic primordial dwarfism type II (MOPDII; OMIM #210720) is a rare genetic disorder characterized by severe prenatal and postnatal growth restrictions, progressive bone dysplasia and skeletal anomalies, microcephaly, and characteristic facial gestalt with prominent billed nose and micrognathia. 2 This form of primordial dwarfism was first described by Majewski, Ranke, and Schinzel 1 and is the most common distinctive diagnostic entity within the group of MOPD syndromes. This syndrome was distinguished from Seckel's syndrome due to the severity of growth restriction, presence of bone abnormalities, and mild or absent mental retardation.…”

“…Microcephalic osteodysplastic primordial dwarfism type II (MOPDII; OMIM #210720) is a rare genetic disorder characterized by severe prenatal and postnatal growth restrictions, progressive bone dysplasia and skeletal anomalies, microcephaly, and characteristic facial gestalt with prominent billed nose and micrognathia. 2…”