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Case Reports
. 2015 Jan 31;49(1):41-46.
doi: 10.17096/jiufd.73283. eCollection 2015.

Majewski osteodysplastic primordial dwarfism type II: clinical findings and dental management of a child patient

Arslan Terlemez  1 , Mustafa Altunsoy  2 , Hakki Celebi  3
Affiliations
Case Reports

Majewski osteodysplastic primordial dwarfism type II: clinical findings and dental management of a child patient

Arslan Terlemez et al. J Istanb Univ Fac Dent. .

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.

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Conflict of interest statement

Conflict of interest: None declared.

Figures

Figure 1.
Figure 1.
The patient with MOPD II at left and her older sister at right. Patient is five years old and the older sister is fourteen years old. Relatively proportionate short stature is noted.
Figure 2.
Figure 2.
Patient’s chest radiograph at age 2 shows narrow chest, hypoplastic scapula, hypoplastic pelvis and slender bones.
Figure 3.
Figure 3.
Hand photo of the patient at age 5 (A) and hand radiograph of the patient at age 2 (B).
Figure 4.
Figure 4.
(A) Her height is only 62 cm. (B) Large and prominent nose and small pinnae. Hair is thin and dry. (C) Oral examination of the patient revealed small incisors and posterior teeth with severe caries.
Figure 5.
Figure 5.
Periapical (A,B,C,D) and lateral cephalometric (E) radiographs show short-rooted incisors, rootless primary molars, micrognathia and hypoplastic alveolar bone. (D) shows the extraction area after two weeks. In (E), large sella turcica and premature closure of cranial sutures are observed.
Figure 6
Figure 6
(A)Extracted teeth.(B) Extraction area after two weeks.
See this image and copyright information in PMC

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