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Journal of Indian Society of Pedodontics and Preventive Dentistry Official publication of Indian Society of Pedodontics and Preventive Dentistry
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CASE REPORT
Year : 2006  |  Volume : 24  |  Issue : 5  |  Page : 44-46
 

Osteogenesis imperfecta


1 Department of Pediatric Dentistry, Nair Hospital Dental College, Mumbai, India
2 Department of Oral and Maxillo Facial Surgery, Nair Hospital Dental College, Mumbai, India
3 Department of Anesthesia, Nair Hospital, Mumbai, India

Correspondence Address:
Tejashri Gupte
Lecturer, Nair Hospital Dental College, Mumbai
India
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Source of Support: None, Conflict of Interest: None


PMID: 16891753

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  Abstract 

Osteogenesis imperfecta is an inherited disorder of the connective tissue. The extreme bone fragility seen in patients suffering from osteogenesis imperfecta pose a series of problems with regard to behavior management and rendering of quality dental treatment. Presented here a case of a four year old child suffering from osteogenesis imperfecta.


Keywords: Blue sclerae, bone fragility, osteogenesis imperfecta


How to cite this article:
Gupte T, Iyer V, Damle S G, Malik N, Halbe A. Osteogenesis imperfecta. J Indian Soc Pedod Prev Dent 2006;24, Suppl S1:44-6

How to cite this URL:
Gupte T, Iyer V, Damle S G, Malik N, Halbe A. Osteogenesis imperfecta. J Indian Soc Pedod Prev Dent [serial online] 2006 [cited 2019 Jul 24];24, Suppl S1:44-6. Available from: http://www.jisppd.com/text.asp?2006/24/5/44/26040



  Introduction Top


Osteogenesis imperfecta is a serious inherited disorder which is commonly known to have an autosomal dominant pattern of inheritance. However, autosomal recessive and non hereditary types are also known to occur. The clinical features commonly observed in patients with osteogenesis imperfecta include abnormal bone formation, growth deficiency, bone fragility, blue sclerae, hearing loss, skin thinness, joint laxity and hypermobility and dentinogenesis imperfecta.[1],[2],[3] The disease causes either a decrease in collagen synthesis or the production of structurally defective collagen hence all tissues rich in type I collagen may be affected.[1],[4] Four types of ostoegenesis exist based on the classification of Sillence et al[5] [Table - 1]. Peterson and Wetzel have evaluated the recent findings in classification of osteogenesis imperfecta on the basis of existing dental symptoms[6] [Table - 2].


  Case Report Top


A four year old female child reported to the department of Pediatric dentistry, Nair hospital dental college with the complaint of pain in the lower right and left posterior teeth. The parents gave a history of swelling in the lower left posterior teeth accompanied by severe pain and slight fever. The medical history of the child revealed a history of three episodes of fractures of long bones (left leg twice and left hand once) within a span of the past two years; following minor trauma. The family history revealed the child's seven year old sibling also had history of five fractures.

On examination, the child was found to be very frail, having a small stature, thin built and blue sclerae [Figure - 1]. The child's mother and elder sister too had blue sclerae.

Intraoral examination revealed carious maxillary and mandibular right and left primary first and second molars [Figure - 2]. Dentoalveolar abscess was seen in relation to mandibular right and left first primary molars. Discoloration of the primary maxillary right central incisor was evident. The occlusion showed a deep bite with excessive overjet [Figure - 3].

An OPG along with intra oral periapical radiographs of mandibular molars were taken. Severe bone loss in relation to the mandibular first molars was noted. The child was referred for Pediatric opinion in view of blue sclerae and the past history of frequent fractures. X-rays of the long bones [Figure - 4][Figure - 5], along with her medical, family history and the clinical findings confirmed the diagnosis of Osteogenesis imperfecta type I.

The dental treatment was planned under general anesthesia as the child was very uncooperative and the use of physical restraints was not advisable in view of fragility of her bones.

Mandibular primary right and left first molars were extracted and the other carious teeth were restored using glass ionomer cement. The post-operative recovery and healing was satisfactory and regular follow ups were carried out. The child is presently asymptomatic.


  Discussion Top


Osteogenesis imperfecta (OI) may be accompanied by dentinogenesis imperfecta (DI) and hence, craniofacial, oral and dental manifestations may be observed. These can be of great significance if the overt physical signs of osteogenesis imperfecta are not evident and the diagnosis is uncertain. Teeth affected by dentinogenesis imperfecta present with an opalescent grayish-brown hue. The enamel may be of normal thickness, but frequently is dislodged exposing the softer dentin. Enamel dislodgement may be attributed to the smooth dentino-enamel junction (DEJ), which is normally scalloped, in the teeth of such patients.[7]

The radiographic findings reveal ampullar extensions of the pulp chamber,[6] shortened roots and bulbous crowns that constrict at the cervix. Pulp chamber and root canals may be partially or totally obliterated.

The histological examination of dentin often reveals presence of irregular dentinal tubules, amorphous areas, embedded cells and poor calcification.[8],[9]

Skeletal class III malocclusion has been described in many patients with type III and type IV osteogenesis imperfecta. However, contrary to this, our case showed a developing class II occlusion. Anterior and posterior crossbites have also been reported.[4]

Oral manifestations specific to dentinogenesis imperfecta are commonly observed in patients with osteogenesis imperfecta and the evidence of disturbances in dental development can be crucial for establishing the diagnosis of osteogenesis imperfecta.[1]

Regarding the dental management of children with Osteogenesis imperfecta, it is noteworthy that many clinicians appear reluctant to provide dental care to such children. This could be due to the possible fear of causing maxillary / mandibular fractures as these children have an increased susceptibility to bone fractures. It should be emphasized that these children should be handled gently, avoiding excessive force during extractions. The administration of prophylactic antibiotics is not indicated for routine dental treatment procedures.[10]

Palmidronate, a biphosphonate drug is now being advocated for the treatment of osteogenesis imperfecta. It is administered intravenously in a dosage of 7.5 mg/kg/year at four to six month intervals. This treatment has been reported to yield biochemical, radiological and histomorphometric evidences of decreasing the rate of bone loss, reducing chronic bone pain and improving patient mobility.

The parents must be instructed regarding oral hygiene maintenance, as poor oral hygiene will cause dental problems that will bring additional pain, discomfort and complications for their children.

 
  References Top

1.Malmgren B, Norgren S. Dental aberrations in children and adolescents with osteogenesis imperfecta. Acta Odontol Scand 2002;60:65-71.   Back to cited text no. 1    
2.Cole WG. Advances in osteogenesis imperfecta. Clin Orthop 2002;402:6-16.   Back to cited text no. 2    
3.Mahoney EK, Widmer RP, Sillence DO. Opalescent dentin in two affected siblings. N Z Denr J 2001;97:15-8.   Back to cited text no. 3    
4.O' Connel AC, Marini JC. Evaluation of oral problems in an osteogenesis imperfecta population. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87:189-96.   Back to cited text no. 4    
5.Sillence DO. Osteogenesis imperfecta: an expanding panorama of variants. Clin Orthop 1981;159:11-25.   Back to cited text no. 5    
6.Petersen K, Wetzel WE. Recent findings in classification of osteogenesis imperfecta by means of existing dental symptoms. J Dent Child 1998;65:305-9.   Back to cited text no. 6    
7.Lindau BM, Dietz W, Hoyer I, Lundgren T, Storhuag K, Noren JG. Morphology of dental enamel and dentin-enamel junction in osteogenesis imperfecta. Int J Paediatr Dent 1999;9:13-21.  Back to cited text no. 7    
8.Koreeda-Minra M, Oaishi T, Oshima T. Significance of histopathologic examination in the diagnosis of dentin defects associated with type IV osteogenesis imperfecta. Two case reports. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;95:85-9.  Back to cited text no. 8    
9.Malmgren B, Lindskog S. Assessment of dysplastic dentin in osteogenesis imperfecta and dentinogenesis imperfecta. Acta Odontol Scand 2003;61:72-80.   Back to cited text no. 9    
10.Advisory statement. Antibiotic prophylaxis for dental patients with total joint replacements. American Dental Association: American Academy of Orthopaedic Surgeons. J Am Dent Assoc 1997;128:1004-8.  Back to cited text no. 10    


    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]

    Tables

[Table - 1], [Table - 2]


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   Abstract
   Introduction
   Case Report
   Discussion
   References
   Article Figures
   Article Tables

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