Dental Considerations in the Management of Glanzmann's Thrombasthenia

Abstract

Glanzmann's thrombasthenia, is one of the rarest congenital, genetically inherited platelet disorder. It has an incidence of about 1:1,000,000, but is more common in populations with increased consanguinity. Glanzmann's thrombasthenia is characterized by deficiency or dysfunction of glycoprotein (GP) lib and Ilia, which are the receptors of fibrinogen. Both sexes are equally affected. Typical mucocutaneous bleeding occurs at birth or early infancy. Obtaining appropriate dental history of excessive bleeding after dental extraction, unexplained spontaneous mucocutaneous bleeding, gingival bleeding during teething or shedding of deciduous teeth and petechiae, ecchymoses or purpura on mucous membranes can play an important part in diagnosis. Hence, the pediatric dentist plays a very crucial role for prompt diagnosis and management of Glanzmann's thrombasthenia. Presenting here is a known case of Glanzmann's thrombasthenia, of a 6-year-old girl who required to undergo dental extraction and its successful management using an "acrylic-splint" along with the placement of "Calgigraf-Ag Foam".

Keywords: Glanzmann’s thrombasthenia; pediatric dentist; platelet disorders; “Acrylic-splint” appliance..

Fig. 1:

Girl diagonosed with Glanzmann’s thrombasthenia

Fig. 2:

Maxillary occlusal photograph showing grossly destructed 65

Fig. 3:

Mandibular occlusal photograph showing proximal caries on 75 and arrested grossly carious 85

Fig. 4:

IOPA of 65 showing radiolucency involving enamel dentin and pulp with severe periradicular bone loss

Figs 5A and B:

IOPA of 75 and 85 showing radiolucency involving enamel and dentin

Fig. 6:

85 restored with a stainless steel crown and 75 with composite

Fig. 7:

Healing of socket satisfactory after 7 days

Fig. 8:

“Liver-clot” seen in socket of 65

Fig. 9:

Calgigraf AG-foam

Fig. 10:

Fabricated acrylic splint appliance

Fig. 11:

Appliance positioned in the child’s mouth

Fig. 12:

Healing satisfactorily complete at 2 month recall visit