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UPDATE: Dental Management of a Rare Hereditary Disease: HHT

Terry Pannkuk, DDS, MScDDiagnostic Puzzles, Instructional, Sample Cases, All by Date


Preoperative periapical radiograph


Preoperative radiograph (horizontal angle)


Telangiectases noted on the dorsum of the tongue


A red rash noted on the lower lip


The throat revealed an accumulation of blood from a recent episode of epistaxis


Gross caries noted (tooth #5)


Initial caries control aided with caries indicator dye


Stepwise removal of caries with some remaining soft dentin in the distal box area


Completed caries control


Cleaning and shaping of the buccal and lingual canal systems


Completed toughing of the interconnecting buccal-lingual fin


A small layer of Cavit after placement of intracanal calcium hydroxide


Wedging of a matrix band prior to placement of the temporary


Placement of the Fuji IX LC temporary material. Gingivectomy was subsequently performed to capture a 1mm ferrule for restoration


Gingivectomy of the distal tissue facilitated matrix band placement and aseptic isolation at the obturation visit


Immediate post-treatment radiograph

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Patient: 68 year old female with hereditary hemorrhagic telangiectasia (HHT)

Chief Complaint: cold sensitivity and a toothache

Dental History: The patient had visited her dentist who performed a full mouth exam with radiographs finding that the maxillary right first bicuspid (tooth #5)had extensive Class II distal caries with a pulp exposure

Significant Findings (tooth #5): The patient presented with multiple scarlet-tinged telangiectases on the dorsum of the tongue and an irregular rash on the lower lip. Examination of the throat revealed accumulation of blood from a recent nosebleed that had been treated with laser surgery earlier in the morning. Tooth #5 revealed a sharp transient response to cold thermal stimulation and moderate percussion sensitivity. A mesial marginal ridge fracture was noted with normal periodontal probing depths. The radiographic series revealed extensive caries involving the pulp.

Pulp and Periradicular Diagnosis (tooth #5): Degenerating pulp with acute periradicular periodontitis

Treatment Prognosis (tooth #5): Guarded to Fair

Treatment Plan: Nonsurgical endodontic therapy (tooth #5)

Special Considerations of Performed Treatment:

Hereditary hemorrhagic telangiectasia (HHT) is also called Osler-Weber-Rendu Syndrome and is an autosomal dominant hereditary disease characterized by nosebleeds (epistaxis), arteriovenous malformations, related pulmonary disease, digestive tract bleeding, and involvement of other organs. Classic telangiectases spot the tongue as multiple red vesicular lesions and the lips display an irregular red rash. Antibiotic prophylaxis is advised before dental treatment due to the risk of a transient bacteremia causing a paradoxical brain embolism or infection.

In this case the patient presented with an emergency need for relief of a toothache. The patient was taking Amoxicillin for the recent incident of epistaxis and was covered for initiation of endodontic treatment. Tooth extraction was considered undesirable compared to endodontic treatment due to bleeding concerns.

The image series shows the endodontic emergency procedure including caries control with the aid of indicator dye. A Fuji IX temporary restoration was placed after pulp extirpation and placement of calcium hydroxide. A second visit was planned two weeks later to finish endodontic treatment and build-up the tooth in preparation for restoration. The AAE guidelines recommend a 1mm minimum circumferential ferrule. In this case crown lengthening will be required before the crown is placed.

Surgical gingival reduction was performed and the bleeding was unremarkable. At two weeks treatment was finished with final obturation via the vertical compaction of warmed gutta percha technique. A "floating" matrix band was fixed utilizing Dycal during the placement of a carbon fiber post and resin bonded core (DT Light Post, Photobond, and Luxacore). The patient was then referred back to her restorative dentist for a full-coverage crown restoration.

Key Learning Points:

1. Patients with hereditary hemorrhagic telangiectasia require antibiotic prophylaxis before dental treatment

2. A 1mm circumferential ferrule is the minimum acceptable remaining dentin collar required for predictable stable restoration according to the AAE guidelines

3. Gingival reduction or crown-lengthening is essential if a resin-bonded restoration is be placed subgingivally

4. Team communication between the endodontic specialist and restorative dentist is important to make sure the appropriate restorative design and plan is executed

5. Dycal is a useful "floating" matrix band luting material

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