UPDATE: Dental Management of a Rare Hereditary Disease: HHT

Terry Pannkuk, DDS, MScD Instructional


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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