Introduction:
Hereditary hemorrhagic telangiectasia (HHT) is a major autosomal dominant disease of genetic origin that is also referred to as ‘OWRD- Osler-Weber-Rendu Disorder’ with a global incidence of one person for every 5000 individuals. Seldom are the oral manifestations of this genetic disease highlighted in medical literature. However, research in dental and maxillofacial surgery has focused on the oral manifestations of this disease, which particularly sheds light on generalized chronic periodontitis (a chronic inflammatory disease in which the gums and teeth are affected due to aggregation of dental plaque) and the multiple tooth avulsions (knocked-out teeth) that may occur in individuals affected by this syndrome. Oral aspects of this genetic syndrome are discussed in detail in this article.
Why Are the Oral Manifestations in HHT Patients Underestimated?
The oral manifestations in HHT patients are underestimated because:
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HHT is primarily an angiogenesis disorder that is accompanied by several arteriovenous malformations in the affected individuals. The other major diagnostic feature of this syndrome is nose bleeds. Nose bleeding is not only one of the common clinical symptoms but is a frequently occurring feature in many patients.
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The dual features or complications associated with direct bleeding found in these patients affected by HHT make them highly prone to an increased hemorrhagic (bleeding) risk that needs constant monitoring by physicians. Further, this also needs to be considered during the intervention or adoption of surgical strategies for addressing the issue of hemorrhages.
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Not many physicians or even general surgeons focus on the maxillofacial aspects of the disease. As frequent nose bleeding is one of the diagnostic symptoms mentioned earlier, this can be a factor in diagnosing the condition where maxillofacial surgeons can play an important role in modifying and addressing the patient's dental functions or care.
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Dental care in these patients requires a lot of implications that need to be considered by the maxillofacial surgeon because these patients would be at an increased risk of bleeding post-oral surgical or dental procedures.
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Just as is the case of hemorrhages in HHT patients as with any other surgical procedure, the same implications apply to routine dental care as well. Hence, these hemorrhage risks need to be addressed by the maxillofacial surgeon through careful pre-operative evaluation, antibiotic therapy, and interdisciplinary collaboration with the physician regarding drug dosage and administration to prevent bleeding risk during the dental procedure. Post-operative care also needs to be taken accordingly after the dental or oral surgical procedure.
What Are the Dental Management Protocols for HHT Patients in an Ambulatory Setting?
Concerning the oral cavity, chronic periodontal disease or periodontitis could manifest in these patients due to breached oral immunity and underlying hemorrhagic systemic disease patterns. There is the possibility of many teeth being prone to grade 2 or grade 3 mobility (moderate to severe mobility) and would be further prone to avulsions or severe displacement of the tooth from the jaw or the alveolar bone socket. Hence, HHT patients suffering from chronic periodontal disease or chronic periodontitis would need multiple extractions, gingival and periodontal therapy, and dental rehabilitation with prosthetics.
However, owing to the hemorrhagic risk, dental or maxillofacial surgical surgeons can perform dental or oral surgical procedures and periodontal therapies only in the operating room of an ambulatory surgical unit or in a hospital setting, to facilitate the monitoring of the patient post-operatively. Tooth avulsion surgeries or dental extractions can be performed under local anesthesia and the technique of conscious sedation can be done with pre-operative and post-operative antibiotic prophylaxis administration.
Care should be taken to achieve adequate hemostasis (the mechanism of cessation of bleeding) during and after the procedure. Further, avulsed maxillary (upper jaw) molar teeth in these cases would be prone to be displaced within the maxillary sinus if left untreated, which can lead to life-threatening complications. These complications include septic shock (a life-threatening condition that occurs when blood pressure falls to a dangerously low level following an infection), cavernous sinus thrombosis (a blood clot in the cavernous sinuses), or intracranial complications that can increase the risk of mortality.
In such cases of sudden avulsion injuries in the infected patients of HHT, alveolar crestal surgery (a surgical technique in which incisions are made in the alveolar ridge and sinus wall to expose the bone and elevate the Schneiderian membrane) or Caldwell Luc surgery (a surgical procedure to treat severely impacted maxillary sinuses) or operation would be deemed suitable. though risky, in an ambulatory setting or surgical unit of the hospital with extreme precautions to establish proper blood control or hemostatic control.
How Can Nasal Epistaxis Be Managed in HHT Patients?
Often HHT patients who report severe epistaxis (nasal bleeding) from the nose clinically would require frequent admissions to the emergency department of a hospital or imminent hospitalization. Also, physicians would treat HHT patients by prescribing them frequent iron infusions to prevent hemorrhagic risks. For nasal bleeding, the maxillofacial surgeon or the plastic surgeon would treat it with collagen compresses given in the nasal vestibular region with interrupted sutures to prevent bleeding recurrence.
The nasal cannula would be positioned near the jaw or the oral cavity region and compression or hemostasis would be achieved by antifibrinolytic agents like Tranexamic acid.
What Is the General Management of HHT Patients?
The general management of HHT patients involves treating the arteriovenous malformations and their hemorrhagic complications encountered by the general surgeon and the physician. Rapid hemostatic methods to control nasal bleeding, and during dental or maxillofacial surgeries as elaborated above in a hospital setting, surgical closure and management of the nasal cavity through procedures like Young's operation (in severe cases), management alongside orofacial implications, laser photocoagulation or sclerosing therapies in hemorrhagic management, and so on are some of the options that are generally considered during preoperative and postoperative evaluation by the physician and general surgeon. Timely diagnosis and management play an important role in modifying and addressing the patients' concerns preventing life threats, and improving the post-operative quality of life of the patients.
Conclusion:
To conclude, several oral and maxillofacial manifestations with a high risk of bleeding or hemorrhage and neurovascular infections, and dental, periodontal, or sinus infections if left untreated can lead to life-threatening complications in these patients. The role of the maxillofacial surgeon in the management of HHT patients is hence an imperative protocol that needs interdisciplinary collaboration with physicians and other accessory specialists.
Proper or rather strict oral hygiene measures, regular dental follow-ups with the dental or maxillofacial surgeon, and customized dental management on the nature of clinical symptoms such as chronic periodontitis, avulsion injuries, tooth extractions, and so on considering the hemorrhagic and infection risk is essential in the dental or maxillofacial management of HHT patients.
