INTRODUCTION

Sickle cell anemia (AF) is an autosomal recessive genetic disorder, in which hemoglobin (Hb) is structurally abnormal, resulting in episodic sickle-shaped erythrocytes formation¹. The clinical manifestations of the disease, such as tissue ischemia (particularly in the bones, lungs, brain, kidneys and spleen), the high susceptibility to infections and attacks of pain, are the result of two main pathological processes: vessel-occlusion and hemolysis^2,3.

In the maxillofacial region, patients with FA present characteristics such as mandibular retrusion, convex profile and maxillary protrusion³, pallor of the mucosa, radiographic abnormalities, delayed dental eruption, disturbances of dental mineralization, hypercementosis, higher incidence of caries and more severe periodontitis than normal ones in children^3-5. Furthermore, during a vaso-occlusive episode within the inferior alveolar artery, pulp necrosis can occur, even in healthy teeth6-8. Ischemic areas can be infected secondary by hematogenous route or by local invasion through the periodontal ligament, resulting in osteomyelitis^1,9.

All these characteristics require special care. The patient must be accompanied by a multidisciplinary health team throughout his life, with the main objectives of preventing acute crises and infections and adequate pain management10. Within the health team, the dentist also contributes to the achievement of these objectives and, for this reason, it is extremely important that you are familiar with the management of these patients.

The objective of this study was to present a review of the literature on the care that the dentist should consider during dental care of patients with sickle cell anemia.

 

Materials and methods

For this literature review, a search of the PubMed and SciELO databases was performed, using an association of words related to disease and dentistry as a search strategy (table 1). Articles including information on dental care for patients with sickle cell anemia, published between January 1990 and July 2018, in Portuguese, English or Spanish were included. Literature review studies or articles whose full text was not found were excluded.

 

Table 1. Search strategy in the Pubmed database.

El filtro del año de búsqueda se agregó a esta búsqueda: a partir de 1990. Esta búsqueda se adaptó a la base de datos Scielo.

Results

Initially, 67 articles were found in the PubMed database and 27 in SciELO. After reading titles and abstracts, 37 articles remained. Finally, after reading the full text, 18 were included in this qualitative review (Figure 1), all them were published in English.

 

Figure 1. Flow chart of article selection.
Fuente: Los autores

Among the articles included, nine were case reports, one was a series of cases, four were cross-sectional, one was a guidelines type and three were retrospective studies (table 2).

Table 2. Characteristics of the articles included.

AF: anemia falciforme
Fuente: Los autores

Preventive dental measures were found to be essential for patients with sickle cell anemia11. It is important to keep in mind that during dental care of patients with FA, certain precautions must be taken to reduce the factors that promote acute crises such as: acidosis, hypotension, hypoxia, infection, vasoconstriction, and stress¹².

 

Prevention

A good history is essential to understand the patient´s medical problems, concerns, and expectations. The dentist should be familiar with hematology tests (eg. biometry,coagulogram) that help identify the patient´s systemic health status and the risk of undergoing an invasive dental procedure. Likewise, it is very important to remember that the care of this patient is carried out within a multidisciplinary team, and so, it is necessary to maintain communication with other health professionals^13-16.

Access to preventive and basic dental care (for example, prophylaxis, active caries lesion control, fluoride application) is essential for patients with FA, with the aim of reducing acute use of health services and hospitalization^11,17,18. In addition to outpatient preventive procedures, patients should also receive nutritional counseling to promote a non-cariogenic eating plan and instructions on tooth brushing, flossing, and tongue cleaning4. Adequate dental care improves the quality of life of the patient, avoiding feeding difficulties, oral diseases and aesthetic problems, and facilitates the management of the disease by the hematologist¹⁹.

 

Invasive procedures

AF patients are exposed to major risk of infection due to inadequate neutrophil response and decreased splenic function. At the same time, infections can trigger a sickle cell crisis²⁰. To prevent infectious adverse events, prophylactic antibiotics should be prescribed before surgical, endodontic, and periodontal scaling procedures^12,14,21. Penicillin V, amoxicillin, and clindamycin are oral antibiotics that can be administered 1 hour before the procedure. Intravenous clindamycin or ampicillin are also options that can be administered 30 minutes before the procedure20. In the case of pediatric patients, amoxicillin is the antibiotic most prescribed by pediatric dentists and hematologists for antibiotic prophylaxis before dental procedures²².

Oral surgical procedures are feasible if the anemia is stable and there is no history of recent vaso-occlusive events. Blood transfusion is only warranted in very specific circumstances and is not administered to patients undergoing oral surgery who have hemoglobin levels greater than 7 g / dL^12,23.

Obtaining deep local anesthesia is important to reduce anxiety and the need for sedation. Theoretically, local anesthetics with vasoconstrictors could contribute to the occurrence of thrombotic or vaso-occlusive events, but there is no real evidence that these agents are contraindicated in patients with AF^20,24.

The use of resources that facilitate the healing of the oral mucosa reduces the duration of bone exposure to the oral environment. Therefore, it is recommended to use platelet rich plasma membranes or fibrin glue (fibrin sealant). The increased risk of bleeding can be controlled with local hemostatic procedures, such as manual compression, sutures, hemostatic sponge application, or fibrin glue¹².

 

Osseo integrated implants

A single study indicated that implant placement is not recommended in patients with sickle cell anemia, due to the risk of osteomyelitis and poor bone irrigation, which can affect osseo integration¹².

 

Stress management

Stress peaks are a trigger for crisis. The patient's anxiety can lead to holding his breath, decreasing oxygenation. For this reason, the use of sedation is recommended in anxious patients. However, this should be applied with caution, because deep sedation can cause episodes of apnea and deoxygenation^12,20.

Inhalation sedation with nitrous oxide is indicated (at least 50% O2), as well as the cautious use of intravenous sedation. The administration of oxygen for at least 5 minutes before sedation, and the maintenance of an oxygen cannula during and after the procedure is important^23,24.

The use of general anesthesia in patients with FA should be avoided whenever possible. The change in the partial pressure of oxygen, the blood flow, the pH and the low temperature produced during general anesthesia can promote the development of intravascular insufficiency and, therefore, a vaso-occlusive crisis24. The protocol for treating a patient with sickle cell disease under general anesthesia usually requires that a prophylactic transfusion be performed prior to treatment, with the aim of ensuring adequate levels of hemoglobin A (at least 60%) and not more than 40% of abnormal hemoglobin²³.

 

Orthodontic treatment

Knowing that many patients with FA can present dentofacial deformity, especially type II, they should be recommended to seek orthodontic treatment early, since most of the manifestations can be easily controlled in children during growth. During orthodontic treatment, it is necessary to avoid emotional stress. Very slight orthodontic forces must be used to avoid causing vaso-occlusive crises in the local blood circulation and the patient's oral hygiene status must be subject to strict control to avoid episodes of gingivitis or infections during treatment¹³.

 

Pain management

To relief pain in the orofacial region, it is advisable to use pain relievers such as paracetamol and codeine24. If the pain is severe, more powerful pain relievers, such as morphine, can be given. Adjuvants such as antidepressants or antihistamines can also be used. These are heterogeneous compounds that increase the effect of pain relievers4. The use of acetylsalicylic acid as an analgesic and other non-steroidal anti-inflammatory drugs should be avoided, since they can have an adverse effect on the acid-base balance, promoting acidosis^13,24.

 

Osteomyelitis

Osteomyelitis is one of the most important complications that the dentist can find in patients with FA. These patients have a 29 to 31% risk of developing osteomyelitis at least once in life, especially in long bones. Fortunately, AF-related osteomyelitis is rarer in the jaw, with a reported incidence of 3% to 5%². As with other infectious bone processes, osteomyelitis caused by sickle cell crisis should be treated with surgical bone debridement along with antibiotic therapy, supported by the result of tissue culture or secretion, and antibiotic sensitivity studies. Systemic management included rehydration, correction of nutritional levels, and pain control, and for this, it may be necessary to hospitalize the patient^2,25,26.

 

Discussion

This literature review showed a lack of prospective studies regarding dental care for patients with sickle cell anemia. This same result was found in a previous Cochrane systematic review27. Prevention measures appear to be clearly important, as are procedures for stress control, oxygenation, infection prevention, and pain control. However, there are controversial points that still need to be clarified and proven, such as the safety of vasoconstrictor local anesthetics and the success of Osseo integrated implants in patients with FA.

Neglect of oral health care has been found to have a negative impact on overall health. This is especially worrying in people with sickle cell anemia, since oral diseases can increase the systemic risk of infection, vaso-occlusive crisis and pain^3,11,17.

Furthermore, the clinical symptoms of the disease are factors that contribute to the construction of a negative family environment and, consequently, to the abandonment of oral health, especially in pediatric patients4,5. This situation highlights the importance of educational and preventive measures. In the life of patients with FA. In a cohort study, it was observed that the introduction of basic dental care (prophylaxis, extraction and simple restoration of carious teeth) in patients with FA can reduce the number of hospital admissions and the total number of days of hospitalization for these patients¹¹, decreasing costs for the health system.

Systemic disease can also cause oral manifestations that must be diagnosed and controlled4. Knowledge and awareness of the risks and recommendations involved in treating patients with sickle cell anemia is essential to reduce complications resulting from the invasive procedures we perform and to allow the diagnosis of oral lesions¹².

It is important to remember that PA patients are more likely to suffer from dental pain of undetermined or unspecified origin17. Many cases of pulp necrosis caused by vaso-occlusive crises, in apparently healthy teeth, can be misdiagnosed8. The same is true with osteomyelitis. Fortunately, osteomyelitis of the jaws caused by AF is a rare condition, however, due to its rarity; it can be under-diagnosed, allowing the disease to progress over a prolonged period².

Studies show that the majority of FA patients visit the dentist only when they feel pain^11,28. In addition, many patients experience some discrimination from dental surgeons, who often end up refusing to treat them for fear of trans and postoperative complications18. This fear is often due to a lack of knowledge of the characteristics of the disease. A Brazilian study observed that the level of knowledge that dental students obtain during the undergraduate course on hematological disorders is insufficient, which requires a curricular reformulation²⁹.

 

Conclussion

Procedures for the prevention and promotion of oral health are essential for the comprehensive maintenance of the health of patients with sickle cell anemia. Even so, during dental care for patients with FA, certain care must be taken, such as antibiotic prophylaxis, the use of sedation and stress reduction, hydration of the patient, good control of oxygen levels and maintenance of an infusion, adequate tissue to avoid vaso-occlusive crisis and infections. Further studies are still needed to clarify certain questions about dental care for FA patients, such as the safety of local anesthetics with vasoconstrictors and the implications of rehabilitation with osseo integrated implants.

 

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	Patricia Verónica Aulestia-Viera; https://orcid.org/0000-0002-3860-2360
	Isadora Dourado Cardoso Alves; https://orcid.org/0000-0002-7992-6724
	Gabriela Moura Chicrala; https://orcid.org/0000-0001-6628-3048
	Paulo Sergio da Silva Santos; http://orcid.org/0000-0002-0674-3759
	Luiz Alberto Valente Soares Junior; http://orcid.org/0000-0003-0717-7354