Taghreed Ahmed Al Harbi et al, 2020;4(3):567–571.
International Journal of Medicine in Developing Countries
Sickle cell anemia in dentistry: manifestations and management
Taghreed Ahmed Al Harbi1*, Saad Dakilallah Al Harbi2, Manal Al Malik3, Omar Abd El Sadek El Meligy4
Correspondence to: Taghreed Ahmed Al Harbi
*Senior Registrar Pediatric Dentist, Assistant Dental Continues Quality Improvement and Patient’s Safety Facilitator, King Fahad Armed Forces Hospital, Jeddah, Saudi Arabia.
Email: Taghreed1985 [at] gmail.com
Full list of author information is available at the end of the article.
Received: 03 january 2020 | Accepted: 13 January 2020
© 2020 IJMDC. Published by Discover STM Publishing Ltd.
Sickle cell disease (SCD) is considered as one of the most common genetic diseases. It causes an alteration in the shape and function of the red blood cells. In addition, hemolytic anemia and vascular occlusion are considered as the main clinical features of sickle cell. The study aimed at declaring the oral manifestations, considerations, and management of dental patients with SCD. A MEDLINE search was completed for the time period from 1990 to 2019 along with a manual search to locate relevant peer-reviewed articles and textbooks published in English. Several keywords were used to obtain all possible articles concerned with the subject. We obtained 25 articles; only 21 articles were specifically related to our subject. The study concludes that few studies have focused on studying the oral manifestations, dental considerations, and management of dental patients with sickle anemia and most of them were trials.
Sickle cell, oral manifestations, dental considerations, management.
Sickle cell anemia (SCA) is a genetic disease caused by the replacement of glutamic acid by valine in position 6 at the N-terminus of the beta-chain of globin . This genetic alteration results in hemoglobin S formation . After that, de-oxygenation (hypoxia) results in shape alteration of erythrocytes that predominantly contain hemoglobin S, which changes into a shape resembling a sickle . The sickle shape is considered reversible according to the oxygen levels, but constant changes in shape result in a rigid cell membrane of the erythrocytes that cannot return to their normal state [4,5]. Diagnosis of SCA can occur in the sixteenth week of gestation, but manifestations normally do not appear till the sixth month after birth, the diagnostic technique is done through neonatal screening test by taking blood drops from the heel of the newborn to allow the formation of hemoglobin electrophoresis . Hypoxia may lead to circulatory difficulties, like vaso-occlusive conditions, which decrease the erythrocytes life span to 20 days . Furthermore, the sickled erythrocytes have a high affinity to adhere to the endothelium of the blood vessel walls and may cause microvasculature obstruction decreasing the blood flow leading to many hazardous effects like pain, tissue anoxia, and tissue necrosis . In Saudi Arabia, sickle cell disease (SCD) was first reported in the Eastern province in the 1960s. A study has reported that the prevalence of sickle cell trait in Saudi Arabia ranges from 2% to 27% and up to 2.6% will have SCD in some areas .
Diagnosis of SCA
The sickle cell test is done to identify the presence of hemoglobin S. First, a blood sample is collected from the neonatal heel then the red blood cells are deoxygenated using a deoxygenating agent like meta-bi sulfate . After that, cells sickling will happen to the patient who has the disease, but the patient who is homozygous for SCA disease would show a rapid rate for red blood cells sickling . Detection of hemoglobin S can occur using solubility tests, but these tests are not decisive for sickle cell diagnosis until performing electrophoretic examination . Many studies have concluded that patients diseased with SCA have a decreased hemoglobin value (5–9 g/dl) while the normal range is 12–17 g/dl. In addition, the normal range of white blood cells in normal healthy patients ranges from 400 to 11,000/mm3 while in SCA patients, the cell count is increased ranging from 12,000 to 20,000/mm3 .
Oral Manifestations of Patients with SCA
There are various oral manifestations that may be accompanied by SCD like yellow tissue coloration, mucosal pallor, gingival enlargement, malocclusion, papillary atrophy of the tongue, nerve damage to the inferior alveolar nerve, pulpal necrosis of healthy teeth, pulpal calcification, radiographic abnormalities, changes to the superficial cells of the tongue, delayed tooth eruption, intrinsic opacity of enamel mandibular osteomyelitis, and dental caries; however, it is little in SCA patients compared with patients with normal hemoglobin A and disorders of enamel and dentin mineralization (hypomineralization), degree of periodontitis, which is unusual in children, diastema, and hypercementosis [14–16].
In the following, the literature tries to summarize the main oral and dental manifestations with sickle cell and its causes.
Pulp necrosis is considered as a common dental pathology in sickle cell patients leading to crown color-changing . Mainly, pulp necrosis originates from the vaso-occlusion of the blood supply. The pulp necrotic teeth do not respond to vitality tests and may be painful or painless . Hence, the prevention of infection in sickle cell patients is highly demanded by prescribing antibiotics. Antibiotic prophylaxis with penicillin is prescribed to children for the long term . On the other hand, the prescription of long-term antibiotics has oral side effects like increased fungal infection and the emergence of normal saprophytic fungi .
Pathologies of the oral mucosa, tongue, and periodontium
Oral mucosa of sickle cell patients usually show altered pallor color due to a decrease in hematocrit and may be yellowish in color due to the existence of hemolytic anemia . Furthermore, papillae of the tongue may undergo atrophy in sickle cell patients, also smooth reddish pigmentation may appear on the tongue . Blood extravasation can occur also in the gingiva leading to gingival enlargement. In addition, in the heterozygous form of sickle cell trait, the density of the trabecular bone is decreased, making it more liable for periodontitis .
Neuropathy of the mental nerve
Infarction of vascularization to the lower mental nerve branches may occur in SCA, leading to loss of sensation in the chin area . Many studies have concluded that mental nerve neuropathy is common in a pain crisis . This condition of sensitivity loss in the chin area, which may affect the lower lip, is reversible and sensitivity returns to the affected area gradually after several months .
Vaso-occlusion of the blood supply may lead to bony ischemia and small areas of bony necrosis. Pain occurrence in the mandibular bone is common in sickle cell patients especially in the posterior region due to various causes, like less arterial replacement with arterial thrombus occurrence . Bone necrosis and infarction occurrence may lead to osteomyelitis and there is a difficulty to differentiate between early bony infarction and osteomyelitis . By radiographic examination of the jaws of SCA patients, many researchers have reported the existence of abnormal manifestations, which appear like thin inferior border of the mandible, increased radiolucent areas in both jaws due to decreased trabeculae in the jawbones, salmonella infection (usually occur leading to generalized osteoporosis), coarse pattern of trabecular bone, existence of radio-opaque areas in the jaws, and creation of stepladder effect by horizontal rows of trabeculation in the interdental alveolar bone .
Many studies reported that cephalometric analysis of SCA patients shows the presence of maxillary protrusion, retroclined maxillary, mandibular incisors, and forward advancement of the mandible .
Systemic Manifestations of SCA
SCA has various systemic manifestations associated with different conditions as described in the following sections.
Painful episodes or crisis
This disease has different systemic manifestations like hyperhemolysis that is the least common type of crisis associated with deficiency of glucose-6-phosphatase, sequestration, vaso-occlusion, and abnormal heart rate, which may occur due to different causes like myocardial damage from infarctions, pulmonary arterial occlusion, chronic anemia, increased breathing, and aplastic crisis characterized by severe anemia due to non-productive bone marrow, which is responsible for erythrocytes production, exaggerated pain, and abdominal tenderness .
Many studies on this disease have found that many precipitating factors occur before the crisis, which may include infection, acidosis, cold feeling, emotional disorder, stress, hypoxia during surgery under general anesthesia, and dehydration, which is a trigger for sickle cell crisis. So that, administration of intravenous fluids is important during and after surgery if general anesthesia is done .
SCA in infancy
This type of SCD occurs mainly around two years of age but can be diagnosed early at the age of six months. It is characterized by a special syndrome called sickle cell dactylitis [30,31]. This syndrome is characterized by swelling of the hands and feet (symmetrical hand-foot syndrome), and this is because of the vaso-occlusion of the microvasculature in the small bone .
This disease occurs mainly in young children. It is characterized by enlarged liver and spleen due to huge blood accumulation in these organs. The liver enlargement causes increased unconjugated bilirubin, which may lead to jaundice and scleral icterus. The spleen may be palpable in children due to its enlargement, but it decreases in size due to fibrosis occurrence . The decreased size of the spleen may lead to increased bacterial infection in the infant, especially those infants who have not developed an adequate immunity to certain bacterial pathogens. Furthermore, gallstones may be detected in the second and third decades of the patient .
Dental Considerations and Management of Sickle Cell Patients
Medical management of SCA
First, the dentist should know the medical history of the sickle cell patient before initiating the treatment. At the present time, no medication has been discovered for SCA, also there is no treatment program for crisis prevention, only analgesics and other medicines are prescribed to decrease the pain but not regularly to avoid drug addiction. Folic acid may be given daily to children to decrease the severity of anemia . In addition, antibiotics are prescribed to prevent infection, also medicines that are important to overcome dehydration and acidosis. Steroidal anti-inflammatory drugs are contraindicated to avoid the risk of triggering serious pathologies like acute chest syndrome. For sickle cell patients, the most preferred safe analgesic treatment is a combination of paracetamol and codeine. In addition, morphine and its derivative analgesics can be used . Aspirin should not be used to avoid the risk of acidosis.
Cautions regarding infection
Sickle cell patients are at high risk for infection, hence prevention of infection is a priority. In those patients who had functional asplenia or had undergone splenectomy, prophylactic antibiotics should be prescribed for them for prevention of infection, also the prophylactic antibiotics are prescribed for cardiac patients to prevent bacterial endocarditis if they will undergo special dental operation like endodontic treatment, teeth extraction, and all other surgical operations . Management of osteomyelitis in the mandibular region can be done in various procedures like curettage, bony sequestration, debridement, or partial bony resection in combination with antibiotics .
Cautions regarding anesthesia
It is preferred to use sedative agents like an equimolar mixture of oxygen and nitrous oxide, or anxiolytic before local anesthesia injection to decrease the patient’s stress. Deep local anesthesia is preferred to avoid stressful situations, which may lead to occlusive vascular pain.
General anesthesia is preferred to be avoided, but if it is unavoidable, it is important to bring the preoperative hemoglobin level to 10 g/dl before general anesthesia for anemia correction .
Oxygen is used mainly for pediatrics that can be used for SCA patients in the case of preservation 50% oxygen concentration with a high flow rate and adequate ventilation.
Light doses of oral sedation are important to decrease anxiety, however, if a moderate dose is needed, the oxygen concentration should be increased through a nasal cannula .
It is considered a very important side during the management of sickle cell patients. Many studies concluded that children patients with SCA are usually short and thin, have abnormal body weight, and unable to keep up physically with their colleagues due to the inability to control the crisis . In addition, these children are often absent from the school and their score in the intellectual exams is low due to their frequent crisis and hospitalization, so the patients may develop low self-esteem. Hence, the dentist and the patient’s family should support the patient strongly for improving their self-confidence .
Restoring the carious hypo mineralized teeth is better achieved with full coverage crowns rather than dental restorations to protect the hypo mineralized nature of the enamel structure in SCD patients. Extreme care should be taken regarding infections. Any source of infection either dental or non-dental source should be treated vigorously with SCD patients to decrease the chance of crisis caused by infections. That means the pulp therapy is a case selection and it is not always a good option especially in the case of the possibility of treatment failure. At that point, extraction is the best option to eradicate the chances of failure. The latter treatment option should be exerted with extreme care to preserve as well as protect the fragile, osteoporotic bone from fracture. In addition, to decrease the risk of future osteonecrosis that may accompany traumatic extractions .
Sickle cell is a genetic disorder, which shows different oral and dental manifestations. It is important for the dentist to take careful precautions during operating any dental procedure for sickle cell patients. There is a scarcity in studies conducted on studying the oral manifestations, dental consideration, and management of the dental patient with SCA, also most of them are trials and showed unclear results. It is recommended to establish more studies, which focus on declaring oral manifestations of SCA and the precautions taken by the dentists during management and performing any dental procedure.
List of Abbreviation
Conflict of interest
The authors declare that there is no conflict of interest regarding the publication of this article.
Consent for publication
Taghreed Ahmed Al Harbi1, Saad Dakilallah Al Harbi2, Manal Al Malik3, Omar Abd El Sadek El Meligy4
|How to Cite this Article|
Harbi TAA, Harbi SDA, Malik MA, . Sickle cell anemia in dentistry: manifestations and management. IJMDC. 2020; 4(3): 567-571. doi:10.24911/IJMDC.51-1578043152
Harbi TAA, Harbi SDA, Malik MA, . Sickle cell anemia in dentistry: manifestations and management. http://www.ijmdc.com/?mno=80440 [Access: March 29, 2020]. doi:10.24911/IJMDC.51-1578043152
AMA (American Medical Association) Style
Harbi TAA, Harbi SDA, Malik MA, . Sickle cell anemia in dentistry: manifestations and management. IJMDC. 2020; 4(3): 567-571. doi:10.24911/IJMDC.51-1578043152
Harbi TAA, Harbi SDA, Malik MA, . Sickle cell anemia in dentistry: manifestations and management. IJMDC. (2020), [cited March 29, 2020]; 4(3): 567-571. doi:10.24911/IJMDC.51-1578043152
Harbi, T. A. A., Harbi, . S. D. A., Malik, . M. A. & (2020) Sickle cell anemia in dentistry: manifestations and management. IJMDC, 4 (3), 567-571. doi:10.24911/IJMDC.51-1578043152
Harbi, Taghreed Ahmed Al, Saad Dakilallah Al Harbi, Manal Al Malik, and Omar Abd El Sadek El Meligy. 2020. Sickle cell anemia in dentistry: manifestations and management. International Journal of Medicine in Developing Countries, 4 (3), 567-571. doi:10.24911/IJMDC.51-1578043152
Harbi, Taghreed Ahmed Al, Saad Dakilallah Al Harbi, Manal Al Malik, and Omar Abd El Sadek El Meligy. "Sickle cell anemia in dentistry: manifestations and management." International Journal of Medicine in Developing Countries 4 (2020), 567-571. doi:10.24911/IJMDC.51-1578043152
MLA (The Modern Language Association) Style
Harbi, Taghreed Ahmed Al, Saad Dakilallah Al Harbi, Manal Al Malik, and Omar Abd El Sadek El Meligy. "Sickle cell anemia in dentistry: manifestations and management." International Journal of Medicine in Developing Countries 4.3 (2020), 567-571. Print. doi:10.24911/IJMDC.51-1578043152
APA (American Psychological Association) Style
Harbi, T. A. A., Harbi, . S. D. A., Malik, . M. A. & (2020) Sickle cell anemia in dentistry: manifestations and management. International Journal of Medicine in Developing Countries, 4 (3), 567-571. doi:10.24911/IJMDC.51-1578043152