Sickle Cell Anemia: A Review for the Pediatric Dentist

Resident: Tyler Roberts
Article title: Sickle Cell Anemia: A Review for the Pediatric Dentist
Author: Fonseca DDS, et al
Journal: Pediatric Dentistry
Volume #: 29(2) pages #: 159 - 169
Year: March 2007
Major topic: review of Sickle Cell Anemia
Type of article: review
Key pts/findings

Sickle Cell Anemia is characterized by a person who carries a homozygous genetic trait - a substitution of valine for glutamic acid at position 6 of the beta globin chain of adult (A1) hemoglobin. This trait affects the structure and synthesis of hemoglobin and causes general complications of painful crises, stroke, pulmonary disease, delayed growth, osteomylitis, organ damage, psychosocial dysfunction. Oral and dental manifestations include orofacial pain, paresthesia of the mental nerve, stepladder appearance of the alveolar bone on radiographs, pulpal necrosis and enamel hypomineralization.

It was originally thought that sickling of the RBC caused micro circulatory obstruction during capillary transit thus leading to a vaso occlusive crisis and manifestations of the disease. Today it is understood that sickling causes RBC adhesion to endothelium, thus leading to the formation of hetero cellular aggregates causing local hypoxia and chronic inflammation within the circulatory system.

The only available curative therapy for the disease is hemotopoeitic stem cell transplantation that must be done prior to organ dysfunction. However, most patients use anti-inflammatories and opoids to manage their conditions. Blood transfusions have also proved important in slowing the progression and easing the painful symptoms of this disease.

Patients that exhibit low risk complications can and should have outpatient dental services performed including restorations and simple extractions. Moderate to high risk patients should be treated in a fully equipped operating room. A consultation with an anesthesiologist as well as a hematologist should be obtained before hand. Lung function as well as the risk for periopertive and postoperative complications should be assessed and the possible need for blood transfusion prior to appointment should be discussed.

Pediatric dentist: restorations are preferable to Extractions. Treatment plans should be completed prior to a patient beginning bisphosphonate therapy( no case reports of ON with children on bisphosphonates have been reported to date). Local anesthetic with vasoconstrictor is appropriate. Nitrous oxide is considered safe as long as concentrations of at least 50% oxygen are administered at all times. Oral sedation can be used, try to avoid drugs that cause respiratory depression. Mild to moderate pain can be managed with NSAIDS or Acetaminophen; avoid aspirin due to Reyes syndrome. The need for prophylaxis is controversial with no consensus at this point in time. Elective surgeries such as extraction for orthodontics should be avoided if possible.

List of other oral complications: delayed eruption, dentin hypomineralization, pulp calcification's, pulpal necrosis, glossitis, gingival enlargement, mental nerve paresthesia, neuropathy, stepladder appearance of trabecullar bone, prominent zygomatic and parietal bones, thin border of the mandible, mandibular radiopaque lesions, osteomylitis, osteoporosis, malocclusion, mucosal jaundice etc

Summary: Sickle cell disease is most severe in those found with the homozygous trait. Anti inflammatories and opoids along with blood transfusions are the main forms of treatment. Complications resulting from this disease can vary and be wide spread. Dentist should always obtain a good medical history and consult with other health professionals before delivering care to these patients.