Thalassemias and their Dental Implications

Meghan Sullivan Walsh March 9, 2011

Literature Review - St. Joseph/LMC Pediatric Dentistry

Thalassemias and their Dental Implications

Resident: Meghan Sullivan Walsh

Program: Lutheran Medical Center- Providence

Article Title: Thalassemias and their Dental Implications

Authors: Antonio Cutando Soriano; Jose Antonio Gil Montoya, Juan de Dios Lopez-Gonzales Garrido.

Journal: Medicina Oral

Volume (number), Year, Page #’s; 7, 2002, pages 36-45

Major Topic: Thalassemias: clinical and orofacial manifestations, medical and dental treatment.

Overview of Method of Research: Current review of literature and author’s recommendations

Findings: Thalessemia is a hereditary form of anemia with 5 clinical classifications.

1) Severe beta-thalassemia or (Cooley’s anemia) can be separated into two parts according to whether regular blood transfusions are required; Thalassemia major (transfusion dependent) and Thalassemia intermedia (no regular transfusion required.) 2) Thalassemia trait is another classification with impaired alpha or beta-chain production. There are no clinical signs and splenomegalia is rare. 3) Hemoglobin H presents with a moderately severe deficiency in hemoglobin alpha-chain production. 4) Fetal hydropexis is a intrauterine manifestation of the severe form of alpha-thalassemia. Lastly there is the 5) silent carrier associated with the parent of a patient with hemoglobin H disease with no clinical signs.

Clinical Manifestations: The severity of clinical symptoms is dependent on the clinical classification. Many of these patients require continuous blood transfusions. Unfortunately due to these transfusions iron overload and accumulation in the blood stream is common and difficult to resolve. A point is reached where secondary hemochromatosis of the heart liver and endocrine glands can occur. Liver impairment is mild but fibrosis may occur leading to cirrhosis. Endocrine involvement may lead to growth and development impairment by age 10-13 resulting in hypogonadism. In older patients diabetes mellitus with hypothyroidism is noted. Patients with severe beta-thalassemia present with cardiac impairment, (pericarditis, congestive heart failure and arrhythmia's). CHF is the most common cause of death in secondary hemochromatosis. Beta thalasemmia patients have many degrees of cardiac impairment related to the number of blood transfusions. Death due to heart failure can occur between 17-19 years of age. Iron deposits will also affect salivary glands causing painful inflammation and diminished salivary flow.

Oral manifestations: Bony changes occur due to ineffective erythropoiesis with the formation of an erythroid mass. Malocclusions are common with severe maxillary protrusion as well as an anterior open and overbite. These malocclusions are commonly corrected by surgery (maxillary osteotomy and tooth extractions.) Orbital hypertelorism is also common hence the term ‘cooley facies’ or ‘mouse facies.’ Radiographically there is a brush-like image in the cranial vault. The alveolar bone shows rarefaction with thinning of the cortical layer. Obliteration of the paranasal sinuses and unilateral hypoplasia of the maxillary sinus are common findings. Intraorally, paleness of the oral mucosa is correlated to the severity of anemia. Caries and gingivitis more often occur in the patient who have received a splenectomy.

Medical treatment: Blood transfusions are common and have improved the life span of these patients, however harmful accumulation of iron is common. Deferoxamine is the most common drug used for these patients to eliminate body iron. This drug, however, does affect the immune system. Splenectomy is often performed to improve erhthropoiesis and prolong half-life. These patients are at risk for sepsis and are commonly given prophylaxis. Severe forms of thalassemia, infections and iron overload are the most common causes of death of these patients.

Dental Treatment: Obtaining a good medical history and diagnosis of the patient is crucial for treatment modalities. Orofacial deformities and malocclusions are often corrected by surgery and extractions however, these treatments are not recommended for patients with thalassemia major. Blood transfusions before and after treatment for the other types of Thalassemias are recommended. It is important to use caution when prescribing hepatotoxic drugs. Knowing your patients liver function and coagulation tests are pertinent information. Should the patient express signs of diabetes secondary to hemochromatosis, strict preventative measures should be employed similar to any diabetic patient. Periodontal bursae, gingivitis and infectious foci are commonly seen in patients with splenectomies. Patients with cardiac issues are always a major concern. Pericarditis and congestive heart failure are important issues and should be watched carefully even during simple dental procedures. Prescription drugs that affect the CNS should be used with caution as well as NSAIDS and certain antibiotics that may affect the GI. Using anesthetics with vasoconstrictors should be used sparingly. If the patient presents with hypersplenism with leukopenia and thrombocytopenia, antibiotic prophylaxis is recommended. In splenectomized patients sepsis is common and the oral cavity must be prevented from constituting a source of bacterial spread.

Key Points: Summary:

Medical improvements in the field of Thalassemia has made it possible for these patients to reach an adult age. Palliative treatment is no longer a standard. We must be acutely aware as dentist to the severity and medical treatment our patients are following so that we can best treat these patients safely.

Assessment of the Article: Thorough article. I appreciated the attention given to specific dental treatment modalities while handling these patients.