3/2/11 Dental Management of a Child with Congenital Sideroblastic Anemia: A Case Report

Department of Pediatric Dentistry
Resident’s Name:Murphy Program: Lutheran Medical Center - Providence
Article title:Dental Management of a Child with Congenital Sideroblastic Anemia: A Case Report
Author(s): Rochelle Lindemeyer, DMD, Erick Goldberg, DMD, Andres Pinto, DMD MPH
Journal: Pediatric Dentistry
Year. Volume (number). Page #’s: 2007. v29 NO4. 315-319
Major topic: Dental management of SA
Minor topic(s): Treatment of SA
Main Purpose: To review a case report of a child with SA
Overview of method of research: Case Report

Findings:
Sideblastic Anemias(SA) are made up of a group of acquired disorders that have the following traits in common.
1. Anemia
2. Presence of ring sideroblast in bone marrow
3. Impaired heme biosynthesis

The primary pathophysiology of sideroblastic anemia is failure to completely form heme molecules, whose biosynthesis takes place partly in the mitochondrion. This leads to deposits of iron in the mitochondria that form a ring around the nucleus of the developing red blood cell. The X-linked pattern of transmission is the most common form and generally occurs in males. Clinically the patient will present with symptoms including fatigue, dizziness, and decreased tolerance to activities. High iron levels may interfere with growth and development, and can cause cardiac arrythmias and congestive heart failure.

Treatment of children that have SA involves routine blood transfusions. This is done to
1. Maintain hemoglobin levels
2. Manage symptoms
3. Allow for normal growth/development.

Potential risks of this treatment is the development of autoimmunity to repeated transfusions, especially if started at a young age. These patients are often on deferoxamine, which is the current drug used to treat excess iron.

Case Report
Dentists at Children’s Hospital of Philadelphia were consulted regarding a 3 yo girl suffering from SA and intermittent neutropenia. The child was receiving transfusions every 4 weeks, and all of her lab values were normal. A dental exam revealed a full primary dentition w/ maxillary incisors that were unrestorable. The hematologist was consulted, and GA was decided to be the best treatment for the child. Pre-op clindamycin was given due to the child history of neutripenia (even though her labs were WNL). She was intubated. Her mouth was swabbed with .12% chlorhexidine to reduce bacterial load, and the teeth were EXT’d. Recovery was uneventful. The child failed to attend any recall appointments, and did not return for three years.

3 years later, at age 7, the child returned to the dental clinic for an emergency appointment. She was now getting transfusions every 3 weeks, and was taking deferoxamine and folic acid. IOE showed mixed dentition, class I occlusion, extremely poor OH, and multiple decayed teeth. Again, the hematologist was consulted, and GA was determined to be the best tx for her. This time, her lab values were not normal, so the hem. Recommended that she receive a transfusion no more than one week before the surgery, which she did. During the surgery she was
1. nasally intubated, given 1g ampicillin, and the mouth was swabbed with .12% chlorhexidine.
2. FMX was taken and read
3. scaling, prophy,
4. All primary teeth either got GI or ssc’s, 6 ym were sealed
5. Pt. Was discharged that day with no complications

Once again, the family failed to return for any follow up appointments.
Key points/Summary:
1. Management of a child with a blood dyscrasias presents a unique challenge
2. Multidisciplinary care is essential
3. In this case report, due to the child’s neutropenia, the use of rinses and antibiotics before surgery was deemed necessary.

Assessment of Article:
Excellent case report highlighting the possible steps and complications when dealing with a child with a blood dyscrasia.