Oral aspects and management of severe graft-vs-host disease in a young patient with Beta-thalassemia: case report

Dan Boboia 1/29/10 Lit. Review

Title: Oral aspects and management of severe graft-vs-host disease in a young patient with Beta-thalassemia: case report
Author: Fonseca et al
Type of Article: Case Report
Past Med Hx.
A 6 y/o Saudi Arabian male was referred to Fred Hutchinson Cancer research Center (FHCRC) in Seattle on 9/94 for evaluation and treatment of Graft-vs-host disease (GVHD). The patient was diagnosed with beta-thalassemia major (bm) at 6 weeks of age and had blood transfusions until he was 12 months of age. He had a transplant in the US at 12 months of age. His mother was the matched donor. Despite having adequate engraftment he presented with several post-op complications including CMV infection, hypertension, and chronic GVHD 82 days s/p transplant. Once he arrived back to Saudi Arabia he received cyclosporine and prednisone for 5 yrs on an irregular schedule due to poor compliance. All primary teeth were removed a few years after his transplant due to gross caries. He had never used fluoride or antibacterial rinses.

Present illness and PE:
At the time of his admission the GVHD involved the skin, eyes, GI tract, and oral cavity. A skin biopsy was active for GVHD. Oral examination revealed compromised mandibular motion. Additionally microstomia as well as perioral scaring and scleroderma made oral hygiene and eating very difficult. Further exam revealed carious lesions on all 4 permanent first molars, as well as crowding, malocclusion, gingivitis, and purulent exudate was observed.
Treatment:
· Intensive OH regimen including rinses of 0.12% chlorhexidine combined with saline, one tuft tooth brush to access posterior area of the mouth
· Daily dexamethasone for 4 weeks
· Intensive physical therapy and oral stimulation exercises
· Ext of all 6 year molars 3 months after admissions
· Pt was d/c back to his country and has a yearly recall at the FHCRC
Discussion:
· Viral, fungal, bacterial infections are the most significant life-threatening head and neck complications in pretransplant patients
· Oral pain and increasing dryness more then 100 days post-transplant are suggestive of the chronic form of GVHD
· This patient had decreased mouth opening causing poor nutrition; his high carb liquid feedings, xerostomia, trismus, and microstomia all contributed to his high caries rate
· Common sequelae of chemoradiation are age dependent; they are more significant in children under 5 years of age and are: tooth agenisis, microdontia, atypical root and crown morphology, hypocalcification, and early apical closure.