Resident: Swan
Article Title: Oral and Dental Findings in Children with Fanconi Anemia
Authors: Tekcicek et al.
Journal: Pediatric Dentistry, V29 No3 June 07
Type of Article: Case Series
Main Purpose: Discuss the oral and dental findings in children with Fanconi anemia

Review of Fanconi Anemia: FA is an autosomal and X chromosomal recessive DNA instability syndrome characterized by 1. progressive bone marrow failure, 2. multiple congenital abnormalities, 3. a predisposition to cancer. Most FA patients have many physical abnormalities, including short stature, abnormal thumbs, microcephaly, and hypo- and hyper-pigmented spots. While reports of dental findings in these patients are rare, the following have been reported: 1. generalized microdontia, 2. supernumerary teeth, 3. congenitally missing teeth, 4. periodontitis and gingivitis. FA patients lack NK cells and are thus more susceptible to infection.
Also, for these patients, the risk of squamos cell carcinoma is increased in the mucosal linings from the mouth to the anus, especially after the second decade. Stem cell transplantation, the only curative treatment for FA, is thought to increase the risk of developing solid tumors in the oral cavity. It results in increased survival, resulting in longer treatment regimens, including radiotherapy. Another study found that functioning FA genes help protect against oral carcinogenesis--in FA patients malfunctioning FA genes do not provide the same support, so these patients are more susceptible to oral SCC.

Methods: 26 FA children were diagnosed and followed between 2004-2006. They were given physical and hematological evaluations. Oral and radiological exams and salivary collection followed. The children’s teeth were assessed by a pediatric dentist using DMFT/DMFS indices. Oral hygiene was assessed using the GI and PI. Saliva was evaluated regarding flow rate, pH, buffering capacity, and S. mutans levels.

Results: Among the 26 children, 16 had never visited a dentist, 6 had been once, and 4 visited regularly. Only 5 children brushed their teeth regularly, 7 had never brushed their teeth previously, and the other 14 brushed rarely. Prevalence of dental caries in this group was 9/26. 9 children had gingivitis, 3 had coated tongue, 1 had papillary atrophy. Salivary flow rate was less than .7 ml/min. in 56% of patients. Salivary buffering capacity of pH less than 5 was detected in 33% of patients. A correlation was found between salivary buffering capacity and DMFT/S in this study. The most common radiographic findings were generalized microdontia (10/23), congenitally missing teeth (6/23) and transposition (2/23).

Discussion: Microdontia was the most common dental finding in this group. This may be related to the common presentation of microcephaly. However, another study of 15 children found none had microdontia. Transposition has not been previously described in these patients. Salivary flow rate and buffering capacity probably contributed to the chidren’s dental infections, but the major culprit is most likely their poor oral hygiene (81% didn’t brush regularly).

Conclusions: 1. Poor oral hygiene, dental decay, gingivitis, and congenital abnormalities are common oral and dental findings in this group of Turkish children with FA
2. Dentists should be aware of these common findings and of their increased risk for squamous cell carcinoma

Assessment: Article was well done. Hard to apply the findings broadly with their small sample size, especially when other small studies contradict their findings, but it provides a good overview of some general dental findings in this population of kids.