IADR Abstract Archives
Dental management and treatment of sanFilippo syndrome.
Objectives: SanFilippo syndrome (Mucopolysaccharidosis type III C ) is a rare and catastrophic genetic disorder named for the person who first discovered it in 1963. It is an autosomal recessive disorder in the gene of the lysosomal enzyme acetyl-CoA:alpha-glucosaminide acetyltransferase, ending with deficiency in the brain and the spinal cord (central nervous system). Patients carrying this genetic disorder display developmental delay, attention deficiency, uncontrollable hyperactivity, and aggressive behavior. At a later stage, the patients develop progressive dementia and die in late adolescence. The aim of the present report is to introduce a case of patient with SanFilippo syndrome undergoing preoperative and dental care management.
Methods: A 25-year-old female with SanFilippo syndrome was referred to our department for dental treatment. Since she was uncooperative for standard dental procedures, we decided to treat her under GA. Thus, she has undergone medical evaluations including complete blood tests. Based on hematologist advice and guidance, her preoperative platelet count (31000 cell/µl) was not sufficient to allow surgical procedures, and therefore the she was given platelet transfusion before start dental treatment. General anesthesia was also performed with oral intubation, in addition to lidocaine local anesthesia. Radiographic x-ray examinations were also performed. Given this, we have completed her treatment plan, namely extraction of massively destroyed teeth, complicated composite restorations, and root scaling
Results: Despite the complexity of the patient's disease, we were able to perform dental procedures including extractions and dental composite restorations, under a standard anesthesia. No abnormality in the structure of the teeth was noticed except germination in tooth 47.
Conclusions: Despite the risks incurred from San Filippo clinical syndrome as well as the risk of the heavy bleeding during intubation and tooth extractions, we have managed to treat the patient. Consultation and supervision of anesthesiologists and hematologists were greatly helpful.
Methods: A 25-year-old female with SanFilippo syndrome was referred to our department for dental treatment. Since she was uncooperative for standard dental procedures, we decided to treat her under GA. Thus, she has undergone medical evaluations including complete blood tests. Based on hematologist advice and guidance, her preoperative platelet count (31000 cell/µl) was not sufficient to allow surgical procedures, and therefore the she was given platelet transfusion before start dental treatment. General anesthesia was also performed with oral intubation, in addition to lidocaine local anesthesia. Radiographic x-ray examinations were also performed. Given this, we have completed her treatment plan, namely extraction of massively destroyed teeth, complicated composite restorations, and root scaling
Results: Despite the complexity of the patient's disease, we were able to perform dental procedures including extractions and dental composite restorations, under a standard anesthesia. No abnormality in the structure of the teeth was noticed except germination in tooth 47.
Conclusions: Despite the risks incurred from San Filippo clinical syndrome as well as the risk of the heavy bleeding during intubation and tooth extractions, we have managed to treat the patient. Consultation and supervision of anesthesiologists and hematologists were greatly helpful.