Birth defect are observed in 3-5% of newborn baby and it is important factor even in the field of dental or oral surgery department. I describe about the pathology of the birth defect and the deep knowledge for genetic factor for them and also mention about the importance of introduction of genetic counseling for the patients.

　Purpose: The nose of patients with cleft lip is deformed, although the extent of the deformity varies with the degree of lip abnormality. This causes esthetic and functional problems, and does not improve if left untreated.

However, treatment strategies, including how and when to correct the deformity, vary between surgeons/orthodontists. Here, a review of the relevant literature was performed to consider the optimal strategy, current situation, and issues remaining in the treatment of cleft lip nasal deformity.

　Issues: The anatomical characteristics of cleft lip nasal deformity are asymmetry in unilateral cases, a short columella and hooding of the alar rims in bilateral cases, and deficiency of maxilla development, a flared piriform aperture, a wide nose, and a broad tip in all types of cases. These characteristics result from the bony deformity, cartilaginous deformities composed of the nasal septum and the upper and lower lateral cartilages, deficit of the nasal lining, and abnormal muscles around the external nose.

　Treatments: Treatment involves both nonsurgical procedures, i.e., maxillary orthopedics using various types of orthodontic device, and surgical procedures. Operative treatments may be performed in infanthood at the time of primary lip repair, at preschool age, and in adolescence at the end of growth.

　Conclusion: The patient can have a nose with a natural shape and patent nasal airway without any growth disturbance if the ideal therapeutic intervention is conducted with appropriate procedures at each time point.

We report two cases of peripheral odontogenic fibroma of the maxillary anterior gingiva. The first patient was a 46-year-old woman with a three-year history of a painless mass near the left maxillary lateral incisor tooth. Intraoral examination revealed a firm nodule with a smooth surface. CT images showed a radiolucent lesion consistent with the mass. The second patient was a 41-year-old woman who had been aware of an asymptomatic hemorrhagic mass situated in the maxillary midline gingiva. The clinical examination revealed a coral pinkish nodule attached to the gingiva. CT examination did not show any abnormal findings. Both lesions were surgically resected with a 2mm safety margin under local anesthesia. The histological characteristics of both lesions included proliferation of fibroblasts and the presence of collagenous stromacontaining inactive odontogenic epithelium. Immunohistochemically, both cases were negative for CD34 and positive for Bcl-2. Both patients showed no evidence of recurrence associated with peripheral odontogenic tumors.

Congenital factor Ⅺ deficiency is a very rare bleeding disorder, also called hemophilia C. It is autosomal recessive and known to occur in both men and women. It is said that bleeding tendency appears strongly during surgery or trauma in areas with high fibrinolytic activity such as the oral mucosa. The patient was a 44-year-old female who was referred to our department for extraction of her left maxillary second molar. We learned that she had a factor XI abnormality, however the details were unknown so we referred her to the Department of Hematology at our hospital, where she was diagnosed with congenital factor XI deficiency. Under hospitalization, six units of fresh frozen plasma were transfused and the left maxillary second molar was removed. There was no abnormal bleeding during and after surgery, and the patient was discharged the following day. Two years later, she revisited our department requesting extraction of her right maxillary third molar. She underwent the same procedure as before, and there was no abnormal bleeding.