We report a case of holoprosencephaly with cleft lip and palate. The patient was a 26-year-old woman who presented with malocclusion and masticatory dysfunction. She had undergone cleft surgery, including lip and palatoplasty, and alveolar bone grafting until 18 years of age, but her occlusion was not effectively managed. Facial anomalies included slight orbital hypotelorism and trigonocephaly, severe mid-facial delayedgrowth with a flat nose and a scar from bilateral chiloplasty. Intraoral examination showed malocclusion, cross bite, a severely contracted dental arch, and multiple tooth defects of the maxilla. We successfully treated her malocclusion by the following procedures:(1) alveolar bone graft for implants, (2) orthodontic treatment of the mandible and dental treatment of the upper and lower teeth, (3) sagital splitting ramus osteotomy (SSRO), (4) application of dental implants and vestibular extension with a skin graft, and prosthetic treatment of the maxilla.
Oto-palato-digital syndrome (OPD syndrome) is a rare but well-defined disorder first described by Taybi in 1962. It is characterized by skeletal dysplasia of the hands and feet, hearing loss, and cleft palate. We report two cases of OPD syndrome type 1 with Pierre Robin sequence. The patients were brothers, and their signs and symptoms were similar. They had U-shaped cleft palate, micrognathia, anomalies of the hands and feet, and a characteristic facial appearance. Their mother and their mother's grandmother had similar facial characteristics, and their mother's brother had cleft palate and deformities of the hands and feet. The patients had airway problems in the neonatal period, but airway distress improved with growth. At the age of 1 year, we performed palatoplasty. Neither patient has deafness or mental retardation. Their speech is normal, with good velar mobility. The elder brother had bronchostenosis caused by a vascular ring. The right aortic arch was therefore divided at the age of 3 years.
We report a case of congenital ranula with frequent recurrences. The patient was a 13-day-old boy who was referred to our clinic because of swelling of the left side of the oral floor. His parents had noticed the swelling, associated with repeated disappearance and recurrence, from birth. The patient was given a diagnosis of mucocele of the oral floor and observed. A stalk-like swelling developed at 4 months of age. Because we suspected a tumor of the oral floor, excisional biopsy was carried out. The pathological diagnosis was granulation tissue. One month after the biopsy, a transparent undulating swelling developed in the left side of the oral floor. At first, the swelling spontaneously disappeared and recurred, but then persisted. We diagnosed a ranula and incised the swelling. Abundant saliva flowed out. Five months after the incision, swelling recurred. Although repeated incisions were made, swelling recurred and resulted in difficulty in eating. Extirpation of the ranula and sublingual glands was performed under general anesthesia. There has been no recurrence of the cyst or dysfunction. We should consider extirpation of the cyst and sublingual glands in infants in whom a ranula is associated with frequent recurrence and dysfunction.
Myofibroma is a solitary myofibroblastic proliferative neoplasm occurring predominantly in infants and children. Myofibroma rarely arises in the oral and maxillofacial region. We report a case of myofibroma of the mandibular gingiva in a 15-year-old girl. She noticed a gradual swelling in the right mandibular molar region 1 month previously and was referred to our hospital. A soft elastic mass arising in the right molar gingiva, measuring 1.2×1.0-cm, was recognized. An incisional biopsy of the mass yielded a diagnosis of myofibroma. Because the tumor grew rapidly after the biopsy, excision of the tumor with alveolectomy of the mandibular molar region was performed. She has no evidence of recurrence 3 years 9 months after surgery.
A 46-year-old homosexual man was referred to our department on a certain day in February, 2005, by his home dentist because of a mass on the left side of the palate. He had been aware of this mass since the end of December 2004. This mass was dark purple, 10×18mm in size, elastic soft, and irregular in shape with clear margins. At presentation, a biopsy was performed, and the mass was diagnosed as Kaposi's sarcoma. On 8th day, laboratory tests revealed that he was HIV-1 positive. On 18th day, the patient was referred to the department of internal medicine. Highly active antiretroviral therapy (HAART) was initiated on 32th day. A complete remission of oral Kaposi's sarcoma was achieved on 130th day.