Akihiko Iida

Blepharo-Cheilo-Dontic (BCD) Syndrome: Case Report

Objective To report a case of blepharo-cheilo-dontic (BCD) syndrome, to compare with the previous cases, and to discuss the possible treatment. Patient The patient was a 14-day-old boy born uneventfully on August 7, 2002. His scalp hair was sparse and curly. A tumor on the top of his head, which was thought to be a dermoid cyst, and a nevus on the back of his left hand were observed. All typical symptoms of blepharo-cheilo-dontic syndrome, such as euryblepharon with ectropion of the lower eyelids, distichiasis, bilateral cleft lip and palate, severe oligodontia, and conical teeth, were observed. Because there was no fatal complication, and growth and development were about normal, it was recommended that a long and active treatment plan be considered for this patient.

Complication of adenoid cystic carcinoma and sialolithiasis in the submandibular gland: report of a case and its etiological background

The authors report a case of adenoid cystic carcinoma (ACC) complicated with sialolithiasis of the submandibular gland. The patient was a 43-year-old female with a history of papillotubular carcinoma of the breast almost at the same time. She had noticed a swelling in her sublingual area for 10 years, which was later diagnosed by her dentist to be due to a sialolith in the left submandibular gland. After several years of observation, the patient was referred to have her left submandibular gland, containing the stone, surgically removed with a diagnosis of atrophic sialadenitis. Histopathologically, the submandibular gland was extensively replaced with fibrous granulation tissue, in which there were small but invasive foci of ACC. The present case indicates that ACC could arise in the background of chronic sialadenitis. It is suggested that long-standing sialadenitis cases should be carefully examined to exclude suspicion of malignancy before surgery.

Craniosynostosis with joint contractures, ear deformity, cleft palate, scoliosis, and other features.

A case of craniosynostosis with joint contractures, ear deformity, cleft palate, scoliosis, and many other features is presented. The patients skull was round with craniosynostosis of the coronal suture. The fingers were slender and long; the finger joints were contracted. Trismus was also present. Contracture of the temporomandibular joint was suspected. The helices were flat, antihelices were minimal, and the ears protruded. Furthermore, the facial and cranial features of this patient included mild hypertelorism, ocular proptosis, short pons nasi, flat radix nasi, mild retrognathia, and small oral fissure. In the oral cavity, a relatively wide cleft extended from the soft palate to the uvula. A frontal chest radiograph revealed a mild scoliosis. Differential diagnoses are discussed in reported syndromes with craniosynostosis.

Progressive Hemifacial Atrophy Treated by Orthodontic Surgery

Abstract Progressive hemifacial atrophy (PHA) is a self-limited atrophy of subcutaneous tissues (and less frequently of hard tissues) on one side of the face. It is a sporadic, slowly progressing disease whose pathogenesis is still unknown. As a rule the asymmetry caused by PHA (usually of soft tissue) is treated by volume augmentation that involves free tissue grafting or a pedicled flap transfer. We describe a rare case of hard tissue PHA. The atrophic changes occurred in the left molar tooth, maxilla and mandible and were associated with moderate soft tissue atrophy. The left side of the patients mouth was higher on the right side, and the occlusal plane was severely inclined in spite of normal occlusion. After no alteration and no progression of the atrophy were established, the patient was treated with orthodontic surgery. To correct the inclined occlusal plane and asymmetry profile, we performed a Le Fort I and intraoral vertical ramus osteotomy. In the 3-year follow-up, there were good occlusal balance and improved symmetrical profile without relapse or recurrence of the atrophy. Thus, orthodontic surgery was effective as a first procedure to treat hard tissue atrophy that appeared with moderate soft tissue atrophy.