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This paper presents a surgically treated case of Pierre Robin syndrome with seven dyspnea and a study on maxillofacial growth of the syndrome.<BR>Case report: The patient was a 19 day old female infant who often choked during feeding, with mild stridor and fever. On the 7th day of life, respiratory distress had markedly appeared. Because her general condition had not improved at the obstetric hospital, she was referred to our department. Examination revealed a marked degree of micrognathia and a partial cleft palate. Efforts were made for respiration and a systemic improvement. However, one week after admission to our hospital, acute respiratory distress appeared again. Endotracheal intubation was the only way to relieve cyanosis and to keep her airway open. Because long term intubation might develop other serious problems, the surgical operation was performed by modified Randall's method under G. O. F. anesthesia on the 9 th day of hospitalization. This resulted in complete relief of symptoms. She was discharged on the 58 th postoperative day. At the age of 10 months, the tongue was detached from the lip by 2 steps in a short interval. After this, dyspnea had not appeared any more. At the age of 28 months, uranoplasty was performed. Now the patient is well grown, and her mandibular growth and phonetic problems are under observation.<BR>Study on maxillofacial growth of Pierre Robin syndrome: Besides the above case, we experienced six cases of Pierre Robin syndrome in our department during the past 15 years. From these we selected 3 cases having similar clinical and therapeutic conditions as reported here. They were all male and averaged 12.1 years old (11.6, 11.8, and 12.8, respectively). In infancy, these 3 presented mild dyspnea and were treated only conservatively. Uranoplasty was received at the age of about 18 months. Roentgen cephalometrics were taken for this study. Measurements were carried out by Downs, Northwestern, and Sakamoto methods. In addition, Sekiguchi's method was used in order to study the influences of the uranoplasty. From the results it was suggested that the underdevelopment of the mandible continues to exist at least before adolescent growth spurt and is due not only to secondary influences of the operation such as uranoplasty but also to the nature of the syndrome itself.
社団法人日本口腔外科学会の論文

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