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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 describe an 8-year girl with atlanto-axial rotatory fixation (AARF) caused accidentally after pharyngeal flap plasty under general anesthesia. On the third postoperative day, she had torticollis without neurologic damage and complained of neck pain when she rotated her head. AARF was diagnosed on the basis of the computed tomographic findings. Glisson traction with 1.5 kilograms of weight for 2 weeks reduced the dislocation, but further treatment was reguired. The deformity was corrected by manipulation under general anesthesia and the patient's head and neck were immoblilized in a Minerva jacket for 6 weeks. A cervical collar was used subsequently in an attempt to maintain normal spinal alignment, and the reduction remained.AARF is a rare disorder caused by a variety of conditions. Diagnosis is often difficult and delayed. During operation, hyperextension of the neck should be avoided. It is important to be aware of AARF and to begin treatment as soon as possible in patients with postoperative manifestations such as head tilt, neck pain, and inability to rotate the head.