kondo suguru

We studied the effect and validity of medical intervention for submucous cleft palate (SMCP) in our center retrospectively.<br>Object: Fifty SMCP cases were referred to our center between April 1992 and December 2008. Of these SMCP cases, 36 were examined, and 14 with complications which affected operation and speech therapy were excluded.<br>Method: SMCP is defined as a congenital deformity in which there is imperfect muscle union across the velum, if they do not satisfy the Calnan's triad. Their gender, age at first visit, chief complaint, complications, Calnan's triad, length and mobility of soft palate, and medical interventions (operation and speech therapy) were investigated.<br>Results: There were 17 males and 19 females, and the age at first visit varied from 8 days to 6 years and 3 months. Chief complaints were morphologic defects such as cleft in 21 cases and functional disability such as speech disturbance in 15. Complications were found in 22 cases; chief complications were as below: mental retardation in 11 cases, 22 q 11.2 deletion syndrome in 4, first and second branchial arch syndrome in 4 (2 cases with auditory imperfections), and Robin's sequence in 3 (combined OSAS in one). Clinical symptoms were: imperfect muscle union across the velum in 36 cases, uvula bifida in 28, deficiency in the bone of the posterior edge of the hard palate in 22, and all of the Calnan's triad in 20. Eighteen cases showed short palate, and palatal lift was poor in 14 cases. Operation was necessary in 19 cases. In 17 cases without surgery, 12 required speech therapy. The effects of speech therapy were: improvement in 10 cases and slight improvement in 2. Satisfactory speech was acquired before starting school, excluding a case speech therapy was started after 5 years. In the operated cases, palatoplasty was performed. In one case, a pharyngeal flap was combined. In 15 cases speech therapy was necessary. The effect of medical intervention was: improvement in 12 cases, slight improvement in one, and no change in 2. For the 2 no-change cases, a PLP was applied in one, and a pharyngeal flap was applied additionally in the other. In 15 cases with surgery, excluding the 2 no-change cases and 2 cases operated after 5 years, satisfactory speech was acquired before starting school.<br>Conclusion: Excluding the 2 cases in which the speech evaluation after primary operation showed no change, the other cases could acquire satisfactory speech before starting school, provided medical intervention was started within 5 years. We conclude that the medical intervention in our center is appropriate.

Russell- Silver syndrome (RSS) is a syndrome characterized by intrauterine growth retardation, short stature without postnatal catch up growth, inverted triangular face with relative large head. Though orthodontic treatment may be performed for the malocclusion caused by growth disturbance and right - left asymmetry of viscerocranium, cleft palate is rarely complicated.<br>We report a case of RSS with cleft palate. The patient was 6-months girl at initial visit to our center, she was born at 39 weeks gestation in August 2007. At birth, her weight was 1,334 g, her height was 39 cm, Apgar score were 4/8. Because of extremely low birth weight, she was transported to the nearest municipal hospital in the following day. She had no problem in breathing and no abnormal findings were detected in the echocardiography. Though cleft palate was confirmed, suckling was possible. No abnormal findings were detected in head CT and MR imaging. She discharged in November, 2,315 g weighted. Though tube feeding was applied, weight gain was poor. She was referred to our center in February 2008, as it was thought that poor suckling was due to cleft palate. We made palatal plate and took a wait-and-see approach with pediatricians. From the appearance of growth delay, inverted triangular face with normal head circumference, asymmetry of the length of lower limb, RSS was suspected. We had cytogenetic analyses, epigenetic mutation of the H19-DMR on chromosome 11p was identified, diagnosis of RSS were confirmed with characteristic clinical features. Development delay was not obvious including speech, so we performed palatoplasty in September 2010, when she was 3 years and 1 month old, 6,365 g in weight. Mouth breadth was narrow, opening was limited, intubation was possible, but Dingman mouth gag was inapplicable. 20mm mouth opening was obtained by forced opening with utility mouth gag, she was operated while pulling and displacing her tongue. Postoperative complications were absent, speech therapy had started. The evolving of epigenetics elucidated the pathogenesis and the way for early diagnosis for RSS. In the treatment of RSS infants with cleft palate, it was thought to be important to respond in well considering for the pathogenesis.

A clinico-statistical investigation was conducted with 1112 cleft lip and/or palate patients (excluded 18 cases in which data were inadequate) in the Cleft Lip and Palate Center, Fujita Health University Hospital, since its foundation in April 1992.<br>The results were as follows:<br>1) Primary cases were 1073, secondary cases were 39, and average number of patients registered per year was 75.5 from 1993 to 2006.<br>2) The distribution by cleft type was: 368 cases (33.1%) with unilateral CL (A) P, 279 cases (25.1%) with unilateral CL (A), 275 cases (24.7%) with CP, 157 cases (14.1%) with bilateral CL (A) P, 26 cases (2.3%) with bilateral CL (A), and 7 cases (0.6%) with others.<br>3) The average number of operations per year from 1993 to 2006 was as follows: chelioplasty (primary lip operation) 61.7, palatoplasty (one stage operation for CP) 13.1, palatoplasty (soft palate in two stage operation) 26.5, palatoplasty (hard palate in two stage operation) 23.4, secondary alveolar bone graft 17.8, and secondary operation for velopharyngeal incompetence 3.<br>4) The number of patients registered for speech evaluation and training was 741 until June 2007.

The purpose of this study was to determine whether the development of masseter muscle activity at mandibular rest position is characteristic for different angle classifications. The sample consisted of 64 boys and 108 girls, aged ten to 18. Electromyographic (EMG) activity from the right and left masseter muscles was recorded using an EMG scanner. First or second order polynomials were used to separately model the age-related changes in resting muscle activity of each gender and Angle&apos;s classification. The EMG activity of Class I boys and girls were expressed in positive second order polynomials. The EMG activities of Class II and Class III subjects were expressed in first order polynomials, and the EMG levels in Class III subjects were lower than in Class II subjects. These results indicated that development of resting activity levels might distinguish and characterize the anteroposterior mandibular position relative to craniofacial region.