The authors studied 73 patients diagnosed as having deletion at chromosome 22q11.2on the basis of genetic analysis. The results were as fo llows:
1. Specific facial appearance was observed in 99% of patients. Small stature and asymmetric crying face were also found in most patients.
2. Cardiac anomalies were found in 53 patients (73%), and TOF was dominant. Other findings were as follows: hypocalcemia, hypoparathyroidism, finger anomalies, ear anomalies, cervical and spinal abnormalities, and so on.
3. Mental Or developmental retardation (IQ or DQ<60) was found in 65% of patients.
4. In speech findings, there were eleven patients who had articulation disorders including glottal stop without velo-pharyngeal closure insufficiency (VPI). Twenty-nine patients with VPI have undergone surgical operations, and pharyngeal flap operation seemed to be effective. These patients tend to need a long time for restoration of normal speech in conversation even if their VPI improved.

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Three-dimensional growth changes of maxillary alveolar arch in complete unilateral cleft lip and palate (UCLP) children were analyzed. The subjects consisted of 31 UCLP children whose surgical operations were carried out in Kyushu University Dental Hospital. The materials were maxillary plaster models which were taken at both their cheiloplasty and palatoplasty. Serial dental casts were read by a 3D-laser scanner (Vivid-700)and were superimposed on their palatal rugae longitudinally. Three-dimensional changes of the maxillary arch were measured. The results were as follows: 1. The maxillary tuberosity of the minor segment moved to the buccal side and downwards, and that of the major segment moved backwards and downwards.2. The anterior alveolar ridge moved to the palatal side and downwards.3. The primary canine parts moved labially and downwards, but that of the minor segment moved palatally. The space growth was the largest in the maxillary tuberosities, followed by the anterior alveolar ridge and the space growth of the canine regions was the smallest.
Details of the three-d imensional change of the maxillary arch became obvious by superimposing on the palatal rugae of the serial dental casts.

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This study was undertaken to examine the effects of secondary bone grafting (SBG)on the stability of the maxillary dental arch form after maxillary expansion in orthodontic patients with repaired complete unilateral cleft lip and palate (UCLP). The subjects examined in this study consisted of 8 patients who underwent secondary bone grafting after maxillary expansion (SBG group: mean age of 11 years 3 months), and 7 patients who did not undergo secondary bone grafting after maxillary expansion (control group: mean age of 11 years 0 month). Oral plaster models at the initial stage, the post treatment stage and the retention stage were analyzed. The changes and change rates of overj et, overbite, dental arch width, and dental arch length were evaluated at cleft and noncleft sides. The results were as follows:
1. The changes of the overjet and overbite during the retention stage in the SBG group were smaller than those in the control group.
2. The changes of the dental arc h width during the retention stage in the SBG group were smaller than those in the control group at both sides.
3. The change rates of the dental arch length at the cleft side during the retention stage in the SBG group were higher than those in the control group.
These results suggested that secondary bone grafting after maxillary expansion affected the stability of the maxillary dental arch form.

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Velopharyngeal competency and articulation in 37 patients at 4 years of age (group F4) and 33 patients at 5 years of age (group F5) with cleft lip and palate were examined. These patients had an operation on their soft palate closure using Furlow's double-opposing Z-plasty at one and a half years of age under the management of two-stage palatoplasty combined with Hotz plate in our clinic.
We also investigated their speech characteristics after Widmaier's method for soft palate closure as control groups at each age (group W4 and W5) and compared them with those of Furlow's method.
The resul ts were as follows:
1. Velopharyngeal competen cies in the Furlow groups improved more and earlier than those in the Widmaier groups.
2. The development process of normal articulation acquisition of the Furlow group was more normal than that of the Widmaier group, and competency articulation was found to disappear gradually without any speech therapy.
3. The result of the comparison average of the substituted phoneme of speech intelligibility was better than that of the Widmaier group at the same age. In conclusion, it is suggested that patients in the Furlow group were superior in Velopharyngeal competency and articulation at 4 and 5 years of age.

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Objective: In recent years, dental implant therapy for alveolar cleft has been reported in several papers. However, no study has documented a sufficient number of cases with a long follow-up period. This study assessed the modality through a multicenter study with a questionnaire.
Methods: The questionnaire concerned the general information of the patients, bone grafts, implant surgery, prosthodontic treatment, soft tissue management, survival rates of the implants, and psychological effects.
Results: Eleven institutions participated in this study. One hundred and three patients,47males and 56 females,78 unilateral and 25 bilateral clefts, were registered. All of them had undergone autogenous bone grafts for repairing alveolar clefts 5-121 months (average 32months) before the implant placement. At the implant placement, the patients were 9 years 8 months-61 years old (average 22 years 7 months old). The implants installed were mainly 13-15 mm in length and 3.25-4.0 mm in diameter. Twenty-four patients had undergone bone grafts simultaneously. Soft tissue managements around the implants were performed in 19patients. Fixed-type superstructures were settled in all cases. The overall survival rates were 98.6% in five years and 95.4% in 10 years. Almost all patients were satisfied with the treatment results.
Conclusions: These results revealed that the modality is a very useful option for treating patients with alveolar cleft.

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Cleft lip and/or palate or isolated cleft palate patients treated in our department during a twelve-year period, April 1990-March 2002, were analyzed statistically.
1) We studied 393 primary operative cases which included 308 patien ts with cleft lip and/or palate (unilateral 246, bilateral 62) and 85 patients with isolated cleft palate.
2) Since 1996, the annual numbers of surgical operations and primary operations performed on cleft lip or cleft palate in our clinic have increased.
3) One hundred and eighty-eight patients (47.8% ) were from Okayama Prefecture,102 (26% )from Hiroshima Prefecture and 38 (9.7%) from Ehime prefecture. The remaining patients came from other prefectures, but most of them were residents of the Chugoku and Shikoku districts.
4) Complete forms (186 patients) were more common than incomplete forms (122 patients). Cleft lip was more frequent in incomplete forms, cleft of the lip and palate equally frequent in incomplete and complete forms, and cleft of the lip, alveolus and palate, more frequent in complete forms.
5) The l eft-right ratio of the cleft side in unilateral cleft cases was 1.9: 1 with left side predominance in every cleft type.
6) Cleft lip and cleft palate were more frequent in males while isolated cleft palate patients were mostly females.
7) Consanguin eous marriage was found in 4.8% of patients. The incidence of isolated cleft palate was twice as much as in the cleft lip and/or palate group.
8) The frequency of familial expression was 9.9%.9) Concomitant malformation was found in 1 9.6% of all patients. Patients with isolated cleft palate were associated with a variety of anomalies with highest incidence and regarded to be at high risk.

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In patients with cleft lip and palate, severe malocclusion occasionally occurs due to hypoplastic maxilla. In such cases, the advancement of maxilla is limited by scar tissue formed in the palate region; therefore, distraction osteogenesis of the maxilla has been applied as an effective treatment instead of conventional osteotomy. In this report, distraction osteogenesis of the maxilla using subcutaneous devices and sagittal split ramus osteotomy were performed in 3 patients with cleft lip and palate, in whom malocclusion was caused by hypoplastic maxilla.
High Le Fort I osteotomy in the maxillary bone was performed, and skeletal distraction was initiated 7 days after surgery. The lengthening device was activated 1.0 mm/day, and we completed the lengthening after advancing the maxillary bone by 10 mm. Approximately 6months after distraction osteogenesis of the maxilla, lengthening devices were removed, and the bone lengthening area was fixed using mini -plates. Simultaneously, the mandibular bone was posteriorly shifted by sagittal split ramus osteotomy.
As a result, a good facial profile and occlusal conditio n were obtai ned, suggesting that distraction osteogenesis of the maxilla by asing the subcutaneous device was a useful method in patients with cleft lip and palate.

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