In some cases of Hemophilia A patients who are treated with factor VIIII replacement therapy, factor VIIII inhibitor appears, making bleeding control difficult. The authors describe their experience with factor VIII bypassing therapy by using a factor VIII inhibitor to repair a cleft palate.
The case was a 19-months-old old male child with congenital cleft lip, alveolus, and palate. The lip was repaired at the age of four months, and at that time, prolonged bleeding was noticed. Investigations revealed that the patient had Hemophilia A. Thereafter, the patient was treated with factor VIII replacement therapy, but factor VIII inhibitor appeared, and consequently the factor VIII replacement therapy became ineffective.
The patient was given the ideal amount of prdthrombin complex concentrate (PCC), which is the first choice for Hemophilia A with factor VIII inhibitor, and the cleft palate was repaired using the push back method and vomer flap.
But a day after the operation, bleeding could not be controlled in the bone exposed area of the hard palate, and activated prdthrombin complex concentrate (APCC), which is the second choice for Hemophilia A with factor VIII inhibitor, was started, and five days after the operation, the vomer flap was returned.
Bleeding was controlled after the second operation by using a combination of PCC and APCC, and the patient was discharged 37 days after the operation.
Up to now, therapy for Hemophilia A with a factor VIII inhibitor has been described only in some minor cases, like tooth extraction. In the present case, bleeding control was an unknown quantity. In advance, the authors confirmed that APTT reached 86 seconds at six hours after instillation of PCC, but controllable APTT time was level of fifty second.
The authors describe that the instillation quantity of PCC and APCC should be decided on a case-by-case basis because of few relationship between the clinical effect and shortening of PT and APTT.

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[in Japanese]

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The purpose of this study was to establish a method to predict the effects of maxillary protraction in cleft lip and palate patients using linear correlation and stepwise multiple regression analyses based on pretreatment facial morphology. The materials of this study were 29 complete unilateral cleft lip and palate patients (12 males and 17 females), and the mean age at the start of the treatment was 6 years and 8 months. Pretreatment facial morphology and treatment changes in the maxilla and mandible during one year of the protraction were cephalometrically measured.
The results were as follows:
1. The forward displacement of the maxilla was significantly correlated with the curvature of the nasal septum and the posterior upper facial height. This suggested that the effects of maxillary protraction were dependent on the individual growth characteristics associated with surgical repair of the palatal cleft.
2. The relationships of the treatment changes to pretreatment variables were exanined by stepwise multiple regression analysis. Maxillary forward displacement was explained by the following variables: the curvature of the nasal septum, the posterior upper facial height, and the S-N length. The multiple correlation coefficient was more than 0.7, explaining more than 50% of the total variance of the treatment change.
3. For the changes of the mandible and the upper and lower jaw relationship, each multiple correlation coefficient was relatively low, explaining the total variance of the change ranging from 22% to 39%, therefore it was insufficient for prediction.
4. The results suggested that multiple regression equations give more accurate prediction for the treatment effects on maxillary forward displacement by protraction compared with linear correlation based on individual pretreatment variables.

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This study investigated the treatment effects of maxillary protraction in isolated cleft palate patients with the Hellman's dental stage of III A. Thirty-seven children (10 boys and 27 girls) with isolated cleft palate were examined. seventeen children (7 boys and 10 girls) were treated with a maxillary protraction appliance (MPA group), and 20 (3 boys and 17 girls) underwent chin cap therapy (CC group). Lateral cephalograms were used to analyze skeletal changes during 2 years of treatment in both groups. For 7 of the MPA group children (3 boys and 4 girls), cephalograms at a deciduous dentition stage were also used, to compare maxillary growth changes between the deciduous dentition stage and the treatment periods.
The following results were obtained:
1) From comparison of the pretreatment skeletal patterns between both groups, the MPA group showed more severe jaw discrepancy due to more anterior positioning of the mandible than the CC group. However, there were no significant differences in maxillary morphology between the two groups.
2) During the treatment, the MPA group showed greater forward displacement of the maxilla, as well as a more forward growth direction, than the CC group, but there were no significant differences in the jaw relationships and mandibular position.
3) Cases showing stronger forward growth of the maxilla during the deciduous dentition period still have maxillary forward growth potential during maxillary protraction. This suggested that effects of maxillary protraction in isolated cleft palates depend on individual growth characteristics of the maxilla.

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Facial morphology and occlusion at the stage of early mixed dentition in cleft palate patients treated with Furlow palatoplasty were examined and compared with patients treated with the pushback procedure. The subjects selected for this study were 15 patients with a cleft of the soft palate, who were divided into two different groups: The Furlow group consisted of 3 male and 5 female patients, whose cleft palates were repaired with Furlow double-opposing Z-plasty, at an average age of 1 year and 8 months, and the pushback group consisted of 2 male and 5 female patients, whose cleft palates were closed with the pushback procedure, at an average age of 1 year and 7 months.
Cephalometric radiographs (mean age,8 years for the Furlow group, and 7 years and 11 months for the pushback group) and dental study casts (mean age,7 years and 10 months for the Furlow group, and 8 years for the pushback group) were analyzed. Six angles and 4 distances were measured in the cephalograms, and 9 measurements of dental arch width and 2 measurements of dental arch length, were obtained. The results indicated that midfacial growth was significantly better (angle ANB, angle NAPog, distance A' -Ptm' ) and upper dental arches were significantly larger (inter deciduous canine distance, inter first and second deciduous molar distances, dental arch length) in the Furlow group, compared with the pushback group. No subject in the Furlow group had crossbite, while in the pushback group,6 of 7 subjects had anterior and/or posterior crossbite. As Furlow palatoplasty avoids the need for a lengthening procedure that uses mucoperiosteal flaps from the hard palate, it could allow adequate maxillary growth.

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The purpose of this study was to compare the two-dimensional sizes of adenoidal tissue and the upper airway in children with and without unilateral cleft lip and palate (CLP). The sample consisted of 45 boys with unilateral CLP (the CLP group; mean age,9.2 years) and 45 age-matched boys without CLP (the control group; mean age,9.8 years). Parameters related to adenoidal tissue, the upper airway, and craniofacial structures were evaluated using lateral cephalograms. An unpaired t-test was used to compare mean values of each cephalometric measurement between the CLP and control groups. Adenoidal tissue was significantly larger (p< 0.01), while the anteroposterior dimensions of the upper airway were significantly smaller (p< 0.01), in the CLP group compared with the control group, With regard to skeletal characteristics of craniofacial structures, the maxilla was significantly retruded (p< 0.01) in the CLP group compared to with the control group, whereas there were no significant differences in the anteroposterior position of the mandible between the two groups. A significant negative correlation coefficient was determined between the size of adenoidal tissue and upper-airway dimensions. These findings suggest that juvenile patients with unilateral CLP have larger adenoidal tissue and a more restricted upper airway than patients without unilateral CLP.

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It was well known that such anomalies as the number, form, and misdirection of eruption of the teeth adjacent to the alveolar cleft sometimes occur. It became common to adopt secondary alveolar bone grafting as a continuous treatment of cleft lip and palate. It was possible to recover the form and function by teeth movement to the bone grafted site, if such anomalies were little. It was difficult to reconstruct the alveolar cleft, if great anomalies, such as missing teeth, existed. It was considered that almost all severe cases were treated by prosthodontic treatment, previously. Damage to adjacent teeth has been unavoidable, in many cases. On the other hand, autotransplantation and dental implants have been developed in treatment of missing teeth. We have applied autotransplantation (5 cases), autotransplantation of cryopreserved teeth (2 cases), and dental implants (2 cases) to bone-grafted sites in patients with cleft lip and palate.
All cases underwent secondary alveolar bone grafting from the iliac crest. The vertical width of grafted bone ranged from 8 to 16mm. All teeth in transplant or implant sites were upper lateral incisors that were congenitally missing or extracted by dwarf. The duration from bone grafting to plantation ranged from 4 to 50 months. Although some part of root absorption was observed in both cases of autotransplantation of cryopreserved teeth, all cases were successfully treated with final prosthodontic treatment.
It was considered that autotransplantation and dental implants were very useful for the treatment of patients with alveolar cleft, and it was also considered that transplantation of cryopreserved teeth must be developed through clinical andbasic investigations.

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This study investigated factors influencing the results of bone grafting by analyzing changes in the density and contour of grafted bone following secondary bone grafting to the alveolar cleft of patients with unilateral cleft lip and palate.
The weight of grafted iliac bone, and the cubic measurement of the grafting space in the alveolar cleft, were measured during graft surgery on 20 patients with unilateral cleft lip and palate.
The contour and density of the bony bridge were analyzed as well, on dental X-ray films taken 1 week,1 month,3 months, and 6 months following the surgery. Specially-made X-ray film holders combined with an individual bite-splint were prepared for each subject. An aluminum wedge with a thickness of 0 to 16mm was mounted on the film holder, so that the alveolar bone and the aluminum wedge could be visualized on the dental film simultaneously. The bone density on the dental X-ray film was changed to an aluminum-equivalent (Al-Eq) value based on the mm thickness of aluminum.
The levels of the lower and upper margins of the bone bridge were evaluated by a scale graded on the root of an adjacent central incisor, from level 0.00 (the position of the root apex) to level 4.00 (the position of the cervical margin).
All 20 cases were divided into two groups by the level scores for the upper and lower margins, at 6 months postoperatively. When the upper margin was higher than level 1, and the lower margin was lower than level 3, the case was classified into the successful group. When the upper margin was lower than level 1, or the lower margin was higher than level 3, the case was classified into the failure group.
The results were as follows:
1. Eleven of 20 subjects were classified in the successful group, and 9 subjects in the failure group, by the level scores for the upper and lower margins of the grafted bone at 6 months postoperatively.
2. The values of bone density, derived from bone weight (g) and cubic measurement (cm³) at the time of surgery, were significantly higher in the successful group than in the failure group. The discriminant value between the two groups was 2.98g/cm³.
3. In the failure group, the values of Al-Eq of the bone bridge at 6 months postoperatively were significantly lower than those in the first week postoperatively. In the successful group, there were no significant changes observed. A significant difference was observed between the Al-Eq values of the two groups at 6 months postoperatively.

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This study investigated the change in velopharyngeal movement following pharyngeal flap surgery. The subjects were six patients who underwent pharyngeal flap surgery because of velopharyngeal incompetence (the pharyngeal flap group). Seven normal adults (the normal group), and seven surgically treated cleft palate patients with normal speech (the recovered cleft group), were selected as control groups.
EMG of m. levator veli palatini, and the strength of velopharyngeal closure and intraoral air pressure during phonation, blowing, and swallowing, were measured preoperatively, and at 3 months,6 months, and 1 year postoperatively. Nasal air flow rates were analyzed in the pharyngeal flap group as well. Speech intelligibility testing was carried out on the pharyngeal flap group and the recovered cleft palate group.
Results were as follows:
1. prepearatively, EMG activities, strength of velopharyngeal closure and intraoral air pressure during blowing and pressure consonants were significantly lower than in the normal group and the recovered cleft palate group. Following pharyngeal flap surgery, those values increased significantly, becoming close to the level in the recovered cleft palate group.
2. The preoperative value of EMG activities and strength of velopharyngeal closure were the lowest during phonation of nasal consonant sounds. These parameters were as low as those of the two control groups, and no remarkable changes were observed until 1 year postoperatively.
Preoperative values of EMG activity and strength of velopharyngel closure during swallowing were as high as those of the recovered cleft palate group, and there were no remarkable postoperative changes.
3. There was no correlation between EMG activity and intraoral air pressure preoperatively, but these parameters were correlated postoperatively.
4. Speech intelligibility improved remarkably following pharyngeal flap surgery. Still, at one-year postoperatively, it remained lower than that in the recovered cleft palate group.

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Three-Dimensional changes of the maxillary alverolar arch shape were compared between before and after lip repair of unilateral cleft lip and palate patients, on the materials of Thailand (group T) and our university (group J) in last year. In the present study, we analyzed upper dental casts of pre-operated unilateral cleft lip and alveolar patients from each group who needed not any plates, by means of a three-dimensional dental cast analyzing system using laser scanning (UNISN Co. ).
The results were as follows: The alveolar width (W) in group J was significantly smaller than in group T; however, the alveolar length (L) and the ratio (L/W) were not significantly different between the two groups. additionally, the tomographic patterns illustrated by horizontal and frontal views showed a small difference between the two groups.

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The purpose of this study was to learn the condition of telling patients with cleft lip and/or palate the name of disease, and to examine the best way to give such information. Questionnaire consisting 26 items, the Yatabe-Guilford personality test, and the Cornell Medical Index test for patients, and 44 items for parents was performed to 64 patients with CL/CP aged from 13 to 27 years old and 73 parents of the patients from Jan.1997 to Feb.2000.
The results were as follows:
(1)The parents recognized informing the name of the disease positively and there were discrepancies between the patients and their parents as to the recognition of informing the name of the disease.
(2)32.8% of the patients wanted to know the reason for seeing a doctor, and 78.0% of them knew it actually, which meant that they did not always want to know the reason.
(3)When the patients wanted to know the name of the disease, they seemed to accept being told the name of the disease more positively than the patients who did not want to know the name (p<.10).
(4)According to the Yatabe-Guilford personality test, the group of the patients belonged to categories B and E, which represented emotional unstability, more than the control group (p<.01), and they also belonged to category D, which represented positive stability, less than the control group (p<.01).
(5)According to the Cornell Medical Index test (CMI) the group of the patients belonged to areas I and II, which represented a high degree of mental health, significantly higher than the contlol group (p<.01).
(6)According to the Family Adaptability and Cohesion Evaluation Scale (FACES III), the group that had disagreement on being told the name of the disease between the parents and the patients, belonged to the group of 'Extreme' significantly higher than the control group (p<.01).
These findings indicated that patients who want to know the name of their disease should be told it, along with a careful explanation on the treatment planning by parents or medical staff, so that the patients can accept the disease without disturbance.

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This study evaluated bone bridge formation after bone grafting using X-ray computed tomography, in complete unilateral cleft lip and palate patients. Subjects were 14 patients (5 females and 9 males), and all patients received two-stage palatoplasty combined with a Hotz plate, followed by bone grafting using autogenous particulate cancellous bone and marrow harvested from the iliac crest. X-ray computed tomography was performed before and after bone grafting (mean,3.6 months after), and an axial image was used. Then, both the shapes of cleft defects before bone grafting, and bone bridge formation after bone grafting, were classified into three types on basal and alveolar bone side level.
Results were as follows:
1) The cleft defect shape was classified into 5 types.1; on the basal and alveolar bone side, the anterior bone defect was larger than posterior (6 cases).2; on the basal bone side, the anterior was larger than the posterior but on the alveolar side, the anterior and posterior were the same (3 cases).3; on the basal bone side, the anterior and posterior were the same, but on the alveolar side, the anterior was larger than the posterior (2 cases).4; on the basal bone side, the anterior was larger than the posterior, but on alveolar side, the posterior was larger than the anterior (2 cases).5; on the basal and alveolar bone side, the anterior and posterior were the same (1 case).
2) Except for two cases, satisfactory bone bridge formation was found.
3) The mean age of bone grafting was 10 years and 3 months (range,7 to 15 years).
4) Before bone grafting, the cleft side canine had, in 4 cases, already erupted, while in the other 10 cases it had not. Root formation of the cleft side canine ranged between 1/3 to complete.
5) Two cases showed poor bone bridge formation on the basal bone side, and they were characterized by anterior bone defects that were larger than the posterior on the basal bone side, and widths of the basal bone defects that were larger than 20mm.
In conclusion,
1) Satisfactory bone bridge formation was gained at a high rate with two-stage palatoplasty combined with a Hotz plate.
2) The width of a bone defect has strong influence on bone bridge formation.

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A change from the current piecework payment system to the Diagnosis-Related Group/Prospective Payment System (DRG/PPS) has been under discussion for the “ rationalization” of ever-increasing medical expenses. Success of the DRG/PPS partly depends on how appropriate the classification of diagnosis-related groups is going to be. We calculated the hospital charges by method or the progress of medical treatment adopted in cleft lip and palate cases. Also discussed is how to classify diagnosis-related groups, and the feasibility of introducing the DRG/PPS.
The objects were 51 patients that were hospital-treated for cleft lip and palate between April 1998 and December 1999. The breakdown of the 51 is as follows: primary lip plasty,9 cases; primary palatoplasty,14 cases; bone graft to the alveolar cleft,18 cases; and secondary lip and nose repair,10 cases. The hospitalization charge for each case was calculated, based on the table of medical care fee points, from totaling the piecework charges for hospital room and board, surgery and anesthesia, laboratory tests, and imaging. It was thought that the DRG/PPS would be feasible in primary cases of lip plasty and palatoplasy, provided their surgery costs were reexamined. We found that a closer classification would be in order for bone graft and secondary repair cases.
We would also urge a detailed examination of the adequacy of the current medical care fee points, prior to eventual introduction of the DRG/PPS.

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To evaluate the adaptability of children with cleft lip and/or palate to school, their mental images of the school environment were investigated by a semantic differential methed survey using 23 pairs of adjectives. The following eight places inside and outside of school were chosen as environmental factors: classroom, gymnasium, health care room, teachers' office, playground, passageway, road leading to school, and road leading back to home. Fifty children with cleft lip and/or palate (ages 10-11 years) were enrolled in this study. The children with cleft lip and/or palate had a feeling of liberation in the teachers' office and health care room. They did not have an established feeling of relaxation or affinity in the classroom, gymnasium, playground, hallway, road to school, and road leading back to home, which were the main areas of their school activity. This suggested that self-expression and adaptation were difficult for these children.

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We report a patient with Cornelia de Lange syndrome (CDLS) who experienced airway obstruction at palate plasty. The patient was a 21-month-old boy diagnosed as having CDLS with a cleft palate with mental and growth retardation, peculiar facial features, and other congenital malformations. His general condition, low weight, an atrial septal defect (ASD), and recurrent pneumonia delayed palate plasty until he was five years old. At the palate plasty, the narrow hypopharynx and micrognathia were difficult to intubate. Thirty minutes after the extubation, airway obstruction occurred. We inserted a tracheal tube and cured secondary bronchopneumonia using antibiotics. In this case of repeated respiratory disorder and difficult intubation, we concluded the patient should be carefully observed.

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Severe reversed occlusion due to marked maxillary undergrowth is often observed in patients with cleft lip and palate.
In this case, maxillary distraction osteogenesis (DOG) was applied to an adult cleft lip and palate patient with reversed occlusion. Taking into account that his malocclusion was caused by disharmony between the maxilla and the mandible, two-stage orthognathic surgery was performed between-pre and post-orhodontic treatment.
The first surgery, a Le-Fort I osteotomy, was applied 32 months after the presurgical orthodontic treatment, followed by DOG at one week after the first surgery. The distraction pinned in zygomatic bones was activated for 20 days at a rate of 0.5mm/day.
The second surgery, a sagittal splitting ramus osteotomy (SSRQ), was carried out seven months after the first.
The features of the distraction device used in this patient were as follows,
1. The shaft of the device could protrude from the skin, minimizing skin damage.
2. The device can be used as a fixation appliance after the distraction.
The following evaluations were performed before and after distraction, and during retention, based on the results obtained by the treatment.
1. Facial profile and occlusion.
2. Amount and direction of jaw and tooth movement.
3. Occulusal contact area and occlusal force for each area utilizing pressure sensitive occlusal force sheets.
The facial profile was improved by fan after treatment because of the balance between the upper lip and lower lip.
The amounts of maxillary extention and mandibular set-back were 11.4mm and 10.0mm, respectively.
No difference in the occlusal contact area between the pre-distractive stage and the retention stage was observed. On the other hand, the occlusal force for each area at the retention stage increased compared with those at the pre-distractive stage.
These findings suggested that the combined use of maxillary DOG and SSRO was useful in treatment of patients with cleft lip and palate with marked undergrowth of the maxilla and overgrowth of the mandible.

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