We studied retrospectively 43 patients with cleft lip and palate who underwent a series of comprehensive treatments and were followed up until the completion of the final treatment in adulthood at the Maxillofacial Surgery, Tokyo Medical and Dental University Dental Hospital. As for sex, 23 patients were male and 20 female, and as for cleft type, 32 were classified as unilateral (UCLP) and 11 as bilateral (BCLP).
The mean age of cheiloplasty of patients with UCLP was performed at 4.7 months and 4.9 months at the first treatment, and 8.1 months at the second treatment with BCLP. Palatoplasty was done at a mean age of 1.8 years with UCLP and 1.9 years with BCLP. Alveolar bone grafting was carried out on 36 patients. Forty patients had a lip revision or a lip and nose correction. Orthognathic surgery was carried out on 11 patients. Six patients underwent a pharyngeal flap operation.
All patients were referred to orthodontics. Thirty-two patients showed no crossbite at the end of treatment. Fifteen of those patients did not require prosthodontic treatment of the cleft area. One patient underwent a dental implant procedure, 23 patients were treated using a bridge, and 4 patients had placement a denture as a final prosthodontic treatment.
Based on cephalometric roentgenogram analysis, the SNA angle was under −1SD on 24 patients. The SNB angle was between ±1SD on 21 patients. The Ls to E-plane was a mean of −3.7mm with UCLP and a mean of −3.6mm with BCLP. The Li to E-plane was 0.4mm for both UCLP and BCLP.
At the end of the treatment stage, all patients had acquired normal velopharyngeal closure; however, articulation disorders were observed in 5 patients.

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Abnormal articulation of some cleft palate patients do not improve even though their velopharyngeal incompetence and malformation of articulating organs were repaired. As the malfunction of speech discrimination is considered to be one of the reasons for not improving articulation, so that we evaluated the external and internal speech discrimination ability. Then we analyzed the correlation between residual abnormal articulation and speech discrimination ability.
1. Assessment of external speech discrimination ability to normal articulation sounds and of internal speech discrimination ability to abnormal articulation sounds
Eight cases with cleft palate (8 to 60 years of age, mean age of 28.6) were evaluated their external ability to normal articulation sounds and internal ability to their own pronouncing abnormal articulation sounds.
With the 100 percent of correct response, they could discriminate the normal sounds. On the other hand, they could not discriminate their own internal articulation sounds they produced with 0 percent of concordance rate with a speech therapist.
2. Assessment of external speech discrimination ability to abnormal articulation sounds
Eight cases of cleft palate patients (7 to 13 year of age, mean age of 10.1) with abnormal articulation were analyzed their ability to discriminate the misarticulated sound samples and recorded mal-articulated voice of their own. As the control, eight cases of cleft palate with normal articulation and three cases of non palate-cleft subjects took part in this assessment. The patients could not distinguish the abnormal sample sounds, which is either similar or different to their own mal-articulation. There was no difference between the results obtained by these similar and different sounds. In addition, control groups showed no significant difference. However there was some difference of discrimination ability depending on the kinds of abnormal articulation. The "glottal stop" was easy and "lateral articulation" was difficult to distinguish. The recorded sounds of patients' voice were difficult to recognize.
These results indicate that the speech discrimination ability of the patient is not adequate enough to recognize their abnormal articulation and that makes it difficult to improve their mal-articulation.

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Objectives; The aim of this study was to determine the optimal timing of hard palate closure with low surgical damage and to assess the difficulty in achieving early acquisition of normal articulation using Furlow's palatoplasty (F-group), which provides earlier velopharyngeal competence than by Perko's method (P-group).
Subjects and Methods;
1. Serial study cast models of the F-group (unilateral cleft lip and palate, N=47) were used to evaluate the reduction in the residual cleft size in the hard palate.
2. The surgical results of the F-group, such as the maximum cleft width, operation time, blood loss and fistula rate were compared with those of the P-group.
3. The surgical damage and difficulty of hard palate closure in the F-group was also subjectively evaluated by inspecting randomly-selected cast models at four years of age and at the time of hard palate closure.
Results;
1. The anterior cleft width showed a significant reduction by two years of age.
2. The posterior cleft width also showed a significant reduction by three years of age. On the other hand, the posterior alveolar width showed a significant increase by four years of age.
3. The maximum cleft width and the blood loss in the F-group and P-group were 4.06 mm and 15.3 g, and 6.02 mm and 34.6 g, respectively (p < 0.001). There was no difference in the operation time or the fistula rate.
4. The subjective evaluation of the surgical damage and difficulty in achieving hard palate closure in the F-group was low. The agreement between the evaluations at four years of age and at the time of hard palate closure was high.
Conclusion; This study showed that the hard palate could be closed at 4 years of age with low surgical damage and little difficulty by applying Furlow's method.

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It is common for mothers of children with cleft lip/palate to feel anxious about heredity and uneasy during pregnancy with their second child. This survey was conducted to clarify how health-care staff involved in the treatment of cleft lip/palate provide information on the second pregnancy. In the survey, a self-administered questionnaire was sent to health-care staff by mail between September and November 2008 and 768 replies were obtained (recovery rate, 35.6%; valid response rate, 99.7%), with the following results:
1. Orthodontists were the most common, accounting for 57.4% of the staff, followed by oral surgeons, plastic surgeons, dentists, speech pathologists, otolaryngologists, and others (nurses and clinical psychologists).
2. The number of staff who "have been consulted about a second pregnancy" was 309 (40.2%). The support (multiple answers) conducted at that time was as follows: "listening to mothers", "talking about the recurrence rate", "referring to genetic counselors", "referring to family support groups", and "advising not to worry". Forty-nine staff "were at a loss how to proceed", the reasons for which included: gaps in knowledge of the recurrence rate, impossibility of providing support, lack of professional staff (counselors), increasing psychological anxiety, and wondering to what extent they could explain things.
3. The number of staff who "explained matters concerning the second pregnancy from their own perspective" was 140 (18.2%), comprising orthodontic, oral, and plastic surgeons. The rates of the above three types of surgeon were similar. Regarding the time to explain, the time taken for the first consultation was the most consistent.
4. Other professional staff currently coordinating with were "genetic counselors" in only 15 respondents and "clinical medical geneticists" in only 49. Other professional staff wishing to coordinate with in the future were "genetic counselors" in 175 respondents and "clinical medical geneticists" in 154.
In the future, it is necessary to ask genetic professionals to join health-care teams to accommodate mothers' needs, and to discuss how to actually provide information and support, considering off-site coordination when appropriate.

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The purpose of this study was to clarify the current status of initial cleft lip and palate repair in Japan by analyzing the data on 1) techniques and timing of initial cleft lip surgery, 2) techniques and timing of initial cleft palate surgery and 3) timing of alveolar bone grafting, contained in the booklet "Cleft Lip and Palate Treatment Plan —Current Practice of 111 Multidisciplinary Teams in Japan—" published by the Japanese Cleft Palate Association. The following results were obtained.
1. Approximately 70% of the initial operations for unilateral cleft lips are performed using the technique classified as the Millard or modified Millard procedure. Many of these operations are undertaken at age 3 months.
2. Bilateral cleft lip reconstruction is performed in a single stage at 68.8% of the institutions surveyed. The most frequently used techniques are the Mulliken and modified Mulliken techniques, accounting for 46.5%. The most common timing of the operation is 3 months after birth.
3. Cleft palate repair is carried out in one stage at 82.5% of the institutions. Pushback techniques account for 60% of the palate repair techniques used.
4. As for criteria for the timing of alveolar bone grafting, approximately 50% of institutions use calendar age, 30% use tooth eruption status, while some institutions base their decisions on input from orthodontists.

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Semi-structured interviews were conducted with seven mothers who had recently given birth to infants with a cleft lip and palate to identify the principal factors causing them maternal stress and ways to alleviate this stress when rearing their children and getting them treated. Analysis of the interview transcripts led to several findings, as summarized below.
(1) We identified three main categories of maternal distress: postpartum distress, child-rearing distress, and treatment distress. These categories were then broken down into nine subcategories: a sense that something wrong had happened, feeling of not being allowed to provide sufficient early mother-infant contact, feeling of not receiving sufficient information about their child's condition, sadness that their child was segregated from other children, sense of remorse for giving birth to a child with a defect, worried that the child would attract attention when outdoors, anxiety about not being able to breastfeed well, anxiety over cleft-related surgery, and feeling of sadness for infant undergoing cleft treatment.
(2) We then identified ways to alleviate this stress from the transcripts and grouped them into four categories: early mother-infant physical contact, working closely with cleft medical specialists, recalling previous experiences of raising children, and peer support. These were further grouped into eight subcategories: early mother-infant physical contact, clear explanations from cleft specialists, assurance of full recovery, assurance of ability to breastfeed, smooth weaning progression, knowing that the infant is developing well, meeting other children with cleft condition, and support from other mothers while in the hospital.
These findings suggest the best ways that medical professionals can provide psychological support and encouragement to new mothers with cleft lip/palate infants are: (1) to put the mother in contact with specialized cleft medical personnel as soon as possible, to fully explain the nature of the cleft condition early on, and to make sure the infant is getting the proper treatment; and (2) to make sure the mother understands the significance of peer contact and to provide a forum early on where mothers can exchange information and provide mutual support.

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Since 1990, several kinds of specialists in Hokkaido University Hospital have gathered under a team approach for cleft lip and palate patients. Prosthetic treatment is the final procedure by this team approach, and has a good prognosis that can be predicted well. The final restoration for cleft patients should maintain good function for a long time, and has an important role in preventing post-orthodontic relapse. We investigated the validity of prosthodontic treatment over the past 16 years.

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This report presents the use of ex vivo produced oral mucosa equivalent (EVPOME) applied to an oral mucosa defect in two patients with cleft lip and palate. Autologous keratinocytes were placed and propagated in a chemically-defined, animal product and a xenogeneic feeder layer free culture system. Cells were then seeded and cultured on an acellular dermal substrate, AlloDerm^®, which provides strength, durability, and elasticity to the graft.
In case 1, a 20-year-old female underwent closure of the oronasal fistula in the midline of the hard palate using a rotational palatal flap. A circular EVPOME graft (approximately 20 mm in diameter) was grafted onto the bone surface of the donor site of the flap. In case 2, a 16-year-old male underwent vestibuloplasty in the premaxilla region. After trimming, two circular EVPOME grafts were placed over the intact periosteum.
The grafts were easily fixed in place to the surrounding scarred mucosa. Although the underlying tissue of the EVPOME graft sutures was scarred, graft adherence was achieved within a week. The successful clinical outcome reported here suggests the efficacy of EVPOME grafts onto a less-vascularized, scarred tissue, and that the use of EVPOME grafts is highly applicable for patients with cleft lip and palate. EVPOME grafting could be extended especially toward pediatric patients with cleft lip and palate for reconstruction of oral mucosa defects by using cryopreserved autologous keratinocytes and by developing a novel material to ensure a steady supply of scaffold instead of the man-made AlloDerm^®.

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We reported a case of bilateral cleft lip and palate treated by surgical rotation of tooth germ of the lateral incisor adjacent to the alveolar cleft along with bone graft. The patient was a 7-year, 4-month-old boy at the time of the operation. The alveolar cleft existed only on the right side. The image findings revealed that the upper right lateral incisor was conical, infraversed and inclined mesialy at about 90 degrees. The degree of completion of root formation described by Moorrees was Ri to R_1/4. The right upper second premolar was also absent. During the operation, the tooth germ of the lateral incisor was exposed on the cleft margin of the minor segment. After extraction of the upper right deciduous canine and removal of the dental sac of the crown of the lateral incisor, the lateral incisor was inclined toward the socket of the deciduous canine in order not to move the location of the root apex. Finally, particulate cancellous bone and marrow from the iliac crest were grafted and the wound was completely closed. The lateral incisor erupted at 8 months and the root formation was completed by 2 years after the operation. There has been no evidence of root resorption, curved root or pulpa canal obliteration in the 7 years since the operation.
In order to achieve successful results of surgical rotation of tooth germ, it is important to operate at the initial root formation stage, with less surgical damage, and not to move the location of the root apex. It is also recommended that the degree of root rotation should not exceed 90 degrees. If these conditions are satisfied, the surgical repositioning of the tooth becomes a treatment option for occlusal formation of the alveolar cleft region where misdirection and dislocation of teeth occur frequently.

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A submucous cleft palate (SMCP) patient presenting with a fistula often develops hypernasal speech that necessitates surgical repair; however, there have been no reports describing the appropriate timing for surgery. Here, we report two SMCP cases presenting with a congenital and acquired fistula of the palate. The first patient was a 5-year-old boy with SMCP who had a congenital fistula with hypernasality and in whom palatal repair with an excision of the fistula was apparently indicated. The second patient was a 2-year-old boy with SMCP who had an acquired palatal fistula. This patient was not mature enough for an adequate speech evaluation; nevertheless, he underwent a palatal repair with an excision of the fistula based on the consideration that SMCP presenting with an acquired fistula often results in the development of hypernasality. We consider that it is advisable to perform a surgical repair of SMCP presenting with a congenital or acquired fistula as soon as possible, if the patient is older than the age at which cleft palate is generally operated on. Otherwise, this repair should be performed at the general timing for cleft palate repair.

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In recent years, cleft lip and palate has been treated by a team approach, resulting in consistent treatment plans for each patient. However, treatment planning and treatment are sometimes difficult in patients who previously underwent surgery in another hospital. We operated on a patient with jaw deformity who had undergone pharyngeal flap surgery in another institution. The patient was a 16-year-old female who had undergone palatoplasty and pharyngeal flap operation under a diagnosis of submucous cleft palate in another institution at 6 years of age. Subsequently, treatment was initiated at the department of orthodontic dentistry of our hospital. At the age of 16 years, a Le-Fort I osteotomy was planned due to maxillary undergrowth. Though nasotracheal intubation was difficult, she wished to avoid tracheotomy. Therefore, in August 2005 we performed oropharyngeal intubation using a bite plate in an open-mouth state and Le-Fort I osteotomy. After the operation, occlusion became stable, and good results without relapse have been observed for 4 years to the present.

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Treacher Collins Syndrome (TCS) is an autosomal dominant disorder of craniofacial development. The major features of the disease include midface hypoplasia, micrognathia, microtia, conductive hearing loss and cleft palate. Birth prevalence has been estimated at 1:50,000 newborns and approximately 60% are caused by new mutations.
The Pierre Robin Sequence (PRS) is a relatively rare symptom complex characterized by glossoptosis, micrognathia and respiratory obstruction. The pathogenesis of PRS is multifactorial and syndromic in nearly half of the patients including Stickler Syndrome, Velocardiofacial Syndrome, and Treacher Collins Syndrome.
We report a case of TCS and PRS with feeding dysfunction in a 2-month-old girl. She has underwent tracheotomy soon after birth, and respiratory distress was improved. She required nasogastric feeding as do most infants with PRS. For feeding dysfunction, we used the modified pacifier with tube and syringe installed by us. This pacifier enabled the volume of milk to be measured and made desensitization short. Our modified pacifier may be effective for patients with feeding dysfunction.

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We encountered a case of bilateral cleft lip and palate in which, although palatal fistula closure surgery was performed twice using tongue flaps after completing orthodontic treatment, sufficient closure was not achieved. Therefore, to reduce the width of the alveolar cleft and palatal fistula, we performed maxillary alveolar bone movement using interdental distraction osteogenesis and orthodontic treatment, and a good result was obtained.
The patient was a 16-year and 9-month-old boy with bilateral cleft lip and palate, in whom a space was present in the distal area of the maxillary central incisor, and a 16 mm×14 mm palatal fistula remained in the palate. As the treatment plan, we set the mesial area of the bilateral maxillary first molars as the osteotomy line to move the lateral segment together with its alveolar bone in front of the bilateral maxillary first molars in the anterior direction using distraction. We produced a tooth-tooth type distractor in which a Zurich type ramus distractor (Martin Co.) was soldered to orthodontic bands on the right first and left second premolars and first molars. Regarding the distraction method, after a latency period of 1 week, distraction was initiated at the rate of 0.5 mm×2 per day, and the left side was distracted by 5.0 mm, and the right side by 7.5 mm. The consolidation period was 1 month, and the distractor was removed 1 month after distraction. One month after completing distraction, orthodontic treatment using edgewise appliances was started, and the maxillary first molars were anteriorly moved to the bone area which was newly generated by distraction. Thereafter, 1 year after performing distraction, the remaining palatal fistula was closed using auricular cartilage grafting and local flaps. Furthermore, β-TCP, platelet-rich plasma, and medullary fluid filled the alveolar cleft. Thereafter, orthodontic treatment was continued to close the space, and a good occlusal relationship was obtained. The active orthodontic treatment period was 2 years and 11 months.
Application of interdental distraction osteogenesis showed the following advantages:
1. The size of the palatal fistula was reduced and closure of the fistula became easy.
2. The alveolar cleft was closed, and prosthetic treatment became unnecessary.

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The 18q deletion (18q-) syndrome is a multiple-anomaly disorder associated with craniofacial, limb, and genitourinary malformations as well as neuropsychiatric abnormalities. The estimated frequency of occurrence of this syndrome is reported to be approximately 1 in 40,000 live births.
Here, we present the case of a 3-year-old boy with 18q- syndrome, complicated by midfacial hypoplasia, cleft palate, auricle deformities, limb anomalies, heart defects, neuropsychiatric abnormalities, and growth retardation. After the palatal plasty was operated on, he suffered from frequent apnea attacks, and was administered oxygen as treatment. Thereafter, the frequency of the attacks gradually reduced and he was discharged 12 days after the operation. The attacks seemed to be autonomic seizures due to 18q- syndrome, which were induced by general anesthesia. Extreme caution should be taken when using general anesthesia for patients with 18q- syndrome because they could present with various congenital abnormalities. Remarkable points of perioperative management have been reported, including pulmonary complications, heart failure, difficulty of tracheal intubation, and tendency for infection.

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We perform palatal closure in two stages to prevent growth disturbances in the bony maxillary bone structures. In this study, among 164 patients with unilateral cleft lip and palate who received continuous treatment from the period immediately after birth in the Department of Oral Surgery of our college, 68 patients whose long-term follow-up was possible during the treatment period in our orthodontic department were selected; 4 of these patients, in whom orthognathic surgery was finally required, were evaluated.
Regarding the evaluation method, we investigated 16 angle measurement items in lateral roentgenographic cephalograms at the time of the first examination in our orthodontic department, initiation of orthodontic treatment after completion of permanent dentition, and orthognathic surgery. Furthermore, facial prophilograms were produced, and patterns of facial profiles were classified.
Case 1: A male patient who had macroglossia and a tongue-thrusting habit, and had already presented with open bite at the time of the first examination in our orthodontic department. Le Fort type I osteotomy, sagittal split ramus osteotomy, and tongue-reducing surgery were performed at 18 years of age.
Case 2: A female patient who presented with slight maxillary retrusion at the time of the first examination in our orthodontic department. Although the values of items in the maxilla were almost within the normal range after entering adolescence, mandibular hyper-growth became marked. Sagittal split ramus osteotomy was performed at 16 years of age.
Case 3: A male patient who presented with slight maxillary retrusion at the time of the first examination in our orthodontic department and initiation of orthodontic treatment after completion of permanent dentition. Although only orthodontic treatment was planned at first, since the patient requested a shorter treatment period, sagittal split ramus osteotomy was performed at 18 years of age.
Case 4: A male patient who presented with maxillary retrusion and marked mandibular protrusion at the time of the first examination in our orthodontic department. The hyper-growth of the mandible progressed at the time of initiation of orthodontic treatment after completion of permanent dentition, and Le Fort type I osteotomy and sagittal split ramus osteotomy were performed at 19 years of age.
In all of these 4 cases, the reasons for orthognathic surgery were considered to be more closely related to factors other than the influence of palatoplasty.

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