RESUMEN
Hypoglossia-hypodactyly, or aglossia-adactylia syndrome with or without limb anomalies, is an extremely rare congenital condition. It is characterized by a narrow, V-shaped mandibular dental arch with micrognathia, and is typically challenging to treat. We have previously reported 3 patients with hypoglossia-hypodactyly syndrome without limb anomalies who were treated with transverse distraction osteogenesis at the mandibular symphysis during childhood. In this report, we present the long-term prognoses of these 3 cases, until 18 years of age. Of the 3 total cases, sufficient and stable results in occlusion, speech, mastication, and facial appearance were obtained in 2 cases with subsequent orthodontic treatments. Similar results were not observed in the remaining case when continuous orthodontic treatments were not performed. Based on these results, we propose that the transverse distraction osteogenesis procedure at the mandibular symphysis during childhood could be a reliable method to correct the V-shaped mandibular arch of hypoglossia-hypodactyly syndrome, when subsequent orthodontic treatments are provided during the patient's growing period.
RESUMEN
Maxillary hypoplasia is a major issue in cleft lip and palate patients, and predictable surgical maxillary advancement is required. In the present study, the changes and stability of the maxilla and soft tissue profile achieved after the application of anterior maxillary distraction osteogenesis (AMDO) using intraoral expander in unilateral cleft lip and palate and isolated cleft palate patients were investigated by comparing to the Le Fort I osteotomy (LFI) and maxillary distraction osteogenesis (DO) with rigid external distraction (RED) system.Ten patients who underwent orthognathic treatment with AMDO were examined (AMDO group). Changes in the positions of soft and hard tissue landmarks were calculated from the lateral cephalograms taken before the distraction, at the end of the distraction, and 1 year after the surgery. They were compared with the changes in 7 other unilateral cleft lip and palate patients who underwent LFI (LFI group) and 6 others who underwent DO with RED (RED group).The mean maxillary advancement of the AMDO group was similar to that of the RED group, judged by the change of point A. During DO, the AMDO group showed less clockwise rotation of mandible compared to the RED group. The soft tissue advancement of the upper lip and nose in the AMDO group was similar to that in the RED group, which was significantly larger than that in the LFI group.Our results indicate that AMDO can be surgical option to cleft lip and palate patients with less invasive but excellent improvement in both midfacial skeletal and soft tissue similar to DO-RED.
Asunto(s)
Labio Leporino/cirugía , Fisura del Paladar/cirugía , Maxilar/cirugía , Osteogénesis por Distracción/métodos , Adolescente , Adulto , Cefalometría , Femenino , Humanos , Labio , Masculino , Mandíbula , Nariz , Osteotomía Le Fort/métodos , Resultado del Tratamiento , Adulto JovenRESUMEN
OBJECTIVE: To investigate current trends in primary treatment for children with cleft lip and/or cleft palate in Japan. DESIGN: Nationwide, retrospective study under the direction of the Academic Survey Committee of the Japanese Cleft Palate Association based on analysis of data obtained via a booklet-style questionnaire completed by institutions providing primary treatment for cleft lip and/or palate patients. PARTICIPANTS, PATIENTS: Patients were 4349 children undergoing primary repair for cleft lip and/or palate at 107 participating institutions between 1996 and 2000. MAIN OUTCOME MEASURE(S): Cleft type, laterality; use of infant palatal plate; and timing and technique of primary repair for cleft lip and/or palate were evaluated by cleft surgeons at 107 participating institutions. RESULTS: Of a total of 2874 patients with cleft lip and palate or cleft palate only, infant palatal plates were used with 1087 (37.8%) and were not used with 1787 (62.2%). Primary unilateral lip repair was performed at the age of 2 to 6 months in more than 90% of patients. Bilateral cleft lip was treated by one-stage repair in 285 patients (44.5%) and by two-stage repair in 258 (40.2%). Primary one-stage palatal repair was performed in 2212 (76.9%) and two-stage palatal repair in 262 (9.1%) cleft palate patients. Information on treatment of the remaining 400 (14%) patients was unavailable. CONCLUSION: This investigation clarified current trends in primary treatment for cleft lip and/or palate in Japan. The results suggest the need for an increase in regional core hospitals and greater variation in treatment options.
Asunto(s)
Labio Leporino/cirugía , Fisura del Paladar/cirugía , Procedimientos Quirúrgicos Orales/métodos , Procedimientos de Cirugía Plástica/métodos , Pautas de la Práctica en Medicina/estadística & datos numéricos , Preescolar , Femenino , Humanos , Lactante , Japón , Masculino , Evaluación de Procesos y Resultados en Atención de Salud , Estudios RetrospectivosRESUMEN
PURPOSE: The purpose of this study was to investigate the changes in and stability of the maxilla and soft tissue profile achieved after the application of distraction osteogenesis (DO) by use of rigid external distraction (RED) with a retention plate system in unilateral cleft lip and palate (UCLP) adult patients. We compared 2 treatment methods in the management of maxillary hypoplasia: Le Fort I osteotomy and DO. MATERIALS AND METHODS: Six UCLP adult patients who underwent treatment with the RED retention plate system were examined (DO group). Changes in the positions of soft and hard tissue landmarks were calculated from lateral cephalograms taken before distraction, at the removal of the halo, and 1 year after surgery and were compared with those in 7 other UCLP patients who underwent Le Fort I osteotomy (LF1 group). RESULTS: The mean maxillary advancement was significantly larger in the DO group than in the LF1 group after distraction. During the follow-up period, the relapse rate of the maxilla was significantly smaller in the DO group. An undesirable labial inclination of the upper incisors was found in the LF1 group, which may have been due to relapse. The DO group tended to have a higher soft tissue-to-hard tissue anterior movement ratio from the time of distraction to follow-up. CONCLUSIONS: The RED retention plate system improved the midfacial profile by advancement of soft and hard tissue and minimized the risk of injury to the upper lip. Using the RED system with retention plates prevented the undesirable labial inclination of upper incisors that was found in the LF1 group.
Asunto(s)
Labio Leporino/cirugía , Fisura del Paladar/cirugía , Fijadores Externos , Maxilar/cirugía , Procedimientos Quirúrgicos Ortognáticos/métodos , Osteogénesis por Distracción/instrumentación , Adolescente , Adulto , Placas Óseas , Cefalometría , Labio Leporino/complicaciones , Labio Leporino/rehabilitación , Fisura del Paladar/complicaciones , Fisura del Paladar/rehabilitación , Cara/anatomía & histología , Asimetría Facial/etiología , Asimetría Facial/cirugía , Lateralidad Funcional , Humanos , Técnicas de Fijación de Maxilares , Masculino , Maxilar/anomalías , Desarrollo Maxilofacial , Procedimientos Quirúrgicos Ortognáticos/instrumentación , Osteogénesis por Distracción/métodos , Osteotomía/métodos , Resultado del Tratamiento , Adulto JovenRESUMEN
OBJECTIVE: The purpose of this study was to evaluate the long-term follow-up of dental implants placed in the grafted alveoli of patients with cleft lip or palate clinically. PATIENTS AND METHODS: Sixteen patients (8 males and 8 females) who had dental implants placed in alveoli grafted using particulate cancellous bone and marrow (PCBM) from the iliac crest were evaluated. The marginal bone level around the implant was evaluated radiologically at 1 (stage I), 3 (stage II), and 6 years (stage III) after connecting the abutment. The interdental alveolar bone height (IABH) was also evaluated radiologically for up to 6 years. RESULTS: During the follow-up period of an average of 8.6 +/- 0.6 years (range: 7.2 to 9.4 years), only 2 implants were lost in 1 patient, the cumulative survival rate was 90.9%, and the clinical outcome was uneventful in all implants. The marginal bone levels around the implants were 0.29 +/- 0.18, 0.29 +/- 0.19, and 0.28 +/- 0.15 mm at stages I to III, respectively. Moreover, IABH was reduced only in 2 of 16 (12.5%) of the implant-placed grafted alveoli, and was maintained after implant placement for up to 6 years. CONCLUSIONS: These findings indicate that the use of dental implants placed in grafted alveoli is beneficial for maintenance of the grafted bone in patients undergoing secondary bone grafting for cleft repair.
Asunto(s)
Pérdida de Hueso Alveolar/prevención & control , Proceso Alveolar/diagnóstico por imagen , Alveoloplastia , Trasplante Óseo , Fisura del Paladar/cirugía , Implantación Dental Endoósea , Adolescente , Adulto , Labio Leporino/rehabilitación , Fisura del Paladar/rehabilitación , Fracaso de la Restauración Dental , Femenino , Estudios de Seguimiento , Humanos , Masculino , Radiografía , Resultado del Tratamiento , Dimensión VerticalRESUMEN
OBJECTIVE: Documentation of the application of maxillary distraction osteogenesis using rigid external distraction (RED) with skeletal anchorage combined with predistraction alveolar bone grafting (ABG) in cleft maxilla. DESIGN: Case report. PATIENT: A patient with numerous congenital missing teeth and severe maxillary deficiency related to complete bilateral cleft lip and palate with large alveolar bone defect. INTERVENTION: The patient received preoperative orthodontic treatment, predistraction ABG, and maxillary distraction osteogenesis using RED with skeletal anchorage. RESULTS: Predistraction ABG completely united the cleft maxilla. The united maxilla was successfully advanced by the RED system with skeletal anchorage, despite unsound dentition with numerous congenital missing teeth. CONCLUSION: The present study demonstrates that the combination of predistraction ABG and RED system with skeletal anchorage is effective for the treatment of severe maxillary deficiency related to complete bilateral cleft lip and palate with large bone defect and numerous congenital missing teeth.
Asunto(s)
Anomalías Maxilomandibulares/cirugía , Maxilar/anomalías , Maxilar/cirugía , Procedimientos Quirúrgicos Orales , Osteogénesis por Distracción/métodos , Adolescente , Anodoncia/etiología , Anodoncia/rehabilitación , Trasplante Óseo , Cefalometría , Labio Leporino/complicaciones , Fisura del Paladar/complicaciones , Implantes Dentales , Femenino , Humanos , Anomalías Maxilomandibulares/complicaciones , Ferulas Oclusales , Ortodoncia Correctiva , Osteogénesis por Distracción/instrumentación , Retrognatismo/cirugíaRESUMEN
Cleidocranial dysplasia (CCD) is a rare autosomal dominant skeletal dysplasia. In order to define the morphologic characteristics of the masseter muscle incidental to bone abnormalities, we present 3 cases of CCD with the masseter muscle thickness and maxillofacial bone abnormalities, using computed tomography (CT) and panoramic radiographs. In CCD patients (a) the masseter muscles were less thick than in age- and sex-matched control subjects, (b) the zygomatic arch was discontinuous with the hypoplastic zygomatic bone, (c) the ascending ramus of the mandible had parallel-sided borders, and (d) the coronoid process pointed upwards and/or posteriorly. We have concluded the masseter muscles are less thick than normal, alongside the maxillofacial bone abnormalities in CCD patients.
Asunto(s)
Displasia Cleidocraneal/patología , Músculo Masetero/patología , Adulto , Estudios de Casos y Controles , Cefalometría , Niño , Displasia Cleidocraneal/diagnóstico por imagen , Femenino , Humanos , Mandíbula/diagnóstico por imagen , Mandíbula/patología , Músculo Masetero/diagnóstico por imagen , Maxilar/diagnóstico por imagen , Maxilar/patología , Radiografía Panorámica , Hueso Temporal/diagnóstico por imagen , Hueso Temporal/patología , Tomografía Computarizada por Rayos X , Cigoma/diagnóstico por imagen , Cigoma/patologíaRESUMEN
OBJECTIVE: Documentation of the application of mandibular widening by distraction osteogenesis and orthodontics. PATIENTS: Three patients with telescopic bite resulting from an extremely constricted mandible related to hypoglossia-hypodactyly syndrome. INTERVENTION: Mandibular widening by distraction osteogenesis using an extraoral device and subsequent orthodontic treatment. RESULTS: The extremely constricted mandible and telescopic bite were dramatically improved by mandibular widening using distraction osteogenesis and subsequent orthodontics. Two of the three patients had transient complications; one reported temporomandibular joint pain and the other showed evidence of periodontal damage. CONCLUSION: Mandibular widening by distraction osteogenesis is an effective technique for the treatment of telescopic bite resulting from an extremely constricted mandible.
Asunto(s)
Maloclusión/cirugía , Mandíbula/cirugía , Micrognatismo/cirugía , Procedimientos Quirúrgicos Orales , Osteogénesis por Distracción , Niño , Femenino , Deformidades Congénitas del Pie , Deformidades Congénitas de la Mano , Humanos , Maloclusión/terapia , Mandíbula/anomalías , Micrognatismo/terapia , Ortodoncia Correctiva , Síndrome , Lengua/anomalíasRESUMEN
OBJECTIVES: To clarify the need for secondary alveolar bone grafting (ABG) in incomplete alveolar clefts identified clinically from its appearance and test the usefulness of alveolar ridge notching in the edentulous stage as a predictor for ABG. DESIGN: A prospective, cross-sectional study comparing three groups of patients at two stages. PATIENTS: Twenty-four patients with unilateral incomplete cleft of primary palate who underwent either no surgery or cheiloplasty only. METHODS: Cleft severity was assessed at two stages. Initially, the appearance of untreated clefts was assessed before cheiloplasty or at 3 months of age in the patient without cheiloplasty and graded by severity into three groups. Later alveolar bone defects were assessed with computed tomography in the primary or mixed dentition period. A decision regarding the requirement for ABG was then made. Finally, the relationship between untreated clefts and the need for ABG was determined. MAIN OUTCOME MEASURES: Nine (75%) of 12 patients with alveolar ridge notching in the edentulous stage were diagnosed as fulfilling the criteria for ABG. RESULTS: The alveolar bone defects correlated with the severity of untreated cleft conditions (Spearman r = 0.65, p =.002). The need for ABG was greater in patients with alveolar ridge notching, compared with those without notching (9/12 versus 0/12, p =.00034). CONCLUSIONS: Most patients with incomplete alveolar clefts clinically identified in the edentulous stage required ABG, and alveolar ridge notching in the edentulous stage could therefore serve as a predictor for ABG.
Asunto(s)
Proceso Alveolar/anomalías , Proceso Alveolar/cirugía , Trasplante Óseo , Proceso Alveolar/diagnóstico por imagen , Labio Leporino/patología , Fisura del Paladar/patología , Estudios Transversales , Toma de Decisiones , Femenino , Humanos , Lactante , Masculino , Evaluación de Necesidades , Valor Predictivo de las Pruebas , Probabilidad , Pronóstico , Estudios Prospectivos , RadiografíaRESUMEN
OBJECTIVE: This study examined characteristics of nonverbal behavior that patients with cleft lip and palate (CLP) presented during interpersonal communication. DESIGN: This was a case-control design comparing nonverbal behavior of adult women with CLP with females without CLP. PARTICIPANTS: Subjects were 20 adult women with CLP and 20 noncleft control women matched for age and educational experience. MAIN OUTCOME MEASURES: Subject gestures and facial expressions were videotaped during interviews and analyzed with a computer-based kinematic measurement system. RESULTS: The clinical group displayed significantly fewer head movements and a lower smile frequency than the control group. Furthermore, head and hand movements and smiles were less coordinated or congruent for the subjects with CLP than for the comparison group. CONCLUSIONS: Even slight facial disfigurement could have a harmful effect on communication behavior in female patients with CLP.