Pierre Robin sequence: appearances and 25 years of experience with an innovative treatment protocol

Purpose

The aim of the study was to evaluate the largest number of Pierre Robin sequence (PRS) cases to date and its treatment outcome.

Design

This is a retrospective study.

Settings

The study was conducted in a tertiary care university hospital.

Patients

One hundred eighty-eight patients with PRS have been subjected to analysis, defined by the clinical triad of glossoptosis, retro/micrognathia, and cleft or agenesis of the palate, for incidence, risk factors, associated syndromes, other concomitant abnormalities, airway and feeding difficulties, and outcome regarding the neonatal and perinatal surgical and nonsurgical management.

Results

The incidence of PRS was 6.02%. Risk factors were found in 41.5% of mothers with children with PRS. Eleven other syndromes/appearances were associated. Feeding difficulties were seen in 51.8% of the patients. Glossopexy (6.9%) and tracheotomy (2.13%) for airway management were performed very seldom. The suction and drinking plate as well as the surgical treatment protocol may be considered to be the reasons for reduced airway (up to 91.0%), feeding problems (up to 79.9%), and mortality rate (2.1%).

Conclusion

The Pierre Robin sequence, as seen as a heterogeneous group, presents with variation of the cleft palate defects with glossoptosis and concomitant micrognathic mandible. The surgical management and certain prepalatal intervention as nonsurgical management with an innovative treatment strategy were evaluated.     

Bilateral mandibular distraction in micrognathism or hypoplasia of mandible,hazrat fatemeh hospital

ObjectiveTo determine the results of Bilateral Distraction Osteogenesis (DO) versus traditional methods in treatment of micrognathia.MethodsDuring 6 years we had 67 patients with micrognathia. In group A, randomly 35 were treated with traditional methods (Saggital osteotomy and/or bone grafts) and group B 32 cases were treated with Bilateral External Distraction Osteogenesis. Follow up was 36+/− 6 months. X-Ray, CT scan and cast modelling were done for all of the patients. SNA, SNB angles were measured in cephalograms. The patients were examined after one, two, three, six, 12 months for any complications and signs of bone resorption and relapse. Bony and soft tissue advancements were measured and compared with each other.ResultsDuring 6 years we had 67 patients with class II malocclusion. Mean SNA angle in group A was 80.8 (80–81.5) and 80.9 (80–82) in group B. Mean SNB angle in group A was 77.6 (76.5–78) and 77.4 (76.5–78.5). After treatment mean SNB in group A was 79.1 and the difference was significant comparing to pre-op measurement (P<0.005). Mean SNB in group B after treatment was 79.2 which was significantly different comparing with SNB angle before surgery. The mean SNA in both group were not statistically and significantly different. The mean SNB after surgery in both group were not statistically and significantly different. Mean Mandibular advancement in group A was 11.9 mm (7.5–18 mm) and group B with DO treatment was12.1 mm (8–19 mm) which was not significantly different. The patient in group A evaluate the surgical results as excellent (60%) and good (28.5%). The patient in group B evaluate the results as excellent (78.1%) and good (15.6%).ConclusionThe Bilateral Distraction Oseogenesis method can bring forwards the retruded mandible effectively and its results has no difference with older methods. It is an effective and reliable procedure for hypoplasia of Mandible and the results are comparable with older methods.     

三维彩超诊断胎儿小下颌畸形

目的:运用二维彩色多普勒及实时三维成像探讨胎儿小下颌畸形的超声表现及各指标综合诊断方法,提高其超声诊断准确率,总结其临床应用价值。方法:应用二维彩色多普勒超声测量6 300例孕周为18-32周的胎儿下颌骨纵径(FML),左右径,下颌骨纵径与双顶径的比值即下颌指数(JI),胎儿下颌面部角度(IFA)。观察胎儿正中矢状面下颌曲线及冠状面下颌显像,并应用实时三维表面成像技术进一步显示胎儿下颌结构。结果:正常胎儿下颌骨左右径及纵径FML与其孕周正相关(R分别为0.952,0.954,P均<0.01);小下颌组胎儿JI、IFA较正常对照组胎儿显著减小,差异有统计学意义(P<0.05),FML明显小于正常同孕周胎儿。结论:胎儿FML明显小于同孕周正常值,JI<31%,IFA<45%为产前诊断小下颌畸形的超声综合数据指标,二维及实时三维彩超联合应用诊断胎儿小下颌畸形明显优于传统诊断方法,具有其他影像学检查不可取代的临床应用价值。     

Mandibular distraction osteogenesis: at what age to proceed

Objective

To evaluate if there is a difference in mandibular distraction osteogenesis (MDO) treatment success rates and adverse outcomes in newborns, early infants, and older pediatric patients.

Design

Retrospective medical review spanning a nine-year period. Ten newborn (≤35 days old), five early infant (36 days-5 months) and eight older pediatric (>5 months) patients underwent MDO for treatment of micrognathia with a severe tongue-based obstruction. Success was defined as avoidance of tracheostomy or CPAP, and decannulation of patients with tracheotomies. Postoperative complications were grouped into minor and major.

Results

MDO successfully treated 90% of newborns, 100% of early infants and 100% of older pediatric patients. There was no difference in the rates of success (p = 0.48), minor (p = 1.00) and major (p = 1.00) postoperative complications between newborns and early infants. Older pediatric patients had no treatment failures, tended to have fewer minor (p = 0.18) and significantly fewer major (p= 0.04) postoperative complications compared to younger patients. The distractor pin mobility (9%) and scar revisions (13%) were uncommon.

Conclusions

Mandibular distraction osteogenesis is a reliable method for relieving severe tongue-based obstructions in pediatric patients. When comparing newborns and early infant patients, treatment success rates and the occurrence of complications were not found to be different. Older pediatric patients had no treatment failures, and tended to have fewer postoperative complications compared to younger patients.     

中重度小颏畸形整形21例

目的探讨中重度小颏畸形的综合美容整形方法。方法手术采用口内入路,于下唇唇龈沟唇侧黏膜设计“V”形切口,切开黏膜的长度约为4cm。切开粘骨膜达颏骨表面,骨膜剥离子剥离显露颏骨骨面。对于中度小颏畸形,可单纯采用Medpor植入术,对于重度小颏畸形,先通过截骨前移的方法予以矫正,如果仍显不足,可在术后半年,在截骨前移的基础上,再采用Medpor植入,进一步增加颏部的前突度和高度。结果本组共21例,其中女性患者19例,男性2例,年龄17～36岁,平均24岁。单纯采用Medpore假体植入隆颏术12例,颏部截骨前移6例,颏部截骨前移加二期Medpore假体植入隆颏3例。术后小颏畸形明显改善,形态良好。结论对于中重度小颏畸形可采用Medpore假体隆颏、颏部截骨前移或二者结合的手术方法予以矫正。Medpore是一种良好的隆颏材料,其隆颏效果优于传统的硅胶假体。     

Dynamic orthognathic surgical procedure (DOSP) in asymmetric maxillomandibular dysmorphism secondary to unilateral micrognathia: Outcomes of 12 consecutive cases

This study aimed to evaluate the outcomes following a dynamic orthognathic surgical procedure performed at the end of growth to treat asymmetric maxillomandibular deformities linked to unilateral micrognathia when conventional orthognathic surgery was not feasible.The dynamic orthognathic surgical procedure (DOSP) combined concomitant mandibular distraction osteogenesis with contralateral poorly stabilized sagittal split osteotomy and Le Fort I osteotomy. Cephalometric studies were retrospectively conducted on pre- and postoperative lateral and frontal cephalographs, and maxillomandibular movements were calculated. Outcome scores were computed by both experts and laypersons based on photographic analyses.There was a significant postoperative increase in height of the micrognathic ramus in all patients (n = 12; p = 0.002). The angle between the occlusal cant and horizontal reference plane decreased significantly in all of the patients, as did the angle between the midline sagittal plane and mandibular tilt (p < 0.001). Postoperative outcome scores showed significant improvements in all cases, according to both expert and layperson groups.This procedure allows correction of maxillomandibular asymmetries linked to micrognathia. However, it cannot resolve all the factors participating in facial asymmetry, such as those originating in the oculo-auriculo-ventricular spectrum or complex tumor sequelae, and second-step procedures may be required.     

Advantages and disadvantages of mandibular distraction in Robin sequence

Robin sequence (RS) is diagnosed in infants born with micrognathia, glossoptosis and varying degrees of upper airway obstruction (UAO). Due to the variable levels of hypoxia, severe breathing and feeding problems can occur. Treatment is determined by clinical severity, ranging from conservative interventions for mild cases to surgical interventions for severe cases. Mandibular distraction osteogenesis (MDO) is a surgical technique that gradually lengthens the mandible after an osteotomy by using an internal or external distraction device, directly correcting the micrognathia. This review will focus on advantages and disadvantages of mandibular distraction in infants with RS.     

骨牵引技术治疗TMJ强直小下颌畸形伴阻塞性睡眠呼吸暂停低通气综合征

目的:探讨牵引成骨术(d istraction osteogenesis DO)在治疗小下颌畸形伴阻塞性睡眠呼吸暂停低通气综合征(obstructive sleep apnea hypopnea syndrom e,OSAHS)中的应用。方法:9例小下颌畸形伴OSAHS患者行下颌骨牵张成骨术,3个月后行颞下颌关节成形术,术前术后行头颅侧位测量和多导睡眠监测仪测试。结果:9例牵引完成后所有患者后气道间隙(posterior airway space,PAS)值扩大,睡眠呼吸暂停综合指数下降,血氧饱和度增加,OSAHS症状消失或减轻,面部畸形得到明显改善。结论:DO技术在矫治颞下颌关节强直导致的小下颌畸形伴OSAHS中具有重要的临床价值。