颅缝早闭症术前三维CT测量及术后评估

目的 探讨术前、术后三维CT重建在颅缝早闭症患儿修复术中的意义.方法 对17例颅缝早闭症者于术前行CT三维重建,并运用计算机测量颅底俯位、颅骨与轴线相交点之间的距离,以及颅长、颅宽,并求出比值.分别对短头、舟状头、斜头(包括颅顶不对称指数)进行测量.将17例中9例术后的三维CT测量结果与术前进行对比.结果 17例颅缝早闭症术前的CT三维重建,短头前移距离在1.5～3 cm.舟状头后缩2～4 cm.斜头则前移后缩2 cm左右,9例术前与术后测量结果相吻合.结论 术前三维cT测量计算,可为颅缝早闭症手术提供颅骨瓣和眶额桥前移的数据及预估手术效果,术后用CT三维重建测量可以评价手术效果.     

全颅骨切开成形矫正早期矢状缝早闭头颅畸形

目的：探讨矢状缝早闭舟状头畸形的早期手术治疗方法。方法：患儿采取改良的俯卧位,通过头皮冠状切口显露从眉间至枕骨大孔后唇的整个颅盖,切开颅骨分离双侧额骨和双侧顶-枕部骨块。对额骨和枕骨进行放射状切开、塑形纠正前后部隆突畸形;切除部分额、顶骨块缩短头颅前后径;在颞、顶骨下部采用“木桶板”样截骨以及放射状切开、塑形两侧顶骨纠正头颅狭窄畸形。结果：手术后4例舟状头畸形明显改善,外形良好,无严重并发症。术后随访3～10月,头颅外形维持良好,畸形无复发。结论：包括双侧额、顶、枕、颞全颅骨切开重新塑形的手术方法能够有效地矫正婴幼儿矢状缝早闭舟状头畸形。     

颅盖成形术治疗先天性颅缝早闭症

目的探讨治疗先天性颅缝早闭症的多种颅盖手术方法。方法采用下述方法治疗37例先天性颅缝早闭症：①眶额前移额骨瓣交叉旋转顶骨支撑术治疗短头、尖头及塔头畸形;采用梅花形颅骨瓣治疗矢状缝早闭。②额眶成形术治疗三角头。③双侧额骨瓣旋转,额眶带前移、患侧额骨瓣前倾术治疗单侧冠状缝早闭引起的前斜头畸形。④双侧顶枕骨瓣旋转交错,梅花形骨瓣成形术治疗单侧人字缝早闭引起的后斜头畸形;⑤额面前移术治疗双侧冠状缝早闭及颅底缝早闭形成的短头畸形,及合并Apert或Crouzon综合征的颅面畸形。结果37例先天性颅缝早闭症均痊愈出院,术后随访2～3年无明显并发症,头颅外形均得到了改善。结论在治疗先天性颅缝早闭症时,采用大骨瓣的颅盖成形术仍不失为治疗颅面畸形的好方法。     

先天性颅缝早闭的颅骨成形——附6例报道

目的更好的对先天性颅缝早闭的颅骨进行颅骨成形.方法用多种颅骨瓣旋转、调换及结合梅花瓣骨成形重建颅骨外形.结果 6例患儿均获成功,外形满意.结论颅骨成形方法采用梅花瓣式的塑形法,可以在术中任意塑造形状.颅缝早闭症一定要扩大颅底才能彻底改变颅骨外形,减少以后中面部发育不良.采用钛板加钛条固定颅底和骨瓣,即坚强又牢固.     

先天性颅缝早闭的颅骨成形——附6例报道

目的　更好的对先天性颅缝早闭的颅骨进行颅骨成形。方法　用多种颅骨瓣旋转、调换及结合梅花瓣骨成形重建颅骨外形。结果　 6例患儿均获成功 ,外形满意。结论　颅骨成形方法采用梅花瓣式的塑形法 ,可以在术中任意塑造形状。颅缝早闭症一定要扩大颅底才能彻底改变颅骨外形 ,减少以后中面部发育不良。采用钛板加钛条固定颅底和骨瓣 ,即坚强又牢固。     

先天性颅缝早闭的颅骨成形—附6例报道

目的 更好的对先天性颅缝早闭的颅骨进行颅骨成形。方法 用多种颅骨瓣旋转，调换及结合梅花瓣骨成形重建颅骨外形。结果 6例患儿均获成功，外形满意，结论 颅骨成形方法采用梅花瓣式的塑形法，可以在术中任意塑造形状，颅缝早闭症一定要扩大颅底才能彻底改变颅骨外形，减少以后中面部发育不良，采用钛板加钛条固定颅度和骨瓣，即坚强又牢固。]     

数字化和三维打印导板定位技术在同期矫治颅缝早闭继发眶距增宽症中的应用

目的探讨数字化技术及三维打印导板定位技术系统应用于同期矫治先天性颅缝早闭症继发眶距增宽症中的临床效果。方法2015年6月至2019年8月中国医学科学院整形外科医院收治4例先天性单侧冠状缝早闭继发眶距增宽患儿,其中男1例,女3例,年龄3~8岁,采用额眶前移联合倒U形截骨同期矫治头颅畸形及眶距增宽症。术前通过数字化技术设计手术方案,三维打印头颅模型及手术导板,术中以数字化导板指导截骨,术后通过数字化技术评价其效果。测量患者术前、术后头颅三维数据,计算前颅不对称性指数(ACVAI)及眶内壁间距,并通过ProPlan CMF 3.0对术后头颅三维模型和术前模拟进行配准,制作颜色梯度图,判断术后颅骨瓣及眼眶位置与术前设计是否相同。结果4例均按照术前数字化设计方案顺利完成手术,术中截骨导板高度吻合,导板的放置未损伤周围组织,截骨时间缩短至1~2 h。术后无脑脊液漏、感染、颅内血肿及眼球损伤等并发症。术后随访4个月至3年,4例患儿头颅、眶外形都获得很大改善,ACVAI均降至3.5%以下(-1.5%~3.0%),术后的眶内壁间距减小至正常范围(22~28 mm)。颜色梯度图显示,术后效果与术前手术设计模拟效果高度吻合。结论数字化技术及三维打印导板定位技术系统、规范化地应用于复杂先天性颅缝早闭继发眶距增宽症的治疗中,可明显提高截骨的精准性,降低手术风险,缩短手术时间,获得更为满意的外形。     

儿童及青少年先天性头颅畸形颅骨重塑和牵张成骨后骨吸收的定量分析

目的定量分析儿童及青少年先天性头颅畸形患者经颅骨重塑术后骨吸收的情况。方法选取2014年3月至2018年12月在上海交通大学医学院附属第九人民医院整复外科进行颅骨重塑手术治疗的先天性头颅畸形(Crouzon综合征)患儿14例,平均年龄7.7岁,给予改良monobloc截骨牵引成骨和颅骨重塑术。调取患儿术后1周(t1)及术后1年(t2)的头颅CT扫描数据,以Dicom 3.0格式保存后,输入Mimics 18.0软件进行颅骨CT三维重建,在三维重建的颅骨上采用ROI(region of interest)曲线进行定量研究。于重建的颅骨上选取术区的自体骨移植部分,标记为目的选区,读取该区域骨体积,Vt1作为成骨牵引前骨体积,Vt2作为成骨牵引后骨体积,以(Vt1-Vt2)/Vt1×100%作为骨吸收率。采用配对Student’s-t检验对患儿术后1周和术后1年的骨体积进行比较。结果在14例患者中,有11例患者发生了骨吸收,术后1年的骨吸收率为3.482%。术后1年与术后1周骨组织体积比较,差异无统计学意义(t=0.851,P=0.410)。结论儿童及青少年先天性头颅畸形患儿经颅骨重塑和牵引成骨术后1年时出现骨吸收,但骨吸收程度可以接受。因此,对1岁以上的儿童及青少年先天性头颅畸形患儿进行颅骨重塑和牵引成骨的手术治疗方案是可行的。     

婴幼儿颅缝早闭的额眶畸形矫正

目的　探讨手术治疗婴幼儿颅缝早闭引起的额眶骨畸形的手术方法和手术时机。方法　采用额骨上眶骨联合截骨塑形前移的手术方法。连续收治了 11名患儿 ,2名女性 ,9名男性 ,年龄 6～ 9个月。其中 ,额缝早闭 6例 ,非综合征性单侧冠状缝早闭 2例 ,多骨缝早闭 1例 ,Apert综合征和Saethre Chotzen综合征累及冠状缝等骨缝早闭各 1例。结果　随访 2～ 11个月 ,均取得满意的矫正效果。其中 1例术后发现颅骨顶部 (非术区 )局部隆起 ,经戴头盔 3个月得以控制。未发生明显并发症。结论　额骨上眶骨联合截骨塑形前移方法能够安全、有效地矫正颅缝早闭引起的额眶发育不良。     

标准大骨瓣减压术后早期颅骨修补32例疗效分析

目的探讨标准大骨瓣减压术后颅骨缺损早期修补的效果。方法对32例标准大骨瓣减压术后颅骨缺损患者,在术后1～3个月行颅骨修补术,观察l临床效果。结果所有患者手术均顺利,伤FI愈合良好,无继发性硬膜外或硬膜下血肿发生,术后无癫痫发作。头颅CT复查示无钛网变形及移位发生,术后随访3个月,颅骨缺损综合征消失,原有神经功能障碍均有不同程度好转。结论在标准大骨瓣减压术后1～3个月．颅内压正常时修补颅骨缺损有助于脑功能恢复．使患者早日康复。     

Rotational osteotomy at the diaphysis of the radius in the treatment of congenital radioulnar synostosis

Four cases of congenital radioulnar synostosis in three patients were treated by rotational osteotomy of the distal radius. The diaphysis of the radius was osteotomized transversely, and then the forearm was supinated manually. Following surgery, the palm was immobilized in a fully supinated position by a long-arm cast. The average age at surgery was 4 years 5 months old (range 3 years 11 months to 4 years 11 months), and the average follow-up was 21 months (range 12-36 months). Bone union was obtained in all patients without any complications or correction loss. Functional improvements were achieved in all patients. This procedure is simple and safe in the treatment of congenital radioulnar synostosis.     

Mental development after modified pi procedure: dynamic cranioplasty for sagittal synostosis

A prospective developmental assessment was performed on 26 patients operated on with dynamic cranioplasty for sagittal synostosis. Because this technique entails the application of compressive force, it was of great concern to assess the effect of surgery on development and mental status. The surgical technique used was a modified pi procedure. Perioperative variables were recorded. Six patients underwent preoperative intracranial pressure (ICP) measurements. To evaluate objectively the developmental outcome, the Griffiths' Mental Development Scales was used for analysis before and after surgery. A parental questionnaire was used for subjective outcome measurement. Preoperative ICP recordings during sleep ranged from 12.8 to 22.8 mmHg (mean, 16.1 mmHg). The mean age at the time for surgery was 6.9 months (range, 4-16 months; standard deviation [SD], 2.32 months). The surgical technique included shortening of the anteroposterior diameter of the skull by a mean of 16.6 mm. The mean global development quotient (GDQ) preoperatively was 104.5 (range, 82-144; SD, 12.4) and the mean GDQ postoperatively was 101.4 (range, 62-129; SD, 13.6). Mean age at follow-up was 16.3 months (range, 9-40 months; SD, 4.04 months). There was no significant correlation between the amount of intraoperative shortening and mental development. In comparison of means, the GDQ preoperatively did not differ significantly from the GDQ postoperatively. The modified pi procedure is safe and efficient. When surgery was performed before 1 year of age, no significant (p = 0.33) effect on mental development-either detrimental or beneficial-was demonstrated.     

Sagittal synostosis: I. Preoperative morphology of the skull.

The aim of this study was to characterise the preoperative morphology of the skull in sagittal synostosis in an objective and quantified way. The shapes of the skulls of 105 patients with isolated premature synostosis of the sagittal suture (SS group) were studied and compared with those of a control group of 72 children with unilateral incomplete cleft lip (UICL). A standardised radiocephalometric technique was used to obtain the images. A modification of a method developed by Kreiborg was used to analyse the radiocephalograms, which included the digitisation of 88 landmarks in the calvaria, skull base, and orbit (42 in the lateral and 46 in the frontal projections), the production of plots of mean shape for each group, and the intergroup comparison of a series of 81 variables (linear distance between selected landmarks, and angles defined by groups of three landmarks). Data from a subgroup of 66 patients aged 5 to 8 months were further compared to age-matched normative data in terms of seven angular and linear calvarial, cranial base and orbital variables. In a comparative analysis of the mean lateral plots, the foreheads of the study group (SS) had a more pronounced anterior slope and were also more convex. The vertex area was located more anteriorly, and was less convex. The occipital curvature was more prominent. Analysis of the mean frontal plots revealed a lack in convexity and lateral projection of the upper parietal regions, as well as a lower location of the line of maximum skull width. Comparison of the mean values of an SS subgroup to age-matched normative data showed a longer (p<0.001) and narrower skull (p<0.001) and a greater interorbital distance (p<0.001). The cranial base angle, the sella to nasion, and sella to basion lengths did not differ significantly. Sagittal synostosis is characterised by an extensive deformity of the cranial vault, with an essentially normal cranial base. The widened interorbital distance is probably related to compensatory metopic hyperactivity.     

Correction of sagittal synostosis using a modified Pi method

The authors' technique for the treatment of sagittal synostosis, which involves total cranial vault reconstruction and is termed the Pi procedure, is a safe, effective method for correcting the deformities associated with sagittal synostosis. It provides an immediate correction that does not require any further manipulations to the skull, such as a molding helmet. The technique addresses all the aspects of the deformity. It increases the width as well as the central height of the skull and decreases the length of the skull to produce a rounder cranial vault.     

Endoscopic treatment of sagittal suture synostosis — a critical analysis of current management strategies

While many centers nowadays offer minimally invasive techniques for the treatment of single suture synostosis, surgical techniques and patient management vary significantly. We provide an overview of how scaphocephaly treated with endoscopic techniques is managed in the reported series and analyze the crucial steps that need to be dealt with during the management process. We performed a review of the published literature including all articles that examined sagittal-suture synostosis treated with endoscopic techniques as part of single- or multicenter studies. Fourteen studies reporting results of 885 patients were included. We identified 5 key steps in the management of patients. A total of 188 patients were female and 537 male (sex was only specified in 10 articles, for 725 included patients, respectively). Median age at surgery was between 2.6 and 3.9 months with a total range from 1.5 to 7.0 months. Preoperative diagnostics included clinical and ophthalmologic examinations as well as neuropsychological and genetic consultations if needed. In 5 publications, a CT scan was routinely performed. Several groups used anthropometric measurements, mostly the cephalic index. All groups analyzed equally recommended to perform endoscopically assisted craniosynostosis surgery with postoperative helmet therapy in children < 3 months of age, at least for non-syndromic cases. There exist significant variations in surgical techniques and patient management for children treated endoscopically for single suture sagittal synostosis. This heterogeneity constitutes a major problem in terms of comparability between different strategies.

     

Sagittal synostosis: II. Cranial morphology and growth after the modified pi-plasty

The aim of this study was to characterise the postoperative cranial growth and morphology after a modified pi-plasty for sagittal synostosis. The shape of the skull of 82 patients with isolated premature synostosis of the sagittal suture (SS group) operated on with a modified pi-plasty was studied longitudinally. Forty-five children with unilateral incomplete cleft lip (UICL), evaluated longitudinally at the ages of 2.4 and 23.2 months were used as controls. A standardised radiocephalometric technique was used for image acquisition. The radiocephalograms were analysed using a modification of a method developed by Kreiborg, which included the digitisation of 89 landmarks of the calvaria, cranial base, and orbit (43 in the lateral and 46 in the frontal projections), the production of mean shape plots for each group, and the intergroup comparison of a series of 78 variables (linear distance between selected landmarks, and angles defined by groups of three landmarks). Paired and unpaired t tests were used to assess the differences between the variables studied. These were accepted as significant for values of p<0.01 and were presented as coloured segments or areas in the respective plots. In a comparative analysis with the mean UICL lateral plots, the mean preoperative lateral plots of the study group (SS) showed that the anterior slope of the forehead was more pronounced and it was also more convex. The vertex area was located more anteriorly and was less convex. The occipital curvature was more prominent. Comparison of the mean frontal plots showed a deficiency in convexity and lateral projection of the upper parietal regions, and the line of maximum skull width was lower. The postoperative mean lateral plots of the study group showed a correction of the exaggerated anterior inclination of the forehead and a reduction of the abnormal occipital convexity. However, there was little change in the vertex region and it remained flatter than in the control group. In the mean frontal plots, the increase in convexity and in the lateral projection of the upper parietal areas led to a shape that was similar to that of the UICL group. The mean (SD) cephalic index changed from 64.9% (1.8%) to 71.4% (3.5%) (p<0.001). The longitudinal comparison between the mean postoperative plots at 3 and 5 years of age showed that there had been little change in cranial shape. In conclusion, after a modified pi-plasty for sagittal synostosis, significant objective changes in cranial shape towards normality were produced. The postoperative profile cranial shape was improved except in the vertex area, which remained flatter than normal. In the frontal projection an almost normal shape was obtained. The postoperative cranial shape obtained at 3 years of age had remained stable at the age of 5 years.     

Multiple-revolution spiral osteotomy for cranial reconstruction. Technical note

Various combinations of cranial remodeling techniques are used in an attempt to provide optimal cosmetic results and to reduce possible sequelae associated with craniosynostosis. One element of deformity that is difficult to correct directly is an overly flattened area such as that found in the parietal area in sagittal synostosis, unilaterally in lambdoid synostosis, or even in severe positional molding. The authors present a novel application for recontouring cranial bone, namely the multiple-revolution spiral osteotomy. The advantages of this technique include the avoidance of large areas of craniectomy and immediate correction of the cranial deformity. The surgical procedure, illustrative cases, early results, and apparent benefits of this technique are discussed.     

Sagittal synostosis: II. Cranial morphology and growth after the modified pi-plasty.

The aim of this study was to characterise the postoperative cranial growth and morphology after a modified pi-plasty for sagittal synostosis. The shape of the skull of 82 patients with isolated premature synostosis of the sagittal suture (SS group) operated on with a modified pi-plasty was studied longitudinally. Forty-five children with unilateral incomplete cleft lip (UICL), evaluated longitudinally at the ages of 2.4 and 23.2 months were used as controls. A standardised radiocephalometric technique was used for image acquisition. The radiocephalograms were analysed using a modification of a method developed by Kreiborg, which included the digitisation of 89 landmarks of the calvaria, cranial base, and orbit (43 in the lateral and 46 in the frontal projections), the production of mean shape plots for each group, and the intergroup comparison of a series of 78 variables (linear distance between selected landmarks, and angles defined by groups of three landmarks). Paired and unpaired t tests were used to assess the differences between the variables studied. These were accepted as significant for values of p<0.01 and were presented as coloured segments or areas in the respective plots. In a comparative analysis with the mean UICL lateral plots, the mean preoperative lateral plots of the study group (SS) showed that the anterior slope of the forehead was more pronounced and it was also more convex. The vertex area was located more anteriorly and was less convex. The occipital curvature was more prominent. Comparison of the mean frontal plots showed a deficiency in convexity and lateral projection of the upper parietal regions, and the line of maximum skull width was lower. The postoperative mean lateral plots of the study group showed a correction of the exaggerated anterior inclination of the forehead and a reduction of the abnormal occipital convexity. However, there was little change in the vertex region and it remained flatter than in the control group. In the mean frontal plots, the increase in convexity and in the lateral projection of the upper parietal areas led to a shape that was similar to that of the UICL group. The mean (SD) cephalic index changed from 64.9% (1.8%) to 71.4% (3.5%) (p<0.001). The longitudinal comparison between the mean postoperative plots at 3 and 5 years of age showed that there had been little change in cranial shape. In conclusion, after a modified pi-plasty for sagittal synostosis, significant objective changes in cranial shape towards normality were produced. The postoperative profile cranial shape was improved except in the vertex area, which remained flatter than normal. In the frontal projection an almost normal shape was obtained. The postoperative cranial shape obtained at 3 years of age had remained stable at the age of 5 years.