The rational strategies for detecting developmental dysplasia of the hip at the age of 4-6 months old infants: a prospective study

Using ultrasound in evaluation of infant's hip development can reduce surgical procedures, hospitalization and late presentation of developmental dysplasia of the hip (DDH). The increasing incidence of DDH after ultrasound examination is observed and published by many authors. In a prospective study, radiograph of every single ultrasonographic positive hip in infants older than three months, was taken and analyzed in order to see whether it affects infants splintage rate in treating DDH. In a period of 30 months, clinical and simple static ultrasonographic examinations according to Graf were performed on 1430 consecutive infant hips in patients aged between 4 and 6 months. Sonographic positive hips were radiographed and acetabular index (AI) values on simple AP radiographs were analyzed. The sonographic DDH incidence was 51.75 per 1000 hips (51.75 per thousand). After X-ray examination of all 74 ultrasonographic positive hips, only 44 remained abnormal and required treatment indicating a true DDH incidence of 30.77 per 1000 hips (30.77 per thousand). The difference in incidence per ultrasonographic and X-ray positive hips is statistically significant p < 0.01 (t = 5,536). The rational approach in detection of DDH in a child more than 3 months old is to do radiographic assessment of every sonographic positive hip.     

Incidence and Treatment of Developmental Hip Dysplasia in Mongolia: A Prospective Cohort Study

Background

In Mongolia, adequate early diagnosis and treatment of developmental hip dysplasia (DDH) have been unavailable and its incidence was unknown. We determined the incidence of ultrasonographic DDH in newborns and established adequate procedures for diagnosis and treatment of DDH at the largest maternity hospital in Ulaanbaatar, Mongolia.

Methodology/Principal Findings

During one year (Sept 2010 – Aug 2011) we assessed the hips newborns using ultrasound and Graf’s classification of DDH. 8,356 newborns were screened; median age at screening was 1 day. We identified 14,873 Type 1 (89.0%), 1715 Type 2a (10.3%), 36 Type 2c (0.2%), 70 Type D (0.4%), 14 Type 3 (0.08%), and 4 Type 4 hips (0.02%). Children with Type 1 hips (normal) were discharged. Children with Type 2a hips (physiologically immature) received follow-up ultrasounds at monthly intervals. Children with Type 2c to 4 (DDH; deformed or misaligned hip joint) hips were treated with a Tubingen hip flexion splint and also followed up. The hip abnormalities resolved to mature hips in all children who were followed up. There was no evidence for severe treatment related complications.

Conclusion/Significance

This study suggests that the incidence of DDH in Mongolian neonates is comparable to that in neonates in Europe. Early ultrasound-based assessment and splinting treatment of DDH led to mature hips in all children followed up. Procedures are feasible and will be continued.     

Problems in the Early Recognition of Congenital Hip Dislocation

Seven cases of infants whose hips were clinically normal at birth, and in whom hip dislocation was later recognized, are reported. In spite of the widespread practice of routine examination of the hips of newborn babies, infants and young children are still presenting with congenital dislocation of the hip. This is probably owing to there being two aetiological types. Every opportunity to re-examine the hips of children under 2 or even 3 years of age must be taken and a high level of suspicion maintained.     

基于三维CT的模拟手术在大龄DDH患儿外科治疗中的应用研究

目的:通过对大龄发育性髋关节脱位(developmental dysplasia of the hip,DDH)患儿进行术前模拟手术,实现术中精确截骨及旋转角度,从而达到个体化治疗,改善患儿预后的目的。方法:本研究按照术前规划方式分为两组,一组为传统手术组;另一组为模拟手术组。共20例患儿均采用骨盆三联截骨术+股骨截骨术治疗,传统手术组10例,模拟手术组10例,手术时平均年龄为11.3岁,平均随访时间24.2个月。所有患儿均于术前行骨盆三维重建CT检查,测量CE角、股骨前倾角及髋臼指数,在mimics软件中,模拟手术方案,确定术中股骨截骨需要旋转的角度及骨盆截骨的位置,术中按照模拟手术的结果进行操作。术前评价指标使用Tonnis分级,术后评价指标使用改进的Trevor评分系统。结果:模拟手术组Tonnis分级3级4髋,Tonnis分级4级8髋;传统手术组Tonnis分级3级4髋,Tonnis分级4级9髋,两组患儿术前严重程度无显著性差异。依据Trevor评分,模拟手术组8髋(67%)优秀,3髋(25%)良好,1髋(8%)一般。传统手术组5髋(38%)优秀,5髋(38%)良好,3髋(23%)一般。两组有显著性差异。并发症:术后传统手术组3例患儿有不同程度的股骨头坏死。结论:大龄DDH患儿术前模拟手术,可以达到术中精确截骨及旋转角度,可改善患儿预后,实现该类患者的个体化治疗。     

Prognostic value of refined Wiberg's angle in hip development

There are many angles measured on hip radiographs, but their prognostic value for hip development in patient with DDH is controversial. Our aim was to find out how the development of congenitally luxated hip could be predicted after reduction using Wiberg's CE angle and CE angle refined according to Ogata. 119 hips of 61 patients were retrospectively evaluated after reduction from 3 months of age until at least full osteomaturity. According to results of CE angles measurements good and poor hip development group were formed. Healthy hips of the patients with unilateral DDH were considered to be the control group. Negative linear trend of the curve connecting Wiberg's CE angle values measured within first postreductive years is a poor prognostic sign of hip development as are absolute negative values of refined CE angles. In cases where negative initial refined CE angles are combined with negative linear trend of Wiberg's CE angles prognosis is extremely poor. Both Wiberg's and Ogata's CE angle are of prognostic value regarding hip development and should be measured when evaluating AP radiographs of infant's hip.     

Development and growth of immature hips

The authors investigated the development of the bony acetabulum in type IIa+ immature hips using ultrasound follow-up evaluation of the alpha and beta angle. The study comprised 900 hips in one-month old infants. In the initial ultrasound examination the alpha angle measured less than 60 degrees and the beta angle more than 55 degrees. In the second ultrasound examination at the age of two months a discrepancy between the bony and cartilaginous acetabular component was found in 15% of the hips, which means that the type IIa+ hips were transformed into the type IIa-. The infants with type IIa- hips had more than two risk factors for developmental dysplasia of the hip. The statistical relative risk was 17. These hips were treated with the Pavlik harness during two months on average. After treatment all hips had normal values the alpha and beta angles (more than 60 degrees and less than 55 degrees respectively). This study showed that each newborn and infant with a sonographically confirmed hip development disorder has to be included in the follow-up evaluation of acetabular development, which makes it possible to choose the most adequate therapeutic and prophylactic measures.     

European Society of Biomechanics S.M. Perren Award 2018: Altered biomechanical stimulation of the developing hip joint in presence of hip dysplasia risk factors

Fetal kicking and movements generate biomechanical stimulation in the fetal skeleton, which is important for prenatal musculoskeletal development, particularly joint shape. Developmental dysplasia of the hip (DDH) is the most common joint shape abnormality at birth, with many risk factors for the condition being associated with restricted fetal movement. In this study, we investigate the biomechanics of fetal movements in such situations, namely fetal breech position, oligohydramnios and primiparity (firstborn pregnancy). We also investigate twin pregnancies, which are not at greater risk of DDH incidence, despite the more restricted intra-uterine environment. We track fetal movements for each of these situations using cine-MRI technology, quantify the kick and muscle forces, and characterise the resulting stress and strain in the hip joint, testing the hypothesis that altered biomechanical stimuli may explain the link between certain intra-uterine conditions and risk of DDH. Kick force, stress and strain were found to be significantly lower in cases of breech position and oligohydramnios. Similarly, firstborn fetuses were found to generate significantly lower kick forces than non-firstborns. Interestingly, no significant difference was observed in twins compared to singletons. This research represents the first evidence of a link between the biomechanics of fetal movements and the risk of DDH, potentially informing the development of future preventative measures and enhanced diagnosis. Our results emphasise the importance of ultrasound screening for breech position and oligohydramnios, particularly later in pregnancy, and suggest that earlier intervention to correct breech position through external cephalic version could reduce the risk of hip dysplasia.     

Pavlik吊带治疗早期发育性髋关节脱位的研究进展

发育性髋关节脱位(Developmental dysplasia of the hip,DDH)是指一系列髋关节结构异常的疾病,病变严重程度涵盖轻度髋臼发育不良到不可逆的髋关节脱位。DDH的治疗手段进展迅速,但早期闭合复位使用最多的仍是Pavlik吊带。通过超声可监测髋关节复位情况从而评估Pavlik吊带的治疗效果。同时,随着超生髋关节检查技术在我国的推广,使得早期诊断DDH成为可能,这也促进了Pavlik吊带在临床的应用。未来研究的方向在于制定基于询证医学证据的Pavlik吊带使用规范和开发出效果更好、并发症更少的新型支具。     

转化生长因子5在发育性髋关节发育不良中的作用研究进展

发育性髋关节发育不良(developmental dysplasia of the hip,DDH)是一种主要因髋臼、股骨近端和关节囊等存在结构性畸形而导致的不稳定关节病变,进而发展成为髋关节的脱位。髋关节内软骨发育不良、骨骼及肌腱的异常均可导致髋关节结构的畸形,最终造成DDH。因此,早期预防与诊断是DDH治疗的关键。研究表明,DDH具有遗传基础,其易感基因包括GDF5、HOXD9、COL2AL、PAPPA2等,其中遗传因子转化生长因子5(growth differentiation factor 5,GDF5)对软骨细胞的增殖、分化具有重要作用,是当前研究治疗DDH的热点之一。因而,了解GDF5基因对软骨发育及分化的影响,对于DDH的发病机制和治疗具有重要意义。基于此,综述了国内外近期探讨的GDF5在基因层面上对DDH的影响,以及通过关节内注射重组人GDF5等基于GDF5的DDH治疗方案,以期为DDH的临床治疗提供新的策略。     

Brief communication: developmental dysplasia of the hip in medieval London

Developmental dysplasia of the hip (DDH) is a spectrum of disease starting in childhood and in many cases persisting into adulthood. The spectrum ranges from acetabular dysplasia, through hip subluxation to dislocation. The aim of this research was to determine the prevalence and pathoanatomy of acetabular dysplasia and subluxation in excavated human skeletal remains, to complement past research on dislocation in DDH. The material under study was the medieval cemetery of St. Mary Spital in London, in use from c.1100 to 1539 AD. A series of 572 adults with both hips preserved were analyzed. Acetabular dysplasia was indicated by a shallow acetabulum with upward sloping roof. Subluxation was suggested by degenerative change along the margin of the acetabulum suggestive of labral tears, and degenerative change in the outer part of the acetabular roof suggestive of osteoarthritis. The prevalence of DDH (acetabular dysplasia, subluxation, or dislocation) was 1.7%. Because this a congenital musculoskeletal disorder of relatively high frequency, with significant variation in prevalence between populations around the world, it is a topic that warrants targeted research from physical anthropologists studying past populations.     

Ultrasonography in screening for developmental dysplasia of the hip in newborns: systematic review

Objective To assess the accuracy and effectiveness of the screening of all newborn infants for developmental dysplasia of the hip (DDH) using ultrasound imaging, as is standard practice in some European countries but not in the United Kingdom, the United States, or Scandinavia.Design Systematic review.Data sources Twenty three medical, economic, and grey literature databases (to March 2004), with no limitations of design or language; some references were provided by experts.Selection of studies Only diagnostic accuracy studies and comparative studies conducted in an unselected newborn population were eligible for the review. Two reviewers independently selected the studies and performed the quality assessment.Results The review identified one diagnostic accuracy study, and this was of limited quality. In this study the reference standard was treatment up to age of 8 months or an abnormal ultrasound finding at age 8 months. Ultrasound screening had a sensitivity of 88.5% (95% confidence interval 84.1% to 92.1%), specificity of 96.7% (96.4% to 97.4%), a positive predictive value of 61.6% and a negative predictive value of 99.4%. Ten studies evaluated the impact of ultrasound in screening, but these too had various methodological weaknesses, limiting the reliability of their findings. Compared with clinical screening, general ultrasound screening in newborns may increase overall treatment rates, but ultrasound screening seems to be associated with shorter and less intrusive treatment.Conclusions Clear evidence is lacking either for or against general ultrasound screening of newborn infants for DDH. Studies that investigate the natural course of the disorder, the optimal treatment for DDH, and the best strategy for ultrasound screening are needed.     

Association analysis between four vitamin D receptor gene polymorphisms and developmental dysplasia of the hip

Developmental dysplasia of the hip (DDH) is a congenital condition characterized by abnormality in acetabulum size and/or shape. The incidence rate of DDH differs between different populations with risk factors including positive family history, breech presentation, sex, firstborn status, side of the hip, mode of delivery and oligohydramnios. It is recognized that DDH has a genetic component that exhibit autosomal dominant patterns. Many candidate genes have been studied and found to be associated with the disease; most of them are normally involved in cartilage development and joint metabolism. In this study, the association of four single-nucleotide polymorphisms (SNPs) (rs731236, rs1544410, rs7975232 and rs2228570) in the vitamin D receptor (VDR) gene was studied by a case–control analysis. The study sample involves 50 cases with confirmed DDH presentation and 50 nonDDH controls. SNPs were genotyped using conventional polymerase chain reaction (PCR) and restriction fragment-length polymorphism (RFLP) techniques. Genotype and allele frequencies were analysed using SPSS software. No significant associations were found between the VDR polymorphisms analysed and DDH. Further work need to be performed using genomewide analysis to elucidate the genetic basis of DDH.     

Difficulties in diagnosing and managing congenital dislocation of the hip.

In a 10-year retrospective study of 25 921 consecutive deliveries in a neonatal unit in Newcastle upon Tyne 271 cases of congenital dislocation of the hips were identified. Of these, the outcome was unsatisfactory in 12: four diagnoses were missed at birth and eight children required further surgical treatment. Radiological abnormalities were detected in a further five children at long-term follow-up examination. From the results of this study and other published series it was concluded that lack of attention to detail was the main cause of inadequate diagnosis and management of congenital dislocation of the hips. More detailed instruction of junior staff, confirmation of the diagnosis by senior staff, the use of a non-removable splint early in treatment, and thorough follow-up by senior staff are all important.     

Whole genome sequencing of pairwise human subjects reveals DNA mutations specific to developmental dysplasia of the hip

Developmental dysplasia of the hip (DDH) is a common congenital malformation characterized by mismatch in shape between the femoral head and acetabulum, and leads to hip dysplasia. To date, the pathogenesis of DDH is poorly understood and may involve multiple factors, including genetic predisposition. However, comprehensive genetic analysis has not been applied to investigate a genetic component of DDH. In the present study, 10 pairs of healthy fathers and DDH daughters were enrolled to identify genetic hallmarks of DDH using high throughput whole genome sequencing. The DDH-specific DNA mutations were found in each patient. Overall 1344 genes contained DDH-specific mutations. Functional enrichment analysis showed that these genes played important roles in the cytoskeleton, microtubule cytoskeleton, sarcoplasm and microtubule associated complex. These functions affected osteoblast and osteoclast development. Therefore, we proposed that the DDH-specific mutations might affect bone development, and caused DDH. Our pairwise high throughput sequencing results comprehensively delineated genetic hallmarks of DDH. Further research into the biological impact of these mutations may inform the development of DDH diagnostic tools and allow neonatal gene screening.     

人工髋关节置换术在320例股骨头缺血性坏死中的治疗体会

目的：探讨人工髋关节置换术在治疗股骨头缺血性坏死（ANFH）中的临床疗效。方法：选择2007年2月-2011年2月我院收治的320例（340髋）股骨头缺血性坏死患者,均采用人工髋关节置换术对患者进行治疗,其中160例（172髋）患者应用骨水泥型假体进行治疗,另外160例（168）患者采用非骨水泥型假体进行治疗。采用Harris评分对患者手术前后的髋关节功能情况进行评价,并比较骨水泥治疗组和非骨水泥治疗组的临床疗效。结果：患者均获得随访,随访时间为3～18个月。全部患者手术后的Harris评分明显高于手术前,差异有统计学意义（P〈0．05）。骨水泥治疗组和非骨水泥治疗组在术后出血量、术后Harris评分及住院时间方面的差异无统计学意义（P〉0．05）,但非骨水泥治疗组的并发症发生率明显低于骨水泥治疗组（P〈0．05）。结论：采用人工髋关节置换术治疗ANFH疗效显著,能明显改善患者的生活质量,骨水泥型假体与非骨水泥型假体的治疗效果相当,应根据患者的具体情况进行合理的选择。     

Hip contact stress during normal and staircase walking: the influence of acetabular anteversion angle and lateral coverage of the acetabulum

Hip contact stress is considered to be an important biomechanical factor related to development of coxarthrosis. The effect of the lateral coverage of the acetabulum on the hip contact stress has been demonstrated in several studies of hip dysplasia, whereas the effect of the anterior anteversion remains unclear. Therefore, the joint hip contact stress during normal level walking and staircase walking, in normal and dysplastic hips, for small and large acetabular anteversion angle was computed. For small acetabular anteversion angle, the hip contact stress is slightly increased (less than 15%) in staircase walking when compared with normal walking. In hips with large angle of acetabular anteversion, walking downstairs significantly increases the maximal peak contact stress (70% in normal hips and 115% in dysplastic hips) whereas walking upstairs decreases the peak contact stress (4% in normal hips and 34% in dysplastic hips) in comparison to normal walking. Based on the presented results, we suggest that the acetabular anteversion should be considered in biomechanical evaluation of the hips, especially when the lateral coverage of the acetabulum is small.     

人工髋关节置换术在320 例股骨头缺血性坏死中的治疗体会

目的：探讨人工髋关节置换术在治疗股骨头缺血性坏死（ANFH）中的临床疗效。方法：选择2007 年2 月-2011 年2 月我院收 治的320 例（340 髋）股骨头缺血性坏死患者,均采用人工髋关节置换术对患者进行治疗,其中160 例（172 髋）患者应用骨水泥型 假体进行治疗,另外160 例（168）患者采用非骨水泥型假体进行治疗。采用Harris评分对患者手术前后的髋关节功能情况进行评 价,并比较骨水泥治疗组和非骨水泥治疗组的临床疗效。结果：患者均获得随访,随访时间为3～18 个月。全部患者手术后的 Harris评分明显高于手术前,差异有统计学意义（P<0.05）。骨水泥治疗组和非骨水泥治疗组在术后出血量、术后Harris 评分及住 院时间方面的差异无统计学意义（P>0.05）,但非骨水泥治疗组的并发症发生率明显低于骨水泥治疗组（P<0.05）。结论：采用人工 髋关节置换术治疗ANFH疗效显著,能明显改善患者的生活质量,骨水泥型假体与非骨水泥型假体的治疗效果相当,应根据患者 的具体情况进行合理的选择。     

Association of the D repeat polymorphism in the ASPN gene with developmental dysplasia of the hip: a case-control study in Han Chinese

Introduction

Developmental dysplasia of the hip (DDH) is a common skeletal disease, which is characterized by abnormal seating of the femoral head in the acetabulum. Genetic factors play a considerable role in the etiology of DDH. Asporin (ASPN) is an ECM protein which can bind to TGF-β1 and sequentially inhibit TGF-β/Smad signaling. A functional aspartic acid (D) repeat polymorphism of ASPN was first described as an osteoarthritis-associated polymorphism. As TGF-β is well known as an important regulator in the development of skeletal components, ASPN may also be involved in the etiology of DDH. Our objective is to evaluate whether the D repeat polymorphism of ASPN is associated with DDH in Han Chinese.

Methods

The D repeat polymorphism was genotyped in 370 DDH patients and 445 control subjects, and the allelic association of the D repeat was examined.

Results

From D11 to D18, eight alleles were identified. D13 allele is the most common allele both in control and DDH groups, the frequencies are 67.3% and 58.1% respectively. In the DDH group, a significantly higher frequency of the D14 allele and significantly lower frequency of D13 was observed. The association of D14 and D13 was found in both females and males after stratification by gender. There was no significant difference in any other alleles we examined.

Conclusions

Our results show an obvious association between the D repeat polymorphism of ASPN and DDH. It indicates that ASPN is an important regulator in the etiology of DDH.     

骨性关节炎生物学标志物研究进展

骨性关节炎(oseteoarthritis,OA)是一种随着年龄增长发病率明显升高的退行性变,常累及脊柱、髋、膝等人体负重关节,以关节缓慢发展的疼痛、肿胀,伴功能障碍为临床表现,主要有滑膜增生、软骨破坏、软骨下骨骨化及骨赘形成等一系列病理表现。OA对人类的健康和生活质量影响很大,随着老龄化社会的到来,本病的发病率日趋升高,其研究已成为医学领域中的重要课题。目前,OA的早期诊断、病变监测和有效防治仍是骨科领域亟待解决的疑难问题。随着分子生物学的发展和研究手段的提高,许多研究者都在试图寻找用于临床评价OA的生物学标志物。本文将就OA研究中所使用的主要标志物进行综述,为深入研究OA提供方便。     

Development of acetabulum after closed reduction in developmental hip dysplasia

The radiographic study of acetabulum development after closed reduction of 87 congenitally dislocated hips in 64 patients who were treated between 1980 and 1990 was conducted retrospectively. An average patient's age at the moment of closed hip reduction was 16 months (range between 6 to 24 months). On the average, patients were 16 months old (range between 6 to 24 months) at the moment of closed hip reduction. Development of bony acetabulum was followed for 8 years by measuring the AC angle. According to the age at the moment of closed reduction patients were sorted into three groups, the first group from 6 months to 12 months old (39 hips), the second group from 12 to 24 months old (28 hips) and the third group from 24 to 36 months old (20 hips). At the beginning of the treatment mean values of the AC angle in the first group were 37.2 degrees +/- 3.31 degrees; in the second group 39.7 degrees +/- 3.2 degrees; and in the third group 43.8 degrees +/- 2.7 degrees. The final values of the AC angle in the first group were 14.7 degrees +/- 3.21 degrees, in the second group 21 degrees +/- 3.02 degrees; and in the third group 24 degrees +/- 3.76 degrees. Normally and mildly dysplastic hips were achieved in 80.5% treated hips, while in 19.5% treated hips medium and serious dysplasia retarded. The acetabulum development in dysplastic hip after retained stable concentric reduction was the same as in the normal hip. The operative reconstruction of acetabulum should not be done before the third year of life.