Ultrasound hip joint screening in newborn infants. Correlation of anamnestic risk factors and hip dysplasia]

BACKGROUND: Congenital dysplasia of the hip (CDH) is the most frequent inborn deformity of the locomotor apparatus. Hereditary, pelvic respectively breech presentation or abdominal delivery, premature as well as post-term birth and twin pregnancy are considered to be anamnestic risk factors for congenital dysplasia of the hip. The results of ultrasound hip screening from July 87 until December 94 are presented with special regard to the correlation of these risk factors and the occurrence of pathologic hips. PATIENTS: 19 different orthopaedic surgeons examined the hips of 3739 newborns (female: 1837-49.1%; male: 1902-50.9%) by ultrasound (screening). 96% of the examinations were performed within a period of 5 days after birth, in a few cases the babies were up to 19 days old. METHOD: The ultrasound examinations, the assessment of the echograms and classification into types of hip were performed according to Graf's technique. Two types of ultrasonographs were used: SL-1, Siemens--5 MHz scanner; LSC 7500, Picker--7.5 MHz scanner). All investigations were assessed retrospectively over the period of time with the help of documentation forms (data of newborn baby, case history, clinical and sonographical findings, kind of therapy and procedure) and statistically checked (program SPSS 7.5, Chi-Quadrate-Test, logistic regression). RESULTS: In 239 children (6.4%) we found hips required therapy respectively control investigations (type IIa, alpha < 55 degrees or worse; Graf's classification). For the entire group we achieved the following types of hips (right/left side): Ia--214 (5.7%)/224 (6.0%); Ib--2069 (55.3%)/2008 (53.7%); IIa (> or = 55 degrees)--1318 (35.3%)/1322 (35.4%); IIa (< 55 degrees)--65 (1.7%)/74 (2.0%); IIc--45 (1.2%)/71 (1.9%); D--18 (0.5%)/30 (0.8%); IIIa--8 (0.2%)/7 (0.2%); IIIb--1 (< 0.1%)/2 (0.1%); IV--1 (< 0.1%)/1 (< 0.1%). With regard to the risk factors the distribution was as follows: hereditary--302 babies (8.1%), pelvic respectively breech presentation--149 (4%), abdominal delivery--359 (6.5%), premature birth--188 (5.0%), post-term birth--164 (4.4%), twin pregnancy--73 (2%). CONCLUSIONS: In newborn babies with cases of hip dysplasia in their family (heredity) and pelvic respectively breech presentation at birth we found a significant higher rate (p < 0.05) of hips required therapy respectively control investigations (type IIa, alpha < 55 degrees or worse; Graf's classification). Also, for girls and the left hip a significant higher rate was achieved. A correlation of the other mentioned risk factors abdominal delivery, premature and post-term birth as well as twin pregnancy was not evident.     

Is hip sonography in premature infants necessary as a screening procedure?

Screening sonography of the hip in 132 premature infants indicates that prematurity is not a risk for congenital dysplasia/dislocation of the hip. However, for reasons explained in the text, screening should be carried out before discharge from hospital. Follow-up examinations are necessary only for infants with known risk factors for dysplasia/dislocation of the hip.     

Ultrasonographic evaluation of breech presentation as a risk factor for hip dysplasia

In order to gain more information of breech position as a risk factor for congenital hip dysplasia or dislocation, the hips of 408 newborns delivered in the breech position were examined by ultrasound. Clinical examination was performed by both experienced paediatricians and orthopaedic surgeons. The infants were re-examined by ultrasound at 2–3 months of age. Twenty-five newborns (6.1%) had neonatal hip instability. Breech presentation as a risk factor was confirmed, with first borns, breech position with extended knees, and high birthweight as special high-risk groups. Ultrasound showed subluxation in most of the unstable hips. The main benefit of using ultrasound was that direct visualization permitted more reliable evaluation, especially when the clinical findings were uncertain. Normal ultrasound findings in false positive and uncertain Ortolani tests reduced the frequency of unnecessary treatment. Because ultrasound was used in follow-up, the need of radiography was reduced. There were no late-detected cases of hip dysplasia or dislocation, indicating that routine follow-up is not necessary in breech infants with normal hips at birth, provided that the neonatal screening is optimal.     

Auditing hip ultrasound screening of infants at increased risk of developmental dysplasia of the hip.

BACKGROUND: Clinical examination, while useful, has been shown to be insufficient as the sole screening method in infants. Ultrasound examination at 8 weeks in high risk infants is an integral part of the screening process in some units. AIMS: To show the efficiency of hip sonography in detection of developmental dysplasia of the hips in those without clinically dislocated hips. METHODS: All infants born at the National Maternity Hospital between January 1994 and December 2001 were included. All those with clinically dislocated hips were treated by a Pavlik harness and referred for follow up to a paediatric orthopaedic surgeon. An 8 week hip ultrasound scan was performed for those infants with stable hips on examination but who met the following criteria: (1) a first degree relative with congenital dislocation of hips; (2) breech presentation at birth; and (3) a persistent "click" at birth in an otherwise stable hip. RESULTS: During the period of study a total of 52 893 infants were born in the National Maternity Hospital. Based on the criteria above, 5485 hip ultrasound scans were performed. Of those scanned, 18 (0.33%) were found to have dislocated hips and 153 (2.78%) to have dysplasic hips. The 18 infants with dislocation were treated with Pavlik harness; the remaining 153 were followed up by serial ultrasound examinations but did not require active intervention. CONCLUSIONS: Among the population of infants at increased risk of developmental dysplasia of the hip, the hip screening programme identified 18 cases among 5485 infants; a rate of 3.2 per 1000. Hip sonography is therefore worthwhile.     

Fr��herkennung der H��ftdysplasie

Background

Ultrasound screening is more sensitive for developmental dysplasia of the hip than clinical examination, but we do not know which form of ultrasound screening is the best.

Patients and methods

A retrospective analysis was performed of ultrasonographic findings of an universal ultrasound screening in the first week of life between January 2006 and June 2009 to calculate the sensitivity and the specificity of the risk factors gathered by the German two-part general ultrasound screening. Ultrasound examination and classification was done according to the method of Graf.

Results

During the period of study a total of 5,100 hips were screened. Graf??s type I was seen in 4,290 hips (despite positive risk factors in 881 hips), corresponding to a prevalence of 84.1%. Graf??s type IIa was seen in 736 hips (despite positive risk factors in 221 hips), corresponding to a prevalence of 14.4%. Graf??s type IIc stable/instable was seen in 16 hips (despite a lack of risk factors in 13 hips), corresponding to a prevalence of 0.3%. Graf??s type D was found in 37 hips, 18 of which exhibited no risk factors, corresponding to a prevalence of 0.7%. Graf??s type III was diagnosed in 20 hips (despite a lack of risk factors in 8 hips, corresponding to a prevalence of 0.4%). One hip without any risk factors was screened as Graf??s type IV corresponding to a prevalence of 0.02%. Thus, the specificity of the risk factors gathered by the two-part screening for developmental dysplasia of the hip in Germany is 78.1% and the sensitivity 48.6%.

Conclusion

In 78.1% it is possible to detect a healthy hip as such in the third check-up for children between the fourth and sixth week of life with the German two-part ultrasound screening programme. However, only 48.6% of dysplastic or dislocated hips can be detected in the first week of life with this screening programme. Thus, less than 50% of dysplastic or dislocated hips will be treated as early as possible. Thus, every newborn should have an ultrasound screening of the hip during the first week of life independently of symptoms and history.     

Perinatal hip assessment in very low birth weight infants

Fifty healthy low birth weight infants (<1500 g) without risk factors for congenital hip dysplasia (CHD) were examined by ultrasonography (100 hips) at 40 weeks corrected age and controlled at 3 months postnatal age. Sonographic assessment was performed using the classification of Graf. A physiological delay of hip maturation was observed in 10% of the patients (type IIa). At follow-up all of them had normal hip morphology without any pathological finding. Our results suggest that prematurity alone should not be considered a risk factor for CHD.     

Developmental dysplasia of the hip: Controversies and current concepts

Developmental dysplasia of the hip is an important but poorly understood disorder as evidenced by the vast amount of literature published to date on this topic. The precise definition of hip dysplasia is controversial and it encompasses a spectrum of abnormalities, rather than a single condition, which at one end overlap with normal hip maturation. We review the risk factors, clinical examination and radiological imaging of the hip in the infant and young child, the controversies of ultrasound screening for hip dysplasia and the current management, both operative and non‐operative, of this condition according to patient age.     

Genetic aspects of congenital cerebellar ataxia

Congenital cerebellar ataxia comprises a heterogeneous group of birth defects involving brain stem and cerebellum, characterised by ataxia, spasticity, choreoathetoid movements and mental retardation. Three children with congenital cerebellar malformations are described. The presenting features were benign enlargement of head, myoclonic jerks, episodes of hypernoia, nystagmus, ataxia, motor delay and mental retardation. Computerised tomography revealed Dandy-Walker cyst in one case and in two cases cerebellar vermis agenesis was associated with a variable degree of brain stem involvement. Retinal dysplasia and post-axial polydactyly were associated abnormalities in one case as reported in Joubert's syndrome, an autosomal recessive type of familial cerebellar vermis dysgenesis. Genetic factors in the pathogenesis of congenital cerebellar ataxia are reviewed. The relevance of computerised tomography, electroretinography and prenatal diagnosis by fetal ultrasound is discussed. Genetic counselling in congenital cerebellar ataxia presenting in the first year of life is important because of the high risk of recurrence.     

围孕期营养因素与先天性心脏病发病的关系

先天性心脏病是人体在胚胎发育时期受各种因素影响导致心脏及血管发育异常的先天性畸形.先天性心脏病是人类最常见的先天畸形,是导致婴儿发病和病死的重要原因.即使可以经过外科手术矫治畸形,先天性心脏病患儿也要面临各种手术并发症甚至疾病复发的风险,给社会带来巨大的经济压力.目前普遍认为先天性心脏病的发生受遗传因素和环境因素的共同影响.近年来围孕期营养因素与先天性心脏病的发病关系成为研究热点.研究结果显示,叶酸及其他围孕期营养素与先天性心脏病的发生有密切关系,围孕期母亲适当补充这些营养素可降低子代先天性心脏病的发生风险.该文就叶酸、维生素A、锌、维生素B、维生素E围孕期关键营养因素与先天性心脏病的关系,从病因、发病机制及预防方面进行综述,为疾病的预防提供基础.     

The relationship between alpha angle and resistive index of the femoral epiphysis in the normal and abnormal infant hip

Background The arterial vascularity of the hip has been investigated in normal infants using duplex Doppler sonography. This study addressed the differences in hip vascularity in infants with respect to gender and acetabular morphology. Objective To determine whether there is a relationship between the resistive index of the vessels of the femoral chondroepiphysis and the alpha angle in normal infant hips and in those with developmental dysplasia of the hip.Materials and methods We studied 76 hips (38 patients) with gray-scale and power Doppler US. The patients were referred because of a possible abnormal clinical hip examination or had risk factors for developmental dysplasia of the hip. The infants ranged in age from 1 day to 6 weeks. There were 13 boys and 25 girls. Gray-scale images were initially performed in the coronal and transverse planes to evaluate acetabular morphology, alpha angle and position of the femoral chondroepiphysis relative to the acetabulum. The hips were then examined with power Doppler US, in both sagittal and transverse planes, to identify arterial vessels within the femoral epiphysis. Resistive indices were then recorded from the spectral analysis in each vessel identified. Each examination was performed by one of five pediatric radiologists. Mixed model regression was used to assess the relationship between resistive index and alpha angle, age and gender. Results Of the 76 hips, 34 had an alpha angle of 60° or greater and were classified as normal, 26 had an alpha angle between 50° and 59° and were classified as immature, and 13 had an alpha angle of less than 50° and were either subluxed or dislocated at the time of examination. At least two vessels were documented in each femoral epiphysis except in three hips, in which no vessels could be documented because of technical factors. There was a statistically significant linear relationship between the alpha angle and resistive index, such that the resistive index tended to rise with increasing alpha angle (P=0.0022). In addition, female infants had a significantly higher average resistive index than the average resistive index in male infants with the same alpha angle (P=0.0005). Conclusion There is a direct linear relationship between alpha angle and resistive index in the infant hip. Female infants have a higher average resistive index than male infants. We believe that these results might serve as a model for predicting an infant hip at risk of ischemia. In addition, the fact that lower resistive indices of the femoral epiphysis are associated with acetabular dysplasia might help explain the documented low incidence of avascular necrosis in untreated hip dysplasia.This paper was presented at the 2005 SPR meetings in New Orleans.     

发育性髋关节发育不良发病危险因素的研究进展

发育性髋关节发育不良(developmental dysplasia of the hip,DDH)是小儿骨科常见的下肢发育畸形,严重危害患者及其家长身心健康。影响DDH发病的因素可大致分为两部分,即遗传因素和环境因素。随着20世纪80年代高检出率髋关节超声的应用及国家二胎政策的开放,可预见未来一段时期内DDH患者数量将会增加。早诊断、早治疗是该病国内外公认的诊治原则,0~6月龄更是其治疗的黄金时期。但目前其发病的确切病因、发病机制尚不完全清楚,因此对婴儿进行早期筛查和及时干预,对早期诊治意义重大。笔者通过简要综述发育性髋关节发育不良发病危险因素的研究进展,以期为DDH早期筛查和诊断提供参考。     

Echographic diagnosis of hip dysplasia in neonates. Reflections on two years' work at our ultrasonography center

The paper evaluates the role of ultrasound examinations of the hip joint in neonates by comparing the results of a one-year screening study (non-selected group) with the findings in a selected group of neonates with a high risk of hip dysplasia. The introduction to the paper contains a general discussion of the clinical aspects of hip dysplasia and describes the main anatomical findings using echography.     

发育性髋关节脱位发病相关因素的研究进展

发育性髋关节脱位(developmental dislocation of the hip,DDH)是小儿骨科常见的疾病、严重危害儿童健康.髋关节发育不良包括髋臼发育不良、髋关节半脱位及全脱位等病理类型.导致该病发病的常见因素包括:阳性家族史、性别、臀位生产、不正确的襁褓方式和母体激素水平等.但是具体病因尚不明确.本文从DDH的发病率、DDH的危险因素、激素对DDH的影响、DDH的易感基因4个方面综述了DDH的研究现状,从而进一步探讨DDH的发病因素.     

新生儿髋关节筛查资料分析

目的 探讨超声及临床髋关节检查在新生儿发育性髋关节发育不良（DDH）早期筛查中的意义.方法 采用前瞻性的方法,分两阶段对我院2011年8月1日至2013年3月29日出生的新生儿分别进行髋关节临床检查和超声检查,并对筛查结果进行分析.第一阶段为2011年8月1日至2013年1月29日,筛查出生3 ～ 10天的新生儿,了解我院新生儿DDH的患病率、DDH发生的高危因素,以及髋关节超声筛查和临床物理检查两者之间的吻合度等.第二阶段为2013年1月30日至2013年3月29日,对初诊与复诊的一致性及灵敏度和特异度进行调查.结果 第一阶段共筛查5193例新生儿,临床髋关节检查阳性616例（11.86％）,超声检查阳性556例（10.71％）.男、女超声阳性率分别为6.41％和15.78％.臀位及羊水量少的新生儿超声检查阳性率分别为10.55％和13.00％.男、女左、右髋超声分度比较和男、女左髋、右髋、双髋超声检查比较显示,女婴、臀位、羊水量少、右髋发生DDH的风险高,差异有统计学意义（P＜0.05）.第二阶段共筛选出符合超声初查和复查双条件的新生儿108例,初诊与复诊结果差异无统计学意义（P＞0.05）.ROC曲线下面积为0.675（95％ CI：0.183～1.000）.阳性预测值5.88％,阴性预测值98.90％.灵敏度及特异度的95％可信区间分别为50.00％ （95％ CI：1.26％ ～ 98.70％）,84.90％（95％ CI：76.60％ ～91.10％）.结论 超声进行新生儿髋关节DDH检查排除性诊断的意义大.运用髋关节临床及超声检查筛查新生儿DDH简便、安全,可早期发现可疑及异常病例,有利于门诊随访和早期干预.     

Evaluation of risk factors in developmental dysplasia of the hip: results of infantile hip ultrasonography

In this study, risk factors of developmental dysplasia of the hip (DDH) were evaluated. History, clinical examination and risk factors for DDH of the babies were recorded. The hips were evaluated with ultrasonography. Infantile hip ultrasonography is one of the best methods for screening of DDH. Ultrasonography is easy, repeatable and provides visualization of the cartilage part of the hip joint. Graf's method of infantile hip ultrasonography was used to evaluate the hip in this study. Both hips of 371 babies and 32 unilateral hips of 32 babies were included in the study. In 403 babies, 14 (3.4%) had DDH. There were 5 type IIB, 7 type IIC, 1 type D, and 1 type IV hips. Physiological immaturity was present in 81 hips (19% of babies). According to risk factor analysis, the only risk factor in unilateral analysis was presence of oligohydramnios (odd ratio-OR: 11.8, confidence interval-CI: 2.7-52.7). In correlation analysis, there was a correlation between female gender and swaddling. There was overall increase in DDH in female babies who were swaddled compared to those who were not. The results of this study showed that the most important risk factor was oligohydramnios for DDH. Swaddling and female gender increased the risk of the disease, but further studies in larger series are necessary for the confirmation of these results.     

Performance, treatment pathways, and effects of alternative policy options for screening for developmental dysplasia of the hip in the United Kingdom.

AIMS: To compare, using a decision model, performance, treatment pathways and effects of different newborn screening strategies for developmental hip dysplasia with no screening. METHODS: Detection rate, radiological absence of subluxation at skeletal maturity and avascular necrosis of the femoral head, as favourable and unfavourable treatment outcomes respectively, were compared for the following strategies: clinical screening alone using the Ortolani and Barlow tests; the addition of static and dynamic ultrasound examination of the hips of all infants (universal ultrasound) or restricted to infants with defined risk factors (selective ultrasound); "no screening" (that is, clinical diagnosis only). RESULTS: Universal or selective ultrasound detects more more affected children (76% and 60% respectively) than clinical screening alone (35%), results in a higher proportion of affected children with favourable treatment outcomes (92% and 88% respectively) than clinical screening alone (78%) or no screening (75%), and the highest proportion of these achieved without recourse to surgery (64% and 79% respectively) compared with clinical screening alone (18%). However, ultrasound based strategies are also associated with the highest number of unfavourable treatment outcomes arising in unaffected children treated following a false positive screening result. The detection rate of clinical screening alone becomes similar to that reported for universal ultrasound when based on studies using experienced examiners (80%) rather than junior medical staff (35%). CONCLUSION: From the largely observational data available, ultrasound based screening strategies appear to be most sensitive and effective but are associated with the greatest risk of potential adverse iatrogenic effects arising in unaffected children.     

Development of late congenital hip dysplasia: Significance of ultrasound screening

Ultrasound for detecting congenital hip dysplasia was performed in 5970 infants, 2121 of whom were reinvestigated. Of those reinvestigated 726 belonged to the screened group not at risk examined as newborns, 70 were infants at risk first investigated as newborns and 363 were infants at risk first examined after the newborn period; the remainder were infants not at risk first investigated after the newborn period. Pathological findings were obtained in 7.7% of the entire group, in 3.9% of children at no risk, and in 5% of patients at risk. Deterioration of Graf's classification of types was seen in 3.2%. 13 children, 3 of the screened group and 10 of the nonscreened group developed hip dysplasia. None of them would have been missed since they were borderline type IIA on at least one side, which warranted mandatory follow-up.     

Performance,treatment pathways,and effects of alternative policy options for screening for developmental dysplasia of the hip in the United Kingdom

Aims: To compare, using a decision model, performance, treatment pathways and effects of different newborn screening strategies for developmental hip dysplasia with no screening. Methods: Detection rate, radiological absence of subluxation at skeletal maturity and avascular necrosis of the femoral head, as favourable and unfavourable treatment outcomes respectively, were compared for the following strategies: clinical screening alone using the Ortolani and Barlow tests; the addition of static and dynamic ultrasound examination of the hips of all infants (universal ultrasound) or restricted to infants with defined risk factors (selective ultrasound); "no screening" (that is, clinical diagnosis only). Results: Universal or selective ultrasound detects more more affected children (76% and 60% respectively) than clinical screening alone (35%), results in a higher proportion of affected children with favourable treatment outcomes (92% and 88% respectively) than clinical screening alone (78%) or no screening (75%), and the highest proportion of these achieved without recourse to surgery (64% and 79% respectively) compared with clinical screening alone (18%). However, ultrasound based strategies are also associated with the highest number of unfavourable treatment outcomes arising in unaffected children treated following a false positive screening result. The detection rate of clinical screening alone becomes similar to that reported for universal ultrasound when based on studies using experienced examiners (80%) rather than junior medical staff (35%). Conclusion: From the largely observational data available, ultrasound based screening strategies appear to be most sensitive and effective but are associated with the greatest risk of potential adverse iatrogenic effects arising in unaffected children.     

Riesgo de displasia del desarrollo de la cadera en pacientes sometidos a versión cefálica externa

Introduction

Developmental dysplasia of the hip (DDH) refers to the spectrum of abnormalities of maturation and development of the hip. Breech presentation is associated with DDH. This risk factor can be modified by external cephalic version (ECV). The aim of this study is to evaluate the incidence of DDH in patients who successfully underwent ECV, as well as to evaluate need for these children (breech for a period during gestation) to be included in the DDH screening protocol.

The congenital dislocation of the hip joint in ultrasound examination--frequency, diagnosis and treatment

BACKGROUND: A real congenital dislocation of the hip joint is very rare. Because of the severeness of the disease an immediate diagnosis and begin of treatment is required. PATIENTS: Between 1984 and 1995 clinical and sonographic screening examinations at 4177 newborns were performed at our hospital. METHODS: We analyzed our patients retrospectively and found out all cases of congenital dislocation of the hip joint. All these cases were classified and the results of treatment were determined. The clinical success of our strategy of treatment was described. RESULTS: Among the 4177 observed newborns 39 cases of congenital dislocation of the hip joint in 27 children were found. We diagnosed the hip type IIIa, IIIb and IV according to Graf in 28.2 and 9 cases respectively. After 12 month a complete healing rate of 95 percent was exhibited with the functional management strategy. Five percent of the affected joints showed a residual dysplasia. The rate of residual dysplasias was 2.5%. CONCLUSIONS: The good prognosis after our early treatment strategy severe congenital dislocation of the hip joint underlines the use of earliest therapy together with a hip screening program.