Needs for special-care beds for the newborn in the Witwatersrand area

The requirements for different levels of neonatal care in the Witwatersrand area were estimated from a review of neonatal unit records of all infants born at Johannesburg Hospital during 1983 and 1984. When extrapolating these figures to the greater population of the Witwatersrand and referral areas, adjustments were made for the increased number of low-birth-weight and complicated deliveries at Johannesburg Hospital. Given the low-birth-weight rate of 8% for this population, it was calculated that 3.3 intermediate-care beds and 1.2 intensive-care beds were justified per 1,000 annual live births. A total of 25 beds for mechanical ventilation of neonates were required over this study period, approximately double the number available. Facilities for other population groups, who have higher rates for low birth weight, were even less adequate. For the country as a whole it is recognised that postneonatal mortality is a greater problem amenable to less costly intervention than neonatal mortality; nevertheless, existing facilities for neonatal care should be used more efficiently, and a co-ordinated regional service for all population groups in the area should be established.     

Late diagnosed congenital dislocation of the hip

During the years 1975 to 1979, 82 574 children from five counties in southeast Norway were live-born, 197 of whom were treated for late detected CDH. for an incidence of 2.4 per 1 000 live births, i.e., 0.4 promille dislocation. 0.6 promille subluxation, and 1.4 promille dysplasia of the acetabulum without dislocation. All the hips had been found stable at birth. Eighty-six percent were girls, and the left hip was affected in 48 percent, the right hip in 31 perent, and both hips in 21 percent. Only 6.5 percent were delivered in the breech position. This and the stability found neonatally may indicate an etiologic difference between neonatally and late diagnosed CDH.     

Late diagnosed congenital dislocation of the hip

During the years 1975 to 1979, 82,574 children from five counties in southeast Norway were live-born, 197 of whom were treated for late detected CDH, for an incidence of 2.4 per 1,000 live births, i.e., 0.4 promille dislocation, 0.6 promille subluxation, and 1.4 promille dysplasia of the acetabulum without dislocation. All the hips had been found stable at birth. Eighty-six percent were girls, and the left hip was affected in 48 percent, the right hip in 31 perent, and both hips in 21 percent. Only 6.5 percent were delivered in the breech position. This and the stability found neonatally may indicate an etiologic difference between neonatally and late diagnosed CDH.     

Screening of neonatal instability and of developmental dislocation of the hip. A survey of 132,601 living newborn infants between 1956 and 1999

Between 1956 and 1999, 132,601 living children were born in Malm?, and screened for neonatal instability of the hip. All late diagnosed patients have been followed and re-examined clinically and radiologically. During the first years of screening, less than five per 1,000 living newborn infants were treated. This figure increased to 35 per 1000 in 1980, but later diminished again to about six per 1,000 annually after 1990. The number of referred cases decreased from 45 per 1,000 in 1980 to between 10 to 15 per 1,000 from 1990. During the period of high rates of referral and treatment a larger number of paediatricians were involved in the screening procedure than during the periods with low rates of ferral and treatment. Altogether 21 patients (0.16 per 1,000) with developmental dislocation of the hip were diagnosed late, after one week. At follow-up, 18 were free from symptoms and 15 considered to be diologically normal.     

Results of a 25-year screening programme for neonatal hip instability

From 1962 to 1986, 117,256 neonates were screened for congenital dislocation of the hip (CDH). When the primary physical examination was performed by the junior paediatric staff there was a persistent late diagnosis rate of 0.5 per 1000 live births. When the primary examination was undertaken by experienced orthopaedic personnel (1982 to 1984) the late diagnosis rate fell and fewer infants were splinted.     

Congenital dislocation of the hip in the American black

Between 1977 and 1982, 19 cases of congenital dislocation of the hip (CDH) were encountered in black infants. Six of these cases were associated with other anomalies (atypical CDH); 13 were typical CDH. The incidence of complete CDH in the white population studied is 1.5/1000; in the black population studied it is 0.46/1000. The increased incidence in comparison to previous studies may possibly reflect genetic heterogeneity in the control population relative to the African black.     

Ultrasound screening for hips at risk in developmental dysplasia. Is it worth it?

Between May 1992 and April 1997, there were 20,452 births in the Blackburn District. In the same period 1107 infants with hip 'at-risk' factors were screened prospectively by ultrasound. We recorded the presence of dislocation and dysplasia detected under the age of six months using Graf's alpha angle. Early dislocation was present in 36 hips (34 dislocatable and 2 irreducible). Of the 36 unstable hips, 30 (83%) were referred as being Ortolani-positive or unstable; 25 (69%) of these had at least one of the risk factors. Only 11 (31%) were identified from the 'at-risk' screening programme alone (0.54 per 1000 live births). Eight cases of 'late' dislocation presented after the age of six months (0.39 per 1000 live births). The overall rate of dislocation was 2.2 per 1000 live births. Only 31% of the dislocated hips belonged to a major 'at-risk' group. Statistical analysis confirmed that the risk factors had a relatively poor predictive value if used as a screening test for dislocation. In infants referred for doubtful clinical instability, one dislocation was detected for every 11 infants screened (95% confidence interval (CI) 8 to 17) whereas in infants referred because of the presence of any of the major 'at-risk' factors the rate was one in 75 (95% CI 42 to 149). Routine ultrasound screening of the 'at-risk' groups on their own is of little value in significantly reducing the rate of 'late' dislocation in DDH, but screening clinically unstable hips alone or associated with 'at-risk' factors has a high rate of detection.     

A disproportionately high incidence of symptomatic coarctation of the aorta in white infants in the Transvaal

A retrospective study of Johannesburg Hospital records revealed that during a 4-year period (1978-1981) 49 infants who had been born in the Transvaal had presented with symptomatic coarctation of the aorta in the first year of life. The total number of live births for this period was 92,697. This incidence of 0.529 new cases per 1,000 live births, or 1/1,892 births, is three times higher than that observed in a careful study in the USA. The age at presentation and sex ratio were similar to other reported series. There was no definite seasonal incidence. Two of the patients had siblings with coarctation of the aorta. The exact reasons for the unusually high number of cases which occurred in the years 1979 and 1981 (1/1,405 and 1/1,241 live births respectively) could not be determined. It is suggested that it is probably due to a combination of genetic predisposition and as yet unidentified environmental factors.     

Treatment of congenital dislocation of the hip after neonatal diagnosis

During 1966-1975, 920 infants with a congenital dislocation of the hip diagnosed neonatally were born in Uusimaa county in southern Finland. The first examination of the hips was made by a pediatrician, but the diagnosis was generally confirmed by a pediatric surgeon before treatment was started. The mean age of beginning treatment was 7 days. An abduction pillow was the only treatment in 852 cases, six of whom later developed complications, notably two with avascular necrosis. The duration of the pillow treatment shortened noticeably during the time under review but this caused no increase in the number of failures. In 64 cases the routine pillow treatment could not be carried through, mostly because the hip dislocated on the pillow. The most apparent reason for this was inappropriate control of the pillow by the parents. In this group bilateral dislocations and associated calcaneovalgus feet were found more often than in the rest of the neonatal group. Non-operative treatment was successful in 96 per cent of the 920 cases.     

A population-based study of congenital diaphragmatic hernia outcome in New South Wales and the Australian Capital Territory, Australia, 1992-2001

Purpose

The aim of the study was to describe the incidence and survival of infants born with congenital diaphragmatic hernia (CDH) in the state of New South Wales (NSW) and the Australian Capital Territory (ACT), Australia.

Methods

A population-based cohort study of all infants inclusive up to 1 month of age diagnosed with CDH in NSW and the ACT between 1992 and 2001 was conducted. Data sources were the NSW and ACT Neonatal Intensive Care Data Collection (Neonatal Intensive Care Units Study), NSW Birth Defects Register, Population Health Research Centre of ACT Health, and NSW Midwives Data Collection.Individual risk factors for mortality were assessed using the χ² test with P < .05 being considered statistically significant. Multivariate analysis was performed using logistic regression to adjust for potential confounding variables.

Results

From the databases used, the incidence of CDH in NSW and the ACT was 1 per 3800 births. Of 242 infants identified with CDH, 8% underwent termination of pregnancy, 10% were stillborn after 20 weeks' gestation, and 82% were liveborn.Most liveborn infants (70%) were delivered at term with a 64% survival, whereas 30% were preterm with a 35% survival. For liveborn infants, the overall preoperative mortality was 35% with 56% surviving to discharge.Logistic regression identified a low 5-minute Apgar score, prematurity, and air leak as independent risk factors for mortality.

Conclusions

This population-based study of CDH provides us with baseline data for our states. Mortality is high in preterm infants and in the preoperative period. Avoiding preterm delivery and improving preoperative stabilization are the measures most likely to improve survival.     

Neonatal screening for hip joint instability. Its clinical and economic relevance.

Effort to determine the efficacy of neonatal hip examination began in 1967. During the subsequent 23 years, there have been three analyses of the study's data: (1) a retrospective review reported in 1981, (2) a prospective examination in 1989, and (3) an economic evaluation in 1990. The retrospective review concluded that a neonatal hip screening program effectively screens for acetabular dysplasia, and early treatment seems to alter the outcome of congenital dislocation of the hip. The prospective examination, involving 62 newborns diagnosed as having dislocated or dislocatable hips, established that such cases can be effectively treated from birth with flexion/abduction splints monitored by routine clinical and follow-up examinations. An economic evaluation of the data demonstrated an economic benefit of more than $15,000 Cdn per 1000 infants screened. This study, through these three analyses, presents a compelling argument in favor of a routine, standardized neonatal hip examination.     

Pitfalls of early diagnosis and treatment of congenital dislocation of the hip joint

The efficiency of the routines for early diagnosis and treatment of congenital dislocation of the hip joint (CDH) practiced in the 1960s in the city of G?teborg were reviewed. Between 1961 and 1970 there were 65,875 live births in G?teborg. Eighty-five percent of the estimated number of cases of CDH were diagnosed during the first month of life. Of the children "missed" at the primary screening on the maternity wards, 75% (15/20) were premature babies and/or had been delivered during weekends. In all, 475 newborn children were judged to have unstable hips (dislocated or dislocatable hips), corresponding to an incidence of 7.2 per thousand live births. In 19 children, abduction treatment with braces commenced during the first 10 days of life failed to prevent dislocation. Eighteen of these children were treated primarily with a Frejka pillow and one with a von Rosen splint. A history of CDH among relatives was obtained in 47% of these 19 children including four of five children who had developed pathological hips after supplementary treatment with a hip spica cast and/or surgery. Radiological examination at the age of 1-4 months was helpful in revealing failure of early abduction treatment to prevent dislocation, which in most cases was difficult to detect by clinical examination at this early age.     

Adverse outcome of congenital diaphragmatic hernia is determined by diaphragmatic agenesis, not by antenatal diagnosis

BACKGROUND/PURPOSE: The authors studied their congenital diaphragmatic hernia (CDH) cases retrospectively to ascertain if classical CDH and diaphragmatic agenesis (DA) have separate clinical manifestations, whether antenatally diagnosed cases behave differently from those not diagnosed antenatally, and if antenatal diagnosis before 25 weeks carries a worse prognosis. METHODS: The authors performed a retrospective review of 23 infants with CDH treated at their institution between January 1996 and March 1999. The patients were divided into 3 groups that were analyzed: DA and classical CDH, antenatally diagnosed and nonantenatally diagnosed, and antenatally diagnosed before 25 weeks and after 25 weeks. RESULTS: There were 8 cases of DA and 11 cases of classical CDH. Four infants died without operation and could not be classified. Neonates with DA had significantly longer mean duration of preoperative stabilization compared with classical CDH (5.25+/-2.76 days v 1.36+/-1.0 days) and postoperative mechanical ventilatory support (15.7+/-3.0 days v 4.9+/-3.0 days). Fifty percent of DA patients died; all classical CDH patients survived. Twelve cases were diagnosed antenatally, 6 before 25 weeks' gestation. Antenatally diagnosed cases had no statistically significant difference in mortality rates from those not diagnosed antenatally; 50% of those diagnosed before 25 weeks survived. CONCLUSIONS: DA cases require more preoperative preparation and postoperative ventilation and have a bad prognosis compared with classical CDH. Antenatal diagnosis of CDH does not convey a different prognosis. Fifty percent of CDH patients with antenatal diagnosis before 25 weeks survive.     

Recent advances in the prevention, early diagnosis, and treatment of congenital dislocation of the hip in Japan

Results of animal experiments and then of clinical study of newborn infants suggested that the main mechanical factor causing hip dislocation is prolonged tension on the hamstrings and iliopsoas and that each of these muscles has a synergistic effect in the production of hip dislocation during the perinatal period. It was, therefore, presumed that the high incidence of congenital dislocation of the hip (CDH) in Japan might be due mainly to the Japanese tradition of forcibly maintaining the legs of infants in an extended position with a "swathing diaper." The incidence of CDH in Japanese infants prior to 1965 was as high as 1.1%-3.5%. In an attempt to reduce the incidence of CDH, examination of newborns was performed on a national scale. However, early screening methods for hip abnormalities were not adequately reliable in Japan to allow identification of conditions that would later become CDH. In 1975 a national campaign to avoid prolonged extension of the hips and knees of infants during the early postnatal period was initiated. As a result, there has been a remarkable reduction in the incidence of CDH in infants, to less than 0.2%.     

Radiographic screening at four months of infants at risk for congenital hip dislocation.

We report on a radiographic screening programme at four months of age for infants who were clinically normal at neonatal examination but were considered to be 'at risk' for congenital dislocation of the hip because of their family history, breech presentation, or a persistent click. From a total population of 13,662 live births over a two-year period, 357 (2.6%) infants at risk were identified. Of these 46 had abnormal radiographs (six subluxations, 40 acetabular dysplasia). In 12 infants treatment resulted in a normal hip; 34 required no treatment but were followed up until their radiographs were normal and walking had begun. Of the 311 infants with normal radiographs, 256 (82%) were examined after 15 months of age; none had any detectable abnormality. We suggest that radiography of the hip at four months is a valuable adjunct to neonatal screening for infants at increased risk of congenital dislocation of the hip.     

Acetabular dysplasia at the age of 1 year in children with neonatal instability of the hip

Background and purpose As much as one-third of all total hip arthroplasties in patients younger than 60 years may be a consequence of developmental dysplasia of the hip (DDH). Screening and early treatment of neonatal instability of the hip (NIH) reduces the incidence of DDH. We examined the radiographic outcome at 1 year in children undergoing early treatment for NIH.

Subjects and methods All children born in Malmö undergo neonatal screening for NIH, and any child with suspicion of instability is referred to our clinic. We reviewed the 1-year radiographs for infants who were referred from April 2002 through December 2007. Measurements of the acetabular index at 1 year were compared between neonatally dislocated, unstable, and stable hips.

Results The incidence of NIH was 7 per 1,000 live births. The referral rate was 15 per 1,000. 82% of those treated were girls. The mean acetabular index was higher in dislocated hips (25.3, 95% CI: 24.6–26.0) than in neonatally stable hips (22.7, 95% CI: 22.3–23.2). Girls had a higher mean acetabular index than boys and left hips had a higher mean acetabular index than right hips, which is in accordance with previous findings.

Interpretation Even in children who are diagnosed and treated perinatally, radiographic differences in acetabular shape remain at 1 year. To determine whether this is of clinical importance, longer follow-up will be required.     

Pathophysiology of congenital diaphragmatic hernia. III: Exogenous surfactant therapy for the high-risk neonate with CDH.

Exogenous surfactant therapy (EST) in surfactant-deficient premature infants has been shown to improve lung compliance, decrease morbidity, and improve survival. Reports have demonstrated that newborns with congenital diaphragmatic hernia (CDH) have lung compliance, pressure-volume curves, and hyaline membrane formation resembling those changes seen in surfactant deficient premature newborns. We hypothesize that EST may also benefit infants with CDH. All high risk cases of prenatally diagnosed CDH at Children's Hospital of Buffalo from November 1988 to February 1991 were prospectively evaluated for EST. In those families who chose to participate, the surfactant preparation, Infasurf (100 mg/kg), was instilled into the newborn's lungs prior to the first breath. The remainder of the perinatal, neonatal, and surgical care was performed in a routine manner. Three high-risk prenatally diagnosed newborns with left CDH were treated with EST. All showed signs of decreased pulmonary compliance, but could still be adequately oxygenated and ventilated. Surgical correction was performed after stabilization and all required patch closures. Two of the three infants suffered no life-threatening episodes of pulmonary hypertension and all survived. These infants had many known indicators for poor outcome in CDH with an expected survival of less than 20%. We believe that EST in these neonates with CDH contributed to their survival with minimum morbidity. These results suggest that surfactant replacement for the high-risk neonate with CDH warrants further consideration and a randomized clinical trial is being planned.     

Pitfalls of early diagnosis and treatment of congenital dislocation of the hip joint

Summary The efficiency of the routines for early diagnosis and treatment of congenital dislocation of the hip joint (CDH) practiced in the 1960s in the city of Göteborg were reviewed. Between 1961 and 1970 there were 65 875 live births in Göteborg. Eighty-five percent of the estimated number of cases of CDH were diagnosed during the first month of life. Of the children missed at the primary screening on the maternity wards, 75% (15/20) were premature babies and/or had been delivered during weekends. In all, 475 newborn children were judged to have unstable hips (dislocated or dislocatable hips), corresponding to an incidence of 7.2 per thousand live births. In 19 children, abduction treatment with braces commenced during the first 10 days of life failed to prevent dislocation. Eighteen of these children were treated primarily with a Frejka pillow and one with a von Rosen splint. A history of CDH among relatives was obtained in 47% of these 19 children including four of five children who had developed pathological hips after supplementary treatment with a hip spica cast and/or surgery. Radiological examination at the age of 1–4 months was helpful in revealing failure of early abduction treatment to prevent dislocation, which in most cases was difficult to detect by clinical examination at this early age.     

Inguinal hernia in patients with congenital dislocation of the hip. A sign of general connective tissue disorder

During a 5-year period, all the children born in Malm?, Sweden, were examined for congenital dislocation of the hip (CDH) and for inguinal hernia. Girls with CDH had a hernia five times more frequently than other girls, and boys with CDH three times more frequently. The children with CDH sustained their hernia abnormally early in life. We suggest that relaxin, which stimulates collagenase, could alter the connective tissue and be of importance for the development of both CDH and the hernia.     

A population-based study of congenital diaphragmatic hernia: impact of associated anomalies and preoperative blood gases on survival.

BACKGROUND/PURPOSE: Although neonatal care has improved over the past 20 years, mortality rate with congenital diaphragmatic hernia (CDH) remains 50% to 60%, possibly reflecting differences in management or selection biases. The authors determined the incidence, outcome, effect of coexisting anomalies, and prognostic indicators for neonates with CDH in a single inborn population older than 13 years. METHODS: Forty-three neonates with CDH, those symptomatic within the first 6 hours of life, were identified using a validated neonatal database and diagnosis coding data from medical records among 180,643 live inborn neonates delivered at Parkland Memorial Hospital between 1983 and 1995. Charts were reviewed for prenatal history, demographic variables, presence of coexisting malformations, preoperative arterial blood gases, surgical findings, and outcome. Survival to hospital discharge was the primary outcome variable. RESULTS: The incidence of CDH was 1 in 4,200 live births; overall survival rate was 51%. Thirty-two (74%) neonates underwent surgical repair, often at less than 8 hours of life; postoperative mortality rate was 31%. Eighteen (42%) had coexisting major anomalies or chromosomal abnormalities. Eighty percent of neonates with isolated CDH survived, whereas 89% with CDH and associated defects died. Nonsurvivors had lower birth weights and Apgar scores, were more acidotic, and had more severe respiratory compromise. When best preoperative pH was > or = 7.25 or PaCO2 < or = 50 mm Hg, 80% of neonates survived. CONCLUSION: In this inborn population-based review of neonatal CDH between 1983 and 1995, the best predictors of survival were the presence or absence of other anomalies and the best preoperative PaCO2 and pH.