The Alberta Congenital Anomalies Surveillance System: a 40-year review with prevalence and trends for selected congenital anomalies, 1997-2019. / Le système de surveillance des anomalies congénitales de l'Alberta : compte rendu des données sur 40 ans avec prévalence et tendances de certaines anomalies congénitales entre 1997 et 2019.

INTRODUCTION: Current published long-term provincial or territorial congenital anomaly data are lacking for Canada. We report on prevalence (per 1000 total births) and trends in 1997-2019, in Alberta, Canada, for selected congenital anomalies. Associated risk factors are also discussed. METHODS: We used data from the Alberta Congenital Anomalies Surveillance System (ACASS) to calculate the prevalence and perform chi-square linear trend analyses. RESULTS: From 1997 to 2019, the overall prevalence of neural tube defects was stable, at 0.74 per 1000 total births. The same was true for spina bifida (0.38), orofacial clefts (1.99), more severe CHDs (transposition of the great arteries, 0.38; tetralogy of Fallot, 0.33; and hypoplastic left heart syndrome, 0.32); and gastroschisis (0.38). Anencephaly, cleft palate and anorectal malformation significantly decreased with a prevalence of 0.23, 0.75 and 0.54 per 1000 total births, respectively. Significantly increasing trends were reported for anotia/microtia (0.24), limb reduction anomalies (0.73), omphalocele (0.36) and Down syndrome (2.21) and for hypospadias and undescended testes (4.68 and 5.29, respectively, per 1000 male births). CONCLUSION: Congenital anomalies are an important public health concern with significant social and societal costs. Surveillance data gathered by ACASS for over 40 years can be used for planning and policy decisions and the evaluation of prevention strategies. Contributing genetic and environmental factors are discussed as is the need for continued surveillance and research.

Congenital limb deficiencies and major associated anomalies in Alberta for the years 1980-2012.

There is a wide range of the proportion of congenital anomalies associated with limb deficiencies reported in the literature. This variation is primarily attributed to methodology and classification differences. The distribution of associated anomalies among cases with congenital limb deficiencies in Alberta born between January 1, 1980 and December 31, 2012 is described. Of the 170 cases identified, most were live born (75.3%), male (61.8%), had longitudinal limb deficiencies (78.8%), and had associated anomalies outside the musculoskeletal system (77.6%). Significant associations between the preaxial longitudinal group and the central nervous, gastrointestinal, and cardiovascular systems are reported as well as between the postaxial longitudinal group and congenital hip and foot anomalies. Probable and possible syndrome diagnoses are described for cases with recognized malformation patterns.

Views on the Oberg-Manske-Tonkin Classification System for Congenital Anomalies of the Hand and Upper Limb.

A new classification system was proposed by Tonkin et al as a replacement for the Swanson/International Federation of Societies for Surgery of the Hand system. We have reviewed their aims and have pointed out a number of problems that will make it difficult to be universally accepted.

Congenital limb deficiencies in Alberta-a review of 33 years (1980-2012) from the Alberta Congenital Anomalies Surveillance System (ACASS).

The birth prevalence of limb deficiencies in Alberta has been fluctuating. The objectives were to examine patterns and temporal trends of congenital limb deficiencies in Alberta and compare rates with those of other jurisdictions. The Alberta Congenital Anomalies Surveillance System data on live births, stillbirths, and terminations of pregnancy (<20 weeks gestation) occurring between 1980 through 2012 with the ICD-10 Royal College of Paediatrics and Child Health Adaptations codes Q71-Q73 (limb reduction defects), Q79.80 (congenital constriction bands), and Q87.24 (sirenomelia syndrome) were reviewed. Cases were classified as having amelia, transverse, longitudinal (preaxial, postaxial, central, or mixed), intercalary, split hand/split foot, complex, or other type of limb deficiency. Phenotypes were classified as associated, which included cases with a known etiology and cases with at least one other type of anomaly, or isolated. From 1980 through 2012, 795 cases were ascertained from 1,411,652 live births and stillbirths, giving a prevalence of 5.6/10,000 total births. Mixed longitudinal deficiencies were the most common (22.4%). The upper limbs (63.9%) were affected more often than the lower limbs (25.3%). Isolated limb deficiencies occurred in 43.6% of cases, 28.4% had Mendelian or other known conditions, 21.9% had multiple congenital anomalies, 5.4% had chromosome abnormalities and 0.6% were due to teratogens. The associated group, showed a significant increasing trend (P = 0.023). While the overall limb deficiency rates show very little differences across diverse populations and differing time periods, comparisons of subgroups should be made with caution, because variations in terminology and classification contribute to reported differences.

Comparison of cast materials for the treatment of congenital idiopathic clubfoot using the Ponseti method: a prospective randomized controlled trial.

BACKGROUND: The Ponseti method of congenital idiopathic clubfoot correction has traditionally specified plaster of Paris (POP) as the cast material of choice; however, there are negative aspects to using POP. We sought to determine the influence of cast material (POP v. semirigid fibreglass [SRF]) on clubfoot correction using the Ponseti method. METHODS: Patients were randomized to POP or SRF before undergoing the Ponseti method. The primary outcome measure was the number of casts required for clubfoot correction. Secondary outcome measures included the number of casts by severity, ease of cast removal, need for Achilles tenotomy, brace compliance, deformity relapse, need for repeat casting and need for ancillary surgical procedures. RESULTS: We enrolled 30 patients: 12 randomized to POP and 18 to SRF. There was no difference in the number of casts required for clubfoot correction between the groups (p = 0.13). According to parents, removal of POP was more difficult (p < 0.001), more time consuming (p < 0.001) and required more than 1 method (p < 0.001). At a final follow-up of 30.8 months, the mean times to deformity relapse requiring repeat casting, surgery or both were 18.7 and 16.4 months for the SRF and POP groups, respectively. CONCLUSION: There was no significant difference in the number of casts required for correction of clubfoot between the 2 materials, but SRF resulted in a more favourable parental experience, which cannot be ignored as it may have a positive impact on psychological well-being despite the increased cost associated.

CONTEXTE: La méthode de Ponseti pour la correction du pied bot congénital idiopathique a de tout temps spécifié l'utilisation du plâtre de Paris comme matériau de choix; il y a toutefois certains inconvénients associés au plâtre de Paris. Nous avons voulu déterminer l'influence du matériau utilisé (plâtre de Paris c. fibre de verre semi-rigide) sur la correction du pied bot selon la méthode de Ponseti. MÉTHODES: Les patients ont été assignés aléatoirement soit au plâtre de Paris soit à la fibre de verre semi-rigide en vue de l'intervention de Ponseti. Le principal paramètre mesuré était le nombre de plâtres requis pour corriger le pied bot. Les paramètres secondaires incluaient le nombre de plâtres en fonction de la gravité, la facilité de retrait du plâtre, la nécessité de sectionner le tendon d'Achille, le port assidu de l'attelle, le retour de la difformité, la nécessité d'autres plâtres et interventions chirurgicales auxiliaires. RÉSULTATS: Nous avons inscrit 30 patients : 12 ont été assignés au plâtre de Paris et 18 à la fibre de verre. On n'a noté aucune différence entre les groupes quant au nombre de plâtres requis pour la correction du pied bot (p = 0,13). Selon les parents, le retrait du plâtre de Paris était plus difficile p < 0,001), prenait plus de temps (p < 0,001) et nécessitait le recours à plus d'une méthode (p < 0,001). Au moment du dernier suivi à 30,8 mois, les intervalles moyens avant un retour de la difformité nécessitant la pose d'un autre plâtre et/ou une chirurgie ont été de 18,7 et 16,4 mois dans les groupes traités au moyen de la fibre de verre semi-rigide et du plâtre de Paris, respectivement.. CONCLUSION: On n'a noté aucune différence significative entre les 2 matériaux quant au nombre de plâtres requis pour corriger le pied bot, mais la fibre de verre a donné lieu à une expérience plus agréable pour les parents, ce qui ne peut être ignoré en raison de l'impact potentiellement positif sur le bien-être psychologique, et ce, malgré un coût plus élevé.

Principles of rotationplasty.

Rotationplasty is a reconstructive, limb-sparing option for management of lower extremity bone deficiency. This technique involves an intercalary resection, followed by 180° rotation of the distal limb to allow the ankle to function as a knee joint when it is fitted with a modified below-knee prosthesis. Gait analysis and functional outcome studies have reported favorable results with rotationplasty compared with those of above-knee amputation. Moreover, patient satisfaction with rotationplasty is higher than with other limb salvage procedures. The primary drawback of this procedure is patient acceptance of the limb's appearance.

Reliability and predictability of the centre-edge angle in the assessment of pincer femoroacetabular impingement.

PURPOSE: The aim of the study was to assess the use of the centre-edge (CE) angle in the assessment of pincer femoro-acetabular impingement (FAI) for reliability and predictability in the diagnosis. METHODS: Between 2004 and 2008, 55 patients underwent surgical treatment for FAI. A control group of 30 was identified among patients attending the emergency department with normal radiographs. Radiographs were assessed by two independent observers both before and after the operation. Nine patients with trauma were excluded. The magnetic resonance arthrogram reports of the remaining 46 patients were assessed for pincer FAI. Nineteen patients were identified and underwent repeat radiographic assessment. All underwent surgical dislocation of hip (SDH), acetabular, with/without femoral osteochondroplasty. Acetabular depth and version were also assessed. The intraclass correlation (ICC) was used to assess reliability of the CE angle. The paired t test and independent groups t test were used to assess the difference between the pincer FAI group, both pre-op and post-op and against controls. RESULTS: The control and pincer groups were similar in demographics (p=0.1769). Coxa profunda was present in 14 patients with eight also having retroverted acetabuli. Of the rest two had retroverted acetabuli and one protrusio. The mean CE angle in the control group was 31.4°, in the pre-op pincer group 46.2° and in the post-op pincer group 38.3°. The ICC for intra-observer correlation was 0.977 and 0.992 pre-op and 0.986 and 0.974 post-op. The ICC for inter-observer correlation was 0.960 and 0.957 pre-op and 0.979 and 0.967 post-op.The p value was <0.001 between the controls, the pre-op and post-op pincer groups. The test characteristics using the CE angle ≥ 40 is a reasonably good predictor of FAI, with a sensitivity of 84.2% and a specificity of 100%. CONCLUSIONS: The pincer FAI can be reliably assessed with the CE angle and can be predicted in patients presenting with FAI.

Opinion survey regarding pediatric orthopaedic trauma call and emergency trauma management.

BACKGROUND: To determine the attitudes and practices of pediatric orthopaedic surgeons regarding on-call coverage and emergency fracture management. METHODS: A 32-question online survey was sent to all 597 active members of the Pediatric Orthopaedic Society of North America. There were 296 completed surveys, for a response rate of 49.6%. RESULTS: Of the respondents, 85.1% were male. The respondents ranged in age from 30 to older than 70 years, with 54% between 36 and 50 years of age, corresponding to an average of 15 years in practice. Seventy-seven percent of the respondents felt that taking trauma call is an integral aspect of being a pediatric orthopaedist. Of the respondents, 64.9% take call 1 to 9 times per month, 15.8% take 10 to 19 calls, 2.7% take 20 or more, and 16.6% take no call. The number of orthopaedists taking call per practice was fairly evenly distributed between 3 and 10. Call was shared equally in 32% of practices, and mandatory in 72%. Twenty-eight percent of the respondents were additionally compensated for taking calls, in amounts ranging from $100 to $2000 per night, with 1000 dollars the most common rate. One third of operative cases are done that night; one third, the next day; and one third, later in the week. Twenty-four percent of the respondents have dedicated operative block time on the day after the call. Forty-seven percent have a dedicated fracture clinic, of which 51% receive institutional support. CONCLUSIONS: Providing emergency trauma care for children is an integral aspect of pediatric orthopaedics. This survey provides information on the attitudes and strategies of practicing pediatric orthopaedic surgeons in the face of decreasing manpower and increasing demand for such services.

Femoral fractures in children younger than three years: the role of nonaccidental injury.

BACKGROUND: Nonaccidental injury (NAI) in children is a major cause of morbidity and mortality, with fractures being the second most common presentation. The presence of a femur fracture has been reported to be suggestive of nonaccidental trauma in 30% to 60% of young children. The purpose of this study was to determine the percentage of NAI in children younger than 3 years presenting with a femur fracture to a single institution within a western Canadian population. METHODS: A retrospective cohort study was performed for children younger than 3 years who presented to the Alberta Children's Hospital during the years 1994 to 2005. The primary outcome variable was the percentage of NAI associated with femur fracture. Secondary outcome variables included patient demographics, injury characteristics, radiological and other workup, and suspicion of NAI. RESULTS: The overall percentage of NAI was 11% (14/127 patients) and 17% (10/60 patients) in children younger than 12 months. Age younger than 12 months (P = 0.04), nonambulatory status (P = 0.004), delayed presentation (P = 0.002), mechanism of injury unwitnessed or inconsistent (P = 0.008), and other associated injuries (P = 0.006) were significant risk factors for NAI. CONCLUSIONS: Children younger than 3 years who present with femoral fracture are at risk for associated NAI, although perhaps this risk is not as high as previously thought. Regardless, a high index of suspicion is mandatory when these children are encountered, and careful screening with a thorough history, physical examination, and other investigations, where indicated, is warranted to rule out associated NAI. LEVEL OF EVIDENCE: Retrospective cohort study, level IV.

Physeal bridge resection.

Growth arrest secondary to physeal bridge formation is an uncommon but well-recognized complication of physeal fractures and other injuries. Regardless of the underlying etiology, physeal bridges may cause angular and/or longitudinal growth disturbances, with progression dependent on the remaining physeal growth potential. Physeal bridge resection and insertion of interposition material releases the tethering effect of the bridge. Physeal bridge resection has become an accepted treatment option for patients with existing or developing deformity and for those with at least 2 years or 2 cm of growth remaining. Current experimental research is focused on the use of gene therapy and other factors that enhance chondrocyte proliferation to improve the management of growth arrest. The use of cartilage and cultured chondrocytes as interposition material after physeal bridge resection is an area of active research.