MRI of the Spine in Patients who Toe Walk: Is There a Role?

BACKGROUND: Little is known about the prevalence of intraspinal pathology in children who toe walk, but magnetic resonance imaging (MRI) may be part of the diagnostic workup. The purpose of this study was to examine the role of MRI for children who toe walk with a focus on the rate of positive findings and associated neurosurgical interventions performed for children with said MRI findings. METHODS: A single-center tertiary hospital database was queried to identify a cohort of 118 subjects with a diagnosis of toe walking who underwent spinal MRI during a 5-year period. Patient and MRI characteristics were summarized and compared between subjects with a major abnormality, minor abnormality, or no abnormality on MRI using multivariable logistic regression. Major MRI abnormalities included those with a clear spinal etiology, such as fatty filum, tethered cord, syrinx, and Chiari malformation, while minor abnormalities had unclear associations with toe walking. RESULTS: The most common primary indications for MRI were failure to improve with conservative treatment, severe contracture, and abnormal reflexes. The prevalence of major MRI abnormalities was 25% (30/118), minor MRI abnormalities was 19% (22/118), and normal MRI was 56% (66/118). Patients with delayed onset of toe walking were significantly more likely to have a major abnormality on MRI ( P =0.009). The presence of abnormal reflexes, severe contracture, back pain, bladder incontinence, and failure to improve with conservative treatment were not significantly associated with an increased likelihood of major abnormality on MRI. Twenty-nine (25%) subjects underwent tendon lengthening, and 5 (4%) underwent neurosurgical intervention, the most frequent of which was detethering and sectioning of fatty filum. CONCLUSIONS: Spinal MRI in patients who toe walk has a high rate of major positive findings, some of which require neurosurgical intervention. The most significant predictor of intraspinal pathology was the late onset of toe walking after the child had initiated walking. MRI of the spine should be considered by pediatric orthopedic surgeons in patients with toe walking who present late with an abnormal clinical course. LEVEL OF EVIDENCE: Level III Retrospective Comparative Study.

Jumping on the Bandwagon: Comparing the Efficacy of Chlorhexidine Versus Povidone-Iodine Preoperative Skin Antiseptic in Preventing Surgical Site Infections Following Pediatric Orthopaedic Surgery.

BACKGROUND: Adult literature has demonstrated chlorhexidine (CH) superiority at preventing surgical-site infections when compared with povidone-iodine (P-I). The purpose of this study is to compare the rate of postoperative infections after preoperative skin cleansing with either CH or P-I in pediatric orthopaedic surgery in an effort to identify superiority. METHODS: We retrospectively identified all patients (18 y and below) that underwent orthopaedic surgery at our institution in 2015, when P-I was the preoperative skin antisepsis of choice, and in 2018, when a change in protocol resulted in more frequent use of CH. Open fractures, infections, neuromuscular, and tumor surgeries were excluded. Orthopaedic surgeries were classified according to their subspecialty (sports-related/upper extremity, hip and lower extremity, trauma-related, or spine procedure). A 1:1 propensity score matching was conducted within each procedure group on the basis of age, sex, and year using nearest-neighbor matching. Spine procedures could not be matched and were subsequently excluded from analyses. RESULTS: Propensity score matching matched 1416 CH cases with 1416 P-I controls. The infection rate for CH was 19 infections per 1000 cases (27/1416; 1.9%) compared with an infection rate of 11 infections per 1000 cases (16/1416; 1.1%) for P-I subjects. No difference was detected in infection rate across preoperative skin antisepsis groups (P=0.12). Moreover, it was found that CH and P-I resulted in significantly equivalent infection rates to within ±1.5% (P=0.004). When stratified by procedure type, CH used in sports/upper extremity procedures resulted in 29 more infections per 1000 cases compared with P-I use (16/450; 3/450; P=0.005). No difference was detected in infection rate across CH and P-I skin antisepsis groups in lower extremity procedures (9/792; 8/792; P=1.00) or in trauma-related procedures (3/174; 4/174; P=1.00). CONCLUSIONS: CH and P-I are both protective against postoperative infections after sports/upper extremity, lower extremity, and trauma-related pediatric orthopaedic procedures. P-I may provide improved protection over CH as a preoperative skin antisepsis in upper extremity and sports-related procedures. LEVEL OF EVIDENCE: Level III-comparative cohort.

Prospective Evaluation of Tarsal Coalition Excision Show Significant Improvements in Pain and Function.

BACKGROUND: Excision of pediatric tarsal coalition has been successful in most patients. However, some patients have ongoing pain after coalition excision. This study prospectively assessed patient-based clinical outcomes before and after surgical excision of tarsal coalition, with particular emphasis on comparison to radiologic imaging. METHODS: We prospectively studied 55 patients who had symptomatic coalition excision for 2 years postoperatively. Patients filled out the modified American Orthopaedic Foot and Ankle Society score, the University of California Los Angeles activity score, and the simple question "does foot pain limit your activity" at 4 different time points: preoperative, 6 months postoperative, 12 months postoperative, and 24 months postoperative. Comparisons were done utilizing patient demographics, imaging parameters, and patient-reported outcomes. RESULTS: Compared with preoperative levels, patients showed improvements in all outcome parameters. Patients with calcaneonavicular coalitions showed initial rapid improvement with later slight decline, while patients with talocalcaneal coalitions showed more steady improvement; both were similar at 2 years postoperatively. CONCLUSIONS: This prospective study demonstrated remarkable clinical improvements after tarsal coalition excision regardless coalition type, though postoperative courses differed between calcaneonavicular and talocalcaneal types. Finally, a subset of patients has ongoing activity limiting foot pain after coalition excision which could not be explained by the data in this study. LEVEL OF EVIDENCE: Level II-prospective cohort study.

Epiphysiodesis for Leg Length Discrepancy: A Cost Analysis of Drill Versus Screw Technique.

Research has demonstrated similar efficacy of drill epiphysiodesis and percutaneous epiphysiodesis using transphyseal screws for the management of adolescent leg length discrepancy. A cost analysis was performed to determine which procedure is more cost-effective. Patients seen for epiphysiodesis of the distal femur and/or proximal tibia and fibula between 2004 and 2017 were reviewed. A decision analysis model was used to compare costs. Two hundred thirty-five patients who underwent either drill (155/235, 66%) or screw (80/235, 34%) epiphysiodesis were analyzed with an average age at initial procedure of 13 years (range, 8.4 to 16.7 years). There was no significant difference in average initial procedure cost or total cost of all procedures across treatment groups (n = 184). The cost difference between drill and screw epiphysiodesis is minimal. In order for screw epiphysiodesis to be cost-favored, there would need to be a significant decrease in its cost or complication rate. (Journal of Surgical Orthopaedic Advances 30(3):181-184, 2021).

Congenital Pseudarthrosis of the Fibula: Controlling Ankle Valgus in a Heterogenous Condition.

BACKGROUND: Congenital pseudarthrosis of the fibula (CPF) is a rare disorder characterized by a deficiency in the continuity of the fibula and can lead to progressive ankle valgus malalignment. An existing classification system for CPF is imperfect and may contribute to heterogeneity in reporting and discrepancy of outcomes in the literature. METHODS: Fifteen patients with CPF treated at our institution between 1995 and 2017 were retrospectively identified. Only patients with dysplasia leading to spontaneous fracture or pseudarthrosis were included in this series. The median age at presentation was 2.5 years (range: 3 mo to 13.4 y). The median duration of follow-up from the initial presentation was 11.8 years (range: 2.0 to 24 y). Chart review and serial radiographs were analyzed to assess natural history and outcomes following surgery. RESULTS: The coexistence of tibial dysplasia in CPF is very common. Patients were classified into 3 groups based on the degree of tibial involvement-group 1: no evidence of tibial dysplasia, group 2: mild tibial dysplasia, and group 3: significant tibial dysplasia. Age at presentation and age at which fibular fracture occurred were progressively younger with a greater degree of tibial involvement (P<0.05). In the absence of surgical intervention, group 1 patients did not undergo progressive ankle valgus (defined as the valgus change in tibiotalar angle by ≥4 degrees), whereas all patients in groups 2 and 3 did (P<0.001). Fibular osteosynthesis was performed in 6 patients, with union seen only in group 1 patients. Ten patients underwent distal tibiofibular fusion, with no cases of nonunion seen. Distal tibiofibular fusion with or without medial distal tibial hemiepiphysiodesis halted the progression of ankle valgus in 8 of the 10 patients. Further progression of ankle valgus occurred only in patients who did not undergo concurrent medial distal tibial hemiepiphysiodesis and with considerable wedging of the distal tibial epiphysis at the time of fusion. CONCLUSIONS: Tibial dysplasia and CPF are intimately related. Grouping patients on this basis may help guide natural history and treatment and may explain discrepancies in findings in the literature. Fibular osteosynthesis, distal tibiofibular fusion, and medial distal tibial hemiepiphysiodesis may all have an important role in the treatment of CPF. LEVEL OF EVIDENCE: Level IV-case series.

Multisource Evaluation of Surgeon Behavior Is Associated With Malpractice Claims.

OBJECTIVE: We merged direct, multisource, and systematic assessments of surgeon behavior with malpractice claims, to analyze the relationship between surgeon 360-degree reviews and malpractice history. BACKGROUND: Previous work suggests that malpractice claims are associated with a poor physician-patient relationship, which is likely related to behaviors captured by 360-degree review. We hypothesize that 360-degree review results are associated with malpractice claims. METHODS: Surgeons from 4 academic medical centers covered by a common malpractice carrier underwent 360-degree review in 2012 to 2013 (n = 385). Matched, de-identified reviews and malpractice claims data were available for 264 surgeons from 2000 to 2015. We analyzed 23 questions, highlighting positive and negative behaviors within the domains of education, excellence, humility, openness, respect, service, and teamwork. Regression analysis with robust standard error was used to assess the potential association between 360-degree review results and malpractice claims. RESULTS: The range of claims among the 264 surgeons was 0 to 8, with 48.1% of surgeons having at least 1 claim. Multiple positive and negative behaviors were significantly associated with the risk of having malpractice claims (P < 0.05). Surgeons in the bottom decile for several items had an increased likelihood of having at least 1 claim. CONCLUSION: Surgeon behavior, as assessed by 360-degree review, is associated with malpractice claims. These findings highlight the importance of teamwork and communication in exposure to malpractice. Although the nature of malpractice claims is complex and multifactorial, the identification and modification of negative physician behaviors may mitigate malpractice risk and ultimately result in the improved quality of patient care.

Lower-extremity growth patterns and skeletal maturation in children with unilateral fibular hemimelia.

BACKGROUND: Fibular hemimelia is the most common congenital long-bone deficiency. It is usually unilateral and results in a limb-length discrepancy. The literature generally subscribes to the concept of constant inhibition, a process by which limb-length ratios between the shorter and longer extremity remain constant throughout growth, but scientific data supporting this concept are sparse. Additionally, recent literature suggests that these children have abnormal skeletal maturation. OBJECTIVE: To elucidate the lower-extremity long-bone growth patterns and skeletal maturation of children with unilateral fibular hemimelia. MATERIALS AND METHODS: We reviewed medical records of children with unilateral fibular hemimelia seen at a large pediatric hospital over a 17-year period. Inclusion criteria were: at least two scanograms prior to any shortening/lengthening procedure, and no other congenital or acquired disorders. We collected the study cohort's femoral and tibial lengths (scanogram reports), plotted them against patient chronological ages and compared them to published growth standards. When these children's bone ages (Greulich and Pyle) were available, we plotted them against the children's chronological ages. RESULTS: Twenty-three children were included (total=115 scanograms). At least 1 bone-age assessment was performed in 19 children (total=84 bone ages). All bone growth curves were within normal growth standards for the femur and tibia. Length ratios between shorter and longer limbs remained constant. Skeletal maturation was within two standard deviations of normal in 90% of bone ages. CONCLUSION: Lower-extremity long bones of children with unilateral fibular hemimelia have relatively normal growth curves, supporting and confirming the concept of constant inhibition. Most children show normal skeletal maturation.

Reconstruction of Symptomatic Congenital Anterior Cruciate Ligament Insufficiency.

BACKGROUND: Hypoplasia or congenital absence of the anterior cruciate ligament (ACL) is a rare disorder occurring in ∼1 in every 6000 births. Although some patients with hypoplasia or agenesis of the ACL may not complain of instability, others desire to participate in more demanding activities that require the stability of a competent ACL. There are limited reports of surgical treatment of this patient population. The purpose of this study was to report ACL reconstruction in a case series of patients with symptomatic congenital ACL deficiency. METHODS: A retrospective medical record review of the surgical treatment of 14 knees (13 patients) with congenital absence of the ACL at a tertiary care institution from 1995 to 2012 was performed. Patients with a minimum of 1 year of clinical follow-up were eligible for inclusion. RESULTS: The mean age at time of surgery was 12.6 (range, 3 to 22), including 6 patients <12 years of age. Mean follow-up was 2.9 years (range, 1 to 6.6). Nine of 13 patients (69%) had underlying congenital abnormalities/associated syndromes. Preoperative Lachman and pivot shift examination was International Knee Documentation Committee grade C or D in all but 1 knee. ACL reconstruction was performed with combined intra-articular/extra-articular physeal sparing reconstruction with iliotibial band (n=5), autograft hamstring (n=2) or bone-patellar tendon-bone (n=3), or allograft (n=4). Multiligament reconstruction of associated ligamentous deficiency was performed in 7 knees (50%). Postoperative Lachman and pivot shift testing was International Knee Documentation Committee (IKDC) grade A or B in all but 1 knee. One patient with congenital absence of multiple knee ligaments required revision ACL reconstruction surgery, with concurrent first-time posterior cruciate ligament reconstruction, due to persistent instability. None required revision surgery due to graft tear at a minimum of 1-year follow-up. CONCLUSIONS: Surgical stabilization of symptomatic congenital ACL insufficiency, with associated ligamentous reconstruction as required on a case-by-case basis, results in improved stability at early clinical follow-up, with low complication rates. LEVEL OF EVIDENCE: Level IV-retrospective case series.

Constant inhibition in congenital lower extremity shortening: does it begin in utero?

BACKGROUND: Limb-length discrepancy (LLD) in children with congenital lower extremity shortening is constant in proportion from birth to skeletal maturity (known as constant inhibition), but its developmental pattern in utero is unknown. The popular prenatal multiplier method to predict LLD at birth assumes constant inhibition in utero to be true. Verifying the in utero developmental pattern of LLD, and thus confirming the validity of the prenatal multiplier method, is crucial for meaningful prenatal parental counseling. OBJECTIVE: To elucidate the in utero developmental pattern of LLD in fetuses with congenital lower extremity shortening. MATERIALS AND METHODS: Clinical indications for 3,605 lower extremity radiographs performed on infants (<1 year old) at a large tertiary hospital over a 17-year period were reviewed. Inclusion criteria were (1) diagnosis of congenital lower extremity shortening, (2) bilateral lower limb postnatal radiographs documenting LLD and (3) fetal ultrasound (US) documenting LLD. Available measurements of femoral, tibial and fibular lengths on fetal US and postnatal radiographs were collected. Prenatal and postnatal length ratios of shorter-to-longer bones were calculated and compared. RESULTS: Eighteen infants met inclusion criteria. Diagnoses were proximal focal femoral deficiency=4, congenital short femur=2, tibial hemimelia=3, posteromedial tibial bowing=6 and fibular hemimelia=3. The correlations between postnatal and prenatal length ratios were high for the femur, tibia and fibula (R>0.98, P<0.0001). The relative differences in the postnatal and prenatal length ratios of these bones were small (|average|<0.026, standard deviation <0.068). CONCLUSION: Our data indicate that the postnatal and prenatal length ratios were equivalent, supporting the constant inhibition pattern of LLD in utero, thus validating the prenatal multiplier method for predicting LLD.

Subtalar Coalitions: Does the Morphology of the Subtalar Joint Involvement Influence Outcomes After Coalition Excision?

Posteromedial subtalar (PMST) coalitions are a recently described anatomic subtype of tarsal coalitions. We compared with clinical patient-based outcomes of patients with PMST and standard middle facet (MF) coalitions who had undergone surgical excision of their coalition. The included patients had undergone surgical excision of a subtalar tarsal coalition, preoperative computed tomography (CT), and patient-based outcomes measures after surgery (including the American Orthopaedic Foot and Ankle Society [AOFAS] scale and University of California, Los Angeles [UCLA], activity score). Blinded analysis of the preoperative CT scan findings determined the presence of a standard MF versus a PMST coalition. The perioperative factors and postoperative outcomes between the MF and PMST coalitions were compared. A total of 51 feet (36 patients) were included. The mean follow-up duration was 2.6 years after surgery. Of the 51 feet, 15 (29.4%) had a PMST coalition and 36 (70.6%) had an MF coalition. No difference was found in the UCLA activity score; however, the mean AOFAS scale score was higher for patients with PMST (95.7) than for those with MF (86.5; p = .018). Of the patients with a PMST, none had foot pain limiting their activities at the final clinical follow-up visit. However, in the group with an MF subtalar coalition, 10 (27.8%) had ongoing foot pain limiting activity at the final follow-up visit (p = .024). Compared with MF subtalar tarsal coalitions, patients with PMST coalitions showed significantly improved clinical outcomes after excision. Preoperative identification of the facet morphology can improve patient counseling and expectations after surgery.

Surgical Reconstruction of Metatarsal Type Preaxial Polydactyly Using an Amalgamating Osteotomy.

BACKGROUND: Polydactyly of the foot is a relatively common condition. Approximately 15% of cases are preaxial, with one third of these cases involving duplication of the metatarsal [metatarsal type preaxial polydactyly (MTPP)].Surgical reconstruction of polydactyly is indicated to improve shoe tolerance. Reconstruction of MTPP has traditionally involved resection of the hypoplastic lateral ray in addition to soft tissue reconstruction to correct hallux varus. Poor postoperative results have frequently been reported, primarily due to residual hallux varus. We present a novel surgical technique for the treatment of children with MTPP presenting with a cosmetic lateral hallux, involving an amalgamating osteotomy that permits retention of the stable medial metatarsotarsal joint while avoiding the complication of residual hallux varus. METHODS: This was a retrospective case series describing the surgical technique of an amalgamating osteotomy in the treatment of patients with MTPP and a cosmetic lateral hallux. The surgical technique involves corresponding metatarsal osteotomies of the medial and lateral halluces, with amalgamation of the metatarsals and ablation of the residual medial hallux, without the need for extensive soft tissue reconstruction. Clinical and radiologic outcomes were evaluated at a minimum of 2 years postoperatively in 2 patients who underwent this technique. RESULTS: Two children, 1 female and 1 male, underwent an amalgamating osteotomy at the age of 31 and 18 months, respectively. At latest follow-up, 7.3 and 2.8 years after osteotomy, respectively, both patients displayed an excellent functional result according to the Phelps and Grogan clinical outcome scale. Plain radiographs in both cases demonstrated a well-aligned first ray with no growth abnormality and no hallux varus. CONCLUSIONS: We have presented a novel surgical technique for the reconstruction of MTPP presenting with a cosmetic lateral hallux, involving an amalgamating osteotomy without extensive soft tissue reconstruction. This simple technique maintains the stable medial metatarsotarsal joint, permits ongoing longitudinal metatarsal growth, and avoids the complication of hallux varus. LEVEL OF EVIDENCE: Level IV-case series.

Maternal cigarette, alcohol, and coffee consumption in relation to risk of clubfoot.

BACKGROUND: Clubfoot is associated with maternal cigarette smoking in several studies, but it is not clear if this association is confined to women who smoke throughout the at-risk period. Maternal alcohol and coffee drinking have not been well studied in relation to clubfoot. METHODS: The present study used data from a population-based case-control study of clubfoot conducted in Massachusetts, New York, and North Carolina from 2007 to 2011. Mothers of 646 isolated clubfoot cases and 2037 controls were interviewed about pregnancy events and exposures, including the timing and frequency of cigarette smoking, alcohol intake, and coffee drinking. RESULTS: More mothers of cases than controls reported smoking during early pregnancy (28.9% vs. 19.1%). Of women who smoked when they became pregnant, those who quit in the month after a first missed period had a 40% increase in clubfoot risk and those who continued to smoke during the next 3 months had more than a doubling in risk, after controlling for demographic factors, parity, obesity, and specific medication exposures. Adjusted odds ratios for women who drank >3 servings of alcohol or coffee per day throughout early pregnancy were 2.38 and 1.77, respectively, but the numbers of exposed women were small and odds ratios were unstable. CONCLUSIONS: Clubfoot risk appears to be increased for offspring of women who smoke cigarettes, particularly those who continue smoking after pregnancy is recognisable, regardless of amount. For alcohol and coffee drinkers, suggested increased risks were only observed in higher levels of intake.

Patient-reported Outcomes of Tarsal Coalitions Treated With Surgical Excision.

PURPOSE: There are little patient-reported data on functional outcomes of tarsal coalition resection in children and adolescents. The purpose of this study is to evaluate the medium-term (>2 y) outcomes in patients who have had surgical excision of their symptomatic tarsal coalition and to compare patient-based outcomes in patients who have calcaneonavicular (CN) coalitions to those with talocalcaneal (TC) coalitions. METHODS: A billing query was conducted to identify patients who had surgical excision of their tarsal coalition between 2003 and 2008. Eligible patients were mailed questionnaires consisting of a modified American Orthopaedic Foot and Ankle Society (AOFAS) score and the University of California at Los Angeles (UCLA) activity scale. Patients were also specifically asked if their activity level was limited by their foot pain. Only patients who returned questionnaires were included. Demographics and diagnostic images were reviewed. A nonresponder analysis was completed. Complications such as infection and reoperation were reported. RESULTS: Sixty-three patients (22 females, 41 males) who returned questionnaires were included in the analysis. Twenty-four patients had bilateral surgery. TC coalitions were present in 20 patients (32%); CN coalitions were present in 43 patients (68%).Overall, mean modified AOFAS score was 88.3 and mean UCLA activity score was 8.33 at an average of 4.62 years after surgery. Patients who had TC coalitions had similar modified AOFAS scores (88.4) and UCLA activity scores (8.4) when compared with those with CN coalitions (88.0 and 8.3, both not significant).Of the 73% (46/63) patients who reported that their activity levels were not limited by their foot pain, the mean AOFAS score was 93.9 and the mean UCLA activity score was 8.9; 32 of these were CN and 14 were TC coalitions. Of the 27% (17/63) patients who reported that their activity levels were limited by their foot pain, the mean AOFAS score was 72.9 and the mean UCLA activity score was 6.9; 11 of these were CN and 6 were TC coalitions. There was a statistically significant difference in these groups both in modified AOFAS score (P<0.0001) and UCLA activity score (P=0.006). There was no difference in outcomes between those who were treated for a TC and CN coalition. CONCLUSIONS: Patient-reported outcomes after surgical excision of tarsal coalition reveal that >70% of patients' activities are not limited by pain and their functional outcome is terrific. A few patients continue to have problems with ongoing foot pain and activity limitations. The type of coalition does not seem to be an indicative factor in determining outcome.

Medial versus anterior open reduction for developmental hip dislocation in age-matched patients.

BACKGROUND: The difference between medial (MAOR) and anterior (AAOR) approaches for open reduction of developmental hip dysplasia in terms of risk for avascular necrosis (AVN) and need for further corrective surgery (FCS, femoral and/or acetabular osteotomy) is unclear. This study compared age-matched cohorts undergoing either MAOR or AAOR in terms of these 2 primary outcomes. Prognostic impact of presence of ossific nucleus at time of open reduction was also investigated. METHODS: Institutional review board approval was obtained. Nineteen hips (14 patients) managed by MAOR were matched with 19 hips (18 patients) managed by AAOR based on age at operation (mean 6.0; range, 1.4 to 14.9 mo). Patients with neuromuscular conditions and known connective tissue disorders were excluded. Primary outcomes assessed at minimum 2 years' follow-up included radiographic evidence of AVN (Kalamchi and MacEwen) or requiring FCS. RESULTS: MAOR and AAOR cohorts were similar regarding age at open reduction, sex, laterality, and follow-up duration. One hip in each group had AVN before open reduction thus were excluded from AVN analysis. At minimum 2 years postoperatively (mean 6.2; range, 1.8 to 11.7 y), 4/18 (22%) MAOR and 5/18 (28%) AAOR met the same criteria for AVN (P=1.0). No predictors of AVN could be identified by regression analysis. Presence of an ossific nucleus preoperatively was not a protective factor from AVN (P=0.27). FCS was required in 4/19 (21%) MAOR and 7/19 (37%) AAOR hips (P=0.48). However, 7/12 (54%) hips failing closed reduction required FCS compared with 4/26 (16%) hips without prior failed closed reduction (P=0.024). Cox regression analysis showed that patients who failed closed reduction had an annual risk of requiring FCS approximately 6 times that of patients without a history of failed closed reduction (hazard ratio=6.1; 95% CI, 1.5-24.4; P=0.009), independent of surgical approach (P=0.55) or length of follow-up (P=0.78). CONCLUSIONS: In this study of age-matched patients undergoing either MAOR or AAOR, we found no association between surgical approach and risk of AVN or FCS. In addition, we identified no protective benefit of a preoperative ossific nucleus in terms of development of AVN. However, failing closed reduction was associated with a 6-fold increased annual risk of requiring FCS. SIGNIFICANCE: To the best of our knowledge, this is the first study comparing these 2 surgical techniques in an age-matched manner. It further corroborates previous studies stating that there may be no difference in risk of AVN based on surgical approach or presence of ossific nucleus preoperatively. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Likelihood of surgery in isolated pediatric fifth metatarsal fractures.

BACKGROUND: Fractures of the fifth metatarsal bone are common and surgery is uncommon. The "Jones" fracture is known to be in a watershed region that often leads to compromised healing, however, a "true Jones" fracture can be difficult to determine, and its impact on healing in pediatric patients is not well described. The purpose of this study was to retrospectively assess patterns of fifth metatarsal fracture that led to surgical fixation in an attempt to predict the likelihood for surgery in these injuries. METHODS: A retrospective review was performed on patients aged 18 and under who were treated for an isolated fifth metatarsal fracture from 2003 through 2010 at our pediatric hospital. Patient demographics, treatment, and complications were noted. Radiographs were reviewed for location of fracture and fracture displacement. Patients and fracture characteristics were then compared. RESULTS: A total of 238 fractures were included and 15 were treated surgically. Most surgical indications were failure to heal in a timely manner or refracture and all patients underwent a trial of nonoperative treatment. Jones criteria for fracture location were predictive of needing surgery (P<0.01) but confusing in the clinic setting. Fractures that occurred between 20 and 40 mm (or 25% to 50% of overall metatarsal length) from the proximal tip went on to surgery in 18.8% (6/32) of the time, whereas those that occurred between <20 mm had surgery in 4.9% (9/184). This was a statistically significant correlation (P=0.0157). CONCLUSIONS: Although fractures of the fifth metatarsal are common, need for surgery in these fractures is not. However, a region of this bone is known to have trouble healing, and it can be difficult to identify these "at-risk" fractures in the clinical setting. We found simple ruler measurement from the proximal tip of the fifth metatarsal to the fracture to help determine this "at-risk" group and found a significant difference in those patients with a fracture of <20 mm compared with those 20 to 40 mm from the tip; this can help guide treatment and counsel patients. LEVEL OF EVIDENCE: Level 3.

Medication use in pregnancy in relation to the risk of isolated clubfoot in offspring.

Clubfoot, a common major structural malformation, develops early in gestation. Epidemiologic studies have identified higher risks among boys, first-born children, and babies with a family history of clubfoot, but studies of risks associated with maternal exposures are lacking. We conducted the first large-scale, population-based, case-control study of clubfoot with detailed information on maternal medication use in pregnancy. Study subjects were ascertained from birth defect registries in Massachusetts, New York, and North Carolina during 2007-2011. Cases were 646 mothers of children with clubfoot without other major structural malformations (i.e., isolated clubfoot); controls were mothers of 2,037 children born without major malformations. Mothers were interviewed within 12 months of delivery about medication use, including product, timing, and frequency. Odds ratios were estimated for exposure to 27 medications in pregnancy months 2-4 after adjustment for study site, infant sex, first-born status, body mass index (weight (kg)/height (m)(2)), and smoking. Odds ratios were less than 1.20 for 14 of the medications; of the remainder, most odds ratios were only slightly elevated (range, 1.21-1.66), with wide confidence intervals. The use of antiviral drugs was more common in clubfoot cases than in controls (odds ratio = 4.22, 95% confidence interval: 1.52, 11.73). Most of these results are new findings and require confirmation in other studies.

Prenatal screening for clubfoot: what factors predict prenatal detection?

OBJECTIVE: Routine prenatal ultrasound has often resulted in the early detection of musculoskeletal disorders. The purpose of this study was to determine which socioeconomic factors are associated with prenatal detection of clubfoot. METHODS: The Slone Epidemiology Center at Boston University identified infants in three states (Massachusetts, New York, and North Carolina) who were reported as having a clubfoot. Mothers of these children were contacted and interviewed, and medical records obtained. Data were analyzed by using logistic regression to estimate odds ratios (ORs) and 95% confidence intervals (CIs). RESULTS: Overall detection of the clubfoot prenatally was 62.3% (421/676) but this varied considerably by state, 81.1% in Massachusetts (154/190), 58.5% in New York (124/212), and 52.2% in North Carolina (143/274). Multivariate analysis revealed that the strongest predictors for prenatal detection were maternal age ≥35 years (OR: 3.54), non-Hispanic black race (OR: 0.49), the presence of another birth defect (OR: 2.61), residing in Massachusetts (OR: 2.64), and the presence of a bilateral clubfoot (OR: 1.90). CONCLUSIONS: We found a statistically significantly higher rate of prenatal detection of clubfoot in Massachusetts and lower rate in younger mothers (age <35) and black mothers, even after adjustment for other sociodemographic variables.

Anesthetic considerations in Sheldon-Hall syndrome.

Arthrogryposis is characterized by multiple, nonprogressive joint contractures which may be caused by maternal disorders such as oligohydramnios as well as fetal akinesia resulting from primary disorders of muscle, connective tissue, or neurologic tissue. Its prevalence is about 1 : 3000. Distal arthrogryposis (DA) is a heterogenous group of genetic disorders with a characteristic flexion of the joints of the hands and feet divided into different types with additional features. Sheldon-Hall Syndrome (SHS), also known as distal arthrogryposis type 2A (DA2A), has some nonorthopedic features of specific importance to anesthetic care.

Loss-of-function mutations in PTPN11 cause metachondromatosis, but not Ollier disease or Maffucci syndrome.

Metachondromatosis (MC) is a rare, autosomal dominant, incompletely penetrant combined exostosis and enchondromatosis tumor syndrome. MC is clinically distinct from other multiple exostosis or multiple enchondromatosis syndromes and is unlinked to EXT1 and EXT2, the genes responsible for autosomal dominant multiple osteochondromas (MO). To identify a gene for MC, we performed linkage analysis with high-density SNP arrays in a single family, used a targeted array to capture exons and promoter sequences from the linked interval in 16 participants from 11 MC families, and sequenced the captured DNA using high-throughput parallel sequencing technologies. DNA capture and parallel sequencing identified heterozygous putative loss-of-function mutations in PTPN11 in 4 of the 11 families. Sanger sequence analysis of PTPN11 coding regions in a total of 17 MC families identified mutations in 10 of them (5 frameshift, 2 nonsense, and 3 splice-site mutations). Copy number analysis of sequencing reads from a second targeted capture that included the entire PTPN11 gene identified an additional family with a 15 kb deletion spanning exon 7 of PTPN11. Microdissected MC lesions from two patients with PTPN11 mutations demonstrated loss-of-heterozygosity for the wild-type allele. We next sequenced PTPN11 in DNA samples from 54 patients with the multiple enchondromatosis disorders Ollier disease or Maffucci syndrome, but found no coding sequence PTPN11 mutations. We conclude that heterozygous loss-of-function mutations in PTPN11 are a frequent cause of MC, that lesions in patients with MC appear to arise following a "second hit," that MC may be locus heterogeneous since 1 familial and 5 sporadically occurring cases lacked obvious disease-causing PTPN11 mutations, and that PTPN11 mutations are not a common cause of Ollier disease or Maffucci syndrome.

Satisfactory patient-based outcomes after surgical treatment for idiopathic clubfoot: includes surgeon's individualized technique.

INTRODUCTION: Treatment of idiopathic clubfoot has shifted towards Ponseti technique, but previously surgical management was standard. Outcomes of surgery have varied, with many authors reporting discouraging results. Our purpose was to evaluate a single surgeon's series of children with idiopathic clubfoot treated with a la carte posteromedial and lateral releases using the Pediatric Outcomes Data Collection Instrument (PODCI) with a minimum of 2-year follow-up. METHODS: A total of 148 patients with idiopathic clubfoot treated surgically by a single surgeon over 15 years were identified, and mailed PODCI questionnaires. Fifty percent of the patients were located and responded, resulting in 74 complete questionnaires. Median age at surgery was 10 months (range, 5.3 to 84.7 mo), male sex 53/74 (71.6%), bilateral surgery 31/74 (41.9%), and average follow-up of 9.7 years. PODCI responses were compared with previously published normal healthy controls using t test for each separate category. Included in the methods is the individual surgeon's operative technique. RESULTS: In PODCIs where a parent reports for their child or adolescent, there was no difference between our data and the healthy controls in any of the 5 categories. In PODCI where an adolescent self-reports, there was no difference in 4 of 5 categories; significant difference was only found between our data (mean = 95.2; SD = 7.427) and normal controls (mean = 86.3; SD = 12.5) in Happiness Scale (P = 0.0031). DISCUSSION: In this group of idiopathic clubfoot patients, treated with judicious posteromedial release by a single surgeon, primarily when surgery was treatment of choice for clubfoot, patient-based outcomes are not different from their normal healthy peers through childhood and adolescence. While Ponseti treatment has since become the treatment of choice for clubfoot, surgical treatment, in some hands, has led to satisfactory results. LEVEL OF EVIDENCE: Level III.