BackgroundThe prevalence of untreated congenital clubfoot among children older than walking age is higher in developing countries due to limited resources for early care after birth. The Ponseti method represents an intervention option for older, untreated children.MethodsA metanalysis was conducted of observational studies selected through a systematic review of articles included in electronic databases (Medline, Scopus, Embase, Lilacs, and the Cochrane Library) until June 2017. A pooling analysis of proportions with 95% confidence intervals (CIs) and a publication bias assessment were performed as routine. Estimates of success, recurrence, and complication rates were weighted and pooled using the random effects model.ResultsTwelve studies, including 654 feet diagnosed with congenital clubfoot in children older than walking age (older than 1 year old), were included for analysis. The rate of satisfactory outcomes found via a cluster metanalysis of proportions using the random effects model was 89% (95% CI = 0.82–0.94, p < 0.01), relative to the total analysed. The recurrence rate was 18% (95% CI = 0.14–0.24, p = 0.015), and the rate of casting complications was 7% (95% CI = 0.03–0.15, p = 0.19).ConclusionApplication of the Ponseti method in children with untreated idiopathic clubfoot older than walking age leads to satisfactory outcomes, has a low cost, and avoids surgical procedures likely to cause complications. The results obtained exhibited considerable heterogeneity.

Gómez-López-Hernández (GLH) syndrome or cerebello-trigeminal dysplasia is a neurocutaneous syndrome whose etiology is unknown at the present time. We report two additional Brazilian patients, including the oldest one known to date (age 29). Here, we review the expanded phenotype in four patients with new clinical, psychiatric, radiological, and molecular investigations. One patient may have hypomania within the bipolar spectrum disorder with onset in childhood and adolescence. Primary growth hormone (GH) deficiency was ruled out in all patients, although one of them might have developed secondary GH deficiency due to partial hypopituitarism following severe hydrocephalus. Brain magnetic resonance angiography disclosed no azygous anterior cerebral artery (ACA) but only normal variants. Molecular analysis of the lysosomal acid phosphatase gene (ACP2) was performed, but no pathogenic mutations were identified. We present an overview of the phenotypic features of all patients described to date. There are currently 12 unrelated patients reported in the literature, 5 of whom are Brazilian. We discuss new molecular insights and speculate about the pathogenesis of GLH syndrome.

The Ponseti technique for treating clubfoot has been popularized for idiopathic clubfoot and more recently several syndromic causes of clubfoot. We asked whether it could be used to treat recurrent clubfoot following failed posteromedial release. We retrospectively reviewed 58 children (83 clubfeet) treated by the Ponseti technique for recurrent deformity after posteromedial release in three centers. The minimum followup was 24 months (average, 45 months; range, 24-80 months). We determined initial and final Pirani scores and range of motion of the ankle and subtalar joint. Plantigrade and fully corrected feet were obtained in 71 feet (86%); 11 feet obtained partial correction; one patient failed treatment and underwent another posteromedial release. Recurrences occurred in nine

Background: Complex clubfoot is a term used to describe those feet that present after treatment with a short first metatarsal, severe plantar flexion of all metatarsals, rigid equinus, and deep folds through the sole of the foot and above the heel. Ponseti has described a modification of his original technique for the treatment of the deformity. Few series have reported the treatment outcomes of this group of patients. The purpose of this study is to analyze mid-term results and complications of a large multicenter cohort. Methods: Patients with complex clubfoot treated at 6 tertiary-care institutions with a minimum of 1-year follow-up were retrospectively analyzed. Demographic data, previous treatment, number of casts, Achilles tenotomy, recurrences, complications, and additional procedures were documented. The patients were clinically evaluated at the time of presentation, after treatment, and at the last follow-up according to the Pirani score. All variables had a nonparametric distribution and are thus described as median (interquartile range (IQR), minimum-maximum). A comparison between the variables was performed using a Mann-Whitney U test, the change within each group was performed with a Wilcoxon-designated range test. A P-value <0.05 was used to indicate statistical significance. Results: One hundred twenty-four feet (79 patients) were evaluated. The median age at initial treatment was 7 months (IQR, 15; min-max, 1 to 53 mo). The mean follow-up was 49 months (IQR, 42; min-max, 12 to 132 mo). A median of 5 casts (IQR, 5; min-max, 3 to 13) was required for correction. Percutaneous tenotomy of the Achilles tendon was performed in 96% of the feet. One hundred twenty-two feet (98%) were initially corrected; 2 feet could not be corrected and required a posteromedial release. The Pirani score improved significantly from a pretreatment mean of 6 points (IQR, 1; min-max, 4.5 to 6) to 0.5 (IQR, 0.5; min-max, 0 to 2.5) at the last follow-up (P <0.001). Seven feet (6%) presented minor complications related to casting. Relapses occurred in 29.8% (37/124). In this subgroup, the number of casts required at initial treatment was higher (6; IQR, 5; min-max, 1 to 12 vs. 4 IQR, 4; min-max, 1 to 13; P<0.001), and follow-up was significantly longer (62 mo; IQR, 58; min-max, 28 to 132 vs. 37 mo; IQR, 48, min-max, 7 to 115; P<0.001). Conclusions: Ponseti method is safe and effective for the correction of complex clubfeet. Early diagnosis and strict adherence to the Ponseti principles are key to achieve deformity correction. Patients with complex clubfoot require frequent follow-up because of a higher recurrence rate. Level of Evidence: Level III—therapeutic study.