Congenital vertical talus in multiple pterygium syndrome

BACKGROUND: Congenital vertical talus (CVT) is a rare foot deformity, but it is a commonly associated anomaly in patients with multiple pterygium syndrome (MPS). If left untreated, it can cause pain and morbidity, which will affect the patient's ambulation and quality of life. The aim of this study was to assess the prevalence of CVT among patients with MPS, to characterize the clinical and radiological features and examine the outcome of treatment. METHODS: We reviewed the medical records from 1969 to 2009, and detected 14 patients with a diagnosis of MPS. Data regarding clinical findings, radiographs, associated anomalies, and treatment were collected and analyzed. RESULTS: CVT was seen in 10 of 14 patients (71%). All of them had bilateral involvement. Eight of the 10 (80%) were girls, and 3 of these 10 (30%) were nonambulatory patients. All 7 ambulatory patients had manipulation and casting, followed by a single-stage surgical release. The mean age at surgery was 3.0±3.7 years (range, 3 mo-9 y 2 mo). At the last follow-up, all of the 7 patients (100%) had painless plantigrade feet and a reduced talonavicular joint, and none had recurrence of the deformity. The overall mean follow-up was 6 years (range, 2-19 y) and the mean age at the last follow-up was 9 years (range, 2-23 y). The commonly associated anomalies were scoliosis (93%), tethered cord (14%), hip dislocation (43%), cardiac (29%), respiratory (43%), and gastrointestinal anomalies (29%). CONCLUSIONS: CVT is common in MPS. The other common anomalies included scoliosis, hip dislocation, and respiratory problems. Treatment with manipulation and casting followed by, a single-stage surgical release resulted in a good outcome.