Volume of tarsal bones in congenital clubfoot

To elucidate the growth of the tarsal bones in congenital clubfoot, relative to the growth of these bones in the unaffected feet and compared to growth in the feet of normal volunteers, we used a computed tomography (CT) scanner to measure the volume of all tarsal bones. The subjects of the study were 10 adults (7 men and 3 women) with unilateral congenital clubfoot (average age 20 years and 1 month). As controls, we examined 11 healthy volunteers. We calculated the ratio of the volume of each tarsal bone to the total bone volume and the ratio of the volume of each tarsal bone in clubfoot to the corresponding bone in the unaffected foot. The volume ratio of each tarsal bone was compared between clubfeet and unaffected feet because the differences of each tarsal bone ratio between the normal foot group and unaffected foot group were not significant. In the clubfeet (n=10), the talus and the medial cuneiform bones were smaller than those in the unaffected feet (n=10) but the cuboid bone was larger. The growth of the navicular did not differ from as that in unaffected feet. Our results suggested hypoplasia on the medial side of the foot in adult patients with congenital clubfoot. The 3 patients who had undergone medial release showed particularly marked hypoplasia of the medial side. In congenital clubfoot cases with severe deformities who had undergone wide soft-tissue release operations, there were clear growth suppressions in the talus and the medial cuneiform. We could not determine whether the cause of the growth suppression was the hypoplastic nature of tarsal bones themselves or the surgical obstacles to tarsal bone growth.     

Evaluation of the treatment of idiopathic clubfoot by using the Ponseti method

The initial treatment of congenital idiopathic talipes equinovarus (clubfoot) is most often nonsurgical. However, surgical treatment in the form of posteromedial release is often undertaken after failure of conservative measures. The prevalence of both immediate and long-term complications in surgically treated clubfeet has cultivated a renewed interest in nonsurgical treatment. The Ponseti method for treating clubfoot has seen a revived interest among those caring for infantile clubfeet. We report on our first 34 infants (57 clubfeet) treated by using the techniques and principles described by Ponseti. Using a standard scoring system, 54 of 57 clubfeet were successfully corrected without requiring posteromedial release. Only 2 patients (3 clubfeet) required extensive surgical correction. There were 6 relapses. In all recurrent cases, there was a lack of compliance with the straight-last shoe and foot abduction bar regimen. Based on this level of initial success, we believe that posteromedial release is no longer necessary for the majority of cases of congenital clubfeet.     

Peroneal muscular atrophy with Poland syndrome

A 22-year-old male college student had a syndactyly between the second and third fingers of his left hand, which was congenitally small in size. His left pectoralis muscles were absent. He first walked at the age of 12 months, but soon developed difficulties in walking due to weakness of the legs. Atrophy and weakness of the legs aggravated gradually. He was diagnosed as having peroneal muscular atrophy and Poland syndrome, an association of which has not been reported before. A small number of similar cases of peroneal muscular atrophy with various skeletal abnormalities in the literature suggest that the association is not incidental, but of clinical significance.     

Congenital Pseudarthrosis of Femur and Sciatic Nerve Palsy with Congenital Constriction Band Syndrome: Report of the First Case in Literature

Congenital constriction band syndrome has varied clinical presentations ranging from small, incomplete skin deep constriction band to in utero amputation. Pseudarthrosis of underlying bone most commonly tibia has been reported by many authors. We report the first case of congenital pseudarthrosis of the femur with congenital constriction band syndrome. Nine-day-old female presented with the constriction band in the left thigh with open pseudarthrosis of the femur. The left femur had gross recurvatum deformity and the posterior apex of the pseudarthrosis was exposed via skin ulceration. She had an ipsilateral paralytic clubfoot. She was treated with single-stage excision of constriction band and Z-plasty. Spontaneous union of the femur was achieved at 3 months. Procurvatum deformity of the femur improved gradually over 3 years. This happens to be the first and only reported case of congenital pseudarthrosis of the femur with sciatic nerve palsy due to congenital constriction band.     

Experimental approach to the pathogenesis of the anomalies of amniotic disease

By intra-adnexal injection of glucose in the rabbit embryo, we were able to stimulate all the anomalies associated with "Amniotic Disease". Since we were even able to obtain amniotic bands, this study provides an excellent experimental model of this disease. Resulting lesions occur early in development, corresponding to the first trimester of human gestation. All of the anomalies can ultimately be explained by the destruction of the most superficial cells: epiblastic cells of the embryo and the amnion, subjacent mesenchyme, and endothelial cells. The subsequent lack of interaction between these cells and the importance of the anatomical localizations of resulting hematomas can lead to the pathogenetic approach to this disease. In light of the present study, the disease appears to be caused by an external factor within the amniotic fluid. The exact nature of the destructive agent(s) remains a mystery in man.     

Accelerated Ponseti method: First experiences in a more convenient technique for patients with severe idiopathic club feet

BackgroundCongenital Idiopathic Talipes Equinovarus (CTEV), or clubfoot, is a complex deformity that involves pathological anatomy in the foot with ankle equinus, hindfoot varus, midfoot cavus and forefoot adductus [1]. Universal agreement is established about Ponseti technique as the initial management for this deformity. This preliminary study aims to investigate the possibility of having a braceable foot through a proposed accelerated Ponseti method by which, manipulations, 5 castings and Achilles tendon tenotomy are implemented in a week.MethodsThis study included 11 patients with 16severe congenital idiopathic clubfeet treated by an accelerated Ponseti method. The method involves manipulation of the deformed foot, and 1st casting in one day, with the 2nd, 3rd, 4th, 5th castings in the 4th, 5th, 6th, 7th day post-manipulation. After the 4th cast removal, Achilles tenotomy was performed with subsequent three-week casting for all patients. Nonparametric tests were used for comparing the Pirani scores before starting the treatment and after removal of final cast.ResultsFive patients had bilateral club foot deformity. Average age at treatment was 54.8 days (range 8–150 days). All patients, who had severe congenital idiopathic club feet with a Pirani score of 6, underwent the accelerated Ponseti technique. After removal of the three-week cast, the scores median was 0.59, (range 0–1.5), indicating a correction of the deformity and having braceable feet in all patients without experiencing any short-term complication.ConclusionsThe first step accelerated Pnoseti technique was found to be safe and effective for initial correction of severe idiopathic clubfoot deformity in children below three months of age , though it is an initial study that needs more studies with more follow up data.     

Results of Modified Ponseti Technique in Difficult Clubfoot and a review of literature

IntroductionSerial Ponseti casting achieves deformity correction in early presenting idiopathic clubfoot cases normally in around 7 casts. However, there are resistant patients where correction requires more casts than usual. In such patients a modification in standard technique might be required right from the beginning. Such patients were collectively called as difficult clubfoot. The aim of this study was to assess the outcome of our modification to Ponseti technique in difficult clubfoot.MethodsAll idiopathic clubfoot cases who were 75th percentile or more in WHO age for weight chart (chubby infants) or untreated clubfoot patients presenting for first time to our clinic at more than 5 months age (late presenters and neglected cases) were included in the study. Patients who had been previously surgically intervened elsewhere, patients over 7 years of age, patients with syndromic clubfoot or clubfoot associated with neurological conditions were excluded from the study. The patients were treated by early tenotomy of tendoachillis and a plantar fascia release before starting serial casting by Ponseti technique. Post correction, strict bracing protocol was followed with regular follow up. Pirani scoring was done at each stage. Measurement of Talocalcaneal angle on AP radiograph, maximum degree of abduction and dorsiflexion was noted once every year.ResultsThere were total 28 patients in our study. In all, 47 feet were subjected to modified Ponseti protocol. There were 21 male patients. Median age at presentation was 4 months. Mean centile of weight for age as per WHO growth chart was 64. Mean Pirani score at presentation was 5.86 (S.D. ± 0.34). Mean number of casts required for correction was 3.75 ± 1.10. Maximum followup period was 25 months.ConclusionThis modification of Ponseti casting for difficult clubfoot patients achieves correction in shorter duration with less number of casts.