Prevalence of unsuspected myopathy in infants presenting for clubfoot surgery

The objective of this study was an evaluation of the prevalence of myopathies in paediatric patients scheduled for orthopaedic surgery (clubfoot) performed under regional anaesthesia. Seventeen infants scheduled for lower limb orthopaedic surgery were studied to verify coexisting neuromuscular disorders with electromyography and muscle biopsy during surgery. All surgical procedures were performed under caudal block or spinal anaesthesia, associated with light general anaesthesia. No major cardiorespiratory, neurological or malignant hyperthermic complications (muscle rigidity, arrhythmias, hyperpyrexia) were observed. Combined neurological, electromyographic and biopsy studies showed a high rate of myopathic changes (70%). Performance of clubfoot surgery under light general anaesthesia with regional techniques was free from any problems. The high rate of myopathic changes (70%) observed in the muscle biopsies suggests that precautions should be taken with paediatric patients for clubfoot surgery and a regional anaesthesia technique with adequate monitoring may be helpful to prevent possible malignant hyperthermia related problems.     

Late recurrence of clubfoot deformity: a 45-year followup

Idiopathic clubfoot is one of the most common congenital deformities. Regardless of the mode of treatment, clubfoot has a tendency to relapse until the age of 5 years. Relapses are rare in patients after 5 years of age. A 45-year followup of a patient with idiopathic clubfoot treated as a newborn with the Ponseti method is reported. The patient had both clubfeet well-corrected as seen clinically and radiographically with this method. However, recurrent deformities developed bilaterally when the patient was 8 years old. Because of the late recurrence of this deformity, the patient had a thorough neurologic evaluation, which was normal. Treating physicians should be aware of the possibility of late recurrence in patients who have had complete correction of their clubfoot deformity.     

Neuro-myopathic flexion deformities of the cervical spine

A study is presented of six patients who suffered flexion (chin-on-chest) deformity of the cervical spine on a neuropathic or myopathic basis. An awareness of this possibility is recommended, differentiating these patients from those with similar deformities due to ankylosing spondylitis, trauma, or primary degenerative change. Recognition is based on a detailed history, weakness of neck extension, electrodiagnostic studies, and muscle biopsy. A mildly elevated creatine phosphokinase (CPK) was the only consistent laboratory finding. Appropriate surgical correction of severe deformity involves anterior surgical release of contracted sternomastoid muscles, halo-dependent traction, posterior vertebral inferior facet resection, and spinal fusion over an adequate length, supplemented with internal fixation. Where extension correction involves extensive vertebral body separation anteriorly, additional anterior keystone strut grafting is indicated.     

Accessory soleus muscle in clubfoot deformity: a report in four feet

The commonest presentation of accessory soleus muscle is a swelling at the posteromedial aspect of the ankle in adolescents or young adults. Accessory soleus is rarely encountered in children undergoing surgical release for congenital clubfoot, and only a few isolated reports are available in the literature. The purpose of this study is to heighten awareness about the role of accessory soleus muscle in clubfoot deformity. Four cases of accessory soleus muscle in patients undergoing surgical release for clubfoot deformity are reported here in which, a distinct anomalous muscle deep to the tendoachilles was identified. Hindfoot varus and equinus persisted in each of these cases despite an adequate posteromedial soft tissue release, which could be corrected only on tenotomizing the tendon of the accessory soleus muscle at its insertion. An awareness about the accessory soleus muscle is important, particularly when non-operative methods of clubfoot management with tendoachilles tenotomy or limited surgery are employed. Failure to recognize this muscle if present in patients with congenital clubfoot may lead to persistent hindfoot deformity. A high index of suspicion should be maintained in cases in which hindfoot deformity persists despite an otherwise adequate soft tissue correction.     

Evertor muscle activity as a predictor for recurrence in idiopathic clubfoot

Background

Clubfoot is a complex three-dimensional deformity. Although brace compliance after initial correction was previously found to be significantly associated with recurrence in clubfoot, few previous studies have specifically examined evertor muscle function as a factor that contributes to recurrence in children with idiopathic and non-idiopathic clubfoot. The aim of this study was to investigate the relationship among brace compliance, evertor muscle grading, and recurrence rate in pediatric clubfoot patients.

Methods

Children with idiopathic clubfoot who were treated and followed for a minimum of 2 years were included. Patients who used their brace <20–23 h a day for the first 3 months and then <8–10 h per day during sleep and nap times thereafter were classified as group I. Patients who complied fully by using the brace 23 h a day for the first 3 months and then 8–10 h per day during sleep and nap times thereafter were classified as group II. Demographic and clinical data including age, gender, follow-up time, recurrence, evertor muscle grading, types of surgery, brace compliance, severity of initial deformity, age at onset, number of casts required for initial correction, and the need for Achilles tenotomy were collected and analyzed.

Results

Seventy-nine children with clubfoot were included. There were 47 males and 32 females, mean age was 3.2 years (range 2.1–6.3), and the mean follow-up time was 31.4 months. All patients had follow-up of at least 2 years. Primary correction was obtained in all children. There was no significant difference in mean age, mean follow-up time, or recurrence rate between groups. There was, however, a statistically significant difference in mean brace time between groups (p = 0.002). The recurrence rate was 26.2% in group I and 22.2% in group II. The recurrence rate in group a (Pirani score 0) was 3.9%, group b (Pirani score 0.5) 43.8%, and group c (Pirani score 1) 75% (p < 0.001). No significant association was found between severity of the initial deformity, age at the onset of treatment, number of casts required for correction, or reported brace compliance and recurrence or rates of surgery. Only poor or absent evertor muscle activity was found to be statistically significantly associated with risk of recurrence.

Conclusion

Good evertor muscle grading was found to be a significant protective factor against recurrence of idiopathic clubfoot. Thus, improvement in muscle balance around the ankle, especially the evertor muscle, should be emphasized to parents after the casting regimen is completed and correction is achieved.

     

Diastematomyelia presenting as progressive weakness in an adult after spinal fusion for adolescent idiopathic scoliosis.

BACKGROUND CONTEXT: Diastematomyelia is uncommon and rarely presents in adulthood. This report draws attention to the fact that patients who underwent spinal fusion for deformity before the widespread use of computed tomography (CT) and magnetic resonance imaging (MRI) may have unrecognized spinal cord abnormalities. This should be considered if revision surgery is contemplated. PURPOSE: This case report focuses on the late presentation of lower-extremity weakness in a 44-year-old woman with a split cord malformation (diplomyelia), diastematomyelia and tethered cord syndrome. STUDY DESIGN/SETTING: METHODS: The patient underwent instrumented posterior spinal fusion with a Harrington rod as a child for progressive thoracolumbar scoliosis. As an adult, she developed paraparesis after a traumatic event.The patient underwent decompressive laminectomy, subtotal resection of the old fusion mass and resection of the osseous septum. Postoperatively, an anterior spinal fluid leak in the lower thoracic region required repeated fascial grafting, resection of a pseudomeningocele and reverse left latissimus dorsi flap transfer. The leak was controlled, and the patient had near complete resolution of her paraparesis 1 year after her surgery. RESULTS: The case described herein is unusual in that patients with diplomyelia and diastematomyelia rarely are symptomatic in adulthood. However, trauma may precipitate the onset of neurologic symptoms. This patient underwent spinal surgeries to address deformity, pain and progressive lower-extremity weakness. Preoperative CT and MRI studies showed a split cord malformation and diastematomyelia at L1-L2 with spinal stenosis and tethering of both hemicords. CONCLUSIONS: Progressive weakness without any previous neurologic deficit or neurocutaneous stigmas of an underlying spinal cord abnormality may develop in the adult with unrecognized diastemotomyelia. This case demonstrates that a thorough preoperative workup of patients with complex spinal deformities is imperative.     

Dorsal bunion after clubfoot surgery: outcome of reverse Jones procedure

BACKGROUND: The dorsal bunion deformity consists of the elevation of first metatarsal head, plantar flexion contracture at the first metatarsophalangeal joint, and dorsiflexion contracture of the tarsometatarsal joint. A reverse Jones procedure with transfer of the flexor hallucis longus to the metatarsal head has been an effective method in correcting this deformity. METHODS: This is a retrospective review of 27 patients with 33 feet who had reverse Jones procedure with or without metatarsal osteotomy between 1983 and 2002. All patients had previous soft tissue releases for clubfoot deformity. Clinical reviews included muscle function test and radiographic evaluation before and after procedures. We used the American Orthopaedic Foot and Ankle Society Hallux Metatarsophalangeal-interphalangeal scale for functional outcome results. RESULTS: The average follow-up was 4.96 years. There were 21 boys and 6 girls. Average age at time of procedure was 13.7 years. With the reverse Jones procedure, there were 18 first metatarsal osteotomies and 12 split anterior tibial tendon transfers. Before surgery, decreased muscle strength in triceps surae (73%), tibialis posterior (76%), peroneus longus (67%), and extensor hallucis longus (76%) was noted. Patients (84.9%) had normal tibialis anterior and flexor hallucis longus power. In radiographic evaluations, the operation resulted in decreased elevation of the first metatarsal by measuring the metatarsal-horizontal angle. The lateral metatarsophalangeal angle improved from 23 degrees plantar flexion to 1 degree in dorsiflexion. The average global American Orthopaedic Foot and Ankle Society Hallux Metatarsophalangeal-interphalangeal score was 70 preoperatively and 92 postoperatively with improvement of subscores in pain, activity, footwear, range of motion, callus, and alignment. CONCLUSIONS: Dorsal bunion is a recognized long-term complication after clubfoot surgery. The causes of the deformity are weakness of Achilles tendon, overpowering of flexor hallucis longus, and strong anterior tibial tendon with weakness of peroneus longus. The reverse Jones procedure improved the condition in this series and provided a long-lasting and effective correction of the dorsal bunion deformity. LEVEL OF EVIDENCE: Level 4.     

Peroneal Nerve Dysfunction in Patients with Clubfoot Deformity: Evaluation of Clinical Presentation and Treatment

BackgroudComplete peroneal nerve dysfunction associated with congenital clubfoot is uncommonly reported. Our retrospective study highlights the recognition of clinical presentation and mid-term outcomes of treatment in these patients.MethodsEight out of 658 patients undergoing treatment for clubfoot were identified with unilateral complete peroneal nerve dysfunction associated with congenital clubfoot. Three patients presented primarily to our center; 5 were treated elsewhere initially. All patients were treated with Ponseti casts, Achilles tenotomy, and subsequent foot abduction bracing. Diagnosis of complete peroneal nerve dysfunction was confirmed using nerve conduction velocity studies in all patients. After full-time bracing, an insole polythene molded ankle foot orthosis was given. Three patients underwent tibialis posterior transfer to improve foot dorsiflexor power.ResultsThe mean age at presentation was 1.3 years (range, 1 week–5 years). All patients had prominence of lateral 3 metatarsal heads and dimpling of intermetatarsal spaces. At a mean follow-up of 5.1 years, mean shortening of 1.2 cm in tibia (range, 1–2.5 cm) and mean calf wasting of 4.4 cm were observed. There was no relapse of any clubfoot deformity till the final follow-up.ConclusionsProminence of lateral metatarsal heads and dimpling of intermetatarsal spaces should raise early suspicion of peroneal nerve dysfunction. Standard Ponseti protocol is useful in treatment of these patients. Tibialis posterior transfer to dorsum partially restores the ankle dorsiflexion.     

Flexor digitorum accessorius longus muscle is associated with familial idiopathic clubfoot

The etiology of idiopathic and familial clubfoot remains elusive. The purpose of this study was to document the prevalence of the flexor digitorum accessorius longus muscle in a large series of children treated with an extensive soft tissue release for idiopathic clubfoot deformity and compare those patients with and without this anomalous muscle in terms of various demographic data, including a positive family history of clubfoot. A retrospective review was conducted to identify infants treated with an extensive soft tissue release for idiopathic clubfoot deformity at the authors' institutions between 1980 and 2000. Patient characteristics and family demographic data were analyzed using multiple logistic regression analysis modeling the probability of having the anomalous flexor muscle. A flexor digitorum accessorius longus muscle was identified in 55 (6.6%) of the 835 patients at the time of surgical correction of the clubfoot deformity. It was present in 4.5% of patients without a family history (33/741) and 23.4% of patients with a family history (22/94) (P < 0.0001). Children with first-degree relatives with clubfoot are 6.6 times more likely to have the anomalous flexor muscle than children without first-degree relatives with clubfoot (odds ratio 6.6; 95% confidence interval 3.63-11.84; P < 0.0001).     

Anterior stabilization, instrumentation, and decompression for post-traumatic kyphosis

Thirty-seven patients underwent surgery for late post-traumatic kyphosis in the lumbar, thoracolumbar, or thoracic spine. Indications for surgery included: increasing deformity, pain, and persistent neurologic deficit with paraparesis in eight, and development of late spinal stenosis in a further nine patients. All patients underwent anterior correction with Kostuik-Harrington instrumentation. Seventeen patients with neurologic deficit underwent decompression over appropriate levels as well. No posterior fusions or instrumentation were carried out. Stable arthrodesis with correction of the deformity occurred in 36 of 37 patients with only one nonunion. Pain was reduced significantly in 78% of patients. Late neurologic improvement of a significant functional degree occurred in three of eight paraparetics. All patients with spinal stenosis had relief of their symptoms and signs.     

Resistant talipes equinovarus associated with congenital constriction band syndrome

Thirty-seven clubfeet in 28 patients with concomitant congenital constriction band syndrome were reviewed. The bands were considered to be of significance if located in the calf region (zone 2). The severity of the bands was classified. Grade 1 bands involved subcutaneous tissue, grade 2 bands extended to the fascia, grade 3 bands extended to the fascia and required release, and grade 4 bands were congenital amputations. The patients were divided based on neurologic deficit. Group A consisted of 26 clubfeet without neurologic deficit and had 1.4 surgeries per clubfoot. Group B consisted of 11 clubfeet with neurologic deficit and had 3.7 surgeries per clubfoot. Children with grade 3 bands in zone 2 were most likely to have a neurologic deficit. Twenty-three clubfeet had a good clinical result, seven clubfeet fair, and seven clubfeet poor. Group B had poorer results than Group A. These feet ultimately required numerous and more extensive surgeries to obtain correction, and ultimately had a poorer result.     

Ponseti Idiopathic and Nonidiopathic Clubfoot Correction With Secondary Surgeries

The Ponseti method has revolutionized clubfoot treatment for not only idiopathic clubfoot but also non-idiopathic clubfoot. This study aimed to validate the existing literature with respect to the Ponseti method serving as first line treatment for clubfoot. The purpose of this study was to compare clubfoot type and recurrence with secondary surgical procedures following Ponseti method. Kaiser Permanente Northern California database was queried to identify clubfoot children under 3 years old with a consecutive 3-year membership. Associated comorbidities and operative procedure codes were identified. Chart review was performed on all surgical clubfoot patients who completed Ponseti method. Patients’ average age at time of surgery, frequency of surgeries, and types of procedures performed were recorded. A logistic regression analysis assessed the adjusted association between surgery status and clubfoot type. Clubfoot incidence was about 1 in 1000 live births. Of the 375 clubfoot children, 334 (89%) were idiopathic and 41 (11%) were non-idiopathic. In the total study population, 82% (n = 309) patients maintained Ponseti correction without a secondary surgery; 66 patients (18%) underwent subsequent secondary surgeries. The non-idiopathic clubfoot underwent surgery more frequently compared to idiopathic clubfoot patients (41.5% vs 14.7%, respectively, p = .0001). Non-idiopathic clubfoot children underwent surgery at a younger age. This study validates the Ponseti method is the first line treatment for clubfoot correction despite etiology. However, patients with recurrent clubfoot may require secondary surgery following Ponseti method. Clubfoot recurrence surveillance is key for identifying early symptomatic recurrence in order to minimize foot rigidity and the need for osseous procedures.     

Midterm results following revision surgery in clubfeet

Relapse rates of surgically treated clubfeet are about 25%. We reviewed 43 patients (57 feet) treated for relapsed clubfoot deformity between 1992 and 2001 in our department. The average age of the patients at the time of revision surgery was 5.1 years, the mean follow-up was 6.6 years. Surgical therapy was performed using an algorithm according to age groups. The mean Atar score at follow-up was 77 points, representing a good outcome. Out of 57 feet, 20 (35%) were rated excellent, 24 (42%) good, 5 (9%) fair, and 8 (14%) poor. The number of previous surgical interventions had no influence on the outcome. Using an age related surgical algorithm, good postoperative results could be achieved in most of our patients, thus improving their functional situation. This emphasises the usefulness of the proposed algorithm in the difficult situation of recurrent clubfoot, while thorough analysis of the underlying deformity remains essential.     

Occipitocervical fusion in patients with rheumatoid arthritis

Instability and deformity of the cervical spine caused by rheumatoid arthritis is a well known entity. Operative intervention is indicated for patients with progressive deformity and when pain is resistant to conservative treatment. In a series of 39 patients who underwent posterior occipitocervical fusion with a Y plate, 22 patients were observed clinically and radiographically at average 41.5 months after surgery. In 35 of the 39 patients the main indication for surgery was pain, and in 30 of the 39 patients additional neurologic deficit (radiculopathy or myelopathy) was present. Thirty-one of the 39 patients had atlantoaxial instability. The atlantoaxial instability was associated with cranial migration of the dens in 19 patients. According to the classification of Conaty and Mongan 77.3% patients had satisfactory results and 22.7% had unsatisfactory results. Of the 30 patients with neurologic deficit, nine patients had a significant improvement. No patient had a worse result after surgery. Solid fusion was seen in all 22 patients at followup. Seven patients experienced complications directly related to the surgical procedure. Posterior fixation combined with anterior decompression in the presence of spinal stenosis represents a useful and safe method to treat instability and deformity caused by rheumatoid arthritis. Early surgical procedures may reduce the complication rate.     

Radical soft-tissue release of the arthrogrypotic clubfoot

The purpose of this study was to evaluate the results of primary radical soft-tissue release of arthrogrypotic clubfeet in children less than 1 year of age. We performed a retrospective review of six patients (12 feet) who underwent radical release of clubfoot deformity. Primary surgery was performed at an average of 7.4 months and the average follow-up was 4.3 years. We graded our results using a modified functional clubfoot rating system. We had two excellent, four good, three fair and three poor results. Revision surgery was performed on one foot for residual equinus. All patients ambulated independently with orthoses. Our short-term results with primary radical release of clubfoot deformity in arthrogryposis in infants under 1 year of age have been very encouraging. Correction of hindfoot equinus is excellent, and the recurrence rate remains low. Salvage talectomy has not been necessary in this population of children.     

Neuromuscular issues in cavovarus foot

Differential muscle weakness can cause a cavus foot deformity. Presenting complaints in the hindfoot may include ankle instability, secondary arthritis, or peroneal tendonitis. Presenting complaints in the forefoot may include stress fractures, callus formation over the lateral border of the foot, claw toes, first ray overload, and metatarsalgia. More general presenting complaints can include a drop-foot gait, decreased walking tolerance, and difficulty with shoe or orthotic fitting. To surgically correct the foot shape, soft tissue contractures need to be released, bone deformity corrected, and muscles balanced to optimize their strength and prevent recurrence of the deformity. This article reviews the diagnosis and management of the cavovarus foot secondary to longstanding muscle imbalance.     

An Unusual Aberrant Muscle in Congenital Clubfoot: An Intraoperative Finding

Congenital clubfoot is a common congenital deformity, characterized by equinus of the hindfoot and adduction of the midfoot and forefoot, with varus through the subtalar joint complex. A cavus deformity will also be present. The etiology of this congenital deformity remains elusive. Muscle anomalies are not commonly found in patients with idiopathic clubfoot, and, when present, their significance is not clear. The presence of a flexor digitorum accessorius longus muscle and an accessory soleus muscle found at surgical correction of clubfoot deformity has been previously reported. Our case was a female child, aged 2 years, 3 months, who developed bilateral relapsed congenital clubfoot. She was found to have an unusual aberrant muscle in both legs. This was discovered accidentally during surgical correction of her deformity through posteromedial soft tissue release. This muscle might have contributed to the hindfoot varus and equinus in the clubfoot deformity, because the latter were completely corrected after release of the muscle from its insertion. Awareness of such a new anatomic variant, with the other anatomic variants found in clubfoot deformity, will not only improve our understanding of normal lower limb development, but could also lead to improved genetic counseling and diagnostic and treatment methods of such a common congenital deformity.     

Management of idiopathic clubfoot in toddlers by Ponseti's method

The Ponseti method has been reported to have successful results in clubfoot patients less than 6 months of age but the literature on its efficacy in older clubfoot patients still remains sparse. In our study, we prospectively evaluated 55 clubfeet (37 patients) to determine clinically whether the Ponseti method is effective in the management of clubfoot in older children between the age of 12 and 36 months (mean: 24.8 months). All the patients belonged to moderate or severe grades of deformity as per the Pirani scoring. Painless, supple, plantigrade and cosmetically acceptable feet were achieved in 49 clubfeet. Seven patients (seven feet) developed recurrence of adduction, varus and equinus deformity whereas three patients (five feet) developed isolated recurrence of equinus deformity. These seven patients responded to repeat treatment and obtained satisfactory outcome. Four of these seven patients underwent tibialis anterior transfer to third cuneiform for dynamic supination. Three patients, those developed isolated recurrence of equinus deformity, underwent repeat tenotomy. One foot achieved satisfactory amount of dorsiflexion, three feet underwent tendoachilles lengthening whereas another foot underwent posterior release to obtain satisfactory dorsiflexion. Six to 12 numbers of casts (mean: 10) were required to obtain correction of clubfoot deformities. Mean period of immobilization in a cast was 13.9 weeks (10-15 weeks). We found that the Ponseti method is effective in children between the age of 12 and 36 months.     

Clubfoot with supernumerary soleus muscle. Report of 2 cases

In 2 cases of clubfoot with severe and rigid varus deformity, an accessory soleus muscle with attachment on the medial side of the calcaneus was found. After cutting the distal attachment of this muscle, the deformity diminished. The accessory soleus muscle is not interpreted as the primary cause of the clubfoot, but as a highly contributing cause to the rigid varus deformity.     

Tibialis anterior tendon transfer for residual dynamic supination deformity in treated club feet

Twenty-seven previously treated club feet in 25 patients were evaluated retrospectively following tibialis anterior tendon transfer to the dorsum of the foot to correct residual dynamic supination deformity. In 11 feet, the transfer was combined with additional soft tissue and or bony procedures to treat other accompanying deformities. Electrophysiologically demonstrated peroneal weakness causing muscle imbalance contributed to the etiology of this dynamic deformity. At follow-up, none of the patients had dynamic supination deformity during ambulation. All showed active contraction of the transferred tibialis anterior tendon. There was no case of overcorrection. Functional and cosmetic results were assessed by parents and surgeons as being excellent. Tibialis anterior tendon transfer is recommended to correct residual dynamic supination deformity and to restore muscle balance after satisfactory correction of idiopathic club foot contractures.