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Potential treatment for clubfeet based on growth factor blockade.

Select soft tissues in clubfeet are contracted, resulting in stiffness. These contracted tissues share ultrastructural characteristics with palmar fibromatosis (Dupuytren contracture), in which the growth factors transforming growth factor-beta (TGF-beta) and platelet-derived growth factor (PDGF) are expressed and play a role in regulating cell behavior. More contracted tissue (medial side of the foot) and less contracted tissue (lateral side of the foot) from 20 clubfeet were studied using reverse transcription-polymerase chain reaction and western analysis for expression of TGF-beta and PDGF (along with collagen type I and type III). Cell cultures were established from the more contracted tissues to determine the effect of blockade of these factors with neutralizing antibodies on proliferation, chemotaxis, and collagen expression. Both growth factors were expressed at higher levels by the contracted tissues, and blockade led to decreased collagen expression, proliferation, and chemotaxis. Growth factor blockade has the potential to change the behavior of these cells in a way that would lessen the severity of the contractures, perhaps improving the outcome of clubfoot treatment.

Cells, Cultured↗

Smallpox vaccination during pregnancy.

The obstetric outcome (abortions, stillbirths, prematurity, mature births, and congenital abnormalities) in a group of 1522 consecutive pregnant patients who had smallpox vaccinations during recent pregnancies was compared to that in a similar control group of 2024 consecutive pregnant patients who did not receive any antenatal vaccination. Results showed that smallpox vaccination during pregnancy did not increase the rate of stillbirths, premature births, or congenital abnormalities in patients who had been vaccinated at least once before. However, it was noted that women vaccinated during the first trimester of pregnancy more often had children with clubfoot. Therefore, unless there is an immediate risk, we recommend delaying smallpox vaccination during pregnancy until the second or third trimester. However, during epidemic, vaccination should be done regardless of gestational age.

Abortion, Spontaneous↗

[Results of surgical correction of congenital talipes equinovarus].

Different surgical procedures in the treatment of clubfoot were analyzed, especially in correlation to over-correction and inadequate correction. Indications for surgery, surgical errors and their influence on outcome were assessed. 82 children (28 females and 54 males) with 130 congenital equinovarus underwent surgery between 1988 and 1994. Age at the time of operation ranged from 6 to 13 months (average: 9 months). Posterior release (i.e. partial subtalar release) was conducted in 68 cases of clubfeet and complete subtalar release in 62 cases. During follow-up 44 children with 64 clubfeet were reviewed. Magone's criteria were used to assess final results. 15 (23%) feet showed very good results, 26 (41%)--good results, 14 (22%)--satisfactory and 9 (14%)--poor or no correction.

Adolescent↗

[Child orthopedics].

Children's fractures are a challenge to the treatment system, in that 17,000 children are treated in Denmark each year. The true burden on patients and society is unknown. It is estimated that 1000 new-born infants are treated for hip instability in Denmark per year, and 30 are diagnosed late. Ultrasound scanning is recommended for secondary screening and selective screening of children at risk. Identification of acetabular dysplasia followed by surgical correction could be an important prophylactic measure, with a potential reduction in expenses to society. To some extent, congenital clubfoot is hereditary. Environmental factors are important, maternal smoking being one. Early operative treatment yields good results. There is a need for prospective registration. Perthes' disease is an idiopathic necrosis of the capital femoral epiphysis. Passive smoking is a significant factor. A young age (< 7 years) and limited necrosis carry a good prognosis, and active treatment can improve the prognosis for the other patients.

Child↗

Identification of decomposed human remains from radiographic comparisons of an unusual foot deformity.

A case of positive identification from decomposed human remains using an unusual foot deformity is presented. Scrutiny of the decedent revealed foot deformities, which upon examination, prompted further inquiry. Radiographic comparisons and defleshing each foot established bilateral talipes equinovarus (TEV, clubfoot). Positive identification was based upon unique skeletal features present in the radiographs.

Adult↗

[Treatment of the deformities associated with hypoplasia or agenesis of the sacral bone].

On the basis of follow-up of 5 children treated because of the deformities associated with sacral agenesis or hypoplasia as well as on the literature the authors came to the conclusion that congenital dislocation of the hip, clubfoot and flatfoot are the most common. The aim of the treatment is to prepare the child for walking before the end of the first year of life. Since the deformities mentioned are resistant to conservative treatment they require a very sophisticated surgery with the constant adjustment of the operating plan in every stage of the management. The type of hip surgery should be selected individually.

Abnormalities, Multiple↗

Revision surgery in clubfeet.

The reoperated clubfeet of 29 children aged one to 12 years were reviewed. The surgical procedure most often used in revision surgery was recomplete soft-tissue release alone or combined with plantar release, calcaneocuboid fusion, and capsulotomies of the navicular-first cuneiform-first metatarsal joint. In 27 of 29 feet, acceptable results were achieved. Nineteen were excellent and good results. An algorithm that suggests surgical solutions to a variety of clubfoot deformities in different age groups has been developed, as well as an objective rating system, to evaluate the long-term results of revision surgery of clubfeet.

Child↗

Congenital club foot with survival of motor neuron 1, telomeric (SMN1) gene deletion.

A boy with nonreducible bilateral congenital talipes equinovarus had delayed milestones with early-onset generalized hypotonia and muscular weakness. The condition remained stable until he was 8 years old. A slow worsening of motor abilities, with myopathic signs, was observed thereafter. A homozygous deletion of exons 7 and 8 of the survival of motor neuron 1, telomeric (SMN1) gene was found, without neuronal apoptosis inhibitory protein (NAIP) gene deletion, leading to the diagnosis of spinal muscular atrophy. Independent ambulation was lost when he was 13 years old. The occurrence of congenital clubfoot with early onset of neurologic signs, but with a very slowly progressive course, has not been reported in spinal muscular atrophy until now.

Adolescent↗

Clubfeet associated with hydrocephalus: new evidence of gradual dynamic development in utero.

Clubfoot, a relatively frequent congenital malformation, may be associated with several genetic syndromes or other malformations, or may appear as an isolated idiopathic anomaly. We describe the intrauterine development of clubfeet subsequent and in parallel to fetal hydrocephalus formation, as followed by serial transvaginal sonographic scans at 11-16 weeks' gestation. The possibility of a causal relationship between the central nervous system malformation and the clubfeet is suggested. To the best of our knowledge, this is the first description of the dynamic, in vivo fetal development of hydrocephalus and clubfeet at 11-16 weeks' gestation.

Adult↗

[Anesthetic management of a patient with xeroderma pigmentosum].

Xeroderma pigmentosum (XP) is a rare autosomal recessive disease, which is characterized by hypersensitivity of the skin to ultraviolet (UV)-radiation and progressive neurological complications. Patients with XP show a failure to properly repair UV-induced DNA lesions by the nucleotide excision repair (NER) mechanism. This results predominantly in a high frequency of UV-induced skin tumors at an early age. Therefore, patients with XP must avoid exposure to UV-radiation by use of protective clothing, sunscreen and UV-blocking film. We report a 15-year-old girl, who underwent surgical treatment twice for clubfoot under general anesthesia using propofol and fentanyl. We protected her skin from light with UV blocking film in the operating room. Both surgical procedures were performed uneventfully except for delayed awakening. A number of precautions must be taken in the perioperative management of XP patients, which include proper shielding from damaging light, avoidance of all drugs that harm DNA such as halothane and careful evaluation of neurological abnormalities. There is a possibility of difficult endotracheal intubation or prolonged effect of muscle relaxation due to skin atrophy and joint contracture.

Adolescent↗

Patient location strategies for pediatric long-term follow-up studies.

BACKGROUND: Poor follow-up rates greatly diminish the validity of prospective and long-term studies. Therefore, locating patients is of critical importance. This is especially true in populations treated during childhood because addresses will change several times in intervening years. Recent publications have reported new strategies for patient location. The purpose of this study is to test an algorithm proposed by King et al., as well as other search methods, using a cohort of patients treated for clubfoot in childhood METHODS: The study population included 126 patients with clubfeet treated between 1950 and 1967. We followed the search algorithm proposed by King et al. In addition, we used state driver's license records, Reunitetonight.com, and Intelius.com. Patients were considered to be found when they returned a postage-paid reply letter or were contacted by phone. RESULTS: Using web pages recommended by King et al. we located 26 of 126 (21 percent) patients. Operator directory assistance failed to locate any patients not located by free internet sources. Additional websites had varied results. State driver's license records found 25 patients. Reunitetonight.com found none with thirty attempted. The best search engine was Intelius.com which located 68 out of 126 (54 percent) patients. CONCLUSION: The algorithm proposed by King et al. is not effective for long-term follow-up studies of pediatric populations. Intelius.com is worth the small fee charged (dollar 22.45) as it was the most effective method of locating patients.

Algorithms↗

Accessory soleus muscle as a cause of congenital talipes equino varus. A case report.

The authors report a case of congenital clubfoot in a one-year-old male child, in which an accessory soleus muscle was noted intraoperatively, running anteromedially to the Achilles tendon and with a distinct insertion on the postero-medial aspect of the calcaneus. Correction of the varus and equinus of the hindfoot could only be achieved after cutting the tendon of the accessory soleus muscle at its insertion on the calcaneus.

Clubfoot↗

Anterior tibial tendon insertion: an anatomical study.

Anatomical variations of the anterior tibial tendon insertion were studied by dissection in 44 normal feet of 22 embalmed adult cadavers. Three types of insertion were found. The most common insertion (56.8%) was a division of the tendon into two slips, equal in width, the one slip being inserted into the first cuneiform and the other slip into the base of the first metatarsal. The next most common (27.3%) was a division of the tendon into a large slip, inserted into the first cuneiform, and a small slip, inserted into the base of the first metatarsal. The third type, and least common tendon insertion (15.9%), was by a single slip into the first cuneiform only. Knowledge of these anatomical variations will assist surgeon in performing anterior tibial tendon transposition for recurrent congenital clubfoot.

Adult↗

Foot-progression angle in clubfeet.

Foot-progression angle was determined using the Shutrack carbon paper system for 52 feet treated by clubfoot release without wide subtalar release and 43 age-matched controls. Mean foot progression angle was -5 degrees for the clubfeet. This represented 13 degrees of inturning from the normal mean of 8 degrees. Forty-eight percent of the clubfeet had inturning greater than 2 SD from the normal mean. The causes of inturning included adductus (p = 0.005) and, in some cases, internal tibial torsion or internal femoral torsion. Varus of the foot was usually associated with inturning, but inturning was not always associated with varus.

Anthropometry↗

Arterial abnormalities in talipes equinovarus as assessed by angiography and the Doppler technique.

Preoperative angiography in 30 uncorrected clubfeet demonstrated abnormal vascular patterns in all but two limbs with hypoplasia or premature termination of the anterior tibial and medial plantar arteries in the remainder. Postoperative Doppler studies in nine of the limbs with abnormal vessels indicated that these arteries were present. We suggest that the continuous-wave Doppler technique is less useful for identifying major arteries than either dissection or angiography. Furthermore, arterial dysgenesis may play a role in the etiology of clubfoot. Since the posterior tibial artery usually provides the sole arterial supply to the foot, this vessel must be preserved at surgery and during subsequent ankle dorsiflexion.

Angiography↗

Choices in surgical treatment of rigid neurogenic and arthrogrypotic clubfeet.

Clinical experience in treating clubfoot suggests that the equinovarus deformity in the arthrogrypotic child is often comparable to the equinovarus deformity in certain neurologically impaired children. Both types of feet tend to be rigid, to resist correction, and to present problems of management different from those of idiopathic equinovarus feet. I describe a series of 18 rigid neurogenic or arthrogrypotic clubfeet in 10 children illustrating problems of diagnosis and management and pointing up the desirability of access to a broad range of surgical procedures to solve these unique problems.

Arthrogryposis↗

[Talus necrosis and its treatment].

Aetiopathogenesis of the necrosis of the talus has not yet been definitely clarified, and neither has that of the other aseptic necroses. We were able to study the aetiopathogenesis, course of the disease and therapy in 20 of our own patients by follow-up; two of these developed necrosis of the talus in both feet. We definitely excluded patients suffering from osteochondrosis dissecans. Even though fracture of the talus is on the whole relatively rare, it remains the most frequent cause of necrosis of the talus. We also found talonecrosis after surgical correction of clubfoot, after Sudeck's disease (Sudeck-Leriche syndrome, Sudeck's atrophy or dystrophy), suppurative arthritis of the ankle joint, subtalar luxation and haematogenic osteomyelitis. Only few patients required surgery. In most cases a special boot constructed for arthrodesis patients proved sufficient. Each patient developed arthrodesis to a different degree. Depending upon the complaints and stiffening of the ankle joint or of the talo-calcanonavicular joint, the capacity of the patients to be gainfully employed was reduced by an amount between 20 and 30 per cent.

Adolescent↗

The complete subtalar release in clubfeet.

CSTR is a release of the subtalar joint and the talonavicular joint that corrects calcaneal rotation, a major deformity of the clubfoot, as well as the other major deformities of the midfoot and hindfoot. Indications for the CSTR are presented. These include failure of conservative treatment or unsatisfactory surgical results with residual varus deformity, and a foot longer than 8 cm. Contraindications to CSTR include radiographic evidence of a flat-top talus and uncorrected anterior ankle contractures. The Cincinnati incision, which permits excellent visualization of all the relevant structures and good cosmesis, is described and compared with other approaches. The technique of the CSTR is described in detail. Key steps include marking the knee for later alignment with the foot (a major advantage of this procedure), superficial medial dissection, posterior dissection, lateral dissection, and deep medial dissection. Technical details and criteria for evaluation are provided. Four supplemental procedures and their indications are described. These include metatarsal osteotomy, calcaneocuboid capsulotomy, calcaneal wedge osteotomy, and plantar release. Procedures for pinning and casting are described, with special attention to accuracy of realigning the foot and measures for avoiding avascular necrosis and other complications. The importance of intraoperative radiographs is presented, together with methods for obtaining and assessing these films to assure that surgical correction is complete. It is imperative that these films be taken before tendon repair or wound closure so that pinning procedures can be repeated, if necessary.

Bone Nails↗