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Measurement of tibial torsion by ultrasound.

A method of measuring tibial torsion with ultrasound, using reference lines on the tibia, was developed and tested on dry adult tibiae. This technique was used for evaluation of a group of children with apparently normal tibiae and another group of children with spina bifida, cerebral palsy, or clubfoot. The study showed that it is possible to measure true tibial torsion (rather than tibiofibular torsion) with ultrasound. The technique was found to be reliable and accurate enough for routine clinical use and had advantages over clinical methods and other radiographic imaging techniques. Values obtained by this technique are higher than those obtained by methods using the bimalleolar axis as the distal reference line.

Adult↗

Orthopaedic manifestations of Goldenhar syndrome.

Goldenhar syndrome-hemifacial microsomia has been studied extensively with regard to its typical asymmetric craniofacial manifestations but little attention has been paid to its orthopaedic findings. The cases of 23 patients with this syndrome were reviewed in conjunction with a review of the literature. Associated orthopedic abnormalities included mainly spinal anomalies, clubfoot, congenital dislocation of the hip, Sprengel's deformity, and radial limb defects.

Abnormalities, Multiple↗

Congenital contractural arachnodactyly. Report of four additional families and review of literature.

We report here four families with congenital contractural arachnodactyly (CCA) in which a wide range of phenotypic expression is observed. In one family with a large number of affected individuals the condition is mildly expressed. These individuals usually have crumpled ears, camptodactyly with ulnar deviation of the fingers, adducted thumbs, limited elbow and/or knee extension, and hypoplasia of the calf muscles. Arachnodactyly is not a constant feature. No spinal deformities are present and only the proband has clubfoot deformities. With time, affecteds have experienced spontaneous improvement of their contractures and their condition in adulthood has not interfered with a normal lifestyle. Within this family there is little phenotypic variation between affected individuals. Those affected within each of the other families have had varying degrees of severity of the condition. A review of 29 other kindreds described in the literature with congenital contractural arachnodactyly shows that in this condition the most common features are abnormally formed ears, camptodactyly, arachnodactyly, adducted thumbs, limited movement of the elbows and knees, and underdevelopment of the calf muscles. Spontaneous improvement of the contractures with age is reported in 94% of cases. Kyphosis, scoliosis or kyphoscoliosis occurred in 50% and these defects were present in those who where more severely affected with CCA. No ocular problems have been reported in this syndrome, but congenital heart defects have occurred in 14.7%. Marfan syndrome is the most important condition to differentiate from congenital contractural arachnodactyly since these two conditions are similar phenotypically. However, in the former there are frequently serious ocular and cardiovascular problems which lead to significant morbidity and/or early death.

Adolescent↗

A previously unreported variation related to the insertion of the tibialis anterior muscle and the superficial fibular (peroneal) nerve.

During dissection classes in 2003 in the Department of Anatomy at Ege University Faculty of Medicine, an unexpected variation of the insertion of the tibialis anterior tendon was determined on the right side of a formalin-fixed cadaver of a 40-year-old man without any trace of scars, adhesions or signs of trauma or operation. The insertion of the tibialis anterior tendon was very abnormal, not on the medial side of the foot but on the lateral side. The tibialis anterior is an important muscle because of its function and use in tendon transfer as a treatment of recurrent congenital clubfoot and paralytic equinovarus foot deformities in cerebral palsy and arthroscopy, as discussed herein.

Adult↗

Early clinical symptoms and incidence of aspartylglucosaminuria in Finland.

Early clinical symptoms were analyzed from all known 43 children with aspartylglucosaminuria, born during 1974-1989 in Finland. Pre- and perinatal histories appeared normal for all children, except for muscular hypotonia and weak sucking in some babies. Three infants had abduction stiffness in the hips, needing follow-up. Other abnormalities found in infancy were umbilical or inguinal hernias and unusual susceptibility to respiratory and ear infections. This susceptibility diminished clearly in most patients after six years of age. Episodic diarrhea, described earlier, appeared to be a rather infrequent symptom and a less valuable diagnostic clue. New clinical phenomena were talipes planovalgus or clubfoot, needing surgical treatment, and aggressive behavior, needing, occasionally, child psychiatric consultation or treatment. In addition, angiokeratoma of the skin, not an infrequent phenomenon among adult patients, was found in one child. The main indications for further studies were delayed speech, attention deficit and clumsy or delayed motor functions. The disease is easily misdiagnosed and, universally, probably underdiagnosed. Its incidence in Finland was recalculated and appeared to be at least 1 in 18,500 live-born babies in this country.

Aspartylglucosylaminase↗

Arthrogryposis multiplex congenita. Search for prenatal factors in 66 sporadic cases.

In a family and epidemiological survey of 66 cases of arthrogryposis multiplex congenita all cases were found to be sporadic and no family association with clubfoot, congenital dislocation of the hip, or hereditary neuromuscular disease was found. The mothers were significantly older than average. Oligohydramnios was noted in only one-third of cases but many other complications of pregnancy, including probable attempts at abortion, had occurred. It is likely that most cases of arthrogryposis are nongenetic and result from a defective intrauterine environment, whether hormonal, vascular, mechanical, or possibly infective.

Arthrogryposis↗

Birth prevalence of malformations in members of different ethnic groups and in the offspring of matings between them, in Birmingham, England.

STUDY OBJECTIVES: The aims were: (1) to compare the birth prevalence of malformations in different ethnic groups and (2) to explore the reasons for the ethnic variations found by examining birth prevalence in the offspring of matings between ethnic groups. DESIGN: Analysis of data from a register of malformations and register of births. SETTING: Birmingham, England. SUBJECTS: A total of 432,778 infants (including stillbirths) born in 1960-84. MAIN RESULTS: Significant differences (p < 0.01) between ethnic groups were exhibited by the birth prevalence of neural tube defects (NTD), cleft palate, cleft lip, oesophageal atresia/fistula, hypospadias, hip dislocation, clubfoot, polydactyly, and syndactyly. In the offspring of matings between parents of European and Caribbean origin, the birth prevalence of NTD, cleft lip, hypospadias, hip dislocation, polydactyly, and syndactyly seemed more likely to be influenced by the ethnicity of both parents than by that of the mother alone. The reverse was true for the birth prevalence of NTD in subjects with one parent of Irish origin and on of British. CONCLUSIONS: Genetic differences may be responsible for Europeans being at lower risk of polydactyly and at higher risk of NTD, cleft lip, hypospadias, hip dislocation, and syndactyly than Caribbeans. Variations in the intrauterine environment are more likely to account for NTD being more common in Irish than in British subjects.

Asia↗

Risk of selected birth defects by maternal residence close to power lines during pregnancy.

AIMS: To evaluate selected birth outcomes from a published Norwegian cohort study in a nested case-control design with improved exposure data. METHODS: Two controls matched for sex, year of birth, and municipality were selected randomly for children with the following defects: central nervous system (CNS) defects, cardiac defects, respiratory system defects, oesophageal defects, and clubfoot. The distances between maternal addresses, during pregnancy, and power lines were obtained from maps mainly of scale 1:5000. The magnetic fields in the residences were estimated based on distance, current, voltage, and configuration. RESULTS: The highest increased risks were seen for hydrocephalus (OR 1.73, 95% CI 0.26 to 11.64) and for cardiac defects (OR 1.54, 95% CI 0.89 to 2.68). CONCLUSION: This study does not support the hypothesis that residential exposure to electromagnetic fields from power lines causes any of the investigated outcomes.

Abnormalities, Radiation-Induced↗

Risk of birth defects by parental occupational exposure to 50 Hz electromagnetic fields: a population based study.

OBJECTIVES: To study the risk of birth defects by parental occupational exposure to 50 Hz electromagnetic fields. METHODS: The Medical Birth Registry of Norway was linked with census data on parental occupation. An expert panel constructed a job exposure matrix of parental occupational exposure to 50 Hz magnetic fields. Exposure to magnetic fields was estimated by combining branch and occupation into one of three exposure levels: <4 hours, 4-24 hours, and >24 hours/week above approximately 0.1 mu T. Risks of 24 categories of birth defects were compared across exposure levels. Out of all 1.6 million births in Norway in the period 1967-95, 836,475 and 1,290,298 births had information on maternal and paternal exposure, respectively. Analyses were based on tests for trend and were adjusted for parents' educational level, place of birth, maternal age, and year of birth. RESULTS: The total risk of birth defects was not associated with parental exposure. Maternal exposure was associated with increased risks of spina bifida (p=0.04) and clubfoot (p=0.04). A negative association was found for isolated cleft palate (p=0.01). Paternal exposure was associated with increased risks of anencephaly (p=0.01) and a category of "other defects" (p=0.02). CONCLUSION: The present study gives an indication of an association between selected disorders of the central nervous system and parental exposure to 50 Hz magnetic fields. Given the crude exposure assessment, lack of comparable studies, and the high number of outcomes considered, the results should be interpreted with caution.

Adult↗

Single-shot fast spin-echo MR imaging of the fetus: a pictorial essay.

Ultrasonography (US) is the modality of choice for prenatal screening, but occasionally additional imaging information is needed. Magnetic resonance (MR) imaging is an attractive alternative but until recently has been limited by motion artifact. Single-shot fast spin-echo MR imaging was used to depict normal and abnormal anatomy in 26 fetuses. Thirteen studies were performed for maternal indications and 13 were performed to evaluate fetal abnormalities identified or suspected at US. Three of the fetal abnormalities involved the central nervous system (CNS) and 10 involved other anatomic sites. Results were correlated with findings at postnatal clinical examination, imaging, and pathologic analysis. MR imaging demonstrated normal fetal anatomy without substantial motion artifact. CNS structures were well visualized as early as 18-20 weeks gestation, as were most other normal anatomic structures except the heart. MR imaging also allowed characterization of a variety of abnormalities of the CNS (Arnold-Chiari malformation, Walker-Warburg syndrome, amniotic band syndrome) as well as of other structures (renal agenesis, multicystic dysplastic kidney, abdominal masses, severe limb-body wall defect, clubfoot with arthrogryposis, diaphragmatic hernia). US findings were confirmed in most cases, and additional information about the precise diagnosis or the severity or location of the anomaly often helped guide clinical management. Single-shot fast spin-echo MR imaging of the fetus is a useful adjunct to US in difficult diagnostic situations.

Congenital Abnormalities↗

Clinical heterogeneity of skeletal dysplasia in Roberts syndrome: a review.

Roberts syndrome is a rare autosomal recessive disorder with highly variable clinical features. The most notable manifestations include pre- and postnatal growth retardation, craniofacial anomalies and improper development of all four extremities. We reviewed 50 cases whose clinical evaluation has been vigorously pursued. A relationship of deformities exists between humerus versus femur, and radius and ulna versus tibia and fibula. Only six cases had clubfoot. The number of fingers and toes was variable. The sex ratio was 1:1. Most individuals died in early infancy, although the longest follow-up survival was 13 years in one case. Premature centromere separation, centromere splitting and puffing were common chromosomal abnormalities. An annotated bibliography on notable cases is also provided which should serve as an aid for clinicians who wish to further understand the genetic and clinical heterogeneity noted in their cases.

Abnormalities, Multiple↗

Plantarflexion opening wedge medial cuneiform osteotomy for correction of fixed forefoot varus associated with flatfoot deformity.

BACKGROUND: Flatfoot presents as a wide spectrum of foot deformities that include varying degrees of hindfoot valgus, forefoot abduction, and forefoot varus. Medial displacement calcaneal osteotomy, lateral column lengthening, and subtalar fusion can correct heel valgus, but may not adequately correct the fixed forefoot varus component. The purpose of this study was to determine the effectiveness of plantarflexion opening wedge medial cuneiform (Cotton) osteotomy in the correction of forefoot varus. METHODS: Sixteen feet (15 patients) had plantarflexion opening wedge medial cuneiform osteotomies to correct forefoot varus associated with flatfoot deformities from several etiologies, including congenital flatfoot (six feet, average age 37 years), tarsal coalition (five feet, average age 15 years), overcorrected clubfoot deformity (two feet, ages 17 years and 18 years), skewfoot (one foot, age 15 years), chronic posterior tibial tendon insufficiency (one foot, 41 years), and rheumatoid arthritis (one foot, age 56 years). RESULTS: Standing radiographs showed an average improvement in the anterior-posterior talo-first metatarsal angle of 7 degrees (9 degrees preoperative, 2 degrees postoperative). The talonavicular coverage angle improved an average of 15 degrees (20 degrees preoperative, 5 degrees postoperative). The lateral talo-first metatarsal angle improved an average of 14 degrees (-13 degrees preoperative, 1 degree postoperative). Correcting for radiographic magnification, the distance from the mid-medial cuneiform to the floor on the lateral radiograph averaged 40 mm preoperatively and 47 mm postoperatively (average improvement 7 mm). All patients at followup described mild to no pain with ambulation. There were no nonunions or malunions. CONCLUSIONS: Opening wedge medial cuneiform osteotomy is an important adjunctive procedure to correct the forefoot varus component of a flatfoot deformity. Advantages of this technique in comparison to first tarsometatarsal arthrodesis include predictable union, preservation of first ray mobility, and the ability to easily vary the amount of correction. Because of the variety of hindfoot procedures done in these patients, the degree of hindfoot correction contributed by the cuneiform osteotomy alone could not be determined. We have had excellent results without major complications using this technique.

Adolescent↗

Dorsal modified calcaneal plate for extensive midfoot arthrodesis.

BACKGROUND: Extensive midfoot fusions can be challenging because of bone loss, deformity, and soft tissue anatomy. Several options have been advocated, including multiple screw fixation, medial plating, and plantar plating. We report a new technique using a dorsally-placed, modified calcaneal plate for treatment of this difficult clinical problem. METHODS: Patients undergoing extensive (more than four joints) midfoot arthrodeses with a dorsally-placed, modified calcaneal plate between 2000 and 2003 were retrospectively reviewed. Diagnoses included Charcot arthropathy (four), osteoarthritis (two), posttraumatic osteoarthritis (two), massive bone loss from previous infection (one), and residual clubfoot deformity (one). Patients with active midfoot infections were excluded. During the study period, midfoot arthrodeses with a dorsal calcaneal plate were done in 10 patients. Of these, nine patients were available for review. Arthrodeses were attempted in 62 joints in these nine patients. Autogenous grafting was used in three patients (23 joints), allograft was used in six patients (39 joints). Patients were maintained nonweightbearing until radiographs or computed tomography conclusively showed union. RESULTS: One of the 10 patients died from an unrelated cause. In the nine remaining patents, 95% (59 of 62) of joints fused within 4 months of surgery. Postoperative complications included nonunion with broken screws in one patient, and three wound problems successfully treated with local dressings. Secondary procedures included one revision arthrodesis and two hardware removals. Patient satisfaction with this procedure was very high (eight of nine). CONCLUSIONS: The use of a dorsal calcaneal plate is a viable method of fixation for achieving fusion in extensive midfoot arthropathy. The plate is low-profile and easily moldable to conform to dorsal midfoot anatomy. It can be placed without extensive plantar or medial foot dissection and maintains midfoot alignment until bony fusion occurs. In patients with complex midfoot pathology requiring multijoint fusions, the results have been satisfactory.

Adult↗

The use of a working model for teaching functional and applied surgical anatomy of the subtalar joint.

BACKGROUND: Some aspects of functional and applied anatomy may be difficult to conceptualize if taught by conventional methods. In such situations, alternative methods of teaching may need to be adopted. This study was undertaken to evaluate whether a working model can facilitate understanding of the functional and applied surgical anatomy of the subtalar joint. METHODS: A working model of the articulated talus and calcaneus was constructed and used to explain the mechanics of motion of the subtalar joint, the rationale of release of structures to correct hindfoot deformity in clubfoot, principles of tendon transfers, and the rationale of extra-articular subtalar joint arthrodesis. Twenty-four orthopedic residents were divided into two groups (A and B). Group A was taught these specific concepts of functional and applied anatomy with the help of the model, while Group B was taught the same concepts with a written text and diagrams. The level of knowledge of both groups was evaluated by a test with multiple-choice questions. A week later, Group A was given the text while Group B was shown the model. Both groups then answered a questionnaire assessing the value of the model in enhancing their understanding of the subject. RESULTS: In the test, the performance of students in Group A was better than those in Group B. Students of both groups felt that the model gave them a much clearer understanding of the functional anatomy of the subtalar joint. CONCLUSIONS: We conclude that this simple, inexpensive model is a valuable teaching aid that helps students to understand the surgical anatomy of the subtalar joint. CLINICAL RELEVANCE: Surgeons can use this model to effectively teach trainees in this field about the mechanics and anatomy of the subtalar joint and other relevant applications.

Adult↗

The treatment of rigid metatarsus adductovarus with the use of a new hinged adjustable shoe orthosis.

A retrospective review of the early results of treatment of metatarsus adductovarus with a new hinged adjustable shoe orthosis, the Bebax shoe, is presented. The results in 120 cases (210 feet) are included in the study. Criteria for severity of the initial deformity were developed, and all cases of true clubfoot and those cases with prior treatment were excluded. The average age at institution of treatment was 4.5 months, and treatment time averaged 2.7 months (range 1-7 months). Overall, 96% of the cases were completely resolved with the use of the orthotic alone, 3% required additional casting, and 1% required a bar and shoes for complete correction of the deformity. No treatment complications were encountered except some minor problems with hinge loosening, particularly in the child of walking age. We believe that the Bebax shoe is a lightweight, convenient, safe, and both cost- and treatment-effective device in the management of metatarsus adductovarus.

Female↗

Parent satisfaction comparing two bandage materials used during serial casting in infants.

Plaster of Paris was compared with semirigid fiberglass casting material during serial casting in 17 infants with clubfoot or rigid metatarsus adductus. Semirigid fiberglass was statistically superior in its durability, convenience, performance, and ease of removal. The average amount of time for home cast removal by the parents was 55 minutes for plaster of Paris and 21 minutes for semirigid fiberglass. Complications such as skin abrasions and cast slip-off were similar for both casting materials. Ninety-four percent of parents strongly preferred semirigid fiberglass rather than plaster of Paris for their child's serial casting.

Casts, Surgical↗

A new modified technique for harvest of calcaneal bone grafts in surgery on the foot and ankle.

Reported sites for retrieval of cancellous bone for grafts include the iliac crest, greater trochanter, proximal tibia, and distal tibia. A new lateral technique for retrieval of cancellous bone from the calcaneus is evaluated through anatomic review, quantitative analysis, and retrospective clinical assessment. Of 22 patients managed with this technique over a 2-year period, 17 returned for an evaluation by questionnaire, physical examination, and radiographic follow-up at an average of 7 months after surgery (range, 4-16 months). Complaints/complications were minor: three had minor incisional symptoms, five had medial heel pain (3 caused by plantar fasciitis), and one had unchanged preoperative heel pain secondary to clubfoot deformity. Compared with more extensive bone-grafting procedures, this procedure offers the advantages of bone harvested under local anesthesia using a readily accessible ipsilateral extremity and producing minor complications.

Adolescent↗

Louis Stromeyer (1804-76): German orthopaedic and military surgeon and his links with Britain.

Louis Stromeyer was a pioneer of orthopaedic surgery and military medicine in Germany. The 200th anniversary of his birth was celebrated on 6 March 2004. He was much admired in Britain, which he visited on three occasions and where he met many of the country's leading surgeons. He was a friend and colleague of the London surgeon W J Little (1810-94), whose clubfoot he corrected by the operation he developed, and which Little introduced to Britain: subcutaneous tenotomy. A bibliography of Stromeyer's publications is appended.

Germany↗