[Risk Factors for the Development of Distal Tibiofibular Synostosis after Unstable Ankle Fractures]. / Rizikové faktory pro vznik distální tibiofibulární synostózy po luxacních zlomeninách hlezna.

PURPOSE OF THE STUDY The study analyses a cohort of patients with surgically treated ankle fractures who developed complete distal tibiofibular synostoses. It focuses on their occurrence and association with the extent of tibiotalar dislocation of the ankle joint on the trauma X-ray and its relation to the choice of surgery. MATERIAL AND METHODS The cohort of a total of 824 patients with type B and C fractures according to Weber classification was followed up for 9 years. The cohort consisted of 403 (48.9%) men and 421 (51.1%) women. The exclusion criteria included associated talus fractures, calcaneus fractures and fractures of the other bones of the foot. The studied data were obtained retrospectively from medical documentation and by evaluation of trauma X-rays and X-rays obtained during the postoperative checks. The ankle fractures were classified based on the Weber classification and the basic epidemiologic data (age and gender), type of fracture and extent of tibiotalar dislocation of ankle fractures on the trauma X-ray were evaluated. Posttraumatic ankle dislocation was divided into tibiotalar dislocation > 10 mm, tibiotalar dislocation < 10 mm and the group with regular ankle joint. When evaluating the treatment method, the cohort was divided into three groups: Group 1 with one-stage osteosynthesis, Group 2 with temporary K-wire transfixation or external fixation and subsequent secondary conversion to internal osteosynthesis, and Group 3 with definitive transfixation or external fixation of the ankle. The results were statistically evaluated using the Pearson s chi-square test, or the Fisher s exact test for low frequencies. A multivariant logistic regression model was created to identify statistically significant factors contributing to the development of synostosis. The results with the p-value < 0.05 were considered statistically significant. RESULTS In the whole cohort, the synostosis of distal tibiofibular joint was observed in a total of 131 (15.9%) patients. In men it was in 85 (21.1%) cases and in women in 46 (10.9%) cases, which was statistically significant (p < 0.0001). There was a statistically significant difference (p = 0.0020) between the mean age in the group of patients with synostosis (54.4 years) and the mean age in the group of patients without synostosis (49.1 years). Complete distal tibiofibular synostoses were found in 78 (12.7%) fractures classified as type B according to the Weber classification and in 53 (25.5%) type C fractures. When taking into account the gender, synostoses occurred more frequently in men in both types of fractures classified based on the Weber classification, only in type C fractures no statistical significance was established (p = 0.3026). Various size of posttraumatic tibiotalar dislocation was present in both types of fractures. The group with less severe type B ankle fractures showed a statistically significant dominance of synostosis development in cases with large tibiotalar dislocation of more than 10 mm (p<0.0001). In the group with type C fractures different results were obtained. The highest frequency of cases with synostosis was reported in the group with dislocation smaller than 10 mm (p = 0.0698). In the entire cohort, 615 (74.6%) one-stage osteosyntheses were performed and synostoses developed in 77 (12.5%) cases. In 165 (20.0%) patients, transfixation with K-wires or external fixation with subsequent conversion to secondary osteosynthesis were used and synostoses were identified in 50 (30.3%) cases (p < 0.0001). The open fractures showed an insignificantly smaller number of synostoses than the closed fractures (p = 0.5902). DISCUSSION Posttraumatic distal tibiofibular synostoses have varied morphology. A number of studies confirmed that they do not affect much the functional status of the ankle, even despite their extensive finding in the area of syndesmosis is evident on the Xray. Etiologically, a certain role in their development is reported to be played by posttraumatic hematoma in case of damage to deep soft and bony structures of the ankle. CONCLUSIONS A higher occurrence of synostoses was observed in male population, older age patients and also in type C fractures according to the Weber classification. Larger tibiotalar dislocation showed statistical significance in the development of synostoses in type B fractures according to the Weber classification, whereas in type C fractures it was not the main factor contributing to the development of synostosis. In cases where one-staged osteosynthesis was performed, the occurrence of synostoses was statistically significantly lower than in secondary osteosynthesis after temporary stabilisation. Key words: ankle fracture, distal tibiofibular synostosis, ankle joint dislocation, Weber classification, acute surgery, delayed surgery.

Multiple synostoses syndrome: Clinical report and retrospective analysis.

Multiple synostoses syndrome (SYNS1; OMIM# 186500) is a rare autosomal dominant disorder reported in a few cases worldwide. We report a Chinese pedigree characterized by proximal symphalangism, conductive hearing loss, and distinctive facies. We examined the genetic cause and reviewed the literature to discuss the pathogeny, treatment, and prevention of SYNS1. Audiological, ophthalmological, and radiological examinations were evaluated. Whole-exome sequencing (WES) was performed to identify mutations in the proband and her parents. Sanger sequencing was used to verify the results for the proband, parents, and grandmother. The literature on the genotype-phenotype correlation was reviewed. The patient was diagnosed with multiple synostoses syndrome clinically. WES and bioinformatic analysis revealed a novel missense mutation in the NOG gene, c.554C>G (p.Ser185Cys), cosegregated in this family. The literature review showed that the phenotype varies widely, but the typical facies, conductive hearing loss, and proximal symphalangism occurred frequently. All reported mutations are highly conserved in mammals based on conservation analysis, and there are regional hot spots for these mutations. However, no distinct genotype-phenotype correlations have been identified for mutations in NOG in different races. Regular systematic examinations and hearing aids are beneficial for this syndrome. However, the outcomes of otomicrosurgery are not encouraging owing to the regrowth of bone. This study expanded the mutation spectrum of NOG and is the first report of SYNS1 in a Chinese family. Genetic testing is recommended as part of the diagnosis of syndromic deafness. A clinical genetic evaluation is essential to guide prevention, such as preimplantation genetic diagnosis.

Sinostosis cervical asociada a síndromes de Down y de Schneider. Implicaciones clínicas y médico-legales a propósito de un caso autópsico / Cervical synostosis associated with Down's syndrome and Schneider syndrome. Clinical and medico-legal implications of an autopsy case

Con ocasión de un caso de asociación entre síndrome de Down y sinostosis vertebral congénita que sufrió lesión medular traumática, se revisa la asociación de estos cuadros y sus implicaciones clínicas y forenses. La literatura no ha comunicado hasta ahora una asociación prevalente entre síndrome de Down y sinostosis cervicales. Ambos cuadros por separado propenden a la mielopatía cervical, bien de manera degenerativa, bien traumática. En consecuencia, la asociación de ambos debe verse como un factor de riesgo de daño medular o de manera espontánea o, sobre todo, después de traumatismos. En estos casos, la exploración radiológica debe ser exhaustiva incluyendo RMN ya que pueden existir lesiones medulares incluso ante traumatismos mínimos. En caso de fallecimiento, la autopsia medular es obligada ya que puede revelar lesiones subclínicas, clarificar la naturaleza y extensión de las lesiones medulares y esqueléticas, así como ayudar a establecer una mejor correlación anatomoclínica

This paper reviews the association of clinical symptoms and their clinical and forensic implications in a patient with Down's syndrome and congenital cervical synostosis who experienced a traumatic spinal cord injury. To date, no prevalent association between Down's syndrome and cervical synostosis has been reported in the literature. Given that both entities are prone to cause degenerative or traumatic cervical myelopathy, the combination of both conditions must be seen as a risk factor for spontaneous, and particularly traumatic, spinal cord damage. In these cases, radiological examination must be exhaustive, including MRI, given the possibility of spinal cord injury even after minimal trauma. In the event of death, spinal cord autopsy is mandatory as it may reveal subclinical lesions, clarify the nature and extension of the spinal cord and skeletal injuries and help to establish an improved anatomo-clinical correlation

Carpal Coalitions on Radiographs: Prevalence and Association With Ordering Indication.

PURPOSE: Carpal coalitions are common and usually incidental to the indication for wrist radiographs. It is not clear if, or when, carpal coalitions cause pain. The aim of this study was to assess the prevalence of incidental carpal coalitions by evaluating radiographs taken for various indications and to test the association of demographic variables and ordering indications with the finding of a carpal coalition. METHODS: We reviewed 1,119 posteroanterior wrist radiographs for the presence of carpal coalition. We used bivariate and multivariate analyses to assess demographic factors for their independent associations with the presence of carpal coalitions and to compare the difference in the prevalence of carpal coalitions between radiographs obtained to evaluate traumatic wrist pain (623 wrists), nontraumatic wrist pain (175 wrists), and other reasons (321 wrists). RESULTS: Radiographs of 98 out of 1,119 patients (8.8%) showed a carpal coalition. Carpal coalitions were equally likely on radiographs obtained for traumatic wrist pain and nontraumatic wrist pain. Patients with no wrist trauma or wrist pain were less likely to have a carpal coalition on their radiograph. CONCLUSIONS: We consider carpal coalitions an unlikely cause of wrist pain. The lower prevalence in radiographs obtained for causes other than wrist trauma or wrist pain remains unexplained, although it may be spurious. In the evaluation of a patient with nonspecific wrist pain, clinicians should be careful ascribing symptoms to anatomical variations on radiographs. These incidental findings should not usually affect management. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic III.

Distal tibiofibular synostosis after surgically resolved ankle fractures: An epidemiological, clinical and morphological evaluation of a patient sample.

INTRODUCTION: Ankle fractures comprise a highly morphologically and etiologically diverse group of injuries, which includes various degrees of impairment of bone and ligamentous structures. The complete synostosis and incomplete bony bridging of tibiofibular syndesmosis are among the local late complications after surgically treated ankle fractures. PATIENTS AND METHOD: 269 patients were evaluated, including 203 patients with Weber type-B fractures, and 66 patients with Weber type-C fractures. All patients underwent ankle radiography at standard intervals (post-operatively, 6 and 12 weeks, 6 and 12 months). The final assessment one year after osteosynthesis was performed. The study analyzed age, sex, fracture morphology, the location and morphology of ossification, functional outcomes and subjective evaluations of patient status. RESULTS: As risk factors there were found male sex, tibiotalar dislocation, syndesmotic screw fixation and Weber type-C fractures. The severity of subjective difficulties and objective status were not dependent on the size of distal tibiofibular synostosis. DISCUSSION AND CONCLUSION: Despite relatively extensive imaging findings of complete synostosis or incomplete bony bridging, they only limited functional outcomes to a minimal extent.

Incidence and clinical relevance of tibiofibular synostosis in fractures of the ankle which have been treated surgically.

In this retrospective cohort study, we analysed the incidence and functional outcome of a distal tibiofibular synostosis. Patients with an isolated AO type 44-B or C fracture of the ankle who underwent surgical treatment between 1995 and 2007 were invited for clinical and radiological review. The American Orthopaedic Foot and Ankle Society score, the American Academy of Orthopaedic Surgeons score and a visual analogue score for pain were used to assess outcome. A total of 274 patients were available; the mean follow-up was 9.7 years (8 to 18). The extent of any calcification or synostosis at the level of the distal interosseous membrane or syndesmosis on the contemporary radiographs was defined as: no or minor calcifications (group 1), severe calcification (group 2), or complete synostosis (group 3). A total of 222 (81%) patients were in group 1, 37 (14%) in group 2 and 15 (5%) in group 3. There was no significant difference in incidence between AO type 44-B and type 44-C fractures (p = 0.89). Severe calcification or synostosis occurred in 21 patients (19%) in whom a syndesmotic screw was used and in 31 (19%) in whom a syndesmotic screw was not used.(p = 0.70). No significant differences were found between the groups except for a greater reduction in mean dorsiflexion in group 2 (p = 0.004). This is the largest study on distal tibiofibular synostosis, and we found that a synostosis is a frequent complication of surgery for a fracture of the ankle. Although it theoretically impairs the range of movement of the ankle, it did not affect the outcome. Our findings suggest that synostosis of the distal tibiofibular syndesmosis in general does not warrant treatment.

Incidence and variation of interpretably bone (os incae) in northeastern Thailand.

BACKGROUND: The squamous segment of occipital bone consists of cartilaginous and membranous origin. The cartilaginous part develops to supra-occipital bone. The membranous part has three primary ossification centers on each side. The first pair ossification center lies above the cartilaginous part between the superior nuchal line and the highest nuchal line and fuse with the cartilaginous part to form a supra-occipital segment of occipital bone. The second and third pairs have two nuclei each forming lateral and medial plates. All of these ossification centers fuse to form squamous segments of occipital bone. The fusion failure between ossification centers of second and third pair nuclei with each other or supra-occipital segment causes separated bone(s) called interparietal bone(s) or os incae. The interparietal bone should be differentiated from Wormian (intrasutural) bone. The incidence from various studies ranges from 0.37% to 9.50% of the population. OBJECTIVE: To study the incidence and variation of interparietal bone in Northeastern Thailand as compared with other studies. MATERIAL AND METHOD: A total of 400 Thai native skulls (276 male and 124 female) from the collection of Anatomical Museum of the Faculty of Medicine Khon Kaen University aged from 16 to 93 years old were examined by naked eye and photographed. Wormian bone was excluded by shape and site. The statistical method used was percentage of relative frequency. RESULTS: The incidence of interparietal bone in Northeastern Thailand is 7.25% (29 from 400). Males have a two times higher incidence rate than females, (8.33% versus 4.84%). Eleven patterns of interparietal bone were found. Fusion failure of a third pair ossification center is more common than second pair CONCLUSION: Knowledge of interparietal bone is useful for neurosurgeons and radiologists to avoid missed diagnosis of skull fracture. Presented interparietal bone may cause difficulty in surgery of occipital and parietal bone. Forensic scientist can use interparietal bone for personal identification.

Adult post-traumatic radioulnar synostosis.

Post-traumatic radioulnar synostosis is a rare complication of forearm fracture. Resulting in loss of forearm axial rotation, it is functionally very disabling. The surgical indication, timing of operation, surgical technique, interest and type of adjuvant treatment are all issues with which physicians managing radioulnar synostosis must deal. No therapeutic consensus yet exists, but a wide variety of surgical techniques and adjuvant treatments are suggested. A literature review sought to identify risk factors for synostosis, with a view to prevention and determining a suitable therapeutic attitude in the light of existing data.

Management of posttraumatic radioulnar synostosis.

Posttraumatic radioulnar synostosis is a rare complication following fracture of the forearm and elbow. Risk factors for synostosis are related to the initial injury and surgical management of the fracture. Typically, patients present with complete loss of active and passive forearm pronation and supination. Evidence of bridging heterotopic bone between the radius and ulna can be seen on plain radiographs. Although nonsurgical management is sufficient in some cases, surgical excision is typically required. The timing of surgical intervention remains controversial. However, early resection between 6 and 12 months after the initial injury can be performed safely in patients with radiographic evidence of bony maturation. Surgical management consists of complete resection of the synostosis with optional interposition of biologic or synthetic materials to restore forearm rotation. A low recurrence rate can be achieved following primary radioulnar synostosis excision without the need for routine adjuvant prophylaxis.

Radial longitudinal deficiencies with hypoplastic/absent thumbs and cutaneous syndactyly of the most radial digits.

PURPOSE: To describe radial longitudinal deficiency with hypoplastic or absent thumb and cutaneous syndactyly between the most radial digits. In addition, to discuss the clinical relevance and unique treatment decisions involved in optimizing functional outcomes in these patients. METHODS: A total of 163 extremities of 122 patients with radial longitudinal deficiencies were reviewed. We reviewed radiographs and clinical images that were available, with most radial hypoplastic digit and cutaneous syndactyly to the adjacent finger. RESULTS: There were 7 hands with this type of deformity. Four cases had a hypoplastic thumb associated with cutaneous syndactyly between the thumb and index finger. Three cases had a deformity in which the thumb was absent and the hypoplastic index finger was syndactylized to the long finger. Proximal and distal radioulnar synostoses were associated with these deformities in 3 patients. CONCLUSIONS: Because the most radial digit is severely hypoplastic in this type of deformity, pollicization is usually indicated. However, the pollicization procedure must be modified due to associated syndactyly with different degrees of hypoplasia or absence of the intrinsic muscles. This type of deformity should be distinguished from hypoplastic thumb without syndactyly. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Treatment of unilateral coronal synostosis by endoscopic strip craniectomy or fronto-orbital advancement: Ophthalmologic findings.

BACKGROUND: Unilateral coronal synostosis results in ipsilateral retrusion of the forehead and superior orbital rim, shortening the elevation of the orbital roof and contralateral frontal bossing and orbital roof depression. This craniosynostosis is associated with the development of V-pattern strabismus and aniso-astigmatism. Since 2004 endoscopic strip craniectomy performed in patients by 3 months of age has been offered as an alternative to fronto-orbital advancement at 9 to 11 months of age. We compare the incidence and severity of V-pattern strabismus and aniso-astigmatism in children treated by these 2 procedures. METHODS: A retrospective review identified 37 children with unilateral coronal synostosis treated with either fronto-orbital advancement or endoscopic strip craniectomy. Incidence and severity of V-pattern strabismus, fundus excyclotorsion, and aniso-astigmatism was recorded for an "early" examination (between 2 and 14 months of age) and a "late" examination (between 14 and 45 months of age). RESULTS: Early examination revealed no statistical difference in severity of V-pattern strabismus or aniso-astigmatism between the 2 groups. At late examination there was a trend toward greater severity of V-pattern strabismus, an increase in excyclotorsion, and a statistically significant increase in the standard deviation of aniso-astigmatism in the cohort of children treated by fronto-orbital advancement. CONCLUSIONS: Children with unilateral coronal synostosis treated by early endoscopic strip craniectomy may develop less severe V-pattern strabismus, excyclotorsion, and range of aniso-astigmatism than those treated by later fronto-orbital advancement. This is an early, retrospective, nonrandomized study with a short follow-up period; longer follow-up is necessary to confirm these results.

MRI of tarsal coalition: frequency, distribution, and innovative signs.

OBJECTIVE: Tarsal coalition is uncommon, but not rare. Since it may present with chronic symptoms and since prior studies have shown that magnetic resonance (MR) has a high accu- racy for diagnosis, we evaluated the prevalence, frequency, location, and types of coalition, as well as secondary signs, as seen in a large clinical MR population. SUBJECTS AND METHODS: Initially a "word search" program was used to ind all diagnoses of "tarsal coalition" in our RIS database as part of 27,483 ankle MRIs performed over a ten-year period. We revaluated 101 of 169 identiied cases for location (calca- neonavicular vs. talocalcaneal/subtalar) and type (osseous, cartilaginous, or ibrous, utilizing previously described MR criteria) of coalition. Additional imaging indings, including marrow edema and fractures, and associated secondary signs, such as talar "beaks," the "anteater" (hyperplastic anterior calcaneal process) sign, and the innovative "reverse anteater" (hyperplastic navicular) and "drunken waiter" (hypo/dysplastic sustentaculum tali) signs, were reported as well. As a second part to the study, in order to more ac- curately determine prevalence in a population requiring an MRI of the ankle, two observers reviewed 667 consecutive ankle MRIs performed on 640 patients. Patients less than 12 years of age (N = 20, 3.0%) or with nondiagnostic exams (N = 73, 10.9%) were excluded. Note was made: 1. if a coalition was present, 2. if it was deinite or possible, 3. of the location, 4. of the type, 5. of secondary signs, and 6. the frequency of bilaterality. RESULTS: In the initial retrospective study, coalitions were identiied in 0.6% of exams performed, including 78 calcaneonavicular coalitions. Seven fractures were seen, all in the ibrous calcaneonavicular subgroup. We found only eight cases (10.3%) of the "anteater" sign, while identifying 19 cases (24.4%) of the "reverse" anteater sign in the calcaneonavicular group. Talar "beaks" were seen in 15 cases (19.2%) of the calcaneonaviuclar group and nine (39.1%) of the subtalar group. Sixteen (69.6%) of the subtalar cases demonstrated the "drunken waiter" sign, a dysplastic sustentaculum. We saw a dramatic difference in frequency of tarsal coalitions when we applied a prospec- tive approach; 11.5% of all patients (66/574) had a coali- tion, while 12.2% of studies (70/574) demonstrated tarsal coalitions: 25.7% (18/70) were subtalar, 71.4% (50/70) were calcaneonavicular, and 2.9% (2/70) had both. The calcaneonavicular coalitions were roughly evenly divided between the cartilaginous (56%) and ibrous (44%) subtypes, without any osseous coalitions being identiied. The subtalar coalitions were also nearly evenly divided between osse- ous/partially osseous (33.3%), cartilaginous (27.8%), and ibrous (38.9%). Talar "beaks" were seen in 25 cases (50%) of the calcaneonavicular group and ive (27.%) of the sub- talar group. We identiied six cases (12%) of the "anteater" and nine cases (18%) of the "reverse anteater" signs in our calcaneonavicular group. CONCLUSION: Tarsal coalitions ap- pear to be more common than previously described. This is likely to be because ibrous and cartilaginous coalitions are infrequently recognized by plain radiography. The frequency of tarsal coalitions may be as high as 11%. We also found a disproportionately higher relative frequency of calcaneo- navicular coalitions, either because subtalar coalitions are more subtle on MRI or because calcaneonavicular coalitions can be overly diagnosed. Calcaneonavicular coalitions tend to be overwhelmingly nonosseous, while there is a more even distribution for subtalar coalitions. Newly described signs, such as the "reverse anteater" and "drunken waiter," are commonly seen in calcaneonavicular and subtalar coali- tions, respectively.

Clinical manifestations of type IV ulna longitudinal dysplasia.

PURPOSE: Ulna longitudinal dysplasia is an uncommon congenital anomaly that demonstrates a wide variety of clinical manifestations. The clinical manifestations and function of patients with Bayne type IV ulna longitudinal dysplasia have not been well characterized. The purpose of this study was to report the clinical features of type IV ulna longitudinal dysplasia and the extent to which this affects a patient's ability to perform activities of daily living. METHODS: The medical records of children diagnosed with ulna longitudinal dysplasia in our institution between 1960 and 2004 were reviewed. The children found to have ulna longitudinal dysplasia with radiohumeral synostosis (Bayne type IV ulna dysplasia) were studied. The laterality of the deformity, associated musculoskeletal and nonmusculoskeletal anomalies, and treatments were recorded. Patients were interviewed regarding their ability to perform activities of daily living. RESULTS: One hundred twenty-five patients with 146 affected limbs were identified with ulna dysplasia. Seventeen limbs in 14 patients (12% of affected limbs) demonstrated radiohumeral synostosis (RHS). Three of 14 patients with RHS had bilateral involvement. The elbows were fixed in 20 degrees to 90 degrees of flexion. No elbows were positioned in full extension. Eleven of the 17 involved limbs with RHS had digital anomalies. Nine of the 17 limbs had surgical reconstruction. The majority of these procedures were performed on the hand. CONCLUSIONS: The elbow, forearm, wrist, and hand clinical findings associated with type IV ulna longitudinal dysplasia are variable. Surgical treatment usually focuses on correction of hand abnormalities. Many patients function satisfactorily and are able to perform daily activities without surgical intervention.

Variations in calcaneonavicular morphology demonstrated with radiography.

PURPOSE: To determine and classify radiographically demonstrated variations in calcaneonavicular morphology and to estimate prevalence in a clinically relevant patient population. MATERIALS AND METHODS: Retrospective review was performed of foot radiographs obtained during diagnostic evaluation of 460 consecutive patients who presented to the emergency department with acute foot pain. Variations in calcaneonavicular morphology depicted on the medial oblique view (obtained at a 45 degrees angle) were classified into four groups according to morphologic type (types 1-4), and the prevalence of each type was calculated. Chi2 analysis was used to compare the prevalence of each type in male patients and in female patients. One-way analyses of variance were used to compare mean ages of patients for each type and mean calcaneonavicular gaps for each type. RESULTS: The prevalence of morphologic types 1, 2, and 3 was 94.3%, 2.8%, and 2.8%, respectively. The combined prevalence of types 2 and 3 (calcaneonavicular coalitions produced by synchondrosis and syndesmosis, respectively) was 5.6% (95% CI: 3.5%, 7.8%). There were no patients with type 4 morphology (synostosis). The numbers of male patients and female patients with morphologic types 1-3 were approximately equal (P =.9), and there was no statistically significant correlation between any of these three morphologic types and patient age (P =.2). The calcaneonavicular gap was significantly narrower in types 2 and 3 than in type 1 (P =.01), which was characterized as the normal morphology. CONCLUSION: The general prevalence of calcaneonavicular coalition (synchondrosis and syndesmosis) may be greater than previously reported, but further research is needed to prove the validity of this hypothesis.

An aetiological classification for developmental synostoses at the elbow.

Synostoses at the elbow joint are rare. The literature divides them into three groups based on the nature of bony ankylosis; the commonest are humeroradial synostoses. Approximately 150 cases have been reported. There are 29 reported cases of humeroradioulnar synostosis and five of humeroulnar synostosis. An anatomical classification was previously described for humeroradial synostoses. Due to significant phenotypic variability we believe a classification based solely on anatomical characteristics will in some cases be misleading. No classification exists for humeroradioulnar and humeroulnar synostosis. By re-examining the literature we have produced a combined classification for all elbow synostoses which more accurately predicts causes. Congenital elbow synostoses often cause little functional disability. Treatment by soft tissue release and osteotomy has been attempted, but although range of movement is initially, improved re-ossification is the norm. Investigation is more complicated and may be helped by classification which identifies syndrome association, risk of organ anomaly, and inheritance pattern.

[Congenital synostoses of the tarsus. Concept, classification, diagnosis and therapeutic approach]. / Sinostosis congénitas del tarso. Concepto, clasificación, diagnóstico y planteamiento terapéutico.

The incidence of congenital tarsal coalition is about 1%. It is recognized as the main etiology of painful, rigid flatfoot in the pediatric population. Talonavicular (50%) and talocalcaneal (40%) coalition are the most common presentation. We must suspect a tarsal coalition in a child with mechanical pain and shoes deformity. Rigid and painful planovalgus deformity are found in physical exam which led to describe in the pass this entity as peroneal spastic flatfoot. Lateral and oblique (35 degrees-45 degrees) radiographs must be practice to observe the coalition. The presence of a beak in the head of the talus or a half moon condensation image as the result of the superposition of the talus over the calcaneus are commonly described. CT-scan is also useful to delineate the size of the coalition and its location. Resection of the bar is the surgical treatment of choice. Excision of the coalition and interposition of fat or a graft must be tried in young patients in order to preserve foot biomechanical properties and to avoid long term problems associated with arthrodesis. Triple arthrodesis or subtalar arthrodesis must be used in older patients with degenerative signs in radiographs or in those cases of multiple coalition or if resection has failed.

Craniosynostosis in Western Australia, 1980-1994: a population-based study.

A craniomaxillofacial unit was established recently in Western Australia, and a study was carried out to provide some baseline characteristics of primary craniosynostosis in Western Australia and to investigate whether there has been any significant temporal change in birth prevalence. A case control study was conducted, using cases identified from a population-based register of birth defects, and a random sample of all births without a birth defect formed the control group. All subjects were born in Western Australia over the period 1980-1994 inclusive. The prevalence of craniosynostosis over the period 1980-1994 in Western Australia was 5.06 per 10,000 births. There was a significant linear increase in lambdoid synostosis over this period of 15.7% per year. Craniosynostosis was significantly more common among male infants, infants born preterm (<37 weeks gestation), breech presentation or presentations other than vertex, and infants born to fathers 40 years of age or older, even after accounting for known autosomal dominant syndromes. Other major birth defects were found in 11.2% of children with nonsyndromic craniosynostosis. Only 43 children (25.3%) with craniosynostosis were reported to have been seen by a geneticist. Thus, the prevalence of craniosynostosis in Western Australia is among the lowest reported. There is no current explanation for the increase in lambdoid synostosis. The increased risk of so-called nonsyndromic craniosynostosis with paternal age raises the possibility of undiagnosed new dominant mutations. This, along with the excess of other birth defects in children with craniosynostosis emphasises the need to ensure that these families are offered genetic counseling.

Mechanism for superior subluxation of the glenohumeral joint in fibrodysplasia ossificans progressiva.

Progressive heterotopic ossification leads to ankylosis of the major joints in patients who have fibrodysplasia ossificans progressiva. Joint subluxation has not been recognized widely in patients with this disease. The clinical records and radiographs of 79 patients with fibrodysplasia ossificans progressiva were reviewed and, it was found that humeral to chest wall synostosis and subluxation of the glenohumeral joint had occurred in 21% of skeletally immature patients and in 74% of skeletally mature patients. In fibrodysplasia ossificans progressiva, synostosis of the humeral shaft to the chest wall commonly occurs by 7 years of age, well before the age of proximal physeal closure. The continued growth of the proximal humeral physis in the presence of a humeral to chest wall synostosis causes the humeral head to migrate superiorly, thus promoting growth related subluxation. The clinical significance of this finding for patients who have fibrodysplasia ossificans progressiva is unknown, but this unique model will be useful in the study of shoulder biomechanics and growth plate physiology.