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Purpose: To analyze the outcome of surgical treatment of tarsal coalition, assess the role of the surgical technique, as well as of coalition size and type on outcomes., Methods: The search followed the Preferred Reporting Items of Systematic Review and Meta-Analysis and was performed in four databases: MEDLINE, Central, Scopus and Web of Science. The protocol has been registered in the international prospective register of systematic reviews. Patient-reported outcomes (PROMs), complications, revisions and radiographic recurrence were collected. Risk of bias was assessed using MINORS criteria. A random-effects model for meta-analysis was applied for analysis of data heterogeneity., Results: Twenty-five studies including 760 tarsal coalitions were included and had a weighted average follow-up of 44 months. Studies scored fair to poor on the risk of bias assessment with a mean MINORS score of 67% (44-81%). In 77.8% (37.5-100%) of surgically treated tarsal coalitions, good/excellent/non-limiting or improved PROMs were reported. Calculated data heterogeneity was moderate (I 2  = 57%). Open bar resection with material interposition had a clinical success rate of 78.8% (50-100%). Complications occurred in 4.96% of cases. Coalition size did not prove to be a determining factor in postoperative outcome. The influence of the coalition type was not investigated by any of the studies., Conclusion: Data on outcomes of surgical management for tarsal coalitions is limited to retrospective case series with high risk of bias and moderate data heterogeneity. In about ¾ of cases, open resection and interposition of material results in improved PROMs. The arbitrary margin of ≥ 50% of TC coalition size in relation to the posterior facet has little importance in surgical decision-making. None of the studies reported on the influence of the coalition type on postoperative clinical success., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Case: A 30-year-old woman underwent open reduction and internal fixation for multiple segmental rib fractures status post a motor vehicle collision. A year later, the patient presented with extensive intercostal heterotopic ossification associated with multilevel, hemithoracic, rib synostoses compromising her ventilation. The patient subsequently underwent synostoses excision and hardware removal. Pulmonary function tests (PFT), imaging, and patient-reported outcome scores demonstrate resolution of impairment., Conclusion: Resection of multilevel, intercostal, rib synostoses provided an effective treatment for pulmonary restrictive disorder secondary to traumatic rib synostosis. This is the first patient with documentation of prereconstructive and postreconstructive PFTs for chest wall synostosis excision., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/B851)., (Copyright © 2022 by The Journal of Bone and Joint Surgery, Incorporated.)

Case: A 43-year-old woman presented with pain, paresthesia, and coldness of the right upper extremity suggestive of the diagnosis of thoracic outlet syndrome. Three-dimensional computed tomography angiography revealed that the right subclavian artery was constricted because it traveled over an abnormal first rib. After anticoagulation and antithrombotic therapy, the patient underwent resection of the abnormal first rib. Postoperative angiography documented improvement over time of the poststenotic dilatation and recanalization of the subclavian artery capable of delivering almost normal distal flow., Conclusion: Arterial reconstruction is not always necessary for the treatment of arterial thoracic outlet syndrome associated with poststenotic dilatation of the subclavian artery., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/B827)., (Copyright © 2022 by The Journal of Bone and Joint Surgery, Incorporated.)

Cases: Two pediatric cases of post-traumatic distal radioulnar synostosis are reported, accompanied by a literature review summarizing evidence on the management of these conditions. Radioulnar synostosis is a rare complication of distal forearm fractures, which impairs upper-extremity function. The numerous surgical procedures that have been described to treat this condition in adults typically involve synostosis resection and an interposition graft to reduce recurrence. The optimal treatment in children has not been established., Conclusions: Post-traumatic radioulnar synostoses are rare conditions in pediatric patients who can be successfully treated with surgical excision of the synostoses and without the use of interposition grafting., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/B790)., (Copyright © 2022 by The Journal of Bone and Joint Surgery, Incorporated.)

49,XXXXY is the rarest X and Y chromosomal variation, with an incidence of 1 in 80,000-100,000 live male births and has been associated with numerous musculoskeletal abnormalities. Data was collected from an international cohort of boys with 49,XXXXY over 10 years. Children were evaluated by a multidisciplinary team consisting of a pediatric orthopedist, a neurogeneticist, a neurodevelopmentalist, and two physical therapists. Increased rates of torticollis (32.4%), hamstring tightness (42%), radioulnar synostosis (67.6%), pes planus (65.2%), and other foot abnormalities (86.9%) were observed. Several anomalies increased with age, specifically hamstring tightness, kyphosis, and scoliosis. The elucidation of the orthopedic profile of this population is necessary in order to provide healthcare providers with current medical information. This research further supports the necessity for the comprehensive multidisciplinary treatment of boys with 49,XXXXY., (© 2020 Wiley Periodicals, Inc.)

Intradiploic hematomas are extremely rare, particularly in newborns. Caused by bleeding between the inner and outer tables of the calvarium, they manifest with bony swelling of the skull. The authors present the first case of an intraosseous hematoma associated with synostosis, and the first report in a female patient. The clinical, radiological, surgical, and pathological characteristics of this lesion are discussed.

Pediatric care providers, pediatricians, pediatric subspecialty physicians, and other health care providers should be able to recognize children with abnormal head shapes that occur as a result of both synostotic and deformational processes. The purpose of this clinical report is to review the characteristic head shape changes, as well as secondary craniofacial characteristics, that occur in the setting of the various primary craniosynostoses and deformations. As an introduction, the physiology and genetics of skull growth as well as the pathophysiology underlying craniosynostosis are reviewed. This is followed by a description of each type of primary craniosynostosis (metopic, unicoronal, bicoronal, sagittal, lambdoid, and frontosphenoidal) and their resultant head shape changes, with an emphasis on differentiating conditions that require surgical correction from those (bathrocephaly, deformational plagiocephaly/brachycephaly, and neonatal intensive care unit-associated skill deformation, known as NICUcephaly) that do not. The report ends with a brief discussion of microcephaly as it relates to craniosynostosis as well as fontanelle closure. The intent is to improve pediatric care providers' recognition and timely referral for craniosynostosis and their differentiation of synostotic from deformational and other nonoperative head shape changes., Competing Interests: This document is copyrighted and is property of the American Academy of Pediatrics and its Board of Directors. All authors have filed conflict of interest statements with the American Academy of Pediatrics. Any conflicts have been resolved through a process approved by the Board of Directors. The American Academy of Pediatrics has neither solicited nor accepted any commercial involvement in the development of the content of this publication. POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2020 by the American Academy of Pediatrics.)

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., (© 2020 Wiley Periodicals, Inc.)

Mesoaxial synostotic syndactyly (MSSD) with phalangeal reduction is an uncommon congenital limb abnormality characterized by central osseous synostosis at a metacarpal level, mesoaxial reduction of the fingers, and preaxial cutaneous syndactyly in toes. In rare cases, the disease is also associated with fifth finger clinodactyly and postaxial polydactyly. It has autosomal recessive inheritance pattern caused by homozygous variants in the gene BHLHA9 mapped at chromosome 17p13.3. In the present study, a consanguineous family of Pakistani origin segregating MSSD in autosomal recessive form was characterized at clinical and genetic levels. Clinically, the diseased individuals have MSSD associated with clinodactyly and polydactyly. Homozygosity mapping followed by Sanger sequencing of BHLHA9 revealed a novel frameshift variant NM_001164405.1: c.409-409delC; p.(His137Thrfs*61) segregating with the disease phenotypes in the family. This is the second report providing evidence of association of polydactyly with MSSD caused by frameshift variant in the gene BHLHA9. The present molecular investigation will support genetic counselling of the local population carrying diseased variants., (Copyright © 2019. Published by Elsevier Masson SAS.)

Treatment of chronic Monteggia fracture-dislocations remains controversial in skeletally immature patients. The present study aimed to review the clinical outcomes of surgical treatment with an Ilizarov mini-fixator for chronic Monteggia fracture-dislocations in children. From April 2003 to March 2014, 5 pediatric patients (4 males, 1 female) with chronic Monteggia fracture-dislocation were treated with an Ilizarov mini-fixator at our institution. The median age at the time of surgery was 9 years (range 5-14 years), median duration from injury to surgery was 31 months (range 2-125 months), and median duration of follow-up was 12 months (range 11 months-10 years). All patients underwent opening wedge osteotomy of the proximal ulna followed by the application of an Ilizarov mini-fixator. Although closed reduction was attempted after ulnar osteotomy and application of the Ilizarov mini-fixator, open reduction of the radial head was required in all patients. In 4 patients, dense scar tissue in the radiocapitellar joint was excised to enable reduction of the radial head; the remaining patient had traumatic radioulnar synostosis, and underwent separation of the synostosis followed by anconeus interposition arthroplasty. No patient received bone grafting at the ulnar osteotomy site, repair or reconstruction of the annular ligament, or temporary fixation of the radial head with transarticular wire. The median period of external fixation was 10 weeks (range 8-13 weeks). Although there were no severe complications such as deep infection and neurovascular disturbance, asymptomatic radial head subluxation occurred in 2 patients. The patient with traumatic synostosis had residual posterior subluxation with limitation of forearm rotation, and another patient with radial head enlargement had residual anterior subluxation. The median postoperative ranges of motion in pronation, supination, extension, and flexion were 90°, 90°, 0°, and 140°, respectively. The median Kim's elbow performance score was 65 (range 50-75) preoperatively, which improved to 94 (range 80-100) at final follow-up. The outcome was rated as excellent in 4 cases, and good in 1. In pediatric chronic Monteggia fracture-dislocations, ulnar osteotomy followed by the application of an Ilizarov mini-fixator is a viable option that is less invasive than plate fixation.

CASE DESCRIPTION Over a 2-year period, 6 horses (4 Selle Français, 1 Hanoverian, and 1 Thoroughbred) were referred for evaluation of forelimb lameness. All horses had radiographic evidence of synostosis of the first and second ribs (SFSR). CLINICAL FINDINGS For 1 horse, the SFSR was considered the probable cause of the lameness (grade 3/5), with a shortening of the cranial phase of the stride in the affected limb. For 3 horses, it was considered a possible cause of the lameness (grade 1/5) for the same reason. For 2 horses, SFSR was considered an incidental finding unassociated with any clinical signs. The 4 horses with lameness suspected as attributable to SFSR had a moderate to severe amount of irregularly marginated new bone formation at the site of the SFSR, with a cranial displacement of the first rib, compared with findings for the 2 horses in which the SFSR was considered incidental. A likely congenital abnormality of the first rib was first suspected on nuclear scintigraphy in the 1 horse for which it was performed or on radiography of the caudal cervical portion of the vertebral column (3 horses) or shoulder joint (2 horses). TREATMENT AND OUTCOME The horse in which SFSR was considered the probable cause of the lameness was retired to the field and remained chronically lame. Two of the 3 horses in which SFSR was considered a possible cause of lameness received an IV infusion of tiludronate disodium and mesotherapy over the caudal cervical and cranial thoracic regions; both returned to competition but with poor results. One of the 2 horses with subclinical SFSR never developed lameness on the affected side. No follow-up information was available for the other 2 horses. CLINICAL RELEVANCE SFSR can be an incidental finding in horses, with or without clinical manifestations. This abnormality should be considered as a differential diagnosis for horses with forelimb lameness and associated shortening of the cranial phase of the stride that fails to improve with diagnostic analgesic techniques.

Congenital dislocation of the patella is a rare and difficult pathology to treat. We present a case of bilateral congenital dislocation of the patella with synostosis of proximal tibiofibular and proximal radioulnar joints without genu valgum deformity of both knees in a 30-year-old man. To our knowledge, congenital dislocation of the patella associated with synostosis of proximal tibiofibular and proximal radioulnar joints has not been reported in the literature yet.

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., (Copyright © 2017 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

Case: We describe the case of a 40-year-old man with Minnaar type-III congenital synostosis of the lunate and the triquetrum who presented with Lichtman stage-I Kienböck disease. Surgical treatment consisted of capitate shortening with a capitate-hamate fusion., Conclusion: This case demonstrates the tenuous regional nature of the vascularity to the lunate, even in the setting of a complete lunotriquetral synostosis.

Introduction: There is still a controversial discussion on the treatment of talocalcaneal coalition with and without planovalgus deformity. From 2002-2014 80 cases of talocalcaneal coalition in children and adolescents under 18 years of age were surgically treated by J. Hamel. The objective of this study is a retrospective analysis, especially of unfavourable results., Methods: Patients with minimum follow up of 12 months were included (35.7 months on average). In 31 cases treatment consisted of resection and fat-grafting (group 1), in 26 additional cases tarsal osteotomy was added because of planovalgus-deformity (group 2), and in 23 cases primary fusion of the talocalcaneal joint was performed (group 3)., Results: In group 1 one patient was lost to follow-up early with a severe peroneal spasticity and unknown further course. Two further patients still had remarkable pain after 15 months, and another patient had to undergo secondary fusion because of persistent pain. In group 2 secondary fusion was undertaken in two cases and offered in four other cases. In group 3 two patients still suffered from moderate tarsal pain, although complete fusion occurred; one of these patients underwent bilateral treatment. All other patients were pain free or nearly pain free on the last follow-up visit, with marked improvement in comparison to the preoperative situation., Discussion: Surgical treatment of talocalcaneal coalition is successful in most cases in the short to medium follow-up, but unfavourable results are not uncommon and may require secondary fusion. Differential indication between resection, additional deformity correction, or primary fusion seems to be most important.

Objective: To describe the intraoperative findings and outcomes of stapedectomy surgery in Teunissen-Cremers syndrome., Patients: A family of three patients with bilateral conductive hearing loss because of Teunissen-Cremers syndrome., Intervention: Six exploratory tympanotomies and stapedectomies, including one revision operation., Main Outcome Measures: Intraoperative findings and postoperative hearing results., Results: There was an increased distance between the incus and the vestibule, a thicker long process of the incus, and slight variation in the relative position of the ossicles in all patients. Surgery resulted in closure of the air-bone gap to less than 10 dB in all five operated ears., Conclusion: We describe anatomic abnormalities of the ossicular chain in Teunissen-Cremers syndrome. Prosthesis availability should include prostheses that can adapt to these potential anatomic abnormalities. Stapedectomy resulted in good long-term hearing outcomes in this series.

Purpose: There are little patient-reported data on functional outcomes of tarsal coalition resection in children and adolescents. The purpose of this study is to evaluate the medium-term (>2 y) outcomes in patients who have had surgical excision of their symptomatic tarsal coalition and to compare patient-based outcomes in patients who have calcaneonavicular (CN) coalitions to those with talocalcaneal (TC) coalitions., Methods: A billing query was conducted to identify patients who had surgical excision of their tarsal coalition between 2003 and 2008. Eligible patients were mailed questionnaires consisting of a modified American Orthopaedic Foot and Ankle Society (AOFAS) score and the University of California at Los Angeles (UCLA) activity scale. Patients were also specifically asked if their activity level was limited by their foot pain. Only patients who returned questionnaires were included. Demographics and diagnostic images were reviewed. A nonresponder analysis was completed. Complications such as infection and reoperation were reported., Results: Sixty-three patients (22 females, 41 males) who returned questionnaires were included in the analysis. Twenty-four patients had bilateral surgery. TC coalitions were present in 20 patients (32%); CN coalitions were present in 43 patients (68%).Overall, mean modified AOFAS score was 88.3 and mean UCLA activity score was 8.33 at an average of 4.62 years after surgery. Patients who had TC coalitions had similar modified AOFAS scores (88.4) and UCLA activity scores (8.4) when compared with those with CN coalitions (88.0 and 8.3, both not significant).Of the 73% (46/63) patients who reported that their activity levels were not limited by their foot pain, the mean AOFAS score was 93.9 and the mean UCLA activity score was 8.9; 32 of these were CN and 14 were TC coalitions. Of the 27% (17/63) patients who reported that their activity levels were limited by their foot pain, the mean AOFAS score was 72.9 and the mean UCLA activity score was 6.9; 11 of these were CN and 6 were TC coalitions. There was a statistically significant difference in these groups both in modified AOFAS score (P<0.0001) and UCLA activity score (P=0.006). There was no difference in outcomes between those who were treated for a TC and CN coalition., Conclusions: Patient-reported outcomes after surgical excision of tarsal coalition reveal that >70% of patients' activities are not limited by pain and their functional outcome is terrific. A few patients continue to have problems with ongoing foot pain and activity limitations. The type of coalition does not seem to be an indicative factor in determining outcome.

Diaphyseal tibiofibular synostosis is a rare cause of symptomatic shin pain with exertion. In this case, a 14-year-old male soccer player presented with atraumatic right shin pain made worse with running. Computed tomography revealed heterotopic ossification, or synostosis, of the tibial-fibular syndesmosis. The patient's symptoms improved with rest, without the need for operative intervention., (Copyright © 2015 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.)

We present 2 cases of combined arterial and neurogenic thoracic outlet syndrome triggered by trauma in patients with congenital synostoses of the first and second ribs. These patients were successfully treated with supraclavicular resection of the first and second ribs and scalenectomy. We review these cases and the associated literature on thoracic outlet syndrome and rib synostosis., (Copyright © 2014 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

The peroneal tubercle is an osseous structure on the lateral side of the calcaneus present in 90% of individuals. Hypertrophy of the peroneal tubercle resulting in stenosing peroneal tenosynovitis has been well described in the literature. Repair of this condition involves operative treatment to remove the hypertrophied peroneal tubercle and repair any resulting tendon pathology. The authors report a unique case of a hypertrophied peroneal tubercle with an associated tarsal coalition, resulting in complete bony encasement of the peroneal tendons. In this case, a 50-year-old white man presented with worsening bilateral foot and ankle pain for several years. On examination, he had fixed hindfoot varus and bilateral equinocavovarus feet. Magnetic resonance imaging and weight-bearing radiographs showed a calcaneonavicular coalition. Intraoperatively, the authors discovered complete bony encasement of the peroneal longus and brevis tendons. On examination, the peroneal longus and brevis were severely stenotic, with the peroneal brevis to the point of near laceration. This painful condition was repaired by takedown of the calcaneonavicular coalition, the peroneal tubercle was resected, and the peroneal tendons were freed from their bony encasement. Tenodesis of the peroneus brevis to longus was performed and the hindfoot varus was corrected with wedge osteotomy of the calcaneus. The patient reported excellent postoperative results. At 3 months postoperatively, he was pain-free and his calcaneal osteotomy was well healed. This case appears to be the first of its type to be reported in the literature. The details of the case are presented along with a review of the relevant literature., (Copyright 2014, SLACK Incorporated.)

Object: Unilateral fusion of the frontoparietal suture is the most common cause of synostotic frontal plagiocephaly. Localized fusion of the frontosphenoidal suture is rare but can lead to a similar, but subtly distinct, phenotype., Methods: A retrospective chart review of the authors' craniofacial database was performed. Patients with isolated frontosphenoidal synostosis on CT imaging were included. Demographic data, as well as the clinical and radiographic findings, were recorded., Results: Three patients were identified. All patients were female and none had an identifiable syndrome. Head circumference was normal in each patient. The mean age at presentation was 4.8 months (range 2.0-9.8 months); 2 fusions were on the right side. Frontal flattening and recession of the supraorbital rim on the fused side were consistent physical findings. No patient had appreciable facial angulation or orbital dystopia, and 2 patients had anterior displacement of the ipsilateral ear. All 3 patients were initially misdiagnosed with unilateral coronal synostosis, and CT imaging at a mean age of 5.4 months (range 2.1-10.8 months) was required to secure the correct diagnosis. Computed tomography findings included patency of the frontoparietal suture, minor to no anterior cranial base angulation, and vertical flattening of the orbit without sphenoid wing elevation on the fused side. One patient underwent CT scanning at 2.1 months of age, which demonstrated a narrow, but patent, frontosphenoidal suture. The patient's condition was assumed to be a deformational process, and she underwent 6 months of unsuccessful helmet therapy. A repeat CT scan obtained at 10.7 months of age demonstrated the synostosis. All 3 patients underwent fronto-orbital correction at mean age of 12.1 months (range 7.8-16.1 months). The mean duration of postoperative follow-up was 11.7 months (range 1.9-23.9 months)., Conclusions: Isolated frontosphenoidal synostosis should be considered in the differential diagnosis of atypical frontal plagiocephaly.

Poland's syndrome is a rare congenital anomaly characterised by partial or complete absence of sternocostal head of pectoralis major muscle and anomalies of ipsilateral hand and digits. Other associated anomalies involving anterior thoracic wall, breast, diaphragm and vertebrae have also been reported in various cases. We report a case of a 10-year-old girl, with features of left-sided Poland's syndrome associated with spina bifida, dextroposition of the heart and left-sided diaphragmatic hernia. These are rare associations of Poland's syndrome. She was investigated with chest X-ray, contrast-enhanced CT of the thorax, ultrasonography of abdomen and echocardiography which helped in arriving at an accurate diagnosis and assessing all the associated abnormalities.

Objective: To investigate the diagnosis and mapagement of plagiocephaly resulted from For frontosphenoidal synostosis, frontosphenoidal synostosis and squamosal suture synostosis., Methods: orthotic nolding therapy should be selected for patients under 3-month-old. If it can' t make effect after treatment for 3 months, transcranial surgery should be adopted with the horizontal advancement of orbitofrontal bar at the affected side. Patients with squamosal suture synostosis should receive prohotic molding From 2005 to 2012, five patients were therapy for six month, remolding the cap every month., Results: treated with satisfactory result, including 3 patients with frontosphenoidal synostosis who received surgery and 2 patiens with squamosal suture synostosis who receive orthotic molding therapy only., Conclusions: Few patients with plagiocephaly are caused by frontosphenoidal synostosis and squamosal suture synostosis. The orthotic molding therapy should be selected at an early time, but mostly, surgery may be needed for

Congenital fusion of the mandible to the maxilla (syngnathia) is a rare disorder. The first case was reported in 1936 and only a few cases have been reported in the literature since then. This paper reports three new cases of syngnathia. Clinical and radiographic features are presented, as well as surgical management and complications., (Copyright © 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

A 64-year-old man, 7 years after cervical trauma, presented with severe dysphagia of 3-month duration. Computed tomography showed an unusual synostosis between the thyroid cartilage and the cervical spine at C5-6-7 on the right side. A barium swallow study revealed no laryngeal elevation during swallowing. Surgical resection of the bony fusion was performed, and the patient's dysphagia immediately improved without any complications. We report a case of delayed synostosis between the thyroid cartilage and the cervical spine causing severe dysphagia 7 years after cervical trauma. Surgical resection of the bony fusion resulted in immediate improvement of the dysphagia., (Copyright © 2012 Elsevier Inc. All rights reserved.)

This is a report of mandibulo-zygomatic arch synostosis in a dog 7 mo after trauma to the maxilla. Advanced diagnostic imaging was considered essential for characterization of the condition and treatment planning. Surgical excision of the bony proliferation and physiotherapy resulted in improved function within 6 wk.

This paper presents a case report of persistent low back pain and suspected lumbar radiculopathy. A synostosis at the level of the proximal tibiofibular joint was diagnosed. After successful resection of the synostosis, the low back symptoms resolved completely. This is the first report of a proximal tibiofibular synostosis as a possible cause of referred pain proximally.

Surgical wrist denervation involves division of the anterior and posterior interosseous nerves and articular branches of the superficial radial nerve. In this outcome study, 37 patients were individually assessed and deemed suitable for denervation surgery due to appreciable symptom resolution following a local anesthetic wrist block. At a mean of 18 months following denervation surgery, median activity pain scores had decreased by 60% (p < 0.001) from initial assessment levels, and more than three quarters (30/37) of patients reported continued improvement in their activity pain (p < 0.001). More than two thirds of patients had a satisfaction VAS of greater than 50, with less postoperative resting pain and a greater reduction in postoperative activity pain as the important predictors of patient satisfaction. Thirty-one out of the 37 patients had not represented to our department for revision wrist surgery by a mean of 10.3 years follow-up. We have found this procedure useful in ameliorating symptoms for some patients who would conventionally have required partial or total wrist fusions with greater residual functional limitation.

Congenital bony fusion of the maxilla and mandible, especially as an isolated occurrence, is a very rare condition. The very few cases reported in the literature are mostly inadequate in description and confusing in nomenclature. An isolated case of syngnathia in a 60-year-old female patient with unilateral bony fusion (synostosis) of the maxilla and mandible associated with fibrous adhesions (synechiae) of the opposite site is reported. The existent literature is also reviewed.

Congenital maxillomandibular fusion (syngnathia) is rare. The first case was reported in 1936. By 2004, only 25 cases of syngnathia had been reported including 6 cases with fusion of the ascending rami of the mandible with the maxilla and zygomatic complex. This report concerns a 6-month-old girl with a type of congenital fusion of the ascending rami of the mandible with the upper alveolar arch and zygomatic complex that leads to restriction of mouth opening. The clinical features of this anomaly and a brief review of literature are presented., (Copyright © 2010 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Congenital fusion of the jaws is the inability to open the mouth at the time of birth. It occurs as a result of fusion of the jaws or fusion of the mandible to zygoma or temporal bone. The authors report four case studies and describe their management protocol., (Copyright © 2010 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

A rare case of syngnathia (maxillomandibular fusion) is described in a 2-year-old boy who presented from a rural area of Saudi Arabia. The child had the problem since birth and had undergone two surgical attempts to release the fusion at another institution but both had failed and recurrence occurred. In the authors' institute, the patient underwent two separate surgical procedures at the age of 2 and 3 years. Both procedures were followed by a period of aggressive physiotherapy, but the patient presented with re-fusion of the mandible and the maxilla 1 year after the first operation and 5 months after the second operation. It was decided to defer treatment until puberty. The cause of the relapse may be the high osteogenic potential because of his youth or the failure of the parents to maintain the active physiotherapy protocol because they live in a rural area and close follow-up was difficult. A possible genetic predisposition for bone formation at this site should be investigated., (Copyright © 2010 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Summary: This report summarizes the clinical management of an infant with a proximal radio-ulnar synostosis and inherited bone marrow failure syndrome (PRUS/IBMFS). Molecular studies were negative for the characteristic HOXA11 mutation described earlier. He was successfully treated with a non-myeloablative hematopoietic stem cell transplantation from an human leukocyte antigen-identical sibling donor at the age of 3 months. We reviewed the literature on PRUS/IBMFS with an emphasis on the current understanding of the molecular mechanisms involved in the disease pathogenesis. Absence of the HOXA11 mutation in this case implies that molecular mechanisms beyond the HOXA11 gene, yet to be discovered, may contribute for the development of PRUS/IBMFS.

We report the case of a talar body fracture occurring in association with a previously undiagnosed talocalcaneal coalition. A rotational injury during a football tackle produced a grossly deformed ankle that was reduced in the emergency department. The unusual appearance of the medial sub-talar region on plain radiographs prompted further imaging by computerised tomography, which confirmed an osseous talocalcaneal coalition. Operative findings included a talar body fracture that could only be reduced after excision of the osseous coalition. The fracture was reduced and fixed. The patient made a good postoperative recovery with favourable AOFAS scores in the medium term at 1 year., (Copyright 2009 European Foot and Ankle Society. Published by Elsevier Ltd. All rights reserved.)

We have evaluated and treated 3 cases of acute onset elbow flexion contracture in children ranging in age from 6 to 13 years, with type-IV (Cleary and Omer) congenital proximal radioulnar synostosis. All were resolved by manipulative traction under general anesthesia during which an audible elbow "snap" was noted. Two of the cases required surgical treatment of a deformed radial head with good results after a 2 to 4-year follow-up. Our clinical radiographic and intraoperative evidence suggests that the acute onset fixed-flexion contracture results from an anterosuperiorly overgrown dislocated radial head becoming trapped under hypertrophied annular ligament-type tissue after elbow hyperflexion. Excision of the dislocated radial head after release from its entrapment was effective in 2 of the 3 patients in this report, and should be considered.

Symptomatic middle facet talocalcaneal coalition is frequently associated with rearfoot arthrosis that is often managed surgically with rearfoot fusion. However, no objective method for classifying the extent of subtalar joint arthrosis exists. No study has clearly identified the extent of posterior facet arthrosis present in a large cohort treated surgically for talocalcaneal coalition through preoperative computerized axial tomography. The authors conducted a retrospective review of 21 patients (35 feet) with coalition who were surgically treated over a 12-year period for coalition on at least 1 foot. Using a predefined original staging system, the extent of the arthrosis was categorized into normal or mild (Stage I), moderate (Stage II), and severe (Stage III) arthrosis. The association of stage and age is statistically significant. All of the feet with Stage III arthrosis had fibrous coalitions. No foot with osseous coalition had Stage III arthrosis. The distribution of arthrosis staging differs between fibrous and osseous coalitions. Only fibrous coalitions had the most advanced arthrosis (Stage III), whereas osseous coalitions did not. This suggests that osseous coalitions may have a protective effect in the prevention of severe degeneration of the subtalar joint. Concomitant subtalar joint arthrosis severity progresses with age; surgeons may want to consider earlier surgical intervention to prevent arthrosis progression in patients with symptomatic middle facet talocalcaneal coalition.

Surgical reconstruction of symptomatic flatfoot associated with middle facet tarsal coalition is becoming more widely used. This article demonstrates that coalition-concomitant flatfoot is a pathologic entity that is worthy of surgical management. The literature, although limited, has suggested that poor outcomes with isolated simple coalition resection may have been related to the preoperative pes planus that was not addressed. More recently studies have demonstrated improved clinical and radiographic postoperative outcomes when the flatfoot correction is combined with the coalition resection. This article reviews a surgical treatment algorithm that considers the presence of varying degrees of pes planus and rearfoot arthrosis associated with coalition.

Tarsal coalition is a congenital condition characterized by the aberrant union (osseous or fibrous) between 2 bones in the rearfoot, most commonly talocalcaneal coalition, calcaneonavicular coalition, and talonavicular coalition, that results in a restriction or absence of motion. The association between tarsal coalition and a variety of coexisting conditions has been reported over the past 60 years and continues to be better understood. These coexisting conditions (the stigmata of tarsal coalition) have been believed to be secondary effects of the coalition and/or fixed rearfoot position. Advanced imaging has provided significant insights into the concomitant pathology and understanding of tarsal coalition that the symptoms associated with tarsal coalition can be present for a myriad of different reasons. One should consider all the stigmata of tarsal coalition when considering a surgical reconstruction.

Verloes-David-Pfeiffer mesomelia-synostoses syndrome is an autosomal-dominant form of mesomelic dysplasia comprising typical acral synostoses combined with ptosis, hypertelorism, palatal abnormality, CHD, and ureteral anomalies. Since the original reports in 1995, two other patients have been described with this syndrome, one of them the patient reported in 1998 by Day-Salvatore. In this article, we report on the follow-up of some of the original cases and review the literature. We confirm that the Verloes-David-Pfeiffer syndrome (VDPS) is a progressive skeletal disorder that despite repeated corrective surgical intervention leads to severe limb deformities. No mutations were detected in the FLNB gene. To date, the cause and the pathogenesis of VDPS remain unknown. The latter is characterized in this study as a syndromic type of skeletal dysplasia because besides congenital malformations and multiple acromelic synostoses arising prenatally, VDPS manifests in postnatal life as a severe osteochondrodysplasia.

Brachydactyly refers to shortening of the hands and/or feet due to missing, deformed, or shortened bones. It may occur as an isolated trait or as part of a syndrome. According to their pattern of skeletal involvement, the isolated brachydactyly forms have been categorized in the groups A-D including several subgroups. As in many other genetic conditions, there is considerable phenotypic overlap between the groups. The identification of the molecular causes of these conditions has offered insights into their pathogenesis. The generation of animal models has facilitated research on the pathogenic events during digit development that lead to the brachydactyly phenotype. These studies have shown that the BMP pathway plays a pivotal role in the normal development of digits and joints and that the majority of brachydactyly disease genes are directly or indirectly linked to this pathway. Together, these genes function in a regulatory network which is deregulated in the disease state. As a consequence of the close interactions within the network, overlapping phenotypes are generated that are, nevertheless, characterized by specific recognizable patterns. This principle does not only apply for the brachydactylies but is also valid for many other disease entities. Groups of diseases that show a common phenotypic pattern due to the deregulation of a molecular network are suggested to be called molecular disease families.