Prevalence of Lumbosacral Transitional Vertebrae in Patients With Symptomatic Femoroacetabular Impingement Requiring Hip Arthroscopy.

PURPOSE: The primary aim of this study was to determine the prevalence of lumbosacral transitional vertebrae (LSTVs) in patients with symptomatic femoroacetabular impingement (FAI) requiring hip arthroscopy. The secondary aim was to determine whether there is an association between LSTV anatomy and patient-reported outcomes. METHODS: This retrospective study included patients aged 18 to 45 years with symptomatic FAI who underwent arthroscopy between March 2010 and March 2016 and had anteroposterior pelvic radiographs. The exclusion criteria included lack of an FAI diagnosis, hip osteoarthritis (Tönnis grade ≥ 2), prior spinal fusion surgery, prior total hip arthroplasty, indications for total hip arthroplasty, and revision surgery on the affected hip. All radiographs were assessed by an interventional spine and sports fellow. The primary outcome was the prevalence of LSTVs, classified using the criteria of Castellvi et al. Secondary outcomes included the modified Harris Hip Score, Hip Outcome Score, and International Hip Outcome Tool 33 score. RESULTS: A total of 1,880 patients were included. Review of the patients' radiographs yielded 262 LSTVs, for an overall prevalence of 13.9% (type IA in 104 [5.5%], type IB in 53 [2.8%], type IIA in 60 [3.2%], type IIB in 25 [1.3%], type IIIA in 8 [0.4%], type IIIB in 0 [0%], and type IV in 12 [0.64%]). The prevalence of type II, III, and IV LSTVs was 5.6% (n = 105). Unilateral LSTV sidedness did not correlate with symptom laterality (κ = 0.07). There were no differences in patient-reported outcomes between patients with LSTV anatomy and those without it. CONCLUSIONS: In this large cohort of 1,880 patients with symptomatic FAI, the prevalence of LSTVs was 13.9%. There was no correlation between sidedness of unilateral LSTVs and the symptomatic hip. Furthermore, there was no association between LSTV anatomy and patient-reported outcomes. The prevalence of LSTVs in this cohort was similar to the prevalence rates previously reported in patients with low-back pain. LEVEL OF EVIDENCE: Level IV, case series.

Three-Dimensional Analysis of Hemimetameric Segmental Shift in Congenital Scoliosis.

STUDY DESIGN: This is a retrospective cohort study. BACKGROUND: Hemimetameric segmental shift (HMMS) is defined as a hemivertebral deformation in which 2 or more hemivertebrae exist on both sides of the spine and are separated by at least 1 normal vertebra. Reports of HMMS are rare and based on simple anterior x-ray images. No reports have used 3-dimensional computed tomography (3D-CT) to analyze both the anterior and posterior elements. The objective of this study was to analyze the morphology and clinical features of HMMS 3 dimensionally. METHODS: HMMS was confirmed in 32 (6.6%, 16 males and 16 females) of 483 patients diagnosed with congenital scoliosis at the study institution between 1998 and 2013. The average age at the first visit was 6 years and 3 months. 3D-CT imaging was performed for 30 patients older than 2 years (average age: 9 y and 8 mo) and used to classify cases according to posterior elements. RESULTS: With regard to the number of hemivertebrae present, 21 patients had 2 hemivertebrae, 7 patients had 3 hemivertebrae, and 2 patients had 4 hemivertebrae. Patients with 2 hemivertebrae predominantly had hemivertebrae in the thoracolumbar spine. Patients were classified into 2 categories: malformation existing at an equal level in anterior and posterior sides (unison HMMS) and malformation existing at nonequal levels (discordant HMMS). Nine patients had unison HMMS and all of them had 2 hemivertebrae (average: 4.6 vertebrae). Twenty-one patients had discordant HMMS, with 12 having 2 hemivertebrae, 7 having 3 hemivertebrae, and 2 having 4 hemivertebrae. CONCLUSIONS: Through 3D-CT analysis, HMMS was categorized as unison or discordant. Discordant HMMS was observed in 21 of 30 (70%) patients and in all patients with >3 hemivertebrae. Diagnosing HMMS, whether unison or discordant, is clinically important and should be done with careful analysis of bone models and/or radiologic images to determine the correct spinal levels. LEVEL OF EVIDENCE: Level IV-diagnostic study.

Posttraumatic Tibiofibular Synostosis After Treatment of Distal Tibiofibular Fractures in Children.

INTRODUCTION: Posttraumatic pediatric distal tibiofibular synostosis is a rare complication following fracture. This is a retrospective, multicenter case series of synostosis of distal tibiofibular fractures in children. The purpose was to evaluate the incidence and pattern of posttraumatic distal tibiofibular synostosis in children. METHODS: Of the 604 pediatric distal tibiofibular fractures, 20 patients (3.3%) with synostosis after treatment of distal tibiofibular fractures were identified at 3 tertiary referral centers. There were 12 boys and 8 girls, with a mean age of 8.4±2.0 years (range, 3.7 to 11.5 y) at the time of injury. Medical records were reviewed, and serial radiographs were analyzed to determine fracture configuration, pattern of synostosis, and changes in the relative positions of the proximal and distal tibial and fibular physes and in the alignment of the ankle. RESULTS: The time from the occurrence of fracture until the recognition of the synostosis ranged from 2 to 6 months (mean, 2.8 mo). The most common fracture configuration was oblique tibial fracture combined with comminuted fibular fracture. There were 12 focal types and 8 extensive types. The proximal tibiofibular distance was decreased in 13 patients. Proximal migration of the distal fibular physis developed in all cases. Five patients exhibited ankle valgus of 10 degrees or greater with moderate or severe distal fibular shortening. Eight patients were symptomatic after synostosis and 12 patients were asymptomatic. CONCLUSIONS: We identified 2 patterns of synostosis after the treatment of pediatric distal tibiofibular fracture: focal and extensive. The focal type was more prevalent than the extensive type, which was more likely to occur due to high-energy injury. When a tibiofibular cross-union develops, it creates growth abnormalities that warrant observation and potential treatment, as it may lead to progressive deformity or ankle pain. LEVEL OF EVIDENCE: Level IV.

Carpal Coalitions and Metacarpal Synostoses: A Review.

Background: Carpal coalition and metacarpal synostosis are uncommon congenital anomalies of the carpus and hand. Methods: A comprehensive review of the literature was performed to help guide surgical and non-surgical treatment of carpal coalition and metacarpal synostosis. Results: The embryology, epidemiology, medical and surgical management, and associated outcomes are detailed. Conclusions: Most patients with these disorders will likely benefit from conservative measures. Surgery should be considered in patients with pain and limitations in wrist and hand function.

Isolated ring-little finger metacarpal synostosis: a new classification system and treatment strategy.

PURPOSE: To devise a comprehensive classification system for isolated ring-little finger metacarpal synostosis that offers a clear guide to specific treatment for each variation of the deformity. METHODS: Based on the experience of 13 cases (20 hands) of isolated ring-little finger metacarpal synostosis, we devised a classification system that takes into account 2 key pathological features of this malformation: the fourth-fifth intermetacarpal angle and the severity of hypoplasia of the fifth ray. In our classification, all patients were divided into 3 types, according to the fourth-fifth intermetacarpal angle, and each type was further subdivided into 2 subtypes according to the length of the fifth ray. RESULTS: All 20 hands could be classified according to our classification, including 2 hands of type A (both A1), 10 hands of type B (9 B1; 1 B2), and 8 hands of type C (7 C1; 1 C2). Patients of different classification types received different treatments according to our proposed classification-related guidelines. It was also possible to classify all the cases found in literature according to this scheme. CONCLUSIONS: Our classification for ring-little finger metacarpal synostosis is simple and easy to remember. It is applicable to all possible variations of the congenital anomaly and can guide treatment for the whole spectrum of the deformity. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.

Occipitalization of the atlas in children. Morphologic classification, associations, and clinical relevance.

BACKGROUND: Occipitalization is defined as a congenital fusion of the atlas to the base of the occiput. We are not aware of any previous studies addressing the morphologic patterns of occipitalization or the implications of occipitalization in children. We present data on what we believe is the largest reported series of children with occipitalization studied with computed tomography and/or magnetic resonance imaging, and we provide a description of their clinical characteristics. METHODS: We retrospectively reviewed all cases of occipitalization in children included in our spine database. Patient charts and imaging studies were reviewed. A new morphologic classification of occipitalization was developed from the two-dimensional sagittal and coronal reformatted computed tomographic reconstructions and/or magnetic resonance images. The classification includes four patterns according to the anatomic site of occipitalization (Zones 1, 2, and 3 and a combination of those zones), and it was applied to this group of patients. Imaging studies were also reviewed for evidence of cervical instability and for other anomalies of the craniovertebral junction. RESULTS: Thirty patients with occipitalization were identified. There were twenty-four boys and six girls with a mean age of 6.5 years. The morphologic categorization was Zone 1 (a fused anterior arch) in six patients, Zone 2 (fused lateral masses) in five, Zone 3 (a fused posterior arch) in four, and a combination of fused zones in fifteen. Seventeen patients (57%) had atlantoaxial instability, and eight of them had an associated C2-C3 fusion. Eleven patients (37%) had spinal canal encroachment, and five of them had clinical findings of myelopathy. The highest prevalence of spinal canal encroachment (63%) was noted in patients with occipitalization in Zone 2. CONCLUSIONS: Occipitalization is associated with abnormalities that lead to narrowing of the space available for the spinal cord or brainstem. The risk of atlantoaxial instability developing is particularly high when there is an associated congenital C2-C3 fusion. Two-dimensional sagittal and coronal reformatted computed tomographic reconstructions and/or magnetic resonance images can help to establish the diagnosis and permit categorization of occipitalization in three zones, each of which may have a different prognostic implication.

Analysis of anatomic variations in cleft hands.

PURPOSE: This study presents an overview of the clinical and x-ray findings observed in 54 cleft hands of 31 patients. The emphasis was on a detailed analysis of typical malformation-associated x-ray patterns such as aplasia and synostosis, with findings arranged as a function of cleft location and of the Manske and Halikis classification. METHODS: The charts and radiographs of 31 patients with 54 cleft hands were reviewed retrospectively and compared with data reported in the literature. Important clinical and x-ray findings as well as the typical morphology of cleft hands were analyzed. Cleft hands were differentiated according to their location and according to the Manske and Halikis classification. In the different locations the deformities were arranged in teratologic sequences. RESULTS: In cleft hands syndactylies were seen in 30 of the 54 hands, most commonly between the ring and the small finger. In 3 hands polydactylies were noted. Analysis of the x-ray morphology showed 2 typical patterns: aplasia and synostoses. Location-specific teratologic sequences showed that radial cleft hands were bilateral more frequently and were associated significantly more frequently with cleft feet. Aplasias predominated in radial cleft hands, whereas synostoses were more common in central cleft hands. In terms of the Manske and Halikis classification the unilateral cleft hands often corresponded to type I whereas bilateral cleft hands with cleft feet mainly were type IV and type V deformities. Cleft hands with synostoses often were seen in types I to III whereas cleft hands with aplasias were classified most frequently as type V. CONCLUSIONS: Analysis of the patients' clinical data and x-rays showed differences between radial and central cleft hands, as well as between the different Manske and Halikis types.

Surgical management of the hand in Apert syndrome.

In patients with Apert syndrome, the hands demonstrate many disturbances of soft tissue and bony structures. These include a short thumb with radial clinodactyly, complex syndactyly with a bony fusion involving the index, long and ring fingers, symphalangism and simple syndactyly of the fourth web space. The soft tissue anomalies involve the intrinsic muscles, the extrinsic tendon insertions and the neurovascular bundles. We have reviewed 52 patients who underwent surgical reconstruction of their hands. The aim of this study is to propose a better surgical management in the light of recent publications and to improve our understanding of the syndrome, attempting to reduce the number of procedures and to select the best possible procedures for each patient.

[The cleft hand. Proposal of a classification based on 279 cleft hands]. / Die Spalthand. Vorschlag einer Klassifikation auf der Basis von 279 Spalthänden.

PURPOSE: We propose a new classification of cleft hand based on the analysis of our own cases and a literature search. METHODS: We analysed clinical data and radiographs of 31 patients with 54 cleft hands in addition to 225 cleft hands from the literature, and classified them with the help of schematic drawings of radiographs in extension of the proposals of Blauth and Schneider-Sickert. RESULTS: Classification of cleft hand: 1. Median cleft hands without involvement of thumb and small finger. 2. Medioradial cleft hands with thumb affected but with detectable residuals preserved. 3. Radial cleft hands with thumb aplasia. Medioulnar cleft hands with small finger affected but detectable residuals preserved. 5. Ulnar cleft hands with small finger aplasia. Median cleft hands more frequently appear unilateral and are more seldom combined with cleft feet as medioradial and radial cleft hands. In radiographs, aplasia predominate in radial cleft hands while synostoses often appear in median cleft hands. CONCLUSIONS: The new classification enables us to arrange the great diversity of this malformation and takes medioulnar and ulnar cleft hands into account.

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.

Posttraumatic radioulnar synostosis treated with a free vascularized fat transplant and dynamic splint: a report of two cases.

Two cases of posttraumatic radioulnar synostosis are presented. The patients were treated with excision of the cross-union and interposition of a free vascularized fat transplant. A newly devised pronation-supination dynamic splint was employed for 3 months postoperatively in both patients. After a 1-year postoperative follow-up, an increased range of motion was restored in both cases, and there was no evidence of recurrent synostosis formation in subsequent radiographs. We suggest that an interposed vascularized fat graft may be an ideal biologic barrier to fill the space created by cross-union excision.

Phalangeal osteotomy for the treatment of metacarpal synostosis: a case report.

Congenital synostosis between the fourth and fifth metacarpals is a rare congenital anomaly. We present a case of type IIIB metacarpal synostosis according to the Buck Gramcko's classification with the camptodactyly of the little finger. It was treated successfully with the phalangeal angulation osteotomy and soft tissue release procedures.

Missense mutations in the homeodomain of HOXD13 are associated with brachydactyly types D and E.

HOXD13, the most 5' gene of the HOXD cluster, encodes a homeodomain transcription factor with important functions in limb patterning and growth. Heterozygous mutations of human HOXD13, encoding polyalanine expansions or frameshifts, are believed to act by dominant negative or haploinsufficiency mechanisms and are predominantly associated with synpolydactyly phenotypes. Here, we describe two mutations of HOXD13 (923C-->G encoding Ser308Cys and 940A-->C encoding Ile314Leu) that cause missense substitutions within the homeodomain. Both are associated with distinctive limb phenotypes in which brachydactyly of specific metacarpals, metatarsals, and phalangeal bones is the most constant feature, exhibiting overlap with brachydactyly types D and E. We investigated the binding of synthetic mutant proteins to double-stranded DNA targets in vitro. No consistent differences were found for the Ser308Cys mutation compared with the wild type, but the Ile314Leu mutation (which resides at the 47th position of the homeodomain) exhibited increased affinity for a target containing the core recognition sequence 5'-TTAC-3' but decreased affinity for a 5'-TTAT-3' target. Molecular modeling of the Ile314Leu mutation indicates that this mixed gain and loss of affinity may be accounted for by the relative positions of methyl groups in the amino acid side chain and target base.

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.

Metacarpal synostosis: a simple classification and a new treatment technique.

The current classification of metacarpal synostosis is based on the extent of the synostosis. The authors propose a new classification that takes into account the shape of the metacarpal bones, the curvature of the epiphysis, and the discrepancy in length between the two bones. This classification provides better guidelines for the correction of all components of the deformity. The classification is based on the authors' observations of and experience with 36 cases of metacarpal synostosis; 13 of the deformities were surgically corrected. The I-shaped deformity, whether with distinct (type d) or fused (type f) metacarpophalangeal joints, does not require surgical correction. The U-shaped deformity has parallel epiphysis and does not require surgery unless the two metacarpals are asymmetrical in length (type a) or tightly fused (type t); in these cases, simple lengthening or widening of the space with a bone graft is sufficient. Y-shaped synostosis should be separated whether the branches are symmetrical or asymmetrical, the latter having one branch shorter than the other. Because the epiphyses are already divergent, simple separation does not effectively correct Y-shaped synostosis. The authors propose an osteotomy to isolate a trapezoidal segment of bone from the bifurcation. The isolated bone segment is then reversed in the proximal-distal direction to provide a "plateau" upon which the two distal metacarpals can be realigned. Two cases of Ys (symmetrical) synostosis were successfully treated with this technique; one case of Ya (asymmetrical) synostosis also required distraction lengthening of the shorter metacarpal to achieve an excellent result. One of the most difficult types of metacarpal synostosis to treat is k-shaped synostosis, observed only between the fourth and fifth metacarpals; in this type, the head of the short fifth metacarpal abuts the metaphysis of the fourth. Osteotomy and distraction lengthening provide predictable results for correction of this deformity. The authors suggest that k-shaped synostosis might represent a late evolution of untreated Ua synostosis.

[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.

Synostosis of the ring-small finger metacarpal in Apert acrosyndactyly hands: incidence and treatment.

SUMMARY: This study reports on the incidence of a synostosis between the ring-small metacarpal bases in patients with Apert syndrome and describes a technique to resect the synostosis and insert silicone sheets to improve hand function. Records of 9 patients (18 hands) were evaluated. Average age at follow-up was 9.5 years. Three relationships between the ring-small metacarpals were observed. Type I hands (22%) had no abnormal interconnections; type II hands (33%) possessed a synostosis since birth; type III hands (44%) did not possess a synostosis at birth, but one later developed at average age of 79 months. Total incidence of synostosis was 77%. Five patients underwent excision of the synostosis. At an average of 16 postoperative months, 4 of these patients experienced improvement in hand prehension. In patients with Apert syndrome, resection of abnormal interconnections between the ring-small metacarpals is highly recommended to improve hand prehension based on the high incidence of a synostosis in this region.

Congenital radioulnar synostosis in an active duty soldier: case report and literature review.

Congenital radioulnar synostosis (CRS) is a developmental deformity that interferes with pronation and supination of the upper extremity. CRS often results in functional, cosmetic, and cultural limitations. The purpose of this paper is to present a patient with CRS who is currently functioning as a generator mechanic in the U.S. Army and to review the literature for current treatment options. We know of no other individual in the U.S. Army who is on active duty status with this deformity.