The latissimus dorsi creates a dynamic track for the inferior angle of the scapula during arm abduction in humans.

BACKGROUND: The importance of several scapulothoracic muscles, including trapezius and serratus anterior, in maintaining physiological scapula kinematics has been highlighted in the past. However, the relationship between the scapula and the latissimus dorsi muscle remains unclear. Our clinical surgical observation is that the latissimus dorsi does not directly attach but rather runs superficial to the inferior angle of the scapula. Based on this observation, we hypothesise that the latissimus dorsi creates a dynamic track on which the scapula glides under the muscle belly during elevation of the arm, creating the latissimus-scapula overlap (LSO). METHODS: All consecutive patients who had a whole-body computed tomography scan (CT) in case of polytrauma evaluation between 2018 and 2021, with complete depiction of the scapula and latissimus dorsi muscle, were analysed. 150 shoulders in 90 patients with arms up were matched according to their age (within five years), gender, and affected side with 150 shoulders in 88 patients with arms down. Patients with pathologies of the upper extremities or thorax that potentially could alter LSO measurements were excluded. LSO was calculated as a ratio of the measured area of the latissimus dorsi projection on the scapula and the total scapula area. RESULTS: The mean age of the 178 patients (48 females; 13 males) was 60 years. The arms-up group showed a significantly higher LSO than the arms-down group (19.9 ± 6.3% vs. 2.7 ± 2.2%; p < 0.0001). In the arms-up group, approximately one fifth of the scapula was overlapped inferiorly by the muscle belly of the latissimus dorsi, contrary to the almost non-existing LSO in the arms-down group. CONCLUSION: With arms up, humans show a significantly higher LSO in comparison to arms down indicating that the latissimus dorsi indeed creates a dynamic track on which the scapula is forced to travel during abduction of the arm. This finding of increased LSO during the elevation of the arm warrants further consideration of the role of the latissimus dorsi in scapula kinematics and potentially scapular dyskinesis. LEVEL OF EVIDENCE: Level two diagnostic study.

Prenatal and Neonatal Ultrasound and Magnetic Resonance Imaging Diagnosis of Sprengel's Deformity with Unusual Associations.

INTRODUCTION: Sprengel's deformity is a rare congenital anomaly of the shoulder rim. It is the most common congenital anomaly of the shoulder, associated with cosmetic deformity and abnormal shoulder function. Nonsurgical management can be considered for mild cases. Surgical intervention is indicated in moderate to severe cases with the goal of improving cosmetic appearance and function. The best surgical results are obtained in children aged 3-8 years. Correct diagnosis is very important because Sprengel's deformity can be accompanied by additional abnormalities, even in mild cases, and lack of a diagnosis delays proper treatment of the child. The severity of the defect may progress, so it is important to correctly identify children with Sprengel's deformity, even those with a mild form of the defect. CASE PRESENTATION: We report a case of prenatal sonographic diagnosis of Sprengel's deformity with additional features, as yet undescribed and missed - although visible - on prenatal magnetic resonance imaging (MRI). Cesarean delivery was performed due to preterm rupture of membranes, and a postnatal MRI confirmed the unusual constellation of Sprengel's anomaly with lateral meningocele, vestigial posterior meningocele, and lipoma tethering of the cord to the dural sac at the cervical-thoracic junction. CONCLUSION: Diagnosis of Sprengel's deformity is possible with prenatal ultrasound. Asymmetry of the cervical spine, discontinuity of the vertebral arch and abnormal vertebral bodies, as well as the asymmetric position of the shoulder blades with the presence of an omovertebral bone are signs that can help diagnose the defect.

Unilateral accessory scapular ossicle with sprengel deformity in a trauma patient: A case report.

Herein we present a rare anatomic variation of unilateral accessory scapular ossicle in a trauma patient and its rare association with a common scapular anomaly, Sprengel deformity. Foci that appear near the inferior angle of the scapula due to failure of bony fusion during bone maturation are called accessory scapular ossicles. Sprengel deformity is defined as the congenitally high position of the scapula. The recognition of the normal variants of scapula is important, since they could be confused with other pathologies, such as fracture and pulmonary nodule in a trauma patient. Therefore, radiologists should be familiar to these entities even rarely seen.

Utilization of intraoperative neuromonitoring during the Woodward procedure for treatment of Sprengel deformity.

BACKGROUND: Brachial plexus injury (BPI) leading to palsy of the upper extremities is the most serious complication of the Woodward procedure for treatment of Sprengel deformity. Intraoperative neuromonitoring (IONM) is widely used for detecting emerging spinal cord or peripheral nerve injury during spinal and shoulder surgery. However, to date, its utilization in pediatric patients with Sprengel deformity is limited. Furthermore, it remains unclear whether IONM can help prevent BPI during surgery. The purpose of the current study was to assess the feasibility and effectiveness of IONM for early identification and prevention of nerve injury during the Woodward procedure. METHODS: We retrospectively reviewed the records of patients who underwent the Woodward procedure for Sprengel deformity at our institution between January 2017 and January 2020. IONM, including somatosensory evoked potentials (SEP) and motor evoked potentials (MEPs), was performed in all patients. Detailed IONM data were collected and analyzed. Preoperative and postoperative cosmetic appearance (according to the Cavendish classification), shoulder joint abduction function, and radiologic evaluation of the scapula were reviewed. Surgical complications were recorded. RESULTS: Forty-six patients (19 girls, 27 boys) were included (mean age, 5.1 ± 2.1 years). Both SEP and MEP (amplitude of the abductor pollicis) were successfully performed (100%). MEP alerts occurred in 3 patients (6.5%). After scapula position adjustment, signals recovered in 2 patients and remained unchanged in 1 patient-this patient exhibited postoperative motor deficits that resolved completely by 4 months recovery. The SEP amplitudes decreased in all 3 patients but did not reach the warning criteria. Forty patients were classified as grade III and 6 as grade IV in the Cavendish classification, whereas 35 patients were classified as grade II and 11 as grade III in the Rigault scale. The preoperative Cavendish grade was III (III, IV) and the postoperative Cavendish grade was I (I, II) (χ2 = 88.098, P < .001). The preoperative Rigault grade was II (II, III) and the postoperative Rigault grade was I (I, II) (χ2 = 62.133, P < .001). The mean arc of shoulder joint abduction improved from 99° ± 8° to 167° ± 7° (t = -45.871, P < .001) after surgery. Except for temporary motor deficits detected in 1 patient, no other postoperative complications were observed through the time of final follow-up. CONCLUSION: IONM during the Woodward procedure for Sprengel deformity is feasible and effective in detecting intraoperative neurologic changes and may be effective in preventing BPI associated with surgery.

Is it the coracobrachialis superior muscle, or is it an unidentified rare variant of coracobrachialis muscle?

The coracobrachialis muscle (CBM) originates from the apex of the coracoid process, in common with the short head of the biceps brachii muscle, and from the intermuscular septum. The CBM demonstrates variability in both the proximal and distal attachment, with some extremely rare varieties, such as the coracobrachialis superior, coracobrachialis longus and coracocapsularis muscle. This case report describes an extremely rare variant of the coracobrachialis superior muscle, or a very rare variant of the CBM. Our findings highlight the importance of muscle variants in the shoulder region, especially the coracoid region, and are significant for radiologists, anatomists, physiotherapists and surgeons specializing in the shoulder joint.

Cervical myelopathy secondary to omovertebral bone in the pediatric patient with Sprengel deformity.

Sprengel deformity is a congenital anomaly arising mainly in the shoulder girdle, associated with elevation of dysplastic scapula. skeletal anomalies, mainly Klippel-Feil syndrome, hemivertebrae, and omovertebral bone may be present along Sprengel anomaly. The omovertebral bone is an abnormal bone that originates from the superomedial edge of the scapula with different insertion points along the posterior cervical spine, seen in about third of the patients with Sprengel anomaly. While cosmetic to functional impairment is a common presentation to the omovertebral bone, cervical myelopathy is a rare presentation. Here, we described our experience, management and follow up of 13-year-old boy presented with cervical myelopathy secondary to the omovertebral bone.

Reinforcing the vascular disruption theory of the genesis of Poland's syndrome: a rare association of diaphragmatic eventration in a preterm infant with severe musculoskeletal defects.

A preterm female infant was admitted at birth with respiratory distress. On examination, she had an asymmetric right chest wall and ipsilateral small hand. Air entry was reduced over the right chest. A clinical diagnosis of Poland's syndrome was made based on the hypoplasia of the right pectoral muscles, absent nipple, deformed ribs and symbrachydactyly of the ipsilateral hand. Chest X-ray suggested and ultrasound confirmed eventration of the right hemidiaphragm. 'Subclavian artery supply disruption sequence' (SASDS) theory by Bavnick and Weaver remains the most accepted pathogenic mechanism in Poland's syndrome. This case reinforces SASDS theory associated with the genesis of Poland's syndrome that relates to the pathogenicity of vascular disruption of subclavian artery, characteristics of which are unilateral pectoral defects, symbrachydactyly and eventration of the diaphragm. At 2 months, she underwent diaphragm plication. She is under review by our multidisciplinary surgical team for reconstruction of the chest deformity.

Sprengel Deformity with Omovertebral Bone Encroaching the Spinal Canal Causing Progressive Cervical Myelopathy: A Technical Case Report.

An adult with Sprengel deformity and Klippel-Feil syndrome associated with an omovertebral bone has rarely been reported in literature. The omovertebral bone is an abnormal cartilaginous connection between the scapula and the cervical spine. Limited cases have previously been reported in the literature describing surgical intervention when neurologic deficits such as cervical myelopathy or radiculopathy are present. In the present case, an omovertebral bone extended into the cervical lamina resulting in cervical myeloradiculopathy requiring resection of the bony anomaly and cervicothoracic fusion. The omovertebral bone as an etiology for radiculopathy or myelopathy is rarely seen in an adult population, and surgical decompression and fusion should be considered with this constellation of anomalies.

Functional improvement in patients with Sprengel's deformity following Modified Green's procedure and simplified clavicle osteotomy-a study of forty cases.

PURPOSE: Sprengel's deformity though rare is the most common congenital anomaly of the shoulder region. The aim of the study was to check the effectiveness of the modified Green's procedure with simplified clavicle osteotomy in providing significant functional and radiological improvement without neurovascular complications. METHODS: Sixty-eight children of Sprengel deformity were operated by the modified Green's release. Of which forty patients (15 male, 25 female) with more than two years follow-up were evaluated retrospectively. Simplified clavicle osteotomy was done in 34 patients. Severity was graded clinically by Cavendish grading (Grade II-6, III-34) and radiologically by Rigault grading (Grade II - 35, III - 5). The average age at surgery was 5.05 years with a mean follow-up of 62 (24-145) months. RESULTS: The average improvement in Cavendish grade was 2.6 grades, and Rigault grading was 1.07 grades. Shoulder abduction improved by a mean of 360. There was no neurovascular complication in children who had simplified clavicle osteotomy, and one patient without clavicle osteotomy developed transient brachial plexus palsy. Other complications were wound gape, pleural tear and scapular winging. CONCLUSION: The modified Green's procedure with simplified clavicle osteotomy allows for excellent correction of function and cosmesis. We describe a simpler technique of clavicle osteotomy that is quick, safe and equally effective in preventing neurovascular complications. We also describe a surrogate clinical test to avoid potential neurological injury in the absence of neuromonitoring. To the best of our knowledge, this is the second-largest operative series of Sprengel's deformity.

Sprengel Deformity in Biological Sisters.

Sprengel deformity is the main congenital shoulder deformity seen in children. First described by Otto Sprengel in 1891, it consists of a high-riding, dysplastic, and malrotated scapula leading to functional and cosmetic impairments. It has never been reported in siblings. Case Presentation: Two sisters, 8 and 9 years old, presented for an evaluation of atraumatic limitation in the shoulder range of motion and neck webbing with an unknown family history. Physical examination revealed a small high-riding scapula, webbed neck, and painless limitation in shoulder abduction (<70°) and flexion (<80°). The 9-year-old sibling had a bilateral shoulder involvement, and the younger had unilateral. Imaging revealed bony and fibrous omovertebral connections between the dysplastic scapulas and cervical spine along with Klippel-Feil deformities. Both sisters underwent scapula repositioning via a modified Woodward procedure. The omovertebral connection was resected followed by scapula derotation and inferior migration. Both had a dramatic improvement in cosmesis and near-complete restoration of shoulder function at follow-up. Conclusions: Although uncommon, Sprengel deformity results in notable derangement of shoulder function. If untreated, children experience difficulty with most overhead activities and often have cosmetic reports. Although no previous genetic link has been identified, its presence in biological sisters suggests that more research is needed.

Modified Woodward Technique for Sprengel Deformity and a Modification of the Cavendish Classification.

INTRODUCTION: Sprengel deformity (SD) is the most common congenital anomaly of the shoulder. Surgery is required for moderate and severe forms. The modified Woodward procedure is the most widely used procedure for the surgical treatment. METHODS: SD patients who applied to our institution between 2005 and 2018 were retrospectively reviewed. A modification of the Cavendish classification was used for preoperative and postoperative evaluations. RESULTS: Eighteen shoulders of 17 (mean age: 8.5 y, range: 2 to 18 y, 5 males and 12 females) patients were included. The right, left, and bilateral shoulders were affected in 9, 7, and 1 cases, respectively. The mean follow-up time was 62.9 months (12 to 161 mo). Preoperatively, 3 shoulders were type 2, 12 shoulders were type 3, and 3 shoulders were type 4 according to the Cavendish classification and 2 shoulders were type 0, 6 shoulders were type 1, and 10 shoulders were type 2 postoperatively. CONCLUSIONS: Periscapular congenital malformations play a significant role in range of motion limitation. The modified Woodward procedure is a viable alternative in the surgical treatment of SD and the proposed modification of Cavendish classification (grade 0) is functional.

Sprengel Deformity: Comprehensive Evaluation of Concomitant Spinal and Extraspinal Anomalies in 90 Patients.

STUDY DESIGN: A retrospective, case series. OBJECTIVE: The aim of this study is to evaluate the concomitant anomalies in patients with Sprengel deformity (SD). SUMMARY OF BACKGROUND DATA: SD is the most common congenital anomaly of the shoulder. One or more associated anomalies may coexist in SD patients, similar to congenital scoliosis (CS); however, these anomalies and their relationship have not been studied in detail previously. METHODS: SD patients who have applied to our institution between 2005 and 2019 were retrospectively reviewed. The patients were evaluated clinically and radiologically. The patients were divided in two groups as SD patients with CS and without CS, to analyze if these anomalies are present due to CS or SD. Physical examination findings, MRI, CT, and USG reports were analysed for accompanying pathologies. Patients with missing data were excluded. Student-t and Fisher's exact tests were used to compare the groups. Significance value was set as p = 0.05. RESULTS: Ninety patients met inclusion criteria. The most common spinal anomaly was omovertebra, followed by spina bifida and Klippel-Feil. Tethered cord and diastematomiyelia were associated with CS (P = 0.0026 and P = 0.0057, respectively). The most common extra-skeletal anomaly was rib anomalies, followed by urinary and cardiac system anomalies. Rib anomalies were associated with CS (P = 0.00001). CONCLUSION: Concomitant anomalies may accompany SD. The prognosis of SD may be affected by these anomalies. Therefore, patients should be evaluated for possible coexistent congenital anomalies. LEVEL OF EVIDENCE: 4.

Surgical correction of Sprengel's deformity by modified Woodward procedure: outcomes in twenty eight patients.

PURPOSE: Sprengel's deformity is a congenital failure of the descent of the scapula with main concerns regarding cosmetic deformity and restricted function of the affected shoulder. The purpose of this study was to present the results of Modified Woodward procedure in 28 patients with Sprengel's shoulder. METHODS: Twenty-eight patients (9 males, 19 females) of Sprengel's shoulder treated with modified Woodward procedure from 2010 to 2016 were retrospectively studied. Clinical assessment was done using Cavendish grading and Rigault's classification for radiographic evaluation. RESULTS: Mean age at the time of surgery was six years and three months. Mean follow-up was 20 months. We found a mean increase in abduction of 34.2° (15-50) and flexion of 20.6° (10-25), mean scapular lowering of 2.3 cm (0.5-4.5 cm), and a Cavendish grade I in 18 (65%), grade II in 10 (35%). Thus, we had 65% satisfactory results in terms of cosmesis and 85% with the function of the affected limb. Rigault grading improved by one grade in 16 children and by two grades in 12 children. Subjectively, all parents were satisfied with the cosmetic appearance of the achieved scapular lowering. We had one case of delayed superficial wound healing which resolved spontaneously. CONCLUSION: Modified Woodward procedure for Sprengel's shoulder deformity gives good cosmetic correction and improvement in shoulder function with minimal complications. This procedure is good for selective cases of Cavendish grade II with cosmetic concerns and all cases of Cavendish grade III and IV.

Bilateral Multilevel Cervical Rib and Bilateral Omovertebra in Klippel-Feil Syndrome.

BACKGROUND: Klippel-Feil syndrome was first described in 1912; a short neck, low posterior hairline, and decreased cervical joint range of motion are the classical triad of this disease. In this syndrome, which is rarely observed, the characteristics that have been reported include the following: scoliosis; Sprengel deformity; cervical rib; ear, nose, oral, and laryngeal abnormalities; structural abnormalities of the urinary system; and congenital heart diseases. However, bilateral omovertebra and bilateral multilevel cervical ribs have not been reported. CASE DESCRIPTION: We aimed to present this rare syndrome via radiologic findings from cases with bilateral multilevel cervical rib and bilateral omovertebra. CONCLUSIONS: Cases of Klippel-Feil syndrome may be accompanied by multiple abnormalities. We want to highlight the need for detailed examination of patients and lifestyle modification at an early age, before symptom appearance, as well as adaptation to habitual exercise.

Surgical management of Sprengel's deformity by a modification of Green's procedure : A single center experience.

BACKGROUND: Sprengel's deformity is a rare congenital anomaly that can present in children, leading to cosmetic and functional impairment. This study investigated clinical results of this deformity among cases managed using a modified Green procedure. METHODS: From February 2008 to September 2015 a total of 34 patients with Sprengel's deformity were treated with a modified Green procedure. The mean age of patients at the time of surgery was 4.8 years (range 2-12 years) and the average follow-up time was 6.1 years (range 4-10 years). The shoulder abduction, Cavendish classification, Rigault classification, scapular elevation, and postoperative complications were evaluated. RESULTS: The mean shoulder abduction was 102.3° (range 70-140°) preoperatively and 142.6° (range 120-170°) postoperatively. The scapular elevation was 4.2 cm (range 2-6.5 cm) preoperatively and 1.35 cm (range 0-2 cm) postoperatively. Improvement by at least one Cavendish and/or Rigault grade was attained in all cases postoperatively. The differences in preoperative and postoperative shoulder abduction, scapular elevation, Cavendish grade and Rigault grade were statistically significant (p < 0.001). CONCLUSION: The modified Green procedure is a relatively safe and reliable method in the treatment of severe Sprengel's deformity, which ensures successful shoulder function as well as a good cosmetic appearance.

Klippel-Feil Syndrome with Sprengel Deformity.

Coexistence of Klippel-Feil syndrome with Sprengel deformity and omovertebral bone is a rare complex bone abnormality with unknown incidence and etiology. Herein, we report a case of a 6-year-old girl with coexistence of these congenital abnormalities evaluated by three-dimensional computed tomography. We also make a brief review and discuss in details the role of this imaging modality in the evaluation of such complex cases.

Demographics, presentation and symptoms of patients with Klippel-Feil syndrome: analysis of a global patient-reported registry.

INTRODUCTION: Klippel-Feil syndrome (KFS) occurs due to failure of vertebral segmentation during development. Minimal research has been done to understand the prevalence of associated symptoms. Here, we report one of the largest collections of KFS patient data. METHODS: Data were obtained from the CoRDS registry. Participants with cervical fusions were categorized into Type I, II, or III based on the Samartzis criteria. Symptoms and comorbidities were assessed against type and location of fusion. RESULTS: Seventy-five patients (60F/14M/1 unknown) were identified and classified as: Type I, n = 21(28%); Type II, n = 15(20%); Type III, n = 39(52%). Cervical fusion by level were: OC-C1, n = 17(22.7%), C1-C2, n = 24(32%); C2-C3, n = 42(56%); C3-C4, n = 30(40%); C4-C5, n = 42(56%); C5-C6, n = 32(42.7%); C6-C7, n = 25(33.3%); C7-T1, n = 13(17.3%). 94.6% of patients reported current symptoms and the average age when symptoms began and worsened were 17.5 (± 13.4) and 27.6 (± 15.3), respectively. Patients reported to have a high number of comorbidities including spinal, neurological and others, a high frequency of general symptoms (e.g., fatigue, dizziness) and chronic symptoms (limited range of neck motion [LROM], neck/spine muscles soreness). Sprengel deformity was reported in 26.7%. Most patients reported having received medication and invasive/non-invasive procedures. Multilevel fusions (Samartzis II/III) were significantly associated with dizziness (p = 0.040), the presence of LROM (p = 0.022), and Sprengel deformity (p = 0.036). CONCLUSION: KFS is associated with a number of musculoskeletal and neurological symptoms. Fusions are more prevalent toward the center of the cervical region, and less common at the occipital/thoracic junction. Associated comorbidities including Sprengel deformity may be more common in KFS patients with multilevel cervical fusions. These slides can be retrieved under Electronic Supplementary Material.

Effect of antioxidants on BPA-induced stress on sperm function in a mouse model.

In the past few years, bisphenol A, (BPA) an endocrine-disrupting chemical, has received increasing attention because of its detrimental health effects. There is ample evidence to support that BPA interferes with the reproductive health of humans and animals. In spermatozoa, BPA-induced adverse effects are mostly caused by increased oxidative stress. Using an in vitro experimental model, we examined whether antioxidants (glutathione, vitamin C, and vitamin E) have defensive effects against BPA-induced stress in spermatozoa. The results showed that antioxidants inhibit the overproduction of reactive oxygen species (basically cellular peroxides) and increase intracellular ATP levels, thereby preventing motility loss and abnormal acrosome reaction in BPA-exposed spermatozoa. In particular, glutathione and vitamin E reduced the protein kinase A-dependent tyrosine phosphorylation in spermatozoa and, thus, prevented the precocious acrosome reaction from occurring. Furthermore, we found that the compromised fertilisation and early embryo development mediated by BPA-exposed spermatozoa can be improved following their supplementation with glutathione and vitamin E. Based on these findings, we suggest that antioxidants reduce oxidative stress in BPA-exposed spermatozoa, thus preventing detrimental effects on their function and fertility.