Sprengel's deformity: an analysis of surgically and nonsurgically treated patients.

BACKGROUND: Sprengel's deformity is a congenital anomaly of the scapula and can be associated with reduced shoulder mobility and esthetic concerns. Controversies remain regarding its natural history, radiographic findings, and optimal treatment strategy. METHODS: A total of 74 Sprengel's shoulders in 71 patients presenting to a pediatric referral hospital were retrospectively reviewed including 24 surgically treated shoulders. Median age at initial presentation was 3.8 years. Median age at the final follow-up was 9.6 years. RESULTS: Twenty shoulders were analyzed for range of motion across time. The median composite abduction range was initially 105° and at the last time-point 98°, with a median loss of 15° over 5 years (P = .007). A total of 26% of the nonsurgical shoulders reported pain at the latest follow-up, compared with 1 surgical patient (4%) (P = .028). Older age was independently associated with pain (P < .001); this effect was only seen in nonsurgical patients. Radiographically, the glenoid inclination angle (GIA) independently correlated with the abduction range (P = .001). Surgically treated shoulders underwent a modified Woodward procedure at a median 4.8 years of age. At a median follow-up of 41 months, the abduction range improved in the surgical group from 90° preoperatively to 110° postoperatively, with a median gain of 40° (P < .001). The median GIA changed from -2° to 4° with a median gain of 9° (P = .004). CONCLUSIONS: The natural history of untreated Sprengel's deformity may be one of gradual decline in the abduction range, with a proportion of patients developing pain in adolescence. The GIA correlates with the shoulder abduction range. Surgery with a modified Woodward procedure may alter the natural history of the condition by improving motion and reducing risk of pain later in life.

Sprengel's deformity of the shoulder joint treated by Woodward operation.

BACKGROUND: Sprengel's shoulder deformity is a rare condition that happens because of the abnormal termination of the caudal migration of the scapula during the embryonic period. METHODS: This retrospective clinical study included 10 consecutive children who underwent a Woodward operation to correct the Sprengel's shoulder deformity. RESULTS: The average preoperative Cavendish grade for cosmetic evaluation was 3.5 (range 3-4), which decreased to 1.4 (range 1-2) at the final review (statistically significant, p > 0.002, Wilcoxon signed-rank test). Cavendish grade improvement and increase in abduction had a strong positive association (r = 0.681, Spearman correlation coefficient. CONCLUSION: The results showed that both functional and cosmetic outcome had a positive correlation with Woodward procedure. More cosmetic and functional improvement was seen at the last follow-up visit especially in cases where the operation was done in the younger years. LEVEL OF EVIDENCE: IV.

Contributions of muscle imbalance and impaired growth to postural and osseous shoulder deformity following brachial plexus birth palsy: a computational simulation analysis.

PURPOSE: Two potential mechanisms leading to postural and osseous shoulder deformity after brachial plexus birth palsy are muscle imbalance between functioning internal rotators and paralyzed external rotators and impaired longitudinal growth of paralyzed muscles. Our goal was to evaluate the combined and isolated effects of these 2 mechanisms on transverse plane shoulder forces using a computational model of C5-6 brachial plexus injury. METHODS: We modeled a C5-6 injury using a computational musculoskeletal upper limb model. Muscles expected to be denervated by C5-6 injury were classified as affected, with the remaining shoulder muscles classified as unaffected. To model muscle imbalance, affected muscles were given no resting tone whereas unaffected muscles were given resting tone at 30% of maximal activation. To model impaired growth, affected muscles were reduced in length by 30% compared with normal whereas unaffected muscles remained normal in length. Four scenarios were simulated: normal, muscle imbalance only, impaired growth only, and both muscle imbalance and impaired growth. Passive shoulder rotation range of motion and glenohumeral joint reaction forces were evaluated to assess postural and osseous deformity. RESULTS: All impaired scenarios exhibited restricted range of motion and increased and posteriorly directed compressive glenohumeral joint forces. Individually, impaired muscle growth caused worse restriction in range of motion and higher and more posteriorly directed glenohumeral forces than did muscle imbalance. Combined muscle imbalance and impaired growth caused the most restricted joint range of motion and the highest joint reaction force of all scenarios. CONCLUSIONS: Both muscle imbalance and impaired longitudinal growth contributed to range of motion and force changes consistent with clinically observed deformity, although the most substantial effects resulted from impaired muscle growth. CLINICAL RELEVANCE: Simulations suggest that treatment strategies emphasizing treatment of impaired longitudinal growth are warranted for reducing deformity after brachial plexus birth palsy.

Correlation between clinical findings and CT scan parameters for shoulder deformities in birth brachial plexus palsy.

PURPOSE: The shoulder is the most common site of secondary deformities after birth brachial plexus palsy. The severity and the pattern of deformity vary in patients and have implications for clinical decision making. This study aimed to find the correlation between clinical findings and computed tomography (CT) scan parameters for these deformities. METHODS: This prospective study included 75 patients aged 3 to 23 years. The clinical parameters included age, extent of involvement (nerve roots affected), degree of shoulder abduction, active and passive external rotation, and Mallet score. These were correlated with 3 CT scan parameters: elevation of the scapula above the clavicle, relative glenoid version, and percentage of the humeral head anterior to the scapular line. RESULTS: There was a significant correlation between lack of active and passive external rotation and relative glenoid version and humeral head subluxation. There was a significant correlation between active abduction and elevation of the scapula above the clavicle. There was no significant correlation between age or Mallet score with any of the CT scan parameters. CONCLUSIONS: These results suggest that presence of active and passive external rotation beyond 10° is associated with significantly lesser shoulder deformity irrespective of the degree of shoulder abduction. Hence, a patient with more than 10° external rotation does not need a screening CT scan evaluation regardless of the degree of shoulder abduction present. Conversely, a lack of external rotation beyond 10° strongly suggests relative glenoid retroversion and posterior subluxation of the humeral head and should be considered a clinical indicator of shoulder deformation. TYPE STUDY/LEVEL OF EVIDENCE: Diagnostic II.

Geometry of the proximal humeral articular surface in young children: a study to define normal and analyze the dysplasia due to brachial plexus birth palsy.

BACKGROUND: Little is known regarding the morphology of the proximal humerus in growing children. This study reports bilateral magnetic resonance imaging measurements in children with internal rotation contractures from birth palsy, hypothesizing that dysplasia alters normal humeral sphericity and symmetry. METHODS: We studied 25 children with unilateral internal rotation contractures (mean age, 3.7 years) for humeral shape by bilateral magnetic resonance imaging studies at the mid-glenoid level. Local radii of curvature were compared for symmetry and orientation. RESULTS: Neither side showed uniform radii (sphericity), but normal humeri showed symmetry lost in dysplasia. Internal rotation contractures were correlated with flattening of the anterior humeral head (P = .0002). All heads were flatter in the region of articular contact. The skew axis (the largest cross-sectional diameter of the proximal humerus) was collinear with the articular surface centerline in normal humeri, an alignment often lost with dysplasia, resulting in a skew axis angle. The severity of glenoid deformity correlated with progressive posterior displacement of the humeral head center (P < .0003). CONCLUSION: The normal humeral articular surface in the young child is not spherical and is flatter in the middle than at the periphery but is symmetric about its central axis. Internal rotation contractures result in loss of this symmetry with characteristic flattening of the anterior humeral head and development of a skew axis angle. CLINICAL RELEVANCE: Posterior displacement of the humeral head center of rotation beyond 50% of the calculated head radius warrants vigilance and possibly surgical intervention because there is a high likelihood for development of a pseudoglenoid.

Radiographic correlation of clinical shoulder deformity and patient perception following scapula fracture.

BACKGROUND: Interest in operative management of scapular fractures is increasing based upon defined radiographic displacement criteria and growing awareness that certain extra-articular fractures will not do well and result in dysfunction and deformity (slumped shoulder). We intend to quantify clinical deformity, analyze correlations of these novel measures with defined radiographic measures of fracture displacement and with the patients' reported perception of their deformity. METHODS: Prospectively enrolled patients underwent standardized questioning regarding their perception of the deformity. Radiographs were utilized to measure glenopolar angle medial/lateral displacement, and angulation of the displaced scapula fracture. Novel measurements of clinical deformity (shoulder area, shoulder angle and shoulder height difference) were calculated. All measurements were repeated post-operatively for patients undergoing operative treatment. RESULTS: Fifty-one patients (39 operative) were examined within 30 days of injury. Follow-up (≥2 months post-op) was obtained for 31/39 (79%). Medial-lateral displacement significantly correlated with all measures of clinical deformity and with patient reported shoulder appearance bothersome score. Angulation significantly correlated with patient perception and two clinical measures (shoulder area and shoulder angle difference). All post-operative radiographic measures, clinical measures of deformity, and patient reported scores statistically improved from baseline measures. DISCUSSION: Patients with scapula fracture do perceive deformity, and there is a significant correlation between the patients' perception, radiographic and clinical measurements of deformity after scapula fracture. All measures statistically improved in patients with operative treatment compared to baseline measurements. This study reinforces the importance of the clinicians' clinical examination and observation of shoulder deformity in the scapula fracture patient. LEVEL OF EVIDENCE: IV.

Sprengel's Deformity.

Various shoulder bone deformities have been identified in the pediatric age group, with the most common being undescended scapula. Sprengel's deformity is dysplasia and malposition of the scapula, especially in the supraspinatus portion, mainly due to abnormal descent in the embryonic period. The clavicle is shorter and has a different contour. In some patients cervical spine deformities are also noted. This is a case of 3 years old girl presented to the clinic with difficulty in abducting right shoulder and sleeping with an internal rotation of the right arm. With physical examinations and radiological investigations sprengel's deformity was diagnosed. Sprengel's deformity is a male predominant disease which almost never occurs in isolation and is usually associated with deformities in the thoracic rib cage and the cervical and thoracic vertebrae. Our patient also had hyperpigmentation along the lines of Blaschko, which has never been previously reported with Sprengel's deformity. Sprengel's may have an association with cutaneous mosacism syndrome.

Congenital anterior shoulder dislocation in a newborn treated with closed reduction.

Case: This rare case presents an isolated congenital shoulder dislocation in a twin delivery, without traumatic delivery. Delivered by emergent cesarean section at 33 weeks gestation, the infant presented with a lateral shoulder crease with x-rays showing anterior and inferior dislocation. Treatment included prompt reduction and stabilization, with follow-up ultrasound demonstrating a physeal injury. Conclusions: This case report presents the only published congenital shoulder dislocation in an infant after an atraumatic twin cesarean delivery. Prompt reduction, stabilization, and ultrasound imaging to assess for physeal injury is our recommended management for this scenario.

Value of Soft Tissue Release Procedure around the Shoulder to Improve Shoulder Abduction in Birth Brachial Plexus Palsy and Analysis of the Factors Affecting Outcome.

BACKGROUND: A lack of shoulder abduction in spontaneously recovered birth brachial plexus palsy (BBPP) is a common presentation. We have performed a soft tissue release operation in these patients to remove the tethering effect of the tight and cocontracting shoulder adductors. This study was undertaken in order to assess the outcome of this surgical procedure. METHODS: We performed a retrospective analysis of 120 patients who displayed spontaneous recovery from BBPP and subsequently underwent soft tissue release procedure to improve shoulder abduction. The operation involved release of the pectoralis major, latissimus dorsi (LD) and teres major (TM) with axillary nerve neurolysis and transfer of LD and TM to teres minor at a lower position. Outcomes were assessed at a minimum follow up of two years. The primary outcome measures were range of shoulder abduction and Mallet score. Parents were interviewed and their satisfaction was graded on a Likert scale. We also explored prognostic factors responsible for better outcomes namely, age at operation, extent of involvement; preoperative shoulder abduction range, internal rotation deformity, triceps power and Mallet score. RESULTS: The average patient age was 5.8 years(range 1-17). Follow up ranged from 2-6 years. Average preoperative shoulder abduction was 85° (range 30°-140°). Postoperatively the average shoulder abduction was 161° (range 80°-180°). The mean improvement in abduction was 76° (range 20°-110°) [p < 0.001]. Mallet score improved from 16.4 to 19.5 (p < 0.001). High parental satisfaction was recorded by Likert scale assessment. Regression analysis indicated a favourable outcome in patients who are younger, have a better preoperative abduction range and a preoperative triceps power > grade 3. CONCLUSIONS: Soft tissue release procedure employed in this series is effective in improving shoulder abduction. Patients who are of younger age, have better preoperative abduction and triceps power of > grade 3 are expected to achieve the best outcome.