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Background Children with arthrogryposis have impaired motion and strength; however, they develop compensatory strategies to achieve better function than may be anticipated. Impairment measures, such as range of motion and strength, describe physical function, but do not characterize a person’s psychosocial function, or their ability to perform activities and participate in life roles. The Patient Reported Outcome Measurement Information System (PROMIS®) measures domains of function from the perspective of people with a chronic disease, compared to a reference population. We hypothesized that PROMIS® scores would discriminate between children with arthrogryposis and a pediatric reference population, and that children with arthrogryposis would report impaired upper extremity function and mobility, but normal pain interference with activities and normal peer relationships. Methods This is a retrospective cohort study of children with arthrogryposis aged 5-17 years who responded to four pediatric PROMIS® physical and psychosocial domain questionnaires (Mobility, Upper Extremity (UE) Function, Pain Interference, and Peer Relationships) during clinic visits at Shriners Hospital for Children, Northern California from April 2017-May 2019. Responses were converted to a T-score for comparison to the reference population (mean score = 50, standard deviation = 10). Results PROMIS® questionnaires were administered to 65 children with arthrogryposis with a mean age of 9.5 years. Participants reported moderately impaired Mobility (38±9) and UE Function (32±12), both significantly lower than the reference population; 63% reported moderate or severe mobility impairment, and 77% reported moderate or severe impairment with UE Function. Regarding psychosocial function, participants reported excellent Peer Relationships (average 54±8), with 98% reporting excellent or good Peer Relationships. They also reported normal Pain Interference (average 49±10), with 82% reporting normal or mild Pain Interference. Conclusions PROMIS® effectively discriminates between children with arthrogryposis and the reference population. Children with arthrogryposis report moderately impaired physical function (Mobility and UE Function) but normal psychosocial function (Peer Relationships and Pain Interference.) PROMIS® is a useful tool to evaluate and understand the challenges that children with arthrogryposis face.

To determine the prevalence of perinatal factors associated with brachial plexus birth injury (BPBI) in affected infants and their relationship with BPBI severity.Retrospective study of BPBI infants prospectively enrolled in a multicenter registry. The prevalence of perinatal factors was calculated. Infants were stratified by injury severity and groups were compared to determine the association of severity and perinatal factors.Seven-hundred-ninety-six BPBI infants had a mean 4.2 ± 1.6 perinatal factors. Nearly all (795/796) reported at least one factor, including shoulder dystocia(96%), no clavicle fracture (91%), difficult delivery(84%), parity1(61%) and birthweight4000 g(55%). Ten-percent (74/778) had Horner's syndrome and 28%(222/796) underwent nerve surgery. Birth asphyxia and NICU admission were significantly associated with injury severity.NICU admission and asphyxia were associated with BPBI severity. An improved understanding of the relationship between perinatal factors and BPBI severity may be used to guide early referral to BPBI providers and support prevention efforts.

BACKGROUND Patient-reported Outcomes Measurement Information System (PROMIS) for pediatrics is a validated patient-reported or parent-proxy-reported outcomes assessment tool used to evaluate health-related quality of life in children and adolescents with chronic medical conditions. The health-related quality of life of children with brachial plexus birth injury (BPBI) as measured by PROMIS is not well understood. We hypothesized that children with BPBI would report impaired upper extremity (UE) function but normal mobility, pain interference, and peer relationships compared with a reference pediatric population, and that UE function PROMIS scores would be associated with BPBI severity and patient age. METHODS This is a retrospective cohort study of 180 children with BPBI ages 5 to 17 years old who responded to 4 pediatric PROMIS domains (mobility, pain interference, peer relationships, and UE function) between April 2017 and April 2019. Responses were converted to a T score, which allows comparison with a reference pediatric population (mean reference score=50). Multivariable linear regression was used to quantify the association between PROMIS scores and age, sex, Narakas type, and composite Mallet score. RESULTS Children with BPBI had normal PROMIS mobility (49.6±8.5), pain interference (44.6±9.7), and peer relationships (52.4±10.6) scores, but reported mild impairment in UE function (40.8±12.1). Age (P

Forearm supination contractures occur in 7% of children with brachial plexus birth injuries (BPBI). Biceps rerouting is proposed when pronation has deteriorated but is passively correctable to at least 0° (neutral). The purpose of this investigation was to evaluate long-term outcomes of biceps rerouting for this indication, including magnitude and maintenance of correction, complications, and subsequent osteotomy.We conducted a retrospective review of all children with BPBI and forearm supination contractures treated with biceps rerouting alone, for the above indications, from 1993 to 2017 with at least 2 years follow-up. Demographic information, BPBI characteristics, surgical details, and ranges of motion were obtained from medical records. Pre- and postoperative active pronation (AP) and supination (AS), elbow flexion contracture, and arc of forearm rotation (Arc) were analyzed using linear mixed-effect models.Twenty-five children (13 females; 13 left forearms; 15 global BPBI) underwent biceps rerouting at age 7 ± 3 years and were followed for 6 ± 3 years. Before surgery, the mean AP and AS were 6° ± 29° and 62° ± 27°, respectively. At the final follow-up, the mean AP, AS, and Arc were 39° ± 36°, 18° ± 34°, and 57° ± 42°, respectively. AP was significantly improved and AS was significantly decreased by 2 years after surgery and at the final follow-up. Neither Arc nor elbow flexion contracture changed significantly. Two of 25 (8%) children underwent subsequent forearm osteotomy.Biceps rerouting in children with BPBI improves the forearm position when pronation is deteriorating by shifting the arc from supination to pronation without decreasing the arc of motion or worsening elbow flexion contractures. There is a low risk of complications and a limited need for subsequent forearm osteotomy. These results are maintained over time. When performed before passive pronation is reduced beyond neutral, this procedure may prevent severe supination contractures and reduce the need for forearm osteotomy.Therapeutic IV.

Madelung’s deformity is an uncommon congenital wrist condition characterized by premature closure of the volar-ulnar aspect of the distal radius physis, volar carpal subluxation, and distal ulna prominence. It is classified as a malformation of the radio-ulnar axis involving the entire upper limb, according to the Oberg-Manske-Tonkin Classification. It accounts for less than 2% of congenital upper extremity differences. Madelung’s deformity is most commonly idiopathic, but a Madelung-like deformity may result from trauma, infection, multiple hereditary exostoses (MHE), and Ollier’s disease. Additionally, it is associated with skeletal dysplasias involving mutations of the short stature homeobox (SHOX) gene. Madelung’s deformity predominantly affects females and becomes clinically apparent during adolescence. Affected individuals may present with wrist pain, restricted range of motion of the wrist and forearm, decreased grip strength, and function difficulties, as well as aesthetic concerns. Several surgical options have been described for children with Madelung’s deformity, depending on their age and degree of deformity, and include physiolysis, soft tissue release, and osteotomies, with promising outcomes.

Surgical techniques to restore nerve function in infants with brachial plexus birth injuries (BPBI) include nerve grafting, neurolysis, and nerve transfers. These techniques result in reliable recovery of shoulder abduction, shoulder flexion, and elbow flexion in infants with upper and middle trunk injuries who have delayed or absent nerve recovery (Gilbert et al. Microsurgery. 26(4):334–342, 2006; Lin et al. Plast Reconstr Surg. 123(3):939–948, 2009; Manske et al. Plast Reconstr Surg. 143(5):1017e–26e, 2019; Boome and Kaye, J Bone Joint Surg Br. 70(4):571–576, 1988; Pondaag et al. Neurosurgery, 57(3):530–537, 2005; Malessy, J Bone Joint Surg Am. 96(20):e174, 2014). Infants with global brachial plexus injuries, for whom reconstructive strategies include reconstruction of hand function, experience more variable results, with less robust recovery of shoulder and elbow function compared with upper and middle trunk injuries; although some recovery of hand motor function is expected, it is not clear that children recover meaningful hand use. (Gilbert and Whitaker, J Hand Surg Edinb Scotl. 16(5):489–491, 1991; Kirjavainen etal. J Bone Joint Surg Br. 90(3):349–355, 2008; Haerle and Gilbert, J Pediatr Orthop. 24(2):194–200, 2004; Pondaag and Malessy, J Neurosurg. 105(1 Suppl):33–40, 2006; Terzis and Kokkalis, Plast Reconstr Surg. 122(2):516–526, 2008; Maillet and Romana, J Child Orthop. 3(2):101–108, 2009). However, each infant’s injury is unique, and despite postoperative improvements, persistent upper extremity impairment and the need for further intervention are common in both groups (upper trunk and global palsies), regardless of surgical technique.

BACKGROUND Shoulder external rotation recovery in brachial plexus birth injury is often limited. Nerve grafting to the suprascapular nerve and transfer of the spinal accessory nerve to the suprascapular nerve are commonly performed to restore shoulder external rotation, but the optimal surgical technique has not been clearly demonstrated. We investigated whether there was a difference between nerve grafting and nerve transfer in terms of shoulder external rotation recovery or secondary shoulder procedures. METHODS This is a multicenter, retrospective cohort study of 145 infants with brachial plexus birth injury who underwent reconstruction with nerve grafting to the suprascapular nerve (n = 59) or spinal accessory nerve to suprascapular nerve transfer (n = 86) with a minimum follow-up of 18 months (median, 25.7 months [interquartile range, 22.0, 31.2 months]). The primary outcome was the Active Movement Scale (AMS) score for shoulder external rotation at 18 to 36 months. The secondary outcome was secondary shoulder surgery. Two-sample Wilcoxon and t tests were used to analyze continuous variables, and the Fisher exact test was used to analyze categorical variables. The Kaplan-Meier method was used to estimate the cumulative risk of subsequent shoulder procedures, and the proportional hazards model was used to estimate hazard ratios (HRs). RESULTS The grafting and transfer groups were similar in Narakas type, preoperative AMS scores, and shoulder subluxation. The mean postoperative shoulder external rotation AMS scores were 2.70 in the grafting group and 3.21 in the transfer group, with no difference in shoulder external rotation recovery between the groups (difference, 0.51 [95% confidence interval (CI), -0.31 to 1.33]). A greater proportion of the transfer group (24%) achieved an AMS score of >5 for shoulder external rotation compared with the grafting group (5%) (odds ratio, 5.9 [95% CI, 1.3 to 27.4]). Forty percent of the transfer group underwent a secondary shoulder surgical procedure compared with 53% of the grafting group; this was a significantly lower subsequent surgery rate (HR, 0.58 [95% CI, 0.35 to 0.95]). CONCLUSIONS Shoulder external rotation recovery in brachial plexus birth injury remains disappointing regardless of surgical technique, with a mean postoperative AMS score of 3, 17% of infants achieving an AMS score of >5, and a high frequency of secondary shoulder procedures in this study. Spinal accessory nerve to suprascapular nerve transfers were associated with a higher proportion of infants achieving functional shoulder external rotation (AMS score of >5) and fewer secondary shoulder procedures. LEVEL OF EVIDENCE Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Purpose Shoulder internal rotation contractures (IRC) are common sequela of brachial plexus birth injuries (BPBI). Botulinum toxin A (BTX-A) injection into targeted muscles has been described to facilitate functional improvement at the shoulder joint and prevent glenohumeral dysplasia. The purpose of this study was to assess the outcomes of BTX-A injections on shoulder IRC in children with BPBI. Methods We conducted a retrospective analysis of 47 children with shoulder IRC due to BPBI, who were treated with BTX-A. Shoulder passive external rotation in adduction and Active Movement Scale external rotation scores were recorded before and after BTX-A injection. We also recorded the number of children who underwent secondary surgical balancing procedures to improve shoulder motion after BTX-A injection. Results Mean age at the time of injection was 12 months (range, 5–23 months). Subjects demonstrated a significant increase in passive external rotation of 46° (range, 10° to 90) at 4 months; an average improvement of 18° (range, –30° to 80°) persisted at 11 months after injection. A total of 28 patients (60%) underwent subsequent external rotation tendon transfer. At 5-year follow-up, 7 patients (15%) had adequate functional shoulder range of motion and did not undergo external rotation tendon transfer. Conclusions Botulinum toxin A injections result in improvement in IRC due to BPBI, which is sustained beyond the expected half-life of 3 months. As many as 15% of patients who have this treatment avoid external rotation tendon transfer. Type of study/level of evidence Diagnostic IV.

Background Infants with brachial plexus birth injury who do not recover motor function spontaneously in a timely manner are candidates for brachial plexus reconstruction with nerve autograft. Outcomes of this intervention are incompletely understood. The authors present the long-term outcomes of brachial plexus reconstruction with sural nerve autograft in infants with brachial plexus birth injury. Methods The authors retrospectively reviewed all infants with brachial plexus birth injury who underwent brachial plexus reconstruction with sural nerve autograft between 1992 and 2014 with a minimum 2-year follow-up. The authors used Active Movement Scale scores to determine the presence and timing of shoulder, elbow, and wrist recovery. They assessed recovery of hand function in infants with global brachial plexus birth injury with the Raimondi scale. The number and type of secondary reconstructive procedures were identified. Results Forty-three infants who underwent brachial plexus reconstruction at age 7 ± 2 months old were followed for 7 ± 5 years. Most infants recovered antigravity elbow flexion (91 percent) and shoulder abduction (67 percent), but fewer recovered antigravity shoulder external rotation (19 percent) and wrist extension (37 percent). Mean postoperative times until observed antigravity motor strength (Active Movement Scale score >5) at the shoulder, elbow, and wrist were all greater than 12 months; evidence of initial motor recovery (Active Movement Scale score >2) was observed earlier. The mean Raimondi score in infants with global brachial plexus birth injury was 2.2 (range, 0 to 5) at final follow-up. Thirty-three children underwent 2 ± 1.2 secondary reconstructive procedures. Conclusions Brachial plexus reconstruction with sural nerve autograft reliably results in recovery of shoulder abduction and elbow flexion, but recovery of shoulder external rotation and wrist extension is less predictable, and recovery often takes more than 1 year. Secondary procedures are often performed to optimize function. Clinical question/level of evidence Therapeutic, IV.

Background: The anatomy of the scapholunate interosseous ligament (SLIL) has been described qualitatively in great detail, with recognition of the dorsal component’s importance for carpal stability. The purpose of this study was to define the quantitative anatomy of the dorsal SLIL and to assess the use of high-frequency ultrasound to image the dorsal SLIL. Methods: We used high-frequency ultrasound imaging to evaluate 40 wrists in 20 volunteers and recorded the radial-ulnar (length) and dorsal-volar (thickness) dimensions of the dorsal SLIL and the dimensions of the scapholunate interval. We assessed the use of high-frequency ultrasound by comparing the length and thickness of the dorsal SLIL on ultrasound evaluation and open dissection of 12 cadaveric wrists. Student’s t test was used to assess the relationship between measurements obtained on cadaver ultrasound and open dissection. Results: In the volunteer wrists, the mean dorsal SLIL length was 7.5 ± 1.4 mm and thickness was 1.8 ± 0.4 mm; the mean scapholunate interval was 5.0 mm dorsally and 2.5 mm centrally. In the cadaver wrists, there was no difference in dorsal SLIL length or thickness between ultrasound and open dissection. Conclusions: The dorsal SLIL is approximately 7.5 mm long and 1.8 mm thick. These parameters may be useful in treatment of SLIL injuries to restore the native anatomy. High-frequency ultrasound is a useful imaging technique to assess the dorsal SLIL, although further study is needed to assess the use of high-frequency ultrasound in detection of SLIL pathology.

Posterior elbow capsulotomy plus triceps lengthening facilitates passive elbow flexion in children with arthrogryposis multiplex congenita, allowing independent function for activities of daily living, such as feeding and self-care of the face and hair. DESCRIPTION: The posterior aspect of the distal end of the humerus and the olecranon are identified by palpation and exposed via a curvilinear incision over the posterior aspect of the elbow. Identifying the osseous landmarks can be challenging in some patients. The ulnar nerve is identified and protected. The triceps tendon is isolated, and z-lengthening is performed. Next, the posterior elbow capsule is incised proximal to the tip of the olecranon to expose the joint surface, and the arthrotomy is continued incrementally along the medial and lateral capsule until elbow flexion increases by ≥40°, or past 90° (maximum, 120°), with contact between the lengthened ends of the triceps tendon for repair. The triceps tendon is then repaired in the elongated position. After the wound is closed, the elbow is placed in flexion and immobilized in a cast. ALTERNATIVES: Alternative treatments include passive stretching exercises to increase elbow flexion. RATIONALE: Elbow extension contractures result in substantial limitations in the activities of daily living for children with arthrogryposis multiplex congenita. Those who fail to attain at least 90° of elbow flexion with passive stretching in the first year of life benefit from posterior elbow release and triceps lengthening. In addition, children with

Purpose To assess recurrence and complications in children with radial longitudinal deficiency treated with or without external fixator soft tissue distraction prior to centralization. Methods Thirteen upper extremities treated with centralization alone were compared with 13 treated with ring fixator distraction followed by centralization. Resting wrist position between the 2 groups was compared before surgery, approximately 2 years after surgery (midterm), and at final follow-up, which was at a mean of 10 years for the centralization-alone group and 6 years for the distraction group. Radiographs were reviewed for hand-forearm angle, hand-forearm position, volar carpal subluxation, ulnar length, and physeal integrity. Results The clinical resting wrist position was improved significantly after surgery and at final follow-up in both groups, but recurrence was worse at final follow-up in the distraction group patients. Radiographically, in the centralization alone group, the hand-forearm angle improved from 53° before surgery to 13° at midterm but worsened to 27° at final follow-up. In the distraction group, the hand-forearm angle improved from 53° before surgery to 21° at midterm but worsened to 36° at final follow-up. The hand-forearm position improved between preoperative and final assessment in both groups, but at final follow-up, the centralization-alone group had a significantly better position. Volar subluxation was 4 mm improved in the centralization alone group and 2 mm worse in the distraction group at final follow-up. Conclusions Centralization, with or without distraction with an external fixator, resulted in improved alignment of the wrist. Distraction facilitated centralization, but it did not prevent deformity recurrence and was associated with a worse final radial deviation and volar subluxation position compared with wrists treated with centralization alone. Type of study/level of evidence Therapeutic III.