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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

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.

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.