Natural History of Poland Syndrome: A Long-term Study of Functional and Psychosocial Outcomes.

BACKGROUND: Poland syndrome (PS) is a congenital condition characterized by pectoralis hypoplasia and an ipsilateral hand anomaly that frequently necessitates surgery in childhood. This study aimed to determine long-term functional and psychosocial implications of PS. METHODS: Patients underwent strength testing of their affected limb by comparing with their contralateral arm. They completed Disabilities of the Arm, Shoulder, and Hand (DASH), 36-item Short-Form Health Survey (SF-36), and several Patient-Reported Outcome Measurement Information System (PROMIS) surveys. Aggregate scores were compared with population norms. RESULTS: Twenty-eight patients were enrolled, and 16 returned for strength testing. The average age was 42 years (range, 18-65 years), and the average follow-up was 25 years (range, 1-52 years). Dynamometer testing showed decreased strength in shoulder internal rotation and abduction/adduction, hand grip, and key pinch/tip pinch. Average DASH and SF-36 scores were comparable with population norms. The PROMIS Upper Extremity score (46.2) was significantly lower than the average 50 (SD, 10.0); the Social Roles score (57.3) indicated significantly less disability than the general population. Seventy-nine percent reported higher than average satisfaction with life, 82% had normal self-esteem, and 21% and 4% met screening criteria for depression and anxiety, respectively. The Derriford Appearance Scale revealed that 68% of patients identified PS as their most self-conscious feature, with 36% reporting subsequent life adjustments. CONCLUSIONS: This cohort of PS patients self-reported more favorable functional, psychological, and emotional outcomes than expected based on significant shoulder and hand strength deficits. Despite these deficits, patients make adaptations, allowing fulfilling lives with psychosocial functioning similar to their peers.

Single Forearm Vessel Injury in a Perfused Hand: Repair or Ligate? A Systematic Review.

Background: The purpose of this study was to systematically review available literature reporting vessel patency and how this correlates with cold symptoms following the treatment of a single forearm artery injury when the hand remains perfused. The outcomes of those treated by ligation were compared to those treated with vessel repair. Methods: Electronic databases including PubMed, Embase (Elsevier) and Cochrane Central Register of Controlled Trials (Willey) were searched for studies that reported the outcomes of patients who underwent either ligation or repair of single vessel injuries to hands that remained perfused at time of presentation. Level of evidence was determined by two independent reviewers. Studies were then sorted based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and application of inclusion and exclusion criteria. A total of 19 studies were included for statistical analysis. The patency of repaired vessels was calculated (with comparison between those with radial versus ulnar repair) as was the prevalence of cold symptoms in both ligation (or repairs that went on to occlusion) and repair groups. Results: The average patency of radial and ulnar artery repairs was 68.39% and 65.56% respectively. There was no significant difference between the success rates of these repair groups (pooled estimates for odd ratios was 1.02, p=0.867). The average incidence of cold symptoms in those who underwent ligation (or repair that when on to occlusion) and those that had patent repairs were 19.82% and 17.27% respectively. There was no significant difference between the incidence of cold symptoms between these groups (pooled estimate for proportion of patients with cold symptoms was 0.223, p=0.573). Conclusions: This review showed there to be no significant difference in patency of isolated radial or ulnar artery repairs. There was also no significant difference in the prevalence of cold sensitivity in patients who underwent vessel ligation compared to those who underwent repair (and subsequently remained patent). These results support the conclusion of there being no clear benefit to attempting repair of a single vessel, although further studies are needed given the often incomplete reporting of clinical outcomes in this patient population. Additionally, though a cost-benefit analysis was not included in this review, exploring this aspect of the decision making process could be valuable.Level of Evidence: IV.