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

Overview

Journal Hand (N Y)

Publisher Sage Publications

Specialties General Surgery
Orthopedics

Date 2021 May 18

PMID 34002631

Authors

Sarah M Schippers

Hailee Reist

Qiang An

Joseph A Buckwalter V

Affiliations

Soon will be listed here.

Abstract

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.

References

Bohannon R . Grip strength: a summary of studies comparing dominant and nondominant limb measurements. Percept Mot Skills. 2003; 96(3 Pt 1):728-30. DOI: 10.2466/pms.2003.96.3.728. View

Taghinia A, Yorlets R, Doyle M, Labow B, Upton J . Long-Term Functional Upper-Extremity Outcomes in Adults with Apert Syndrome. Plast Reconstr Surg. 2019; 143(4):1136-1145. DOI: 10.1097/PRS.0000000000005479. View

Hudak P, Amadio P, Bombardier C . Development of an upper extremity outcome measure: the DASH (disabilities of the arm, shoulder and hand) [corrected]. The Upper Extremity Collaborative Group (UECG). Am J Ind Med. 1996; 29(6):602-8. DOI: 10.1002/(SICI)1097-0274(199606)29:6<602::AID-AJIM4>3.0.CO;2-L. View

Beals R, Crawford S . Congenital absence of the pectoral muscles. A review of twenty-five patients. Clin Orthop Relat Res. 1976; (119):166-71. View

Stark T, Walker B, Phillips J, Fejer R, Beck R . Hand-held dynamometry correlation with the gold standard isokinetic dynamometry: a systematic review. PM R. 2011; 3(5):472-9. DOI: 10.1016/j.pmrj.2010.10.025. View

Kessler R, Wang P . The descriptive epidemiology of commonly occurring mental disorders in the United States. Annu Rev Public Health. 2008; 29:115-29. DOI: 10.1146/annurev.publhealth.29.020907.090847. View

Stutz C, Mills J, Wheeler L, Ezaki M, Oishi S . Long-term outcomes following radial polydactyly reconstruction. J Hand Surg Am. 2014; 39(8):1549-52. DOI: 10.1016/j.jhsa.2014.05.006. View

Buckwalter V J, Shah A . Presentation and Treatment of Poland Anomaly. Hand (N Y). 2017; 11(4):389-395. PMC: 5256661. DOI: 10.1177/1558944716647355. View

Mysnyk M, Johnson D . Congenital absence of the pectoralis muscles in two collegiate wrestling champions. Clin Orthop Relat Res. 1991; (265):183-6. View

10.

Carr T, Moss T, Harris D . The DAS24: a short form of the Derriford Appearance Scale DAS59 to measure individual responses to living with problems of appearance. Br J Health Psychol. 2005; 10(Pt 2):285-98. DOI: 10.1348/135910705X27613. View

11.

Ware Jr J, Sherbourne C . The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care. 1992; 30(6):473-83. View

12.

Moir C, Johnson C . Poland's syndrome. Semin Pediatr Surg. 2008; 17(3):161-6. DOI: 10.1053/j.sempedsurg.2008.03.005. View

13.

Ozols D, Butnere M, Petersons A . Methods for Congenital Thumb Hypoplasia Reconstruction. A Review of the Outcomes for Ten Years of Surgical Treatment. Medicina (Kaunas). 2019; 55(10). PMC: 6843639. DOI: 10.3390/medicina55100610. View

14.

Jones H . Congenital Absence of the Pectoral Muscles. Br Med J. 2010; 2(3418):59-60. PMC: 2522945. DOI: 10.1136/bmj.2.3418.59-a. View

15.

Goldfarb C, Klepps S, Dailey L, Manske P . Functional outcome after centralization for radius dysplasia. J Hand Surg Am. 2002; 27(1):118-24. DOI: 10.1053/jhsu.2002.30078. View

16.

Martinez G, Daniels K, Febo-Vazquez I . Fertility of Men and Women Aged 15-44 in the United States: National Survey of Family Growth, 2011-2015. Natl Health Stat Report. 2018; (113):1-17. View

17.

Baban A, Torre M, Costanzo S, Gimelli S, Bianca S, Divizia M . Familial Poland anomaly revisited. Am J Med Genet A. 2011; 158A(1):140-9. DOI: 10.1002/ajmg.a.34370. View

18.

Yiyit N, Isitmangil T, Oksuz S . Clinical analysis of 113 patients with Poland syndrome. Ann Thorac Surg. 2015; 99(3):999-1004. DOI: 10.1016/j.athoracsur.2014.10.036. View

19.

Doring A, Nota S, Hageman M, Ring D . Measurement of upper extremity disability using the Patient-Reported Outcomes Measurement Information System. J Hand Surg Am. 2014; 39(6):1160-5. DOI: 10.1016/j.jhsa.2014.03.013. View

20.

Al-Qattan M . Classification of hand anomalies in Poland's syndrome. Br J Plast Surg. 2001; 54(2):132-6. DOI: 10.1054/bjps.2000.3505. View