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Clinical Presentation and Patient-Reported Function in Children with Sprengel's Deformity
Julianna Lee, MD
1; Eliza Buttrick, BA
1; Carley Vuillermin, MBBS, FRACS
2; Lindley B Wall, MD
3; Julie Balch Samora, MD, PhD
4; Apurva S Shah, MD MBA
1,51The Children's Hospital of Philadelphia, Philadelphia, PA; 2Upper Extremity Surgery/Orthopaedics, Boston Children's Hospital/Harvard University, Boston, MA; 3Washington University School of Medicine, St. Louis, MO; 4Nationwide Children's Hospital, Columbus, OH; 5Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA
Introduction:Sprengel's deformity is a rare congenital anomaly of the scapula with limited data of the resulting impact on physical function. We hypothesize that children with Sprengel's deformity will have reduced shoulder range of motion, but that metrics of psychosocial wellbeing will demonstrate less anxiety and depression than the normal population.
Materials & Methods:Children with Sprengel's deformity were prospectively enrolled into the multicenter Congenital Upper Limb Differences (CoULD) Registry between 2012-2023. Shoulder range of motion and patient-reported outcomes (PROs) data were collected from the patient's most recent pre-operative follow-up and compared to normative values. PROs included the Pediatric Outcomes Data Collection Instrument (PODCI) and PROMIS Depression, Anxiety, Peer Relations, and Upper Extremity domains. Univariate analysis was performed between bilateral, syndromic, and Cavendish subgroups.
Results:In total, 59 patients (31, 52.5% female) were included with median age of 5.9 years at most recent evaluation. Most patients (53, 89.9%) presented with a unilateral deformity and a minority of cases (13, 22.0%) were syndromic, predominantly Klippel-Feil Syndrome (9/13, 69.2%). Cavendish Grade III deformity was most common (31/61, 50.8%) followed by 24.6% Grade II, 14.8% Grade I, and 9.8% Grade IV. Average shoulder abduction (113°) and forward elevation (120°) were significantly less than normal range (p<0.001) (
Table 1). Children with Sprengel's deformity reported less anxiety/depression than the normative population as indicated by a mean PROMIS Anxiety score of 44.5 (p=0.017) and a mean Depression score of 45.9 (p=0.028). Mean PROMIS Upper Extremity scores also trended lower than normative values. Patients scored significantly lower on PODCI Upper Extremity Function, Sports, and Global Function modules than the normative population (p<0.05) (
Table 1). PODCI Transfer sub-scores significantly varied by Cavendish classification (p=0.011) with Grade 1 and 2 deformities scoring within the normal range (average 100.0 and 97.0, respectively) versus Grades 3 and 4 scoring below normal (average 91.0 and 93.5, respectively). Neither bilateral presentation nor syndromic association significantly affected PROs (p>0.05).
Conclusions:Children with Sprengel's deformity have decreased shoulder range of motion, specifically abduction and forward elevation which is worse in children with Cavendish Grades 3 and 4 deformities. Regardless of bilateral or syndromic presentation, patients generally demonstrated significantly lower physical function and sports abilities, but decreased anxiety and depression compared to unaffected populations.
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