Sprengel's deformity: an analysis of surgically and nonsurgically treated patients

Abstract

Background: Sprengel's deformity is a congenital anomaly of the scapula and can be associated with reduced shoulder mobility and esthetic concerns. Controversies remain regarding its natural history, radiographic findings, and optimal treatment strategy. Methods: A total of 74 Sprengel's shoulders in 71 patients presenting to a pediatric referral hospital were retrospectively reviewed including 24 surgically treated shoulders. Median age at initial presentation was 3.8 years. Median age at the final follow-up was 9.6 years. Results: Twenty shoulders were analyzed for range of motion across time. The median composite abduction range was initially 105 and at the last time-point 98 , with a median loss of 15 over 5 years (P ¼ .007). A total of 26% of the nonsurgical shoulders reported pain at the latest follow-up, compared with 1 surgical patient (4%) (P ¼ .028). Older age was independently associated with pain (P < .001); this effect was only seen in nonsurgical patients. Radiographically, the glenoid inclination angle (GIA) independently correlated with the abduction range (P ¼ .001). Surgically treated shoulders underwent a modified Woodward procedure at a median 4.8 years of age. At a median follow-up of 41 months, the abduction range improved in the surgical group from 90 preoperatively to 110 postoperatively, with a median gain of 40 (P < .001). The median GIA changed from À2 to 4 with a median gain of 9 (P ¼ .004). Conclusions: The natural history of untreated Sprengel's deformity may be one of gradual decline in the abduction range, with a proportion of patients developing pain in adolescence. The GIA correlates with the shoulder abduction range. Surgery with a modified Woodward procedure may alter the natural history of the condition by improving motion and reducing risk of pain later in life.

Surgical Treatment of Sprengel’s Deformity: A Systematic Review and Meta-Analysis

“…Most studies reported the patients’ sex, except three [ 34 , 43 , 57 ], and the affected side, except eight [ 21 , 23 , 43 , 47 , 50 , 54 , 57 , 59 ]. In the preoperative evaluation, 28 studies classified patients according to the Cavendish clinical scoring system [ 2 , 9 , 14 , 16 , 17 , 19 , 20 , 21 , 23 , 33 , 35 , 36 , 37 , 38 , 41 , 42 , 44 , 45 , 46 , 47 , 48 , 49 , 52 , 53 , 55 , 58 , 59 , 60 ] and 15 studies [ 14 , 15 , 16 , 17 , 19 , 21 , 32 , 36 , 41 , 45 , 48 , 53 , 57 ,…”

“…Ninety-seven patients (22%) were grade 4, 283 patients (64.2%) were grade 3, 51 patients (11.5%) were grade 2 and 10 patients (2.3%) were grade 1. Fifteen studies (283 patients) used Rigault’s classification [ 14 , 15 , 16 , 17 , 19 , 21 , 32 , 36 , 41 , 45 , 48 , 53 , 57 , 58 , 60 ]: 41% reported score 3 (116/283), 59% score 2 (167/283), whereas no patient had score 1 at the preoperative assessment. Only two papers did not report any vertebral-shoulder connection in their patients [ 9 , 40 ]; the other studies identified a connection between the scapula and spine in 305 patients (46.9%), represented by an omovertebral bone bar (264 patients), fibrous bridge (22 cases) or cartilaginous bar (19 cases).…”

“…The Putti–Schrock procedure was reported only by Farsetti [ 32 ] and Sulamaa [ 13 ] in nine cases. The original and modified Green procedure was reported in 14 papers [ 14 , 15 , 23 , 34 , 35 , 37 , 39 , 40 , 44 , 45 , 47 , 48 , 51 , 53 ], accounting for 280 patients; Woodward’s procedure (or modified techniques) was performed in 18 studies [ 2 , 9 , 16 , 17 , 23 , 32 , 33 , 42 , 43 , 46 , 49 , 50 , 54 , 55 , 56 , 57 , 58 , 60 ], accounting for 234 patients. Bhasker [ 41 ], Masquijo [ 19 ] and Mears [ 18 ] treated 29 patients overall with Mears’s technique.…”

“…The mean follow-up was 6.25 years (range: 12 months–28 years). All studies, except for seven of them [ 16 , 23 , 33 , 37 , 42 , 57 , 61 ], reported the length and type of immobilization. In the studies reviewed, the mean period of immobilization was 3 weeks (range, 0.29–6 weeks), usually with a Velpeau or similar bandages.…”

“…Of them, 16 studies (236 patients) [ 14 , 17 , 19 , 20 , 21 , 35 , 36 , 37 , 41 , 44 , 46 , 52 , 55 , 58 , 59 , 60 ] reported preoperative and postoperative raw data. Fourteen studies (219 patients) reported the postoperative Rigault classification [ 14 , 15 , 16 , 17 , 19 , 21 , 32 , 36 , 41 , 45 , 48 , 57 , 58 , 60 ]; of these, eight studies [ 14 , 15 , 17 , 21 , 32 , 36 , 58 , 60 ] reported preoperative and post-operative radiological raw data. A total of 18 studies (236 patients) reported preoperative and postoperative raw data for the Cavendish score (10 studies), Rigault score (two studies) or both (six studies), thus allowing for meta-analysis.…”