Sprengel deformity

SPRENGEL DEFORMITY PRESENTED BY DR JOYDEEP MALLIK
GANGA HOSPITAL, COIMBATORE,TAMILNADU

1. Sprengel deformity Presented by: Dr. Joydeep Mallik GANGA HOSPITAL COIMBATORE

2. Definition: Rare congenital anamoly which arises from interruption of normal caudal migration of the scapula and is characterized by elevation & medial rotation of scapula.

3. Historical significance: First described by Eulenberg (1863) who described 3 patients. Willet and Walsham reported 2 cases with anatomic descriptions of this clinical entity (1883) It is named after Otto Gerhard Karl Sprengel (1852-1915), a German surgeon who described four cases in 1891.

4. Frequency: most common congenital malformation of the shoulder girdle. Age – Mostly noticed at birth Gender : Equal distribution in both sexes Side – Left side more common than right, bilateral only in 10%

5. Genetics: The condition is sporadic. Rarely, autosomal dominant pattern of inheritance.

6. Embryology: The scapula is a cervical appendage that normally differentiates opposite the C4,C5,C6 vertebrae at 5 weeks of gestation. normally descends to the thorax by the end of the third month of intrauterine life. Interruption in the normal caudal migration of the scapula results in a hypoplastic , elevated scapula, known as the Sprengel deformity .

7. Pathophysiology : Occurs between the 9th and 12th week of gestation. An arrest in the development of bone, cartilage, and muscle also occurs. The trapezius , rhomboid , or levator scapulae muscle may be absent or hypoplastic . The serratus anterior muscle may be weak, leading to winging of the scapula. Other muscles, such as the pectoralis major, latissimus dorsi , or the sternocleidomastoid may be hypoplastic and similarly involved.

8. SCAPULA : Dysplastic. High up than normal. Smaller in the vertical plane and larger horizontally. Inferior angle is rotated medially, causing the glenoid to face inferiorly. Convexity of the upper ( supraspinous ) portion of the scapula is increased and curvature of the clavicular shaft is decreased, forming a narrower scapuloclavicular space , may contribute to brachial plexus compression postoperatively.

9. Another anomaly seen in approximately one third of patients with a Sprengel deformity is the omovertebral bone. This is a rhomboid- or trapezoid-shaped structure of cartilage or bone. Usually lies in a strong fascial sheath, which extends from the superomedial border of the scapula to the spinous processes, lamina, or transverse processes of the cervical spine C4 to C7. Omovertebral bone is best visualized on a lateral or oblique radiograph of the cervical spine.

10. Klippel-Feil syndrome and Sprengel’s deformity Congenital fusion of at least 2 cervical vertebrae with/without additional spinal/ extraspinal manifestations Associated Sprengel’s deformity: 7%-42% Most common congenitally fused segment in Sprengel’s deformity: C6-C7;extensive fusion patterns common Thorough neurological examination to be done preoperatively to avoid complications during surgery and anesthesia Short neck Low hair line Restriction of neck movement

11. CLINICAL FEATURES Cosmetic   High position of the scapula Scoliosis  Torticollis   Caput obstiosum (asymmetric distortion of the skull) Facial asymmetry Functional Restricted motions of scapula and shoulder joint

12. Clinically the severity of the elevation of scapula has been described by Cavendish (1972) as: Grade I (Very mild) Shoulders level; deformity invisible when patient is dressed Grade II (Mild) Shoulders almost level; deformity visible as a lump in the web of the neck when patient is dressed Grade III (Moderate) Shoulder joint is elevated 2-5 centimeters; deformity visible Grade IV (Severe) Shoulder joint is elevated; superior angle of the scapula near the occiput Sprengel’s shoulder

13. DIAGNOSIS The x-ray appearances are characteristics,showing the unduly high situation of the scapula.

14. Radiological criteria

15. Radiographic Rigault’s classification grade I: superomedial angle lower than T2 but above T4 transverse process grade II: superomedial angle located between C5 and T2 transverse process grade III: superomedial angle above C5 transverse process

16. Computed tomography (CT) scan CT scans with 3-dimensional (3-D) reconstruction may be performed to visualize the pathoanatomy of the affected region and to visualize the omovertebral bar. CT scans may also help in planning surgery.

17. CT scan and 3D reconstruction show the omovertebral connection arising from the medial border of the scapula and the vertebral column, anterior curving of the supraspinous portion of the scapula, the convex medial border and the concave lateral border of scapula

18. DIFFERENTIAL DIAGNOSIS Elevated scapula due to old paralysis. Spinal accessory nerve injury Long thoracic nerve injury

19. PROGNOSIS Even if operation is undertaken, the prognosis is not very favorable. Literatures indicate that while the mobility of the shoulder may be improved, asymmetry almost always persists.

20. SURGICAL TREATMENT Factors to be assessed severity of the deformity, functional impairment, Age associated comorbid conditions. Surgery is best advisable for a patient between 3 and 8 years of age with moderate or severe cosmetic/ functional deformity. The presence of associated congenital anomalies may be contraindications to operation. Surgical intervention before the age of 2 years is extensive and is technically more difficult. Best results are obtained if surgery is performed below the age of 5 years

21. SURGICAL TREATMENT

22. SURGICAL OPTIONS Putti’s procedure Shrock modified Putti’s procedure Woodward procedure Modified Woodward's procedure Green scapuloplasty Modified Green’s procedure Mears procedure

23. Putti’s procedure: detachment of the scapular insertion of the rhomboids and trapezius , omovertebral bar resection, followed by lowering the scapula and fixing its inferior angle to a rib at the corrected level

24. Shrock modified Putti’s procedure: subperiosteal dissection of the musculature and adding an osteotomy of the supraspinous scapular region and the acromial base to facilitate scapular descent

25. Green scapuloplasty : resection of the prominent superior scapular border and extra- periosteal division of the muscular attachments of scapula to allow the scapula to be displaced inferiorly and muscular reattachment at the newer corrected level at the scapula

26. Trapezius muscle disinrection step from its scapula and clavicle attachments G. Andrault , F. Salmeron , J.M. Laville Green's surgical procedure in Sprengel's deformity: Cosmetic and functional results Orthopaedics & Traumatology : Surgery & Research, Volume 95, Issue 5, 2009, 330 - 335 http://dx.doi.org/10.1016/j.otsr.2009.04.015

27. supraspinatus fossa bone resection, omovertebral bone resection, figure of L type lenghtening of levator scapulae, global lowering, rhomboid muscles reattachment at a higher site and distal tip scapula fixation G. Andrault , F. Salmeron , J.M. Laville Green's surgical procedure in Sprengel's deformity: Cosmetic and functional results Orthopaedics & Traumatology : Surgery & Research, Volume 95, Issue 5, 2009, 330 - 335 http://dx.doi.org/10.1016/j.otsr.2009.04.015

28. Modified Green scapuloplasty : Andrault et al . suggested modifications to Green’s procedure (a) dis -insertion of supraspinatus , (b) clavicular osteotomy and (c) a limited release of the serratus anterior to facilitate the descent of the scapula. incidence of brachial plexus palsy could be reduced by clavicular osteotomy , and that scapular winging could be prevented by doing only a limited release of serratus anterior from the medial scapular border

29. Woodward procedure: Transfer of the origin of the trapezius muscle to a more inferior position on the spinous processes. This was maintained by placing the scapula in a pocket of the trapezius muscle.

30. Modified Woodward's procedure: for achieving better abduction and correction of the glenoid tilt the scapula was anchored to the lower dorsal vertebrae by a stout absorbable suture placed through the superomedial scapula, so as to externally rotate it and cause lateral displacement of the inferior angle, thereby achieving correction of glenoid vara

31. Prone position Preparation of parts done till occiput MODIFIED WOODWARD’S PROCEDURE

32. MODIFIED WOODWARD’S PROCEDURE Midline vertical incision

33. MODIFIED WOODWARD’S PROCEDURE Detachment of attachment of trapezius and rhomboids from spinous processes

34. MODIFIED WOODWARD’S PROCEDURE Release of omovertebral band Excision of superomedial angle of scapula

35. MODIFIED WOODWARD’S PROCEDURE Relocation of scapula to new position

36. MODIFIED WOODWARD’S PROCEDURE Suturing of trapezius to inferior Spinous processes

37. Closure in Layers MODIFIED WOODWARD’S PROCEDURE

38. Mears procedure In a report by Mears, the author described a novel approach-- (a) subperiosteal elevation of the scapular musculature, (b) extraperiosteal resection of the omovertebral bone, (c) supraspinatous fossa osteotomy , (d) release of long head of triceps and a portion of the origin of teres minor from the scapula and (e) resection of the superolateral border of the scapula to gain abduction He reported a significant improvement in function following this procedure.

39. The shaded region represents the area to be osteotomized (A – Reflected trapezius ; B – Rhomboids; C – Levator scapulae; T – The detached triceps)

40. Int J Shoulder Surg. 2011 Jan-Mar; 5(1): 1–8. doi :  10.4103/0973-6042.80459

41. Postoperative complications Winging of the scapula that may result from incomplete reattachment of the serratus anterior muscle Brachial plexus injury To avoid brachial plexus palsy, several authors recommended morcellization of the clavicle on the ipsilateral side as a first step in the operative treatment of Sprengel deformity. Keloid formation.

42. PHYSIOTHERAPY AFTER SURGERY Gradual relaxed passive mobilization of the shoulder and scapula. Suitable pain relieving modality like TENS, IFT and hydrocollator packs may be used to induce relaxation . Special attention is given to achieve early mobility of the scapula and the shoulder abduction and elevation. Overall mobilization and strengthening of the shoulder girdle muscles. Emphasize maximum possible correction of the posture of shoulder and maintain it. 5/6/2016 50

43. THANK YOU