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Sprengel deformity
This document provides an overview of Sprengel's deformity, a rare congenital anomaly where the scapula is elevated and rotated medially. Key points: - It results from interrupted migration of the scapula during embryonic development. - Clinical features include an elevated shoulder, restricted shoulder movement, and sometimes scoliosis. Severity is graded based on elevation level. - Diagnosis is made through x-rays showing elevated scapula position. CT scans can show associated bone anomalies. - Surgical treatment aims to lower the scapula through muscle releases and reattachments, with the goal of improving function and asymmetry. Complications can include brachial plexus injury or sc
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Sprengel deformity
- 1. Presented by: Dr.Joydeep Mallik GANGA HOSPITAL COIMBATORE
- 2. Definition: Rare congenitalanamoly which arises from interruption of normal caudal migration of the scapula and is characterized by elevation & medial rotation of scapula.
- 3. Historical significance: Firstdescribed 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 congenitalmalformation 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%
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- 7. Genetics: The conditionis sporadic. Rarely, autosomal dominant pattern of inheritance.
- 8. Embryology: The scapulais 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.
- 9. Pathophysiology: Occurs betweenthe 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.
- 10. SCAPULA: Dysplastic. Highup 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.
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- 12. Another anomalyseen 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.
- 13. Klippel-Feil syndrome andSprengel’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
- 14. 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
- 15. Clinically the severityof 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
- 16. DIAGNOSIS The x-rayappearances are characteristics,showing the unduly high situation of the scapula.
- 18. Radiological criteria • Withshort vertebral border • (The scapula resembles equilateral triangle). Elevated scapula. • Either toward the spine or less commonly to the opposite direction. Rotation of the inferior angle: • Connecting the superior angle to the cervical spine. Omovertebral bone
- 19. 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
- 20. 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.
- 21. CT scan and3D 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
- 23. DIFFERENTIAL DIAGNOSIS Elevatedscapula due to old paralysis. Spinal accessory nerve injuryLong thoracic nerve injury
- 24. PROGNOSIS Even ifoperation is undertaken, the prognosis is not very favorable. Literatures indicate that while the mobility of the shoulder may be improved, asymmetry almost always persists.
- 25. SURGICAL TREATMENT Factorsto 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
- 29. SURGICAL TREATMENT The surgicalprocedures involve a combination of • (a) scapular lowering with either the shift of the origin or the insertion of the scapular muscles on the spine/ scapula, • (b) resection of the superomedial border and • (c) omovertebral bar resection A clavicular morselization is sometimes recommended as a concomitant deformity of this bone may reduce the correction obtained.
- 30. SURGICAL OPTIONS Putti’sprocedure Shrock modified Putti’s procedure Woodward procedure Modified Woodward's procedure Green scapuloplasty Modified Green’s procedure Mears procedure
- 31. Putti’s procedure: detachmentof 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
- 32. Shrock modified Putti’sprocedure: subperiosteal dissection of the musculature and adding an osteotomy of the supraspinous scapular region and the acromial base to facilitate scapular descent
- 33. Green scapuloplasty: resectionof 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
- 34. Trapezius muscle disinrectionstep 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
- 35. supraspinatus fossa boneresection, 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
- 36. 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
- 37. Woodward procedure: Transferof 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.
- 38. 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
- 39. Prone position Preparation of parts done till occiput MODIFIED WOODWARD’S PROCEDURE
- 40. MODIFIED WOODWARD’S PROCEDURE Midline vertical incision
- 41. MODIFIED WOODWARD’S PROCEDURE Detachment of attachment of trapezius and rhomboids from spinous processes
- 42. MODIFIED WOODWARD’S PROCEDURE Release of omovertebral band Excision of superomedial angle of scapula
- 43. MODIFIED WOODWARD’S PROCEDURE Relocation of scapula to new position
- 44. MODIFIED WOODWARD’S PROCEDURE Suturing of trapezius to inferior Spinous processes
- 45. Closure inLayers MODIFIED WOODWARD’S PROCEDURE
- 46. Mears procedure Ina 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.
- 47. The shaded regionrepresents the area to be osteotomized (A – Reflected trapezius; B – Rhomboids; C – Levator scapulae; T – The detached triceps)
- 48. Int J ShoulderSurg. 2011 Jan-Mar; 5(1): 1–8. doi: 10.4103/0973-6042.80459
- 49. Postoperative complications Wingingof 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.
- 50. 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
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