Sprengel deformity

Presented by: Dr. Joydeep Mallik

GANGA HOSPITAL

COIMBATORE

Definition:

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

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.

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%

Distribution

Unilateral(common)

Bilateral(rare)

10%

Distribution

Unilateral(common)

Bilateral(rare)

10%

Genetics: The condition is sporadic.

Rarely, autosomal dominant pattern of inheritance.

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.

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 serratusanterior muscle may be weak, leading to winging of the scapula. Other muscles, such as the pectoralismajor, latissimus dorsi, or the sternocleidomastoid may be hypoplastic and similarly involved.

SCAPULA:

Dysplastic.

High up than normal.

Smaller in the vertical plane and larger horizontally.

Inferior angle is rotated medially, causing the glenoidto face inferiorly.

Convexity of the upper (supraspinous) portion of the scapula is increased and curvature of the clavicularshaft is decreased, forming a narrower scapuloclavicularspace, may contribute to brachial plexus compression postoperatively.

Associated anomalies

Spine

Spinabifida

KlippelFeil

anomaly

Hemi-vertebra

Scoliosis

Ribs

Cervical rib

Fused ribs

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.

Klippel-Feil syndrome and Sprengel’s deformity

Congenital fusion of at least 2 cervical vertebrae with/without additional spinal/extraspinalmanifestations

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 neckLow hair lineRestriction of neck movement

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

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

DIAGNOSIS

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

Radiological criteria

• With short 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.

Omovertebralbone

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

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.

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

DIFFERENTIAL DIAGNOSIS Elevated scapula due to old paralysis.

Spinal accessory nerve injuryLong thoracic nerve injury

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.

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

SURGICAL TREATMENT

The surgical procedures 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.

SURGICAL OPTIONS

Putti’s procedure

Shrock modified Putti’s procedure

Woodward procedure

Modified Woodward's procedure

Green scapuloplasty

Modified Green’s procedure

Mears procedure

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

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

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

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

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

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

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.

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

Prone position

Preparation of parts done till occiput

MODIFIED WOODWARD’S PROCEDURE


Midline vertical incision


Detachment of attachment of trapezius and rhomboids from spinous processes


Release of omovertebral band

Excision of superomedial angle of scapula


Relocation of scapula to new position


Suturing of trapezius to inferior Spinous processes

Closure in Layers


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.

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

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

http://dx.doi.org/10.4103/0973-6042.80459

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.

PHYSIOTHERAPY AFTER SURGERY Gradual relaxed passive mobilization of the shoulder and

scapula.

Suitable pain relieving modality like TENS, IFT andhydrocollator packs may be used to induce relaxation.

Special attention is given to achieve early mobility of thescapula and the shoulder abduction and elevation.

Overall mobilization and strengthening of the shoulder girdlemuscles.

Emphasize maximum possible correction of the posture ofshoulder and maintain it.

5/6/201650

THANK YOU

Sprengel deformity

Health & Medicine