Sprengel Deformity Correction

Surgical Indications

Absolute Indications

• Cavendish grade 3 or 4 deformity with a visible, cosmetically unacceptable lump and limited shoulder abduction
• Symptomatic omovertebral bone restricting abduction or causing pain
• Significant functional limitation of shoulder abduction that interferes with daily activities

Relative Indications

• Cavendish grade 2 deformity where the patient and family find the visible neck lump cosmetically unacceptable after counselling about the scar
• Progressive loss of abduction in a growing child within the ideal age window
• Restricted abduction with a confirmed bony block (prominent supraspinous fossa or omovertebral bar)

Contraindications

Absolute:

• A stiff, pain-free shoulder in a near-mature adolescent with a mild (grade 1) deformity, the risks of surgery exceed the benefit
• An uncorrected tethered cord or other major undiagnosed spinal anomaly until neurosurgical evaluation is complete
• A medically unfit child in whom the associated cardiac or renal anomaly is unoptimised

Relative:

• Age greater than about 8 years, the soft tissues and brachial plexus tolerate descent poorly and results are less predictable
• Severe Klippel-Feil with a very short, stiff neck, the increased anaesthetic and neurological risk must be weighed against the modest expected gain
• A very mild grade 1 deformity found incidentally, observation is appropriate

Goals of Surgery

Surgery serves two purposes and both must be discussed with the family:

• Cosmetic: to lower the visible lump in the web of the neck and improve the symmetry of the shoulder girdle
• Functional: to improve shoulder abduction by removing the bony block (omovertebral bone and prominent supraspinous portion) and by repositioning the scapula so the glenohumeral and scapulothoracic rhythm can work

Cavendish Classification and Indication

Timing of Surgery

• Ideal window: between three and eight years of age
• Why early: the scapula and surrounding soft tissues are still mobile, allowing a larger and safer descent, and neuromuscular adaptation is better
• Why not older: beyond about eight years the soft tissues stiffen, the brachial plexus tolerates stretch poorly, and both the neurological risk and the recurrence rate rise
• Late presentation: counsel the family honestly that correction in the older child is less predictable, and that surgery may be withheld if the shoulder is already stiff and the deformity mild

Evidence for Surgery

The Woodward Procedure

The Woodward procedure, described in 1961, relocates the scapula by detaching the origins of the trapezius and rhomboids from the spinous processes and reattaching the muscle mass more distally. It is the most widely performed operation for Sprengel deformity today. Reported outcomes consistently show gains in shoulder abduction in the order of 30 to 40 degrees and a meaningful cosmetic improvement in the majority of grade 3 and 4 deformities operated within the ideal age window.

The Green Procedure

The Green procedure, described in 1957, releases the periscapular muscles extraperiosteally from the scapula (levator scapulae, rhomboids and the omovertebral connection) and repositions the freed scapula, holding it distally with skeletal traction to the opposite iliac crest or ribs. It is less commonly performed than the Woodward but remains a valid alternative, and the two procedures share the key steps of omovertebral excision and supraspinous resection.

What the Series Show

• Improvement in active shoulder abduction is the most reproducible functional gain, typically around 30 to 40 degrees
• Cosmetic correction is generally good for grades 3 and 4 but rarely completely normalises the contour
• Results are best in younger children with mobile soft tissues
• The brachial plexus traction injury is the most feared complication and is over-represented in older children and in over-relocation

Embryology of Scapular Descent

• The scapula forms in the mesenchyme of the upper limb bud at a cervical (around C4 to C5) level
• During the first trimester it descends caudally to its final thoracic position opposite the second to seventh ribs, reaching this position by about the eighth week of gestation
• Sprengel deformity results from a failure of this descent, so the scapula remains high, hypoplastic and adducted (rotated so its glenoid faces inferiorly and its inferior angle faces the spine)
• An omovertebral connection is thought to tether the scapula and prevent descent in around one-third of cases

The Omovertebral Bone

• Present in around one-third of Sprengel deformities
• A bone, cartilage, or fibrous band running from the superomedial border of the scapula to the spinous processes, transverse processes or laminae of the lower cervical vertebrae (usually C4 to C7)
• Tethers the scapula and limits abduction, and must be excised at operation
• Lies close to the vertebral canal, so dissection stays extraperiosteal and away from the spinal canal

Periscapular Muscles — Origins and Innervation

Nerves at Risk

Spinal Accessory Nerve (CN XI)

• Enters the posterior triangle, then runs on the deep (anterior) surface of the trapezius, innervating it segmentally from superior to inferior
• Endangered when the trapezius is detached from the spinous processes and reflected laterally
• Division denervates the trapezius, producing shoulder droop, a winged scapula and weak overhead elevation
• Identify and protect it throughout the dissection

Dorsal Scapular Nerve

• Arises from the C5 root, pierces the scalenus medius and runs with the deep branch of the transverse cervical (dorsal scapular) artery along the medial border of the scapula, deep to the rhomboids
• Supplies the rhomboids; injury weakens scapular retraction

Long Thoracic Nerve

• Arises from C5, C6 and C7 roots and descends on the superficial surface of serratus anterior
• Injury causes a winged scapula, keep the dissection off the chest wall over serratus anterior

The Brachial Plexus and Thoracic Outlet

• As the scapula is drawn inferiorly the clavicle and entire shoulder girdle descend with it, which lengthens the path of the brachial plexus through the thoracic outlet
• The plexus, particularly the upper trunk, is vulnerable to a traction injury
• A clavicular osteotomy or morcellation shortens the rigid bony ring of the thoracic outlet and decompresses the plexus during descent
• Intra-operative neuromonitoring (somatosensory or motor evoked potentials) is used to detect impending traction injury before it becomes permanent

The Prominent Supraspinous Portion

• In many Sprengel scapulae the supraspinous portion of the scapula (the bone above the scapular notch) is hooked or prominent and projects above the superior border
• This bony prominence can impinge on the chest wall or the thorax and block abduction even after the omovertebral bone is removed
• Resection of this prominence (subperiosteally, protecting the suprascapular nerve and the supraspinatus tendon) removes the bony block and improves the gain in abduction

Positioning and Preparation

Patient position: Prone, with longitudinal rolls under the chest and pelvis and the head supported in a headrest. The affected arm is draped free so the scapula and shoulder can be manipulated during the procedure. A lateral decubitus position is an acceptable alternative.

Anaesthesia: General anaesthesia with a secure airway. Klippel-Feil and cervical anomalies make intubation difficult, anticipate this and plan the airway with the anaesthetist. Intra-operative neuromonitoring (somatosensory or motor evoked potentials) is strongly recommended to guard the brachial plexus.

Consent: Specifically counsel the family regarding the risk of brachial plexus traction injury (the most feared complication, transient or occasionally permanent), the posterior scar, incomplete correction or recurrence, winging of the scapula, infection and the need for a period of immobilisation afterwards. Set realistic expectations, the contour improves but rarely normalises.

Woodward Procedure — Step-by-Step

Step 1: Incision and Exposure

With the child prone, mark a long posterior midline incision from the spinous process of the lower cervical spine (around C4 or C5) down to the mid-thoracic spine (around T8), curving gently toward the elevated scapula. Incise the skin and subcutaneous tissue in line with the mark.

Raise skin and subcutaneous flaps laterally to expose the trapezius, the rhomboids and the elevated, hypoplastic scapula.

Step 2: Identify and Excise the Omovertebral Bone

Palpate the superomedial border of the scapula for the bony or cartilaginous omovertebral bar running to the cervical spine. Expose it and define its proximal and distal attachments. Excise it completely in an extraperiosteal fashion, freeing the scapula from its tether to the cervical spine.

Step 3: Detach the Trapezius and Rhomboid Origins (Extraperiosteal)

Working from the midline, detach the origins of the trapezius and then the rhomboids extraperiosteally from the spinous processes, creating a continuous musculotendinous sheet that can be relocated as a unit. Identify and protect the spinal accessory nerve as it runs on the deep surface of the trapezius during this elevation.

Step 4: Resect the Prominent Supraspinous Portion

With the muscle sheet freed, expose the superior border of the scapula. Resect the prominent or hooked supraspinous portion subperiosteally, removing the bony block to abduction while protecting the supraspinatus muscle and tendon and the suprascapular nerve.

Step 5: Clavicular Osteotomy or Morcellation (Older Child)

In the older child, perform a clavicular osteotomy or morcellation on the affected side. This shortens the rigid bony ring of the thoracic outlet and decompresses the brachial plexus as the scapula descends, substantially reducing the risk of a traction palsy. The younger child often does not need this step because the soft tissues are more yielding.

Step 6: Relocate and Reattach the Muscle Mass

Bring the freed scapula and the attached trapezius and rhomboid sheet distally to the level of the contralateral scapula, never lower. Reattach the muscle origins to the spinous processes more distally (for example two or three levels below their original position) using non-absorbable sutures through bone or fascia. Confirm with neuromonitoring that the plexus is not under tension.

Step 7: Closure

Achieve haemostasis and place a drain. Close the wound in layers over the relocated muscle mass with an absorbable deep layer and a skin closure that aims for the least conspicuous scar. Apply a well-padded dressing and immobilise the arm.

The Green Procedure — Summary

The Green procedure achieves the same goals by a different route. Through a similar posterior incision, the periscapular muscles (levator scapulae, rhomboids, and the omovertebral connection) are released extraperiosteally from the scapula rather than from the spinous processes. The freed scapula is then repositioned distally and held there with skeletal traction (a wire through the scapula connected to the opposite iliac crest or to a rib) for several weeks while the muscles heal in their new length. The omovertebral bone is excised and the prominent supraspinous portion is resected just as in the Woodward procedure. It is less commonly performed today but remains a valid option, particularly when the muscle origins are unfavourable for the Woodward relocation.

Post-operative Protocol

Immobilisation Phase (Weeks 0 to 4)

• Immobilisation: A sling or shoulder immobiliser for about four to six weeks to allow the relocated muscle mass to heal in its new position
• Positioning: Keep the arm supported and avoid active abduction or elevation in the early phase
• Analgesia: Paracetamol and an NSAID, with short-course opioids as needed
• Wound care: Inspect at 48 hours and at two weeks; remove the drain when output is minimal
• Neurological review: Examine the arm for brachial plexus function immediately on recovery and at each visit

Rehabilitation Phase (Weeks 4 to 12)

• Begin gentle active and active-assisted range of motion once the muscle mass has healed, focusing on abduction and forward flexion
• Progressive strengthening of the periscapular muscles (trapezius, serratus anterior, rhomboids)
• Scar management: silicone gel and massage from two to three weeks once healed
• Monitor: any new or persistent neurological deficit warrants urgent nerve conduction studies

Return to Function

• School: Within one to two weeks in the sling, avoiding physical education and contact activity
• Sport and full activity: Usually from three months, once range and strength are restored
• Final outcome: Abduction generally continues to improve for six to twelve months

Outcomes

Functional Outcome

• Improvement in active shoulder abduction is the most reproducible gain, typically around 30 to 40 degrees
• Removing the omovertebral bone and the prominent supraspinous portion both contribute to the gain in abduction
• Results are best in younger children with mobile soft tissues

Cosmetic Outcome

• Most grade 3 and 4 deformities show a meaningful cosmetic improvement
• The contour rarely normalises completely, and the family should be told this before surgery
• The posterior scar is a permanent trade-off and must be discussed during consent

Special Case: The Older Child (Greater Than 8 Years)

Why the Older Child Is Different

• The soft tissues and brachial plexus are less yielding, so descent of the scapula is harder and more dangerous
• Both the recurrence rate and the risk of a brachial plexus traction injury rise with age
• The scapula itself is more deformed and the muscle mass more fibrotic

Management Principles

• Counsel the family honestly that the result is less predictable
• Make a clavicular osteotomy or morcellation a routine part of the procedure to decompress the plexus
• Use intra-operative neuromonitoring throughout
• Do not over-relocate, a partial improvement is safer than a full forced descent
• In a stiff, pain-free adolescent with a mild deformity, observation is often the better choice

Special Case: Bilateral and Syndromic Sprengel

Bilateral Deformity

• May be staged or simultaneous depending on anaesthetic fitness and surgeon preference
• Each side is treated on its own merits using the Cavendish grade and the functional deficit
• Plan the second side once the first has healed and the abduction gain is clear

Klippel-Feil and Associated Anomalies

• A difficult airway is anticipated, plan intubation with the anaesthetist
• Screen for and address a tethered cord before elective scapular surgery
• Coexisting congenital scoliosis, renal and cardiac anomalies influence peri-operative care
• The whole child is managed, not just the scapula