May 30, 2024

Sprengel Deformity is called Undescended scapulae or Congenitally elevated scapulae which generally descends within 12 weeks of the intraoperative period.

Congenitally elevated scapulae

Most common congenital anomaly of the shoulder in children

Associated with :

  • Klippel-Feil Syndrome (1/3 cases)
  • Kidney disease
  • Scoliosis
  • Diastematomyelia

Etiopathogenesis

The exact etiology is unknown

The defect is known to occur in early embryonic life/ most particularly at the development of the C-Spine and Upper limb

At 3 months:- Mesenchymal tissue takes form as a cervical spine and at the same level upper limb bud develop then descent to the level of the thorax: permanently high shoulder girdle

Affected organs:

  • Deformities in occiput
  • Cervical spine
  • Upper thoracic spine
  • Ribs
  • Surrounding muscles

Features of Sprengel’s shoulder

Sprengel Deformity

Smaller in vertical diameter

Broad

Arches forward and fit into the superior thoracic cage

The inferior angle is near the spine

The vertebral border is convex

Occasionally it reaches to occiput of the skull

From a superior angle: a sheet/band-like structure is attached to the transverse process (Omovertrbral bar)

Muscles around it are either normal or underdeveloped

  • Rotator cuff muscles
  • Trapezius
  • Levator scapulae
  • Serratus anterior

Classification of Sprengel Deformity

Cavendish (1972)

Grade Features
Grade 1Very MildShoulder joint at the levelThe deformity is invisible when the patient is dressed
Grade 2MildShoulder joint at the levelThe deformity is visible as a lump in a web of the neck when the patient is dressed
Grade 3ModerateShoulder joint elevated 2-5 cmDeformity is visible
Grade 4SevereShoulder joint much elevatedThe superior angle of scapulae is near occiput with or without neck webbing or brevicollis (Short neck)

Rigault

GradeLevel of scapulae
Grade 1Between T2 – T4
Grade 2Between C5 – T2
Grade 3Above C5

Treatment Options for Sprengel’s Shoulder

Surgery is the mainstay of treatment

Result depends upon

  • Age of the child
  • Severity

Operation should be performed between 3 – 6 years ideally. Inferior outcomes are seen if operated after 6 years

Basic Steps

  • Resect the omovertebral bar
  • Excise superolateral margin of the scapulae
  • Excise attachment of Scapular muscle
  • Derotate and lower scapular body
  • The inferior angle of scapulae is usually fixed in the lower position (transverse process or ribs)

Clavicular osteotomy is often needed to avoid brachial plexus injury caused by stretch

Common Methods

  1. Transplantation of Scapulae
    • Woodward’s Procedure (erase muscle from origin)
    • Green’s Procedure (erase muscle from the scapular border)
  2. Mear’s Procedure (Partial Scapulectomy)
  3. Shrock’s Procedure
  4. Vertical osteotomy of scapulae

Summary of treatment of Sprengel Deformity

To summarize the treatment of Sprengel Deformity:

See also: Congenital anomalies of hand (Classification)

See also: Shoulder Examination