Congenital torticollis: treatment approaches
Conservative therapy yields the best results in the first and second years of a child's life. If pathological changes in the sternocleidomastoid muscle (m. sternocleidomastoideus) are pronounced, non-surgical methods can be continued up to three years of age.
With progressive cranial and facial asymmetry, as well as significant muscle hypoplasia, surgical intervention is indicated (usually in children older than three years). The basic surgical technique is Zatepin's operation. It includes:
- Transection of the muscle heads at the site of attachment to the clavicle;
- Dissection of the superficial cervical fascia;
- Resection of the lower portions of both heads by 1.5–2.0 cm (creation of a diastasis to prevent recurrence).
An alternative method is complete removal of the affected muscle followed by plasty with a preserved tendon or a Dacron (lavsan) tape. In the postoperative period, a plaster thoracocranial bandage is mandatory to keep the head in a state of hypercorrection.
Sprengel's deformity: anatomy and pathogenesiss
High scapular standing is a consequence of the action of a teratogenic factor in the early stages of intrauterine organ differentiation. The pathology is often combined with spinal curvature, torticollis, and the presence of additional cervical ribs.
In this malformation, the scapula is reduced in size, elevated, and rotated around the sagittal axis. It literally "sits" on the 1st rib and becomes wedged between it and the clavicle, which mechanically blocks the elevation of the arm above the horizontal level.
The muscular apparatus develops abnormally: the trapezius (m. trapezius) and rhomboid (m. rhomboideus) muscles, and sometimes the serratus anterior (m. serratus anterior), may undergo cicatricial changes, atrophy, or be completely absent. A characteristic bone anomaly is the appearance of an omovertebral bone (usually triangular in shape), which connects the medial border of the scapula with the transverse process of a cervical or thoracic vertebra.
Classification of Sprengel's deformity
Depending on the severity of muscle involvement and the presence of additional bone formations, two forms are distinguished:
- Muscular form. Has three degrees of severity:
- Mild: scapular displacement by 1–2 cm, upper limb function is barely affected (elevation is limited within 10°).
- Moderate: scapula is 2–5 cm higher than normal, the medial border forms an angle of 10–30° with the spine. Arm elevation is possible up to 120–150°, the shoulder girdle is visually shortened.
- Severe: displacement by 6–12 cm (the border can reach the earlobe), pronounced facial asymmetry. Mobility of the inferior angle of the scapula drops to 1–3 cm, and shoulder abduction is limited to 80–110°.
- Osseous form. Occurs on both the left and right sides. Always classified as severe. It is distinguished by a wider scapula (displacement of 6–12 cm), gross functional impairments, and the obligatory presence of an omovertebral bone.
Diagnostics and surgical treatment
The diagnosis is confirmed by radiography of the shoulder girdle (showing rib and spinal deformities and the omovertebral bone) and CT (for detailed visualization of joints and bone anomalies). EMG is mandatory to assess the degree of cicatricial degeneration and muscle atrophy.
Conservative treatment of moderate and severe forms yields no results. Surgical intervention is indicated, which is divided into three groups:
- Lowering the scapula without bone manipulations (mild muscular form);
- Lowering the outer part of the scapula (moderate and some severe muscular forms);
- Partial or subtotal resection of the scapula (severe muscular and osseous forms).
The optimal age for surgery is from 4 to 6 years. Operating earlier is technically difficult due to the immaturity of structures, while delayed intervention threatens the development of secondary deformities and a high risk of brachial plexus palsy.
Briefly: answers to topical questions
What are the main causes of Sprengel's deformity?
The main cause of Sprengel's deformity is exposure to a teratogenic factor in the very early period of intrauterine development. This pathological effect occurs at the stage of organ differentiation.
High scapular standing is merely an external manifestation of a pathology that simultaneously affects the shoulder girdle, spine, and ribs. As a result, concomitant anomalies form (spinal curvatures, cervical ribs, torticollis, internal organ malformations), as well as changes in the muscular apparatus:
- Rhomboid muscle (m. rhomboideus) — frequently affected, may be absent or atrophied.
- Trapezius muscle (m. trapezius) — also prone to frequent anomalies of development and topography.
- Serratus anterior muscle (m. serratus anterior) — affected less frequently.
To remember the optimal age for surgery in Sprengel's deformity, think of school: "Before school (4–6 years), lower the scapula to avoid plexus palsy in high school."
Class and exam questions
Why should Sprengel's deformity not be operated on in adolescents?
In older children, secondary deformities of the shoulder girdle become established, and attempts to lower the scapula sharply increase the risk of postoperative complications, up to severe brachial plexus palsy.
What is the essence of Zatepin's operation for torticollis?
This is the transection of both heads of the sternocleidomastoid muscle and resection of their lower portions by 1.5–2.0 cm. Resection is necessary to create tissue diastasis and reliably prevent recurrence of the deformity.
Which muscle is least commonly affected in Sprengel's deformity?
Unlike the rhomboid and trapezius muscles, which are affected almost always, the serratus anterior muscle (m. serratus anterior) is involved in the pathological process much less frequently.
What else to study on this topic
- Evaluation of EMG results in muscular and osseous forms of Sprengel's deformity
- Technique of omovertebral bone resection and lowering of the outer part of the scapula
- Congenital dysplastic chest wall developmental defects (etiology)
- Rules for applying and managing patients in a plaster thoracocranial bandage
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