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Author: Amulya K Saxena, MD, Attending Pediatric Surgeon, Department of Pediatric Surgery, Medical University of Graz, Austria

Amulya K Saxena is a member of the following medical societies: European Pediatric Surgeons Association, German Society of Pediatric Surgery, German Society of Surgery, and International Pediatric Endosurgery Group

Editors: Diana Farmer, MD, Associate Professor, Departments of Clinical Surgery, Pediatrics, Obstetrics, Gynecology and Reproductive Services, Division of Pediatric Surgery and the Fetal Treatment Center, University of California at San Francisco; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Nicholas A Shorter, MD, Professor of Clinical Surgery and Clinical Pediatrics, State University of New York-Downstate University; Division Chief, Department of Surgery, Division of Pediatric Surgery, State University of New York-Downstate Medical Center; H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina; Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Division of Pediatric Surgery, Department of Surgery, Children's Memorial Hospital of Chicago

Author and Editor Disclosure

Synonyms and related keywords: torticollis, congenital wryneck, unilateral sternocleidomastoid muscle tightness, sternomastoid tumor, congenital muscular torticollis, sternomastoid fibrosis, progressive facial hemihypoplasia, hematomas, plagiocephaly, facial hypoplasia

History of the Procedure

The earliest description of torticollis dates back to writings from the ancient Greek civilization. According to Plutarch, Alexander the Great may have had torticollis.

Problem

Torticollis is a result of unilateral tightness and shortening of one sternocleidomastoid muscle. A visible or sometimes palpable swelling, often referred to as a sternomastoid tumor, appears in a part of the muscle in infants aged 2-3 weeks. It often persists until they are aged 1 year. It is rarely bilateral and may be seen in older children in whom the mass was not previously identified.

Frequency

Torticollis occurs in 0.4% of all births.

Etiology

The etiology is incompletely understood, although several theories have been postulated.1, 2 Reports on the familial transmission of congenital muscular torticollis have been few. An idiopathic intrauterine embryopathy or the intrauterine development of sternocleidomastoid compartment syndrome may be responsible for the sternomastoid fibrosis.

Pathophysiology

An end-arterial branch of the superior thyroid artery supplies the middle part of the sternocleidomastoid muscle. Obliteration of this end artery may be responsible for the development of muscle fibrosis. As an alternative, primary trauma that temporarily and acutely obstructs the veins may lead to intravascular clotting in the obstructed venous tree. In infants, this clotting is evidenced by the development of a sternocleidomastoid mass, which eventually disappears and is replaced by fibrous tissue.

Clinical

The mass is generally 1-3 cm in diameter. It is a painless swelling in the substance of the sternocleidomastoid muscle and develops in neonates aged 2-3 weeks. In infants, the tumor is hard, and the patient's head is tilted and flexed to the side of the fibrosis. However, in older children, the tumor is less discrete than it is in younger children, and the sternocleidomastoid muscle appears thickened and foreshortened along its entire length. This thickening restricts rotation and lateral flexion of the neck.

Older children compensate for the head tilt by elevating their shoulder to maintain a horizontal plane of vision. The head tilting is further compensated by twisting the neck and back, if required, to maintain a straight line of sight. These compensatory mechanisms do not occur in infants, who do not need to maintain a horizontal plane of vision until they stand up. Also, in older patients, muscular spasms play a role or accompany torticollis.



Management for torticollis is primarily nonoperative, generally consisting of parental physiotherapy.

Rare indications for surgical management include persistent sternocleidomastoid contracture limiting head movement, persistent sternocleidomastoid contracture accompanied by progressive facial hemihypoplasia, and torticollis in children older than 12 months.



Surgical management of congenital muscular torticollis is generally avoided until the child is aged at least 1 year,3 until conservative methods (eg, physiotherapy) are unsuccessful, and until other differential diagnoses are excluded.



Lab Studies

  • No specific laboratory blood tests are required in the workup of patients with torticollis.

Imaging Studies

  • Although the diagnosis is easily made upon physical examination by an experienced clinician, ultrasonography is the most commonly ordered test.4, 5

Diagnostic Procedures

  • Clinical examination
    • The entire length of the muscle must be palpated to determine if fibrosis or an area of fibrosis is present along the entire length of the muscle.
    • The anterior border of the muscle must be palpated. It generally stands out as a tight band. This may be difficult to detect in small infants because the neck is relatively short.
    • Alternative differential diagnoses must be considered if the muscle is neither short nor prominent.
  • Differential diagnosis
    • Abnormal position in utero
    • Cervical hemivertebrae
    • Cervical lymphadenitis
    • Cervical abscess
    • Retropharyngeal abscess
    • Ocular muscle torticollis
    • Tumors of the posterior fossa
    • Atlantooccipital subluxation
    • Sandifer syndrome
    • Postural abnormalities

Histologic Findings

Histopathologic findings include fibrous replacement of skeletal muscle fibers that undergo atrophy. The degree of fibrosis and its extent or distribution may vary. Even in neonates, the fibrous tissue is mature. This finding indicates that the disease began before birth.



Medical therapy

Medical therapy involves conservative treatment.6 Sternocleidomastoid fibrosis spontaneously resolves in the vast majority of infants. Physiotherapy may be recommended; however, no evidence shows that this alters the course of the condition.

Botulinum toxin type A has been injected into the sternocleidomastoid muscle for the treatment of congenital muscular torticollis in pediatric and adult patients.7 However, modest benefits with improved range of motion has been observed in very few patients.

Only about 4-5% of patients are surgically treated, generally after the age of 1 year.

Surgical therapy

Surgery is performed with the patient under general anesthesia. A 3- to 4-cm transverse skin incision is made about 1 cm over the sternal and clavicular origins of the affected muscle. The platysma is carefully divided along the line of incision to avoid injury to the external jugular vein. The 2 heads of the sternocleidomastoid muscle are dissected free. The muscle is divided using diathermy to prevent bleeding. The platysma is then sutured with absorbable 4-0 skin suture, and the skin is closed with continuous 4-0 nonabsorbable skin suture.



Hematomas may develop because of inadequate hemostasis during surgery. Incomplete division may cause the condition to persist.



Recurrent torticollis after surgery is rare, with a rate of less than 3%. Secondary effects of untreated torticollis include plagiocephaly, facial hypoplasia, and musculoskeletal effects.

  • Plagiocephaly is an asymmetric skull deformity in infants that is caused by the flattening of one occiput that leads to the secondary flattening of the contralateral forehead. After the torticollis resolves, the plagiocephaly resolves; however, several years may pass before it disappears.
  • Facial hypoplasia is inhibition in the growth of the mandible and maxilla due to muscle inactivity. Clinically significant facial hemihypoplasia develops over 8 months; however, it is obvious in patients at the age of 2-3 years. Facial hypoplasia improves as the child grows, after the torticollis resolves.
  • Musculoskeletal effects include the compensatory ipsilateral elevation of the shoulder, as well as cervical and thoracic scoliosis. Wasting of additional muscles in the neck may be present due to sternocleidomastoid inactivity.



Follow-up should be continued until the torticollis resolves completely, until head and neck movement normalize, and until cervical and thoracic scoliosis is resolved in older children.



Media file 1:  Appearance of torticollis as a result of sternomastoid fibrosis in a young child.
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Media type:  Photo

Media file 2:  Surgical view of sternomastoid fibrosis shows the thyroid gland (1), the inferior thyroid artery (2), fibrosis of the sternal part of the sternocleidomastoid muscle (3), the brachiocephalic trunk (4), and the normal clavicular part of the sternocleidomastoid muscle (5).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo



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Torticollis excerpt

Article Last Updated: Feb 15, 2008