Congenital Muscular Torticollis

Surgical treatment of Congenital Muscular Torticollis has traditionally consisted of lengthening the sternocleidomastoid muscle by cutting it to different levels. Dr. Juan Monreal developed in 2012 an innovative technique, based on the principles of regenerative medicine, which allows a more effective and scar-free correction.

What is Congenital Muscular Torticollis?

Congenital Muscular Torticolis is a postural deformity of the neck that originates in an abnormal and progressive scarring of the sternocleidomastoid muscle of one side of the neck. As a result of this fibrosis, the aforementioned muscle suffers different degrees of atrophy and retraction that generate asymmetries, malpositions of the neck and face and motion limitations. Congenital Muscular Torticollis is the third most common congenital malformation after hip dysplasia and clubfoot.

What Causes Congenital Muscular Torticollis?

Hippocrates described postural neck problems more than 2000 years ago, but although much has been written about the disorder since first descriptions, its exact cause remains unknown. Historically, two theories have been proposed:

• The “intrauterine” theory attributes the unilateral shortening of the sternocleidomastoid muscle to an abnormal fetal position.
• The theory of “birth trauma” suggests that the muscle is damaged during a difficult birth, which causes bleeding in the muscle and ultimately fibrosis and contracture. Regarding this last hypothesis, several studies have shown a higher incidence of problematic deliveries in patients with CMT.

Types of Congenital Muscular Torticollis

Three basic presentations of the CMT have been defined:

• Postural: The newborn shows a postural deficit without tension in the muscle or restriction of the passive movement of the neck.
• Muscular: Characterized by the presence of tension in the sternocleidomastoid muscle and a limitation of the passive movement of the neck.
• Mass in the sternocleidomastoid (the most frequent): the sternocleidomastoid muscle is thickened and has a restricted range of active and passive range of motion. The mass is usually palpated inside the muscle and sometimes is visible.

Diagnosis.

This pathological condition is diagnosed at birth or soon after with one of the following signs:

• Malposition of newborn’s neck and face. Progressive fibrosis inside the muscle causes its retraction and consequently the tilting of the head towards the affected side and the limitation of neck movements.
• Sometimes it is associated with a palpable lump or mass inside the affected muscle.
• Although a physical exam is usually enough to have a correct diagnosis, ultrasound can be used as an adjunct diagnostic tool to reveal internal anomalies in the affected muscle. It is not usually necessary to perform other types of imaging studies such as CT Scan or MRI for early diagnosis. In view of surgical planning, we do request MRI from all patients.
• If not treated, different degrees of asymmetry usually appear in the bone structures of the face and skull.
• If not diagnosed or treated early during the first three months of age, neck deformity becomes increasingly noticeable.

Prognosis.

In very few exceptional cases, spontaneous correction of the neck of the newborn with CMT, during the first weeks after birth, has been documented. The diagnosis and treatment by physiotherapy should be performed as soon as possible. Specific physiotherapy programs, performed during the first months of life (especially during the first 3 months) usually provide complete corrections in more than 90% of cases.

In the event that diagnosis or treatment is delayed more than three months, success rate drops to 60-70%. When it is delayed beyond the first year of life, physiotherapy programs do not usually have a significant success rate and surgical treatment begins to be considered.

Patients with neglected or previous failed attempts at CMT correction usually develop a more pronounced postural neck deformity over the following years, usually accompanied by abnormal shoulder malposition on the affected side. Shoulder elevation is an additional postural anomaly performed relieve the tension caused by the contracture of sternomastoid muscle. It is common that facial asymmetries and craniofacial deformities of different degrees and locations develop over time, usually affecting the forehead, cheekbone and orbit of the affected side.

In adult patients with neglected CMT or with previous failed attempts at surgical correction, cervical spine deformities (scoliosis) of different degrees may develop.

Available treatments.

Congenital Muscular Torticollis can be treated in two different ways depending on the time of diagnosis:

Non surgical treatments.

• • Early physiotherapy, which is curative in the vast majority of cases (more than 90%) if started within the first three months of life. After the first year of life, physiotherapy is no longer as effective and especially after the fourth year of life it usually has a very low curative efficacy.
  • From these ages, and especially in adulthood, the first line of treatment is surgery since physiotherapy does not usually provide any successful improvement. Success rates of traditional surgical techniques are very varied depending on the technique used and surgeon’s experience. In some countries, adult patients are rejected as candidates for surgical treatment due to the high rate of relapses and low success rate.
  • Botulin Toxin based treatments aim to relieve neck tension, but have no effect on long-term behavior of muscular structure and deformity.

Surgical treatments.

Except for endoscopic assisted techniques muscle cutting (myotomies) must be performed through skin incisions located above the clavicle and/or behind the ear. These techniques are intended to release the tension generated by muscle contraction-fibrosis thus correcting posture of the neck and face (this is much like cutting a tensioning rope). Surgical techniques commonly used to treat Congenital Muscular Torticollis are based in:

• Endoscopic assisted muscle release in its central portion or in its clavicular – sternal  junction.
• Open muscle release from its clavicle-sternum junction and/or area of insertion located behind the ear (mastoid process). They are technically known as Unipolar or Bipolar release.
• A simultaneous surgical elongation (known as Z_plasty) can be performed in addition of open bipolar or monopolar releases. The purpose is to avoid the loss of muscle contours.
• In very severe cases, the complete removal of the affected muscle has been described.

To perform unipolar or bipolar release techniques, surgeon will make skin incisions located above the clavicle near the sternum, and/or behind the ear. These incisions usually have a size between 3 and 5 cm. The endoscopic technique uses smaller incisions (± 1 cm). Regardless of the skin incision, muscle release (cut through the muscle also known as «myotomy») must be full thickness near the clavicle – sternum junction, behind the ear or in the center of the muscle.

These surgical techniques aims at improve the tension caused by muscle retraction and thus correct neck and face posture. Any of these surgical procedures requires, during the postoperative period, the use of cervical collars or other types of orthosis, in addition to an intensive rehabilitation program. These supplementary measures are intended to prevent the cuts in the muscle from reattaching, which would cause recurrence of the deformity.