The outcomes of congenital torticollis delayed surgery in older children: A case series

Abstract

Introduction and importance

Congenital muscular torticollis is a benign affection defined as a contracture or fibrosis of the sternocleidomastoid muscle, causing ipsilateral inclination and contralateral rotation of the face and chin. The management is multidisciplinary, usually surgical and should start at very early age in infants to secure better results. Thus, the purpose of our study is to report the outcomes of delayed surgery performed in older children above 5 years old with late diagnosis.

Case presentation

We report the cases of 4 patients aged between 5 and 11 years old and followed in our department for congenital torticollis. They were all born by vaginal delivery with vacuum extraction in two cases of breech presentation. Even if the condition is present at birth, most parents were not bothered by the cervical vicious neck position of their children until a later age, delaying the diagnosis. The clinical examination found a flexed head position on the right side in all cases, with contralateral rotation. Regarding the age, we proposed surgical treatment immediately for two of them, while two were sent to our department after multiple ineffective physiotherapy sessions. The surgery consisted on right distal tenotomy and a cervical collar was prescribed next to physiotherapy. They all had successful results with correction of head position and improvement of cervical range motion.

Clinical discussion

Children treated early with active and well monitored rehabilitation, recover completely and regain normal head position and mobility rapidly. In older children, above 5 years, they are more likely to develop sequels such as asymmetry of facial movement.

Conclusion

Delayed diagnosis after the age of 5 years old still can be managed successfully with a correction of the head position and rotation motion. However, in these older children, physiotherapy alone cannot be effective and should be associated to surgery. Moreover, once the diagnosis is done, surgical treatment should be performed to avoid wasting more time.

Highlights

• •Congenital torticollis is the 3rd most common congenital orthopedic malformation next to hip dysplasia and calcanean foot.
• •In children older than 5 years, physiotherapy alone as a treatment for congenital torticollis cannot be effective.
• •Above 5 years, children with congenital torticollis are more likely to develop sequels such as asymmetry of facial movement.
• •Thanks to surgery, even after the age of 5 years, congenital torticollis can be managed successfully with a correction of the head position and rotation motion.

1. Introduction

Congenital muscular torticollis is a benign affection defined as a contracture or fibrosis of one sternocleidomastoid (SCM) muscle, causing ipsilateral inclination and contralateral rotation of the face and chin. It is the third most common congenital orthopaedic deformity [1].

The management is multidisciplinary, usually surgical and should start at very early age in infants to secure better results. Thus, the purpose of our study is to report the outcomes of delayed surgery performed in older children above 5 years old with late diagnosis.

This case series has been reported in line with the Process Checklist 2020 criteria [2].

2. Presentation of the case series

We present the cases prospective in design of 4 patients followed in our head and neck surgery department for congenital torticollis. They were all aged between 5 and 11 years old, 3 boys and a girl, with no similar cases in the siblings. Their mothers' pregnancies were well followed up and they all gave birth by vaginal delivery with vacuum extraction in two cases of breech presentation. No particular post-partum complication was noted. No signs of obstetrical trauma were reported such as brachial plexus lesion or shoulder dislocation.

Even if the condition is present at birth, most parents were not bothered by the cervical vicious neck position of their children until a later age, when they start getting to school. The age of diagnosis varies between 5 and 10 years old. The clinical examination found a flexed head position on the right side in all cases, with contralateral rotation, rigidity in lateral head movements, vicious drooping position of the right shoulder and asymmetric limb movements. The ipsilateral sternocleidomastoid (SCM) muscle was contracted and facial growth was symmetrical.

Cervical ultrasound (US) realised in 3 patients showed thickness in the SCM muscle (Table 1).

Table 1.

Clinical presentation of the 4 patients.

	Age	Sex	Birth	Clinical diagnosis	Radiology
Patient 1	5	M	Vaginal delivery Vacuum extraction (Breech presentation)	Age of diagnosis: 5 Flexed right head position with contralateral rotation	Cervical US: thickness in the SCM of 3 mm
Patient 2	11	F	Vaginal delivery	Age of diagnosis: 8 Flexed right head position with contralateral rotation	Cervical US: thickness in the SCM of 4.7 mm
Patient 3	8	M	Vaginal delivery	Age of diagnosis: 6 Flexed right head position with contralateral rotation	Cervical US: SCM swelling
Patient 4	14	M	Vaginal delivery Vacuum extraction (Breech presentation)	Age of diagnosis: 10 Flexed right head position with contralateral rotation	–

Regarding the age, we proposed surgical treatment immediately for two of them, while two were sent to our department after multiple [8], [9], [10] ineffective physiotherapy sessions (there was no need to take measures before the surgery).

The surgery has been performed by ENT specialists and consisted on a right distal tenotomy under general anaesthesia. We released of the origin of the two heads of the SCM muscle from the clavicle and the sternum with a section of the contracted part to avoid recurrence (Fig. 1).

Fig. 1.

Surgical steps of right distal tenotomy with section of the contracted muscle parts.

Cervical collar was prescribed to wear daily for 3 months postoperative. The patients received physiotherapy based on motor rehabilitation. At 3 months follow up, major improvement of cervical range motion and head position was observed. At 1 year and 3 years follow up, the head was straight and the movements normal (Table 2) (Fig. 2, Fig. 3).

Table 2.

Surgical management and follow up of the 4 patients.

	Prior physiotherapy	Surgical procedure	Follow up
Patient 1	None	Age of surgery: 5 Right distal tenotomy	Cervical collar for 3 months 10 sessions of Physiotherapy 3 months follow up: Vertical raising of head position Improvement of cervical range motion
Patient 2	8 sessions (No results)	Age of surgery: 8 Right distal tenotomy	Cervical collar for 3 months 10 sessions of Physiotherapy 3 months follow up: Improvement of cervical range motion and vertical head position 3 years follow up: Straight head position Normal head movements
Patient 3	None	Age of surgery: 7 Right distal tenotomy	Cervical collar for 3 months 10 sessions of Physiotherapy 3 months follow up: Improvement of cervical range motion and vertical head position 1 year follow up: Straight head position Normal head movements
Patient 4	10 sessions (No results)	Age of surgery: 11 Right distal tenotomy	Cervical collar for 3 months 10 sessions of Physiotherapy 3 months follow up: Improvement of cervical range motion Vertical head position

Fig. 2.

Before (a) and after (b) surgery pictures in patients 1 and 2. The oblique eyebrows alignment shows the degree of head misplacement.

Fig. 3.

After surgery pictures in patients 3 and 4.

3. Discussion

The clinical term “torticollis” comes from the Latin words: tortum collum, which means “twisted neck”. Congenital muscular torticollis is a benign affection defined as a contracture or fibrosis of one sternocleidomastoid (SCM) muscle, causing ipsilateral inclination and contralateral rotation of the face and chin. And it is the third most common congenital orthopaedic deformity after hip dysplasia and calcaneovalgus feet, with a worldwide incidence rate between 0.3 % and 1.9 %. Slight male predominance (3:2) can be observed, and it is more common in the right side [1].

Congenital torticollis is usually linked to muscular origin (99 % of cases) and more rarely to rachidian one. The main mechanism still totally unclear, but seems related to traumatic baby delivery requiring the use of obstetrical instruments such as vacuum and forceps responsible for direct muscular trauma by disruption of the SCM, and foetus intrauterine malposition leading to compartment syndrome (ischaemia and oedema) and fibrosis of the SCM muscle causing its retraction and shortening. These two conditions favoured with breech or transverse presentation, primiparity, multiple pregnancy, macrosomia, oligohydramnios, cranial asymmetry and uterine malformations [3], [4].

Other causes are reported in the literature, as infectious myositis, primary myopathy of the SCM muscle and reflex muscle contraction due to dysfunction of the accessory nerve or C2-C3 spinal nerves [5], [6].

The diagnosis is clinical and easily made usually in the neonatal period or after birth [1].

It manifests as firm, regular tumefaction of the SCM that can disappear between the age of 2 and 6 months, responsible of flexed head position on one side, with contralateral rotation and rigidity in lateral head movements. It may be accompanied by characteristic vicious attitude of the shoulder permanently raised on the side opposite to the retraction. Occipital plagiocephaly presenting as flattening of the skull can be observed in 85 % of the cases. In case of doubt, ultrasound can confirm the diagnosis by showing echogenic thickening localized in the SCM muscle [7], [8].

After the age of walking, consequences of non-treated head position arise with the duration of congenital torticollis, in particular asymmetry of the height of the shoulders, facial scoliosis defined by hemi-atrophy of the face on the side opposite to the muscular damage and a retreat of the hemi-base of the skull on the injured side, global asymmetry of the trunk and/or limbs with lack of stability in the shoulder girdle (the child cannot free the upper limbs to play), and visual dysfunction with an important impact on the child quality of life [9], [10].

However, in some cases such as ours, the torticollis can be noticed at young age, but it doesn't get linked to pathological condition until the child grows and starts social intercourse, usually in school, in order for the parents to compare with other kids and realise that it's not going to correct itself spontaneously.

The early diagnosis determines the choice of the therapeutic approach, as well as the good post-treatment evolution. Children treated early with active and well monitored rehabilitation, recover completely and regain normal head position and mobility rapidly [11].

The mainstay of treatment is conservative management which include physiotherapy and family education. However, it is the treatment of choice in very young infants over a period of 3 months with multiple sessions per week. The protocol is based on active positioning program and passive stretching first performed by the physiotherapist, and then taught to the parents. It is considered effective for children with a rotation deficit of less than 6° at the age under 4.5 months [1], [12]. According to Lee et al. study, the duration of physiotherapy should depend on the severity of the associated fibrosis with an average duration of treatment for minor fibrosis of 3.2 months, 3.8 months for moderate fibrosis, and 5 months for severe fibrosis [13].

Past this age, next to physiotherapy, tubular torticollis orthosis should be put in place until the rotation deficit is fully recovered. Also, family involvement plays a role in occupational therapy, by positioning the child object of interest the opposite of his natural positioning to improve and correct his motor development (e.g., placing the bottle on the side of the torticollis during feeding, placing light and sound sources in such a way as to promote head rotation in the deficient sector) [14], [15]. In another hand, massage is used in many countries for the treatment of congenital torticollis with high patient compliance since it dilates capillaries, speeds up the circulation of blood and lymph and promotes the nutritional supply of the muscles which promotes their growth and development [12].

However, as in our case series, conservative treatment as an only treatment approach is no longer effective for children diagnosed after the age of 1 year, and even less past the age of 5 [11].

Surgical management is indicated after failure of 6 months of well-conducted physical therapy or for cosmetic reasons, only in children older than 1 year, and remains the safest way to minimize long-term consequences. The surgical procedure consists on tenotomy releasing the SCM attachments. The tenotomy can be unipolar, either distal consisting on releasing the SCM muscle from the clavicula and the sternum, or proximal, releasing the muscle from the mastoid process with a regard to the anatomical proximity of the facial nerve emergence; bipolar tenotomy with both proximal and distal release is usually indicated for significant retraction in older children.

There is much debate about determining which surgical procedure gives the best results with minimum complications; namely the lesion of the great auricular nerve, the spinal nerve, an important scar, recurrent muscle band formation and loss of neck contour [16].

The technique described by Ferkel et al. consists on a bipolar release with an inferior Z-plasty of the SCM tendon which offers an aesthetic advantage of preserving the muscle relief. The main risk still the lesion of the facial nerve since the infant mastoid is not yet pneumatized [17].

Thus, the procedure can be performed through a sky open approach; or an endoscopic approach which offers a better view of the operating field, ensuring a complete and precise release and a protection of the muscle from denervation by damage of the spinal nerve.

One of the endoscopic techniques described is the so-called Wry neck technique, which is based on a simultaneous subperiosteal lengthening of the sternocleidomastoid muscle at its mastoid insertion, and division of the inferior fibrotic bands. Given that the sternomastoid muscle is fixed at a lower level on the mastoid apophysis, the lengthening of the muscle is maintained, as the fibrosing and shortening tendency is minimal, which allows to maintain postoperative cervical mobility [18]. Also, a transaxillary endoscopic technique was described as safe and rapid with excellent access to the muscle with minimal scarring.

Physiotherapy and cervical collar worn at least 3 months are required postoperatively. The outcomes are generally good in terms of function in children operated on before the age of 5 years [1].

In older children, above 5 years, they are more likely to develop sequels such as asymmetry of facial movement. But, with no facial asymmetry, the surgical outcomes still good considering the correction of head position and mobility such as in our case series. After the age of 10, extensive fibrosis alters the surgical results since the majority of patients keeps obvious asymmetry of the face even if they increase their head mobility [19].

Indeed, in older patients, like reported in Chen et al. study on 18 patients (6–22 years old), all except 1 had a satisfactory range of motion of the neck and all except 2 showed improvement of the facial asymmetry after bipolar tenotomy. But the follow-up radiographs showed improvement of the tilt of the odontoid process, but the asymmetry of the articular facets of the axis persisted [20].

4. Conclusion

We conclude that delayed diagnosis after the age of 5 years old still can be managed successfully with a correction of the head position and rotation motion. However, in these older children, physiotherapy alone cannot be effective and should be associated to surgery based on unipolar distal tenotomy, leaving bipolar tenotomy to young teenagers and beyond. Also, if congenital torticollis can be cured, facial asymmetry if associated does not always get improved. Moreover, once the diagnosis is done, surgical treatment should be performed to avoid wasting more time.

Provenance and peer review

Not commissioned, externally peer-reviewed.

Consent

Written informed consent was obtained from the patient's parents for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Funding

None.

Ethical approval

Ethical approval was provided by the authors' institution.

Author contribution

Dr. Khadija El Bouhmadi: Study concept and writing the paper.

Pr. Oukessou Youssef: Study concept and correction of the paper.

Dr. Saout Arrih Badr: Corresponding author and writing the paper.

Pr. Abada Reda: Study concept and correction of the paper.

Pr. Rouadi Sami: Study concept and correction of the paper.

Pr. Mahtar Mohamed: Study concept and correction of the paper.

Guarantor

El Bouhmadi Khadija.

Research registration

Not required.

Declaration of competing interest

The authors declare having no conflicts of interest for this article.