Groningen, The Netherlands
Neck Flexion Deformity
Complaint: flexion deformity of the neck.
Assessment: instead of a normal cervical lordosis there is a kyphosis.
Diagnosis: cervical kyphosis
Explanatory note: cervical kyphosis. A cervical kyphosis in a neutral posture should be considered abnormal. Cervical kyphosis can be caused by one or two under- developed vertebral bodies or an operation in which a laminectomy was performed, or it can be part of a syndrome (Table 2.1). If it is one of the symptoms of a syndrome, the child’s other abnormalities are usually so impressive that the practitioner has not noticed the kyphosis in the neck. It is however important to identify the cervical kyphosis because even during infancy compression of the spinal cord can already occur, resulting in lifelong paraplegia or even death.
Syndromes with a cervical kyphosis. See Appendix for features of syndromes
Campomelic (camptomelic) dysplasia
Neurofibromatosis (Von Recklinghausen disease)
Supplementary assessment: anteroposterior and lateral X-rays as well as a CT-scan and a MRI of the cervical spine.
Primary care treatment: none.
When to refer: all cervical kyphoses should be considered pathological and be referred as quickly as possible.
Secondary care treatment: cervical kyphosis. In milder kyphosis without neurological abnormalities a wait-and-see approach may be taken. One should wait until the age of 18 months before performing a spondylodesis for more severe kyphosis without neurological abnormalities.
Neurological abnormalities necessitate earlier intervention. In addition to the cervical spondylodesis, the spinal cord must be decompressed.
Complaint: short neck.
Assessment: the patient has a short neck (Fig. 2.1). In 50 % of cases there is a triad:
(a, b) Klippel-Feil syndrome. The short neck and the low cranial hair growth
limited mobility of the cervical spine
growth of cranial hair on the back
In less than 20 % of cases extra skin can be seen on the lateral side(s) of the neck with muscular fascial tissue in the form of a wing (pterygium colli), known as “webbed neck”. This extra tissue is spread between the mastoid process and the acromion on both sides.
Diagnosis: Klippel-Feil syndrome1 (congenital brevicollis)
Explanatory note: Klippel-Feil syndrome. The clinical picture was described by the French physicians Klippel and Feil in 1911 as “l’homme sans cou” (man without a neck). The short neck is caused by vertebrae that have fused together. There may also be a unilateral unsegmented bony connection, hemivertebrae, absence of the posterior elements (arches and spinal processes), a basilar impression, an occipitoatlantal synostosis, an atlanto-axial subluxation and a hypoplasia of the dens. In 20 % of cases the abnormality is accompanied by a torticollis, in 60 % there is a kyphoscoliosis at the thoracic level caused by the hemivertebrae and unilateral and unsegmented bony connections. In 30 % of cases there is an elevated posture and under-development of the shoulder blade, known as a Sprengel deformity. In about 25 % of cases there are cardiac and renal abnormalities, and in 30 % deafness. Neurological complications in adults are possible as a result of compression of the myelum or of the exiting spinal nerves.
Supplementary assessment: X-rays as well as a CT-scan and a MRI of the cervical and thoracic spines.
Primary care treatment: if the patient has no pain or neurological symptoms there will be a wait-and-see policy. If there is pain, it is advisable to limit the activities and a cervical collar can be prescribed.
When to refer: in the early phase to the pediatrician before detecting any other anomalies. Referral should be made to the orthopedic surgeon for additional anomalies such as muscular torticollis and scoliosis, and to the neurosurgeon if neurological complications occur at an older age.
Secondary care treatment: Klippel-Feil syndrome. A torticollis based on a shortened sternocleidomastoid muscle can be treated by dividing or lengthening the muscle. Neurological symptoms are caused mostly by an atlantoaxial subluxation, a basilar impression or a congenital occipitoatlantal synostosis (for treatment, see pp. 15, 18, 19).
Complaint: a wry neck is usually present at birth, but sometimes it appears at an older age.
Assessment: lateral flexion of the head towards the affected side, the chin is turned towards the non-deviated side. An easy way to determine on which side the deviation lies is to draw an imaginary line between the pupils. The shoulder that this line points to is the affected side (Fig. 2.2).
(a) Torticollis. Contracture of the left sternocleidomastoideus muscle. An easy way to determine on which side the abnormality lies is to draw an imaginary line between the pupils. The shoulder this line points to is the affected side. (b) Situation 2 years after dividing the left sternocleidomastoid muscle origin
infantile muscular torticollis (congenital muscular torticollis) juvenile muscular torticollis osseous torticollis ocular torticollis
Explanatory note: torticollis. The cause of the infantile as well as juvenile muscular torticollis is unknown.
Infantile muscular torticollis
In 20 % of cases a swelling in the trajectory of the sternocleidomastoid muscle is visible and palpable between 10 days and 4 weeks after birth (Fig. 2.3). The swelling reduces gradually 4 weeks after it has appeared. By the age of 4–6 months the swelling has disappeared. In 80 % of cases the swelling is not observed or not recognized, and the abnormality is later on identified on the basis of a contracture of one or both heads of the sternocleidomastoid muscle.
Swelling in the trajectory of the right sternocleidomastoid muscle (arrow)
The sternocleidomastoid muscle has a sternal and a clavicular origin. The joint muscle belly inserts into the mastoid process and occiput just behind the ear (Fig. 2.4). The cause of the swelling in the sternocleidomastoid muscle is unclear. It used to be assumed that during a difficult birth (breech presentation or forceps delivery) there was bleeding into this muscle, followed by fibrosis of the hematoma. However, blood in the swelling has never been shown. It is now assumed that the fibrous tissue is already present before birth.
(a) The sternocleidomastoid muscle has a sternal and a clavicular origin. The shared muscle belly inserts into the mastoid process and occiput just behind the ear. The sternocleidomastoid muscle can operatively be lengthened or divided at the level of the clavicle and sternum. If there is a recurrence the insertion at the level of the mastoid process and occiput may also have to be divided. (b, c): In this case the sternocleidomastoid muscle is divided at the mastoid process and occiput. The origins of the clavicular and sternal head are lengthened with a Z-plasty (Redrawn from: Ferkel RD, Westin GW, Dawson EG, Oppenheim WL. Muscular torticollis. A modified surgical approach. J Bone Joint Surg Am. 1983;65-A:894–900)
Spontaneous recovery of the torticollis occurs during the first year of life in 90 % of cases. If the abnormality persists, the face and the skull will also become deformed, with a flattening of the skull and the face on the side of the contracture (plagiocephaly). A developmental dysplasia of the hip is found in 20 % of children with an infantile muscular torticollis. If there is no contracture of the sternocleidomastoid muscle, the underlying causes are a congenital anomaly of the skeleton or an ocular abnormality.
Juvenile muscular torticollis
Sometimes a muscular torticollis appears at childhood. In that case there is a contracture of both heads of the sternocleidomastoid muscle. This condition does not recover spontaneously.
Due to the presence of a hemivertebra (half vertebra) or one or several asymmetric nonsegmented bony connections, a wry neck can be present. The patient has Klippel-Feil syndrome2 if the neck is also too short.
Goldenhar syndrome or oculoauriculovertebral dysplasia is a special form of osseous torticollis in which in addition to abnormal vertebrae there are also eye and ear abnormalities such as epibulbar dermoid cysts and preauricular skin anomalies.
This anomaly is present from birth, but is often only noticed around the age of 9 months, after the child has achieved sitting balance. Paresis of the extraocular muscles, generally the superior oblique muscle, causes crossed and double vision when the head is held horizontally. The child must hold his head slanted in the frontal plane to prevent double vision.
Supplementary assessment: radiological assessment of the cervical spine to reveal or rule out an osseous torticollis. In infantile or congenital muscular torticollis an ultrasound of the hips or an anteroposterior X-ray of the pelvis should be made to check for a developmental dysplasia of the hip.
Primary care treatment: a patient with infantile muscular torticollis is usually referred to a pediatric physiotherapist up to the age of 18 months.
The parents do stretching exercises under the supervision of the pediatric physiotherapist. It is not certain whether these stretching exercises influence the natural history. Infantile muscular torticollis disappears spontaneously around the first year of life in 90 % of cases.
When to refer: children with a muscular or osseous torticollis should be referred to an orthopedic surgeon when the child is older than 18 months. If the sternocleido-mastoid muscle is not shortened and there are no osseous abnormalities the child should be referred to an ophthalmologist.
Secondary care treatment: infantile muscular torticollis. If the abnormality is still present at 1 year of age there is little chance of spontaneous recovery. In such cases, the sternocleidomastoid muscle should be operatively lengthened or divided at the clavicular and/or sternal origin (Fig. 2.4). The ideal age for operative treatment is in the third year of life. The asymmetry of the face and the skull restores completely after that. Operating before this age increases the chances of an ugly scar and retraction of the skin at the level where the sternocleidomastoid muscle has been lengthened or divided. Even after this age it is not too late to carry out the correction. Good cosmetic results can be achieved up to the age of 12. The sooner the treatment is implemented, the better the ultimate result will be with regard to facial symmetry.
Postoperative treatment involves the use of a neck orthosis day and night for 6 weeks (Fig. 2.5). In the case of a recurrence, in addition to dividing or lengthening the origin at the level of the clavicle and sternum the insertion at the level of the mastoid process should also be divided.
(a, b) Neck orthosis, in this case a SOMI orthosis (S sternum, O occiput, M mandibula, I immobilization)
Juvenile muscular torticollis
Usually this type is permanent and requires operative treatment.
Spondylodesis over a trajectory as short as possible should be carried out.
Treatment by an ophthalmologist.
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Acute Onset of a Painful Stiff and Possibly Wry Neck
Complaint: the child has a painful stiff neck with a possible abnormal posture.
Assessment: there is no mobility in the cervical spine and there may be a wry neck.
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