There were 47 patients with congenital muscular torticollis who underwent operative release. After a mean follow-up of 74 months (60 to 90), they were divided into two groups, one aged one to four years (group 1) and the other aged five to 16 years (group 2). The outcomes were assessed by evaluating the following parameters: deficits of lateral flexion and rotation, craniofacial asymmetry, surgical scarring, residual contracture, subjective evaluation and degree of head tilt. The craniofacial asymmetry, residual contracture, subjective evaluation and overall scores were similar in both groups. However, group 2 showed superior results to group 1 in terms of the deficits of movement, surgical scarring and degree of head tilt. It is recommended that operative treatment for congenital muscular torticollis is postponed until the patient can comply successfully with post-operative bracing and an exercise programme

Adult presentation of neglected congenital muscular torticollis is rare. We report 12 patients with this condition who underwent a modified Ferkel’s release comprising a bipolar release of sternocleidomastoid with Z-lengthening. They had a mean age of 24 years (17 to 31) and were followed up for a minimum of two years. Post-operatively a cervical collar was applied for three weeks with intermittent supervised active assisted exercises for six weeks. Outcome was assessed using a modified Lee score and a Cheng and Tang score. The mean pre-operative rotational deficit was 8.25° (0° to 15°) and mean lateral flexion deficit was 20.42° (15° to 30°), which improved after treatment to a mean of 1.67° (0° to 5°) and 7.0° (4° to 14°) after treatment, respectively. According to the modified Lee scoring system, six patients had excellent results, two had good results and four had fair results, and using the Cheng and Tang score, eight patients had excellent results and four had good results. Surgical management of adult patients with neglected congenital muscular torticollis using a modified Ferkel’s bipolar release gives excellent results. The range of neck movement and head tilt improved in all 12 patients and cosmesis improved in 11, despite the long-standing nature of the deformity

Benign paroxysmal torticollis is a self-limiting condition occurring during infancy. It resolves by the age of two to three years. Periodic episodes of torticollis may randomly alternate from side to side and be associated with other symptoms. The aetiology is unknown and no treatment is effective. It is relatively uncommon, and has not been previously reported in the orthopaedic literature, although initial referral may well be to an orthopaedic surgeon. We report four cases, and review the literature

Aims

Developmental dysplasia of the hip (DDH) can be managed effectively with non-surgical interventions when diagnosed early. However, the likelihood of surgical intervention increases with a late presentation. Therefore, an effective screening programme is essential to prevent late diagnosis and reduce surgical morbidity in the population.

1. A parallel study has been made of fifty patients presenting with a sternomastoid tumour and fifty-two patients presenting with muscular torticollis. 2. In the birth histories of both these groups there was a high incidence of breech, forceps and primiparous births. The distribution of each was strikingly similar. 3. Sternomastoid tumours were right-sided in three-quarters of all cases and in an even higher proportion of the breech births. There was twice the expected incidence of plagiocephaly. Only one in seven proceeded to muscular torticollis, but in some of the remainder minor residua could be detected. 4. Muscular torticollis presented at any age, but one-third commenced in the first year of life. Only one in five gave a history of previous sternomastoid tumour. The contracture showed a predilection for the clavicular head, and was generally associated with some degree of facial asymmetry. 5. Nine of a combined 102 cases had a first or second degree similarly affected relative. 6. It is concluded that whatever the condition in the muscle at birth, it has three inconstant and variable sequelae. The torticollis may resolve completely; it may become clinically manifest as a tumour; or it may remain clinically latent, subsequently undergoing a variable degree of cicatrisation to produce torticollis. 7. The treatment of established torticollis by open division is described and the follow-up in thirty-six cases recorded. 8. This operation can be relied on to cure the principal deformity, but is accompanied by a number of minor cosmetic defects. Of these the most striking are tight bands apparently due to anomalous reattachment of the clavicular head, and loss of the sternomastoid column of the neck. 9. The method could not be relied on to cure facial asymmetry completely, even in the early years of life. However, there was some evidence to suggest that persistence of asymmetry was allied to persistence of other residua of the torticollis, for example fascial bands

We report a series of 15 children, six male and nine female, of average age 20 months, seen at a paediatric orthopaedic clinic with torticollis. Orthopaedic examination revealed a normal range of neck movement in all cases but in seven there was palpable tightness in the absence of true shortening or contracture of the sternomastoid muscle. The patients were prospectively referred for ocular examination. In five of the 15 an ocular cause for the torticollis was detected with underaction of the superior oblique muscle in three, paresis of the lateral rectus muscle in one and nystagmus in one. Another two patients were found to have an abnormal ocular examination which was thought to be unrelated to their torticollis. Three of the patients with ocular torticollis required extra-ocular muscle surgery to abolish the head tilt and one of these had a tight sternomastoid muscle. Two of the non-ocular group had surgical release of the sternomastoid muscle; in the rest, the condition either resolved with physiotherapy or required no active treatment. We recommend that all patients with torticollis and no clear orthopaedic cause are referred for ocular assessment since it is not possible clinically to distinguish ocular from non-ocular causes

1. One hundred cases of torticollis and 117 cases of sternomastoid tumour have been reviewed. 2. Congenital torticollis can be sub-divided into two groups: postural and muscular. 3. Congenital postural torticollis is present at birth; it is not associated with a sternomastoid tumour; it is transient in nature; and it does not require operation for its relief. 4. Congenital muscular torticollis is preceded by a sternomastoid tumour which is clinically evident in one-fifth of all cases. 5. The ischaemic theory of the causation of sternomastoid tumours is not supported by recent histological investigations. Some other cause, which probably is operative before birth, must be sought. 6. Four-fifths of all cases of sternomastoid tumours resolve spontaneously and leave no deformity. Excision of the tumour in infancy is therefore unjustifiable. 7. Open division of the muscle and of the cervical fascia in congenital muscular torticollis cures the deformity but leaves an unsightly scar. 8. Subcutaneous tenotomy can be relied upon to cure the deformity if post-operative treatment is carried out skilfully and assiduously over a prolonged period. 9. If complete correction is not gained at the time of subcutaneous tenotomy a better result can be assured by open division of the upper end of the muscle through an incision within the hair line

We reviewed seven children with torticollis due to refractory atlanto-axial rotatory fixation who were treated in a halo vest. Pre-operative three-dimensional CT and sagittal CT imaging showed deformity of the superior articular process of C2 in all patients. The mean duration of halo vest treatment was 67 days (46 to 91). The mean follow-up was 34 months (8 to 73); at the latest review six patients demonstrated remodelling of the deformed articular process. The other child, who had a more severe deformity, required C1-2 fusion. We suggest that patients with atlanto-axial rotatory fixation who do not respond to conservative treatment and who have deformity of the superior articular process of C2 should undergo manipulative reduction and halo-vest fixation for two to three months to induce remodelling of the deformed superior articular process before C1-2 fusion is considered

Aims. Although atlantoaxial rotatory fixation (AARF) is a common cause of torticollis in children, the diagnosis may be delayed. The condition is characterised by a lack of rotation at the atlantoaxial joint which becomes fixed in a rotated and subluxed position. The management of children with a delayed presentation of this condition is controversial. This is a retrospective study of a group of such children. Patients and Methods. Children who were admitted to two institutions between 1988 and 2014 with a diagnosis of AARF were included. We identified 12 children (four boys, eight girls), with a mean age of 7.3 years (1.5 to 13.4), in whom the duration of symptoms on presentation was at least four weeks (four to 39). All were treated with halo traction followed by a period of cervical immobilisation in a halo vest or a Minerva jacket. We describe a simple modification to the halo traction that allows the child to move their head whilst maintaining traction. The mean follow-up was 59.6 weeks (24 to 156). Results. Despite the delay in referral, the subluxation was successfully reduced in all children. Only two children required atlantoaxial fusion. Conclusion. The results of our study suggest that normal anatomy with restoration of movement may be achieved even in cases of AARF presenting late, obviating the need for fusion. We also show a simple modification to the halo traction that allows the child to move their head about while still maintaining traction. Take home message: Prompt diagnosis and management with halo traction (with a simple modification as described) is associated with good results in patients with AARF who present late. Cite this article: Bone Joint J 2016;98-B:715–20

We report four children with sternomastoid contracture combined with torticolis secondary to congenital vertebral anomalies. Two had features of Klippel-Feil syndrome and a proximal release of the contracted sternomastoid produced good cosmetic correction initially. Progression of the deformity occurred subsequently without recurrence of sternomastoid contracture. One child had such mild deformity that it was merely observed. The fourth child was born with torticollis without sternomastoid tightness and a vertebral anomaly was later recognised. He slowly developed a sternomastoid contracture and his condition was considerably improved by sternomastoid release. This combination of causes of torticollis has not, as far as we know, been previously reported. The clinician should be aware of it and should also realise that radiographs of the very immature spine may not disclose the bony anomalies

Aims

Early detection of developmental dysplasia of the hip (DDH) is associated with improved outcomes of conservative treatment. Therefore, we aimed to evaluate a novel screening programme that included both the primary risk factors of breech presentation and family history, and the secondary risk factors of oligohydramnios and foot deformities.

We report three cases of atlanto-axial rotatory fixation in adults. Early diagnosis was made by clinical tests showing restricted head rotation in maximal neck flexion and asymmetry of the transverse processes of the atlas, confirmed by cineradiography. Early treatment by traction may obviate long-term problems of torticollis and instability

The moulded baby syndrome comprises: head moulding (plagiocephaly); pelvic obliquity with unilateral loss of hip abduction in flexion; and occasionally scoliosis, torticollis and bat ears. The hips, however, are radiologically normal and do not require the treatment used in the management of congenital dislocation or dysplasia. A review of 67 hips confirms this finding

Three-dimensional CT was used to examine the atlantoaxial joint of a nine-year-old girl who presented with fixed rotation of the head 3.5 months after an apparently spontaneous acute torticollis. The method provided clear, anatomical images to show the site, extent and direction of the type-I rotatory fixation (Fielding and Hawkins 1977). The 3-D reconstructions helped to guide manipulation which was successful in this late case

Eight patients suffering from various forms of idiopathic dystonia are described whose initial referral was for an orthopaedic opinion. The diagnoses of these patients, who were seen over a two-year period, comprised dystonia musculorum deformans, dystonia of the foot, spasmodic torticollis and occupational cramps. Although various musculoskeletal sequelae often occur, the primary underlying neurological cause of these unusual conditions is emphasised

1. A case is described of severe birth injury to the sternomastoid muscle in a breech-delivered two-day-old infant. The affected muscle showed widespread haemorrhage, fragmentation and necrosis of its fibres, and disruption and disorganisation of the endomysial sheaths. 2. Disruptive muscular trauma of this type is known to lead to florid fibroblastic proliferation with formation of a large mass of scar tissue. It is suggested that the "sternomastoid tumour" of infancy develops as a sequel to such trauma occurring during birth. 3. The theories of birth trauma and of ischaemia, in the etiology of the "sternomastoid tumour" of infancy and of congenital muscular torticollis, are not mutually exclusive but may be complementary, the circumstances causing the trauma also leading to ischaemic damage

Aims

Despite the presence of screening programmes, infants continue to present with late developmental dysplasia of the hip (DDH), the impact of which is significant. The aim of this study was to assess infants with late presenting dislocation of the hip despite universal clinical neonatal and selective ultrasound screening.

Aims

The aim of this study was to identify the association between asymmetrical skin creases of the thigh, buttock or inguinal region and pathological developmental dysplasia of the hip (DDH).

The aim of this prospective study was to investigate prematurity as a risk factor for developmental dysplasia of the hip (DDH). The hips of 221 infants (88 female, 133 male, mean age 31.11 weeks; standard deviation (sd) 2.51) who were born in the 34th week of gestation or earlier, and those of 246 infants (118 female, 128 male, mean age 40.22 weeks; sd 0.36) who were born in the 40th week of gestation, none of whom had risk factors for DDH, were compared using physical examination and ultrasound according to the technique of Graf, within one week, after the correction of gestational age to the 40th week after birth or one week since birth, respectively. Both hips of all infants were included in the study. Ortolani’s and Barlow’s tests and restricted abduction were accepted as positive findings on examination. There was a statistically significant difference between pre- and full-term infants, according to the incidence of mature and immature hips (p < 0.001). The difference in the proportion of infants with an α angle < 60° between the two groups was statistically significant (p < 0.001). The incidence of pathological dysplasia (α angle < 50 º) was not significantly different in the two groups (p = 1.000). The Barlow sign was present in two (0.5%) pre-term infants and in 14 (2.8%) full-term infants.

These results suggests that prematurity is not a predisposing factor for DDH.

Cite this article: Bone Joint J 2015; 97-B:716–20

Fracture of the atlas is rare in children. We report a case of fracture of the atlas through a synchondrosis of the anterior arch complicated by atlantoaxial rotatory fixation in a four-year-old girl.

We present the case of an 83-year-old man who developed quadriparesis and respiratory embarrassment following osteomyelitis at the occipito-atlantoaxial junction. He had developed an abscess at this site after an earlier urinary infection with methicillin-resistant staphylococcus aureus. Stabilisation of the neck and antibiotic therapy led to an almost complete neurological recovery without recourse to anterior surgery.

There is controversy whether congenital foot abnormalities are true risk factors for pathological dysplasia of the hip. Previous United Kingdom screening guidelines considered congenital talipes equinovarus (CTEV) to be a risk factor for hip dysplasia, but present guidelines do not. We assessed the potential relationship between pathological dysplasia of the hip and fixed idiopathic CTEV.

We present a single-centre 21-year prospective longitudinal observational study. All fixed idiopathic CTEV cases were classified (Harrold and Walker Types 1 to 3) and the hips clinically and sonographically assessed. Sonographic Graf Type III, IV and radiological irreducible hip dislocation were considered to be pathological hip dysplasia.

Over 21 years there were 139 children with 199 cases of fixed idiopathic CTEV feet. Sonographically, there were 259 normal hips, 18 Graf Type II hips, 1 Graf Type III hip and 0 Graf Type IV hip. There were no cases of radiological or sonographic irreducible hip dislocation.

Fixed idiopathic CTEV should not be considered as a significant risk factor for pathological hip dysplasia. This conclusion is in keeping with the current newborn and infant physical examination guidelines in which the only risk factors routinely screened are family history and breech presentation. Our findings suggest CTEV should not be considered a significant risk factor in pathological dysplasia of the hip.

Cite this article: Bone Joint J 2014;96-B:1553–5.

Fractures of the odontoid in children with an open basilar synchondrosis differ from those which occur in older children and adults. We have reviewed the morphology of these fractures and present a classification system for them.

There were four distinct patterns of fracture (types IA to IC and type II) which were distinguished by the site of the fracture, the degree of displacement and the presence or absence of atlantoaxial dislocation. Children with a closed synchondrosis were classified using the system devised by Anderson and D’Alonzo. Those with an open synchondrosis had a comparatively lower incidence of traumatic brain injury, a higher rate of missed diagnosis and a shorter mean stay in hospital. Certain subtypes (type IA and type II) are likely to be missed on plain radiographs and therefore more advanced imaging is recommended. We suggest staged treatment with initial stabilisation in a Halo body jacket and early fusion for those with unstable injuries, severe displacement or neurological involvement.

The practice of regular radiological follow-up of infants with a positive family history of developmental dysplasia of the hip is based on the widespread belief that primary acetabular dysplasia is a genetic disorder which can occur in the absence of frank subluxation or dislocation. We reviewed all infants who were involved in our screening programme for developmental dysplasia of the hip, between November 2002 and January 2004, and who had a normal clinical and ultrasound examination of the hip at six to eight weeks of age, but who, because of a family history of developmental dysplasia of the hip, had undergone further radiography after an interval of 6 to 12 months. The radiographs of 89 infants were analysed for signs of late dysplasia of the hip and assessed independently by three observers to allow for variability of measurement. There were 11 infants (11%) lost to follow-up.

All the patients had normal radiographs at the final follow-up and none required any intervention. We therefore question the need for routine radiological follow-up of infants with a positive family history of developmental dysplasia of the hip, but who are normal on clinical examination and assessment by ultrasound screening when six to eight weeks old.

Twins are often considered to be at an increased risk of developmental dysplasia of the hip (DDH); we therefore investigated whether multiple births have a higher incidence of DDH, and if selective ultrasound scanning should be considered for these infants.

We reviewed our records of all live births between 1 January 2004 and 31 December 2008 and included 25 246 single and 990 multiple births.

Multiple births did not have a significantly higher incidence of DDH compared with single births (0.0030 vs 0.0023, p = 0.8939). Of the 990 multiple births, 267 had neonatal ultrasound scans and one case of DDH was diagnosed and treated successfully with a Pavlik harness. There were two late-presenting cases at eight and 14 months of age, neither of whom had risk factors for DDH and consequently had not had a neonatal scan. Whereas selective ultrasound scanning of multiple births would have led to earlier detection and treatment of the late-presenting cases, they did not have a significantly higher incidence of DDH compared with single births.

We conclude that being a twin or triplet in itself is not a risk factor for DDH and that selective ultrasound scanning is not indicated for this population.

Cite this article: Bone Joint J 2013;95-B:132–4.

The association between idiopathic congenital talipes equinovarus (CTEV) and developmental dysplasia of the hip is uncertain. We present an observational cohort study spanning 6.5 years of selective ultrasound screening of hips in clubfoot. From 119 babies with CTEV there were nine cases of hip dysplasia, in seven individuals. This suggests that 1 in 17 babies with CTEV will have underlying hip dysplasia.

This study supports selective ultrasound screening of hips in infants with CTEV.

We retrospectively studied the possibility that direct trauma to the biceps muscle might be the cause of poor elbow flexion and supination in 18 consecutive children with birth lesions of the brachial plexus who had delayed or impaired biceps recovery despite neurophysiological evidence of reinnervation. All had good shoulder and hand function at three months of age. Eight recovered a strong biceps after six months, but nine required a pectoralis minor to biceps transfer to augment elbow flexion and supination. One had a delayed but good recovery of the biceps after microsurgical reconstruction of the plexus. All had a clinical ‘pseudotumour’ in the biceps muscle, which was biopsied during pectoralis minor transfer in two patients and showed rupture and degeneration of muscle fibres with a fibro-fatty infiltrate, suggesting previous muscle trauma.

Direct muscle trauma is an uncommon but important cause of delayed or impaired biceps recovery after brachial plexus birth injuries. Surgery to reinnervate the biceps muscle will not work if substantial muscle damage is present when a suitable muscle transfer should be considered.

This prospective cohort study aimed to investigate the relationship between developmental dysplasia of the hip and mode of delivery in 571 consecutive breech infants using a modified Graf’s static morphological method to grade the severity of dysplasia.

In this group, 262 infants were born by planned Caesarian section, 223 by emergency section and 86 vaginally. Taking all grades of hip dysplasia into account (Graf types II, III and IV), there was no statistical difference in the incidence of dysplasia between the groups (elective section 8.4%, emergency section 8.1% and vaginal delivery 7.0%). However, when cases with Graf type II dysplasia, which may represent physiological immaturity, were excluded, the rate of type III and IV hips, which we consider to be clinically relevant, increased in the vaginally delivered group (4.7%) compared with the elective section group (1.1%), with a relative risk of approximately 1:4 (95% confidence interval 1.03 to 15.91). No difference was observed between the emergency and elective section groups, or between the emergency section and vaginally delivered groups.

This study supports previous published work, with the added value that the diagnoses were all confirmed by ultrasound.

In a prospective study over 11 years we assessed the relationship between neonatal deformities of the foot and the presence of ultrasonographic developmental dysplasia of the hip (DDH). Between 1 January 1996 and 31 December 2006, 614 infants with deformities of the foot were referred for clinical and ultrasonographic evaluation. There were 436 cases of postural talipes equinovarus deformity (TEV), 60 of fixed congenital talipes equinovarus (CTEV), 93 of congenital talipes calcaneovalgus (CTCV) and 25 of metatarsus adductus.

The overall risk of ultrasonographic dysplasia or instability was 1:27 in postural TEV, 1:8.6 in CTEV, 1:5.2 in CTCV and 1:25 in metatarsus adductus.

The risk of type-IV instability of the hip or irreducible dislocation was 1:436 (0.2%) in postural TEV, 1:15.4 (6.5%) in CTCV and 1:25 (4%) in metatarsus adductus. There were no cases of hip instability (type IV) or of irreducible dislocation in the CTEV group.

Routine screening for DDH in cases of postural TEV and CTEV is no longer advocated. The former is poorly defined, leading to the over-diagnosis of a possibly spurious condition. Ultrasonographic imaging and surveillance of hips in infants with CTCV and possibly those with metatarsus adductus should continue.

Of the 34 723 infants born between 1 June 1992 and 31 May 2002, the hips of 2578 with clinical instability or at-risk factors for developmental dysplasia of the hip were imaged by ultrasound.

Instability of the hip was present in 77 patients, of whom only 24 (31.2%) had an associated risk factor. From the ‘at-risk’ groups, the overall risk of type-III dysplasia, instability and irreducibility was 1:15 when family history, 1:27 when breech delivery and 1:33 when foot deformity were considered as risk factors. Of those hips which were ultrasonographically stable, 88 had type-III dysplasia.

A national programme of selective ultrasound screening of at-risk factors for the diagnosis of hip dislocation or instability alone cannot be recommended because of its low predictive value (1:88). However, the incidence of type-III dysplasia and hip dislocation or dislocatability in the groups with clinical instability, family history, breech position and possibly postural foot deformity as risk factors could justify a programme of selective ultrasound imaging.