Paediatric Pearls

Torticollis

December 18, 2011, 12:24 am

Torticollis / Wry Neck / Sternomastoid tumour of infancy with thanks to Dr Katie Knight

(From Latin tortus = twisted + collum = neck)

Torticollis can be congenital or acquired, but this article will focus mostly on the congenital form, affecting 0.3% of infants and usually presenting in the first 6 months of life [1]. It is the third most common reason for referral to orthopaedics in this age group. The overwhelming majority of cases seen are due to a benign muscular problem, but some more sinister diagnoses can also present in a similar way, so it is crucial to be aware of these.

What causes torticollis?

Muscular damage:

Most cases of congenital torticollis are the result of damage to the sternocleidomastoid muscle (SCM) at birth (for example in instrumental delivery) or in the uterus (restricted movement or abnormal positioning causing muscle damage).

Damage to the SCM causes it to shorten or contract as fibrosis affects the area. Fibrotic change in the damaged muscle is felt as a hard lump – the ‘pseudotumour’ of torticollis, as it is sometimes called.

This shortening of the muscle in turn makes it difficult for the infant to turn their head, resulting in neck stiffness and a fixed head position, with very limited neck movement.

Risk of muscular torticollis is increased in intrauterine constraint (eg breech presentation or oligohydramnios [2]), and it is also associated with other minor positional deformities. 10% of babies with torticollis have hip dysplasia. [3] One study looking at 1001 babies found that 10% had one or more postural deformities (in decreasing order of frequency: plagiocephaly or torticollis; congenital scoliosis or pelvic obliquity; adduction contracture of a hip and/or malpositions of the knees or feet [4]. This study found that all these deformities were more likely to be observed in:

babies with a greater birth length
breech presentation
oligohydramnios
babies delivered instrumentally
Male infants were also found to be 1.9 x more likely to have positional deformities including torticollis.

With these presenting symptoms described above and nothing else of note, torticollis is clearly the first diagnosis that springs to mind. HOWEVER – to play the devil’s advocate – a baby who presents with ‘a lump in the neck’ and ‘abnormal neurology’ certainly demands a careful history and examination.

Uncommon causes of torticollis

Congenital vertebral abnormalities:

The SCM is supplied by the accessory nerve (CN XI), which exits the skull through the jugular foramen. Anything affecting the structure of the upper cervical spine or skull base could compress the nerve root of CN XI and cause torticollis.

Congenital vertebral abnormalities often come along with other congenital abnormalities, as part of a syndrome (two examples are briefly described below, for interest). For this reason a child presenting with torticollis who is known to have other congenital abnormalities should be carefully examined with the possibility of an unusual syndrome kept in mind. [5]

MURCS syndrome (Müllerian duct/renal aplasia/cervicothoracic somite dysplasia) is a rare condition affecting 1 in 5000 female infants that has been associated with congenital torticollis in some cases due to aplasia of the posterior vertebral arch [6]

Klippel-Feil syndrome – cervical spine fusion is seen along with a host of other symptoms [7]

Posterior fossa tumours, tumours of the cervical spine, atlas and axis - these are very rare and should be part of the differential in older children who present with acquired torticollis. [8, 9] Posterior fossa tumours, when they present with torticollis, usually have accompanying symptoms of intracranial pathology (headache, nausea, vomiting, eye signs) [10]

‘Mimics’ of torticollis

‘Ocular torticollis’ occurs when there is 4^th cranial nerve palsy. The superior oblique muscle, supplied by CN IV, causes the eye to look inwards and downwards. Paralysis of the muscle means the eye cannot adduct or internally rotate, and this causes torsional diplopia, which the child ‘corrects’ by tilting the head position. Adopting this position over a long period of time eventually causes contracture of the SCM. [11] This condition can be ruled out by using the cover test (watch a 7 minute long Youtube video with a rather disconcerting picture of a huge eye in the background here). When the affected eye is covered, the child should spontaneously correct their head position (in the early stages, before muscle contracture has occurred).

Examination

Appearance (see image): The head is tilted to one side (to the side of the affected muscle), and the chin is turned to the other side. There is stiffness, from the lack of movement, so there may be pain when the neck position is passively corrected.

A lump may be felt in the distal SCM.

Lump felt in distal SCM

Investigation

The key is to differentiate between muscular torticollis (ie common, benign, easily correctible) and non-muscular torticollis (ie possibly secondary to neurological, ocular or vertebral pathology, and needing further investigation.

If there is a lump palpable in the SCM, it needs to be differentiated from other causes of a lump in the neck. Ultrasound is the best first line investigation – it detects fibrosis of the muscle (diagnosing torticollis) but would also pick up abnormal lymph nodes or masses.

Fine needle aspiration would be the next step if there was any uncertainty of the diagnosis, but this is rarely needed.

Treatment

Once muscular torticollis is confirmed:

Physiotherapy is the mainstay of treatment. Even when there is severe fibrosis of the SCM, physio is effective in 98% [12]

Neck stretches, performed regularly, moving the neck in the opposite direction to the affected muscle (tilt head sideways towards non-affected side, rotate towards affected side). Physio referral is indicated so parents can be taught the correct way to perform the stretches.
Let the baby spend more time lying on its tummy, to strengthen neck muscles
Use baby chair or Fraser chair to minimise the time the baby spends lying flat
Encourage head turning to affected side by using toys, distraction, feeding from that side
Physiotherapist may advise use of a neck brace in certain cases.

(The above advice adapted from ‘Physio Questions’ [13], a blog by an Australian physio – torticollis featured as a blog entry in August 2010)

Surgical treatment is very rarely needed – only in instances where conservative management has failed after 6 months of treatment. When surgery is performed, the operation is a bipolar release of the SCM, and this has been found to be highly successful, even in patients older than 5 years [14] and into adulthood [15]

Alternatives to surgery?

A recent successful non-surgical development in treating cases resistant to physio is using botox injection. [16] The evidence for chiropractic treatment is weak, isolated successful cases have been described, [17] but there has been no randomised controlled trial. There are also reports of infants with torticollis caused by neurogenic tumours being treated (unsuccessfully) by a chiropractor before the correct diagnosis was made, [18] so it is imperative that parents have consulted a doctor before they choose to seek chiropractic help.

References:

Tags: musculoskeletal, neurology, ophthalmology, paediatric surgery
Category: For Emergency Departments, For General Practitioners | Comment

Paediatric surgery at Whipps Cross

April 26, 2011, 10:40 pm

I thought it might be useful to outline the sort of paediatric surgery Whipps offers. This post is being put together with the help of my surgical colleagues who are keen to continue receiving appropriate paediatric referrals from GPs. I will be updating it as and when I get the information from the different specialties.

A word from the anaesthetists: As a general rule all of our consultants on the on-call rota are currently able to anaesthetise any child, 3 years or above presenting as an emergency as long as they are ASA 1 or 2 and needing non body cavity surgery (appendicectomy excepted) with an expected duration of less than 2 hours. (“ASA 1 and 2″ are children who are previously fit and well and those with a mild underlying systemic illness such as mild asthma.) We have three consultants who have had advanced training in paediatric anaesthesia (Drs. Williams, Singh and Radhakrishnan) who undertake regular elective paediatric lists who are happy to anaesthetise younger children.

GENERAL SURGERY (Mr Stephen Brearley) AND UROLOGY (elective surgery from 1 year of age, emergency surgery from 3 years of age):

Hernias (all types)

Foreskins

Undescended testicles

Lumps of all types

Tongue ties

Thyroglossal cyst

Emergency work in children over the age of 3 eg. suspected appendicitis

Please refer such things as in-grown toenails and verrucae to podiatrists rather than surgeons.

ENT:

(elective work from 6 months of age, emergency work from 3 years of age):

Lead Clinician, Mr. Sam Jayaraj

Dedicated Paediatric ENT clinics at WX:

Mr Jayaraj 3 per month at WX and 2 per month at Forest Medical Centre, Loughton (WX outreach clinic)

Mr Patel 2 per month at WX

Mr Kenyon 1 per month at WX.

N.B. We are in the process of setting up a Paediatric ENT Specialist Community Clinic (SCC) in conjunction with Dr Watkin and our audiological colleagues at Wood Street Clinic – watch this space!

ENT Surgical procedures offered at WX

Tonsillectomy

Adenoidectomy

Oral cavity lesions

Turbinate reduction

Obstructive sleep apnoea assessment

Nasal bone manipulation following trauma

Nasal cautery

Peri-orbital abcess drainage

Middle ear ventilation tubes (Grommets)

Pre-auricular sinus excision

Pinnaplasty (Mr Jayaraj and Mr Kenyon)

Myringoplasty/tympanoplasty

Mastoidectomy (Mr Nitesh Patel)

Cervical lymph node excision

Thyroglossal cyst excision

Branchial cyst excision

Tongue tie division

Thyroid surgery (Mr Papesch)

Tertiary paediatric ENT services not available at WX (these are rare conditions which require highly specialised nursing, anaesthetic and allied therapies support):

Cleft lip & palate surgery and craniofacial disorders

Paediatric head & neck malignancy

Cochlear implant surgery

Paediatric airway reconstruction surgery (i.e laryngotracheal reconstructive surgery etc)

Currently we refer these patients to our tertiary paediatric ENT colleagues at Great Ormond Street Hospital

OPHTHALMOLOGY:

Strabismus, eyelid, lacrimal, cataract (with infants being referred to Great Ormond Street where our ophthalmologist with a paediatric interest also works).

ORTHOPAEDICS:

MAXILLOFACIAL SURGERY:

Tags: ENT, ophthalmology, paediatric surgery
Category: For General Practitioners | Comment

GP version of February 2011′s Paediatric Pearls

March 2, 2011, 11:52 pm

GP February 2011 reminds us all of the NICE guideline on Attention Deficit and Hyperactivity Disorder. We continue our 6-8 week baby check series with information on the absent red reflex and go back to our “from the literature” box to discuss snoring and obstructive sleep apnoea (OSA). We have relaunched our prolonged jaundice guideline. Please leave comments and questions below.

Tags: 6 week check, ENT, jaundice, mental health issues, newborns, ophthalmology
Category: For General Practitioners | Comment

Checking the red reflexes

February 19, 2011, 11:12 pm

6 week check series – The Absent Red Reflex – with thanks to Dr Sarah Prentice

Importance of red reflex examination at the 6 week check

Early detection of potentially sight and life-threatening eye disease. Due to the early and time-limited plasticity and development of the eye, any blockage of light to the retina interferes with development of optic neural pathways and can have profound effects on later vision.

Pathology

Cataracts

Retinoblastoma

High Refractive errors

Vitreal haemorrhage/opacity

Corneal scaring (e.g. ocular toxocariasis)

Retinal tear

Retinopathy of prematurity

Persistence of the tunica vasculosa lentis/Persistent hyperplastic primary vitreous ⁽¹⁾

The Examination

Darkened room

Ophthalmoscope on +3 dioptres

Hold 1 foot away

Red reflexes can only be described as normal if they are:

Equal in colour, intensity and clarity with no opacities or white spots ⁽²⁾

Handy hints

For the child that won’t open his/her eyes: try picking/sitting them up or rocking them from sitting to lying. Having a parent hold them on their shoulder (as if winding them) and looking from behind often works. A feeding child will often open his/her eyes, although breast feeding then makes looking in the eyes logistically tricky.

Children with darker skin tones may have pale retina. If retinal vessels can be seen and followed to the disc and the reflex is equal bilaterally then this is reassuring. Comparison with parents’ red reflexes may also help.

Management:

Normal: No further follow-up. Will have routine ophthalmology review by school nurse/orthoptist in pre-school years. ⁽⁵⁾

Unable to see red-reflexes or unsure: Referral to paediatric ophthalmology primary care clinic (if available)

Absent red reflex: Urgent referral to paediatric ophthalmologists (should be seen in less than 2 weeks)

Family history of neonatal eye disease e.g. retinoblastoma, congenital cataracts: Routine referral to paediatric ophthalmologists.

Low birth-weight/premature infants (at high risk of retinopathy of prematurity): Should have had ROP screening and follow-up arranged as necessary by neonatal unit.

References and resources

1. Robertson’s Textbook of Neonatology. Fourth Edition. 2005. Edited by Janet M. Rennie.
2. American Academy of Pediatrics Policy Statement. Red Reflex Examination in Infants PEDIATRICS Vol. 109 No. 5 May 2002
3. Red Reflex Examination in Neonates, Infants, and Children. PEDIATRICS Vol. 122 No. 6 December 2008, pp. 1401-1404 (doi:10.1542/peds.2008-2624)
4. www.eyesite.ca/7modules/Module5/html/Mod5Sec1.html - Good pictures of cataracts, retinoblastoma and glaucoma
5. http://www.patient.co.uk/doctor/Vision-Testing-and-Screening-in-Young-Children.htm
6. http://www.bartsandthelondon.nhs.uk/docs/poster_red_reflex_print.pdf Poster of red reflexes and referral pathway from Bart’s and The London.

Tags: 6 week check, newborns, ophthalmology
Category: For General Practitioners | Comment

Torticollis

Paediatric surgery at Whipps Cross

GP version of February 2011′s Paediatric Pearls

Checking the red reflexes

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