March 2016: a few odds and ends on asthma this month and assessing a child in an acute exacerbation, Childline survey, Meningococcus W and paediatric neck lumps. Do leave comments below:
BCG Lymphadenitis with thanks to Dr Mujahid Hassan
Lymphadenitis is the most common complication of BCG vaccination, and is of two types – suppurative and non-suppurative.
Normal course post-vaccination:
Intradermal injection -> local multiplication of vaccine -> transport to lymphatics via lymph glands -> haematogenous dissemination of BCG.
No clear definition of ‘BCG lymphadenitis,’ proposed definition is when it becomes palpable or concerning for parents.
Can appear as early as two weeks after vaccination, most within 6 months and almost all cases will be within 24 months.
Normally ipsilateral with one or two palpable lymph nodes, but can involve multiple nodes. Normally axillary but can be with cervical/supraclavicular.
- Isolated lymph node enlargement
- BCG vaccination to ipsilateral side
- Absence of tenderness or heat to lump
- Absence of fever
Non-suppurative will resolve within a few weeks – this is a normal reaction and most of these are sub-clinical so go unnoticed.
Suppurative involves an enlarging lymph node with fluctuant appearances, oedema and erythema. Happens in ’30-80%’ of cases of lymphadenitis.
Treatment of suppurative lymphadenitis:
Antibiotics: Previously erythromycin/rifampicin/isoniazid have been used but their clinical role is of dubious significance, so are not used routinely.
Reassurance and followup are what is needed.
Fine Needle Aspiration: Suppurative lymphadenitis can result in spontaneous perforation and sinus formation, which can result in several unpleasant months of dressing and wound care. FNA is thus recommended to prevent this and reduce time for healing.
Surgical excision: Risks of general anaesthesia - other than in extreme cases of failed FNA/multiloculated lymph nodes - far outweigh the potential benefits.
Management pathway and images courtesy of:
WM Chan, YW Kwan, CW Leung. Management of Bacillus Calmette-Guérin Lymphadenitis, Hong Kong Journal of Paediatrics (New Series). Vol 16. No. 2, 2011, available via http://www.hkjpaed.org/details.asp?id=782&show=1234
J Goraya and V Virdi, Bacille Calmette-Guérin lymphadenitis, Postgrad Med J. 2002 June; 78(920): 327–329,
available via http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1742390/pdf/v078p00327.pdf
Neglect and emotional abuse is the safeguarding topic this month. ED advice on the management of minor head injuries, a report from BPSU in hypocalcaemic fits secondary to vitamin D deficiency, the new UK immunisation poster and a bit on crying babies. Hope you find it all helpful. Comments welcome below
With thanks to Dr Nikolina Kyprianou for this article and to Mr Devesh Misra, consultant paediatric urologist at the Royal London Hospital, for checking it.
Hypospadias is a congenital abnormality where the male urethral opening is displaced ventrally. It may be displaced anywhere within the glans, the shaft of the penis, the scrotum, or the perineum. It results from failure of the urethral folds to close during fetal development and it is often associated with abnormal penile curvature (chordee) due to disrupted development of the penile shaft. Males with severe uncorrected forms of hypospadias may have difficulties in controlling their urinary stream which may require urination in a sitting position. They can also have erectile dysfunction and infertility (due to impaired semen delivery).
The location of the urethral meatus classifies the hypospadias and its severity.
3 classes of severity:
- 1st degree: urethral opening within the glans/subcoronal (50% of cases)
- 2nd degree: urethral opening on the penile shaft (20% of cases)
- 3rd degree: urethral opening within the scrotum or perineum (30% of cases)
Hypospadias results due to disruption of the androgenic stimulation that is required for the normal male external genitalia to develop. Environmental factors have been found to be associated with hypospadias suggesting a multifactorial aetiology. These include:
- Advanced maternal age
- Pre-existing maternal diabetes mellitus
- Gestational age before 37 weeks
- History of paternal hypospadias
- Exposure to smoking and pesticides
Hypospadias is one of the most common congenital abnormalities with an incidence from 0.3 to 0.7% in live male births. It is more common in the Caucasian population and in those of Jewish and Italian descent.
The diagnosis is usually made during the newborn examination. Physical findings include:
- The appearance of two urethral openings: the first one will be in the normal position at the end of the glans, which will be a blind ending urethral pit. The second is the abnormally located true urethral meatus.
- Abnormal foreskin due to its incomplete closure around the glans leading to the appearance of a dorsal hooded prepuce.
- Abnormal penile curvature (chordee).
In 6% of cases the foreskin is normal and the urethral abnormality only becomes evident during/after circumcision when the glans of the penis is visualised.
Isolated hypospadias: this is only rarely associated with upper genitourinary tract abnormalities and therefore there is no need to
routinely perform imaging studies of the tract.
Cryptorchidism and hypospadias: these patients have an increased risk of disorders of sex development. Cryptorchidism is present in 10% of
patients with hypospadias and is higher in patients with proximal hypospadias (eg. scrotal or perineal urethral meatus). Proximal hypospadias on its own is also associated with disorders of sex development. These patients should be referred to a specialist so that they can have the following investigations:
- Pelvic USS to evaluate internal genitalia.
- Karyotype analysis.
- Serum electrolytes as a screen for salt-wasting forms of congenital adrenal hyperplasia.
Hypospadias with other organ system anomalies eg. Congenital cardiac disease, imperforate anus, limb malformations, or cleft lip. These patients require renal and bladder USS because they are at risk of upper tract abnormalities. They should be referred to a specialist who will also perform genetic testing.
Parents should be advised not to circumcise their baby in order to preserve the foreskin and optimise later surgical choices. These patients need to be referred to the local paediatric urologist who will determine the severity of the hypospadias and the need and timing for surgical correction.
The goal of surgery is to create a straight penis by repairing any curvature (orthoplasty), to create a urethra with its meatus at the tip of the penis (urethroplasty), to reform the glans into a more natural conical configuration (glansplasty), to achieve cosmetically acceptable penile skin coverage, and to create a normal-appearing scrotum. Surgery is usually performed within the first year of life because early correction is associated with improved wound
healing, reduced rate of complications and improved emotional and psychological result. The repair is usually planned as a single-stage procedure, but in infants with severe hypospadias a two-stage repair may be necessary.
With the use of modern instruments, sutures and antibiotics and by performing the procedure at a younger age, the repair of hypospadias has been successful.
For a useful leaflet for parents please follow link and download the PDF document: http://www.patient.co.uk/health/Hypospadias.htm
Baskin LS, Erol A, Li YW, Cunha GR. Anatomical studies of hypospadias. J Urol 1998; 160:1108.
Baskin LS, Ebbers MB. Hypospadias: anatomy, etiology, and technique. J Pediatr Surg 2006; 41:463.
Carlson WH, Kisely SR, MacLellan DL. Maternal and fetal risk factors associated with severity of hypospadias: a comparison of mild and severe cases. J Pediatr Urol 2009; 5:283.
Brouwers MM, Feitz WF, Roelofs LA, et al. Risk factors for hypospadias. Eur J Pediatr 2007; 166:671.
With thanks to Dr Jess Spedding for the continuation of her minor injuries series….
Minor Injuries Series, part 2: The Elbow Xray and Supracondylar fracture:
The elbow xray typically strikes fear into the heart of clinicians as there are so many centres of ossification which appear at different stages of a
child’s development and can quite easily be mistaken for bony injury. Remember that the smooth rounded appearance of a centre of ossification does not often mimic the typically sharp edges of a new fracture fragment, but even saying this, distinction can be very difficult. If you have the CRITOL (or CRITOE) acronym in your mind, it will help you to interpret the xray with a sensible approach. The image below highlights each of the six ossification centres with the typical age of the child when each centre appears:
Note: Lateral / External epicondyle are interchangeable terms, giving either CRITOL or CRITOE
Work through in order the centres of ossification – each may appear at slightly different ages in different children, but the sequence in which they
appear should always be C,R,I,T,O,L. For example in a 6 year old you would not expect the lateral condyle to have appeared yet, so if there is a bony fragment at that site, it is suggestive of a fracture.
The supracondylar fracture (of the distal humerus) is the most common upper limb fracture in young school age children. It presents almost always as
a FOOSH (fall on the outstretched hand). They are likely to be in a lot of pain, and may need strong analgesia and immobilisation (with a splint or
sling) before assessment is possible. (A good example of a strong, rapid acting, well tolerated analgesic that can be used in the Emergency Department is intranasal diamorhpine, which is made up with saline into a small volume of fluid and then given as nose drops with a syringe.)
You may well see bruising and swelling around the elbow, with tenderness around the distal humerus, but remember to check the whole limb including
clavicle (and the whole of their body if the mechanism of injury could have caused other serious injuries!)
The elbow contains numerous important neurovascular structures – your assessment must document presence of radial pulse, capillary refill time in
fingertips and function of ulnar, median and radial nerves (not possible formally in younger children, so watch to see if they will hold a toy or parents hand
and be suspicious if the xray shows a displaced fracture). Any concerns about neurovascular compromise require the fracture to be urgently reduced, either by the Emergency Department team or referral to Orthopaedics. This initial reduction is to relieve mechanical pressure of displaced bony fragments on the
neurovascular structures and will not provide the stability required for neatly aligned healing, so a trip to theatre for fixation will happen soon after.
A common classification system for supracondylar fractures is the Gartland classification. In this there are three categories of supracondylar fractures based on their radiological appearance on the lateral elbow view:
1: undisplaced – recognised as clinical suspicion plus xray evidence of fluid in the elbow joint elbow (as demonstrated by a raised anterior fat pad or
the presence of a posterior fat pad) and possibly loss of anterior humeral line (normal is when line along anterior humerus intersects middle third of
2. visible fracture which is hinging on the posterior edge
3. totally displaced
Type 1 can usually be managed conservatively with an above elbow cast and sling, whilst 2 and 3 require operative fixation.
All should be followed up by Orthopaedics in fracture clinic – typical progress is evidence of union at 4 weeks, and then the patient is encouraged to
gradually do away with the sling, to allow mobilisation without too much discomfort.
A rare but debilitating late complication of supracondylar fractures is Volkmann’s ischaemic contracture (when the brachial artery is damaged and months later the patient develops clawing of the thumb and fingers and forearm wasting).
Talipes (Neonatal Clubfoot) with thanks to Dr Mujahid Hasan and the paediatric physiotherapy department at Barts Health
Newborn babies can present with one of two types of Talipes:
- Congenital Talipes Equinovarus (CTEV or fixed/structural Talipes)
- Positional foot problems
Click here for the Whipps Cross physiotherapists and Muj’s complete, illustrated article.
The information for this topic is taken from a recent comprehensive review (August 2012) that appeared in www.UpToDate.com. Bartshealth employees can access the full text via a link from the intranet.
Ingested foreign bodies (UptoDate.com article, August 2012)
Coins — Coins are by far the most common foreign body ingested by children. Approximately two-thirds of ingested coins are in the stomach by the time of x-ray but those that lodge in the oesophagus for 24 hours after ingestion may need to be removed endoscopically as only 20-30% of these will pass into the stomach on their own. Coins that reach the stomach can be managed expectantly, and most will be passed within one to two weeks. A child who develops any signs or symptoms of obstruction, abdominal pain, vomiting, or fever, needs to come back to the ED urgently.
Button batteries — ingestions of “button” batteries are increasing and are associated with significant morbidity. Animal studies have demonstrated mucosal necrosis within one hour of ingestion and ulceration within two hours, with perforation as early as eight hours after ingestion. It may be difficult to differentiate between a disk battery and a coin on a radiograph. This distinction is most important when the foreign body is in the oesophagus, since batteries require immediate removal whereas coins may not.
Magnets — also increasing. Many of the children with complications from multiple magnet ingestion had underlying developmental delay or autism. In one case, an older child inadvertently swallowed these magnets while using them to imitate a pierced tongue. Two or more strong magnets, especially if ingested at different times, may attract across layers of bowel leading to pressure necrosis, fistula, volvulus, perforation, infection, or obstruction. Radiographs of the neck and abdomen should be performed, including a lateral view. X-rays cannot usually determine whether bowel wall is compressed between the magnets, although the finding of magnets that appear to be stacked but are slightly separated is suggestive. Management depends on the number, location and type of magnets, and on the timing of the ingestion. Ingestion of a single magnet can generally be managed conservatively with serial radiographs while multiple magnets need removing. Laxatives may help with faster bowel emptying if they are not in a place easily accessible with the endoscope.
References at www.uptodate.com.
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 . 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?
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 ), and it is also associated with other minor positional deformities. 10% of babies with torticollis have hip dysplasia.  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 . This study found that all these deformities were more likely to be observed in:
- babies with a greater birth length
- breech presentation
- 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. 
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 
Klippel-Feil syndrome – cervical spine fusion is seen along with a host of other symptoms 
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) 
‘Mimics’ of torticollis
‘Ocular torticollis’ occurs when there is 4th 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.  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).
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.
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.
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% 
- 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’ , 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  and into adulthood 
Alternatives to surgery?
A recent successful non-surgical development in treating cases resistant to physio is using botox injection.  The evidence for chiropractic treatment is weak, isolated successful cases have been described,  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,  so it is imperative that parents have consulted a doctor before they choose to seek chiropractic help.