Category Archives: Uncategorized

Benign enlargement of the subarachnoid space in infancy (BESS)

Case history: a 5-month-old boy was referred to clinic because his head circumference had jumped from below the 25th to the 75th centile and his GP felt that he had a prominent anterior fontanelle. He was developmentally normal with some noticeable frontal bossing. There had been concerns about his mother having had “hydrocephalus” when she was a baby.

Benign enlargement of the subarachnoid space in infancy (BESS)

  • usually involves the frontal lobe subarachnoid spaces
  • characterised clinically by a widened fontanelle, macrocephaly and/or frontal bossing
  • M > F
  • often a family history
  • majority are neurodevelopmentally normal
  • head circumference climbs through the centiles, plateauing on one of the top 2 centiles in late infancy
  • unclear pathophysiology
  • a transient accumulation of cerebrospinal fluid in the frontal region or delayed development or function of the arachnoid villi at the sagittal sinus?
  • cranial ultrasound / MRI show extra fluid around the brain frontally but no ventricular enlargement
  • There’s a more scientific and detailed radiological description at https://radiopaedia.org/articles/benign-enlargement-of-the-subarachnoid-space-in-infancy
  • can be complicated by chronic subdural haemorrhage possibly secondary to the stretching of subdural veins (Papasian, 2000)
  • type 1 glutaric aciduria also presents with increasing head size but these children are not developmentally normal and have other signs on their cranial imaging (Biswas, 2016)
  • more information at J Pediatr Neurosci. 2014 May-Aug; 9(2): 129–131 although I’m not convinced of the need for the follow up imaging advocated here, especially if it requires a general anaesthetic
  • The literature suggests that BESS resolves spontaneously by 2 years.
  • The macrocephaly is likely to persist

The head circumference of the baby presented above plateaued between the top 2 centiles at 10 months. He remains neurodevelopmentally normal.

Picture courtesy of Dr Abdel-Rahman Abdel-Halim, from the case https://radiopaedia.org/cases/29

Safeguarding: Run, Hide, Tell

With thanks to Nicci Wotton, safeguarding nurse consultant at Imperial College NHS Trust  for this month’s safeguarding item.
Today’s children are used to filming their lives and sharing with their friends via Snapchat, Instagram etc. Let children know what to do in the
event of a terrorist attack – 5 simple actions:
  1. Run to a place of safety
  2. Hide
  3. Turn your phone onto silent
  4. Turn off vibrate
  5. Only when safe call police on 999

Lyme Disease

The sun’s come out here in the UK and people are venturing into forests for picnics. Timely then for NICE to spoil the fun and publish its guideline on Lyme Disease (NG95, April 2018)
  • Caused by a tick-borne spirochaete of the Borrelia species, which is spread by a bite from  an infected tick
  • Ticks live in many woodland and grassy areas but only a small number carry the bacteria that causes Lyme disease
  • 2,000 to 3,000 diagnoses each year in England and Wales.
  • erythema migrans rash, examples here.
  • Flu-like symptoms to start with. Other symptoms include migratory inflammatory arthritis, uveitis, pain or numbness, trouble with memory, heart block, pericarditis
  • ELISA and immunospot testing are used for diagnosis but false negatives are possible especially in first 4 weeks
  • Treated with doxycycline or amoxicillin
Distribution map of UK cases here as part of a 2017 paper in Brit JGP on Lyme disease as a cause of Bell’s palsy in children as well as adults.

Should I treat an incidentally found high ASOT in a well child?

Antistreptolysin O is an antibody produced by Group A streptococci (GAS). Levels rise 1 – 4 weeks after an infection, peak between week 3 and 5 and may remain detectable for a few weeks after an infection. >200 is abnormal in adults, opinions differ in the literature as to whether children should have the same cut off but most clinicians use this number for everyone.
ASOT does not predict which people will get complications of GAS eg. rheumatic fever, glomerulonephritis. Click here to comment and join the discussion on when ASOT should be measured and what to do with the result

Viral Gastoenteritis: Could it be Cyclical Vomiting?

Children above the age of 5 in the UK can usually cope with viral gastroenteritis without needing medical input. They vomit a few times, move on to the diarrhoeal stage, get thirsty and a bit dehydrated and start drinking just as it all stops, thereby successfully rehydrating themselves and getting on with their lives.  So if a vomiting 9 year old is brought to us by a parent who says they’ve been admitted 4 times before for iv fluids , it is probably worth taking a closer look. The shocked, prostrate child we saw in the ED this weekend (a re-presentation) may have cyclical vomiting. More information about this here. Early treatment with anti-emetics and benzodiazepines may help avoid the need for iv fluids.

table showing stages of cyclical vomiting and therapies
Cyclical Vomiting
Schematic representation of the four phases of Cyclic Vomiting Syndrome and their therapeutic goals. Fleisher et al. BMC Medicine 2005 3:20 doi:10.1186/1741-7015-3-20 Li BUK et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Consensus Statement on the Diagnosis and Management of Cyclic Vomiting Syndrome. Journal of Pediatric Gastroenterology and Nutrition 2008; 47 : 379–393 (full text, doses etc.)

Journal Club: Lumbar Punctures in Children with Febrile Seizures

Journal Club is a revamped monthly feature in the Paediatric Pearls newsletter. I’m happy to receive submissions from any primary or secondary care journal club you are running as long as the paper is relevant to front line health professionals working with children. Please contact me through the contact page.

With thanks this week to Dr Saskia Wills who took us through a paper on the need (or not) for LPs in children with complex febrile seizures. Her full presentation is here.

In brief:

  • The definition of a febrile seizure in this paper is a seizure in a child 6 months to 5 years with a fever >38o and without an underlying CNS infection or a history of afebrile seizures
  • They occur in 2-4% of children <5yrs (peak at 12-18 months)
  • They are classified as complex if they last >15 minutes, have a focal onset, or there are multiple episodes within 24 hours
  • They are often associated with viral infections, especially HHV6
  • The risk is slightly higher in boys and those with a family history of febrile convulsion
  • 1/3 of children will have another febrile seizure in the future, but very few (2.4%) go on to have epilepsy. (The risk of epilepsy, which varies with different presenting features, is discussed here)
  • In a retrospective French study of otherwise well children presenting with complex febrile seizures, only 5 out of 839 (0.7%) had confirmed bacterial meningitis. All of these had had a prolonged seizure plus some ongoing abnormal neurology or sign suggestive of CNS infection. The study concluded that in children with complex febrile seizures but no other signs of CNS infection, LP usually isn’t necessary. The risk of proven CNS disease is higher in those under 1yr and with a prolonged seizure. This study didn’t look at children who had other risk factors for meningitis, such as immunodeficiency.

Paper studied: Guedj R1, Chappuy H2 et al. Do All Children Who Present With a Complex Febrile Seizure Need a Lumbar Puncture? Ann Emerg Med. 2017 Jul;70(1):52-62. PubMed Link.

Managing a Child with a Decreased Conscious Level

With thanks to Dr Dilshad Marikar for looking at the 2016 RCPCH material on managing a child with a decreased conscious level, prompted by his being on call when a 14 year old was brought to the ED with a GCS of 3.

The RCPCH algorithm has a child between 4 weeks and 18 years old enter the Decreased Consciousness (DeCon) pathway if the AVPU is “P” or “U” or if there is a new finding of GCS ≤ 14 which seems quite a low threshold and means that we will all need to use this guideline at some point. See: https://www.rcpch.ac.uk/system/files/protected/page/RCPCH%20DeCon%20Poster%20.pdf – incorporates how to identify and manage the situation and differential diagnoses. The full guideline is available here and the recommendations summary PDF, here. Some salient points:

  • Consider intubation if GCS < 8 and child not improving
  • Give oxygen if O2 saturation is ≤ 95%
  • Check capillary blood glucose within 15 minutes
  • Don’t overlook the possibility of NAI or safeguarding issues

Who Has Not Been Asked About Nappy Rash?

The Avon Longitudinal Study of Parents and Children (ALSPAC) study collected information about nappy rash using self-completed questionnaires answered by parents at the end of the first four weeks of their baby’s life. The study found that 25% of the babies had experienced napkin dermatitis.

NICE has a comprehensive clinical knowledge summary on nappy rash here. Salient points:

  • Skin swabs are not recommended for the management of nappy rash as the results are difficult to interpret.
  • Both Candida and bacteria (such as Staphylococcus aureus) colonize healthy skin and a skin swab may be positive when infection is not present.
  • A swab should only be taken when a secondary bacterial infection is suspected, to guide choice of antibiotic

This picture and more available on the excellent Primary Care Dermatology Society website. Compare this candidiasis picture with ammoniacal dermatitis and napkin eczema.

  • Consider using nappies with the greatest absorbency (for example, disposable gel matrix nappies)
  • Leave nappies off for as long as is practically possible. Clean and change the child as soon as possible after wetting or soiling. Use water, or fragrance-free and alcohol-free baby wipes.
  • Dry gently after cleaning — avoid vigorous rubbing.
  • Bath the child daily — but avoid excessive bathing (such as more than twice a day) which may dry the skin.
  • Do not use soap, bubble bath, or lotions. Advise about skin care.
  • Prescribe a barrier preparation to apply thinly at each nappy change, to protect the skin. Zinc and Castor Oil ointment BP or Metanium® ointment are recommended. Alternatively, white soft paraffin BP ointment or dexpanthenol 5% ointment (Bepanthen®) could be used.
  • For children over 1 month of age, consider prescribing topical hydrocortisone 0.5% or 1% cream once a day for 7 days max.

Signs of Raised Intracranial Pressure (ICP)

From APLS manual 6E

  1. Abnormal oculocephalic reflexes (avoid in patients with neck injuries):
    When the head is turned to the left or right a normal response is for the eyes to move away from the head movement; an abnormal response is no (or random) movement. See video for a demo of normal reflexes.
  2. Abnormal Posture:
    Decorticate (flexed arms, extended legs)
    Decerebrate (extended arms, extended legs)
    Posturing may need to be elicited by a painful stimulus
  3. Abnormal pupillary responses: unilateral or bilateral dilatation suggests raised ICP
  4. Abnormal breathing patterns: There are several recognisable breathing pattern abnormalities in raised ICP. However they are often changeable and may vary from hyperventilation to Cheyne-Stokes breathing to apnoea
  5. Cushing’s Triad: Hypertension, Bradycardia and breathing pattern abnormalities are a late sign of raised ICP

Winter, Vitamin D and Rickets

It’s dark and sun-less again in the UK and everyone’s Vitamin D levels will be at rock bottom over the next couple of months. Rickets is not rare in London and neither are consequent hypocalcaemic fits in our babies and teenagers unfortunately. Hackney CCG has an easy to follow algorithm for prevention and management of Vitamin D deficiency: you can find it here.

There’s even a table which tells you which vitamin preparations are suitable for vegetarians or vegans, which are Kosher and Halal certified and which to avoid in peanut allergy.