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Plagiocephaly

Clinical, Common, General paediatrics, OPD

Differential

The clinical criteria for a unilateral lambdoid synostosis consist of an ipsilateral occipital flattening, a depressed ipsilateral ear lobe (inferior movement) and a parallelogram-like shape in the posterior view. All three of these signs were present in the eight synostotic infants. Furthermore, all children had developed a compensatory contralateral parietooccipital bulging that led to a slanted tree top-like shape of the head at follow-up. Normal posterior view (ie ears level) and anterior movement of the ear excludes LS [but photo looks like ipsi anterior movement in LS – is it contralat in PP??? No mention of anterior bossing, not obvious in photo].

German study – all LS cases obvious clinically. Where positional plagiocephaly was doubted, USS demonstrated patent sutures.

[Arch Dis Child 2015;100:152-157 doi:10.1136/archdischild-2014-305944]

Monitoring

Measure the oblique diameter left (ODL) and oblique diameter right (ODR) lines are drawn from points located 40° either side of the antero-posterior (AP) line. 40° is typically where deformation most notable.  Express as difference (the Oblique diameter difference (ODD) = ODL−ODR) or else ratio between the ODL and the ODR (oblique diameter difference index, or ODDI).

[European Journal of Pediatrics March 2006, Volume 165, Issue 3, pp 149-157]

Treatment

Dutch RCT of 6 months of helmet therapy (n=84 infants aged 5 to 6 months with moderate to severe skull deformation, exclusions were prems, muscular torticollis, craniosynostosis, or dysmorphic features). Full recovery was achieved in 10 of 39 (26%) participants in the helmet therapy group and 9 of 40 (23%) participants in the natural course group (odds ratio 1.2, 95% confidence interval 0.4 to 3.3, P=0.74). All parents reported one or more side effects.

[van Wijk RM BMJ 2014; 348 (); g2741]

Some evidence for bedding pillows (but SUDI risk?) and stretching exercises.

Haemangiomata

Clinical, Common, Dermatology, General paediatrics

2018 classification (ISVVA.org) – rather functional and lacking in poetry!

Basically benign tumours, involving blood vessels.  Seen in 12% of all infants  – more common in girls, whites, premature infants, twins and are babies born to mothers of higher maternal age!  Mostly seen in head and neck region, including the face, but can be anywhere.

Tumours distinguished from malformations.

Infantile Haemangioma

Cutaneous/mucosal haemangiomata usually develop after birth, appearing in the first 8 weeks of life.  They then develop and grow for 6-12 months, often resembling a strawberry.  Most then start to reduce and fade gradually, although it can take up to 9 years.   Often there will be complete disappearance with no cosmetic defect, but there may well be scarring, telangiectasia, or loose fibro-fatty tissue.

Can be further differentiated by depth (superficial tend to be raised and bright red, deep are generally darker red or even purple/blue, they can also be mixed) and extent/pattern (focal or segmental).

Typically they are in the skin and soft tissues, but can sometimes affect the liver or airways.  Associated with GLUT-1 positive staining on biopsy.

Congenital Haemangioma

Much less common. Present at birth and do not progress, although they may grow proportionally with child.  Oval or round, plaques or exophytic.  Some rapidly involute during the first year of life but otherwise they are permanent.

Vascular malformations

Grow slowly compared with vascular tumours.  Usually present at birth but perhaps inconspicuous until child grows.  Can involve arteries, veins, lymphatics in various combinations.

Capillary malformations most common – dilated capillaries, classic port wine stain (naevus flammeus).  Darken over time, do not regress.  Can be associated with bone or soft tissue overgrowth. Multiple can be associated with underlying AVM!

Nevus simplex is the classic “stork bite” at the nape, eyelid or forehead at birth. Lighter, regress.

Venous malformation more ill defined, bluish, easily compressible.  Multifocal tend to be autosomal dominant.  Some syndromes eg Blue rubber bleb naevus syndrome (widespread, including palms/soles).

Lympoedema and cystic hygroma are the lymphatic versions.

Others

  • Pyogenic granuloma – reaction to trauma, well demarcated, raised or even pedunculate
  • Telangiectasia eg Hereditary haemorrhagic telangiectasia (HHT)
  • Angiokeratoma – characteristic of tubersclerosis
  • PHACE syndrome (post fossa malformations, haemangiomata, arterial anomalies, cardiovascular defects, eye anomalies – but also midline defects)
  • Tufted angioma and Kaposiform haemangioendothelioma – similar histologically, but latter bruised, purpuric appearance, infiltrate into muscle/adipose tissue and associated with Kasabach-Merritt syndrome (consumptive coagulopathy).
[Seminar intervent radiol 2017][Ped dermatology 2016]

Leadership

Generic, Management

Leadership is not the same as management: yes, it’s about people and systems and getting things done.  But it’s more about inspiration, long term goal setting, encouraging people in their own journeys.

Can all be a bit alpha and masculine.  Yet lots of evidence that a compassionate style is more effective.  Study by Jonathan Haidt (New York University) shows that if employees are moved by the compassion or kindness of their leaders (a state he terms elevation), the more loyal they become to him or her, even if it isn’t directed at them personally.

We are especially sensitive to signs of trustworthiness in our leaders, and react strongly to “arsehole” behaviour.  

Not only does an angry response erode loyalty and trust, it also inhibits creativity by jacking up stress levels. Positions of power tend to lower our natural inclination for empathy, so it is particularly important as a leader to be self aware, and actively practice seeing situations form their employee’s perspective.

Key challenges – junior doctor training, MCNs, HEAT targets, centralization vs local demand.  Opportunities: improvement, efficiency.

Circle of influence (Steven Covey) – small subset of circle of concerns.  Note potential for stress and disillusionment in face of concerns, at time of need for motivation and creativity!  Always potential for extending circle of influence…

SWOT analysis: strengths, weaknesses, opportunities, threats.  Build on strengths, mitigates weaknesses, capitalize on opportunities, tackle threats head on.

To maintain trust and confidence – stay in touch, know your people.  Have a common platform rather than being seen as separate.

  • Direction and purpose – conflicts?  Wrong activities?
  • Align systems/processes – bureaucracy?  Slow processes? Going through the motions?
  • Know the people – do they say what you want to hear rather than being honest?
  • Release potential – and frees up your own time!
  • Influence and communicate – perception does not always equal reality

Transactional vs transformational styles:

  • Problem solving                   Coaching
  • Power based authority                        Influencing but no authority
  • Conservative                           Creative
  • Lack of growth                       Woolly
  • Work harder as philosophy     Change for change’s sake
  • Vulnerability to change
  • Thorough
  • Safe

Vision – relevant, strategically worthwhile over years, concordant – should stretch capabilities and self-image

Determines Mission: standards and values

Then in turn Goals (organisational), Strategies, Action plans

Having the broader goals and strategies helps services align, and allows stepwise change within a comfort zone rather than radical revolution with panic

SMART Plus objective – specific, measurable etc Plus clarification about why it’s important, acknowledgement and recognition.

Barriers should be flagged up as next steps – need to keep a “wildly important goal” (WIG) on the agenda of each meeting to maintain perspective.

Individual responsibility for health

Ethics, Generic, Patients

Deciding how to distribute health care costs may include looking backwards at what behaviours have contributed to a condition (eg tattoo removal may not be publicly funded, but removal of a disfiguring skin lesion where suffering is equivalent is), or may look forward to how behaviour might affect the effectiveness of a treatment (eg liver transplant with continued alcohol excess).  Sometimes looking forward and looking backwards have the same outcome, but not necessarily.

There are a number of arguments against these attitudes:

  • Humanitarian – a patient’s suffering should be addressed, regardless of the circumstances
  • Libertarian – denying treatment is likely to lead to even worse consequences, with eventual loss of political and civic participation (which is a societal good, as per JS Mill)
  • Fairness – although certain behaviour may increase the risk of a negative health outcome, other factors also play a role which are outside individual control, and rarely straightforward to establish causality.
  • Practical – if a doctor makes decisions based on behaviour, it encourages intrusiveness on their part, and defensiveness on the patient’s part, both impact on doctor-patient relationship
  • Moralistic – who decides which behaviours are acceptable and which not? Rarely non-judgmental

The liberal egalitarian response is to hold individuals responsible for their choice, but not for the consequences of their choice.  The egalitarian view is that everyone should have equal opportunities, regardles of their natural or social advantages/disadvantages at birth.  Of course, it can often be debated whether “choice” is ever truly distinct and independent of circumstance!  The liberal view is that there should be no formal or informal barriers (although not necessarily compensation for the disadvantaged).

So it would be appropriate to tax smokers an amount related to the increased health costs of smoking.  It would not be fair to tax some smokers more than others, even if the costs of their treatment might be more – it is the choice that matters.  This avoids all the objections above, apart from the moralistic one: but at least decisions on lifestyle taxes are made democratically, not by health care providers.

Does not solve the problem of whether behaviours can truly be considered a choice, when they are often predictable based on socio-economic factors.  Plus, not all types of behaviour can be taxed – physical inactivity?  Poor health care seeking behaviour?  Unsafe sex?

Cappelen and Norheim, J Med Ethics 2005;31:476–480. doi: 10.1136/jme.2004.010421

Significant Event Analysis

Clinical governance, Generic, Patients

Traditional M&M (mortality and morbidity) meetings – Many errors are not reviewed, and the key protagonists often not present when a case is being discussed; fail to engage affected families. This lack of transparency in the context of the Francis report is at odds with our duty of candour to patients when things go wrong.

Much energy is spent in the NHS concluding whether errors, adverse incidents and deaths are ‘avoidable’ or ‘preventable’.

‘Avoidability’ is an arbitrary conclusion – what matters, surely, is the care that the child received. Professional analysis of the care given reassures parents that their child’s life is of primary importance, and may provide some comfort that their experience will benefit other children.

Root cause analysis (RCA) tracks the origins of an adverse event back to find causes – too simplistic?

cf ‘Safety-II’ approach – focuses on understanding how things usually go right, and only then exploring why things occasionally go wrong.  Rare serious events, although easy to identify, often have complex aetiology, and factors may be difficult to modify. In contrast, “normal” behaviour may be easier to understand and to influence.

Parents’ own questions should inform professional discussion.  Analysis should go beyond identifying what the child died from, to considering why a child died of that condition, in that place,  at that time.

“The investigation of medical error, adverse events and child mortality each requires a distinct approach that revolves around a continuous cycle of reporting, professional scrutiny and follow-through of SMART actions. These processes should separately feed into a properly formatted clinical governance meeting, the purpose of which is to provide assurance to hospital boards and other regulatory bodies that there exists coordinated oversight of risk management, clinical effectiveness, audit and patient experience.”

[James Fraser, Bristol – Arch Dis Child doi:10.1136/archdischild-2015-309536 ]

Angioedema

Allergy, Clinical, Common, General paediatrics, Immunology

Swelling, usually acute, non-pitting.  May be erythema too.  Typically affects face, especially lips, tongue, eyes, but can be limbs, even internal!

Usually related to urticaria (wheals). As with urticaria, can be allergy – clue is consistent trigger, pattern of recurrent episodes – but can have other causes.

Angioedema without urticaria – consider hereditary or drugs, especially NSAIDs and ACE inhibitors.

Overdiagnosis

Generic, Health Literacy, Patients

Online survey of 400 people aged 50-70, only 2.6% of people could explain what overdiagnosis means.

Definition: when a disease is detected that would not cause any harm during the lifetime of the patient.

Without better public understanding, difficult for them to make informed decisions about their health.

Ghanouni et al. A survey of public definitions of the term ‘overdiagnosis’ in the United Kingdom. BMJ Open 7 April 2016.

Autoimmune Hepatitis

Gastro, General paediatrics, Immunology

Presents with anything from subtle anorexia, fatigue, rashes, abdo/joint pain to acute liver failure.  Jaundice does not relate to degree of histological fibrosis.

Check Prothrombin time, glucose, ammonia, lactate to monitor liver disease. Response to Vitamin K at 8 hours is prognostic, so refer to specialist centre if poor.

Type 1 (60%) is ANA/SMA (=small muscle) positive, usually presents as a viral hepatitis ie jaundice, raised transaminases, but can present insidiously, even with established portal hypertension, or as acute on chronic. Type 2 is LKM1 (liver/kidney/microsomal) positive, similar clinical presentation but probably more jaundice and cirrhosis, less impairment in synthesis. Seronegative hepatitis has been described in up to 20%.  Antibodies to soluble liver antigen described.  Quite common to see pANCA, too (not sensitive or specific).  The role of these autoantibodies in disease is unclear!

As with other autoimmune diseases, strong association with HLA types.  DR4 associated with less severe disease, lower rate of relapse (but older presentation).  Has been reported in association with immune disorders eg autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED).

Alkaline phosphatase rarely exceeds 4x normal and generally remains less than 2x normal. Raised immunoglobulins are a clue, with a selective increase in IgG (up to 3x higher than the upper level of normal) seen in 75-85% (kids and acute less likely).  But not specific – also seen in Wilson’s disease; other causes should be excluded too. Interface hepatitis on biopsy, with sharp differentiation between inflammatory zone and normal liver tissue.  Findings don’t always match biochemistry.  Nonetheless, diagnosis is ultimately clinical!  Diagnostic scoring system available.  Differential also includes chronic hepatitis C and drug induced liver injury.

Treat with steroids, azathioprine (remember TPMT polymorphisms)  when improving, else mycophenylate. 85% achieve remission, biochemically within 6-12 weeks, histologically 6-12 months.  More than double normal enzymes is an indication for treatment, else bridging or multilobular necrosis.  Progressive fibrosis is associated with liver inflammation, and poor response to treatment (inability to suppress inflammation within 12 months) is associated with progression to cirrhosis (54%) and transplantation (15%).  But fibrosis can be stopped or slowed in the majority, and even cirrhosis can regress with treatment.  Aim is normal transaminases, immunoglobulins, histology – biopsy is still gold standard for assessing fibrosis.

Unlikely to outgrow.  Some trials of withdrawing treatment after remission, only a minority manage more than a year without relapse.  Azathioprine 2mg/kg daily effective.  Ciclosporin and MMF are second line agents.  Hepatobiliary cancers and lymphoma risk is increased and should be screened for.

Transplant may be necessary eg severe acute presentation with poor response to treatment.  Results are good, recurrence is rare but well described.

Variants

Can be features of other disorders eg primary biliary cirrhosis, primary sclerosing cholangitis.  Biliary changes are commonly seen in autoimmune hepatitis in any case.

Can be part of polyendocrine syndrome type 1.

[Heneghan, Lancet 2013; 382: 1433–44 http://dx.doi.org/10.1016/S0140-6736(12)62163-1]

See also PMID 22495399

Valproate – MHRA warning

General paediatrics, Neurology,

Updated December 2023 –

High risk of serious developmental disorders if exposed in womb (up to 30-40% risk, including 5x higher risk of autism) and congenital malformations (10%), including:

  • Spina bifida
  • face/skull malformations, including cleft lip/palate
  • Limb, heart, kidney, genital abnormalities
  • Deafness

In boys, pre-clinical data on transgenerational risks, and animal studies suggesting infertility.

Oral Valproate must not be started in new patients (male or female) younger than 55 years, unless two specialists independently consider and document that there is no other effective or tolerated treatment, or there are compelling reasons that the reproductive risks do not apply.”

Patient guide and checklist available.

Testing for antibiotic allergy

Allergy, General paediatrics

See also Penicillin allergy.

Systematic review – just 0.21% of unselected general paediatric outpatients exhibit positive antibiotic allergy tests, and only 6.8% of those with suspected allergy test positive.

No evidence to support using skin prick testing.  Intradermal testing has high false positive rate (64-67% for penicillin and clarithromycin). Caubet did oral provocation test (OPT) regardless of intradermal result to beta lactam, NNT=11 to avoid one OPT! [Ped All Immun 2015 ]. OPT reactions tend to be cutaneous and mild, usually more than 1hr post administration.

Where reaction is severe but non-immediate, eg Stevens Johnson syndrome, Toxic Epidermal Necrolysis (TEN), Drug Rash with Eosinophilia and Systemic Symptoms (DRESS), care needs to be taken with investigation, but studies have reported intradermal and OPT without unsafe adverse effects.  If reaction is anaphylaxis with first dose, then OPT contraindicated.

Wide variety in regimens.  Suggests 1-5 incremental doses of amoxicillin for mild reactions (timing not specified), giving cumulative dose appropriate to child, then continuing for 3 days.  Skin testing may be appropriate for severe reactions according to risk:benefit balance.  Check asthma well controlled, no antihistamines.  Warn that low risk of false negative result (absent co-factors) and low risk of re-sensitization.

Intravenous provocation only where PICU!

[Marrs, ArchDisChild 2015]

Macy article says any rash can have OPT! 5mm pos SPT for penicilloyl-polylysine has good negative predictive value for anaphylaxis with OPT. Recommends 5 days amoxicillin.

[Ann Allergy Asthma Immunol 121(2018):523−529]

Mirakian article suggests SPT for all immediate reactions! Split dose challenges, with a week between first and second doses!

For non immediate reactions (1-72hrs), OPT confirmed in 59%, ID less than 40%.

6 studies showing that benign reactions (ie witnessed macpap or urticarial, no pain/burning, <50% skin surface etc) do not need skin testing.  Geneva have done more than 800 straight to OPT.  New EAACI guidelines in press.

If delay in reaction is unclear, assume immediate.  SPT vs amoxicillin, PPL, MDM.  IgE vs BPL.  0.04ml ID volume.  Note different reference ranges!  See Brockour, Allergy 13

Recent letter claimed OPT after skin test was “unnecessary, dangerous, unethical”!  But 30-100% false negatives!

Clavulanate allergy described.

Test sensitivity falls more than 4/12 after episode, ideally do within 4-6/52????

Basophil activation test using flow cytometry looking promising for IgE mediated drug reactions.  EAACI interest group working on Drug Allergy Passport.