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Encephalopathy with Status Epilepticus during Sleep (ESES)

General paediatrics, Neurology

Poorly understood complication of some epilepsies.

Clinically, global regression esp cognitive and behavioural, associated with paroxysmal epileptic activity during sleep, which may of course not be recognized.  The classic example is Benign Rolandic Epilepsy, where continuous spike waves develop druing slow wave sleep (CSWS).  Also seen in Landau Kleffner syndrome.

The problem can be unmasked by anti-epileptic medication, especially carbamazepine.  Appears to be associated with brain pathologies eg polygyria, migrational disorders plus some chromosomal problems eg 8p-.

Diagnosis should be considered when unexpected cognitive impairment (eg memory, temperospatial skills, language) or behaviour changes (eg hyperactivity, aggression, disorientation).  Motor impairments eg ataxia and dystonia have been described.

Investigations

Dramatic increase in EEG abnormalities of any kind during sleep.

Treatment

Traditional plus newer AEDs have been used, but evidence does not point to any one being superior to any other.

Steroids appear to be effective – unclear whether ACTH or hydrocortisone better.

Outcome

Poor prognosis (ie long term neuropsychiatric problems) appear to be associated with longer duration of ESES, plus frontal neuropsychological deficits and frontal EEG anomalies.

[Epilepsy Research and Treatment 2012 http://dx.doi.org/10.1155/2012/642725]

Benign Rolandic Epilepsy (BECTS, or CECTS)

General paediatrics, Neurology

Latest name “Childhood Epilepsy with centrotemporal spikes”. Not always benign…

Common form of childhood epilepsy, with some characteristic features:

  • Seizures usually in sleep
  • If awake, then start with symptoms in one side of the mouth/tongue eg tingling
  • Speech may be impaired, with gurgling noises, as throat muscles affected – child may be trying to indicate something weird is happening to them!
  • Then twitching of face, spreading to face and arm
  • Can have tonic-clonic seizures
  • Characteristic centro temporal EEG abnormalities.  May need to be done sleep-deprived to provoke.
  • Although focal, MRI not indicated if typical features.

Treatment not always required, many children only ever have one or two seizures! Benign, because almost always goes away in puberty viz before age 16.

NICE recommends treatment with carbamazepine or lamotrigine as first-line treatment.

Be aware that carbamazepine and oxcarbazepine may exacerbate or unmask continuous spike and wave during slow sleep, which may occur in some children with benign epilepsy with centrotemporal spikes.

If these are ineffective, alternatives would be levetiracetam, oxcarbazepine or sodium valproate.

Lower limb variants

Clinical, Common, General paediatrics, OPD, Orthopaedic

Beware 2 problems, with additive or compensatory effects e.g. foot and hip!

Rotation probs ie in-toeing and out-toeing.

Metatarsus adductus is common.  Outer edge of foot is curved, and vertical line through heel misses 2nd/3rd toe space.  Rigid forms need serial casting, else 90% improve without treatment by 6-9 months of age.

Internal tibial torsion can be seen by keeping patellae parallel (sitting, or kneeling), and seeing angle of foot.  No treatment required unless severe (tibial rotational osteotomy).

Femoral ante version is more common in girls and often familial.  W posture sitting, patella points in, eggbeater pattern running.  80% resolve spontaneously, else osteotomy (but high rate of complications).

Out toeing normal in first 24 months.  Usually external tibial torsion; occ femoral retroversion.  External tibial torsion associated with patellofemoral instability.  Beware Perthes and SUFE in school age children, esp  unilateral.

Pes Planus – Flexible or rigid?  Arch reforms on tiptoeing?  Rigid suggests congenital vertical talus (rocker bottom heel) or JIA, either way, usually painful.  Beware CP or muscular dystrophy or connective tissue disorder.  Asymptomatic is considered benign.  Insoles may be useful for pain and shoe deformation, do not correct flat foot!

Angular problems ie genu varum/valgum

(Bow/knock)  Gap between knees (intercondylar distance) should be <6cm, gap between ankles (intermalleolar) should be <8cm.  Beware rickets, renal osteodysplasia, tumours, skeletal dysplasias.  Note association between high impact sport and genu varum – cause or selection?  Increased risk of injury/OA in later life…  Differential includes Blounts disease (also associated with obesity).

Knock knees exacerbated by external tibial torsion, ligamentous laxity, obesity.  Less typical pattern of rickets but seen.

[Yeo, BMJ 2015;351:h3394 – videos too]

 

Ebola persistence and recrudescence

General paediatrics, Infectious disease

Now documented that Ebola can recur, or else persist (and therefore be transmissible) from survivors.

Only 2 documented cases of recurrence.  Must be v low risk.

Where blood/urine PCR positive, always ill. Detectable in urine, sweat for up to 40 days.

Circulating neutralizing antibody would reduce risk from blood, but “immune protected” sites different eg Eyes, Pregnancy / breast milk, semen, Central nervous system.

Only 2 cases of sexually transmitted ebola.

So far no evidence of persistence beyond 1yr.

 

Lymphoedema

General paediatrics, Surgical

In adults, usually after surgery or radiotherapy.  But can be primary, appearing in childhood (but not necessarily in early childhood).  Secondary causes include Klippel-Tranaunay, Noonans, Turners, other venous malformation.

The incidence of primary lymphoedema is approximately 1 in 6,000 births. Approximately 10 children are born each year with primary lymphoedema in Scotland.

Lymphoedema, particularly if not well controlled, “carries significant human, personal, financial and societal costs”. Apart from cosmetic issues, pain is often a significant problem (underestimated), plus risk of cellulitis.  Inadequate treatment increases risk of complications:

  • fibrosis,
  • papillomatosis (warty growths consisting of dilated lymphatics and fibrous tissue)
  • lymphorrhoea (leakage of lymph fluid through the skin),
  • functional limitations
  • psychological morbidity, social isolation and limitation of life choices, including employment opportunities

So early diagnosis and control important.

Examination

Deep pressure for at least 30 seconds! If pits, then lymphatics ok.

Positive Stemmer sign= the inability to pick up a fold of skin at the base of the second toe, indicating thickening of the skin. This is useful in differentiating lymphoedema from other forms of oedema. [http://www.woundsinternational.com/ Journal of Lymphoedema, 2009, Vol 4, No 2]

Investigations

Consider secondary causes, as above.

Consider LFTs, thyroid function (impaired lymphatic drainage seen in hypothyroidism, hence oedema and even effusions).

Imaging to assess venous blood flow eg USS with dopplers.

MRI can show hypertrophy of fat, as in lipoedema (see below), or other tissues (viz Klippel Trenaunay).

Lymphoscintigraphy is gold standard but not readily available.

Differential is Lipoedema – almost exclusively females, presents at or after puberty, symmetrical lower limb enlargement.  Often a history of easy bruising and tenderness in the affected limbs. Lymphoedema can later develop as a secondary complication.

Further advice from http://www.thebls.com/patients/index.php?bls_user=9f0a85c93b84f4e36a2629f6d17c38e7

Patient support from http://www.lymphoedema.org/

[SMASAC Short Life Working Group on Lymphoedema – Lymphoedema Care in Scotland, Achieving Equity and Quality, 2013]

 

Pharyngitis treatment

Common, General paediatrics, Infectious disease,

Pencillin is generally recommended, as resistance in group A streptococcus is unheard of, and risk of rash with amoxicillin if actually Epstein-Barr.  For group A streptococcus a 10 day course has better microbiological clearance but probably no benefit clinically to 5 days.

For scarlet fever, treat for 10 days with any antibiotic except azithromycin (5 days).

Comparative efficacy of antibiotics vs gp A strep pharyngitis – Seventeen trials (5352 participants) were included; mixed adults and kids?

  • no difference in symptom resolution between cephalosporins and penicillin (intention-to-treat (ITT) analysis; N = 5; n = 2018; odds ratio for absence of resolution of symptoms (OR) 0.79, 95% confidence interval (CI) 0.55 to 1.12).
  • Clinical relapse was lower with cephalosporins (N = 4; n = 1386; OR 0.55, 95% CI 0.31 to 0.99; overall number needed to treat to benefit (NNTB) 50), but found only in adults (OR 0.42, 95% CI 0.20 to 0.88; NNTB 33).
  • There were no differences between macrolides and penicillin. Children experienced more adverse events with macrolides (N = 1, n = 489; OR 2.33; 95% CI 1.06 to 5.15).

Evidence is insufficient to show clinically meaningful differences between antibiotics for GABHS tonsillopharyngitis. Based on these results and considering the low cost and absence of resistance, penicillin can still be recommended as first choice.  But not much logic in replacing any other antibiotic with penicillin! [Cochrane Database of Systematic Reviews. 4:CD004406, 2013. UI: 23633318]

Short courses (3-6 days) of amox, co-amox, cefuroxime/cefixime (cefalexin not studied), macrolides etc are actually better than standard 10 day course of penicillin, but more expensive.  [ Cochrane Database of Systematic Reviews 2012, Issue 8. Art. No.: CD004872.]

SAPG 2022 recommends penicillin but if shortage amoxicillin then flucloxacillin. For penicillin allergy, clarithromycin preferred, then erythromycin, then azithromycin. Third line cefalexin, then co-amoxiclav, then co-trimoxazole.

Diabetic Ketoacidosis

Emergency medicine, Endocrinology, General paediatrics

DKA – BSPED guidance 2021.

The potentially serious acute complication of diabetes.  In the absence of adequate insulin, glucose levels start to rise in the blood, spilling over the threshold for kidney resorption and causing a diuresis.  Metabolism switches to ketone bodies, causing acidosis.

Presents with weight loss, tiredness, vomiting, heavy breathing (Kussmaul), reduced consciousness.  Diabetes symptoms of course, if first presentation, which might just be new wetting, or unusually heavy nappies.  Can be confused with pneumonia, or appendicitis! Often missed diagnosis.  In 2020 Lanarkshire audit, half had seen GPs at least once before diagnosis, with many having had bloods done rather than BM, or being asked to hand in urine the next day… 40% had BM done by family member!

Traditionally 15-17% of new presentations of diabetes are DKA, but with pandemic went up to 66%.

ISPAD def DKA = Bicarbonate pH under 7.3 (H+50) PLUS ketones 3+ (blood or urine).

Beware can develop with normal glucose levels IN THOSE TAKING INSULIN.  Suspect if blood ketones above 3 in known diabetic, refer to hospital.  Between 0.5 and 3, follow sick day rules.

Mild (over 7.2 or 63) vs moderate (7.1-7.2 or 79) vs severe (79+) categories.  Treat as 5 vs 7 vs 10% dehydrated respectively.

All get 10ml/kg over 30 mins (assuming you start IV fluids) unless shocked (10/kg over 15 mins, repeat up to 40 then inotropes).

Maintenance fluids (0.9% NaCl with 20mmol KCl per 500ml bag) as per traditional formula (Holliday-Segar method) – 100ml/kg/d for under 10kg; 50ml/kg/d additionally for each kilo between 10-20kg; 20ml/kg/d for each kilo above that.

  • Calculate deficit as above. Subtract initial 10/kg bolus (but not shock boluses) and correct over 48hrs
  • ONLINE CALCULATOR (dka-calculator.co.uk) comes with disclaimer, gives fluid calculations but then prints out 16 pages which you don’t need! [KB]
  • Note SCOTSTAR has separate DKASupportDocument for those likely to need transfer

See graphic in BMJ 2016.

Complications

If acidosis not correcting, check cannula, check fluid calculations, consider sepsis.  Replace insulin syringe! Acidosis can also be caused by hyperchloraemia (hence Plasmalyte preferred at RHC – less chloride).

Risk of thromboembolism due to dehydration and immobility during recovery.

Cerebral oedema

25% mortality from cerebral oedema, 34% long term neurodisability.  Headache, irritability, agitation.  Posturing, focal neurology eg eye movements, pupil asymmetry.  Cushings triad – bradycardia, hypertension, breathing irregularity.

Hypertonic saline (2.7 or 3%, 2.5-5ml/kg over 10-15 mins) or mannitol (20%, 0.5-1g/kg over 15 mins), no preference.  

Switching

Stay on DKA pathway (and not transfer to subcutaneous insulin) until:

  • The patient has been reviewed by a Consultant, or paediatric diabetes team medical staff and
  • The patient has no evidence of dehydration has no nausea or vomiting for 6 hours, and
  • Has blood glucose less than 10mmol/l, and
  • The blood ketones have fallen below 0.9mmol/l

Panton Valentine Leucocidin

General paediatrics, Infectious disease

PVL for short.

Cytotoxin associated with Staph SCCmec type IV (this is the cassette that contains mecA), which is the one sensitive to most non-beta lactam antibiotics, but in the US many MRSA too.

Causes lysis of neutrophils and macrophages.  But clinical effects probably through immune dysregulation.

Associated with higher virulence eg necrotizing, multifocal disease eg osteomyelitis, sepsis, multiorgan failure.

Should be treated with combination of beta lactam and protein synthesis blocking antibiotic (clindamycin, linezolid, rifampicin or gentamycin).  Some evidence to support addition of daptomycin.

Surgery often required to remove foci of infection in necrotic tissue.

Decolonisation therapy with chlorhexidine body wash and mupirocin nasal cream recommended.

Chronic Recurrent Multifocal Osteomyelitis

General paediatrics, Infectious disease, Orthopaedic

Adolescents, longbones, probably 1% of all osteomyelitis. ?mech – biopsies usually negative on culture! Pain, ?arthritis if adjacent to focus. Associated with:

  • pyoderma gangrenosum
  • uveitis
  • palmar plantar pustulosis (as in psoriasis)
  • IBD (may come later)

ie inflammatory, autoimmune sounding. Osteolytic & sclerotic lesions on XR, hotspots on bone scan. Histology not characteristic. Differential diagnosis is Langerhans cell histiocytosis.

Rx NSAIDs, steroids if severe/recurrent, ?pamidronate, ?infliximab.

Self limiting but relapsing over 2-4 yrs. 1 case series showed high rate of deformity, but the largest found no long term complications at all!