Pediatrics

Preoperative

• Carbohydrate loading (400 mL oral carb drink 2 hours before)
• No prolonged fasting
• Nutritional optimization, prehabilitation

Perioperative

• TIVA or low-MAC volatile agent
• NDMR reversal with sugammadex
• Epidural analgesia
• Goal-Directed Fluid Therapy (GDFT)
• Avoid nitrous oxide
• Normothermia
• Lung-protective ventilation

Postoperative

• Early extubation
• Early feeding (day 1)
• Early mobilization
• Remove drains early
• Multimodal analgesia (minimize opioids)
• Thromboprophylaxis

HRS Diagnosis

• Creatinine >133 µmol/L (1.5 mg/dL)
• No hypovolemia, no nephrotoxic drugs
• No improvement with albumin challenge
• No proteinuria >500 mg/day

Type 1 HRS (rapid, <2 weeks) vs Type 2 HRS (gradual, resistant ascites).

Stabilization

• Vasoconstrictors (Terlipressin 1–2 mg IV q6h or Norepinephrine) + Albumin infusion (1 g/kg on day 1, then 20–40 g/day)
• Biliary decompression URGENTLY if cholangitis + AKI

Intraoperative

• Invasive monitoring essential, vasopressors at ready
• Target MAP >75 mmHg (higher threshold in renal compromise)
• Mannitol for renal protection
• AVOID hypotension at all costs

Anesthetic Drug Modification

All drug doses reduced significantly; cisatracurium preferred; remifentanil intraoperatively; paracetamol only analgesic postoperatively.

Mannitol 20% solution, dose: 0.25–0.5 g/kg IV, administered 30–60 minutes before skin incision.

Mechanisms of Renal Protection

1. Osmotic diuresis → dilutes toxic bile salts in tubules
2. Free radical scavenging → reduces oxidative injury
3. Renal vasodilation
4. Maintains tubular patency
5. Reduces tubular cell swelling

Additional Benefits

• Reduces hepatic ischemia-reperfusion injury during Pringle maneuver
• Reduces cerebral edema

Monitoring & Contraindications

Monitoring: Serum osmolality (target <320 mOsm/L), urine output.

Contraindications: Established AKI (anuria), CHF, severe dehydration.

WHO Classification of Dengue (2009 Revised Classification)

The WHO 1997 classification (DF/DHF Grades I–IV/DSS) was revised in 2009 because many severe, life-threatening presentations did not strictly meet DHF criteria and were being missed. The 2009 classification is now the globally preferred, clinically actionable system, dividing dengue into three categories based on presence of warning signs and severity features.

Principles of Management

1. Initial Assessment and Triage

• Careful history: date of fever onset (critical for staging — defervescence around day 3–7 coincides with onset of the critical/leakage phase), warning signs, bleeding, urine output, co-morbidities
• Examination: hydration status, capillary refill, pulse volume, pulse pressure, postural BP change, hepatomegaly, signs of effusion/ascites, rash, bleeding manifestations, tourniquet test
• Baseline investigations: complete blood count with haematocrit and platelet count (trend more important than single value), NS1 antigen (positive from day 1, most useful in first 5 days) and/or IgM ELISA (positive typically after day 5), liver function tests, and further work-up guided by severity

2. The Three Phases of Dengue Illness

3. Fluid Management – The Cornerstone of Therapy

• Dengue without warning signs: encourage adequate oral fluids (ORS, fruit juice, plain water); outpatient management with advice to return immediately if any warning sign develops; daily review if possible, especially around the time of defervescence
• Dengue with warning signs: hospitalise; start IV isotonic crystalloids (Ringer's lactate/normal saline) at 5–7 mL/kg/h, titrated hourly against vital signs, urine output, and haematocrit trend; reduce infusion rate as the patient stabilises, generally not exceeding 24–48 hours of the critical phase
• Severe dengue with shock (DSS): immediate IV bolus of isotonic crystalloid 10–20 mL/kg over 15–30 minutes; reassess; if no improvement, may repeat bolus or switch to a colloid (e.g., 6% dextran/hydroxyethyl starch); once stabilised, taper fluids progressively over the following 24–48 hours as leakage resolves — over-hydration once the leaking phase ends risks pulmonary oedema/fluid overload, so fluids must be actively DE-escalated, not simply continued
• Meticulous monitoring during the critical phase: vital signs and urine output hourly (in shock)/2–4 hourly, haematocrit every 4–6 hours, strict input-output charting

4. Other Management Principles

• Antipyretics: paracetamol only for fever/pain – STRICTLY AVOID NSAIDs (ibuprofen, aspirin) due to bleeding risk and aspirin's association with Reye syndrome
• Platelet transfusion: NOT given prophylactically for low platelet count alone in the absence of bleeding — reserved for significant active bleeding with severe thrombocytopenia, since prophylactic transfusion has not been shown to prevent bleeding and carries transfusion-related risk
• Blood transfusion: for significant, confirmed blood loss (e.g., overt bleeding with falling haematocrit/haemodynamic compromise) — a falling haematocrit WITH clinical deterioration suggests bleeding, not just haemodilution from fluid therapy, and should prompt transfusion
• Avoid unnecessary intramuscular injections and invasive procedures (risk of bleeding at puncture sites)
• Monitor for and manage complications: encephalopathy, myocarditis, acute kidney injury, secondary bacterial infection, and fluid overload during the recovery phase
• Discharge criteria: afebrile for ≥24–48 hours without antipyretics, clinical improvement, rising and stabilising platelet count, stable haematocrit, adequate urine output, no respiratory distress

International (Grading) Classification of VUR

Grading is based on the appearance of the collecting system on voiding cystourethrogram (VCUG), the gold standard investigation for diagnosing and grading VUR.

Management of a Child with VUR

• General measures for all grades: prompt treatment of any UTI, regular bowel and bladder management (treat constipation and voiding dysfunction, which significantly worsen reflux and breakthrough infection risk), maintenance of good hydration, and periodic surveillance for renal scarring (DMSA scan) and growth/blood pressure monitoring
• Grade I–III (low grade): managed conservatively — most resolve spontaneously with growth as the submucosal ureteric tunnel lengthens; continuous antibiotic prophylaxis is considered selectively (particularly in infants, those with bladder-bowel dysfunction, or recurrent febrile UTI) rather than uniformly for every child, reflecting evidence (RIVUR trial) of modest benefit balanced against antimicrobial resistance concerns
• Grade IV–V (high grade) or breakthrough febrile UTI despite prophylaxis, or progressive renal scarring: surgical correction is considered — options include endoscopic subureteric injection of bulking agent (e.g., dextranomer/hyaluronic acid copolymer) as a minimally invasive first-line surgical option, or open/laparoscopic/robotic ureteric reimplantation (e.g., Cohen, Politano-Leadbetter techniques) for definitive correction in higher-grade or persistent reflux
• Regular follow-up VCUG or radionuclide cystography to document resolution/downgrading, alongside DMSA renal scan to monitor for new or progressive scarring, particularly in higher grades
• Family counselling regarding the largely favourable natural history of lower-grade VUR, and the importance of prompt evaluation for fever in a child with known VUR

Antibiotic Prophylaxis of Urinary Tract Infection

• Rationale: continuous low-dose antibiotic prophylaxis (CAP) aims to reduce recurrent febrile UTI and consequent renal scarring in children at higher risk, chiefly infants and young children with moderate-to-high grade VUR
• Commonly used agents (given as a single low nightly dose, roughly 1/4 to 1/3 of the treatment dose): Nitrofurantoin (avoid in infants <1–3 months and in G6PD deficiency), Trimethoprim-sulfamethoxazole (avoid <2 months age due to kernicterus risk), Cephalexin (often preferred in young infants where the above are contraindicated)
• Evidence base (RIVUR and CUTIE trials): prophylaxis reduces the RISK of recurrent febrile/symptomatic UTI, particularly in children with bladder-bowel dysfunction, but does NOT show a clear reduction in new renal scarring on repeat DMSA in unselected populations — hence current practice favours a SELECTIVE, individualised approach (higher grade reflux, recurrent UTI, infancy) rather than blanket prophylaxis for every child with any grade of VUR
• Prophylaxis is generally continued until VUR resolves/downgrades to a low-risk grade, or the child is toilet-trained and bladder-bowel dysfunction is controlled, with periodic reassessment
• Prophylaxis does not replace prompt evaluation and TREATMENT (not just prophylactic dosing) of any breakthrough febrile illness in a child on CAP, which requires a urine culture and full treatment dose if UTI is confirmed

Definition of Macrocephaly

Macrocephaly is defined as an occipitofrontal circumference (OFC/head circumference) >2 standard deviations above the mean for age, sex, and gestation (i.e., above the 97th centile on standard growth charts), OR an OFC increasing across percentile lines on serial measurement. It may be due to a large brain (megalencephaly — anatomic or metabolic), increased CSF volume (hydrocephalus), subdural collections, or a thickened skull, and may be a benign familial variant or reflect underlying pathology — hence serial measurement and comparison with parental head circumference is essential.

Hydrocephalus – Investigation

• Clinical evaluation: serial OFC plotted on a growth chart, anterior fontanelle tension/size, splitting of sutures, ""setting-sun"" sign, dilated scalp veins, irritability/lethargy, vomiting, developmental regression, and (in older children with closed sutures) signs of raised intracranial pressure — headache (worse on waking/with straining), papilloedema, vomiting, VI nerve palsy
• Cranial ultrasound (through the open anterior fontanelle) – first-line, bedside, radiation-free screening tool in infants with an open fontanelle; useful for serial monitoring of ventricular size
• MRI brain – investigation of choice once the fontanelle has closed or for detailed anatomical delineation: identifies the level and cause of obstruction (aqueductal stenosis, posterior fossa lesion/Dandy-Walker malformation, tumour, Chiari malformation), degree of ventriculomegaly, and periventricular changes (transependymal CSF flow); does not require radiation, though may need sedation in young children
• CT brain – useful in the acute/emergency setting (rapid, widely available) to assess ventricular size and detect an acute cause, though less detailed than MRI for defining the underlying aetiology
• Additional work-up as indicated: TORCH screen/CMV PCR if congenital infection suspected, chromosomal/genetic testing if syndromic features present, CSF analysis if infective/inflammatory cause suspected (only after imaging has excluded a mass lesion/risk of herniation), and ophthalmological assessment for papilloedema

Hydrocephalus – Management

1. Medical/Temporising Measures

• Reserved for stabilising a sick infant, buying time before definitive surgery, or managing very slowly progressive, mild ventriculomegaly under close observation
• Serial lumbar puncture (only where communicating hydrocephalus and no contraindication) or acetazolamide and furosemide (reduce CSF production) may be used transiently, but are NOT considered adequate definitive long-term therapy in most progressive hydrocephalus

2. Surgical Management – Definitive Treatment

3. Treating the Underlying Cause

• Excision of an obstructing tumour/mass where feasible, treatment of underlying CNS infection (meningitis/ventriculitis), and management of any associated congenital malformation
• Long-term follow-up: serial head circumference, developmental assessment, and monitoring for shunt complications – mechanical malfunction/blockage, infection (most common in the first month post-insertion), and over-drainage (slit ventricle syndrome, subdural haematoma)

Definition

Cerebral (cortical) visual impairment (CVI) is visual dysfunction caused by damage to or dysfunction of the post-chiasmatic visual pathways and/or visual processing areas of the brain (optic radiations, occipital cortex, and visual association areas), occurring in a child with an otherwise structurally normal or near-normal eye examination. It is now recognised as the LEADING cause of childhood visual impairment in developed countries, most commonly resulting from hypoxic-ischaemic encephalopathy, periventricular leukomalacia (in preterm infants), traumatic brain injury, CNS infection, hydrocephalus, or structural brain malformation.

How CVI Is Diagnosed

• Diagnosis is primarily clinical, based on a history of visual behaviour inconsistent with ocular examination findings, in a child with a relevant history of neurological insult (perinatal asphyxia, prematurity, seizures, CNS infection/trauma)
• Ophthalmological examination is essential to EXCLUDE ocular causes: it typically shows normal or near-normal anterior segment, media, and fundus examination, with normal or only mildly abnormal pupillary responses — the mismatch between poor functional vision and relatively preserved ocular structure is the diagnostic hallmark of CVI
• Characteristic behavioural features reported by caregivers/observed clinically: preference for specific colours (often red/yellow), better response to moving over static targets, difficulty with visual crowding (struggles to identify an object among many, e.g., in a cluttered picture or busy room), light-gazing, absent or inconsistent visual fixation, better vision in the peripheral than central field, and marked variability of visual function with fatigue, illness, or unfamiliar environments
• Neuroimaging (MRI brain) supports the diagnosis by demonstrating structural correlates — periventricular leukomalacia, cortical/subcortical injury, optic radiation involvement, or other structural brain abnormality consistent with the clinical picture
• Electrophysiological tests (visual evoked potentials) may show abnormalities and can help differentiate cortical from purely ocular pathology, though findings can be variable and are interpreted alongside the clinical picture
• Structured CVI assessment tools (e.g., the CVI Range/Roman-Lantzy inventory) are increasingly used to systematically characterise the ten commonly described visual behavioural characteristics of CVI and grade severity, guiding individualised intervention planning

Management

• No specific pharmacological or surgical cure exists — management is centred on structured, individualised habilitation and environmental adaptation, ideally through a multidisciplinary team (paediatric ophthalmologist, developmental paediatrician, vision therapist/CVI-trained teacher, occupational therapist)
• Environmental modification: reduce visual clutter, use high-contrast and brightly coloured (often the child's preferred colour) materials, present one object/item at a time, allow additional processing time (""latency"") before expecting a visual response, use movement to attract and maintain attention
• Multisensory approach: pairing visual targets with sound/touch cues to reinforce learning, since many children with CVI also have other sensory or motor impairments requiring an integrated approach
• Educational support: individualised education plans incorporating CVI-specific strategies, low-vision aids where appropriate, and close liaison with special educators trained in CVI
• Treatment of associated conditions: refractive error correction, strabismus management, and treatment of co-existing epilepsy or other neurodevelopmental impairments, all of which commonly coexist and can further limit visual function if unaddressed
• Prognosis and follow-up: visual function in CVI can genuinely IMPROVE over time with appropriate intervention and brain plasticity, particularly when identified and managed early — unlike many ocular causes of blindness, making early recognition and structured intervention especially valuable; regular reassessment tracks progress and adapts strategies as the child develops

Background

The Duke criteria (originally 1994, subsequently modified in 2000 and further refined in 2023 by the Duke-International Society for Cardiovascular Infectious Diseases/ISCVID) are used for the clinical diagnosis of infective endocarditis (IE), combining major and minor criteria derived from microbiological, echocardiographic, and clinical findings. They apply to children as well as adults, with particular relevance in those with underlying congenital heart disease, prosthetic valves/intracardiac devices, or central venous catheters.

Major Criteria

1. Blood culture positive for IE: typical IE-causing organisms (e.g., Viridans streptococci, Streptococcus gallolyticus, HACEK group, Staphylococcus aureus, community-acquired enterococci without a primary focus) from ≥2 separate blood cultures; OR persistently positive blood cultures with an organism consistent with IE from cultures drawn >12 hours apart, or 3/3 or majority of ≥4 cultures with first and last drawn ≥1 hour apart; OR single positive culture for Coxiella burnetii or phase I IgG titre >1:800
2. Evidence of endocardial involvement: echocardiography (transthoracic; transoesophageal in prosthetic valves/complex cases/high clinical suspicion with negative TTE) showing a vegetation, abscess, new partial dehiscence of a prosthetic valve, or new valvular regurgitation (worsening/changing pre-existing murmur alone is not by itself a major criterion)

Minor Criteria

• Predisposing condition: underlying heart disease (including congenital) or intravenous drug use
• Fever ≥38°C
• Vascular phenomena: major arterial emboli, septic pulmonary infarcts, mycotic aneurysm, intracranial haemorrhage, conjunctival haemorrhages, Janeway lesions
• Immunological phenomena: glomerulonephritis, Osler nodes, Roth spots, rheumatoid factor positivity
• Microbiological evidence not meeting the major criterion (e.g., a single positive blood culture, or serological evidence of infection with an organism consistent with IE)

Diagnostic Categories

Key Points for Paediatric Application

• Congenital heart disease (especially those with prosthetic material, VSD with jet lesions, or cyanotic CHD with prior palliative shunts) is the leading predisposing factor in children, replacing rheumatic heart disease as the commonest underlying substrate in most contemporary paediatric series in developed settings, though rheumatic heart disease remains important in endemic regions
• Blood cultures MUST be drawn (ideally 3 sets from different sites) BEFORE starting antibiotics whenever feasible, as prior antibiotic exposure is a major cause of ""culture-negative"" endocarditis and diagnostic difficulty
• The 2023 Duke-ISCVID update expanded imaging modalities considered under the major criterion (including cardiac CT and PET/CT for prosthetic material) and refined the list of typical causative organisms, reflecting advances since the 2000 modified criteria

Background

Sydenham chorea (rheumatic/St. Vitus' dance) is a major manifestation of acute rheumatic fever (Jones criteria) resulting from autoimmune-mediated dysfunction of the basal ganglia (caudate nucleus and subthalamic nuclei), following molecular mimicry after group A streptococcal infection. It characteristically has a LONG latent period (up to several months) after the preceding streptococcal pharyngitis, often occurring after other features of acute rheumatic fever have subsided, and is more common in girls and in the 5–15-year age group.

Clinical Features

• Chorea: irregular, involuntary, non-repetitive, rapid, jerky movements affecting the face, tongue, and limbs, worsened by stress/excitement and disappearing during sleep — typically bilateral, though hemichorea (unilateral) can occur
• Hypotonia: often marked, may even predominate over the choreiform movements in some children (""soft"" chorea)
• Milkmaid's grip sign: irregular, relaxing/tightening grip when the child squeezes the examiner's fingers, reflecting inability to sustain steady muscle contraction
• ""Spooning"" and ""pronator"" signs: spooning/hyperextension of the fingers with wrists extended when arms are outstretched, and pronation of the outstretched hands/forearms
• ""Darting"" or ""jack-in-the-box"" tongue: inability to maintain sustained tongue protrusion
• Emotional lability and behavioural changes: irritability, emotional outbursts, obsessive-compulsive features, deteriorating school performance and handwriting — often the earliest noticed feature by family/teachers before the movement disorder is fully apparent
• Dysarthria due to involvement of orofacial and speech musculature in more severe cases
• Symptoms typically evolve over 1–2 weeks and, untreated, may persist for weeks to several months (occasionally longer), usually resolving completely, though relapses can occur, particularly with subsequent streptococcal infections or pregnancy/oral contraceptive use later in life (chorea gravidarum)

Management

• Confirm the diagnosis of acute rheumatic fever per revised Jones criteria, and treat as for any episode of acute rheumatic fever, including a search for concurrent carditis (echocardiography is recommended in every case of Sydenham chorea, even without audible murmur, since subclinical carditis is common)
• Eradicate residual streptococcal infection: a single course of benzathine penicillin G (or oral penicillin/erythromycin if penicillin-allergic), even though throat culture is often negative by the time chorea presents, given the latent interval
• Secondary antibiotic prophylaxis (as for any confirmed rheumatic fever) — monthly IM benzathine penicillin G (dose per body weight), continued for a duration determined by presence/severity of carditis (minimum 5 years or until age 21, whichever is longer for chorea without carditis; longer, often lifelong, if carditis/valvular disease present) as per standard rheumatic fever secondary prophylaxis guidelines
• Symptomatic treatment of chorea (only if functionally disabling): chorea itself is self-limiting and does NOT require treatment in mild cases; for significant/disabling movements, options include Sodium valproate (commonly preferred, effective and reasonably well tolerated) or Carbamazepine as first-line symptomatic agents; Haloperidol (low dose) is an alternative but used more cautiously due to extrapyramidal side effects
• Immunomodulatory therapy (corticosteroids, IVIG, or plasmapheresis) has been used in severe, refractory, or prolonged cases, based on the presumed autoimmune basis of chorea, though evidence is less robust than for the antiepileptic symptomatic agents above and is generally reserved for severe disease
• Supportive care: a calm environment to minimise stress/excitement (which worsens chorea), protective padding/environmental safety measures if movements are severe enough to risk injury, and psychological/school support given the frequent emotional and behavioural component

Background

Haemophilia A (Factor VIII deficiency, ≈85% of cases) and Haemophilia B (Factor IX deficiency) are X-linked recessive bleeding disorders. A school-going child with haemophilia requires a structured, multidisciplinary approach combining prophylactic factor replacement, family/child/school education, and lifestyle modification to minimise bleeding episodes (particularly joint bleeds, which drive long-term disability) while allowing as normal and active a childhood as safely possible.

A. Prophylaxis Against Bleeding (Medical)

• Primary prophylaxis with regular factor VIII/IX concentrate infusions (started early, ideally before the onset of recurrent joint bleeding, e.g., after the first joint bleed or by 1–2 years of age in severe haemophilia) is now the standard of care in severe haemophilia, shown to markedly reduce joint bleeds and long-term arthropathy compared to on-demand treatment
• Newer extended half-life factor concentrates allow less frequent dosing (e.g., every 1–2 weeks rather than 2–3 times weekly), improving adherence, particularly convenient for school-going children
• Emicizumab (a bispecific antibody mimicking Factor VIIIa function, given as a subcutaneous injection every 1–4 weeks) represents a major recent advance for Haemophilia A, particularly valuable for children with inhibitors and those seeking reduced infusion frequency and easier subcutaneous (rather than IV) administration — well suited to the school-age population
• Prompt, adequate treatment of any breakthrough bleed with factor concentrate at home (via a home therapy/parent or self-infusion programme once age-appropriate) minimises joint damage — ""treat early, treat adequately"" is a core principle

B. Counselling – Child, Family, and School

• Genetic counselling for the family: explain X-linked recessive inheritance, carrier testing for at-risk female relatives, and reproductive/prenatal testing options for future pregnancies
• Recognise bleeding early: educate family and, age-appropriately, the child to recognise early signs of joint bleeding (a distinctive ""tingling""/""bubbling"" sensation or stiffness the child can often identify before swelling is visible) and to seek prompt factor replacement rather than waiting for overt swelling/pain
• School liaison and education: inform and educate teachers/school staff about the condition, provide a written emergency action plan, ensure factor concentrate/emergency contact details are accessible at school, and identify a designated staff member familiar with first-aid measures for this child specifically
• Activity counselling – encourage safe participation, not restriction: non-contact sports (swimming, cycling with protective gear, badminton, athletics) are encouraged for physical fitness and psychosocial wellbeing and joint health; high-contact/collision sports (rugby, boxing, wrestling, kick-boxing) are generally discouraged due to high trauma risk — the overall aim is to normalise childhood participation as far as safely possible rather than default over-restriction
• Dental and medical procedure planning: factor cover before any dental extraction, surgery, or invasive procedure; avoid intramuscular injections (use subcutaneous/oral routes); STRICTLY avoid aspirin and other NSAIDs (antiplatelet effect increases bleeding risk) — paracetamol is the analgesic/antipyretic of choice
• Psychosocial support: address the emotional impact of a chronic condition, encourage peer support/haemophilia patient organisations, and support the child's growing independence in self-management (age-appropriate transition to self-infusion skills as the child matures)
• Medical identification: a medical alert bracelet/card stating the diagnosis, severity, and emergency contact/treating centre details, to be carried at all times