Paediatrics Marrow Pg 171-180 (CNS )
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Questions and Answers

What is the most common cause of congenital hydrocephalus?

  • Aqueductal stenosis (correct)
  • Subarachnoid hemorrhage
  • Dandy-Walker malformation
  • Choroid plexus papilloma

    • Cyclops is a clinical feature associated with hydrocephalus.

    True

    What is the normal cerebrospinal fluid (CSF) volume in adults?

    150 mL

    A common imaging finding in hydrocephalus is ________ on ultrasound.

    <p>ventriculomegaly</p> Signup and view all the answers

    Match the following conditions with their type:

    <p>Aqueductal stenosis = Obstructive Choroid plexus papilloma = Non-obstructive Meningitis = Non-obstructive Subarachnoid hemorrhage = Non-obstructive</p> Signup and view all the answers

    What is the primary characteristic of seizures?

    <p>Abnormal paroxysmal electrical activity in the brain</p> Signup and view all the answers

    Febrile seizures can occur in patients with previous unprovoked seizures.

    <p>False</p> Signup and view all the answers

    What is the first step taken during the assessment of a first episode of UTI?

    <p>USG KUB</p> Signup and view all the answers

    What age group is primarily affected by febrile seizures?

    <p>6 months to 5 years</p> Signup and view all the answers

    Seizures can manifest with ______ disturbances such as sudden variation in heart or respiratory rate.

    <p>autonomic</p> Signup and view all the answers

    A normal USG KUB indicates further investigation is required.

    <p>False</p> Signup and view all the answers

    Match the types of seizures with their characteristics:

    <p>General tonic-clonic seizures = More common, usually a single episode during febrile illness Focal onset seizures = Less common, may have multiple episodes during the same febrile illness Duration less than 15 min = Associated with typical/simple seizures Duration greater than 15 min = Associated with atypical/complex/complicated seizures</p> Signup and view all the answers

    What condition is abbreviated as VUR?

    <p>Vesicoureteral reflux</p> Signup and view all the answers

    The ______ scan is performed 4-6 months after treatment to detect renal scars.

    <p>DMSA</p> Signup and view all the answers

    Match the following conditions with their descriptions:

    <p>VUR = Urine flows backward from the bladder to the kidneys PUV = Rare condition causing urine accumulation in the bladder DMSA Scan = Detects renal scars after UTI treatment MCUG = Evaluates for VUR after an active UTI</p> Signup and view all the answers

    What characterizes a complicated UTI?

    <p>Renal angle tenderness</p> Signup and view all the answers

    A urinary tract infection (UTI) is defined by the presence of significant bacteria without any symptoms being present.

    <p>False</p> Signup and view all the answers

    What is the minimum colony count in CFU/mL for significant bacteriuria using suprapubic aspiration?

    <blockquote> <p>10^3</p> </blockquote> Signup and view all the answers

    Urinary tract infections can be termed recurrent if there are _____ or more episodes.

    <p>2</p> Signup and view all the answers

    Match the methods of urine collection with their associated probability of infection.

    <p>Suprapubic aspiration = &gt; $10^{3}$ CFU/mL Urethral catheterization = &gt; $10^{4}$ CFU/mL Midstream clean catch = &gt; $10^{5}$ CFU/mL</p> Signup and view all the answers

    What is the most severe cranial neural tube defect that is not compatible with life?

    <p>Anencephaly</p> Signup and view all the answers

    Folic acid supplementation can reduce the risk of neural tube defects by 50%.

    <p>False</p> Signup and view all the answers

    What maternal serum marker is elevated in the case of neural tube defects?

    <p>Alpha-fetoprotein (AFP)</p> Signup and view all the answers

    The main type of encephalocele is located in the ______ area.

    <p>occipital</p> Signup and view all the answers

    Match the following cranial neural tube defects with their characteristic:

    <p>Anencephaly = Not compatible with life Encephalocele = Herniation of brain tissue Iniencephaly = Antenatal Stargazer posture Craniorachischisis = Splitting of the cranium and spine</p> Signup and view all the answers

    What is the gold standard for diagnosing hydrocephalus?

    <p>MRI brain</p> Signup and view all the answers

    Increased intracranial tension can cause bulging anterior fontanelles in infants.

    <p>True</p> Signup and view all the answers

    What medication is used in medical management to reduce cerebrospinal fluid (CSF) production?

    <p>Acetazolamide</p> Signup and view all the answers

    The primary organism associated with shunt-associated meningitis is __________.

    <p>Staphylococcus epidermidis</p> Signup and view all the answers

    Match the following clinical features of hydrocephalus with their descriptions:

    <p>Macrocephaly = Head circumference greater than +2 SD Sunset sign = Visible upper sclera due to down gaze Dilated scalp veins = Decreased venous drainage Wide separation of skull sutures = Separation greater than 0.5 cm</p> Signup and view all the answers

    Which grade of VUR is associated with tortuosity of the ureter?

    <p>Grade 3</p> Signup and view all the answers

    Prophylactic antibiotics are indicated for all grades of VUR.

    <p>False</p> Signup and view all the answers

    What is the most common organism found in urinary tract infections (UTIs) in children?

    <p>E. coli</p> Signup and view all the answers

    Supportive management for VUR includes adequate fluids and __________.

    <p>regular and complete voiding</p> Signup and view all the answers

    Match the following management strategies with their purposes:

    <p>Supportive management = Promotes spontaneous resolution of VUR Prophylactic antibiotics = Prevents UTIs in high-risk VUR grades Surgical correction = Addresses UTI occurrence post-prophylaxis Radionuclide cystogram = Follow-up for VUR with less radiation exposure</p> Signup and view all the answers

    What characteristic is associated with Dandy Walker malformation?

    <p>Cystic expansion of the 4th ventricle</p> Signup and view all the answers

    Dandy Walker malformation is commonly associated with hydrocephalus in Type I.

    <p>False</p> Signup and view all the answers

    What are common symptoms of Type II Dandy Walker malformation?

    <p>Hydrocephalus, meningomyelocele, cerebellar tonsil herniation</p> Signup and view all the answers

    The condition characterized by incomplete development of the cerebellum is known as __________.

    <p>cerebellar hypoplasia</p> Signup and view all the answers

    Match the following types of Dandy Walker malformation with their characteristics:

    <p>Type I = Not associated with hydrocephalus Type II = Associated with hydrocephalus</p> Signup and view all the answers

    Which of the following is NOT a feature of vein of Galen malformation?

    <p>Cystic expansion of the thalamus</p> Signup and view all the answers

    Occipital widening is a feature associated with Dandy Walker malformation.

    <p>True</p> Signup and view all the answers

    What imaging technique is often used to visualize abnormalities in Dandy Walker malformation?

    <p>MRI</p> Signup and view all the answers

    Which of the following is NOT a risk factor for Neural Tube Defects (NTD)?

    <p>Advanced maternal age</p> Signup and view all the answers

    Meningocele is characterized by the presence of spinal cord structures.

    <p>False</p> Signup and view all the answers

    What condition is associated with hydrocephalus in severe caudal NTDs?

    <p>Meningomyelocele</p> Signup and view all the answers

    The failure of closure of the anterior neuropore results in __________ NTDs.

    <p>Cranial</p> Signup and view all the answers

    Match the type of NTD with its description:

    <p>Spina bifida occulta = Asymptomatic with possible skin signs Meningocele = Meninges and CSF filled cavity Meningomyelocele = Spinal cord structures present Arnold-Chiari type II = Associated defect with meningomyelocele</p> Signup and view all the answers

    Which of the following conditions is characterized by a smooth appearance of the brain and absence of gyri?

    <p>Lissencephaly</p> Signup and view all the answers

    Agenesis of the corpus callosum is always lethal in males.

    <p>True</p> Signup and view all the answers

    What genetic defect is associated with holoprosencephaly?

    <p>Sonic hedgehog (SHH) gene</p> Signup and view all the answers

    Porencephaly is characterized by _______ that are lined by white matter.

    <p>cystic spaces</p> Signup and view all the answers

    Match the following conditions with their clinical features:

    <p>Lissencephaly = Absence of gyri Porencephaly = Cystic spaces lined by white matter Agenesis of the corpus callosum = Microcephaly and low IQ Holoprosencephaly = Single lobe brain</p> Signup and view all the answers

    Study Notes

    Hydrocephalus

    • Associated Genetic Disorder: Trisomy 13 (Patau's syndrome)
    • Clinical Features: Midline facial abnormalities including proboscis, cyclops, and cleft lip/palate
    • Note: Increased CSF volume in the brain (>50mL). Normal CSF volume: 150mL (Adults) → 50mL (Infant)
    • Obstructive Causes: Congenital anomalies (aqueductal stenosis, Dandy-Walker malformation, Arnold-Chiari malformation, Vein of Galen malformation), tumors (posterior fossa tumors), TORCH infections (e.g., toxoplasmosis)
    • Non-obstructive Causes: Increased CSF production (choroid plexus papilloma), Decreased CSF absorption (subarachnoid hemorrhage, meningitis)
    • Pathogenesis: Stenosis at the aqueduct of Sylvius leads to blockage of CSF flow from the lateral ventricle to the 4th ventricle.
    • Imaging: Ventriculomegaly on USG (most common finding in aqueductal stenosis)

    Vesicoureteral Reflux (VUR) & Urinary Tract Infections (UTIs)

    • First Episode of UTI: USG KUB (normal - no further testing, abnormal - further evaluation, including post-void urine in the bladder)
    • Further Investigation (Abnormal USG KUB): Micturating Cystourethrogram (MCUG) performed 2-4 weeks after treatment of active infection (if previous treatment unsuccessful, performed immediately after infection)
    • Recurrent UTI: If patient has recurring UTI. Non E.coli UTI requires specific investigation.
    • Abnormalities Seen: VUR, PUV (Posterior urethral valve)
    • Further Investigations (Abnormal findings): DMSA scan performed 4-6 months after treatment to detect renal scars (inflammation from UTI can mimic scarring).

    Seizure Disorders

    • Definition: Abnormal, paroxysmal electrical activity in the brain
    • Manifestations: Motor, sensory, behavioral changes, autonomic disturbances
    • Causes: Epilepsy (provoked, unprovoked) and recurrent seizures
    • Febrile Seizures: Seizures provoked by fever, with normal developmental status in patients
    • Types of Seizures:
      • Typical/Simple: General tonic-clonic seizures (GTCs) - more common; Single seizure during febrile illness; Duration < 15 minutes
      • Atypical/Complex/Complicated: Focal onset - less common; Multiple seizures during the same febrile illness; Duration > 15 minutes

    Dandy Walker Malformation

    • Features:
      • Cystic expansion of 4th ventricle
      • Dilatation of posterior fossa
      • Hydrocephalus
      • Occipital widening
      • Cerebellar hypoplasia
    • Types:
      • Type I: Adults, no hydrocephalus association, cerebellar tonsil herniation, neck pain, headache
      • Type II: Young children, hydrocephalus, meningomyelocele, cerebellar tonsil herniation, cerebellar and brainstem structures involvement, 4th ventricle structures abnormalities
    • Arnold Chiari Malformation: Features associated with Arnold-Chiari malformation.
    • Vein of Galen Malformation (VOG):
      • Increased venous pressure
      • Compression/obstruction of CSF pathway
      • Hydrocephalus
      • Heart failure
      • Cranial bruit
      • Prominent carotid pulsations
    • Imaging: MRI images of brain are shown, depicting various sections of the brain for the Dandy-Walker and vein of Galen malformation conditions.

    Genitourinary System

    • Definitions:
      • Complicated UTI: High-grade fever (>39°C), systemic toxicity, renal angle tenderness, ↑ Serum Creatinine
      • Simple UTI: Low-grade fever, dysuria, ↑ urine frequency
      • Recurrent infection: ≥ 2 episodes of UTI
      • Significant Bacteriuria: Urine culture: Definitive diagnosis of UTI. Assessment of significant bacteriuria by colony forming unit (CFU) count.
      • Asymptomatic bacteriuria: Significant bacteriuria without any symptoms, no treatment required.
      • Leukocyturia: > 10 WBC/mm³ in uncentrifuged urine sample OR >5 WBC/HPF (High Power Field) in centrifuged urine sample, not diagnostic for UTI.
    • Management:
      • Antibiotic Management: Hospitalization for complicated UTI and young infants.
      • Imaging: Renal scan and Ultrasound (USG)
      • Other: Follow-up for UTI to assess for underlying urinary tract anomalies.

    Cranial Neural Tube Defects (NTDs)

    • Anencephaly:
      • Most severe NTD, not compatible with life.
      • Types: Complete (holoencephal) or Partial (meroanencephaly)
    • Encephalocele:
      • Defect in skull, herniation of brain (swelling).
      • Most common site: occipital area.
    • Iniencephaly:
      • Fusion of cervical vertebrae.
      • Antenatal USG finding: Stargazer posture.
    • Craniorachischisis:
      • Cranio: Cranium
      • Rachi: Spinal region
      • Schisis: Splitting
    • Antenatal Diagnosis:
      • USG (14-16 weeks of gestation)
      • Earliest NTD identified: Anencephaly (10-12 weeks)
      • Markers: Acetylcholinesterase (best/specific marker), AFP (Alpha-fetoprotein: elevated at 15-18 weeks in maternal serum only- screening method)
    • Prevention:
      • Folate acid supplementation: decreases NTD risk by 70%
      • Periconceptional supplementation: 1 month before planned conception
      • Dose: 400 mcg/day (Previously affected child: 4000 mcg/day)

    VUR & UTI Management Guidelines

    • Grading of VUR:
      • Grade 1: Partial reflux
      • Grade 2: Complete reflux
      • Grade 2 (+): Associated dilatation of bladder & renal pelvis
      • Grade 3 (+): Tortuosity of ureter
      • Grade 4 (+): Distorted pelvi-calyceal impressions
    • Radionuclide cystogram: For VUR follow-up, less radiation exposure (but less accurate for grading).
    • Management:
      • Supportive Management: Adequate fluids, mix of breast milk and bottle feeding (BBD), regular and complete voiding, relief of constipation.
      • Prevention of UTI: Prophylactic antibiotics (e.g., cotrimoxazole/ nitrofurantoin/ cephalexin) until BBD resolves (indication: Grade 3-5 VUR).
      • Regimen: Until 2 years of age + 1 year after last episode of UTI.
      • If UTI occurs post-prophylaxis: Surgical correction: ureteric re-implementation.

    UTI

    • Significance in Children:
      • Presentation: Fever without a focus
      • Classification: Simple and complicated
      • UTI < 5 years: Investigate for renal scarring, association with urinary tract anomalies.
      • Most common organism in UTI: E.coli

    Congenital Anomalies and Hydrocephalus

    • Clinical Features:
      • Macrocephaly: Increased head circumference greater than +2 standard deviations.
      • Increased Intracranial Tension (ICT): Dilated scalp veins, bulging anterior fontanelles, wide separation of skull sutures (> 0.5 cm), sunset sign (visible upper sclera).
    • Investigations:
      • Screening: Neurosonogram (USG at anterior fontanelle)
      • Gold Standard/Definitive: MRI brain
    • Treatment:
      • Medical Management: Acetazolamide (diuretics, carbonic anhydrase inhibitor)
      • Surgical Management: V-P shunt (ventriculo-peritoneal)
    • Complications:
      • Shunt-associated meningitis: Organism: Staphylococcus epidermidis, Diagnose through CSF analysis from shunt tapping.

    Neural Tube Defects (NTDs)

    • Incidence: Most common CNS congenital malformations (3.5-4/1000 live births).
    • Risk Factors:
      • Folic acid deficiency, maternal drugs (e.g. anticonvulsant, carbamazepine), Uncontrolled maternal diabetes.
    • Pathology:: Failure of closure of anterior neuropore (24 days) results in Cranial NTDs. Failure of posterior neuropore closure (26 days) results in Caudal NTDs.
    • Types of Caudal NTDs:
      • Spina bifida occulta: Asymptomatic (may present with swelling/ tuft of hair over defect).
      • Meningocele: Meninges present (CSF filled cavity, transillumination test +).
      • Meningomyelocele: Most severe caudal NTD, associated defects: Arnold-Chiari type II malformation (80%), Hydrocephalus.
    • Additional Notes:
      • Risk of rupture/ infection
      • Spinal nerve root herniation: can cause lower limb tone/ flaccidity, bowel and bladder incontinence

    Neuronal Migration Defects

    • Lissencephaly: Smooth appearance of brain (agyria - absence of gyri).
    • Schizencephaly: Clefts inside brain (schizis: splitting).
    • Porencephaly: Cystic spaces in brain (lined by white matter) due to cerebral infarctions during intrauterine period or perinatal.
    • Agenesis of corpus callosum:
      • Clinical features: Microcephaly, low IQ, refractory seizures
      • Associated syndromes: Aicardi syndrome (X-linked inheritance, severe in females, lethal in males) with eye manifestations, infantile spasms, vertebral defects.
    • Holoprosencephaly:
      • Failure of division of prosencephalon (single lobe brain).
      • Gene defect: Sonic hedgehog (SHH) gene.

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    Description

    This quiz covers key aspects of hydrocephalus, including its association with Trisomy 13, clinical features, and underlying causes. It also discusses vesicoureteral reflux (VUR) and the implications of urinary tract infections (UTIs) in pediatric patients. Test your knowledge on the imaging findings and pathogenesis of these conditions.

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