Podcast
Questions and Answers
What is the most common cause of congenital hydrocephalus?
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.
Cyclops is a clinical feature associated with hydrocephalus.
True (A)
What is the normal cerebrospinal fluid (CSF) volume in adults?
What is the normal cerebrospinal fluid (CSF) volume in adults?
150 mL
A common imaging finding in hydrocephalus is ________ on ultrasound.
A common imaging finding in hydrocephalus is ________ on ultrasound.
Match the following conditions with their type:
Match the following conditions with their type:
What is the primary characteristic of seizures?
What is the primary characteristic of seizures?
Febrile seizures can occur in patients with previous unprovoked seizures.
Febrile seizures can occur in patients with previous unprovoked seizures.
What is the first step taken during the assessment of a first episode of UTI?
What is the first step taken during the assessment of a first episode of UTI?
What age group is primarily affected by febrile seizures?
What age group is primarily affected by febrile seizures?
Seizures can manifest with ______ disturbances such as sudden variation in heart or respiratory rate.
Seizures can manifest with ______ disturbances such as sudden variation in heart or respiratory rate.
A normal USG KUB indicates further investigation is required.
A normal USG KUB indicates further investigation is required.
Match the types of seizures with their characteristics:
Match the types of seizures with their characteristics:
What condition is abbreviated as VUR?
What condition is abbreviated as VUR?
The ______ scan is performed 4-6 months after treatment to detect renal scars.
The ______ scan is performed 4-6 months after treatment to detect renal scars.
Match the following conditions with their descriptions:
Match the following conditions with their descriptions:
What characterizes a complicated UTI?
What characterizes a complicated UTI?
A urinary tract infection (UTI) is defined by the presence of significant bacteria without any symptoms being present.
A urinary tract infection (UTI) is defined by the presence of significant bacteria without any symptoms being present.
What is the minimum colony count in CFU/mL for significant bacteriuria using suprapubic aspiration?
What is the minimum colony count in CFU/mL for significant bacteriuria using suprapubic aspiration?
Urinary tract infections can be termed recurrent if there are _____ or more episodes.
Urinary tract infections can be termed recurrent if there are _____ or more episodes.
Match the methods of urine collection with their associated probability of infection.
Match the methods of urine collection with their associated probability of infection.
What is the most severe cranial neural tube defect that is not compatible with life?
What is the most severe cranial neural tube defect that is not compatible with life?
Folic acid supplementation can reduce the risk of neural tube defects by 50%.
Folic acid supplementation can reduce the risk of neural tube defects by 50%.
What maternal serum marker is elevated in the case of neural tube defects?
What maternal serum marker is elevated in the case of neural tube defects?
The main type of encephalocele is located in the ______ area.
The main type of encephalocele is located in the ______ area.
Match the following cranial neural tube defects with their characteristic:
Match the following cranial neural tube defects with their characteristic:
What is the gold standard for diagnosing hydrocephalus?
What is the gold standard for diagnosing hydrocephalus?
Increased intracranial tension can cause bulging anterior fontanelles in infants.
Increased intracranial tension can cause bulging anterior fontanelles in infants.
What medication is used in medical management to reduce cerebrospinal fluid (CSF) production?
What medication is used in medical management to reduce cerebrospinal fluid (CSF) production?
The primary organism associated with shunt-associated meningitis is __________.
The primary organism associated with shunt-associated meningitis is __________.
Match the following clinical features of hydrocephalus with their descriptions:
Match the following clinical features of hydrocephalus with their descriptions:
Which grade of VUR is associated with tortuosity of the ureter?
Which grade of VUR is associated with tortuosity of the ureter?
Prophylactic antibiotics are indicated for all grades of VUR.
Prophylactic antibiotics are indicated for all grades of VUR.
What is the most common organism found in urinary tract infections (UTIs) in children?
What is the most common organism found in urinary tract infections (UTIs) in children?
Supportive management for VUR includes adequate fluids and __________.
Supportive management for VUR includes adequate fluids and __________.
Match the following management strategies with their purposes:
Match the following management strategies with their purposes:
What characteristic is associated with Dandy Walker malformation?
What characteristic is associated with Dandy Walker malformation?
Dandy Walker malformation is commonly associated with hydrocephalus in Type I.
Dandy Walker malformation is commonly associated with hydrocephalus in Type I.
What are common symptoms of Type II Dandy Walker malformation?
What are common symptoms of Type II Dandy Walker malformation?
The condition characterized by incomplete development of the cerebellum is known as __________.
The condition characterized by incomplete development of the cerebellum is known as __________.
Match the following types of Dandy Walker malformation with their characteristics:
Match the following types of Dandy Walker malformation with their characteristics:
Which of the following is NOT a feature of vein of Galen malformation?
Which of the following is NOT a feature of vein of Galen malformation?
Occipital widening is a feature associated with Dandy Walker malformation.
Occipital widening is a feature associated with Dandy Walker malformation.
What imaging technique is often used to visualize abnormalities in Dandy Walker malformation?
What imaging technique is often used to visualize abnormalities in Dandy Walker malformation?
Which of the following is NOT a risk factor for Neural Tube Defects (NTD)?
Which of the following is NOT a risk factor for Neural Tube Defects (NTD)?
Meningocele is characterized by the presence of spinal cord structures.
Meningocele is characterized by the presence of spinal cord structures.
What condition is associated with hydrocephalus in severe caudal NTDs?
What condition is associated with hydrocephalus in severe caudal NTDs?
The failure of closure of the anterior neuropore results in __________ NTDs.
The failure of closure of the anterior neuropore results in __________ NTDs.
Match the type of NTD with its description:
Match the type of NTD with its description:
Which of the following conditions is characterized by a smooth appearance of the brain and absence of gyri?
Which of the following conditions is characterized by a smooth appearance of the brain and absence of gyri?
Agenesis of the corpus callosum is always lethal in males.
Agenesis of the corpus callosum is always lethal in males.
What genetic defect is associated with holoprosencephaly?
What genetic defect is associated with holoprosencephaly?
Porencephaly is characterized by _______ that are lined by white matter.
Porencephaly is characterized by _______ that are lined by white matter.
Match the following conditions with their clinical features:
Match the following conditions with their clinical features:
Flashcards are hidden until you start studying
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.
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.
