📘 Essential for Pediatric Nursing, Child Health Nursing, Neurology, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
CNS malformations are structural abnormalities of the brain or spinal cord present at birth, caused by genetic mutations, infections, nutritional deficiencies, or environmental exposures during embryonic development.
✳️ These conditions may lead to neurological impairment, developmental delay, and lifelong disability depending on the severity.
| System Affected | Example | Description |
|---|---|---|
| 🧠 Brain (Cerebrum) | Microcephaly, Hydrocephalus | Abnormal brain size or fluid accumulation |
| 🧠 Midline Defects | Holoprosencephaly | Incomplete division of cerebral hemispheres |
| 🧠 Posterior fossa | Dandy-Walker malformation | Cerebellar and fourth ventricle abnormality |
| 🧠 Neural Tube Defects | Anencephaly, Spina bifida | Failure of neural tube closure |
| 🧠 Migrational Disorders | Lissencephaly | Smooth brain surface (no gyri/sulci) |
| Type | Description |
|---|---|
| 🟢 Spina bifida occulta | Hidden defect; no protrusion; tuft of hair seen |
| 🟡 Meningocele | Protrusion of meninges through vertebral gap |
| 🔴 Myelomeningocele | Protrusion of meninges + spinal cord (most severe, neurological deficits) |
❌ Intellectual disability
❌ Paralysis or motor impairment
❌ Seizures
❌ Hydrocephalus
❌ Urinary/bowel incontinence
❌ Vision or hearing loss
🟡 Neural tube closes by 28th day of gestation
🟡 Folic acid prevents most neural tube defects
🟡 Anencephaly = incompatible with life
🟡 Hydrocephalus = increased CSF → enlarged head
🟡 Spina bifida = failure of vertebral arch closure
Q1. Which of the following is a neural tube defect?
🅰️ Hydrocephalus
🅱️ Microcephaly
✅ 🅲️ Anencephaly
🅳️ Cerebral palsy
Correct Answer: 🅲️ Anencephaly
Q2. Deficiency of which vitamin leads to neural tube defects?
🅰️ Vitamin B12
🅱️ Vitamin D
✅ 🅲️ Folic acid
🅳️ Vitamin A
Correct Answer: 🅲️ Folic acid
Q3. Spina bifida with spinal cord protrusion is called:
🅰️ Meningocele
✅ 🅱️ Myelomeningocele
🅲️ Occulta
🅳️ Encephalocele
Correct Answer: 🅱️ Myelomeningocele
Q4. A child with increased head circumference and sunset eyes likely has:
🅰️ Microcephaly
🅱️ Anencephaly
✅ 🅲️ Hydrocephalus
🅳️ Dandy-Walker
Correct Answer: 🅲️ Hydrocephalus
Q5. Dandy-Walker syndrome affects:
🅰️ Frontal lobe
✅ 🅱️ Cerebellum and fourth ventricle
🅲️ Brainstem
🅳️ Temporal lobe
Correct Answer: 🅱️ Cerebellum and fourth ventricle
Q6. Which condition is associated with smooth brain (lacking folds)?
🅰️ Spina bifida
✅ 🅱️ Lissencephaly
🅲️ Hydrocephalus
🅳️ Cerebral palsy
Correct Answer: 🅱️ Lissencephaly
Q7. Most critical time for neural tube development is:
🅰️ 12–16 weeks
✅ 🅱️ 3–4 weeks
🅲️ 6–8 weeks
🅳️ 16–20 weeks
Correct Answer: 🅱️ 3–4 weeks
📘 Essential for Pediatric Nursing, Child Health Nursing, Anatomy, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Cranial deformities are abnormal shapes or sizes of the skull caused by congenital or acquired causes, such as premature closure of cranial sutures (craniosynostosis), intrauterine constraint, or positional factors.
✳️ These deformities can be cosmetic, neurological, or both depending on severity.
| Type | Cause |
|---|---|
| ✅ Craniosynostosis | Premature fusion of one/more sutures |
| ✅ Positional Plagiocephaly | External pressure on the skull (non-suture-related) |
| Term | Suture Involved | Skull Shape |
|---|---|---|
| Scaphocephaly | Sagittal suture | Long, narrow head (boat-shaped) |
| Brachycephaly | Coronal sutures (bilateral) | Short, broad head |
| Plagiocephaly | Unilateral coronal or lambdoid | Asymmetrical (flattened) skull |
| Trigonocephaly | Metopic suture | Triangular-shaped forehead |
| Oxycephaly | Multiple sutures | Tower-shaped skull |
| Microcephaly | All sutures closed early | Small head, often with brain underdevelopment |
| Feature | Observation |
|---|---|
| 🧠 Skull Shape | Asymmetry, abnormal contour or height |
| 📏 Head Circumference | May be small (microcephaly) or increased (hydrocephalus) |
| 👁️ Forehead | Prominent or flattened |
| 🩺 Fontanelle | Closed early in craniosynostosis |
| 🧪 Neurological | May have developmental delay or seizures (in severe cases) |
❌ Raised intracranial pressure (ICP)
❌ Developmental delay
❌ Vision or hearing problems
❌ Cosmetic and psychosocial concerns
❌ Cranial nerve involvement (in syndromic cases)
🟡 Scaphocephaly = long boat-shaped head (sagittal suture)
🟡 Brachycephaly = broad flat head (bilateral coronal fusion)
🟡 Helmet therapy = used in positional plagiocephaly
🟡 Craniosynostosis = treated with cranial vault surgery
🟡 Early intervention prevents neurodevelopmental complications
Q1. Craniosynostosis refers to:
🅰️ Brain infection
✅ 🅱️ Premature fusion of skull sutures
🅲️ Postural defect
🅳️ CSF leak
Correct Answer: 🅱️ Premature fusion of skull sutures
Q2. Scaphocephaly is caused by fusion of which suture?
🅰️ Coronal
🅱️ Lambdoid
✅ 🅲️ Sagittal
🅳️ Metopic
Correct Answer: 🅲️ Sagittal
Q3. The gold standard imaging for cranial deformity diagnosis is:
🅰️ MRI
🅱️ Skull X-ray
✅ 🅲️ 3D CT scan
🅳️ Ultrasound
Correct Answer: 🅲️ 3D CT scan
Q4. Positional plagiocephaly is best prevented by:
🅰️ Helmet from birth
🅱️ Sleeping upright
✅ 🅲️ Tummy time and alternating sleep position
🅳️ Constant left-side sleep
Correct Answer: 🅲️ Tummy time and alternating sleep position
Q5. Most effective treatment for craniosynostosis:
🅰️ Braces
✅ 🅱️ Surgical remodeling
🅲️ Massage
🅳️ Cranial traction
Correct Answer: 🅱️ Surgical remodeling
Q6. What is a key nursing responsibility post cranial surgery?
🅰️ Increase fluid intake
🅱️ Avoid touching the head
✅ 🅲️ Monitor neuro status and bleeding
🅳️ Keep child in Trendelenburg position
Correct Answer: 🅲️ Monitor neuro status and bleeding
Q7. Early closure of all sutures causes:
🅰️ Hydrocephalus
🅱️ Dandy-Walker syndrome
✅ 🅲️ Microcephaly
🅳️ Encephalocele
Correct Answer: 🅲️ Microcephaly
📘 Essential for Pediatric Nursing, Medical-Surgical Nursing, Child Health, Anatomy, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Spina bifida is a neural tube defect (NTD) where there is incomplete closure of the vertebral column and spinal cord structures during embryonic development, leading to exposure or protrusion of meninges and/or spinal cord.
✳️ “Spina bifida” means “split spine” and usually occurs in the lumbar-sacral region.
| Type | Description | Severity |
|---|---|---|
| 🟩 Spina bifida occulta | Hidden defect; no sac; often asymptomatic | Mild |
| 🟨 Meningocele | Meninges protrude through defect in vertebrae; spinal cord intact | Moderate |
| 🟥 Myelomeningocele | Meninges + spinal cord protrude; major neurological deficits | Severe |
| 🟦 Rachischisis (rare) | Complete failure of neural tube closure | Lethal |
| Type | Signs |
|---|---|
| 🟩 Occulta | Dimple, tuft of hair, small swelling on back |
| 🟨 Meningocele | Fluid-filled sac, no motor/sensory loss |
| 🟥 Myelomeningocele | Visible sac with nerves, paralysis, urinary/fecal incontinence |
| 🔴 All types | Risk of hydrocephalus, scoliosis, clubfoot, learning delay |
❌ Hydrocephalus
❌ Meningitis
❌ Urinary tract infections (UTIs)
❌ Paraplegia or motor loss
❌ Skin ulcers and orthopedic deformities
❌ Psychosocial stress
🟡 Spina bifida = neural tube defect of spinal column
🟡 Most severe = Myelomeningocele
🟡 Folic acid prevents neural tube defects (400 mcg/day)
🟡 First sign in newborn = sac-like swelling on back
🟡 Hydrocephalus is a common associated defect
Q1. Spina bifida is caused due to:
🅰️ Protein deficiency
✅ 🅱️ Neural tube defect
🅲️ Iron deficiency
🅳️ Iodine excess
Correct Answer: 🅱️ Neural tube defect
Q2. Most severe form of spina bifida is:
🅰️ Spina bifida occulta
🅱️ Meningocele
✅ 🅲️ Myelomeningocele
🅳️ Rickets
Correct Answer: 🅲️ Myelomeningocele
Q3. The most important nutrient to prevent spina bifida is:
🅰️ Vitamin D
🅱️ Iron
✅ 🅲️ Folic acid
🅳️ Calcium
Correct Answer: 🅲️ Folic acid
Q4. What is the position of the infant with open spina bifida?
🅰️ Supine
🅱️ Left lateral
✅ 🅲️ Prone
🅳️ Sitting
Correct Answer: 🅲️ Prone
Q5. Associated condition with spina bifida is:
🅰️ Encephalitis
🅱️ Scoliosis
✅ 🅲️ Hydrocephalus
🅳️ Aplastic anemia
Correct Answer: 🅲️ Hydrocephalus
Q6. The sac in myelomeningocele contains:
🅰️ Skin only
🅱️ Meninges only
✅ 🅲️ Meninges + spinal cord
🅳️ Muscle
Correct Answer: 🅲️ Meninges + spinal cord
Q7. Best time for surgical closure of myelomeningocele is:
🅰️ After 1 month
✅ 🅱️ Within 24–48 hours of birth
🅲️ After 1 year
🅳️ At 6 months
Correct Answer: 🅱️ Within 24–48 hours of birth
📘 Important for Pediatric Nursing, Neurology, Child Health, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Hydrocephalus is a condition characterized by an abnormal accumulation of cerebrospinal fluid (CSF) within the ventricular system of the brain, leading to increased intracranial pressure (ICP) and ventricular dilation.
✳️ It may be congenital or acquired, and if untreated, can lead to brain damage and developmental delay.
| Type | Description |
|---|---|
| 🟦 Communicating Hydrocephalus | CSF flows freely but absorption is impaired |
| 🟥 Non-communicating (Obstructive) | CSF flow blocked within ventricles (e.g., aqueductal stenosis) |
| Type | Examples |
|---|---|
| 🟢 Congenital | Aqueductal stenosis, Dandy-Walker malformation, Arnold-Chiari |
| 🔴 Acquired | Post-meningitis, brain tumors, hemorrhage, trauma |
| Sign | Description |
|---|---|
| 📏 Head | Rapid increase in head circumference |
| 🎯 Fontanelle | Bulging anterior fontanelle |
| 👁️ Eyes | Sunset eyes (downward deviation) |
| 📣 Cry | High-pitched cry |
| 🍽️ Feeding | Poor sucking, vomiting |
| 🧠 Others | Seizures, lethargy, irritability |
❌ Shunt malfunction or blockage
❌ Shunt infection (meningitis, peritonitis)
❌ Delayed milestones
❌ Seizures
❌ Visual or hearing impairment
❌ Dependency on shunt for life
🟡 Hydrocephalus = excess CSF in brain ventricles
🟡 Most common treatment = VP shunt surgery
🟡 Sunset eyes = classic sign in infants
🟡 Monitor head circumference in early detection
🟡 Dandy-Walker = hydrocephalus + cerebellar hypoplasia
Q1. Hydrocephalus is due to:
🅰️ Decreased blood in brain
✅ 🅱️ Increased CSF in ventricles
🅲️ Loss of brain cells
🅳️ Low blood sugar
Correct Answer: 🅱️ Increased CSF in ventricles
Q2. Classic eye sign in infant with hydrocephalus:
🅰️ Nystagmus
🅱️ Red eyes
✅ 🅲️ Sunset sign
🅳️ Squint
Correct Answer: 🅲️ Sunset sign
Q3. Gold standard investigation for hydrocephalus is:
🅰️ X-ray skull
🅱️ USG
✅ 🅲️ MRI/CT brain
🅳️ EEG
Correct Answer: 🅲️ MRI/CT brain
Q4. Most common surgical treatment for hydrocephalus is:
🅰️ Craniotomy
🅱️ Burr hole
✅ 🅲️ Ventriculoperitoneal (VP) shunt
🅳️ EEG
Correct Answer: 🅲️ Ventriculoperitoneal (VP) shunt
Q5. Dandy-Walker syndrome includes:
🅰️ Enlarged pituitary
🅱️ Brain hemorrhage
✅ 🅲️ Hydrocephalus + cerebellar defect
🅳️ Thalamic calcification
Correct Answer: 🅲️ Hydrocephalus + cerebellar defect
Q6. Which drug helps reduce CSF formation temporarily?
🅰️ Paracetamol
🅱️ Diazepam
✅ 🅲️ Acetazolamide
🅳️ Ciprofloxacin
Correct Answer: 🅲️ Acetazolamide
Q7. A red flag for shunt infection post-surgery is:
🅰️ Weight gain
🅱️ Sleep
✅ 🅲️ High fever and irritability
🅳️ Dry skin
Correct Answer: 🅲️ High fever and irritability
📘 Essential for Pediatric Nursing, Neurology, Rehabilitation Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Cerebral palsy (CP) is a non-progressive, permanent disorder of movement and posture caused by damage to the immature developing brain (before, during, or shortly after birth).
✳️ It affects muscle tone, motor control, coordination, balance, and may be associated with cognitive or sensory impairments.
| Type | Features | Area Affected |
|---|---|---|
| 🟥 Spastic CP (most common) | Stiff, tight muscles; scissor gait | Cerebral cortex |
| 🟨 Dyskinetic/Athetoid CP | Involuntary writhing movements | Basal ganglia |
| 🟩 Ataxic CP | Poor balance, shaky movements | Cerebellum |
| 🟦 Mixed CP | Combination of two or more types | Multiple areas |
| System | Signs |
|---|---|
| 🧠 Motor | Delayed milestones, abnormal muscle tone (↑ or ↓), poor coordination |
| 🚶♂️ Posture | Abnormal reflexes (Moro, tonic neck), poor head control |
| 🦵 Gait | Toe walking, scissoring, foot drop |
| 🗣️ Speech | Dysarthria (slurred), drooling |
| 🧮 Cognitive | May have intellectual disability |
| 👀 Sensory | Vision, hearing loss; seizures may occur |
| ⚠️ Others | Scoliosis, joint contractures, feeding difficulties |
📌 There is no cure, but early intervention can significantly improve function.
❌ Muscle contractures
❌ Seizures
❌ Aspiration pneumonia
❌ Malnutrition
❌ Emotional and social issues
❌ Learning difficulties
🟡 CP = non-progressive motor disorder due to early brain damage
🟡 Spastic CP is the most common type
🟡 CP affects movement, posture, speech, coordination
🟡 Early rehabilitation = best outcome
🟡 Muscle relaxants (e.g., Baclofen), therapy, and support = key
Q1. Cerebral palsy is a disorder of:
🅰️ Brainstem stroke
✅ 🅱️ Movement and posture due to early brain injury
🅲️ Kidney function
🅳️ Lung development
Correct Answer: 🅱️ Movement and posture due to early brain injury
Q2. Most common type of cerebral palsy is:
🅰️ Ataxic
🅱️ Dyskinetic
✅ 🅲️ Spastic
🅳️ Mixed
Correct Answer: 🅲️ Spastic
Q3. A child with scissor gait and increased tone likely has:
🅰️ Ataxic CP
✅ 🅱️ Spastic CP
🅲️ Flaccid paralysis
🅳️ Muscular dystrophy
Correct Answer: 🅱️ Spastic CP
Q4. Drug used to reduce spasticity in CP:
🅰️ Paracetamol
🅱️ Insulin
✅ 🅲️ Baclofen
🅳️ Ciprofloxacin
Correct Answer: 🅲️ Baclofen
Q5. Which of the following is a common feature in CP?
🅰️ Normal development
🅱️ Hyperactive growth
✅ 🅲️ Delayed motor milestones
🅳️ Increased appetite
Correct Answer: 🅲️ Delayed motor milestones
Q6. Which therapy helps improve communication in CP children?
🅰️ Physiotherapy
✅ 🅱️ Speech therapy
🅲️ Surgery
🅳️ Radiotherapy
Correct Answer: 🅱️ Speech therapy
Q7. Early intervention in CP can:
🅰️ Cure the condition
🅱️ Reverse the brain damage
✅ 🅲️ Improve function and reduce complications
🅳️ Prevent inheritance
Correct Answer: 🅲️ Improve function and reduce complications
📘 Essential for Pediatric Nursing, Neurology, Medical-Surgical Nursing, Child Health, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Meningocele is a type of spina bifida cystica, a neural tube defect where the meninges (protective coverings of the brain and spinal cord) protrude through a defect in the vertebral column, forming a fluid-filled sac, but the spinal cord remains in place and undamaged.
✳️ It is less severe than myelomeningocele and usually does not involve neurological deficits.
| Type | Description | Severity |
|---|---|---|
| 🟩 Spina bifida occulta | Hidden defect, no protrusion | Mild |
| 🟨 Meningocele | Meninges protrude in a sac, spinal cord not involved | Moderate |
| 🟥 Myelomeningocele | Meninges + spinal cord protrude | Severe |
| System | Signs |
|---|---|
| 🧠 CNS | Sac-like swelling over spine (usually lumbosacral) |
| 🟡 Skin | Translucent sac filled with CSF |
| 🧍 Motor | Usually no paralysis or motor deficit |
| 🧪 Sensory | Usually normal sensation |
| 🩺 Others | Normal bladder and bowel control (unlike myelomeningocele) |
✅ In most cases, neurological function is preserved.
❌ Meningitis
❌ CSF leak
❌ Sac rupture
❌ Hydrocephalus (less common than in myelomeningocele)
❌ Tethered cord syndrome (rare)
🟡 Meningocele = protrusion of meninges only, spinal cord intact
🟡 Sac is CSF-filled, covered by thin skin
🟡 Neurological function usually normal
🟡 Surgery done within 24–48 hours of birth
🟡 Folic acid prevents most NTDs
Q1. Meningocele is a:
🅰️ Brain disorder
🅱️ Closed spinal defect
✅ 🅲️ Protrusion of meninges through spine defect
🅳️ Lung deformity
Correct Answer: 🅲️ Protrusion of meninges through spine defect
Q2. In meningocele, which structure is NOT involved in the sac?
🅰️ Meninges
🅱️ CSF
✅ 🅲️ Spinal cord
🅳️ Dura
Correct Answer: 🅲️ Spinal cord
Q3. Best position for neonate with meningocele:
🅰️ Supine
🅱️ Lateral
✅ 🅲️ Prone
🅳️ Trendelenburg
Correct Answer: 🅲️ Prone
Q4. What is the ideal time for surgical repair of meningocele?
🅰️ After 1 month
✅ 🅱️ Within 24–48 hours
🅲️ 6 months
🅳️ 1 year
Correct Answer: 🅱️ Within 24–48 hours
Q5. What is the key nutrient in NTD prevention?
🅰️ Iron
🅱️ Vitamin A
✅ 🅲️ Folic acid
🅳️ Vitamin D
Correct Answer: 🅲️ Folic acid
Q6. The CSF-filled sac in meningocele is usually located at:
🅰️ Thoracic region
✅ 🅱️ Lumbosacral region
🅲️ Cervical region
🅳️ Skull base
Correct Answer: 🅱️ Lumbosacral region
Q7. Which complication is least likely in meningocele?
🅰️ Meningitis
🅱️ CSF leak
✅ 🅲️ Severe paralysis
🅳️ Sac rupture
Correct Answer: 🅲️ Severe paralysis
📘 Important for Pediatric Nursing, Orthopedic Nursing, Child Health, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Skeletal defects are congenital abnormalities of bones or joints that affect the form, size, or function of the musculoskeletal system. They can be isolated or syndromic, and may be due to genetic mutations, intrauterine factors, or environmental causes.
✳️ These may lead to mobility issues, growth retardation, or cosmetic deformities if not managed early.
| Type | Description |
|---|---|
| 🧬 Genetic | Due to mutations (e.g., achondroplasia) |
| 🤰 Developmental | Due to uterine position or amniotic fluid issues (e.g., clubfoot) |
| 🧪 Teratogenic | Drug exposure, infection, radiation |
| ⚙️ Mechanical | Pressure from multiple gestation or fibroids |
| Type | Example | Description |
|---|---|---|
| 🦴 Bone Size Abnormality | Achondroplasia | Dwarfism with short limbs |
| 🔩 Limb Deformity | Clubfoot, Talipes | Foot turned inward/downward |
| 🧍 Spinal Defect | Scoliosis, Kyphosis | Abnormal spinal curvature |
| 🦿 Joint Deformity | Developmental dysplasia of hip (DDH) | Dislocation/subluxation of hip |
| 💀 Skull Defect | Craniosynostosis | Early fusion of skull sutures |
❌ Physical disability
❌ Growth delay or stunted height
❌ Chronic pain
❌ Recurrent fractures
❌ Psychosocial stress
🟡 Clubfoot = congenital inward foot deformity
🟡 DDH = hip dislocation in infants, Ortolani sign
🟡 Achondroplasia = short limb dwarfism
🟡 Scoliosis = lateral curvature of spine
🟡 Early detection + therapy = better mobility outcome
Q1. Clubfoot is also called:
🅰️ DDH
🅱️ Genu valgum
✅ 🅲️ Talipes equinovarus
🅳️ Scoliosis
Correct Answer: 🅲️ Talipes equinovarus
Q2. Ortolani and Barlow tests are used for:
🅰️ Clubfoot
🅱️ Scoliosis
✅ 🅲️ Developmental dysplasia of hip
🅳️ Hydrocephalus
Correct Answer: 🅲️ Developmental dysplasia of hip
Q3. The most common cause of dwarfism is:
🅰️ Rickets
✅ 🅱️ Achondroplasia
🅲️ Spina bifida
🅳️ Osteomalacia
Correct Answer: 🅱️ Achondroplasia
Q4. In scoliosis, which part is affected?
🅰️ Foot
🅱️ Skull
✅ 🅲️ Spine
🅳️ Hip
Correct Answer: 🅲️ Spine
Q5. Which device is used for DDH?
🅰️ Walker
✅ 🅱️ Pavlik harness
🅲️ Crutch
🅳️ Helmet
Correct Answer: 🅱️ Pavlik harness
Q6. Brittle bone disease is called:
🅰️ Achondroplasia
✅ 🅱️ Osteogenesis imperfecta
🅲️ Scoliosis
🅳️ Talipes
Correct Answer: 🅱️ Osteogenesis imperfecta
Q7. Treatment for clubfoot includes:
🅰️ Traction
🅱️ Sling
✅ 🅲️ Ponseti casting or surgery
🅳️ Craniotomy
Correct Answer: 🅲️ Ponseti casting or surgery
📘 Essential for Pediatric Nursing, Child Health Nursing, Medical-Surgical Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Cleft lip is a congenital split or separation in the upper lip, resulting from incomplete fusion of the maxillary and medial nasal processes during early fetal development (4–7 weeks gestation).
✳️ It may occur unilaterally or bilaterally, and may occur alone or with cleft palate.
| Type | Description |
|---|---|
| 🟩 Unilateral | One side of lip is affected |
| 🟨 Bilateral | Both sides of lip are split |
| 🟥 Complete | Extends into the nose |
| 🟦 Incomplete | Does not reach nasal floor |
| Feature | Observation |
|---|---|
| 👄 Lip | Visible gap or notch in the upper lip |
| 👃 Nose | Deformity of nostril on affected side |
| 🍽️ Feeding | Difficulty in sucking/breastfeeding |
| 🗣️ Speech | Delayed or nasal speech (if palate also involved) |
| 🧠 Psychological | Parental anxiety, social stigma |
❌ Feeding difficulty and failure to thrive
❌ Speech and language delay
❌ Ear infections and hearing loss (if palate involved)
❌ Dental malocclusion
❌ Psychological and social issues
🟡 Cleft lip = congenital split in the upper lip
🟡 Surgery = cheiloplasty at 3–6 months
🟡 Rule of 10s = criteria for surgery
🟡 Use special feeding devices pre-op
🟡 Post-op care = no suction, arm restraints, clean suture line
Q1. Cleft lip results from failure of fusion between:
🅰️ Mandibular and maxillary processes
🅱️ Frontal and maxillary processes
✅ 🅲️ Medial nasal and maxillary processes
🅳️ Mandibular and nasal processes
Correct Answer: 🅲️ Medial nasal and maxillary processes
Q2. Best age for cleft lip surgery is:
🅰️ At birth
🅱️ 1 year
✅ 🅲️ 3–6 months
🅳️ 5 years
Correct Answer: 🅲️ 3–6 months
Q3. Which of the following is part of the Rule of 10s for cleft lip repair?
🅰️ 10 hours age
✅ 🅱️ 10 weeks age
🅲️ 10 months age
🅳️ 10 kg weight
Correct Answer: 🅱️ 10 weeks age
Q4. One important post-op nursing intervention after cleft lip repair is:
🅰️ Prone positioning
🅱️ Suctioning frequently
✅ 🅲️ Use of elbow restraints
🅳️ Feed via nasogastric tube
Correct Answer: 🅲️ Use of elbow restraints
Q5. Major concern in infant with cleft lip:
🅰️ High fever
🅱️ Sleep apnea
✅ 🅲️ Feeding difficulty
🅳️ Jaundice
Correct Answer: 🅲️ Feeding difficulty
Q6. Cleft lip is best diagnosed antenatally by:
🅰️ MRI
🅱️ Amniocentesis
✅ 🅲️ Ultrasound
🅳️ Maternal blood test
Correct Answer: 🅲️ Ultrasound
Q7. Haberman feeder is used for:
🅰️ Phototherapy
✅ 🅱️ Cleft lip/palate feeding
🅲️ Oxygen delivery
🅳️ Nebulization
Correct Answer: 🅱️ Cleft lip/palate feeding
Congenital Oral Defect – Cleft Palate📘 Essential for Pediatric Nursing, Child Health Nursing, ENT, Medical-Surgical Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Cleft palate is a congenital split or opening in the roof of the mouth due to incomplete fusion of the palatal shelves during embryonic development (between 7th and 12th weeks of gestation).
✳️ It may occur alone or with cleft lip, affecting the soft palate, hard palate, or both.
| Type | Description |
|---|---|
| 🟩 Incomplete | Only soft palate is involved |
| 🟥 Complete | Involves both soft and hard palate; may communicate with nasal cavity |
| 🟨 Unilateral / Bilateral | One or both sides of the palate are affected |
| 🟦 Submucous cleft palate | Mucosa intact but underlying muscle and bone are cleft |
| Feature | Description |
|---|---|
| 👅 Palate | Visible split/opening in the roof of the mouth |
| 🍽️ Feeding | Nasal regurgitation of milk, poor sucking |
| 🗣️ Speech | Delayed, hypernasal, unintelligible speech |
| 👂 Ear | Recurrent otitis media, hearing loss (due to Eustachian tube dysfunction) |
| 😷 Others | Growth delay due to feeding problems |
❌ Feeding difficulties
❌ Speech delay
❌ Recurrent ear infections (otitis media)
❌ Dental anomalies
❌ Hearing impairment
❌ Psychological or social issues
🟡 Cleft palate = opening in the roof of the mouth
🟡 Leads to feeding problems and nasal speech
🟡 Surgery (palatoplasty) is done at 9–18 months
🟡 Use special feeders before surgery
🟡 Speech therapy is crucial post-op
Q1. Cleft palate results from failure of fusion of:
🅰️ Nasal septum
🅱️ Maxillary processes
✅ 🅲️ Palatal shelves
🅳️ Mandibular arches
Correct Answer: 🅲️ Palatal shelves
Q2. Ideal age for cleft palate repair is:
🅰️ 1 month
🅱️ 6 months
✅ 🅲️ 9–18 months
🅳️ 5 years
Correct Answer: 🅲️ 9–18 months
Q3. Common speech problem in cleft palate is:
🅰️ Aphasia
🅱️ Stammering
✅ 🅲️ Hypernasal speech
🅳️ Aphonia
Correct Answer: 🅲️ Hypernasal speech
Q4. Which feeder is best for cleft palate?
🅰️ Spoon
🅱️ Bottle
✅ 🅲️ Haberman feeder
🅳️ Nipple shield
Correct Answer: 🅲️ Haberman feeder
Q5. Post-op position after palatoplasty is:
🅰️ Supine
✅ 🅱️ Prone or side-lying
🅲️ Trendelenburg
🅳️ Sitting
Correct Answer: 🅱️ Prone or side-lying
Q6. Which of the following is NOT a complication of cleft palate?
🅰️ Ear infections
🅱️ Nasal regurgitation
✅ 🅲️ Hyperthyroidism
🅳️ Speech difficulty
Correct Answer: 🅲️ Hyperthyroidism
Q7. Nursing goal in cleft palate infant includes:
🅰️ Delay feeding
🅱️ Avoid surgery
✅ 🅲️ Promote feeding and weight gain
🅳️ Use pacifier
Correct Answer: 🅲️ Promote feeding and weight gain
📘 Essential for Pediatric Nursing, Medical-Surgical Nursing, Child Health, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Gastrointestinal tract defects are congenital structural abnormalities affecting the development, continuity, or function of the digestive system, which can lead to feeding problems, obstruction, vomiting, or failure to thrive.
✳️ These anomalies may occur anywhere from the mouth to the anus and often require surgical correction soon after birth.
| Condition | Site Affected | Description |
|---|---|---|
| 🟩 Cleft Lip/Palate | Mouth/Oral cavity | Split in upper lip/palate affecting feeding |
| 🟥 Esophageal Atresia with Tracheoesophageal Fistula (TEF) | Esophagus | Esophagus ends blindly or connects abnormally with trachea |
| 🟨 Pyloric Stenosis | Stomach outlet | Hypertrophy of pyloric muscle → projectile vomiting |
| 🟦 Duodenal Atresia | Duodenum | Complete blockage of duodenal lumen |
| 🟧 Malrotation with Volvulus | Small intestine | Twisting of intestine → obstruction & ischemia |
| 🟫 Hirschsprung’s Disease | Large intestine | Absence of ganglion cells → no peristalsis |
| ⚪ Anorectal Malformation (Imperforate Anus) | Rectum/Anus | Absent or abnormal anal opening |
| 🔶 Omphalocele / Gastroschisis | Abdominal wall | Intestines protrude outside the body (with or without sac) |
| Condition | Common Signs |
|---|---|
| TEF/Esophageal Atresia | Frothy secretions, choking, coughing, cyanosis during feeds |
| Pyloric Stenosis | Non-bilious projectile vomiting, olive-shaped mass, dehydration |
| Duodenal Atresia | Bilious vomiting, “double bubble” sign on X-ray |
| Malrotation/Volvulus | Sudden abdominal pain, bilious vomiting, bloody stool |
| Hirschsprung’s Disease | Delayed meconium, abdominal distension, constipation |
| Anorectal Malformation | No anal opening, abdominal distension, no meconium passage |
| Gastroschisis/Omphalocele | Visible bowel loops at birth, risk of infection and fluid loss |
| Condition | Treatment |
|---|---|
| TEF / Esophageal atresia | Surgical repair (ligation of fistula & anastomosis) |
| Pyloric stenosis | Pyloromyotomy (Ramstedt’s procedure) |
| Duodenal atresia | Duodenoduodenostomy |
| Hirschsprung’s disease | Pull-through surgery after colostomy |
| Imperforate anus | Anoplasty or staged reconstruction |
| Gastroschisis / Omphalocele | Sterile cover at birth, staged surgical closure |
❌ Aspiration pneumonia (TEF)
❌ Dehydration and weight loss
❌ Intestinal perforation or sepsis
❌ Delayed growth and development
❌ Stoma-related issues (in colostomy cases)
❌ Nutritional deficiencies
🟡 TEF = 3 C’s: Coughing, Choking, Cyanosis during feeds
🟡 Pyloric stenosis = olive-shaped mass + projectile vomiting
🟡 Duodenal atresia = “double bubble” sign
🟡 Hirschsprung’s = aganglionic colon + delayed meconium
🟡 Omphalocele = bowel covered with membrane, gastroschisis = no membrane
Q1. Which GI defect causes choking and cyanosis during feeding?
🅰️ Hirschsprung’s disease
✅ 🅱️ Tracheoesophageal fistula
🅲️ Pyloric stenosis
🅳️ Gastroschisis
Correct Answer: 🅱️ Tracheoesophageal fistula
Q2. Olive-shaped abdominal mass is characteristic of:
🅰️ Gastroschisis
✅ 🅱️ Pyloric stenosis
🅲️ Duodenal atresia
🅳️ Hirschsprung’s
Correct Answer: 🅱️ Pyloric stenosis
Q3. The “double bubble” sign on X-ray suggests:
🅰️ TEF
🅱️ Gastroschisis
✅ 🅲️ Duodenal atresia
🅳️ Volvulus
Correct Answer: 🅲️ Duodenal atresia
Q4. Which defect involves absence of ganglion cells in colon?
🅰️ TEF
🅱️ Imperforate anus
✅ 🅲️ Hirschsprung’s disease
🅳️ Cleft palate
Correct Answer: 🅲️ Hirschsprung’s disease
Q5. Which condition presents as bowel loops outside the body without sac?
🅰️ Omphalocele
✅ 🅱️ Gastroschisis
🅲️ TEF
🅳️ Hernia
Correct Answer: 🅱️ Gastroschisis
Q6. First nursing action for visible gastroschisis at birth:
🅰️ Cut the cord
✅ 🅱️ Cover with sterile saline-soaked gauze
🅲️ Feed the baby
🅳️ Place baby in Trendelenburg position
Correct Answer: 🅱️ Cover with sterile saline-soaked gauze
Q7. Common finding in imperforate anus:
🅰️ Frothy oral secretions
🅱️ Yellow vomiting
✅ 🅲️ Absent anal opening and no meconium
🅳️ Frequent diarrhea
Correct Answer: 🅲️ Absent anal opening and no meconium
📘 Essential for Pediatric Nursing, Medical-Surgical Nursing, Neonatology, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Esophageal atresia (EA) is a congenital condition where the esophagus ends in a blind pouch, not connected to the stomach.
Tracheoesophageal fistula (TEF) is an abnormal connection between the trachea and esophagus.
✳️ They often occur together and result in feeding difficulty, aspiration, and respiratory distress in newborns.
| Type | Description | Frequency |
|---|---|---|
| 🟥 Type C (Most common) | Esophageal atresia + distal TEF | ~85% |
| 🟨 Type A | Pure EA without TEF | 7–8% |
| 🟩 Type E (H-type fistula) | No atresia, but TEF present | 4–5% |
| 🟦 Other rare types | EA + proximal/distal TEF | <1% |
| Sign | Description |
|---|---|
| 🍼 Feeding | Coughing, choking, cyanosis during feeds (3 C’s) |
| 🫁 Respiratory | Excessive drooling, frothy secretions, respiratory distress |
| 💧 GI | Abdominal distension (air entering stomach via fistula) |
| 🚫 Feeding tube | Cannot pass NG tube into stomach (meets obstruction) |
❌ Aspiration pneumonia
❌ Anastomotic leak
❌ Esophageal stricture
❌ Gastroesophageal reflux (GERD)
❌ Recurrent fistula
❌ Tracheomalacia (soft trachea)
🟡 EA with TEF = newborn with 3 C’s: coughing, choking, cyanosis during feeding
🟡 Most common type = Type C (EA with distal TEF)
🟡 Polyhydramnios = prenatal clue for EA/TEF
🟡 X-ray = coiled NG tube + air in abdomen
🟡 Surgery = ligation + end-to-end anastomosis
Q1. Most common type of esophageal atresia is:
🅰️ EA without TEF
🅱️ EA with proximal TEF
✅ 🅲️ EA with distal TEF (Type C)
🅳️ H-type fistula
Correct Answer: 🅲️ EA with distal TEF (Type C)
Q2. Classic sign of EA with TEF in newborn is:
🅰️ Cyanosis at rest
✅ 🅱️ Coughing, choking, and cyanosis during feeding
🅲️ Green vomiting
🅳️ Projectile vomiting
Correct Answer: 🅱️ Coughing, choking, and cyanosis during feeding
Q3. Which prenatal clue suggests EA/TEF?
🅰️ Breech position
🅱️ Low amniotic fluid
✅ 🅲️ Polyhydramnios
🅳️ Small placenta
Correct Answer: 🅲️ Polyhydramnios
Q4. Chest X-ray in EA with distal TEF shows:
🅰️ Clear lung fields
✅ 🅱️ Coiled NG tube + gas in abdomen
🅲️ Airless abdomen
🅳️ Enlarged heart
Correct Answer: 🅱️ Coiled NG tube + gas in abdomen
Q5. What is the first nursing action in suspected TEF?
🅰️ Feed the baby slowly
🅱️ Administer oral glucose
✅ 🅲️ Keep NPO and insert suction tube
🅳️ Position prone
Correct Answer: 🅲️ Keep NPO and insert suction tube
Q6. A complication after repair of TEF is:
🅰️ Renal failure
✅ 🅱️ Anastomotic stricture
🅲️ Appendicitis
🅳️ Hypoglycemia
Correct Answer: 🅱️ Anastomotic stricture
Q7. TEF is commonly associated with which syndrome?
🅰️ TORCH
🅱️ Marfan
✅ 🅲️ VACTERL
🅳️ Wilson’s
Correct Answer: 🅲️ VACTERL
📘 Essential for Pediatric Nursing, Child Health Nursing, Medical-Surgical Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Pyloric stenosis (also called infantile hypertrophic pyloric stenosis) is a condition where the pyloric muscle (between the stomach and duodenum) becomes abnormally thickened, leading to obstruction of gastric emptying.
✳️ Most commonly affects male infants between 2–6 weeks of age.
🔁 Hypertrophy + hyperplasia of pyloric muscle
⬇️
⛔ Narrowing of pyloric canal
⬇️
🍽️ Obstructed gastric outflow
⬇️
🤮 Forceful (projectile) non-bilious vomiting
⬇️
💧 Dehydration and electrolyte imbalance
| Feature | Description |
|---|---|
| ✅ Projectile vomiting | Non-bilious, occurs after feeds |
| ✅ Palpable olive-like mass | In right upper abdomen |
| ✅ Visible peristalsis | Moves from left to right across stomach |
| Test | Findings |
|---|---|
| 🧪 Electrolytes | ↓ Sodium, ↓ Chloride, ↓ Potassium; metabolic alkalosis |
| 🟡 Ultrasound abdomen | Best choice: thickened pyloric muscle (>3mm) and elongated canal |
| 🟠 Barium meal | “String sign” or “railroad track sign” (less commonly used now) |
| 🧪 Blood gas | Shows metabolic alkalosis (↑ pH, ↑ bicarbonate) |
❌ Severe dehydration
❌ Electrolyte imbalance
❌ Metabolic alkalosis
❌ Malnutrition & weight loss
❌ Aspiration pneumonia
🟡 Pyloric stenosis = hypertrophy of pyloric muscle
🟡 Classic sign = projectile, non-bilious vomiting
🟡 Olive-like mass felt in right upper abdomen
🟡 Metabolic alkalosis due to H⁺ loss
🟡 Treatment = Ramstedt’s pyloromyotomy
Q1. A key symptom of pyloric stenosis is:
🅰️ Bilious vomiting
🅱️ Fever
✅ 🅲️ Projectile non-bilious vomiting
🅳️ Constipation
Correct Answer: 🅲️ Projectile non-bilious vomiting
Q2. Common age of onset for pyloric stenosis is:
🅰️ Newborn
🅱️ >6 months
✅ 🅲️ 2–6 weeks
🅳️ 2 years
Correct Answer: 🅲️ 2–6 weeks
Q3. Classic electrolyte imbalance in pyloric stenosis:
🅰️ Hyperkalemia
🅱️ Hyponatremia and hyperchloremia
✅ 🅲️ Hypokalemia and hypochloremia
🅳️ Acidosis
Correct Answer: 🅲️ Hypokalemia and hypochloremia
Q4. Diagnostic investigation of choice is:
🅰️ X-ray chest
🅱️ Barium enema
✅ 🅲️ Ultrasound abdomen
🅳️ Endoscopy
Correct Answer: 🅲️ Ultrasound abdomen
Q5. Surgery done for pyloric stenosis is:
🅰️ Colectomy
🅱️ Fundoplication
✅ 🅲️ Ramstedt’s pyloromyotomy
🅳️ Laparotomy
Correct Answer: 🅲️ Ramstedt’s pyloromyotomy
Q6. Pre-operative nursing action includes:
🅰️ Start oral feeding
✅ 🅱️ NPO and start IV fluids
🅲️ Give sedatives
🅳️ Encourage prone position
Correct Answer: 🅱️ NPO and start IV fluids
Q7. Metabolic condition associated with pyloric stenosis:
🅰️ Metabolic acidosis
🅱️ Respiratory acidosis
✅ 🅲️ Metabolic alkalosis
🅳️ Respiratory alkalosis
Correct Answer: 🅲️ Metabolic alkalosis
📘 Essential for Pediatric Nursing, Neonatology, Surgery, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Anorectal malformations (ARMs) are congenital defects of the rectum and anus, where the normal anal opening is absent, misplaced, or abnormally connected to other organs.
✳️ The severity ranges from imperforate anus to fistulous connections with genitourinary system.
| Type | Description | Gender |
|---|---|---|
| 🟩 Low-type | Anus close to normal site, no fistula | Boys & Girls |
| 🟥 Intermediate | Rectum ends above pelvic floor | Boys & Girls |
| 🟨 High-type | Rectum ends higher in pelvis, often with fistula | Boys: rectourethral / Girls: rectovaginal |
| 🟦 Fistula types | Abnormal connection with urethra, bladder, vagina, perineum | Common in high ARMs |
| Sign | Description |
|---|---|
| 🟥 Absent anus | No anal opening on inspection |
| 🟨 No passage of meconium within 24–48 hrs | Suggests intestinal obstruction |
| 🟦 Meconium from abnormal opening | E.g., meconium in urine or vagina |
| 🟩 Abdominal distension | Due to fecal retention |
| 🟧 Crying, vomiting, irritability | Obstructive symptoms |
| Surgery | Indication |
|---|---|
| 🟢 Anoplasty | For low-type ARM |
| 🟡 Colostomy + Posterior Sagittal Anorectoplasty (PSARP) | For high/intermediate-type ARM |
| 🟣 Staged repair | 1. Colostomy → 2. PSARP → 3. Colostomy closure |
❌ Constipation or fecal incontinence
❌ Anal stenosis or stricture
❌ Recurrent fistula or abscess
❌ Delayed toilet training
❌ Psychological impact
🟡 ARM = absence or malposition of anal opening
🟡 Imperforate anus is most common presentation
🟡 Initial sign = no meconium in first 48 hrs
🟡 Surgery = Anoplasty / PSARP
🟡 Associated with VACTERL syndrome
Q1. Anorectal malformation is a:
🅰️ Acquired defect
🅱️ Inflammatory disorder
✅ 🅲️ Congenital malformation of anus/rectum
🅳️ Nutritional disease
Correct Answer: 🅲️ Congenital malformation of anus/rectum
Q2. A newborn hasn’t passed meconium in 48 hrs. First suspicion?
🅰️ TEF
🅱️ Pyloric stenosis
✅ 🅲️ Anorectal malformation
🅳️ Intussusception
Correct Answer: 🅲️ Anorectal malformation
Q3. Most appropriate imaging for ARM at birth:
🅰️ Chest X-ray
🅱️ CT scan
✅ 🅲️ Invertogram / cross-table lateral X-ray
🅳️ ECG
Correct Answer: 🅲️ Invertogram / cross-table lateral X-ray
Q4. High-type ARM in males commonly presents with fistula to:
🅰️ Skin
✅ 🅱️ Urethra or bladder
🅲️ Stomach
🅳️ Duodenum
Correct Answer: 🅱️ Urethra or bladder
Q5. Initial nursing action in ARM is:
🅰️ Start feeding
✅ 🅱️ NPO and IV fluids
🅲️ Give laxative
🅳️ Administer oral antibiotics
Correct Answer: 🅱️ NPO and IV fluids
Q6. Surgery done for low ARM is:
🅰️ Colostomy
✅ 🅱️ Anoplasty
🅲️ Appendectomy
🅳️ Herniorrhaphy
Correct Answer: 🅱️ Anoplasty
Q7. ARM is associated with which syndrome?
🅰️ Marfan
✅ 🅱️ VACTERL
🅲️ TORCH
🅳️ Wilson’s
Correct Answer: 🅱️ VACTERL
📘 Essential for Pediatric Nursing, Medical-Surgical Nursing, Child Health Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
A hernia is the protrusion of an organ or tissue through an abnormal opening in the surrounding muscle or tissue wall. In congenital cases, it occurs due to incomplete closure of fetal anatomical passages.
✳️ In children, congenital hernias often involve the abdominal wall and may be visible during crying or straining.
| Type | Site | Description |
|---|---|---|
| 🟩 Inguinal hernia | Groin | Abdominal contents protrude through inguinal canal |
| 🟥 Umbilical hernia | Belly button | Intestine bulges through weak umbilical ring |
| 🟨 Epigastric hernia | Midline above umbilicus | Small fatty mass protrudes through linea alba |
| 🟦 Diaphragmatic hernia | Thoracic cavity | Abdominal organs herniate into chest cavity |
| 🟪 Hiatal hernia | Esophageal opening | Stomach herniates through diaphragm into thorax |
| 🟫 Femoral hernia | Upper thigh (rare in children) | Protrudes through femoral canal below inguinal ligament |
| Site | Signs |
|---|---|
| 🟢 Inguinal | Soft groin swelling, increases with crying, may reduce on lying down |
| 🟡 Umbilical | Soft bulge at umbilicus, especially when baby cries |
| 🔵 Diaphragmatic | Respiratory distress at birth, scaphoid abdomen, cyanosis |
| 🟠 Hiatal | Regurgitation, vomiting, feeding difficulties |
❌ Incarceration (non-reducible hernia)
❌ Strangulation (cut-off blood supply → emergency)
❌ Bowel obstruction
❌ Respiratory failure (in diaphragmatic hernia)
❌ Recurrence (rare with proper repair)
🟡 Hernia = protrusion through a weak muscle wall
🟡 Most common hernia in infants = inguinal (especially in preterm boys)
🟡 Diaphragmatic hernia = respiratory distress at birth + scaphoid abdomen
🟡 Umbilical hernia often resolves by age 2–3 years
🟡 Inguinal hernias always need surgery in children
Q1. Most common type of hernia in newborns is:
🅰️ Femoral
🅱️ Hiatal
✅ 🅲️ Inguinal
🅳️ Ventral
Correct Answer: 🅲️ Inguinal
Q2. Umbilical hernia in a 1-year-old is best managed by:
🅰️ Immediate surgery
✅ 🅱️ Observation (may resolve on its own)
🅲️ Bandage tightly
🅳️ Bed rest
Correct Answer: 🅱️ Observation
Q3. Danger sign in hernia is:
🅰️ Soft reducible swelling
🅱️ Swelling during crying only
✅ 🅲️ Irreducible tender mass with vomiting
🅳️ Flat abdomen
Correct Answer: 🅲️ Irreducible tender mass with vomiting
Q4. Scaphoid abdomen in newborn with respiratory distress suggests:
🅰️ Pyloric stenosis
🅱️ Hiatal hernia
✅ 🅲️ Congenital diaphragmatic hernia
🅳️ Intussusception
Correct Answer: 🅲️ Congenital diaphragmatic hernia
Q5. Surgery for inguinal hernia in a child is called:
🅰️ Fundoplication
✅ 🅱️ Herniotomy
🅲️ Laparotomy
🅳️ Colectomy
Correct Answer: 🅱️ Herniotomy
Q6. Hiatal hernia may present with:
🅰️ Diarrhea
✅ 🅱️ Regurgitation and vomiting
🅲️ Cyanosis
🅳️ Clubbing
Correct Answer: 🅱️ Regurgitation and vomiting
Q7. Best position to reduce respiratory distress in diaphragmatic hernia:
🅰️ Supine
🅱️ Prone
✅ 🅲️ Head elevated with nasogastric decompression
🅳️ Trendelenburg
Correct Answer: 🅲️ Head elevated with nasogastric decompression
📘 Essential for Pediatric Nursing, Child Health Nursing, Surgery, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
🔽 Hypospadias: A congenital condition where the urethral opening is located on the underside (ventral surface) of the penis, anywhere from the glans to the perineum.
🔼 Epispadias: A rarer condition where the urethral opening is on the upper (dorsal) surface of the penis, or in females, near the clitoris.
✳️ Both are congenital malformations and may be isolated or part of genitourinary syndromes.
| Type | Urethral Opening Location |
|---|---|
| 🟢 Glanular | Near tip of glans penis (mildest) |
| 🟡 Coronal/Subcoronal | Below the head of penis |
| 🟠 Midshaft | Middle of penile shaft |
| 🔵 Penoscrotal | Where penis meets scrotum |
| 🔴 Perineal | Behind the scrotum (most severe) |
| Feature | Hypospadias | Epispadias |
|---|---|---|
| 🔽 Meatus location | Ventral (underside) | Dorsal (upper side) |
| 🚽 Urination | Downward/abnormal stream | Upward/abnormal stream |
| 🎯 Associated issues | Chordee (penile curvature), incomplete foreskin (hooded) | Bladder exstrophy (in severe cases) |
| 🧬 Fertility | May affect ejaculation | May affect continence |
| 👶 Appearance | Cosmetic abnormality | Severe cosmetic + functional issues |
| Defect | Surgery | Goal |
|---|---|---|
| Hypospadias | Urethroplasty | Create functional urethra at tip |
| Epispadias | Complex reconstructive surgery | Correct meatus, curvature, continence |
✳️ Severe epispadias may require multi-stage repair and involve bladder reconstruction if part of exstrophy complex.
❌ Urethrocutaneous fistula
❌ Meatal stenosis
❌ Urinary retention or incontinence
❌ Cosmetic dissatisfaction
❌ Emotional/psychosocial issues
🟡 Hypospadias = urethral opening on underside of penis
🟡 Epispadias = urethral opening on upper side
🟡 Do not circumcise babies with hypospadias
🟡 Surgery done at 6–18 months
🟡 Post-op care includes catheter care & infection prevention
Q1. In hypospadias, the urethral opening is located on the:
🅰️ Dorsal surface
✅ 🅱️ Ventral surface
🅲️ Glans tip
🅳️ Inside scrotum
Correct Answer: 🅱️ Ventral surface
Q2. Which is true about epispadias?
🅰️ More common than hypospadias
✅ 🅱️ Urethral opening on dorsal penis
🅲️ No surgical repair needed
🅳️ Affects only females
Correct Answer: 🅱️ Urethral opening on dorsal penis
Q3. Why should circumcision be avoided in hypospadias?
🅰️ It is painful
🅱️ Cosmetic reason
✅ 🅲️ Foreskin needed for surgical repair
🅳️ It causes infection
Correct Answer: 🅲️ Foreskin needed for surgical repair
Q4. Ideal age for surgical correction is:
🅰️ 3–4 years
🅱️ After puberty
✅ 🅲️ 6–18 months
🅳️ At birth
Correct Answer: 🅲️ 6–18 months
Q5. Common complication after hypospadias repair is:
🅰️ Renal failure
✅ 🅱️ Urethrocutaneous fistula
🅲️ Appendicitis
🅳️ Stridor
Correct Answer: 🅱️ Urethrocutaneous fistula
Q6. A child with hypospadias should be referred to:
🅰️ Dermatologist
✅ 🅱️ Pediatric urologist
🅲️ Gastroenterologist
🅳️ Cardiologist
Correct Answer: 🅱️ Pediatric urologist
Q7. Epispadias is more commonly associated with:
🅰️ Clubfoot
✅ 🅱️ Bladder exstrophy
🅲️ GERD
🅳️ TEF
Correct Answer: 🅱️ Bladder exstrophy
📘 Essential for Pediatric Nursing, Neonatology, Urology, Medical-Surgical Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Bladder exstrophy is a rare congenital anomaly in which the anterior wall of the bladder and the lower abdominal wall fail to close during fetal development, resulting in the bladder being exposed outside the body.
✳️ It is part of the Exstrophy-Epispadias Complex, often associated with epispadias, pubic diastasis, and genital defects.
| Type | Description |
|---|---|
| 🟩 Epispadias | Urethra opens on dorsal penis or near clitoris |
| 🟥 Classic Bladder Exstrophy | Bladder mucosa exposed, ureters visible |
| 🟨 Cloacal Exstrophy | Most severe; bladder + intestines + genital defects + imperforate anus |
| System | Findings |
|---|---|
| 🔴 Bladder | Bright red bladder mucosa exposed on lower abdomen |
| 🟠 Ureters | Constant dribbling of urine from ureteral orifices |
| 🟡 Urethra | Epispadias often present |
| 🟢 Pelvis | Wide separation of pubic bones (pubic diastasis) |
| 🔵 Genitals | Undescended testes, small penis in males; bifid clitoris in females |
| ⚪ Others | May be associated with inguinal hernia, umbilical hernia, spine anomalies |
| Stage | Goal |
|---|---|
| 🟢 Primary bladder closure | Done within 48–72 hours of birth |
| 🟡 Epispadias repair | Usually around 6–12 months |
| 🟠 Bladder neck reconstruction | To achieve urinary continence (4–5 years) |
| 🔵 Orthopedic repair | For pubic bone separation if needed |
✳️ In cloacal exstrophy, additional surgeries for intestine, genitalia, and anal canal are required.
❌ Urinary incontinence
❌ Recurrent urinary tract infections
❌ Vesicoureteral reflux (VUR)
❌ Infertility (in severe cases)
❌ Renal damage (if left untreated)
❌ Psychosocial and body image issues
🟡 Bladder exstrophy = bladder exposed through lower abdominal wall
🟡 Strongly associated with epispadias
🟡 Cover exposed bladder with sterile saline gauze
🟡 Surgery done in multiple stages, starting within 72 hrs
🟡 Nursing focus = infection control, fluid management, emotional support
Q1. Exstrophy of the bladder is a defect in:
🅰️ GI tract
✅ 🅱️ Lower abdominal wall and bladder
🅲️ Spinal cord
🅳️ Cranial bones
Correct Answer: 🅱️ Lower abdominal wall and bladder
Q2. Which structure is commonly exposed in bladder exstrophy?
🅰️ Intestine
🅱️ Stomach
✅ 🅲️ Bladder mucosa
🅳️ Umbilical cord
Correct Answer: 🅲️ Bladder mucosa
Q3. Immediate nursing action for bladder exstrophy at birth is:
🅰️ Give oral feed
✅ 🅱️ Cover bladder with sterile moist dressing
🅲️ Apply diaper
🅳️ Turn baby prone
Correct Answer: 🅱️ Cover bladder with sterile moist dressing
Q4. Which surgical step is done first in bladder exstrophy repair?
🅰️ Epispadias correction
✅ 🅱️ Primary bladder closure
🅲️ Bladder neck reconstruction
🅳️ Pubic bone fixation
Correct Answer: 🅱️ Primary bladder closure
Q5. Pubic diastasis refers to:
🅰️ Wide chest
🅱️ Kidney defect
✅ 🅲️ Separation of pubic bones
🅳️ Bowel obstruction
Correct Answer: 🅲️ Separation of pubic bones
Q6. Which of the following is NOT a complication of bladder exstrophy?
🅰️ Urinary incontinence
🅱️ VUR
✅ 🅲️ Congenital heart defect
🅳️ UTI
Correct Answer: 🅲️ Congenital heart defect
Q7. Bladder exstrophy is more common in:
🅰️ Females
✅ 🅱️ Males
🅲️ Twins
🅳️ Preterm only
Correct Answer: 🅱️ Males
📘 Essential for Pediatric Nursing, Medical-Surgical Nursing, Urology, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
🔒 Phimosis: A condition where the foreskin (prepuce) cannot be retracted over the glans penis due to tightness or scarring.
🔓 Paraphimosis: A condition where the retracted foreskin becomes trapped behind the glans and cannot be pulled forward, leading to painful constriction and swelling.
✳️ Phimosis = Cannot pull back foreskin
✳️ Paraphimosis = Foreskin pulled back & stuck
| Type | Phimosis | Paraphimosis |
|---|---|---|
| 🟩 Physiological | Normal in newborns and infants | N/A |
| 🟥 Pathological | Due to infection, scarring (e.g., balanitis) | Retraction-induced emergency |
| Feature | Phimosis | Paraphimosis |
|---|---|---|
| 👀 Appearance | Tight foreskin, can’t retract | Swollen, painful glans with tight ring behind it |
| 🚽 Urination | Ballooning of foreskin during urination | Painful urination or urinary retention |
| 🔴 Pain | Usually painless (unless infected) | Painful and urgent |
| 💧 Discharge | Possible smegma or pus in infection | Edema of foreskin and glans |
| 🩸 Circulation | Normal | Risk of ischemia or gangrene (emergency) |
| Management | Description |
|---|---|
| 🟢 Observation | Normal in infants; resolves by age 3–5 |
| 🟡 Topical steroids | Betamethasone cream for 4–6 weeks |
| 🔵 Gentle stretching | Under supervision, no forceful pulling |
| 🔴 Circumcision | Indicated if recurrent infection, urinary difficulty, or ballooning persists |
| Management | Description |
|---|---|
| ⚠️ Manual reduction | Apply ice/compression → gently reduce foreskin over glans |
| 💉 Dorsal slit procedure | If manual reduction fails |
| 🔪 Circumcision | Definitive treatment to prevent recurrence |
| 💊 Analgesics + Antibiotics | If infection or inflammation present |
| Phimosis | Paraphimosis |
|---|---|
| Recurrent balanitis | Painful ischemia of glans |
| Urinary retention | Gangrene (if untreated) |
| UTIs | Permanent damage to glans |
| Voiding difficulty | Requires emergency intervention |
🟡 Phimosis = foreskin cannot retract
🟡 Paraphimosis = retracted foreskin stuck behind glans
🟡 Phimosis is normal in infants
🟡 Paraphimosis is a surgical emergency
🟡 Circumcision = definitive treatment in recurrent cases
Q1. In phimosis, the foreskin:
🅰️ Can retract easily
🅱️ Is absent
✅ 🅲️ Cannot be pulled back over the glans
🅳️ Becomes swollen
Correct Answer: 🅲️ Cannot be pulled back over the glans
Q2. Paraphimosis is considered a/an:
🅰️ Normal variation
🅱️ Mild infection
✅ 🅲️ Surgical emergency
🅳️ Skin rash
Correct Answer: 🅲️ Surgical emergency
Q3. First-line treatment for mild phimosis in children is:
🅰️ Circumcision
✅ 🅱️ Topical steroid application
🅲️ Antibiotics
🅳️ Foley catheter
Correct Answer: 🅱️ Topical steroid application
Q4. Most serious complication of untreated paraphimosis:
🅰️ UTI
✅ 🅱️ Gangrene of glans
🅲️ Rash
🅳️ Hernia
Correct Answer: 🅱️ Gangrene of glans
Q5. Which of the following is a normal developmental condition in infants?
🅰️ Paraphimosis
✅ 🅱️ Physiological phimosis
🅲️ Bladder exstrophy
🅳️ Inguinal hernia
Correct Answer: 🅱️ Physiological phimosis
Q6. A nursing priority in a child with paraphimosis:
🅰️ Give ORS
✅ 🅱️ Refer immediately for manual reduction
🅲️ Start iron therapy
🅳️ Perform catheterization
Correct Answer: 🅱️ Refer immediately for manual reduction
Q7. Phimosis becomes pathological when:
🅰️ Baby is 3 months old
🅱️ Baby passes urine normally
✅ 🅲️ Pain, infection, and ballooning occur during urination
🅳️ Circumcision already done
Correct Answer: 🅲️ Pain, infection, and ballooning occur during urination
📘 Essential for Pediatric Nursing, Child Health Nursing, Surgery, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Cryptorchidism refers to a condition where one or both testes fail to descend into the scrotum during fetal development and remain in the abdomen or inguinal canal at birth.
✳️ Also called undescended testis, it is the most common genital abnormality in male infants.
| Type | Location | Description |
|---|---|---|
| 🟩 Unilateral | One testis undescended | Most common |
| 🟥 Bilateral | Both testes undescended | Higher risk of infertility |
| 🟨 Palpable | Felt in inguinal canal or upper scrotum | 80% of cases |
| 🟦 Non-palpable | Intra-abdominal or absent | Requires imaging/surgery |
| 🟪 Ectopic testis | Testis descends to abnormal location (e.g., thigh) | Rare |
| Feature | Observation |
|---|---|
| 🩺 Scrotal asymmetry | One side appears underdeveloped or empty |
| 🤲 Absent testis | Not palpable in scrotum during physical exam |
| 🚫 Fertility | Risk of infertility if untreated |
| 🔥 Risk | Higher chance of testicular torsion, trauma, hernia, or malignancy |
✳️ Most testicles descend spontaneously by 3–6 months of age.
| Procedure | Description |
|---|---|
| 🔧 Orchidopexy | Surgical repositioning of testis into scrotum (done between 6–18 months of age) |
| 🧬 Orchidectomy | Removal of atrophic/absent testis (if non-functional or high malignancy risk) |
❌ Infertility (especially in bilateral cases)
❌ Testicular cancer (higher risk if untreated)
❌ Inguinal hernia
❌ Torsion or trauma
❌ Psychological/body image issues in adolescence
🟡 Cryptorchidism = undescended testis
🟡 Most cases resolve by 6 months
🟡 Surgery = orchidopexy, done at 6–18 months
🟡 Increases risk of infertility & malignancy
🟡 Always examine scrotum during newborn checkup
Q1. Cryptorchidism refers to:
🅰️ Scrotal swelling
✅ 🅱️ Undescended testis
🅲️ Hypospadias
🅳️ Enlarged prostate
Correct Answer: 🅱️ Undescended testis
Q2. Ideal age for orchidopexy is:
🅰️ At birth
🅱️ 3 years
✅ 🅲️ 6–18 months
🅳️ After puberty
Correct Answer: 🅲️ 6–18 months
Q3. Most common location of undescended testis is:
🅰️ Abdominal cavity
✅ 🅱️ Inguinal canal
🅲️ Thigh
🅳️ Bladder
Correct Answer: 🅱️ Inguinal canal
Q4. Which hormone is sometimes used in cryptorchidism therapy?
🅰️ Insulin
🅱️ TSH
✅ 🅲️ hCG
🅳️ FSH
Correct Answer: 🅲️ hCG
Q5. Which complication is associated with untreated cryptorchidism?
🅰️ Diabetes
🅱️ Renal failure
✅ 🅲️ Testicular cancer
🅳️ Glaucoma
Correct Answer: 🅲️ Testicular cancer
Q6. In bilateral non-palpable testes, which investigation is essential?
🅰️ ECG
🅱️ Liver function test
✅ 🅲️ Hormonal assay and imaging
🅳️ CBC
Correct Answer: 🅲️ Hormonal assay and imaging
Q7. Orchidopexy helps in:
🅰️ Preventing jaundice
✅ 🅱️ Preserving fertility and preventing malignancy
🅲️ Improving vision
🅳️ Controlling hypertension
Correct Answer: 🅱️ Preserving fertility and preventing malignancy
📘 Essential for Pediatric Nursing, Medical-Surgical Nursing, Child Health Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Polycystic kidney disease (PKD) is a genetic disorder characterized by the development of multiple fluid-filled cysts in the kidneys, which enlarge the kidneys and impair their function over time.
✳️ It can be autosomal dominant (ADPKD) or autosomal recessive (ARPKD).
| Type | Inheritance | Age of Onset | Features |
|---|---|---|---|
| 🟩 Autosomal Dominant PKD (ADPKD) | One affected parent | Adulthood (30–40 yrs) | Bilateral kidney enlargement, cysts in other organs |
| 🟥 Autosomal Recessive PKD (ARPKD) | Both parents carriers | Infancy / childhood | Severe, rapid progression, associated with liver fibrosis |
✳️ ARPKD is the common pediatric type, often diagnosed prenatally or in neonates.
| System | Signs/Symptoms |
|---|---|
| 🧫 Renal | Enlarged kidneys, abdominal mass, decreased urine output |
| 💨 Respiratory | Respiratory distress (due to Potter’s sequence) |
| 💧 Fluid & Electrolyte | Hyponatremia, acidosis |
| 🫀 Hypertension | Common early sign |
| 🧬 Others | Failure to thrive, hepatic fibrosis, portal hypertension |
🔁 No curative treatment; supportive care and symptom management essential
❌ Chronic kidney disease (CKD) / ESRD
❌ Hypertension
❌ Recurrent UTI
❌ Respiratory failure (in neonates)
❌ Hepatic fibrosis and portal hypertension (in ARPKD)
❌ Intracranial aneurysm (in ADPKD)
🟡 PKD = multiple renal cysts + progressive kidney damage
🟡 ARPKD = childhood onset, ADPKD = adult onset
🟡 Antenatal USG can detect ARPKD prenatally
🟡 Common signs in infants = enlarged kidneys, respiratory distress
🟡 No cure → manage BP, infections, prepare for dialysis or transplant
Q1. Polycystic kidney disease is characterized by:
🅰️ Absent kidneys
🅱️ Kidney tumor
✅ 🅲️ Multiple fluid-filled renal cysts
🅳️ Unilateral kidney shrinkage
Correct Answer: 🅲️ Multiple fluid-filled renal cysts
Q2. ARPKD is commonly seen in:
🅰️ Adults
✅ 🅱️ Infants and neonates
🅲️ Teenagers
🅳️ Post-menopausal women
Correct Answer: 🅱️ Infants and neonates
Q3. A key early sign of ARPKD in newborns is:
🅰️ Diarrhea
🅱️ Rash
✅ 🅲️ Respiratory distress
🅳️ Seizures
Correct Answer: 🅲️ Respiratory distress
Q4. The definitive treatment for end-stage PKD is:
🅰️ Diuretics
🅱️ Antibiotics
✅ 🅲️ Kidney transplant
🅳️ High-protein diet
Correct Answer: 🅲️ Kidney transplant
Q5. A major complication of ARPKD involving the liver is:
🅰️ Cirrhosis
✅ 🅱️ Hepatic fibrosis and portal hypertension
🅲️ Hepatitis A
🅳️ Liver cancer
Correct Answer: 🅱️ Hepatic fibrosis and portal hypertension
Q6. A common associated condition in adult PKD is:
🅰️ Scoliosis
🅱️ Jaundice
✅ 🅲️ Intracranial aneurysm
🅳️ Glaucoma
Correct Answer: 🅲️ Intracranial aneurysm
Q7. In PKD, blood pressure is usually:
🅰️ Low
✅ 🅱️ Elevated
🅲️ Normal
🅳️ Fluctuating randomly
Correct Answer: 🅱️ Elevated
📘 Essential for Pediatric Nursing, Neonatology, Endocrinology, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Ambiguous genitalia refers to a congenital condition in which the external genital organs do not appear clearly male or female, making sex identification at birth uncertain.
✳️ It is also called Disorders of Sexual Differentiation (DSD) or Intersex condition.
| Type | Karyotype | Description |
|---|---|---|
| 🟩 46, XX DSD | Female (XX) | Excess androgen → masculinized genitalia (e.g., congenital adrenal hyperplasia) |
| 🟥 46, XY DSD | Male (XY) | Deficient androgen action → underdeveloped male genitalia |
| 🟨 True Hermaphroditism | XX/XY mosaic | Both ovarian & testicular tissue present |
| 🟦 Mixed Gonadal Dysgenesis | Abnormal gonads | Genitalia often ambiguous |
| 🟪 Chromosomal anomalies | Turner (45,X), Klinefelter (47,XXY), etc. | May present with genital abnormalities |
| Feature | Description |
|---|---|
| ⚠️ External ambiguity | Enlarged clitoris / micropenis, hypospadias, fused labia, undescended testis |
| 🧬 Genitalia mismatch | External genitalia don’t match internal organs or karyotype |
| ❌ Absent gonads | Not palpable in scrotum/labia |
| 🚻 Urinary abnormalities | Single perineal opening, abnormal urinary stream |
| 🩸 Salt-wasting crisis | Seen in CAH: dehydration, vomiting, low sodium, shock |
| Test | Purpose |
|---|---|
| 🧬 Karyotyping (Chromosome analysis) | Determines genetic sex |
| 💉 Hormonal profile | Serum 17-OHP, cortisol, testosterone, LH/FSH |
| 🔬 Pelvic ultrasound | Detects uterus, ovaries, testes |
| 🔭 Genitogram / MRI pelvis | Clarifies internal structures |
| 🧪 Electrolytes | Check for salt-wasting in CAH |
| 🧬 Molecular genetic testing | Confirms specific mutations (e.g., CYP21A2 for CAH) |
❌ Psychological distress (in child and family)
❌ Infertility (in some types)
❌ Recurrent urinary tract infections
❌ Social stigma and gender identity conflict
❌ Adrenal crisis (in CAH)
🟡 Ambiguous genitalia = uncertain sex due to abnormal external genitalia
🟡 Most common cause in newborn girls = CAH
🟡 First test = Karyotyping
🟡 Gender should not be assigned at birth until full evaluation
🟡 Requires multidisciplinary team and parental counseling
Q1. Ambiguous genitalia refers to:
🅰️ Unilateral undescended testis
✅ 🅱️ Genitalia not clearly male or female
🅲️ Premature puberty
🅳️ Hernia
Correct Answer: 🅱️ Genitalia not clearly male or female
Q2. Most common cause of ambiguous genitalia in newborn girls is:
🅰️ Turner syndrome
✅ 🅱️ Congenital adrenal hyperplasia (CAH)
🅲️ Androgen insensitivity
🅳️ Testicular torsion
Correct Answer: 🅱️ Congenital adrenal hyperplasia (CAH)
Q3. In CAH, salt-wasting crisis presents with:
🅰️ Hypertension
✅ 🅱️ Vomiting, low sodium, shock
🅲️ Bradycardia
🅳️ Hypothermia
Correct Answer: 🅱️ Vomiting, low sodium, shock
Q4. Primary test to determine genetic sex in ambiguous genitalia is:
🅰️ USG
🅱️ MRI
✅ 🅲️ Karyotyping
🅳️ X-ray
Correct Answer: 🅲️ Karyotyping
Q5. Which of the following is essential in managing ambiguous genitalia?
🅰️ Assign sex immediately
✅ 🅱️ Multidisciplinary evaluation before assigning gender
🅲️ Delay treatment until puberty
🅳️ Start hormone therapy without tests
Correct Answer: 🅱️ Multidisciplinary evaluation before assigning gender
Q6. Genetic defect in CAH is commonly in:
🅰️ SRY gene
🅱️ FSH receptor
✅ 🅲️ CYP21A2 gene
🅳️ LH beta chain
Correct Answer: 🅲️ CYP21A2 gene
Q7. Important nursing action in suspected ambiguous genitalia:
🅰️ Label as male
🅱️ Refer for hernia surgery
✅ 🅲️ Delay sex labeling, maintain support and privacy
🅳️ Start feeding immediately
Correct Answer: 🅲️ Delay sex labeling, maintain support and privacy
📘 Essential for Pediatric Nursing, Cardiology, Child Health Nursing, Medical-Surgical Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Tetralogy of Fallot (TOF) is a cyanotic congenital heart defect consisting of four anatomical abnormalities of the heart, resulting in reduced oxygenation of blood and cyanosis.
✳️ It is the most common cyanotic congenital heart disease in children.
| Defect | Description |
|---|---|
| 1️⃣ Ventricular Septal Defect (VSD) | Hole between the right and left ventricles |
| 2️⃣ Pulmonary Stenosis | Narrowing of pulmonary valve or artery → ↓ blood to lungs |
| 3️⃣ Overriding Aorta | Aorta shifted over VSD → receives blood from both ventricles |
| 4️⃣ Right Ventricular Hypertrophy (RVH) | Thickening of right ventricle due to overwork |
| System | Signs/Symptoms |
|---|---|
| 💙 Cyanosis | Bluish lips, nails (especially during crying or feeding) |
| 🌀 Hypoxia | “Tet spells” – sudden cyanosis, dyspnea, irritability |
| 🚼 Growth | Poor weight gain, fatigue during feeds |
| 💨 Breathing | Dyspnea, fast breathing, squatting posture (relieves dyspnea) |
| 🫀 Murmur | Systolic ejection murmur due to pulmonary stenosis |
| 🧍 Others | Clubbing of fingers, delayed milestones |
| Test | Findings |
|---|---|
| 🩺 Clinical exam | Cyanosis, murmur, squatting |
| 💻 Chest X-ray | “Boot-shaped heart” (due to RVH) |
| 📈 ECG | Right ventricular hypertrophy |
| 🩻 Echocardiography (2D Echo) | Confirms the 4 defects |
| 🧬 Oxygen saturation | Low SpO₂ (cyanotic even with oxygen) |
| 💉 Pulse oximetry / ABG | Hypoxia, polycythemia |
| Type | Description |
|---|---|
| 🟩 Palliative (temporary) | Blalock-Taussig shunt – connects subclavian artery to pulmonary artery |
| 🟦 Corrective (definitive) | Intracardiac repair – closes VSD and relieves pulmonary stenosis (done at 6–12 months) |
❌ Cerebral abscess
❌ Brain stroke (due to polycythemia)
❌ Heart failure
❌ Delayed physical/mental development
❌ Sudden death (if untreated tet spell is prolonged)
🟡 TOF = 4 defects: VSD, pulmonary stenosis, overriding aorta, RVH
🟡 Most common cyanotic CHD
🟡 Tet spells = cyanosis + breathlessness + squatting
🟡 X-ray shows boot-shaped heart
🟡 Definitive surgery = intracardiac repair
Q1. TOF includes all except:
🅰️ VSD
🅱️ Pulmonary stenosis
✅ 🅲️ Atrial septal defect
🅳️ Overriding aorta
Correct Answer: 🅲️ Atrial septal defect
Q2. The first aid for a Tet spell is:
🅰️ Lay flat and give water
🅱️ Elevate head
✅ 🅲️ Knee-chest position and oxygen
🅳️ Give diuretics
Correct Answer: 🅲️ Knee-chest position and oxygen
Q3. Classic X-ray finding in TOF:
🅰️ Snowman sign
✅ 🅱️ Boot-shaped heart
🅲️ Egg-on-string
🅳️ Box-shaped heart
Correct Answer: 🅱️ Boot-shaped heart
Q4. Which shunt is used in palliative surgery for TOF?
🅰️ Glenn shunt
✅ 🅱️ Blalock-Taussig shunt
🅲️ Fontan shunt
🅳️ Waterston shunt
Correct Answer: 🅱️ Blalock-Taussig shunt
Q5. A child squats after running. Likely diagnosis?
🅰️ ASD
🅱️ VSD
✅ 🅲️ Tetralogy of Fallot
🅳️ PDA
Correct Answer: 🅲️ Tetralogy of Fallot
Q6. What is the pathophysiological effect of overriding aorta?
🅰️ Low oxygenation
✅ 🅱️ Mixes oxygenated and deoxygenated blood
🅲️ Hypertension
🅳️ Bradycardia
Correct Answer: 🅱️ Mixes oxygenated and deoxygenated blood
Q7. Most definitive management of TOF is:
🅰️ Iron therapy
🅱️ Diuretics
✅ 🅲️ Intracardiac surgical repair
🅳️ Heart transplant
Correct Answer: 🅲️ Intracardiac surgical repair
📘 Essential for Pediatric Nursing, Cardiology, Neonatology, Medical-Surgical Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Transposition of the Great Vessels (TGV) is a cyanotic congenital heart defect in which the positions of the aorta and pulmonary artery are switched:
✳️ This results in two separate, non-communicating circulations, making survival impossible without a mixing lesion (e.g., ASD, VSD, PDA).
| Type | Description |
|---|---|
| 🟩 D-Transposition (dextro-TGV) | Aorta from RV, pulmonary artery from LV → most common & severe |
| 🟨 L-Transposition (levo-TGV or “corrected”) | A physiologically corrected form (less severe) |
| System | Signs & Symptoms |
|---|---|
| 💙 Cyanosis | Within hours of birth; not relieved by oxygen |
| 💨 Breathing | Rapid breathing (tachypnea), no significant murmur |
| 🚼 Feeding | Poor feeding, fatigue, failure to thrive |
| 💢 Clubbing | Seen in chronic cases |
| 🧬 Dependence on PDA/VSD/ASD | Necessary for blood mixing and survival |
| 🔄 Unresponsive to O₂ | Classic sign – “blue baby” not improving with oxygen |
| Test | Findings |
|---|---|
| 🧪 Pulse oximetry | Persistent cyanosis with low SpO₂ |
| 🩺 Clinical signs | No improvement with 100% O₂ |
| 🖥️ Echocardiogram (2D Echo) | Confirms abnormal origin of great vessels |
| 🧪 Hyperoxia test | Minimal rise in PaO₂ despite 100% oxygen |
| 💻 Chest X-ray | “Egg on a string” appearance (narrow mediastinum) |
| 📊 ECG | May show RV hypertrophy |
| Procedure | Description |
|---|---|
| 🔄 Arterial switch operation (ASO) | Definitive surgery; arteries are reconnected to correct ventricles (usually done within first 2 weeks of life) |
| 🟨 Atrial switch (Senning/Mustard) | Older method; now rarely used |
| ⚙️ Additional surgeries | If associated with VSD/ASD |
❌ Severe hypoxia and cyanosis
❌ Heart failure
❌ Metabolic acidosis
❌ Arrhythmias (post-surgery)
❌ Neurodevelopmental delay (if prolonged hypoxia)
❌ Death (if untreated)
🟡 TGV = Aorta from RV + Pulmonary artery from LV
🟡 Most common cyanotic heart defect in newborns
🟡 Egg-on-string appearance on X-ray
🟡 PGE1 keeps PDA open, Rashkind septostomy creates ASD
🟡 Definitive surgery = Arterial Switch Operation
Q1. In TGV, which arteries are transposed?
🅰️ Aorta and coronary arteries
✅ 🅱️ Aorta and pulmonary artery
🅲️ Carotid and aorta
🅳️ Pulmonary and subclavian
Correct Answer: 🅱️ Aorta and pulmonary artery
Q2. Classic chest X-ray sign in TGV is:
🅰️ Boot-shaped heart
✅ 🅱️ Egg-on-string
🅲️ Snowman sign
🅳️ Box-shaped heart
Correct Answer: 🅱️ Egg-on-string
Q3. First-line emergency drug in TGV is:
🅰️ Digoxin
🅱️ Lasix
✅ 🅲️ Prostaglandin E1 (PGE1)
🅳️ Dopamine
Correct Answer: 🅲️ Prostaglandin E1 (PGE1)
Q4. Why is Prostaglandin E1 used in TGV?
🅰️ Increase blood pressure
✅ 🅱️ Keep ductus arteriosus open
🅲️ Reduce heart rate
🅳️ Correct arrhythmia
Correct Answer: 🅱️ Keep ductus arteriosus open
Q5. Surgical treatment of choice in TGV is:
🅰️ Valve replacement
🅱️ Glenn shunt
✅ 🅲️ Arterial switch operation
🅳️ Fontan procedure
Correct Answer: 🅲️ Arterial switch operation
Q6. A newborn with cyanosis not improving with 100% O₂ likely has:
🅰️ PDA
🅱️ ASD
✅ 🅲️ TGV
🅳️ VSD
Correct Answer: 🅲️ TGV
Q7. The emergency procedure to create an ASD is called:
🅰️ Mustard operation
🅱️ Fontan shunt
✅ 🅲️ Rashkind septostomy
🅳️ Ross procedure
Correct Answer: 🅲️ Rashkind septostomy
📘 Essential for Pediatric Nursing, Neonatology, Cardiology, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
TAPVC (Total Anomalous Pulmonary Venous Connection) is a cyanotic congenital heart defect where the pulmonary veins do not connect to the left atrium as they should, but instead drain abnormally into the right atrium or systemic venous circulation.
✳️ It leads to mixing of oxygenated and deoxygenated blood → cyanosis + heart failure.
| Type | Connection Site | % of Cases |
|---|---|---|
| 🟩 Supracardiac | To SVC via vertical vein | ~50% |
| 🟥 Cardiac | To coronary sinus or right atrium | ~25% |
| 🟨 Infracardiac | To portal vein or hepatic vein | ~20% |
| 🟦 Mixed | Multiple sites | ~5–10% |
🔁 Pulmonary veins carry oxygenated blood, but in TAPVC they connect to the right atrium or systemic vein →
⬇️
📉 Mixing of oxygenated & deoxygenated blood in right atrium →
⬇️
💙 Systemic hypoxia/cyanosis + volume overload →
⬇️
🫀 Right-sided heart failure
🟡 Survival is only possible if there’s a mixing lesion, e.g., ASD, PDA.
| Feature | Description |
|---|---|
| 💙 Cyanosis | Severe, appears shortly after birth |
| 🫁 Respiratory distress | Tachypnea, nasal flaring, chest retractions |
| 🍼 Feeding problems | Poor feeding, failure to thrive |
| 💧 Signs of CHF | Hepatomegaly, tachycardia, edema |
| 🚫 No murmur | Often no loud murmur |
| 🛑 Obstructed TAPVC | Worse symptoms, rapidly fatal without surgery |
| Test | Findings |
|---|---|
| 🧪 Pulse oximetry | Low SpO₂ not improving with oxygen |
| 💻 Chest X-ray | “Snowman sign” in supracardiac type (enlarged SVC) |
| 📊 ECG | Right atrial and ventricular hypertrophy |
| 🩺 2D Echocardiogram | Diagnostic tool; shows abnormal venous connection |
| 🧪 Cardiac catheterization / MRI | For anatomical confirmation (pre-surgery) |
| Surgery | Description |
|---|---|
| 🛠️ Anastomosis of pulmonary veins to LA | Surgical redirection of veins to left atrium |
| 🏥 ASD closure | If present, closed surgically |
| 📅 Timing | Within first few weeks/months; emergency if obstructed TAPVC |
❌ Heart failure
❌ Pulmonary hypertension
❌ Cyanotic spells
❌ Metabolic acidosis
❌ Sudden death if obstructed TAPVC untreated
❌ Post-op complications: stenosis of pulmonary veins
🟡 TAPVC = pulmonary veins drain into right atrium/systemic veins
🟡 Cyanosis appears soon after birth
🟡 Survival needs PDA or ASD for mixing
🟡 Supracardiac type shows “snowman sign” on X-ray
🟡 Surgical correction is the only cure
Q1. In TAPVC, pulmonary veins drain into:
🅰️ Left atrium
🅱️ Lungs
✅ 🅲️ Right atrium/systemic veins
🅳️ Coronary arteries
Correct Answer: 🅲️ Right atrium/systemic veins
Q2. Most common type of TAPVC is:
🅰️ Infracardiac
🅱️ Cardiac
✅ 🅲️ Supracardiac
🅳️ Mixed
Correct Answer: 🅲️ Supracardiac
Q3. Chest X-ray in supracardiac TAPVC shows:
🅰️ Boot-shaped heart
🅱️ Egg-on-string
✅ 🅲️ Snowman appearance
🅳️ Box-shaped heart
Correct Answer: 🅲️ Snowman appearance
Q4. Life in TAPVC depends on presence of:
🅰️ Aortic stenosis
✅ 🅱️ ASD or PDA (mixing lesion)
🅲️ Coarctation of aorta
🅳️ Tricuspid regurgitation
Correct Answer: 🅱️ ASD or PDA (mixing lesion)
Q5. Definitive treatment for TAPVC is:
🅰️ Balloon septostomy
🅱️ Prostaglandin E1
✅ 🅲️ Surgical redirection of pulmonary veins
🅳️ Pacemaker insertion
Correct Answer: 🅲️ Surgical redirection of pulmonary veins
Q6. A neonate with cyanosis not improving with O₂ may have:
🅰️ ASD
✅ 🅱️ TAPVC
🅲️ PDA
🅳️ Tricuspid regurgitation
Correct Answer: 🅱️ TAPVC
Q7. A child with TAPVC develops worsening cyanosis, likely due to:
🅰️ Increased blood pressure
🅱️ Low blood sugar
✅ 🅲️ Obstruction of pulmonary venous return
🅳️ Bradycardia
Correct Answer: 🅲️ Obstruction of pulmonary venous return
📘 Essential for Pediatric Nursing, Cardiology, Child Health Nursing, Medical-Surgical Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Atrial Septal Defect (ASD) is a congenital heart defect in which there is an abnormal opening in the interatrial septum, allowing oxygen-rich blood from the left atrium to mix with oxygen-poor blood in the right atrium.
✳️ ASD is a left-to-right shunt, often acyanotic, but can lead to complications if untreated.
| Type | Location | Frequency |
|---|---|---|
| 🟩 Ostium secundum | Middle part of septum | Most common (75%) |
| 🟥 Ostium primum | Lower part, near AV valves | Associated with AV septal defects |
| 🟨 Sinus venosus | Near entry of SVC or IVC | Rare |
| 🟦 Coronary sinus type | Near coronary sinus | Very rare |
❗ Many children are asymptomatic and diagnosed on routine checkups
| Feature | Description |
|---|---|
| 💨 Fatigue | Easy tiring during play or exercise |
| 🍼 Feeding issues | Poor feeding in infants |
| 🫁 Respiratory | Frequent chest infections |
| 💗 Heart sounds | Wide fixed splitting of second heart sound (S2) |
| 🫀 Murmur | Systolic ejection murmur (pulmonary area) |
| 🫧 Cyanosis | Usually absent unless pulmonary hypertension develops |
| Test | Findings |
|---|---|
| 🩺 Clinical exam | Murmur, wide fixed split S2 |
| 💻 Echocardiography (2D Echo) | Confirms ASD and shunt direction |
| 🩻 Chest X-ray | Cardiomegaly, increased pulmonary vascular markings |
| 📉 ECG | Right atrial enlargement, right axis deviation |
| 💉 Cardiac catheterization | Measures shunt ratio and pressures (if needed) |
| Method | Indication |
|---|---|
| 🛠️ Transcatheter device closure | Preferred for ostium secundum ASD (after 2–4 years) |
| 🔨 Surgical patch closure | For large or non-device-suitable ASDs |
| 🩹 Timing | Usually closed between 2–5 years of age to prevent complications |
❌ Right-sided heart failure
❌ Pulmonary hypertension
❌ Eisenmenger syndrome (reversal to right-to-left shunt)
❌ Arrhythmias (e.g., atrial fibrillation)
❌ Stroke (due to paradoxical embolism)
🟡 ASD = opening in interatrial septum
🟡 Commonest type = ostium secundum
🟡 Wide fixed split of S2 is hallmark
🟡 Most ASDs are left-to-right shunt
🟡 Surgery/device closure done by 2–5 years
Q1. Atrial septal defect causes which type of shunt?
🅰️ Right to left
✅ 🅱️ Left to right
🅲️ Bidirectional
🅳️ Obstructive
Correct Answer: 🅱️ Left to right
Q2. Most common type of ASD is:
🅰️ Sinus venosus
🅱️ Ostium primum
✅ 🅲️ Ostium secundum
🅳️ Coronary sinus
Correct Answer: 🅲️ Ostium secundum
Q3. Hallmark auscultation finding in ASD is:
🅰️ Continuous murmur
🅱️ Loud S1
✅ 🅲️ Wide fixed split of S2
🅳️ Diastolic murmur
Correct Answer: 🅲️ Wide fixed split of S2
Q4. A child with small ASD may:
🅰️ Require immediate surgery
🅱️ Be cyanotic at birth
✅ 🅲️ Close spontaneously over time
🅳️ Need pacemaker
Correct Answer: 🅲️ Close spontaneously over time
Q5. A long-term complication of untreated ASD is:
🅰️ Jaundice
🅱️ Scoliosis
✅ 🅲️ Pulmonary hypertension
🅳️ Diabetes
Correct Answer: 🅲️ Pulmonary hypertension
Q6. Surgical closure of ASD is typically done at:
🅰️ <1 year
✅ 🅱️ 2–5 years
🅲️ Teenage
🅳️ Adulthood
Correct Answer: 🅱️ 2–5 years
Q7. Device closure is best for which ASD type?
🅰️ Ostium primum
✅ 🅱️ Ostium secundum
🅲️ Sinus venosus
🅳️ Coronary sinus
Correct Answer: 🅱️ Ostium secundum
📘 Essential for Pediatric Nursing, Cardiology, Child Health Nursing, Medical-Surgical Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Ventricular Septal Defect (VSD) is a congenital heart defect characterized by an abnormal opening in the interventricular septum, allowing left-to-right shunting of blood between the left and right ventricles.
✳️ It is the most common congenital heart defect.
| Type | Location | Common Features |
|---|---|---|
| 🟩 Perimembranous | Near tricuspid and aortic valves | Most common (~80%) |
| 🟥 Muscular (Trabecular) | In muscular lower septum | May close spontaneously |
| 🟨 Inlet | Near AV valves | Associated with Down syndrome |
| 🟦 Outlet (Supracristal) | Near pulmonary valve | More common in Asians |
🔁 Blood flows from left ventricle (high pressure) to right ventricle (low pressure) →
⬇️
🫁 Increased pulmonary blood flow →
⬇️
🫀 Pulmonary hypertension and right ventricular overload →
⬇️
❌ May lead to Eisenmenger syndrome if untreated
| Feature | Description |
|---|---|
| 💨 Dyspnea | Rapid breathing, shortness of breath, especially during feeding |
| 💓 Murmur | Harsh pansystolic murmur (left lower sternal border) |
| 🍼 Feeding issues | Fatigue during feeds, poor weight gain |
| 💙 Cyanosis | Absent (unless Eisenmenger develops) |
| 🧒 Growth | Failure to thrive, delayed milestones |
| 🔁 Recurrent infections | Especially respiratory tract infections |
| Test | Findings |
|---|---|
| 🩺 Auscultation | Loud pansystolic murmur (holosystolic) |
| 💻 Echocardiography (2D Echo) | Confirms VSD size, location, shunt direction |
| 📉 ECG | Right/left ventricular hypertrophy (based on defect size) |
| 🩻 Chest X-ray | Cardiomegaly, increased pulmonary vascular markings |
| 💉 Cardiac catheterization | For large defects or surgical planning |
| Indication | Approach |
|---|---|
| Large VSD with CHF | Surgical closure (open heart surgery) |
| Moderate VSD with failure to thrive | Surgery at 6–12 months |
| Device closure | For muscular or suitable perimembranous VSDs |
❌ Congestive heart failure (CHF)
❌ Recurrent pneumonia or bronchitis
❌ Growth retardation
❌ Eisenmenger syndrome (irreversible pulmonary hypertension)
❌ Aortic valve prolapse
❌ Infective endocarditis
🟡 VSD = most common congenital heart defect
🟡 Blood shunt = left to right
🟡 Murmur = pansystolic at left lower sternal border
🟡 Small VSD may close spontaneously
🟡 Large VSD → CHF, needs surgical closure
Q1. Ventricular septal defect is an opening between:
🅰️ Atria
✅ 🅱️ Ventricles
🅲️ Aorta and pulmonary artery
🅳️ Left ventricle and aorta
Correct Answer: 🅱️ Ventricles
Q2. VSD causes which type of shunt?
🅰️ Right to left
✅ 🅱️ Left to right
🅲️ Bidirectional
🅳️ Reversed only during sleep
Correct Answer: 🅱️ Left to right
Q3. Most common type of VSD is:
🅰️ Inlet
✅ 🅱️ Perimembranous
🅲️ Muscular
🅳️ Supracristal
Correct Answer: 🅱️ Perimembranous
Q4. Classical murmur in VSD is heard during:
🅰️ Diastole
🅱️ Early systole
✅ 🅲️ Holosystole (pansystolic)
🅳️ Late systole
Correct Answer: 🅲️ Holosystolic (pansystolic)
Q5. Which complication can develop if VSD remains uncorrected?
🅰️ Pulmonary atresia
✅ 🅱️ Eisenmenger syndrome
🅲️ Tricuspid atresia
🅳️ Coarctation of aorta
Correct Answer: 🅱️ Eisenmenger syndrome
Q6. Which condition contraindicates VSD closure?
🅰️ CHF
✅ 🅱️ Eisenmenger syndrome
🅲️ Growth retardation
🅳️ Murmur
Correct Answer: 🅱️ Eisenmenger syndrome
Q7. Device closure is most suitable for:
🅰️ Large inlet VSD
🅱️ Supracristal VSD
✅ 🅲️ Muscular VSD
🅳️ Coronary sinus defect
Correct Answer: 🅲️ Muscular VSD
📘 Essential for Pediatric Nursing, Cardiology, Medical-Surgical Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Coarctation of the Aorta (CoA) is a congenital narrowing (stenosis) of a segment of the aorta, most commonly just distal to the origin of the left subclavian artery near the ductus arteriosus.
✳️ This results in increased blood pressure in the upper body and decreased blood flow to the lower body.
| Type | Description |
|---|---|
| 🟩 Preductal (infantile) | Narrowing occurs before ductus arteriosus → depends on PDA for lower body perfusion |
| 🟥 Postductal (adult) | Narrowing occurs after ductus → collateral circulation may develop |
⬆️ Increased pressure proximal to narrowing → hypertension in upper limbs
⬇️ Decreased pressure distal to narrowing → hypoperfusion of lower limbs
➡️ LV hypertrophy develops due to increased afterload
➡️ Severe cases may lead to congestive heart failure in infancy
| Feature | Infants (severe form) | Older children/adults (milder) |
|---|---|---|
| 🍼 Poor feeding, FTT | Fatigue, irritability | Leg cramps during exercise |
| 💙 Cyanosis (lower body) | Cold feet | Headache, epistaxis |
| 🩺 BP discrepancy | ⬆️ BP in arms, ⬇️ in legs | Absent/delayed femoral pulses |
| 🫀 Murmur | Systolic murmur over back | Inter-scapular area |
| Test | Findings |
|---|---|
| 🩺 BP measurement | Higher in upper limbs than lower limbs |
| 💻 Echocardiography (2D Echo) | Confirms site & severity of narrowing |
| 📉 ECG | Left ventricular hypertrophy |
| 🩻 Chest X-ray | Rib notching, “3 sign” (indentation at coarctation site) |
| 🧬 MRI/CT angiography | Detailed view for surgical planning |
| Procedure | Description |
|---|---|
| 🛠️ Resection with end-to-end anastomosis | Excision of narrowed segment |
| 🩹 Subclavian flap repair | Aortic wall reconstruction |
| 🧰 Balloon angioplasty/stenting | Used in older children/adolescents |
🕒 Surgery is typically done between 2 weeks and 6 months of age in symptomatic neonates
❌ Severe hypertension
❌ Aortic rupture or aneurysm
❌ Heart failure
❌ Stroke (due to hypertension)
❌ Endocarditis
❌ Re-coarctation (narrowing again)
🟡 CoA = aortic narrowing, most often just after left subclavian artery
🟡 Classic sign = higher BP in arms vs. legs
🟡 X-ray shows rib notching + “3 sign”
🟡 Infants need PGE1 to maintain PDA
🟡 Definitive treatment = surgical repair or balloon angioplasty
Q1. Coarctation of the aorta leads to:
🅰️ Low BP in both limbs
✅ 🅱️ High BP in arms, low in legs
🅲️ High BP in legs
🅳️ Cyanosis in face
Correct Answer: 🅱️ High BP in arms, low in legs
Q2. Drug used to maintain PDA in neonates with CoA is:
🅰️ Digoxin
🅱️ Furosemide
✅ 🅲️ Prostaglandin E1 (PGE1)
🅳️ Dopamine
Correct Answer: 🅲️ Prostaglandin E1 (PGE1)
Q3. Classic chest X-ray sign in CoA:
🅰️ Egg-on-string
✅ 🅱️ Rib notching + 3 sign
🅲️ Boot-shaped heart
🅳️ Box-shaped heart
Correct Answer: 🅱️ Rib notching + 3 sign
Q4. Which syndrome is commonly associated with CoA?
🅰️ Down syndrome
🅱️ Marfan syndrome
✅ 🅲️ Turner syndrome
🅳️ Klinefelter syndrome
Correct Answer: 🅲️ Turner syndrome
Q5. Common murmur heard in CoA:
🅰️ Diastolic murmur
🅱️ Continuous murmur
✅ 🅲️ Systolic murmur over back
🅳️ Murmur at apex
Correct Answer: 🅲️ Systolic murmur over back
Q6. A serious untreated CoA can result in:
🅰️ Diabetes
✅ 🅱️ Stroke and heart failure
🅲️ Renal stones
🅳️ Retinopathy
Correct Answer: 🅱️ Stroke and heart failure
Q7. Definitive treatment of CoA is:
🅰️ Long-term diuretics
🅱️ Pacemaker
✅ 🅲️ Surgical resection/balloon angioplasty
🅳️ Iron therapy
Correct Answer: 🅲️ Surgical resection/balloon angioplasty
📘 Essential for Pediatric Nursing, Cardiology, Medical-Surgical Nursing, Child Health Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Patent Ductus Arteriosus (PDA) is a congenital heart condition where the ductus arteriosus (a fetal blood vessel connecting the aorta and pulmonary artery) fails to close after birth, resulting in left-to-right shunting of blood.
✳️ The ductus arteriosus normally closes within 24–72 hours after birth. Persistent patency leads to volume overload of the lungs and left heart.
| Stage | Role of Ductus Arteriosus |
|---|---|
| 🍼 In fetus | Diverts blood away from lungs to aorta |
| 👶 After birth | Closes due to ↑ oxygen and ↓ prostaglandins |
| ❗ In PDA | Stays open → blood flows from aorta to pulmonary artery (left-to-right shunt) |
⬆️ Aortic pressure → pushes blood through PDA → pulmonary artery → lungs → left atrium
⬇️ Results in volume overload, pulmonary congestion, left-sided heart strain
✳️ Large PDA may lead to congestive heart failure and pulmonary hypertension
| Feature | Description |
|---|---|
| 💓 Murmur | Continuous “machinery” murmur (best heard at left upper sternal border) |
| 💨 Tachypnea | Due to pulmonary congestion |
| 💗 Bounding pulse | Wide pulse pressure |
| 🍼 Feeding difficulty | Poor feeding, sweating |
| 🧒 Growth issues | Failure to thrive |
| 💙 Cyanosis | In Eisenmenger syndrome (rare, late-stage reversal) |
| Test | Finding |
|---|---|
| 🩺 Auscultation | Continuous machinery murmur |
| 💻 Echocardiography (2D Echo + Doppler) | Confirms PDA size, shunt direction |
| 🩻 Chest X-ray | Cardiomegaly, ↑ pulmonary vascular markings |
| 📉 ECG | Left atrial/ventricular hypertrophy |
| 💉 Oxygen saturation | Normal or mildly reduced unless Eisenmenger develops |
| Drug | Action |
|---|---|
| 💊 Indomethacin / Ibuprofen | Closes PDA by inhibiting prostaglandins |
| 💉 Paracetamol | Alternative to NSAIDs in some centers |
| ❗Contraindicated in | Bleeding, renal impairment, NEC |
| Indication | Method |
|---|---|
| Moderate to large PDA | Transcatheter coil/device closure (from ~6 months onwards) |
| Not suitable for device | Surgical ligation via thoracotomy |
🛑 In cyanotic heart defects (e.g., TGV) – PDA is life-saving and kept open using Prostaglandin E1 (PGE1).
❌ Congestive heart failure
❌ Pulmonary hypertension
❌ Failure to thrive
❌ Infective endocarditis
❌ Eisenmenger syndrome (late-stage reversal of shunt)
🟡 PDA = persistent fetal connection between aorta and pulmonary artery
🟡 Common in preterm infants and rubella syndrome
🟡 Classic murmur = continuous machinery murmur
🟡 Closure with indomethacin/ibuprofen in preemies
🟡 Definitive closure = device or surgical ligation
Q1. PDA is a communication between:
🅰️ Atria
✅ 🅱️ Aorta and pulmonary artery
🅲️ Ventricles
🅳️ Right atrium and pulmonary vein
Correct Answer: 🅱️ Aorta and pulmonary artery
Q2. Classic murmur of PDA is:
🅰️ Mid-diastolic
🅱️ Holosystolic
✅ 🅲️ Continuous “machinery” murmur
🅳️ Systolic ejection
Correct Answer: 🅲️ Continuous “machinery” murmur
Q3. Drug used to close PDA in preterm neonates:
🅰️ PGE1
🅱️ Digoxin
✅ 🅲️ Indomethacin
🅳️ Dopamine
Correct Answer: 🅲️ Indomethacin
Q4. In PDA, blood flows from:
🅰️ Pulmonary artery to aorta
✅ 🅱️ Aorta to pulmonary artery
🅲️ Right atrium to left atrium
🅳️ Left ventricle to right ventricle
Correct Answer: 🅱️ Aorta to pulmonary artery
Q5. Which condition uses PDA to maintain life pre-surgery?
🅰️ ASD
🅱️ VSD
✅ 🅲️ Transposition of Great Vessels (TGV)
🅳️ Coarctation postductal
Correct Answer: 🅲️ Transposition of Great Vessels (TGV)
Q6. Surgical PDA closure is done through:
🅰️ Craniotomy
✅ 🅱️ Thoracotomy
🅲️ Laparotomy
🅳️ Endoscopy
Correct Answer: 🅱️ Thoracotomy
Q7. Most common complication of large untreated PDA:
🅰️ Diabetes
🅱️ Epilepsy
✅ 🅲️ Congestive heart failure
🅳️ Nephrotic syndrome
Correct Answer: 🅲️ Congestive heart failure
📘 Essential for Pediatric Nursing, Orthopedic Nursing, Child Health Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Congenital Talipes Equinovarus (CTEV), commonly called Clubfoot, is a congenital deformity in which one or both feet are twisted inward and downward at birth, resembling the shape of a club.
✳️ “Talipes” = foot deformity
✳️ “Equino” = plantarflexed (toe down)
✳️ “Varus” = inward turning of heel
| Deformity | Description |
|---|---|
| 🟩 Cavus | High medial arch of the foot |
| 🟥 Adduction | Forefoot turned toward midline |
| 🟨 Varus | Heel turned inward |
| 🟦 Equinus | Ankle pointing downward (plantar flexion) |
| Type | Description |
|---|---|
| 🟢 Idiopathic (true CTEV) | Most common; otherwise healthy child |
| 🟡 Syndromic/Secondary | Associated with disorders like spina bifida, arthrogryposis |
| 🔵 Positional (postural) | Due to intrauterine position, correctable manually |
| Sign | Description |
|---|---|
| 👣 Foot appearance | Small, twisted, and rigid foot at birth |
| 🧭 Foot position | Inversion, adduction, plantar flexion |
| 🛑 Limited motion | Restricted dorsiflexion and eversion |
| ❌ Cannot correct passively | Fixed deformity (not flexible) |
| 🔁 Often bilateral | ~50% cases involve both feet |
🌀 Ponseti Method (Gold Standard)
❌ Recurrence (if brace not used properly)
❌ Skin breakdown or pressure sores
❌ Limb length discrepancy (rare)
❌ Residual deformity or stiffness
❌ Gait disturbances in severe untreated cases
🟡 CTEV = congenital clubfoot deformity (CAVE)
🟡 Ponseti method is the gold standard treatment
🟡 Serial casting starts ideally within first week of life
🟡 Followed by Achilles tenotomy + foot brace
🟡 Complication = recurrence if brace non-compliant
Q1. CTEV is also known as:
🅰️ Flatfoot
✅ 🅱️ Clubfoot
🅲️ Knock knees
🅳️ Bow legs
Correct Answer: 🅱️ Clubfoot
Q2. Most common type of CTEV is:
🅰️ Syndromic
✅ 🅱️ Idiopathic
🅲️ Postural
🅳️ Traumatic
Correct Answer: 🅱️ Idiopathic
Q3. Which method is the gold standard for treating CTEV?
🅰️ Kite method
🅱️ Night splint only
✅ 🅲️ Ponseti method
🅳️ Serial traction
Correct Answer: 🅲️ Ponseti method
Q4. Component ‘Equinus’ in CTEV means:
🅰️ Heel turned inward
✅ 🅱️ Foot pointed downward
🅲️ Toes spread outward
🅳️ Foot curved outward
Correct Answer: 🅱️ Foot pointed downward
Q5. Which device is used after Ponseti correction?
🅰️ AFO
🅱️ KAFO
✅ 🅲️ Dennis Brown splint
🅳️ Milwaukee brace
Correct Answer: 🅲️ Dennis Brown splint
Q6. In CTEV, which part of foot is adducted?
🅰️ Heel
✅ 🅱️ Forefoot
🅲️ Midfoot only
🅳️ Calcaneus
Correct Answer: 🅱️ Forefoot
Q7. Best time to start treatment for CTEV is:
🅰️ After 6 months
🅱️ After walking begins
✅ 🅲️ As soon as possible after birth
🅳️ At 2 years
Correct Answer: 🅲️ As soon as possible after birth
📘 Essential for Pediatric Nursing, Medical-Surgical Nursing, Mental Health Nursing, GNM/BSc Nursing, NHM, GPSC, AIIMS, Staff Nurse Exams
Down’s Syndrome is a genetic disorder caused by the presence of an extra (third) copy of chromosome 21 — also known as Trisomy 21.
✳️ It is the most common chromosomal abnormality, leading to intellectual disability, characteristic facial features, and multiple congenital anomalies.
| Type | Description | % of Cases |
|---|---|---|
| 🟩 Trisomy 21 | Full extra chromosome 21 in all cells | ~95% |
| 🟨 Translocation | Part of chromosome 21 attached to another chromosome | ~3–4% |
| 🟦 Mosaicism | Some cells have extra chromosome 21, others are normal | ~1–2% |
| System | Signs & Symptoms |
|---|---|
| 👶 Appearance | Flat facial profile, upward slanting eyes, small ears, small mouth with protruding tongue |
| ✋ Hands/Feet | Single palmar crease, short broad hands, sandal gap (gap between 1st & 2nd toes) |
| 🧠 Neurological | Intellectual disability (mild to moderate), delayed milestones |
| 🫀 Cardiac | Congenital heart disease (especially AV septal defect, VSD, PDA) |
| 🫁 Respiratory | Recurrent infections due to hypotonia and narrow airways |
| 🧬 Other | Hypotonia (floppy baby), short stature, hearing loss, hypothyroidism, early-onset Alzheimer’s disease |
✳️ No cure – Supportive, developmental, and medical management
| Need | Management |
|---|---|
| 🫀 Cardiac | Early echocardiogram → cardiac surgery if needed |
| 🧠 Developmental | Early intervention: physiotherapy, occupational therapy, special education |
| 📚 Cognitive | IQ testing, behavioral therapy |
| 🧪 Medical | Screen for hypothyroidism, hearing/vision, GI anomalies |
| 💬 Speech delays | Speech therapy |
❌ Congenital heart disease
❌ Hypothyroidism
❌ Atlanto-axial instability
❌ Leukemia (↑ risk for ALL/AML)
❌ Vision & hearing loss
❌ Early Alzheimer’s disease
🟡 Down’s syndrome = Trisomy 21
🟡 Most common chromosomal cause of intellectual disability
🟡 Classic signs = flat face, slanted eyes, single palmar crease
🟡 Confirmed by karyotyping
🟡 Risk increases with maternal age >35 years
Q1. Down’s syndrome is also known as:
🅰️ Trisomy 13
✅ 🅱️ Trisomy 21
🅲️ Monosomy X
🅳️ Turner syndrome
Correct Answer: 🅱️ Trisomy 21
Q2. Most common congenital heart defect in Down’s syndrome:
🅰️ Tetralogy of Fallot
✅ 🅱️ Atrioventricular septal defect (AVSD)
🅲️ ASD
🅳️ Coarctation of aorta
Correct Answer: 🅱️ Atrioventricular septal defect (AVSD)
Q3. Classic hand feature in Down syndrome is:
🅰️ Clubbed fingers
🅱️ Extra digits
✅ 🅲️ Single palmar crease
🅳️ Radial deviation
Correct Answer: 🅲️ Single palmar crease
Q4. Which of the following increases the risk of Down syndrome?
🅰️ Young maternal age
🅱️ Father’s age >35
✅ 🅲️ Mother’s age >35
🅳️ C-section delivery
Correct Answer: 🅲️ Mother’s age >35
Q5. Confirmatory test for Down syndrome is:
🅰️ Triple marker test
🅱️ Ultrasound
✅ 🅲️ Karyotyping
🅳️ X-ray
Correct Answer: 🅲️ Karyotyping
Q6. Down’s syndrome child is more prone to:
🅰️ Diabetes
🅱️ Hypertension
✅ 🅲️ Leukemia
🅳️ Asthma
Correct Answer: 🅲️ Leukemia
Q7. What is the inheritance pattern of typical Down syndrome?
🅰️ X-linked
🅱️ Autosomal recessive
✅ 🅲️ Chromosomal nondisjunction
🅳️ Mitochondrial
Correct Answer: 🅲️ Chromosomal nondisjunction