---

Neonatal Seizures - Complete Quiz Notes

---

1. DEFINITION

• Neonatal seizures = seizures in the first 28 days of life (or up to 44 weeks corrected gestational age in preterms)
• Unlike adults, neonatal seizures are almost always ACUTE SYMPTOMATIC (not epileptic)
• More common in the first 28 days than any other time in life
• Incidence: 1-5 per 1000 live births; higher in preterm infants

---

2. PATHOPHYSIOLOGY (Key Concept - Often Tested!)

Why phenobarbital works poorly: It is a GABA-agonist, but in neonates GABA is actually EXCITATORY - hence the reduced response to phenobarbital.

---

3. ETIOLOGY BY DAY OF PRESENTATION

Mnemonic for Causes: "HIDE MAST"

• H - Hypoxic-Ischemic Encephalopathy (most common, ~50%)
• I - Intracranial hemorrhage (IVH, SDH, SAH)
• D - Drug withdrawal / intoxication
• E - Electrolyte abnormalities (hypocalcemia, hypomagnesemia, hyponatremia)
• M - Metabolic (hypoglycemia, IEM, pyridoxine deficiency)
• A - Anomalies of brain (cortical dysplasia, lissencephaly)
• S - Sepsis / Meningitis (bacterial, viral - HSV)
• T - Trauma (birth injury)

---

4. CLINICAL CLASSIFICATION (Volpe's Classification)

Key fact: Generalized tonic-clonic seizures as seen in older children are NOT seen in neonates due to immature synaptic connections.

---

5. JITTERINESS vs SEIZURES (High-yield comparison!)

---

6. DIAGNOSIS

EEG (Gold Standard)

• Continuous conventional EEG is the gold standard (recommended by American Clinical Neurophysiology Society)
• Many neonatal seizures are subclinical - only 1/3 of EEG seizures have clinical signs
• Only 1/4 of clinically suspected seizures have EEG confirmation
• aEEG (amplitude-integrated EEG): Simplified bedside monitoring; less sensitive but useful when conventional EEG unavailable

Workup

1. Bedside: Blood glucose, electrolytes (Na, Ca, Mg), blood gas
2. Blood: CBC, culture, LFTs, metabolic screen (ammonia, lactate, amino acids)
3. CSF: LP for meningitis/encephalitis (HSV PCR)
4. Imaging: Cranial ultrasound (IVH), MRI brain (preferred - shows HIE, stroke, malformations)
5. EEG: Continuous monitoring

---

7. MANAGEMENT

Step 1: Correct Underlying Cause First!

• Hypoglycemia: 10% dextrose 2 mL/kg IV bolus
• Hypocalcemia: 10% calcium gluconate 2 mL/kg IV slowly
• Hypomagnesemia: MgSO4 0.2 mL/kg of 50% solution IM
• Pyridoxine deficiency: Pyridoxine 100 mg IV (diagnostic and therapeutic)

Step 2: Antiepileptic Drugs

Per OP Ghai / standard Indian pediatric practice:

• Phenobarbital remains the first-line drug in India
• Pyridoxine 100 mg IV should be given empirically if seizures are refractory (pyridoxine-dependent epilepsy)

---

8. NEONATAL EPILEPSY SYNDROMES

---

9. PROGNOSIS

• Prognosis is primarily determined by the underlying etiology
• Of survivors with neonatal seizures: 25-70% develop subsequent epilepsy
• HIE - moderate mortality; high risk of neurodevelopmental disability
• Metabolic causes (hypocalcemia, hypoglycemia corrected promptly) - good prognosis
• Structural causes / Ohtahara / EME - poor prognosis
• Long-term risks: cerebral palsy, cognitive impairment, epilepsy

---

10. QUICK REVISION BULLETS (for last-minute quiz prep)

• Most common cause = HIE (~50%)
• Most common seizure type = Subtle (especially in preterms)
• Clonic seizures - most specific for focal pathology
• EEG is the gold standard - many seizures are subclinical
• Phenobarbital works poorly because GABA is excitatory in neonates (NKCC1 > KCC2)
• "5th day fits" = benign neonatal convulsions on day 4-6
• First line drug in India = Phenobarbital 20 mg/kg IV
• Refractory seizures - always trial Pyridoxine 100 mg IV
• Jitteriness is stimulus-sensitive + suppressible - seizures are NOT
• HSV encephalitis must be considered and acyclovir started empirically in CNS infection

---

• PE 20.15 - Neonatal Seizures (already done above!)
• PE 20.20 - Common Surgical Conditions in Newborn: TEF, Oesophageal Atresia, Anal Atresia, Cleft Lip & Palate, CDH, Cause Abdomen (Prune Belly)

---

SGD 13 - PE 20.20: Surgical Conditions in Newborn

---

1. TRACHEO-ESOPHAGEAL FISTULA (TEF) & ESOPHAGEAL ATRESIA (EA)

Definition

• EA = Congenital interruption of esophageal continuity -> proximal esophageal obstruction
• TEF = Pathological communication between esophagus and trachea
• Incidence: 1 in 1500-3000 live births; mild male predilection

Embryology

• 4th gestational week: Incomplete division of esophagotracheal diverticulum of foregut
• Normally the foregut divides into trachea (anterior) and esophagus (posterior)

Gross Types (Vogt / Waterston Classification)

Memory trick: "C is the Common one = Coded 86%"

Associated Anomalies - VACTERL (in 60-70%)

• V - Vertebral
• A - Anorectal malformations
• C - Cardiac (most common associated anomaly - ~35%)
• T - Tracheal (tracheomalacia)
• E - Esophageal (the condition itself)
• R - Renal
• L - Limb (radial)

Clinical Features

• Maternal polyhydramnios (fetus can't swallow amniotic fluid - esp. isolated EA 86%)
• Excessive salivation / frothing at mouth from birth
• Choking, coughing, cyanosis on first feed
• Respiratory distress - aspiration pneumonia
• Scaphoid abdomen - in pure EA (no gas in abdomen)
• Gaseous distension of abdomen - in EA + distal TEF (air enters through fistula)
• H-type fistula: recurrent pneumonia, chronic cough - may present late

Key Diagnostic Sign

• Failure to pass nasogastric/orogastric tube (coils back at thoracic inlet)
• CXR showing the tube curled up in proximal blind pouch
• Air in abdomen on X-ray = confirms distal TEF
• No air in abdomen = pure EA (isolated)
• CONTRAINDICATED: Oral contrast study (aspiration risk!)

Management

Immediate (Preoperative):

1. Replogle tube (double-lumen sump catheter) in upper pouch for continuous suction
2. Position: Upright prone position (prevents aspiration)
3. IV antibiotics (broad-spectrum)
4. IV fluids - NPO
5. Echocardiography + renal ultrasound (VACTERL workup)
6. Avoid intubation if possible (positive pressure -> air into GI tract via TEF)

Surgical (Definitive):

• Primary repair: Right thoracotomy with extrapleural dissection, division of TEF, primary end-to-end esophageal anastomosis
• Minimally invasive: Thoracoscopic approach now preferred
• Long-gap EA: Staged repair; gastrostomy at birth, anastomosis at 4-6 months
• H-type fistula: Cervical approach for fistula ligation

---

2. CONGENITAL DIAPHRAGMATIC HERNIA (CDH)

Definition & Incidence

• Developmental diaphragmatic defect with herniation of abdominal contents into thoracic cavity
• Incidence: 1 in 2000-5000 births
• Disrupts lung and pulmonary vascular development

Types

Bochdalek = most common = Back/Posterior/Left

Pathophysiology

• Diaphragm develops from septum transversum + pleuroperitoneal folds - complete by 9 weeks
• Incomplete fusion -> defect -> bowel/organs herniate into thorax
• Pulmonary hypoplasia (both lungs affected, ipsilateral more severe)
• Pulmonary hypertension - thick arteriolar smooth muscle -> R-L shunting

Clinical Features

• Prenatal: Polyhydramnios, mediastinal shift on antenatal ultrasound, gastric bubble in chest
• At birth: Respiratory distress - grunting, dyspnea, retractions, cyanosis
• Classic triad on examination:
  1. Respiratory distress from birth
  2. Scaphoid (sunken) abdomen (bowel is in chest)
  3. Bowel sounds in the chest + Absent breath sounds on affected side
• Heart displaced to opposite side (R-side for L-CDH)
• Barrel-shaped chest

Investigations

• CXR: Bowel loops/gaseous shadows in chest + mediastinal shift (DIAGNOSTIC)
• Pulse oximetry: Pre-ductal vs post-ductal SpO2 difference (R-to-L shunting)
• Echo: Assess pulmonary hypertension and cardiac anomalies
• LHR (Lung-Head Ratio): Fetal sonographic prognostic marker

Management

Initial Stabilization (NOT emergency surgery!):

1. Intubate and ventilate - "GENTLE ventilation" (permissive hypercapnia, avoid barotrauma)
2. NGT for GI decompression
3. Nitric oxide (iNO) - for pulmonary hypertension
4. ECMO - if refractory respiratory failure
5. Avoid mask ventilation (inflates bowel in chest)
6. Agents: sildenafil, prostacyclin (PGI2), milrinone for pulmonary HTN

Surgical (once stabilized):

• Timing: Delayed repair (after stabilization) - NOT immediate emergency
• Approach: Subcostal/laparotomy; thoracoscopic repair also done
• Bochdalek: Primary repair if small; patch repair if large defect
• Survival: 65-90% in high-volume centers

Fetal intervention:

• FETO (Fetal Endoluminal Tracheal Occlusion): Balloon placed at 27-29 weeks -> lung growth by retention of lung fluid

---

3. ANAL ATRESIA / ANORECTAL MALFORMATIONS (ARM)

Definition & Incidence

• Failure of urorectal septum to divide the cloaca normally
• Incidence: 1 in 5000 live births; male predominance (58%)

Embryology

• 6th week: Urorectal septum descends to divide cloaca into:
  • Anterior: urogenital sinus
  • Posterior: anorectal canal
• Failure -> fistula between bowel and urinary tract (males) or introitus (females)
• Failure of anal membrane resorption -> anal stenosis or membrane

Classification (Wingspread / Peña)

LOW defects (below levator ani = GOOD prognosis):

• Perineal (cutaneous) fistula - most common low defect
• Anal stenosis
• Anal membrane

HIGH defects (above levator ani = WORSE prognosis):

Cloaca = single perineal opening in females (rectum + vagina + urethra fused)

Clinical Features

• Absent/abnormal anus on perineal inspection at birth
• No meconium passage in 24-48 hours
• Abdominal distension
• Meconium visible in urine (rectourinary fistula - males)
• Meconium at introitus/vestibule (females)
• Perineal fistula - meconium on perineum

Associated Anomalies - VACTERL (25-60% GU anomalies)

• Higher the defect = more associated anomalies
• Recto-bladder fistula / cloaca = 90% GU anomalies
• Low (perineal) fistula = only 10% GU anomalies
• Also: sacral anomalies (Currarino triad), spinal defects, cardiac, vertebral

Investigations

• Invertogram (Wangensteen-Rice): Lateral X-ray with baby inverted - gas bubble to skin distance (now rarely used)
• Distal colostogram (after colostomy) - gold standard to define fistula anatomy
• Renal ultrasound + VCUG
• Spinal ultrasound/MRI (sacral/spinal anomalies)
• Echocardiography

Management

LOW defects:

• Primary single-stage repair without colostomy
• Cutback anoplasty or limited PSARP (Posterior Sagittal Anorectoplasty)
• Anal stenosis: Serial dilatations

HIGH defects - THREE-STAGE approach:

1. Stage 1 (Newborn): Divided loop sigmoid colostomy with mucous fistula
2. Stage 2 (3-6 months): PSARP - Posterior Sagittal Anorectoplasty (Peña procedure)
   • Electrical stimulation to identify sphincter center
   • Midline incision from coccyx to perineum
   • Fistula division + rectal mobilization + muscle reconstruction
3. Stage 3 (Several weeks after Stage 2): Colostomy closure
   • Followed by anal dilations for months

Key: PSARP (Peña procedure) = gold standard for high ARM

---

4. CLEFT LIP AND PALATE

Embryology

• Cleft lip: Failure of maxillary prominence to fuse with merged medial nasal prominences (5th-7th week)
• Cleft palate: Failure of lateral palatine processes (palatal shelves) to fuse (7th-12th week)
• Incisive fossa = landmark dividing anterior (primary) and posterior (secondary) palate

Classification

Epidemiology

• Cleft lip ± palate: 1 in 1000 births; 60-80% are MALE
• Cleft palate alone: 1 in 2000 births; more common in females
• Most common craniofacial anomaly

Clinical Features

• Visible cleft of lip +/- palate at birth
• Feeding difficulties - inability to create suction (especially cleft palate)
• Regurgitation through nose during feeding
• Speech problems (hypernasal speech) - if palate involved
• Risk of otitis media (abnormal eustachian tube function)
• Recurrent respiratory infections
• Dental anomalies

Associated conditions

• Pierre Robin sequence: cleft palate + micrognathia + glossoptosis -> airway obstruction
• Can be part of syndromes: Treacher Collins, Van der Woude, Stickler syndrome

Management - Team Approach (Rule of 10s)

"Rule of 10" for cleft lip repair: 10 weeks old, 10 lbs weight, Hb 10 g/dL

Feeding:

• Special wide-base nipples / Haberman feeder
• Nasogastric tube if severe feeding difficulty
• Mother's milk is preferred

---

5. PRUNE BELLY SYNDROME (Eagle-Barrett Syndrome / Triad Syndrome)

Definition

1. Deficiency/absence of anterior abdominal wall muscles (wrinkled, prune-like abdomen)
2. Bilateral cryptorchidism (undescended testes - intra-abdominal)
3. Urinary tract anomalies - bilateral hydroureteronephrosis, megacystis, vesicoureteral reflux

Epidemiology

• Almost exclusively in males (>95%)
• Females: urinary + abdominal wall defects but no cryptorchidism
• Rare: 1 in 35,000-50,000 births

Pathophysiology

• Likely due to fetal bladder outlet obstruction -> massive bladder distension -> compresses abdominal wall musculature -> prevents normal development
• Ureters: long, tortuous, dilated (mostly non-obstructive; due to decreased smooth muscle + increased collagen)
• ~80% have vesicoureteral reflux (VUR)

Clinical Features

• Wrinkled, lax, prune-like abdomen (abdominal wall is thin/deficient)
• Bilateral undescended testes (intra-abdominal)
• Dilated urinary tract (hydroureteronephrosis, megacystis)
• Respiratory difficulties (abdominal wall weakness affects breathing)
• Constipation (poor abdominal musculature)
• Orthopaedic: clubfoot, hip dislocation (oligohydramnios effect)
• Cardiac anomalies in some

Management

• No universal surgery for ureters (rarely obstructive)
• Bilateral orchidopexy at 6-12 months (along with abdominal wall repair)
• Abdominoplasty (Monfort procedure) - transverse lower abdominal incision, excision of redundant skin
• VUR management: prophylactic antibiotics; ureteral reimplantation if severe
• Infertility is common despite orchidopexy (abnormal spermatogenesis, retrograde ejaculation)
• Renal failure risk: abnormal kidneys + persistent pyelonephritis

---

QUICK COMPARISON TABLE - All Conditions

---

ALSO RECALL FROM PE 20.15 - NEONATAL SEIZURES (previously covered):

• Most common cause: HIE (~50%)
• Most common type: Subtle seizures
• First-line drug: Phenobarbital 20 mg/kg IV
• Why phenobarbital works poorly: GABA is excitatory in neonates (NKCC1 > KCC2)
• Jitteriness vs Seizure: Jitteriness is stimulus-sensitive + suppressible
• EEG = Gold Standard
• Refractory seizures: Trial Pyridoxine 100 mg IV

---

---

What Your SGD Covers Today (PE 20.15 + PE 20.20):

PE 20.15 - Neonatal Seizures (covered in the previous message above)

PE 20.20 - 5 Surgical Conditions in Newborn: