Neonatal stroke and intracranial haemorrhage — formative SAQs

SAQ 1 — The term infant with focal seizures (20 marks, ~15 minutes)

A term infant (39 weeks, birthweight 3400 g) is born by emergency caesarean for failure to progress after an instrumental attempt. Apgar scores are 6, 8, and 9. At 30 hours of age the midwife notes rhythmic jerking of the right arm and right side of the face lasting two minutes. Between events the infant is alert but has a reduced grasp and asymmetry of tone favouring the left side. Blood glucose is 3.2 mmol/L. Cranial ultrasound on day 2 is reported as normal. [1]

Questions

1. What is the most likely diagnosis, and why does the normal cranial ultrasound not exclude it? (4 marks) [1]
2. Outline your immediate management priorities for this infant. (5 marks) [1]
3. Describe the definitive neuroimaging investigation and what it would show. (4 marks) [1] [3]
4. Discuss the prognosis and the follow-up pathway, citing the evidence for outcome prediction. (7 marks) [3] [8]

Model answer (must-hit)

1. The most likely diagnosis is perinatal arterial ischaemic stroke (PAIS), presenting with focal clonic seizures and asymmetric tone at 30 hours. A normal cranial ultrasound does not exclude PAIS because ultrasound is poor at visualising the cortical surface and may miss a focal cortical infarct; MRI with diffusion-weighted imaging is required. [1]
2. Immediate management: maintain airway and oxygenation; check and correct glucose (already 3.2 mmol/L — normal); secure IV access; give phenobarbital 20 mg/kg IV for the seizure; start continuous EEG if available; maintain normoxia, normoglycaemia, normotension, and normothermia; arrange MRI in parallel. [1]
3. MRI brain with diffusion-weighted imaging (DWI) is the definitive investigation. DWI would show restricted diffusion in an arterial territory — most commonly the left middle cerebral artery — confirming acute ischaemia. SWI excludes haemorrhage and MRV excludes cerebral sinovenous thrombosis. [1] [3]
4. Prognosis: the Baak 2023 systematic review establishes that infarct size, corticospinal tract involvement, and early neurological severity predict outcome. Approximately 30 to 60 percent of PAIS survivors develop hemiplegic cerebral palsy, with cognitive impairment and epilepsy in a subset. Follow-up: structured neurodevelopmental surveillance with early detection of CP and a rehabilitation pathway per the Australian clinical consensus guideline (Greenham 2021). [3] [8]

SAQ 2 — The preterm infant with grade III IVH and progressive PHVD (20 marks, ~15 minutes)

A 26-week infant (birthweight 780 g) has a routine cranial ultrasound on day 4 showing bilateral grade III intraventricular haemorrhage with acute ventricular dilation. The infant is haemodynamically stable. On day 14, the head circumference has crossed two centile lines upward, and a repeat ultrasound shows progressive ventricular dilation. [4]

Questions

1. Define the Papile grade and explain the mechanism of the white matter injury in severe IVH. (5 marks) [4] [5]
2. What complication has developed by day 14, and how should it be monitored? (4 marks) [6]
3. Outline the stepwise management of this complication, citing the evidence for intervention thresholds. (6 marks) [6]
4. Discuss the prognosis for this infant and the family counselling points. (5 marks) [4] [5]

Model answer (must-hit)

1. Grade III IVH is intraventricular haemorrhage with acute ventricular dilation (Papile). The white matter injury is not from the intraventricular blood itself but from a periventricular haemorrhagic infarction: the germinal matrix haemorrhage compresses the terminal veins, causing venous obstruction and haemorrhagic infarction of the periventricular white matter. Blood breakdown products are also toxic to oligodendrocyte precursors (Ballabh 2010, 2021). [4] [5]
2. The complication is post-haemorrhagic ventricular dilation (PHVD). It is monitored with serial cranial ultrasound (measuring the Levene ventricular index and anterior horn width) and serial head circumference. The upward crossing of centile lines signals progression. [6]
3. Stepwise management: (1) serial cranial ultrasound monitoring for all grade III–IV IVH. (2) The de Vries 2019 RCT established treatment thresholds based on ventricular dimensions — intervene at the threshold rather than waiting for severe dilation, to reduce shunt surgery. (3) Temporising measures: serial lumbar punctures, or a ventricular access device with CSF drainage. (4) A ventriculosubgaleal shunt if temporising fails. (5) A permanent ventriculoperitoneal shunt for persistent progressive hydrocephalus. [6]
4. Prognosis: grade III–IV IVH carries a high risk (~40–60%) of major neurodevelopmental impairment — cerebral palsy (typically spastic diplegia or quadriplegia from periventricular corticospinal tract injury), cognitive impairment, visual impairment, and epilepsy. The severity of PHVD and periventricular white matter injury independently predict outcome beyond the grade itself. Family counselling: honest discussion of the risks, the monitoring pathway, and the need for long-term neurodevelopmental surveillance and early intervention. [4] [5]