Intraventricular haemorrhage and periventricular leukomalacia — formative SAQs

SAQ 1 — The preterm infant who suddenly deteriorates (20 marks, ~15 minutes)

A 26-week-gestation infant, birthweight 760 g, is intubated and ventilated for respiratory distress syndrome. Antenatal steroids were given. At 36 hours of life, after a witnessed pneumothorax that required drainage, the infant has a sudden fall in haemoglobin from 145 to 88 g/L, a metabolic acidosis, a fall in blood pressure, and three brief focal seizures. The anterior fontanelle is bulging. [1]

Questions

1. Give the most likely diagnosis and the immediate investigation that confirms it. (3 marks) [1]
2. Describe the immediate bedside management, including the haematological correction and the first-line antiseizure medication with its dose and route. (6 marks) [1]
3. Reproduce the Papile grading system and state which grade carries the highest risk of cerebral palsy and post-haemorrhagic hydrocephalus. (5 marks) [1]
4. Outline the cranial-ultrasound surveillance programme for very preterm infants and the monitoring required after any grade III–IV bleed. (6 marks) [9]

Model answer (must-hit)

1. The most likely diagnosis is a large intraventricular haemorrhage, precipitated by the cerebral blood-flow swing from the pneumothorax in an infant with a pressure-passive cerebral circulation. The confirming investigation is an urgent cranial ultrasound, which will show the echogenic bleed, grade it by the Papile system, and reveal any ventricular dilation or parenchymal extension. [1]
2. Immediate management follows ABC with haemodynamic gentleness: maintain the airway and ventilate gently (avoid further blood-pressure swings), drain the pneumothorax, correct the hypotension cautiously with volume or inotrope, and correct the anaemia with a packed-red-cell transfusion. Check and correct the coagulation and platelets. Treat the seizures with phenobarbital 20 mg/kg intravenously as first-line. The principle is minimal handling applied to the whole circulatory system — a rough resuscitation can extend the bleed. [1]
3. The Papile grades are: I, subependymal germinal-matrix haemorrhage; II, intraventricular haemorrhage filling under half the ventricle without dilation; III, intraventricular haemorrhage with acute ventricular dilation; IV, parenchymal venous infarction. The grade IV lesion carries the highest risk of cerebral palsy (roughly 50–70 percent, typically a hemiplegia contralateral to the lesion) and of post-haemorrhagic hydrocephalus. The grade IV lesion is a venous infarct from compression of the terminal veins by the distended ventricle, not simple extension of the bleed. [1]
4. The cranial-ultrasound surveillance programme for very preterm and very-low-birthweight infants is typically performed on day 1, day 3, day 7, and around day 28, plus any time of clinical deterioration — because about half of severe bleeds are clinically silent. After any grade III–IV bleed, serial head circumference measurement (plotted on a preterm chart) and serial ventricular-index measurement on ultrasound detect post-haemorrhagic ventricular dilation before it becomes clinically obvious; an accelerating head circumference and a rising ventricular index prompt escalation. [9]