Paeds Cases · fetal-neonatal-and-perinatal
Intraventricular haemorrhage and periventricular leukomalacia — structured clinical encounter
Structured encounter testing the approach to a preterm infant with a sudden deterioration from a large intraventricular haemorrhage: recognition, Papile grading, immediate management, and the prevention and prognostic-counselling discussion.
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Target exams
Station brief (candidate)
You are the neonatal registrar. 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. The team asks you to establish the diagnosis and immediate management. You then proceed to the cranial-ultrasound result, the grading, the prevention discussion, and the prognostic counselling. You have 12 minutes with the team and 5 minutes for examiner discussion. [1]
Information available on request
- 26 weeks' gestation, birthweight 760 g; antenatal corticosteroids given; intubated and ventilated for respiratory distress syndrome. [1]
- At 36 h: witnessed pneumothorax requiring drainage; haemoglobin falls from 145 to 88 g/L; metabolic acidosis (pH 7.18, base deficit 12 mmol/L); blood pressure falls; three brief focal seizures; anterior fontanelle bulging. [1]
- Urgent cranial ultrasound: echogenic material filling and distending both lateral ventricles, with a wedge-shaped echogenic parenchymal lesion in the right periventricular white matter. [1]
Tasks
- Give the diagnosis and grade the intraventricular haemorrhage using the Papile system, justifying it from the ultrasound. [1]
- Outline the immediate bedside management, including the haematological correction and the first-line antiseizure medication with its dose and route. [1]
- Describe the monitoring required after a grade III–IV bleed, and the management options if post-haemorrhagic ventricular dilation develops. [6]
- Outline the evidence-based prevention strategies for IVH for future preterm births, and explain how you would frame the prognostic counselling with the family. [2] [3] [4] [9]
Marking anchors
Must-hit
- Diagnoses a large intraventricular haemorrhage with parenchymal extension, grading it as a grade IV IVH (a parenchymal venous infarct) on the basis of the distended ventricles and the wedge-shaped periventricular parenchymal lesion; explains that grade IV is a venous infarction from compression of the terminal veins by the distended ventricle, not simple extension of the bleed. [1]
- Begins ABC stabilisation with haemodynamic gentleness, drains the pneumothorax, corrects the anaemia with packed red cells (and corrects coagulation and platelets), and treats the seizures with phenobarbital 20 mg/kg intravenously as first-line; emphasises that a rough resuscitation can extend the bleed. [1]
- Monitors with serial head circumference (preterm chart) and serial ultrasound ventricular-index measurement; intervention options for post-haemorrhagic ventricular dilation are serial lumbar puncture, ventricular access device, DRIFT, and ventriculoperitoneal shunt, citing the Whitelaw 2007 trial and its equipoise. [6]
Merit
- Names the prevention bundle with trial evidence: antenatal corticosteroids, delayed cord clamping (Rabe 2019 Cochrane), caffeine citrate (Schmidt 2006 CAP trial), and prophylactic indomethacin for the extremely preterm (Ment 1994), naming haemodynamic stability as the mechanistic thread. [2] [3] [4]
- Frames the prognostic counselling as a calibrated, revisitable probability rather than a verdict: a grade IV IVH carries a roughly 50–70 percent risk of a spastic hemiplegia contralateral to the lesion and a high rate of cognitive impairment, but the individual outcome is uncertain and the family is supported through surveillance and early intervention. [9]
- Describes the cranial-ultrasound surveillance programme (day 1, 3, 7 and ~28) and the role of MRI at term-equivalent age for the diffuse non-cystic white-matter injury that ultrasound underestimates. [5] [9]
Fail
- Withholds or delays the cranial ultrasound or the haemodynamic stabilisation because the infant 'needs to be more stable first.' [1]
- Starts acetazolamide or furosemide to 'prevent hydrocephalus,' or claims a drug reliably reverses or halts the bleed. [6]
- Counsels the family with a fixed verdict ('your baby will definitely have cerebral palsy') rather than a calibrated probability, or states that nothing could have been done to prevent the bleed. [2] [9]
References
- [1]Papile LA; Burstein J; Burstein R; Koffler H Incidence and evolution of subependymal and intraventricular hemorrhage: a study of infants with birth weights less than 1,500 gm. J Pediatr, 1978.PMID 305471
- [2]Ment LR; Oh W; Ehrenkranz RA; et al Low-dose indomethacin and prevention of intraventricular hemorrhage: a multicenter randomized trial. Pediatrics, 1994.PMID 8134206
- [3]Schmidt B; Roberts RS; Davis P; et al Caffeine therapy for apnea of prematurity. N Engl J Med, 2006.PMID 16707748
- [4]Rabe H; Gyte GM; Díaz-Rossello JL; Duley L Effect of timing of umbilical cord clamping and other strategies to influence placental transfusion at preterm birth on maternal and infant outcomes. Cochrane Database Syst Rev, 2019.PMID 31529790
- [5]Volpe JJ Dysmaturation of Premature Brain: Importance, Cellular Mechanisms, and Potential Interventions. Pediatr Neurol, 2019.PMID 30975474
- [6]Whitelaw A; Evans D; Carter M; et al Randomized clinical trial of prevention of hydrocephalus after intraventricular hemorrhage in preterm infants: brain-washing versus tapping fluid. Pediatrics, 2007.PMID 17403819
- [9]O'Shea TM; Kuban KC; Allred EN; et al Neonatal cranial ultrasound lesions and developmental delays at 2 years of age among extremely low gestational age children. Pediatrics, 2008.PMID 18762501