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Paeds Topicsfetal-neonatal-and-perinatal

Paeds · fetal-neonatal-and-perinatal

Intraventricular haemorrhage and periventricular leukomalacia

Also known as Intraventricular haemorrhage and periventricular leukomalacia · Germinal matrix haemorrhage · Periventricular leukomalacia · Preterm brain injury · White-matter injury of prematurity

Fellowship guide to the two great injuries of the preterm brain: germinal-matrix intraventricular haemorrhage and periventricular leukomalacia — the vascular vulnerability, Papile grading, the white-matter cascade, cranial-ultrasound surveillance, prevention, and family-centred prognostic counselling.

high10 referencesUpdated 12 July 202623 min readVerification in progress

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RACP DWEMRCPCH Theory

Red flags

  • A sudden unexplained drop in haemoglobin, apnoea, seizures, or a bulging anterior fontanelle in a preterm infant is intraventricular haemorrhage until proven otherwise — obtain a cranial ultrasound urgently
  • Post-haemorrhagic ventricular dilation can progress to hydrocephalus within days; serial head circumference and ultrasound monitoring are mandatory after any grade II–IV IVH
  • Grade III–IV IVH and cystic PVL carry a high risk of cerebral palsy, cognitive impairment and post-haemorrhagic hydrocephalus — honest, family-centred prognostic counselling must begin early
  • No drug reverses established IVH or PVL; management is supportive, and prevention through antenatal steroids, delayed cord clamping and haemodynamic stability is the only demonstrably effective strategy

Life stages

fetalneonate

Care settings

nicudelivery-roomretrieval

Clinical exam formats

written-onlyracp-dce-long-casemrcpch-short-clinical

Board mappings

  • Intraventricular haemorrhage and periventricular leukomalacia
  • Long case: intraventricular haemorrhage and periventricular leukomalacia
  • Communication: breaking bad news after severe preterm brain injury
  • Patient Care: intraventricular haemorrhage and periventricular leukomalacia
  • Medical Expert: intraventricular haemorrhage and periventricular leukomalacia
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Saved on this device.

Practise this topic

  • Short-answer question1
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Target exams

RACP DWEMRCPCH Theory

Red flags

  • A sudden unexplained drop in haemoglobin, apnoea, seizures, or a bulging anterior fontanelle in a preterm infant is intraventricular haemorrhage until proven otherwise — obtain a cranial ultrasound urgently
  • Post-haemorrhagic ventricular dilation can progress to hydrocephalus within days; serial head circumference and ultrasound monitoring are mandatory after any grade II–IV IVH
  • Grade III–IV IVH and cystic PVL carry a high risk of cerebral palsy, cognitive impairment and post-haemorrhagic hydrocephalus — honest, family-centred prognostic counselling must begin early
  • No drug reverses established IVH or PVL; management is supportive, and prevention through antenatal steroids, delayed cord clamping and haemodynamic stability is the only demonstrably effective strategy

Life stages

fetalneonate

Care settings

nicudelivery-roomretrieval

Clinical exam formats

written-onlyracp-dce-long-casemrcpch-short-clinical

Board mappings

  • Intraventricular haemorrhage and periventricular leukomalacia
  • Long case: intraventricular haemorrhage and periventricular leukomalacia
  • Communication: breaking bad news after severe preterm brain injury
  • Patient Care: intraventricular haemorrhage and periventricular leukomalacia
  • Medical Expert: intraventricular haemorrhage and periventricular leukomalacia

Overview & Definition

Intraventricular haemorrhage (IVH) and periventricular leukomalacia (PVL) are the two signature brain injuries of the preterm infant, and they share a single anatomical stage: the periventricular region, where the fragile germinal matrix vasculature and the vulnerable premyelinating oligodendrocytes of the white matter lie in close apposition to the lateral ventricles. A haemorrhage from the germinal matrix that breaches the ependyma and enters the ventricular system is an IVH; injury to the periventricular white matter itself — whether from ischaemia, inflammation, or the downstream effect of a large haemorrhage — is PVL. [1] [5]

The two are clinically and prognostically intertwined. A large IVH can infarct the adjacent parenchyma (the grade IV lesion), obstruct cerebrospinal fluid pathways to cause post-haemorrhagic hydrocephalus, and trigger the inflammatory cascade that injures white matter. Conversely, the same haemodynamic instability and infection that cause PVL can also rupture the germinal matrix. For clinician practical point is that an IVH and a PVL are often found together on the same cranial ultrasound, and the severity of each independently shapes the prognosis a family must be helped to understand. [5] [10]

What are IVH and PVL, and why are they grouped together?

An intraventricular haemorrhage is bleeding that originates in the fragile germinal matrix of the preterm brain and enters the ventricular system, graded by the Papile system (grades I to IV). Periventricular leukomalacia is injury to the white matter flanking the ventricles, classically from ischaemia and inflammation of the premyelinating oligodendrocytes. They are grouped because they share the periventricular anatomy, the same risk profile of prematurity and haemodynamic instability, and because a severe IVH often coexists with or directly causes white-matter injury. [1] [5]

The preterm infant who suddenly deteriorates is bleeding until proven otherwise

A sudden unexplained fall in haemoglobin, a new metabolic acidosis, apnoea, hypotension or a bulging anterior fontanelle in an infant under 32 weeks' gestation, or under 1500 g, demands an urgent cranial ultrasound. Most IVH occurs in the first 72 hours of life, and over half of severe bleeds are clinically silent — so the index of suspicion must be high and the ultrasound must be done early. [1]

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References10ShowHide
  1. [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. [2]Ment LR; Oh W; Ehrenkranz RA; Philip AG; Duncan CC; Makuch RW Low-dose indomethacin and prevention of intraventricular hemorrhage: a multicenter randomized trial. Pediatrics, 1994.PMID 8134206
  3. [3]Schmidt B; Roberts RS; Davis P; Doyle LW; Barrington KJ; Ohlsson A; Solimano A; Tin W Caffeine therapy for apnea of prematurity. N Engl J Med, 2006.PMID 16707748
  4. [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. [5]Volpe JJ Dysmaturation of Premature Brain: Importance, Cellular Mechanisms, and Potential Interventions. Pediatr Neurol, 2019.PMID 30975474
  6. [6]Whitelaw A; Evans D; Carter M; Thoresen M; Wrobel C; Odd DE; Cohen N; Brownsdon S; Puntis J; James SR; et al Randomized clinical trial of prevention of hydrocephalus after intraventricular hemorrhage in preterm infants: brain-washing versus tapping fluid. Pediatrics, 2007.PMID 17403819
  7. [7]Luyt K; Jary SL; Lea CL; Young GR; Hurrion E; Garnett E; Smallman LA; Steventon V; Hollands L; Cowan FM; et al Drainage, irrigation and fibrinolytic therapy (DRIFT) for posthaemorrhagic ventricular dilatation: 10-year follow-up of a randomised controlled trial. Arch Dis Child Fetal Neonatal Ed, 2020.PMID 32623370
  8. [8]Volpe JJ Cerebellum of the premature infant: rapidly developing, vulnerable, clinically important. J Child Neurol, 2009.PMID 19745085
  9. [9]O'Shea TM; Kuban KC; Allred EN; Paneth N; Hirtz D; Fichorova RN; Heeren T; Leviton A Neonatal cranial ultrasound lesions and developmental delays at 2 years of age among extremely low gestational age children. Pediatrics, 2008.PMID 18762501
  10. [10]Logan JW; Westra SJ; Allred EN; Bose C; Leviton A; Kuban KC; O'Shea TM; Paneth N; ELGAN Study Group Antecedents of perinatal cerebral white matter damage with and without intraventricular hemorrhage in very preterm newborns. Pediatr Neurol, 2013.PMID 23859853

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