Abstract 2- 0915-0930
Category: Clinical

At the end of the session,
participants will be able to:

  1. Describe the concept of delayed brain development occurring amongst infants suffering from cardiothoracic malformations.
  2. Demonstrate that features of premature-type acute hypoxic ischemic injury (e.g., periventricular leukomalacia and pontosubicular necrosis) are encountered in infants dying shortly after surgical repair for cardiothoracic malformations.
  3. Illustrate that the presence of premature-type acute hypoxic ischemic injury can be used to estimate the degree of cerebral developmental delay in infants suffering from cardiothoracic malformations.

COI Disclosure:

None to disclose


Karina Chornenka is a PGY-3 Neuropathology resident at the University of British Columbia.


Karina Chornenka 1,  Christopher Dunham 2

 1 Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada

2 Division of Anatomical Pathology, Children’s and Women’s Health Centre of BC, Vancouver, BC, Canada

Target Audience:
Pathologists, Residents, Medical Students

Communicator, Collaborator, Health Advocate, Scholar, Professional

Utilizing neurodevelopmental time windows of hypoxic-ischemic pathology to infer brain maturity in patients with congenital cardiothoracic defects


Neuroradiologic investigations have demonstrated that cerebral development is delayed by 2-4 weeks in infants suffering from congenital heart defects and congenital diaphragmatic hernia (CHD/CDH). These estimates are based upon application of the “total maturation score” (TMS) system that evaluates brain development using magnetic resonance imaging (MRI) by assessing myelination, cortical gyration, insular development, T1 white matter signal intensity and the involution of the germinal matrix (N Engl J Med 2007;357: 1928-1938, J Pediatr Surg 2012;47:453-461). These infants often require surgical correction of their malformations shortly after birth, but unfortunately some do not survive. Of those coming to autopsy, it is not uncommon to encounter acute hypoxic ischemic injury (HII). We examined three individuals born at term with CHD/CDH, ranging in age from 3 to 5 months, who died shortly after surgery and displayed evidence of neuropathology that included acute HII characterized by periventricular leukomalacia (PVL) and pontosubicular necrosis (PSN). PVL and PSN are typically encountered in the context of prematurity and occur during specific developmental time windows (i.e., PVL: 24-32 weeks gestational age; PSN: 20 weeks gestational age – 2 months postnatal) (Acta Neuropathol 1995;90:7-10, Acta Neuropathol 2005;110:563-578). Given the ages of the affected individuals herein, we suggest that the presence of premature-type neuropathology in the form of acute HII could be used to support the hat infants with CHD/CDH incur delayed brain development. Moreover, based on our observations we propose that the delay in cerebral development could be longer than previous estimates using MRI.