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Neurological findings in esophageal atresia | Overview

Kagan MS, Wang JT, Pier DB, Zurakowski D, Jennings RW, Bajic D. Infant Perioperative Risk Factors and Adverse Brain Findings Following Long-Gap Esophageal Atresia Repair. J Clin Med. 2023 Feb 23;12(5):1807. doi: 10.3390/jcm12051807. PMID: 36902591; PMCID: PMC10003188.

Infants that undergo long-gap esophageal atresia with Foker process are exposed to significant stress from surgery, sedation, and anesthesia load in our efforts to repair the gap and allow for normal feeding. We measured several easily quantifiable clinical end-point measures (e.g., gestational age at birth; anesthesia exposure; sedation length; muscle relaxation length; days of receiving antibiotics and steroid treatment). Together, they serve as indirect markers in assessing the risk of brain abnormalities following long-gap esophageal atresia repair.


McMahon MJ, Evanovich DM, Pier DB, Kagan MS, Wang JT, Zendejas B, Jennings RW, Zurakowski D, Bajic D. Retrospective analysis of neurological findings in esophageal atresia: Allostatic load of disease complexity, cumulative sedation, and anesthesia exposure. Birth Defects Res. 2023 Nov 7. doi: 10.1002/bdr2.2269. Epub ahead of print. PMID: 37936552.

This is a retrospective study of infants following esophageal atresia repair at our institution (2009–2020). All infants underwent spinal imaging diagnostic studies as part of ruling our VACTERL association.  In contrast, head (brain) imaging findings were identified in 67% (22/33) of infants in the context of clinically indicated imaging (47%; 33/70). We propose that all infants with EA undergo brain imaging in addition to routine spinal imaging given the high burden of abnormal brain findings in our retrospective cohort.


Kagan MS, Mongerson CRL, Zurakowski D, Jennings RW, Bajic D. Infant study of hemispheric asymmetry after long-gap esophageal atresia repair. Ann Clin Transl Neurol. 2021 Nov;8(11):2132-2145. doi: 10.1002/acn3.51465. Epub 2021 Oct 18. PMID: 34662511; PMCID: PMC8607454.

The left side of the brain is expected to be normally bigger in infancy than the right side. Our pilot quantitative MRI study of hemispheric volumes suggests that premature patients might be at risk of altered expected left-greater-than-right forebrain asymmetry following repair of long-gap esophageal atresia.


Bajic D, Rudisill SS, Jennings RW. Head circumference in infants undergoing Foker process for long-gap esophageal atresia repair: Call for attention. J Pediatr Surg. 2021 Sep;56(9):1564-1569. doi: 10.1016/j.jpedsurg.2021.01.030. Epub 2021 Feb 13. PMID: 33722370; PMCID: PMC8362829.

Results suggest uncompromised body and head growth after repair of long-gap esophageal atresia with Foker process irrespective of the gestational age at birth. In this study, we show a discrepancy between head circumference (novel data) and brain size (previously published data) in the same cohort, suggesting that head circumference might not be the best indirect measure of brain size in a selected group of patients undergoing Foker process for long-gap esophageal atresia repair.


Mongerson CRL, Jaimes C, Zurakowski D, Jennings RW, Bajic D. Infant Corpus Callosum Size After Surgery and Critical Care for Long-Gap Esophageal Atresia: Qualitative and Quantitative MRI. Sci Rep. 2020 Apr 14;10(1):6408. doi: 10.1038/s41598-020-63212-3. PMID: 32286423; PMCID: PMC7156662.

This is a qualitative and quantitative MRI study of corpus callosum (major white matter tract that connects left and right brain hemisphere). This non-sedated research brain MRI was acquired for term-born (n= 13) and premature (n = 13) patients following treatment for long-gap esophageal atresia, and controls (n= 20) <1 year corrected age. Our report suggests delayed or diminished CC growth in comparison to controls, with no difference between term-born and premature patients. This work is important because previous studies in preterm infants showed that white matter abnormalities of the corpus callosum can serve as an important predictor of neurodevelopmental outcomes.


Rudisill SS, Wang JT, Jaimes C, Mongerson CRL, Hansen AR, Jennings RW, Bajic D. Neurologic Injury and Brain Growth in the Setting of Long-Gap Esophageal Atresia Perioperative Critical Care: A Pilot Study. Brain Sci. 2019 Dec 17;9(12):383. doi: 10.3390/brainsci9120383. PMID: 31861169; PMCID: PMC6955668.

This is series case report in preterm and full-term infants (n = 3/group) who underwent non-sedated brain MRI twice: before (PRE-Foker scan) and after (POST-Foker scan) completion of perioperative care. A neuroradiologist reported on qualitative brain MRI findings. Our quantitative analysis showed increased intracranial space, and decreased brain and corpus callosum size and growth in the context of complex perioperative critical care (before and after treatment). 


Lee Mongerson CR, Jennings RW, Zurakowski D, Bajic D. Quantitative MRI study of infant regional brain size following surgery for long-gap esophageal atresia requiring prolonged critical care. Int J Dev Neurosci. 2019 Dec;79:11-20. doi: 10.1016/j.ijdevneu.2019.09.005. Epub 2019 Sep 26. PMID: 31563705; PMCID: PMC6897486.

Both full-term and premature critically ill infants undergoing life-saving surgery for long-gap esophageal atresia are at risk of smaller total and regional brain size. Normalized volumes (% of the whole) of the forebrain, deep gray matter, cerebellum and brain stem support globally delayed or diminished brain growth in patients. Future research should look into neurodevelopmental outcomes of infants born with long-gap esophageal atresia irrespective of the gestational age at birth.


Kagan MS, Mongerson CRL, Zurakowski D, Bajic D. Impact of Infant Thoracic Non-cardiac Perioperative Critical Care on Homotopic-Like Corpus Callosum and Forebrain Sub-regional Volumes. Front Pain Res (Lausanne). 2022 Apr 7;3:788903. doi: 10.3389/fpain.2022.788903. PMID: 35465294; PMCID: PMC9021551.

This is a quantitative neuroanatomical study using brain MRI to evaluate volumetric relationship between regions of corpus callosum (major white matter bundle connecting left and right side of the brain) and its corresponding brain region. The results are done in a pilot cohort of term-born and premature infants following repair of long-gap esophageal atresia with Foker process. Results contribute to our previous data reports implicating globally smaller brain size in selected group of critically ill infants.


Mongerson CRL, Wilcox SL, Goins SM, Pier DB, Zurakowski D, Jennings RW, Bajic D. Infant Brain Structural MRI Analysis in the Context of Thoracic Non-cardiac Surgery and Critical Care. Front Pediatr. 2019 Aug 2;7:315. doi: 10.3389/fped.2019.00315. PMID: 31428593; PMCID: PMC6688189.

This was the first study in a pilot cohort of infants demonstrating incidental brain findings on research brain MRI following repair of long-gap esophageal atresia. Full-term infants appear to demonstrate similar brain vulnerability as premature infants. Study included qualitative and quantitative brain MRI metrics.