Ultrasound Obstet Gynecol

. 2021 Mar 16. doi: 10.1002/uog.23630. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/33724570/

Decreased morbidity of ‘bowel-only’ left congenital diaphragmatic hernia patients: implications of prenatal ultrasound diagnosis for prenatal counseling and postnatal management

R A Didier ^1 2 3, E R Oliver ^1 2 3, P Rungsiprakarn ¹, S E DeBari ^1 2, S E Adams ^2 4, H L Hedrick ^2 3 4, N Scott Adzick ^2 3 4, N Khalek ^2 3 4, L J Howell ^2 4, B G Coleman ^1 2 3Affiliations expand

• PMID: 33724570
• DOI: 10.1002/uog.23630

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Abstract

Objectives: To evaluate postnatal outcomes in “stomach-down” left congenital diaphragmatic hernias (CDH) diagnosed by prenatal ultrasound (US) compared to “bowel and stomach” containing left CDH.

Methods: Children with left CDH who underwent prenatal US and postnatal repair at our institution between January 2008 and March 2017 were eligible for this IRB-approved retrospective study. Detailed prenatal US examinations, fetal magnetic resonance imaging (MRI) studies, operative reports, and medical records of children enrolled in our Pulmonary Hypoplasia Program (PHP) were reviewed. Subjects with additional anomalies were excluded. US findings were correlated with postnatal outcomes.

Results: 152 subjects met inclusion criteria and 78 patients had surgically-confirmed liver herniation and were excluded. Of 74 “liver-down” CDH cases, 28/74 (37.8%) were also “stomach-down,” 46/74 (62.2%) had intrathoracic bowel and stomach, and 68/74 (91.9%) had PHP data available for analysis. Of “stomach-down” CDH, 35.7% (10/28) were referred from a referring institution for a suspected lung lesion. There was no significant difference in median observed/expected lung area to head circumference ratio (o/e LHR) measurements between “stomach-down” CDH and “bowel and stomach” CDH (41.5% vs. 38.4%; p=0.41). Furthermore, there was no difference in median MRI o/e total lung volume (TLV) between groups (49.5% vs. 44.0%; p=0.22). However, “stomach-down” CDH patients demonstrated decreased median intubation time (9.5 vs. 18 days, p<0.01), decreased median ECMO time (223.5 vs. 495 hours, p<0.05), decreased oxygen requirement at 30 days of life (11.5% vs. 47.6%, p<0.01), and decreased pulmonary hypertension (9/26 [34.6%] vs. 28/42 [66.7%], p=0.01). No neonatal deaths were observed in “stomach-down” CDH patients and one neonatal death was seen in a patient with intrathoracic stomach herniation.

Conclusions: Despite similar o/e LHR and o/e TLV infants with “stomach-down” CDH have decreased morbidity compared to those with both bowel and stomach herniation. Progressive distention of the stomach over the course of gestation may explain these findings. Furthermore, “stomach-down” left CDH is not infrequently mistaken for a lung mass. Accurate prenatal US characterization is crucial for appropriate prenatal counseling and patient management. This article is protected by copyright. All rights reserved.

Keywords: Congenital diaphragmatic hernia; ECMO therapy; obstetrics; prenatal diagnosis; prenatal ultrasound; pulmonary hypertension; pulmonary hypoplasia.

This article is protected by copyright. All rights reserved.