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CASE LETTER
Year : 2019  |  Volume : 36  |  Issue : 4  |  Page : 361-363  

Prenatal diagnosis and perinatal management of a bilateral anterior congenital diaphragmatic hernia


1 Medical College of Georgia, Augusta University, Augusta, Georgia
2 Department of Pediatric Surgery, Medical College of Georgia, Augusta University, Augusta, Georgia
3 Department of Pediatric Radiology, Medical College of Georgia, Augusta University, Augusta, Georgia

Date of Web Publication28-Jun-2019

Correspondence Address:
Ms. Elyssa Faye Cohen
Medical College of Georgia, Augusta University, Augusta
Georgia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/lungindia.lungindia_485_18

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How to cite this article:
Cohen EF, Pillai RB, Hatley RM, Bajaj M. Prenatal diagnosis and perinatal management of a bilateral anterior congenital diaphragmatic hernia. Lung India 2019;36:361-3

How to cite this URL:
Cohen EF, Pillai RB, Hatley RM, Bajaj M. Prenatal diagnosis and perinatal management of a bilateral anterior congenital diaphragmatic hernia. Lung India [serial online] 2019 [cited 2019 Sep 21];36:361-3. Available from: http://www.lungindia.com/text.asp?2019/36/4/361/261714



Sir,

Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm that allows abdominal viscera to herniate into the thorax resulting in pulmonary hypoplasia.[1] CDH occurs in one of 2000–3000 births.[2] About 97% of CDH are unilateral, 1% bilateral, and 2% are central hernias.[2] Bilateral CDH is challenging to diagnose prenatally because the mediastinal shift is minimal, and the right-sided herniated liver is of similar echogenicity as the lung.[2] Bilateral CDH accounts for five in 1,000,000 births, and bilateral anterior CDH occurs in six of 10,000,000 births.[3] We describe a rare case of a CDH where the anterior half of the diaphragm is deficient bilaterally and centrally, leading to bilateral anterior herniation of the liver and small loop of bowel, as well as secondary complications of narrow intimal tear of the aorta at the level of the right carotid artery.

Bilateral CDH carries significant mortality, with a mortality rate of 65%–74% versus 20%–35% for unilateral CDHs.[1],[2] Prenatal diagnosis is extremely important as it can improve delivery planning, postnatal care, and evaluation for chromosomal and congenital anomalies.[4],[5]

This patient is a male born at 39.1 weeks by planned cesarean section to a G3P2 mother. The infant was suspected to have a CDH on the antenatal ultrasound. The CDH was confirmed by fetal magnetic resonance imaging (MRI) as an anterior CDH with the liver and a small portion of bowel herniating superiorly through the defect [Figure 1].
Figure 1: Fetal magnetic resonance imaging sagittal. T2-weighted image. 37 weeks gestation. Herniated liver (arrow) and pleural effusion. Intact posterior diaphragm (star)

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The boy was born with acute respiratory distress syndrome and needed extensive cardiorespiratory support including extracorporeal membrane oxygenation (ECMO). Once the patient was relatively stable in the early postnatal period, computed tomography imaging was performed to better visualize the CDH [Figure 2]. This imaging showed a bilateral anterior diaphragmatic defect with an intact posterior diaphragm, a hernia containing a large portion of the liver and a bowel loop posterior to it, bilateral hypoplastic lungs, and a superiorly displaced heart.
Figure 2: Coronal computed tomography scan. 29 days of life. Liver herniating through the bilateral anterior congenital diaphragmatic defects (arrowheads). The heart is displaced superiorly (star). Hypoplastic lungs (arrows)

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An echocardiogram demonstrated endothelial disruption of the aorta at the level of the right carotid artery possibly due to the previous ECMO cannula position. The tissue flap was blocking blood flow and created an aortic arch gradient and systemic hypertension that was controlled with esmolol. Consultations between pediatric surgery and pediatric cardiology determined the patient was stable to repair the diaphragmatic hernia and the aortic arch. A bilateral anterior CDH repair was performed successfully, and notable findings during this procedure included multiple blebs and surface irregularity on the right lung, confluent pericardial and diaphragmatic tissue, hypoplastic left lung, and diaphragmatic remnants. Seventeen days later, the infant underwent an uncomplicated aortic arch reconstruction of the intimal flap dissection of the transverse arch.

In our case, although bilateral CDH was suspected based on sonographic evidence, the definitive prenatal diagnosis was based on further evidence from fetal MRI, which demonstrated liver and small bowel herniation into the pleural cavity. There was also evidence of bilateral lung hypoplasia and superior displacement of the heart.

Patients who are diagnosed prenatally are more likely to have severe defects that are easily visualized on fetal ultrasound and carry a worse prognosis.[2] As such, surgical repair for CDH continues to be a challenge requiring a multidisciplinary approach with collaboration between surgery, neonatology, cardiology, and radiology teams. Presurgical management and optimal timing of surgery remain variable; to date, studies evaluating the survival outcomes of early versus delayed surgical repair demonstrate inconclusive evidence.[2] The prognosis of bilateral CDH remains poor, with the severity of pulmonary hypertension and pulmonary hypoplasia being the important prognostic risk factors.[1],[2],[5]

Bilateral CDH is an extremely rare type of CDH, and our case is novel interest because it presents an even rarer variation-a bilateral anterior CDH of an infant who was diagnosed prenatally and presented at birth with heart dysfunction requiring ECMO. Literature/case reports on bilateral anterior CDH are extensively limited. Our case demonstrates how integrative use of different imaging modalities can guide prenatal diagnosis, surgical planning, and neonatal management in hopes of improving survival outcomes of CDH patients.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Neville HL, Jaksic T, Wilson JM, Lally PA, Hardin WD Jr. Hirschl RB, et al. Bilateral congenital diaphragmatic hernia. J Pediatr Surg 2003;38:522-4.  Back to cited text no. 1
    
2.
Botden SM, Heiwegen K, van Rooij IA, Scharbatke H, Lally PA, van Heijst A, et al. Bilateral congenital diaphragmatic hernia: Prognostic evaluation of a large international cohort. J Pediatr Surg 2017;52:1475-9.  Back to cited text no. 2
    
3.
Khandelwal S, Oelschlager BK. Video. Laparoscopic repair of congenital bilateral Morgagni hernia. Surg Endosc 2011;25:2010.  Back to cited text no. 3
    
4.
Song MS, Yoo SJ, Smallhorn JF, Mullen JB, Ryan G, Hornberger LK, et al. Bilateral congenital diaphragmatic hernia: Diagnostic clues at fetal sonography. Ultrasound Obstet Gynecol 2001;17:255-8.  Back to cited text no. 4
    
5.
Hiasa KI, Fujita Y, Fukushima K, Nagata K, Taguchi T, Wake N, et al. Ultrasound and MR images of prenatally diagnosed bilateral congenital diaphragmatic hernia, a rare variation of CDH. Clin Imaging 2012;36:639-42.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]



 

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