Journal of Reproductive Medicine Gynaecology & Obstetrics Category: Medical Type: Case Report
Right Congenital Diaphragamatic Hernia with a Sac - A Case Report
- Cátia Denise Ferreira Rodrigues1*, Sara Bernardes Cunha1, Inês Falcão Reis1, Maria Augusta Pinto1, Teresa Paula Teles1
- 1 Department Of Obstetrics And Gynecology, Centro Hospitalar De Entre O Douro E Vouga, EPE, Aveiro, Portugal
*Corresponding Author:Cátia Denise Ferreira Rodrigues
Department Of Obstetrics And Gynecology, Centro Hospitalar De Entre O Douro E Vouga, EPE, Aveiro, Portugal
Received Date: Oct 17, 2018 Accepted Date: Nov 20, 2018 Published Date: Dec 04, 2018
The incidence of CDH is about 1 in 3000 - 5000 live births and most cases are isolated . Associations with genetic, chromosomal or structural anomalies occur in about 30% of cases and heavily impact on the overall prognosis . Pulmonary compression caused by abdominal viscera is responsible for hypertension and pulmonary hypoplasia, both by mechanical effect as by intrinsic changes that affect the normal development of the lung [3,4]. The most frequently affected side is the left (approximately 80-85% of cases) being rare the bilateral herniation [3,4]. The diagnosis of CDH is made by ultrasound, usually after 24 weeks of gestation. Fetal Magnetic Resonance Imaging (MRI) may be useful when there is doubt in the diagnosis or to optimize the fetal prognosis [1,4]. Despite advances in neonatal intensive care, CDH associated mortality remains high, ranging from 30 to 50% .
Fetal MRI confirmed a massive right CDH that contained the right hepatic lobe surrounded by significant amount of fluid. This defect was associated with a left deviation of the mediastinum and a significant decrease of the right lung area, with about 4cc (Figure 3). The left lung maintained structure and volume preserved. Given the risk of need for postnatal surgical treatment the pregnant woman was transferred to a center with differentiated perinatal support. Delivery occurred at 37 weeks after premature rupture of membranes, resulting in a newborn male with 2860g and Apgar scores of 8 and 9 at 1 and 5 min, respectively.
On the 7th day of life the newborn was submitted to surgery, with a thoracotomy approach, to reduce the herniated viscera and to close the diaphragmatic defect. The surgery was uneventful and confirmed the presence of a hernia sac, previously suggested by the MRI. The newborn was discharged two weeks after surgery. After a year of surveillance in Pediatric consultation the child was discharged, with no need for further medical care.
According to the most recent reviews, right CDH accounts for 8% to 21% of all CDH cases . Right CDH occurs through the defect resulting from failure of the anterior pleuroperitoneal membrane to fuse with the sternum and costal cartilages during embryogenesis. The defect on the right is more difficult to diagnose because the heart remains on the left, the stomach maintains its normal position and the liver can easily be mistaken for the lung. The right CDH is suggested by the direction of portal vein flow, presence of as cites in the chest, or presence of gallbladder in the thoracic cavity. There are several theoretical explanations suggest that impact of right CDH might be worse. First, right lung normally accounts for 57% of total fetal lung volume and any compromise on its development may have a larger clinical impact. Second liver herniation through diaphragm is more frequently seen in right-sided defects, because of anatomic adjacency of the right lobe .
The most common forms of CDH correspond to a direct communication between the abdominal cavity and the thoracic cavity. Anatomically a hernial sac is a plueroperitoneal sac that covers and delimits the herniated contents in the thorax. The presence of this feature was reported in about 20% of the cases. Currently there is only one study that evaluated the prognostic value of the sac. This study concluded that in these situations there is greater preservation of lung volume and consequent better neonatal prognosis . After the diagnosis the conduct should include a careful morphological ultrasound, a fetal echocardiogram and the cytogenetic study .
Although the ultrasound is the method of choice in pregnancy, fetal MRI is increasingly used. In the cases of CDH, MRI easily allows the distinction between liver, lung and intestine, as well as the relation between the liver and the diaphragm and the estimation of the remaining lung volume . The most relevant prenatal prognostic factors are the value of the Lung-to-Head Ratio (LHR) and the presence of intrathoracic liver [9,10]. Current studies are controversial in relation to the severity of right CDH compared to left CDH cases, and it is not clear whether it is associated with higher mortality .
In most centers the treatment remains postnatal. The mean age for surgery is controversial and dependent on the postnatal evaluation, however it is agreed that the surgery should be performed after cardiopulmonary stabilization. Congenital diaphragmatic hernia mortality remains high. However in cases of isolated CDH the survival rate may reach 78% .
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Citation:Rodrigues CDF, Cunha SB, Reis IF, Pinto MA, Teles TP (2018) Right Congenital Diaphragmatic Hernia with Sac – A Case Report. J Reprod Med Gynecol Obstet 3: 014.
Copyright: © 2018 Cátia Denise Ferreira Rodrigues, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.