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J Ped Surg Case Reports 12 (2016) 16e20

Journal of Pediatric Surgery CASE REPORTS

journal homepage: www.jpscasereports.com

A Morgagni hernia with an absent ductus venosus: An unusual casecausing unusual consequences

Maria Phillis, Alejandro V. Garcia, Eric B. Jelin*

Department of General Pediatric Surgery, Johns Hopkins University School of Medicine, 1800 Orleans Street, Baltimore, MD 21287, USA

a r t i c l e i n f o

Article history:Received 1 April 2016Received in revised form21 June 2016Accepted 24 June 2016

Key words:Morgagni herniaDuctus venosusLiver neovascularizationCongenital diaphragmatic hernia

* Corresponding author.E-mail address: ejelin1@jhmi.edu (E.B. Jelin).

2213-5766/� 2016 The Authors. Published by Elsevierhttp://dx.doi.org/10.1016/j.epsc.2016.06.011

a b s t r a c t

A Morgagni hernia is a rare form of congenital diaphragmatic hernia (CDH), comprising only 3e5% of allCDH cases. Agenesis of the ductus venosus with direct umbilical vein blood flow to the heart is a rela-tively uncommon finding that is often fatal in utero. We present a case of a 2-month-old infant withMorgagni hernia and absence of the ductus venosus. These combined defects led to neovascularization ofthe liver, severe pulmonary hypertension and right heart failure. In this report, we describe a Morgagnihernia that’s presentation resembled that of a Bochdalek hernia likely because of concomitant absence ofthe ductus venosus causing severe pulmonary hypertension.� 2016 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND

license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Congenital diaphragmatic hernia (CDH) occurs in approximately1 in 2500 births, accounting for about 8% of major congenitalanomalies [1]. A Morgagni hernia is a rare form of CDH, comprisingonly 3e5% of all CDHs. Morgagni hernias are thought to be causedby a failure of the pars sternalis to fuse with the costal arches [2]. Ininfancy they are typically asymptomatic and usually present laterwith nonspecific symptoms [3]. The most common presentation isrecurrent respiratory infection. Associated anomalies are relativelycommon and are usually cardiac in nature [3]. Surgical repair waspreviously accomplished via laparotomy or thoracotomy, but is nowmore frequently done through minimally invasive approachesleading to shorter hospital stays and fewer complications [4,5].

Pulmonary hypertension is commonly seen in infants withclassic Bochdalek CDH, resulting in right to left shunting, hypox-emia and acute right sided heart failure in those most severelyaffected [6]. Agenesis of the ductus venosus associated with directumbilical return to the heart is a relatively uncommon finding witha prevalence of 6/1000 fetal examinations [7]. These defects areassociated with cardiomegaly, heart failure, hydrops fetalis andpostnatal pulmonary hypertension [8e10].

Neovascularization occurs in ischemic states as a method ofimproving physiological function of organs with ischemic damage[11]. Neovascularization of the liver is most often seen in the liter-ature in associationwith liver transplant and ischemia. Case studies

Inc. This is an open access article u

involving transcapsular arterial collateralization of a liver transplantafter hepatic artery occlusion have been seen adult patients [12,13]and failure of liver transplant in pediatric patients [14].

We present a case of a 2-month-old infant withMorgagni herniaand absence of the ductus venosus. These combined defects led toneovascularization of the liver, severe pulmonary hypertension and

Fig. 1. Chest X-ray (AP) showing opacity obscuring the right medial hemidiaphragm

consistent with Morgagni hernia.

nder the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Fig. 2. Sagittal and coronal CT scans showing the Morgagni hernia.

M. Phillis et al. / J Ped Surg Case Reports 12 (2016) 16e20 17

right heart failure. The authors are aware of no other presentationof Morgagni hernia associated with neovascularization and absenceof the ductus venosus.

1. Case report

The patient was a 66 day old male infant who was born viaCaesarian section as a result of maternal preeclampsia and breechpresentation to a gravida 4 para 3 mother at 34 weeks and 2 days

Fig. 3. Echocardiogram showing m

gestation. The pregnancy was complicated by maternal obesityand maternal insulin dependent diabetes. Prenatal imaging hadrevealed agenesis of the ductus venosus with umbilical veindraining into the right atrium, mild tricuspid and mitral valveregurgitation and dilation, cardiomegaly, shortened limbs, andliver calcifications at 29 weeks gestation. Apgar scores at birthwere 7 and 8 at 1 min and 5 min of life. Birth weight was 3060 g.Several dysmorphic features were noted at birth, including lowset ears, high arched palate, microphallus, unilateral palmar

ass effect of liver on the heart.

Fig. 4. Photographs taken intraoperatively of neovascularization of the liver.

M. Phillis et al. / J Ped Surg Case Reports 12 (2016) 16e2018

crease, multiple focal hemangiomas, cardiomyopathy with mildlydepressed biventricular function, and hepatomegaly with fattyinfiltrates. A TORCH infection work up and genetic SNP analysiswere unremarkable. No other genetic analysis was completed. Thepatient was transferred to the NICU for management of tachyp-nea, pulmonary arterial hypertension, hypoxia, requiring CPAPand hypoglycemia. Follow up post-natal ultrasound confirmedprenatal diagnosis of agenesis of the ductus venosus with um-bilical vein draining into the right atrium, mild tricuspid andmitral valve regurgitation and dilation, cardiomegaly, and livercalcifications.

The patient was able to be weaned down to 1.5 L of 100% FiO2on nasal cannula by day 33 of life. The patient developed mildrespiratory distress on DOL 66 with increasing oxygen re-quirements and positive blood cultures. The patient experiencedpersistent desaturation into the 70s, with rhonchi auscultatedon the right side. Chest X-ray revealed an opacity obscuring theright medial hemidiaphragm contour, consistent with a Mor-gagni hernia containing the liver (Fig. 1). A CT of the chest wasconsistent with a Morgagni hernia that was occupied nearlycompletely by Liver (Fig. 2). Specifically, on echocardiogram, itwas noted that the extension of the liver into the lower portionof the right hemithorax was causing mass effect on the adjacentheart and that there was significant pulmonary hypertension(Fig. 3).

Surgical correction was undertaken laparoscopically on day 69of life after the pulmonary hypertension was medically optimized.The abdominal cavity was carefully examined. Significantneovascularization of the liver was noted (Fig. 4). There was alarge diaphragmatic defect centrally located in the expected

position based on the prior imaging. The umbilical vein connecteddirectly to the right atrium and it was divided using bipolarelectrocautery. The hernia sac was excised, avoiding entry intoeither the pleura or the pericardium. The suprahepatic diaphragmwas mobilized away from the hepatic veins to allow for primaryrepair of the hernia without tension or chest wall deformity.There was no kinking of either the IVC or the hepatic veins. Bloodloss was minimal. Fig. 5 shows a schematic of the defect beforeand after repair. Pre-operatively the patient was taking in 65% ofhis feeds PO. By post-operative day four, he was taking in 90% ofhis feeds PO. At 3 week follow-up, he was off oxygen, feeding andgrowing normally. Three months post-repair he was growing anddeveloping normally without symptomatic respiratory or cardiacconcerns.

2. Discussion

There have been three previous cases [7] reported in theliterature of absent ductus venosus and anomalous drainage of theumbilical vein into the right atrium or coronary sinus in associa-tion with development of right sided congenital diaphragmatichernia and dilation of the right atria and ventricle. One of thesecases also involved desaturation and compression of the rightatrium and ventricle with right to left shunting [7]. The authorsare unaware of Morgagni hernia presenting with an absence of thevenosus ductus in any other published case study or review of theliterature.

Extrahepatic umbilical venous drainage is associated with fetalmalformations that involve significant agenesis of the fetal portalsystem, hepatic tumors and focal nodular hyperplasia [15]. We

Fig. 5. Diagram showing the defect pre and post repair. A) shows the liver herniating through the Morgagni defect and the umbilical vein traveling through the defect with drainagedirectly into the heart. B) shows the repair of the diaphragm (re-attachment to the body wall), reduction of the liver and ligation of the umbilical vein.

M. Phillis et al. / J Ped Surg Case Reports 12 (2016) 16e20 19

hypothesize that the liver recruited increased arterial circulationand became hypervascular in order to compensate for the lack ofportal flow resulting in the neovascularization. The ductus venosusallows a portion of the umbilical venous blood to bypass the liverand reach the main circulation more rapidly. It is able to change itsdiameter to adjust the distribution of blood into the heart. Absenceof this structure and direct drainage of the umbilical vein into theheart causes complete liver bypass. This vascular configuration isassociated with cardiac defects, high volume cardiac failure, car-diomegaly and pulmonary hypertension [9,16,17]. Thus, it likelythat our patient’s pulmonary hypertension was in part due to theneonatal effects of fetal hepatic bypass and an absent ductusvenosus. Furthermore, the liver’s lack of tethering by the falciformligament and ductus venosus likely contributed to its ability tosignificantly herniate into the mediastinum. This allowed the liverto put pressure on the heart and contributed to the overall cardiaccompromise. Thus, the combination of a Morgagni hernia and lackof a ductus venosus likely sets the stage for a hemodynamicallysignificant congenital diaphragmatic hernia.

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