A case of intrapericardial diaphragmatic hernia with a massive pericardial effusion: fetal diagnosis and therapy

doi:10.1016/j.jpedsurg.2005.07.044

Journal of Pediatric Surgery

Volume 40, Issue 11, November 2005, Pages e43-e45

https://doi.org/10.1016/j.jpedsurg.2005.07.044 Get rights and content

Abstract

Intrapericardial diaphragmatic hernia is a very rare phenotype of neonatal diaphragmatic hernia which is thought to be caused by the developmental failure of the septum transversum. There have been only 10 cases reported since 1980, and among them, only 2 cases were diagnosed in fetal life. We herein report a new case that was diagnosed in fetal life, and pericardiocentesis was performed at 27 weeks of gestation. This is the first case to undergo a fetal interventional therapy. After birth, the patient successfully underwent closure of the hernia, despite severe pulmonary hypoplasia.

Section snippets

Case report

A 30-year-old woman was referred to the department of gynecology and obstetrics of our institute for the assessment of her fetus complicated with bilateral massive pleural effusions. By fetal ultrasonography, a massive fluid collection was detected around the heart and severely compressed bilateral lungs (Fig. 1A and B). Cardiac function was not impaired, and hydrops fetalis was not complicated. To prevent severe pulmonary hypoplasia due to the compression by a massive effusion, percutaneous

Discussion

Intrapericardial diaphragmatic hernia is a very rare phenotype of diaphragmatic hernias seen in the neonatal periods. Since 1980, only 10 cases have been reported in the world [1], [2], [3], [4], [5], [6], [7], [8]. This kind of hernia is thought to be caused by the developmental failure of the retrosternal part of the septum transversum in early gestation, which would develop to be the central tendons of the diaphragm.

The intrapericardial diaphragmatic hernia showed clinically characteristic

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Intrapericardial immature teratoma with pericardial effusion in a 4-month-old boy
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Some reports described similar pathophysiological events that caused pericardial effusion. Highly vascular mediastinal tumors can be associated with exudative effusion [25,26] and, in intrapericardial diaphragmatic hernia, massive pericardial effusion is a specific characteristic in fetuses with left hepatic lobe herniation [27]. Because microscopic examination of the resected specimen did not show tumor rupture, we considered that the massive pericardial effusion in our case was due to exudative fluid collection caused by mechanical obstruction of the inferior vena cava or thoracic duct or the result of inflammation caused by rubbing forces between the pericardium and tumor.
Intrapericardial teratoma is an extremely rare mediastinal teratoma that arises at the junction of the ascending aorta and right atrial appendage. The teratoma is often complicated by massive pericardial effusion that compresses the heart and lungs, leading to heart failure and/or respiratory distress and potentially fatal outcomes in early infants. A 4-month-old boy was referred to our institute with slowly progressive appetite loss and respiratory distress and was diagnosed with intrapericardial teratoma with pericardial effusion. The patient underwent simultaneous surgical drainage of the pericardial effusion and tumor resection. The tumor was pathologically diagnosed as an immature teratoma. The postoperative course was uneventful. He is now being followed up in the outpatient clinic and shows no clinical or radiological signs of recurrence.
Prenatal diagnosis and management for congenital intrapericardial diaphragmatic hernia with massive cardiac effusion: A case report and literature review
2015, Journal of Medical Ultrasound
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Nine cases of intrapericardial diaphragmatic hernia had been reported between 1980 and 2002 [10]. Eight more cases, including ours, have been reported thereafter (Table 1) [1–7]. Summarizing these 17 cases, none had cardiac tamponade despite the massive pericardial effusion; this may be attributable to the slow accumulation process to allow continuous distension of the fetal pericardium [2].
Congenital intrapericardial diaphragmatic hernia with massive pericardial effusion is a rare type of Morgagni hernia. Since 1980, there have been only 16 reported cases. We report on the imaging features of such a case that was diagnosed in utero. The prognosis of congenital intrapericardial diaphragmatic hernia is better than the other types of congenital diaphragmatic hernia, but lung hypoplasia due to compression by the pericardial effusion is not uncommon. Early intervention and treatment should be given to improve the perinatal outcome once the prenatal diagnosis has been made. We have summarized current diagnostic methods and management for this rare phenotype, after reviewing previous case reports and articles relating to the intervention for congenital diaphragmatic hernia.
Intrapericardial diaphragmatic hernia: A rare type of congenital diaphragmatic hernia
2011, Journal of Pediatric Surgery
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Of the few cases detected antenatally, most were observed for the rest of the gestational period and were operated postnatally [8,12]. In a few cases, fetal pericardiocentesis was performed so as to prevent severe pulmonary hypoplasia secondary to compression of the lung followed by postnatal surgical repair [6,10]. In most reported cases, the defect was repaired by simple primary closure.
Diagnosis and management of fetal intrapericardial Morgagni diaphragmatic hernia with massive pericardial effussion
2010, Journal of Pediatric Surgery
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In our case, fetal pericardiocentesis and successive fluid aspirations were performed at 21 and 22 4/7 weeks' gestation and were effective for decompressing the lungs. If this kind of therapy had not been applied, the patient might have died soon after birth because of severe pulmonary hypoplasia [4,7]. Progressive and almost complete invasion of right hemithorax by the fetal liver raised the concern of severe respiratory failure previously described in CDHM with pericardial effusion and unplanned delivery with or without fetal therapy [3,4,7].
Herniation of the liver into the fluid-filled pericardial sac resulting in a thoracic mass is a particularly rare form of Morgagni hernia (congenital diaphragmatic hernia of Morgagni). We report an early antenatal diagnosis of congenital diaphragmatic hernia of Morgagni with pericardium effussion at 21 weeks' gestation. Two pericardiocentesis were performed at 21 and 22 4/7 weeks' gestation because of recurrence of pericardial effussion. Regular ultrasound assessments showed progressive herniation of the liver to practically fill the right hemithorax. An ex utero intrapartum treatment procedure was performed at 37 weeks' gestation to rescue maximum intrathoracic space for ventilation of the remaining functional lung tissue and to establish an airway for postnatal support. After birth, the patient successfully underwent early correction of the hernia. Postoperative course was uneventful, and the newborn girl was discharged 18 days later without complications and is currently doing well.
Prenatal Diagnosis and Management of a Rare Central Tendon Defect Type of Congenital Diaphragmatic Hernia with a Massive Pericardial Effusion
2020, Case Reports in Obstetrics and Gynecology
Congenital diaphragmatic hernia with liver herniation into the pericardial sac in a 30-week gestation infant
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A case of intrapericardial diaphragmatic hernia with a massive pericardial effusion: fetal diagnosis and therapy

Abstract

Section snippets

Case report

Discussion

J Pediatr Surg

Ann Thorac Surg

An unusual hernia: congenital pericardial effusion associated with liver herniation into the pericardial sac

Pediatr Radiol

In utero sonographic diagnosis of diaphragmatic hernia with hepatic protrusion into the pericardium mimicking an intrapericardial tumor

Prenat Diagn