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Journal of Pediatric Surgery (2011) 46, E29–E31

Intrapericardial diaphragmatic hernia: a rare type ofcongenital diaphragmatic herniaVishesh Jain⁎, Subhasis Roy Choudhury, Rajiv Chadha, Archana Puri

Department of Pediatric Surgery, Lady Hardinge Medical College and Kalawati Saran Children's Hospital, New Delhi110001, India

Received 20 November 2010; revised 28 December 2010; accepted 1 February 2011

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Key words:Intrapericardialdiaphragmatic hernia;Peritoneopericardialdiaphragmatic hernia

Abstract An extremely rare case of congenital intrapericardial diaphragmatic hernia is presented. Since1981, only 14 cases have been reported in the literature. A 5-year-old girl presented with dyspnea onexertion and easy fatigability. Computed tomography was suggestive of an anterior diaphragmatichernia. Laparoscopy followed by successful open repair of hernia was performed.© 2011 Elsevier Inc. All rights reserved.

Intrapericardial diaphragmatic hernia is a very rare typeof diaphragmatic hernia in which there is herniation ofabdominal contents into the pericardial variety. It may becongenital or traumatic in origin, with the former beingmore common in children. There have been only 14 similarcases previously reported since 1981, and the presentationhad been in the antenatal period or in infancy [1-10]. Weherein report the 15th case of intrapericardial diaphragmatichernia in a 5-year-old girl, who underwent successful openrepair. A detailed case report along with review of literatureis presented.

1. Case history

A 5-year-old girl presented with dyspnea on exertion andeasy fatigability for the past year. The parents noticed thatthe girl got breathless and tired frequently during her daily

⁎ Corresponding author.E-mail address: dr.vishesh79@gmail.com (V. Jain).

022-3468/$ – see front matter © 2011 Elsevier Inc. All rights reserved.oi:10.1016/j.jpedsurg.2011.02.003

routine activities. There was no history of cyanosis, syncope,or any significant trauma. The child had an uneventfulperinatal period with a full-term normal vaginal delivery athome. No antenatal ultrasound had been performed. Ongeneral examination, the vital signs were normal. There wasno external chest or sternal deformity. The heart sounds werenormal in pattern but decreased in intensity. Air entry wasequal on both sides. Plain chest radiographs revealedwidening of cardiac shadow along with the presence offew radiolucent shadows. Computed tomographic scanalong with oral contrast showed presence of contrast-filledbowel loops in the thoracic cavity, surrounding the heartanteriorly and laterally (Fig. 1A, B). The child underwent adiagnostic laparoscopy, revealing herniation of part of thesmall bowel and transverse colon into the pericardial cavitythrough a defect in the anterior diaphragm. On reducing theviscera, the heart was seen directly without any interveningpericardium or peritoneum (Fig. 2). The defect measured 6 ×6 cm; and on its edge, a fleshy falciform ligament waspresent. Because of the large size, laparotomy and openrepair of the defect using falciform ligament to patch thedefect were performed. The falciform ligament was found incontinuity with the left edge of the defect. The posterior free

Fig. 1 A, Coronal view of chest computed tomography with oral contrast showing herniation of contrast-filled bowel loops (black arrows)around the heart (white arrowhead). B, Chest computed tomography with oral contrast showing herniation of contrast-filled bowel loops (blackarrows) around the heart.

E30 V. Jain et al.

edge of the falciform ligament was swung to cover the defectafter detaching it from the parietal abdominal wall and wassutured to the edges of the defect with interrupted 3-0-polypropylene sutures. The child had an uneventful recoveryand remains asymptomatic on follow-up. Plain chestradiograph performed before discharge showed decreasedwidening of the cardiac shadow along with absence of anyradiolucent shadow.

2. Discussion

The incidence of congenital diaphragmatic hernia isreported to be 1 in 2200 births. The most common typeinvolves a posterolateral defect (Bochdalek type), whereasMorgagni hernias with a retrosternal defect are rare.Extremely rare are the congenital intrapericardial diaphrag-matic hernia in which the viscera herniates into the pericardial

Fig. 2 Findings during laparoscopy after reduction of herniatedviscera show large anterior diaphragmatic defect with heart seenwithout any intervening layer.

cavity. This condition has also been referred to as perito-neopericardial diaphragmatic hernia in a few reports[11,12]. This condition is caused by the developmentalabnormality of the retrosternal septum transversum [2,9].

The presentations of these lesions are diverse. Antenatal-ly, they can present as massive pericardial effusion orpericardial mass [3,6-10]. Most affected neonates aresymptomatic in the immediate postnatal period, with severerespiratory distress and cyanosis secondary to massivepleural effusion or pulmonary hypoplasia [1,2,4,5]. Incontrast, the presented case was stable without markedcardiorespiratory compromise. Congenital lesions may goundetected and can present in adulthood with complaints ofdyspnea, palpitations, cyanosis, abdominal pain, chest pain,or fatigue [11,12].

Diagnosis is suggested on radiological investigationslike ultrasonography or computed tomography and con-firmed intraoperatively. The treatment of affected casesespecially when detected in utero is not standardized. Of thefew cases detected antenatally, most were observed for therest of the gestational period and were operated postnatally[8,12]. In a few cases, fetal pericardiocentesis wasperformed so as to prevent severe pulmonary hypoplasiasecondary to compression of the lung followed by postnatalsurgical repair [6,10]. In most reported cases, the defectwas repaired by simple primary closure. In the reportedcase, the defect was too large to be closed primarily; soa musculofascial flap was fashioned. Use of prostheticmesh, although common in repair of classic congenitaldiaphragmatic hernia, is not reported in intrapericardialdiaphragmatic hernia but can be tried when conventionalmeasures fail.

To conclude, intrapericardial diaphragmatic hernia is anextremely rare condition and should be kept in thedifferential diagnosis of anterior diaphragmatic hernia andfetal or neonatal massive pericardial effusion. Surgical repairis the treatment of choice.

E31Intrapericardial diaphragmatic hernia

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