Ann Thorac Surg 2007;84:638-640
© 2007 The Society of Thoracic Surgeons
Case Reports
Asymptomatic Congenital Intrapericardial Diaphragmatic Hernia and Epigastric Hernia in the Adult
Gaetano La Greca, MD, PhDa,*,
Maria Sofia, MDa,
Valentina Randazzo, MDa,
Francesco Barbagallo, MDa,
Rosario Lombardo, MDa,
Pierfranco Soma, MDb,
Domenico Russello, MDa
a Department of Surgical Sciences, Organ Transplantation, and Advanced Technologies, University of Catania, Cannizzaro Hospital, Catania, Italy
b Plastic Surgery Unit, Cannizzaro Hospital, Catania, Italy
Accepted for publication March 19, 2007.
* Address correspondence to Dr La Greca, via Messina, 354, Catania, 95126, Italy. (Email: glagreca{at}unict.it).
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Abstract
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The congenital intrapericardial hernia is a rare kind of diaphragmatic hernia. It is due to an embryologic defect of the central tendon of the diaphragm, often accompanied by other congenital malformations. This work presents a unique case report in the literature of the congenital association between intrapericardial diaphragmatic hernia and epigastric hernia in an adult woman. In spite of herniation of the colon and omentum the patient was completely asymptomatic, requesting surgery for an epigastric hernia for aesthetic reasons. The defect of the diaphragm was sutured and the abdominal wall was repaired with a prosthetic mesh.
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Introduction
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Congenital defects of the pericardium are uncommon with an estimated incidence of 1 to 2 cases per 13,000 [1]. So the congenital intrapericardial diaphragmatic hernia is rare and it can be associated with other anomalies such as septal defects, valvular malformations, and omphalocele. In newborns this hernia is highly symptomatic, and in adults the symptoms are variable to absent. We present this case, unique in the reported literature of the congenital association between intrapericardial diaphragmatic hernia and epigastric hernia in a completely asymptomatic adult.
The patient is a 68-year-old woman who was admitted to our department for a voluminous epigastric hernia. The hernia was present at birth, it was initially small, and it increased in size during the years. The patient’s family history and medical history are unremarkable. Her parents, living in the country, refused surgery when she was young, and afterward she did not request any treatment. The patient was asymptomatic and in good health when she requested surgery for aesthetic reasons due to a cutaneous alteration on the skin covering the hernia (Fig 1). Preoperative assessment included a chest roentgenogram and computed tomography. The first showed an anterior diaphragmatic hernia, Morgagni’s hernia, with large bowel herniated in the right hemithorax. Computer tomography confirmed the anterior diaphragmatic hernia and the epigastric hernia (Fig 2). Preoperative respiratory assessment (including spirometry) was performed, achieving normal values. Surgery was planned and the laparoscopic approach was chosen for aesthetic reasons and minimally invasive technique, even if the epigastric skin needed to be resected. After a difficult viscerolysis of the diaphragmatic hernia contents, a laparotomy was performed. An ovalar 6 x 4 cm defect in the anterior portion of the pericardial diaphragm was identified, which constituted a peritoneal-pericardial communication (Fig 3). The heart was directly exposed after reduction of the hernia contents, omentum, and colon. The hernia had no sac and there were no adhesions to the heart, ascending aorta, or main pulmonary artery. The difficulties during laparoscopy were due to an adhesion between the omentum and the internal edge of the diaphragmatic defect. The defect had thickened edges and was limited only to the pericardial diaphragm without pleural involvement. It was primarily closed with interrupted absorbable sutures, positioning an endopericardic drainage. The defect of the epigastric abdominal wall was repaired with a double layer polyester mesh with hydro-soluble film. The cutaneous defect was repaired through a "Z" plastic technique covering the mesh. The endopericardic drainage was removed on postoperative day 4 after a negative cardiac ultrasonography, and the patient was discharged a week after surgery. The postoperative course was uneventful and there was no recurrence after the 6-month follow-up.
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Fig 2. (a, b) Thorax computed tomographic (CT) scan showing the colon (black arrows) inside the intrapericardial diaphragmatic hernia near the heart wall (open arrows). (c) Slide of the abdominal CT scan at the level of the epigastric hernia with intestinal contents (white arrow).
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Comment
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The intrapericardial diaphragmatic hernia is a very rare event in which peritoneal and pericardial cavities are in direct communication through a defect in the diaphragm. During weeks 4 to 8 of the embryologic period, the diaphragm develops by fusion of four structures: (1) the pleuroperitoneal membranes, (2) the septum transversum, (3) the dorsal mesentery of the esophagus, and (4) the lateral body walls. An early embryologic accident produces Cantrell’s pentalogy, whereas later embryologic errors produce omphalocele, pleuropericardial and peritoneopericardial defects in any combination [2]. Intrapericardial hernia is the rarest of these congenital defects, frequently diagnosed at birth or in infancy due to respiratory and cardiac symptoms. In these patients the defect is often joined with other congenital defects, especially cardiac or abdominal wall malformations like omphalocele [3], but never in association with epigastric hernia. The epigastric hernia of our patient could be considered a mild form of omphalocele, because the skin (despite being very thin) is still represented, and the patient had no umbilicus. Herniation in the pericardial cavity has been reported to produce cardiac compression, tamponade, respiratory distress, and pericardial effusion, and also symptoms related to complications of the herniated gut [4]. However, some patients remain asymptomatic for many years, and the diagnosis of a diaphragmatic hernia is performed incidentally by radiography or by identification of the intestinal sound within the chest [5]. The omentum is usually the first to pass through the opening in the diaphragm, followed by colon, small bowel, liver, or stomach. The pericardium of our patient contained the transverse colon and omentum since birth, despite this she did not have any abdominal symptoms related to colonic abnormal position or cardiac symptoms related to myocardial irritation like arrhythmia. It is certainly significant to point out that this case of intrapericardial diaphragmatic hernia in adulthood was due to the refusal of proposed surgical treatment in childhood, and this incredible patient’s acceptance of an abnormal aesthetic of her abdomen. Concerning diagnosis, a computed tomographic scan is the best mean to identify and characterize the diaphragmatic hernia, even if it is very difficult to identify the thin leaf of pericardium around the hernia. So in our case, the computed tomographic scan misdiagnosed the hernia as an anterior diaphragmatic hernia, or Morgagni’s hernia. Repair of the hernia can be performed with either a thoracic or an abdominal approach, but the latter is the best choice. One case is reported to have been repaired by laparoscopic approach [4]. This approach helped us in the first part of the surgical treatment in which the communication between the peritoneal and pericardial cavity was recognized. Unfortunately, we could not finish the surgical repair by laparoscopy due to the difficulties in reducing the hernia contents into the abdomen. The pericardial diaphragm can often be satisfactorily sutured primarily, but mesh, pericardium, or fascia lata have been occasionally used [2]. We believe that this case represents the first reported in the literature of the association between a congenital intrapericardial diaphragmatic hernia and congenital epigastric hernia in adult. When identified, these defects can be easily repaired without complications.
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References
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- Peter SD, Shah SR, Little DC, et al. Bilateral congenital diaphragmatic hernia with absent pleura and pericardium Birth Defects Res A Clin Mol Teratol 2005;73:624-627.[Medline]
- Meng R, Straus A, Milloy F, et al. Intrapericardial diaphragmatic hernia in adults Ann Surg 1979;189:359-366.[Medline]
- Di Stefano M, Giacomoni MA, Cucchi L, et al. Congenital intrapericardial diaphragmatic hernia associated with an omphalocele in a newborn infant Pediatr Med Chir 1986;8:131-133.[Medline]
- Paci M, de Franco S, Della Valle E, et al. Septum transversum diaphragmatic hernia in an adult J Thorac Cardiovasc Surg 2005;129:444-445.[Free Full Text]
- Sariosmanoglu N, Hazan E, Metin K, et al. Intrapericardial diaphragmatic hernia and atrial septal defect in adults J Thorac Cardiovasc Surg 2002;123:353-354.[Free Full Text]
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Abstract
Introduction
