Hernia (2006) 10:525–527 DOI 10.1007/s10029-006-0133-z
C AS E RE PO RT
Bilateral congenital anterior diaphragmatic hernia: a case report A. Ninos · St. Pierrakakis · V. Stavrianos · G. Papaioanou · A. Ajiazi · Ch. Iordanou · P. Vagenas · M. Vidali · G. Douridas · N. Setakis
Received: 10 April 2006 / Accepted: 28 July 2006 / Published online: 5 September 2006 © Springer-Verlag 2006
Abstract The anteromedial diaphragmatic hernias of Morgagni and Larrey are the least common defects that occur in this organ. Simultaneous occurrence has been described but remains an absolute rarity. We herein present a case of this bilateral herniation with a unique presentation. The paper refers to the basic anatomic, diagnostic and therapeutic considerations for this entity. Keywords
Morgagni · Larrey · Diaphragmatic hernia
Introduction Anterolateral diaphragmatic hernias comprise the 3% of all the defects of this musculotendinous organ [1]. This congenital anomaly is rarely observed and usually identiWed incidentally due to the lack of symptoms [2]. A case of a symptomatic hernia of the Morgagni– Larrey type is presented and its aetiopathogenic aspects together with its diagnostic procedures and its clinical pattern are discussed, because of the absolute rarity of this bilateral defect. Surgical repair is considered the gold standard of treatment [3] for such symptomatic or incidental Wndings. A. Ninos (&) · St. Pierrakakis · G. Papaioanou · A. Ajiazi · Ch. Iordanou · P. Vagenas · M. Vidali · G. Douridas · N. Setakis Surgical Department, Thriasion General Hospital, G. Genimatas Av., 19200 Magula, Athens, Greece e-mail:
[email protected] V. Stavrianos Radiological Department, Thriasion General Hospital, Athens, Greece
Report of a case A 49-year-old male was referred by his general practitioner to the outpatient department of our unit for a surgical consultation. The patient had a history of a recent–onset hypertension. He also referred to some episodes of shortness of breath without any evidence of a provocative factor whatsoever together with nonspeciWc epigastric complaints. During his initial workup, a chest X-ray revealed a huge diaphragmatic hernia with the presence of colon dislocation in the left hemithorax. Abdominal X-rays were unremarkable. Computed tomography (Fig. 1) revealed a large segment of left colon herniating into the left hemithorax, causing a partial compression of the lung. Bilateral diaphragmatic defects were recognized parasternally. During an exploratory laparotomy, we found a bilateral Morgagni–Larrey hernia without a sac. The intraoperative Wndings were compatible with the CT Wndings. Nearly half of the transverse together with the splenic Xexure and a segment of the descending colon were found in the left hemithorax. There was also epiploic fat herniating through the right oriWce anteriorily to the heart. We reduced the herniated organs back into the abdominal cavity, closed the hernia oriWces with separate stitches and reinforced them with an onlay PTFE dual mesh. His postoperative course had no complications and he was discharged on the Wfth day. He remained asymptomatic in his ten-month followup. A remarkable reduction of his systolic hypertension has also been noticed.
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Fig. 1 A CT reconstruction showing the herniating colon and the bilateral diaphragmatic defects
Discussion Two small gaps of various sizes are closed with connective tissue bilaterally between the sternal and costal parts of the muscular part of the diaphragm. These gaps are the right and left sternocostal triangles of Morgagni and Larrey, respectively. The superior epigastric vessels pass through these openings. These are the sites of the homonymous hernias [4]. The anterior diaphragmatic hernia is usually a unilateral congenital defect that occurs on the right in 90% of such cases. It comprises 2–6% of all the congenital diaphragmatic hernia cases [5]. Although a sac is usually present at Wrst, it may later rupture, leaving no trace. During infancy it usually contains liver, but later, in adulthood, colon, omentum or stomach may be found [6]. Both an acquired and a congenital mechanism have been postulated for the development of these defects. According to the Wrst, episodes of vomiting and cough could increase the intraabdominal pressure to such an extent that could provoke the defect. According to the latter, partial agenesis of the costal or sternal part together with insertion alterations could be responsible for the development of these hernias [7]. Morgagni–Larrey hernia is an extremely rare occurrence. It is characterized by protrusion of abdominal organs into the thorax bilaterally. In the great majority of parasternal hernias, no symptoms occur that could reasonably be related to them. When present, the symptoms are predominantly gastrointestinal [8]. In our patient the majority of complaints were of intrathoracic origin. This is the Wrst case
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in the English literature where a hypertension problem has been related to herniation. The diagnosis is easily accomplished by radiographs and computed tomographic scan of the thorax and abdomen. Anterior–posterior Wlms show a mass or gas–Xuid levels in the right or left cardiophrenic angle while lateral ones substantiate the anterior location of the herniation [9]. CT is a useful diagnostic method in patients with solid organ herniation. Magnetic resonance imaging is the best diagnostic tool when mediastinal tumors are being considered and when the mass demonstates fat density on CT [10]. As in the majority of congenital diaphragmatic hernias, the gold standard of management is prompt surgical repair through an abdominal [11] or thoracic [12] approach. The advantages of the abdominal approach [13] in patients with bilateral foraminal hernias are obvious, especially in cases where another intraabdominal disease also requires attention. Furthermore, the visceral contents of the sac can be more readily restored to their normal abdominal position by pulling them down from below than by pushing them from above. The defect is closed by direct tissue approximation or by using prosthesis, especially in cases of diaphragmatic aplasia and very large defects. Many allogenic and autologous materials have been used but there is no systematic study comparing these prostheses. Polypropylene [14] and polytetraXuoroethylene [15] are the most commonly used materials. In recent years, double-layer meshes [16] have become available that can be placed in direct contact with the abdominal viscera, allowing the defect to be closed safely and without
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tension. The size of the mesh, which must overlay the edges of the oriWce by 5 cm [17], is tailored to the defect and is stitched in place using the same material. In cases where a primary repair is feasible, a nonabsorbable suture [18, 19] in an interrupted or continuous technique is preferred. Some suggest the reinforcement of a direct repair with an overlying mesh, as we did in our case too [20]. In selected cases a laparoscopic approach could be successful. This method is usually favoured in adults, especially in nonacute cases [21]. The laparoscopic approach can also be used in the paediatric age group without using a prosthesis [22]. Video-assisted thoracic repair has also been reported [23] to be a safe and eVective method of Wxing a Morgagni hernia. The mesh can be Wxed in place by using a hernia stapler, a helical fastener or an endostich. The surgical outcome obtained by using either of these techniques is the same [24].
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