Injury Extra 41 (2010) 65–67
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Case report
Congenital double diaphragm initially diagnosed as traumatic ruptured diaphragmatic hernia Hooman Yarmohammadi a,*, Alireza Bakhshaeekia b, Leila Ghahramani b, Hamid Reza Abbasi b, Shahram Bolandparvaz b a b
Department of Surgery, University Hospital of Pennsylvania, 3400 Spruce Street, 4 Maloney, Philadelphia, PA 19104, USA Department of Surgery, Faghihi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
A R T I C L E I N F O
Article history: Accepted 16 March 2010
1. Case report An 11-year old boy, a victim of penetrating stab wound to his chest, presented with severe respiratory distress and dyspnoea. The knife had entered the chest at the right mid-axillary line, at the level of 7th–8th ribs. He was initially diagnosed of having a right sided pneumothorax and therefore primary resuscitation was performed and a right sided chest tube was inserted. However patient’s severe shortness of breath did not improve. His respiratory rate was 40/min, blood pressure 90/60 mm Hg, and pulse rate was 140/min. Physical examination demonstrated a small (1 cm) laceration on the right chest at the level of 7th intercostal space just below the tip of the scapula. Patient’s chest X-ray revealed intestinal gas above the diaphragm with chest tubes in the right and left lungs (Fig. 1). The patient was hypoxic (PO2 78.8 mm Hg, PCO2 31.5 mm Hg, pH 7.46, HCO3 21.8 mmol/L and O2 saturation of 96.2%), in spite of 10 L of O2. Base on his chest X-ray findings and his unstable clinical features, he was emergently taken to the operating room (OR) with possible diagnosis of traumatic diaphragmatic hernia. During the operation, a double diaphragm with a large central defect was diagnosed. Hernia repair surgery was done and he was taken to the intensive care unit. His clinical condition progressively improved. He was discharged from the hospital 10 days post-surgery.
2. Surgical technique The abdomen was entered through a midline laparotomy incision. After initial exploration we observed that the diaphragm
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has two muscular layers (Fig. 2). The first layer was attached to the lung and chest wall in its normal anatomical position. The liver and spleen were positioned under the domes of the diaphragm in the right and left side, respectively. The second layer or the lower layer originated from the chest wall in the periphery and formed a large circular muscular layer with a large defect (20 cm 20 cm) in the middle. On the right side, these two muscular diaphragmatic layers were separated by the liver and by the spleen on the left side. A 7– 8 cm distance was observed between the two layers. The stomach had herniated through the central defect of the lower diaphragmatic layer. The stomach had enlarged with gas entrapped due to the patient’s hyperventilation and was pushing on the left lung. Schematic diagram of this anatomical variation is presented in Fig. 3. This defect partially resembled a large Bochdalek hernia; however, unlike Bochdalek hernia the intestinal content had not herniated into the chest, and the chest content was intact. Since the right lower diaphragm was firmly attached to the liver, and only a small potential space was seen it seemed that it would not cause any future problems and no surgical intervention was performed. On the left side the stomach and small bowel had herniated through the defect and after expanding had caused respiratory problems. We hypothesized that by opening the left lower diaphragm and expanding the size of the defect the possibility of strangulation and other future problems would be eliminated. Therefore, the left lower layer just below the spleen was opened on its transverse axis. The incision was 5 cm long (Fig. 4A). The defect caused by the transverse incision was then stretched out and the two layers were approximated and sutured along their longitudinal axis (Fig. 4B). The technique used was similar to the technique which is used for treating stenosis in any structure e.g. spatulating in arterial stenosis, patients with pyloric stenosis, etc. In this manner the lower layer of the congenital diaphragm expanded and the cleft-like appearance was eliminated. A mesh was placed in between in order to provide more support and sutured to the reconstructed diaphragmatic layers. 3. Discussion Various types of congenital defects have been described for the diaphragm2,4,5. Morgagni–Larrey hernias, Bochdalek hernia, diaphragmatic dysontogenetic cyst, congenital sliding and rolling
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Fig. 1. Chest X-ray of the patient on arrival to the urban first-aid centre. The two layers of diaphragm are marked on the left side and are seen separated by the gas of the stomach (arrow) and small bowel, herniated from the defect in the first layer of diaphragm.
Fig. 2. The two layer of diaphragm with the stomach evacuated from the space between them. The first layer (upper arrow) is attached to the lower surface of the lung and the second layer (lower arrow) is below the spleen on the left side.
Fig. 3. Schematic diagram of the anatomical variation of the diaphragm.
hiatal hernia have been previously reported as congenital problems affecting the diaphragm4. In this study, we presented a case of congenital double diaphragm, a rare entity which to our best knowledge is being reported for the first time. The patient’s history, physical examinations and clinical evaluations were in favour of a traumatic ruptured diaphragm2,5. However, gross evaluation at the time of operation revealed a normal diaphragm along with another layer of diaphragm with a defect very similar to Morgagni–Larrey hernia3; however, unlike Morgagni–Larrey, in which the intestinal content herniate into the thoracic cavity, in our case, the intestinal content and most prominently the stomach had herniated through the defect in the lower diaphragmatic layer between the two layers of the diaphragm. Morgagni hernia is a congenital diaphragmatic hernia which occurs through anteromedial defect in the diaphragm. In our patient the defect was centrally located. Meticulous physical examination and radiological evaluations are essential in order to detect these injuries2,4,5. High-resolution CT scan of the chest is the best method of evaluating these defects, however in emergency situations it may not be practical3. Traumatic diaphragmatic hernias may be operated both by laparoscopic or open surgery. Laparoscopic surgery is usually used in patients that have had no symptom or mild symptoms4. Anterolateral thoracotomy, reduction of hernia with repair of the
Fig. 4. (A) Transverse incision made on the first layer of diaphragm (on the left). (B) The two layers of diaphragm sutured towards each other, obliterating the space between them and the mesh used to facilitate, the attachment (on the right).
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foramen of Morgagni with mesh (prolene) has been the most common method of operating these defects. Our patient had become anxious; most likely due to the trauma and pain and had hyperventilated. Conclusively, he had developed gastric hyperaeration and distension. The gastric distension along with the pneumothorax in the right side had most probably caused the symptoms of the patient. The patient was initially managed by inserting a right sided chest tube. However, the patient had not responded to this treatment. In our centre, due to patient’s CXR we thought the patient has ruptured diaphragm and the management for traumatic diaphragmatic rupture was applied1,2. Surgical approach should be individualized. Our case is an example of the vast variations in which a surgeon may confront. It is an emphasis on that although surgical experience and skill is an important requirement, clinical judgment, knowledge of regional anatomy and common sense is also essential.
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Acknowledgement The schematic picture was drawn by Dr A.R. Bakhshaeekia.
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