EXPERIMENTAL AND CLINICAL TRANSPLANTATION

Objectives: Diaphragmatic hernia is a rare complication after pediatric liver transplant. This report presents occurrences of diaphragmatic hernia after living-donor liver transplants in 2 children.

Material and Methods: In 1 of the 2 patients, a right-sided diaphragmatic hernia developed after a living-donor liver transplant due to progressive familial intrahepatic cholestasis where a left lateral segment graft was used. In the other patient, a left-sided diaphragmatic hernia developed after a living-donor liver transplant due to biliary atresia following Kasai portoenterostomy where a left lateral segment graft was used.

Results: After diaphragm repair, the postoperative course was uneventful and there were no recurrences.

Conclusions: A literature review identified nearly 30 cases of diaphragmatic hernia following liver transplants; diaphragmatic hernia should be considered a potential surgical complication after liver transplant.

Key words : Liver transplant, Diaphragmatic hernia, Children

Today, liver transplant is the only treatment option for end-stage liver failure. Although patient survival and graft success have increased significantly with the progression of immunosuppressive and surgical techniques, correction of intensive-care conditions, and multidisciplinary approaches, posttransplant complications continue.¹ Despite the fact that diaphragm injury can occur during liver transplants, formation of a diaphragmatic hernia is rare.² In this study, we present 2 pediatric diaphragmatic-hernia cases that occurred among 199 overall pediatric liver transplant cases undertaken in our center between 2006 and 2014.

Materials and Methods

Case 1
An 8-year-old girl weighing 16 kilograms came to our center due to growth retardation and jaundice. The pediatric end-stage liver disease score was 9. The graft-to-recipient weight ratio was 2%. Progressive familial intrahepatic cholestasis was diagnosed. A left lateral segment living-donor liver transplant was performed on the patient. Her postoperative course was uneventful, and after 12 days she was discharged home. The patient returned to our center after 46 days due to a high fever and vomiting. A chest radiograph showed bowel loops in the thorax. An emergency laparotomy was completed, resulting in a diagnosis of a right-sided diaphragmatic hernia. During the operation, necrosis in the ileal segment and a 3 × 3 centimeter defect to the right diaphragm was detected. A resection and anastomosis were performed. The defect in the diaphragm was repaired using primer nonabsorbable Prolene sutures. The patient was discharged from hospital uneventfully on the sixth day after surgery (Figure 1).

Case 2
A 3.5-year-old girl weighing 8 kilograms came to our center with a history of Kasai portoenterostomy performed due to extrahepatic biliary atresia. The pediatric end-stage liver disease score was 22. Her graft-recipient weight ratio was 3%. A left lateral segment living-donor liver transplant was performed on the patient. Her postoperative course was uneventful, and after 14 days she was discharged home. The patient returned to our center with abdominal pain 7 years after discharge. The results of laboratory tests were normal, but a chest radiograph showed a distended stomach extending through the left hemithorax. A computed tomography scan was performed on the patient because of suspicion of eventration and concern that the stomach was too close to the thorax (Figure 2). A thoracotomy was performed on the patient, with a reduction to the stomach. A repair of a 4 × 4 centimeter defect in the diaphragm also was completed using primer nonabsorbable Prolene sutures. The patient was discharged from hospital uneventfully on the seventh day.

Diaphragmatic hernias can be congenital, traumatic, or iatrogenic.³ Diaphragmatic pathologies such as dysfunction and paralysis are seen in 8% to 15% of cases after pediatric liver transplant.^4,5Diaphragmatic hernias are rare after liver transplant, being seen in 1.8% of 166 cases examined by Shigeta and associates6 and 2.8% in 142 patient series in Moon and associates.² In our 199 pediatric cases, we noted the ratio of diaphragmatic hernias occurring at a rate 1% less than in the other literature. With this ratio, in our clinical approaches, we think that with awareness of potential diaphragmatic-hernia emergence, careful dissection and bleeding control will be effective in reducing diaphragmatic hernia in pediatric cases.

Several reasons have been proposed for diaphragmatic hernia emerging after liver transplants, including the pressure gradient between the chest and abdominal cavity; use of the left lateral segment (right colon and small bowel that migrate into the right-upper quadrant); the thin muscular layer of diaphragm adhering during surgery or injury to the diaphragm due to the heat effect during bleeding control; immunosuppressive regimens begun after transplant; delays in recovery based on diaphragm movements; and delays in wound healing for patients with malnutrition.^1,2 Both of our 2 patients were malnourished and had growth retardation, and left lateral segments were used for both, with the defect localized in each in the posterior medial region. In 1 of the patients, an operation previously performed due to biliary atresia was taken into account. Tacrolimus and steroids were used for both patients as immunosuppressives after transplant. The combination of these factors can explain the emergence of diaphragmatic hernia.

In a diaphragmatic hernia, symptoms include vomiting, abdominal pain, and general lung problems.^1,7 Vomiting and abdominal pain were present in 1 of our patients, and the other was having occasional nonspecific abdominal pain. Several diagnostic methods were used in the diaphragmatic hernia identification, including chest radiographs and computed tomography scans.³ The computed tomography scans were performed to expand on chest radiograph results, and to assess the patients’ livers, and eliminate other accompanying pathologies. Although diaphragmatic hernia was indicated in the right side, cases in the left region and bilateral also have been reported.⁸ In children, showing only left-sided diaphragmatic hernia is rare after transplants.

In pediatric cases, treatment options include laparoscopic, thoracoscopic, or open surgical techniques via an abdominal or thoracic approach. In a study, recurrence rates of 7.9% in endoscopic procedures and 2.6% in open surgery procedures were observed. The reason given for these rates was technical difficulties in liberalizing the posterior muscular diaphragmatic rim.⁹We chose laparotomy for 1 of our patients due to sensitivity in their abdomen, and we chose thoracotomy for the other due to the possibility of adhesions and the position of the liver. Each of these 2 cases made use of primer nonabsorbable Prolene sutures.

Despite these results, larger studies are needed to understand the progress and pathophysiology of diaphragmatic hernia. However, attention paid to bleeding control and reduction in adherences in the diaphragm through reduced-trauma dissections will decrease the emergence of diaphragmatic hernia, especially in malnutrition cases.

Volume: 16 Issue: 3 June 2018

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