Two cases of spontaneous rupture of an umbilical hernia, a rare complication of portal hypertension

Portal hypertension is a severe complication of liver cirrhosis frequently leading to formation of ascites. We describe two patients that presented with a spontaneous rupture of an umbilical hernia, a rare complication of liver cirrhosis. Umbilical hernia itself however is a common complication of portal hypertension occurring in about 20% of the patients. In general, umbilical hernias in patients with liver cirrhosis warrant elective surgical repair, in a center of expertise with liver cirrhosis, after optimal management of ascites. Case Report Two cases of spontaneous rupture of an umbilical hernia, a rare complication of portal hypertension Peter R Oosterwijk1*, Eva Kouw2 and Wouter H. de Vos tot Nederveen Cappel3 1Peter R Oosterwijk, Gastroenterology trainee, Institution Isala hospital Zwolle, Netherlands 2Eva Kouw, gastroenterologist, Institution, Gelre hospital Apeldoorn, Netherlands 3Gastroenterologist Institution, Isala hospital Zwolle, Netherlands Dates: Received: 29 July, 2017; Accepted: 24 August, 2017; Published: 28 August, 2017 *Corresponding authors: Peter R Oosterwijk, Gastroenterology trainee, Institution Isala hospital Zwolle, Netherlands, E-mail:


Introduction
Portal hypertension is a haemodynamic abnormality and one of the most severe complications of liver cirrhosis which include ascites, hepatic encephalopathy and bleeding from gastro esophageal varices [1]. We describe a rare complication of portal hypertension in two patients with alcoholic liver cirrhosis and discuss the treatment strategies to prevent this complication.

Case Reports
Patient A, a 63-year-old male patient, was diagnosed in 2009 with alcoholic liver cirrhosis and an umbilical hernia.
His further medical history included diabetes mellitus with micro-and macrovascular complications and obesity. He presented at our emergency department after he noticed a massive fl uid leak from his umbilicus. Prior to presentation he had suffered from abdominal pain and an umbilical mass for three weeks. On physical examination we saw an alert patient, haemodynamically and respiratory stable, without fever or jaundice. The abdomen was distended by an enormous mass of ascites and a very large reducible umbilical hernia with skin necrosis and leaking ascites ( Figure 1). Laboratory values at admission are shown in the Table 1.
The Child Pugh classifi cation was grade B (score 8) and the MELD-score 9. Ascites white blood count was 0.9 x 10 9 /L, suggestive of infected ascitic fl uid.  After two months of hospitalisation, the patient was discharged for further rehabilitation. One year later the patient was admitted again, now with refractory ascites and encephalopathy, but without signs or symptoms of a recurrrent hernia, both on clinical and ultrasound examination. Patient B, a 52-year old male was diagnosed in 2012 with alcoholic liver cirrhosis. During the past year he developed ascites and an umbilical hernia. He was referred from a regional hospital to our centre because of multiple liters of ascites which had leaked from a spontaneous rupture of his umbilical hernia.
Prior to referral he actively abused alcohol and had no physical complaints.
On physical examination we saw an alert patient, haemodynamically and respiratory stable, without fever or jaundice. The abdomen was not distended and showed an umbilical hernia with a small ulcer of 5 mm, no longer leaking ascitic fl uid. The Child Pugh classifi cation was grade A (score 6) and the MELD-score 7. Diuretics and cefotaxim were already started at the regional center and were continued. There were no signs of infected ascites.
A CT-scan of the abdomen was made which showed a cirrhotic liver, ascites and esophageal varices, but no signs of a patent umbilical vein. Several days later operation followed. The hernial sac was excised and abdominal drainage of ascites took place. The defect had a diameter of 1 centimeter. The peritoneum was closed with PDS sutures. The hernial defect was closed with a 6 x 6 cm soft polypropylene sublay mesh.
The patient recovered quickly from surgery. He was discharged from the hospital after a week when a stable situation was reached with diuretic therapy.

Discussion
Spontaneous rupture of an umbilical hernia with massive ascitic leakage is a rare complication of portal hypertension in liver cirrhosis [2][3][4][5][6]. However, umbilical hernia itself is a common complication of portal hypertension occurring in about 20% of the patients [7]. Any surgical intervention in patients with advanced liver disease has a high risk of complications. Therefore timing of umbilical hernia repair in cirrhotic patients with ascites is diffi cult. Umbilical hernia repair in patients with a patent umbilical vein is contraindicated. Repair of an umbilical hernia necessitates the complete freeing of the umbilical ring and the ligation of the reopened umbilical vein. If the vein is ligated during umbilical hernia repair, the outfl ow of the portal circulation is hampered leading to acute portal vein thrombosis and subsequent acute liver failure necessitating emergency liver transplantation [7,8].
Although bowel incarceration is reported as a rare complication in this specifi c patient group, mortality rates are reported as up to 30% when emergency surgery for incarceration or strangulation is necessary [9][10][11]. Therefore, in general, umbilical hernias in patients with ascites warrant elective surgical repair, in a center of expertise with liver cirrhosis, after optimal management of ascites. Even for patients on a waiting list for liver transplantation elective surgery should be considered due to a long waiting time [7,8,12]. In our cirrhotic patients with an umbilical hernia, and without an open umbilical vein, elective hernia repair would have prevented spontaneous rupture of hernias. We presented two cases which confi rm the need for correction of an umbilical hernia, preferrably in a stable clinical situation.