Hiatal Hernia with Prolapse of the Pancreas Causing Bile Duct Stricture and Liver Function Disorders: A Case Report and Literature Review

Abstract

Hiatal hernia is a common condition in elderly patients, but the additional presence of prolapse of the pancreas is extremely rare. We herein report an 89-year-old woman who presented with liver function disorders and abdominal pain. Her laboratory tests revealed cholestasis, and imaging examinations showed stenosis of the common bile duct pulled toward the hernia sac. She was diagnosed with a common bile duct stricture due to pancreatic herniation and underwent laparoscopic surgery. Our review of the literature identified three types of pancreatic herniations: asymptomatic, bile duct complication, and acute pancreatitis. Pancreatic head herniation tends to induce bile duct complications.

Introduction

Esophageal hiatal hernia is a prevalent condition in elderly patients; however, rare complications secondary to hiatal hernia have been increasingly reported in clinical practice. Among the many types of herniation, a pancreatic component that prolapses with the stomach into the mediastinum is one of the rarest. Pancreatic herniation can be asymptomatic, but it sometimes causes complications, such as jaundice, liver function disorders, and acute pancreatitis. In addition, clinical decisions about indications for surgery are always complicated and challenging because these patients are usually elderly and have many comorbidities.

We herein report a rare case of an 89-year-old woman who presented with hepatic dysfunction due to common bile duct stricture accompanied by pancreatic herniation. In addition, we reviewed the English literature on pancreatic herniation with hiatal hernia and discuss clinical types of this condition and their features.

Case Report

An 89-year-old woman was referred to our hospital with liver function abnormalities. She had noted dull right upper quadrant abdominal pain for three months, but she denied nausea, vomiting, hematemesis, and melena. Her medical history included gastroesophageal reflux disease, hypertension, dyslipidemia, osteoporosis, and an overactive bladder. She had been taking vonoprazan, valsartan, pravastatin, mirabegron, rebamipide, ascorbic acid, and calcium pantothenate. She did not have a habit of consuming tobacco or alcohol.

She was afebrile with blood pressure of 166/79 mmHg. A physical examination showed neither anemia nor jaundice. There was no abdominal tenderness. The laboratory test results revealed liver function abnormalities as follows: total bilirubin, 1.0 mg/dL; aspartate aminotransferase (AST), 100 U/L; alanine aminotransferase (ALT), 132 U/L; alkaline phosphatase (ALP), 717 U/L; and gamma glutamyl transferase (GGT), 1,146 U/L. Other blood chemistry data and tumor markers were within the normal range.

Ultrasonography detected dilatation of the intrahepatic and extrahepatic bile ducts, and the common bile duct was running cephalad (Fig. 1A). Computed tomography (CT) of the chest and abdomen showed a giant hiatal hernia involving the entire stomach, part of the transverse colon, the duodenum, and the entire pancreas in the hernial sac in the mediastinum (Fig. 1B-F). The extrahepatic bile duct was dilated, pulled up toward the hernia sac, and occluded at the level of the hernial orifice in the shape of a beak (Fig. 1C, yellow arrow). The intrahepatic bile duct was also dilated, and the gallbladder was moderately distended. Consecutively, CT with drip-infusion cholangiography was performed, demonstrating the dilated intra- and extrahepatic bile duct and stenosis of the common bile duct, which was pulled toward the hernia sac and deformed (Fig. 2). There was no choledocholithiasis or cholangiocarcinoma. An upper gastrointestinal examination revealed that the whole upside-down stomach and part of the duodenum were located in the mediastinum above the diaphragm (Fig. 3A).

Figure 1.

Ultrasonography (US) and coronal sections of computed tomography (CT) of the abdomen. A (US): The intrahepatic bile duct is dilated and the extrahepatic bile duct is running cephalad. B-F (CT, coronal section): The pancreas, the duodenum, and the transverse colon are herniated in the mediastinum along with the whole stomach. The common bile duct and the portal vein are narrowing at the level of the hernial orifice (C, yellow arrow). P: pancreas, D: duodenum, S: stomach, C: transverse colon

Figure 2.

Three-dimensional reconstruction of computed tomography (CT) in drip-infusion cholangiography (DIC-CT) from the posterior view of the coronal section. The dilated extrahepatic bile duct is pulled toward the thorax and the intrahepatic bile ducts are also extended.

Figure 3.

Upper gastrointestinal examinations (UGE). A: Preoperative UGE showed the whole upside-down stomach and half of the duodenum are located in the mediastinum. B: Postoperative UGE confirmed the normal position of the stomach and duodenum.

Based on these findings, she was diagnosed with a type IV hiatal hernia with herniation of the colon, duodenum, and entire pancreas, resulting in stricture of the common bile duct.

Since the patient had liver function abnormalities and mild symptoms, one month later, she underwent elective laparoscopic surgery with mesh-augmented hiatal repair. During the surgery, the herniated organs were returned to the abdominal cavity, and the hernial orifice, which was enlarged by 5 cm in width, was closed with 3 sutures and a piece of mesh (Hiatus Mesh, 8.0 cm×8.0 cm, Parietex; Medtronic, Minneapolis, USA) in front of the orifice. After the surgery, her stomach and duodenum were located in the abdomen, as confirmed by a fluoroscopic examination with gastrografin contrast (Fig. 3B), and the liver function test findings recovered to their normal ranges throughout her hospitalization. She was discharged home one month after the surgery.

Discussion

Hiatal hernia is a condition involving prolapse of the abdominal contents, most commonly the stomach, into the mediastinum through the diaphragmatic esophageal hiatus, and it is encountered often in daily clinical practice (1,2). Although the exact prevalence of hiatal hernia has been difficult to determine, Kim et al. reported that 9.9% of patients who routinely underwent chest computed tomography had this condition (3). Researchers have proposed that the causes of this disease include increased intraabdominal pressure due to obesity and age-related kyphosis, weakness of the diaphragmatic sphincter muscle, and enlargement of the hiatal orifice (1,2). Skeletal deformities, such as scoliosis and kyphosis, can distort the anatomy of the diaphragm and induce severe hiatal hernia. Accordingly, age, obesity, and female gender are considered to be the most common risk factors for developing this condition (3,4).

Hiatal hernias are roughly divided into sliding hernias (85-95%) and paraesophageal hernias (5-15%) and are further categorized into 4 types according to the spread and extent of herniation (2). Type I hiatal hernia corresponds to a sliding hernia, in which the distal esophagus and stomach prolapse into the posterior mediastinum. Narrowly defined paraesophageal hernia is a type II hiatal hernia. In this condition, the gastric fundus migrates into the mediastinum through the esophageal hiatus while the gastroesophageal junction remains in a normal position. Type III hiatal hernia has combined features of types I and II and is considered a “mixed type.” Both the gastroesophageal junction and the fundus of the stomach herniate through the esophageal hiatus, and a “giant hiatal hernia” sometimes forms. Type IV hiatal hernia involves herniation of the abdominal organs along with the stomach. Abdominal components, such as the colon, greater omentum, duodenum, small intestine, spleen, and pancreas have been reported as herniated organs in the thoracic cavity.

Most mild hiatal hernias are usually asymptomatic and are discovered accidentally. However, as gastroesophageal reflux develops, which is accompanied by the destruction of the lower esophageal sphincter function, patients with a hiatal hernia begin to complain of symptoms, such as heartburn and regurgitation. In contrast, a severe hiatal hernia sometimes causes symptoms, including chest pain, epigastric pain, postprandial fullness, nausea, and vomiting. Gastric volvulus, occlusion, ischemia, and perforation are extremely rare complications related to gastric herniation with a giant hiatal hernia. Even life-threatening conditions, such as cardiopulmonary arrest due to cardiac compression, have been reported (5).

In patients with a type IV hiatal hernia, herniation of the partial or complete pancreas is extremely rare. Although the pancreas is fixed to the retroperitoneum, stretching of the transverse mesocolon after displacement of the transverse colon into the hernia sac and loosening of the posterior adhering fascia of the pancreas can cause migration of the pancreas into the mediastinum (6-8). Patients with a pancreatic herniation may remain asymptomatic or can develop different symptoms, such as abdominal pain, abnormal liver function tests, jaundice, elevated levels of pancreatic enzymes, or pancreatitis.

We reviewed the English literature for cases of pancreatic herniation secondary to hiatal hernia and identified 28 cases (6-33). After adding our case, we summarized information about these 29 patients in Table. The median age was 74 (range: 24-102) years old, and women accounted for 20 of 29 cases (69%). In these patients, we noticed that there were some clinical types and categorized them into three types of pancreatic hernia according to their clinical features, laboratory data, and imaging examination findings (Table). To differentiate the three types, we emphasized objective data and image findings, since abdominal or chest pain only is neither objective nor sufficient for a diagnosis of bile duct obstruction or acute pancreatitis. The first type is pancreatic hernia without any objective abnormalities, i.e. asymptomatic herniation of the pancreas. These patients had neither an increased levels of biochemical factors (e.g., total bilirubin, alkaline phosphatase, amylase, and lipase) nor abnormal image findings (e.g., bile duct dilatation and peripancreatic effusion). The second type has clear evidence of cholestasis, defined as having either an elevated level of biochemical factors, such as total bilirubin and alkaline phosphatase, or bile duct dilatation on imaging modalities. Elevated levels of transaminases (i.e., aspartate aminotransferase, alanine aminotransferase) or gamma glutamyl transferase are usually present in these cases. The third type refers to acute pancreatitis due to pancreatic hernia. This category includes patients who have both abdominal symptoms and elevated pancreatic enzymes (i.e. amylase or lipase) with or without image findings that are specific to acute pancreatitis (i.e. peripancreatic fat stranding or fluid collection). Of note, five patients had the clinical features of both types 2 and 3 (8,15-18).

Table.

Cases of Esophageal Hiatal Hernia with Pancreatic Herniation.

type	Case (ref)	Age, sex	T. Bil. [mg/dL]	ALP* [U/L]	Bile duct dilatation	Amylase [U/L]	Lipase [U/L]	Peripancreatic effusion	Prolapsed part of the pancreas	Treatment
1	(28)	67 F	1.0	ND	-	ND	ND	-	h	Surgery (op)
	(29)	56 M	ND	ND	-	ND	ND	-	bt	Unknown
	(30)	74 M	ND	ND	-	ND	ND	-	bt	Unknown
	(31)	78 F	ND	ND	-	ND	ND	-	hbt	Surgery (op)
	(32)	75 F	ND	ND	-	99	ND	-	bt	Surgery (lp)
	(33)	61 F	ND	ND	-	ND	ND	-	b	Surgery (op)
2	(9)	77 F	3.0	416	+	ND	ND	-	h	Surgery (ND)
	(10)	64 M	1.5	849	+	ND	ND	-	h	Surgery (op)
	(11)	72 F	Normal	153	+	153	Normal	-	h	Surgery (op)
	(12)	83 F	20.0	660	+	ND	ND	-	ND	Surgery (op)
	(13)	59 F	9.2	751	+	ND	ND	-	ND	Surgery (lp)
	(14)	81 M	1.4	464	+	68	ND	-	h	Surgery (lp)
	Ours	89 F	1.0	717	+	81	ND	-	hbt	Surgery (lp)
2 & 3	(15)	80 F	Normal	3×ULN	-	7×ULN	40×ULN	-	hbt	Surgery (ND)
	(16)	70 F	1.8	ND	+	660	ND	-	bt	Observation
	(8)	79 F	0.9	254	+	ND	238	+	hbt	Observation
	(17)	90 F	ND	ND	+	890	3,500	+	bt	Observation
	(18)	86 F	2.0	258	+	2,805	ND	+	bt	Surgery (op)
3	(19)	71 F	1.0	ND	-	1,720	132	+	hb	Surgery (op)
	(6)	70 M	ND	ND	-	3×ULN	3×ULN	-	bt	Surgery (ND)
	(20)	78 F	ND	ND	-	1,543	787	+	bt	Observation
	(21)	68 M	ND	ND	-	284	171	-	bt	Surgery (lp)
	(22)	89 M	1.7	ND	-	5,256	ND	+	b	Observation
	(23)	61 F	ND	ND	-	408	ND	+	b	Surgery (op)
	(24)	88 M	ND	ND	-	273	1,968	-	bt	Observation
	(7)	65 F	ND	ND	-	ND	2,950	+	bt	Observation
	(25)	102 F	ND	ND	-	ND	1,719	+	bt	Observation
	(26)	24 F	ND	ND	-	ND	3,000	+	ND	Surgery (op)
	(27)	65 M	ND	ND	-	ND	2,687	-	bt	Observation

Since our case showed abnormal liver function tests and definite dilation of the bile duct, we categorized this patient as having type 2. Jaundice or liver function test abnormalities due to a hiatal hernia are extremely rare as a cause of biliary congestion, but some cases have been reported (Table, Type 2) (8-18). As seen in our patient, in most of these reported cases, total bilirubin or alkaline phosphatase was elevated remarkably, and the extrahepatic bile duct was dilated and pulled toward the hernia sac. Narrowing was seen in the middle of the biliary tract on imaging. These findings suggested that distortion and stricture of the extrahepatic bile duct induced biliary stasis and led to these biochemistry assay abnormalities. Mandaliya et al. reported a case with a total bilirubin level of 20 mg/dL due to stricture of the common bile duct (12).

However, cases of acute pancreatitis caused by a prolapse of the pancreas with a large hiatal hernia have been reported previously (Table, Type 3) (6,7,19-27). A diagnosis of acute pancreatitis is generally established based on abdominal symptoms accompanied by the significant elevation of pancreatic enzymes (i.e., amylase or lipase) or imaging evidence suggestive of pancreatitis (i.e. peripancreatic fluid or mesenteric stranding). Elevation of pancreatic enzymes and pancreatitis are considered to be triggered by obstruction of the main pancreatic duct secondary to migration of the pancreas. Chevallier et al. demonstrated folding and obstruction of the main pancreatic duct with endoscopic retrograde cholangiopancreatography imaging (6). Repetitive trauma or ischemia of the pancreas resulting from traction with pancreatic migration may be one explanation for this finding (8). Chevallier et al. reported a case with increased levels of pancreatic enzymes (amylase and lipase) and incarceration of the pancreatic body on CT, but these findings were transient and returned to normal after seven days (6). Lu et al. also reported a case of pancreatitis due to a hiatal hernia with the pancreas returning to its normal position two months later (24). These cases strongly suggest that the contents of the hernia sac were neither permanent nor stable and could move spontaneously and dynamically.

In addition, we found five cases with combined characteristics of both types 2 and 3 (Table, types 2 and 3). These cases demonstrated that bile and pancreatic duct involvement can occur simultaneously and might be mutually transitional, according to the location of the prolapsed pancreas.

Table shows an uneven distribution of types 2 and 3 in terms of the areas of the prolapsed pancreas. Depending on the part of the prolapsed pancreas, we roughly divided pancreatic herniation into two clinical patterns, as shown in Fig. 4. Pattern A indicates hiatal hernia including pancreatic head herniation (Fig. 4A), while pattern B indicates hiatal hernia mainly with pancreatic body and tail prolapse (Fig. 4B). Among patients with pure type 2 pancreatic herniation, 5 of 5 patients (100%) had herniation of the pancreatic head (pattern A, Fig. 4A). Since cholestasis is caused by bile duct twists and obstruction, sliding of the pancreatic head with the common bile duct likely played an important role in the onset of this condition. Although our case included not only the head of the pancreas but also the body and tail as prolapsed parts into the mediastinum, we consider this case as one of pattern A. Meanwhile, in patients with pure type 3 pancreatic herniation, only 1 of 10 cases (10%) involved the head of the pancreas as a herniated portion of the hiatal hernia, and in most cases, the body or tail of the pancreas migrated into the mediastinum (pattern B, Fig. 4B). Kumar et al. also pointed out that most cases with pancreatitis secondary to a hiatal hernia had herniation of the pancreatic body and tail (22). Tomida et al. summarized 14 previously reported cases with acute pancreatitis secondary to a large hiatal hernia. In 11 of 14 cases (78.6%), the pancreatic herniation involved the pancreatic body and tail (18). Since pancreatic duct obstruction is considered the main cause of acute pancreatitis, a deformity wherein the pulled pancreatic duct in the body or tail of the pancreas may contribute to the development of acute pancreatitis.

Figure 4.

Schematic illustrations of pancreatic herniation with a hiatal hernia. A (pattern A): Pancreatic head herniation with deformity of the extrahepatic bile duct, as seen in our case. B (pattern B): Pancreatic body and tail herniation with peripancreatic effusion due to acute pancreatitis.

The optimal therapeutic strategy for a large hiatal hernia with a pancreatic herniation remains unknown. In a clinical setting, therapeutic decisions are usually chosen on a case-by-case basis. Our review showed that all patients with pure type 2 pancreatic herniation underwent surgery. In contrast, only 5 of 11 patients (45%) with pure type 3 pancreatic hernia received surgery (Table). Acute pancreatitis may prevent surgeons from considering surgery because of peripancreatic inflammation after acute pancreatitis. Since some cases suggested mobility of the pancreatic position (6,24), and most patients with a pancreatic hernia are elderly with comorbidities, the indication for surgery must be considered carefully and comprehensively.

In conclusion, there are three clinical types of pancreatic herniation with hiatal hernia: type 1 (asymptomatic pancreatic herniation), type 2 (cholestasis due to bile duct involvement), and type 3 (acute pancreatitis). Pancreatic head herniation can cause cholestasis and bile duct dilatation more often than pancreatic body or tail herniation, while the latter can induce acute pancreatitis more frequently. As the prevalence of hiatal hernia has been increasing in our aging society, we will more often encounter these uncommon complications associated with this common disease.

The authors state that they have no Conflict of Interest (COI).