A 62-year-old woman with a history of hiatus hernia status post-Nissen fundoplication, gastroesophageal reflux disease (GERD), hypertension, hyperlipidemia, and hypothyroidism presented with sudden onset of abdominal pain while eating dinner the day before. She reported 10/10 pain in the epigastric and left upper quadrant areas, which she described as constant, severe, and stabbing in nature. Following the sudden onset of pain, she became nauseated and had multiple episodes of nonbloody, nonbilious emesis. She reported that this was her third ED visit and that previous labs and imaging were negative.
The patient reported that she had been seen for abdominal pain many times in the past. The previous episodes of pain were similar in location and occurred postprandial, but were not nearly as severe and never persistent or with vomiting. She said she had seen her surgeon just three weeks earlier with plans to repair the paraesophageal hernia in about one month. The surgeon's note said the patient had roughly one-third of her stomach herniated into her chest. She was scheduled for laparoscopic repair of the symptomatic paraesophageal hernia and a revision of the prior Nissen fundoplication.
The patient appeared ill and in moderate distress due to pain, with dry mucous membranes and mottled extremities. Her vital signs were normal except for sinus tachycardia at 117 bpm. Her abdomen was distended but soft with diffuse tenderness to palpation, worst in the epigastric and left upper quadrant with guarding, but no rigidity was noted. The remainder of the physical exam was unremarkable.
Labs and a CT of the chest and abdomen were ordered. Leukocyte count returned at 9.87 k/ul and lactate of 7.7 mmol/l. CBC, BMP, LFTs, and troponin were within normal limits. An initial ECG showed sinus tachycardia with a rate of 127 with hyperacute T waves in leads V2-V5.
She was treated initially with IV fluids, 1 mg hydromorphone, and 4 mg ondansetron. The chest x-ray showed hazy opacities in the lung bases, left greater than right, but no other obvious abnormalities. Broad-spectrum antibiotics were started.
Find the diagnosis and case discussion on p. 28.
Diagnosis: Acute Gastric Volvulus Secondary to Paraesophageal Hernia
The patient continued to decompensate while in the ED. She became confused, tachypneic, and more tachycardic. The CT returned and revealed pneumomediastinum, gastric pneumatosis, portal venous gas, free intraperitoneal gas, and intraperitoneal contrast extravasation which appeared to be secondary to acute gastric volvulus with ischemia and perforation. This was immediately communicated to the general surgery team, which quickly prepped the patient for the operating room.
A hiatus hernia occurs when there is herniation of abdominal contents through the esophageal hiatus of the diaphragm. Hiatus hernias are divided into sliding (type I) and paraesophageal hernias (types II-IV). Type I occurs when only the gastroesophageal junction is displaced above the diaphragm, and is usually caused by trauma, congenital defects, and iatrogenic factors. Most type I hernias are asymptomatic. These can cause symptoms of GERD, such as heartburn, regurgitation, and dysphagia, if large enough. Complications are rare. A symptomatic sliding hernia involves medical treatment of GERD symptoms and surgical treatment with Nissen fundoplication if symptoms are refractory to medical management. (Diaphragm. In: Surgical Foundations: Essentials of Thoracic Surgery. Philadelphia: Elsevier Mosby, 2004.)
Types II-IV or paraesophageal hernias occur when the gastric fundus is displaced above the diaphragm to varying degrees. The etiology of types II-IV of hernias are usually a complication of surgical dissection of the hiatus after anti-reflux procedures (most commonly Nissen fundoplication), esophagomyotomy, or partial gastrectomy. The majority of types II-IV are asymptomatic or have intermittent symptoms of epigastric/substernal pain or postprandial fullness with nausea. Complications include gastric volvulus, bleeding (from gastric ulceration, gastritis, or erosions) and respiratory complications from mechanical compression. (Diaphragm. In: Surgical Foundations: Essentials of Thoracic Surgery. Philadelphia: Elsevier Mosby, 2004.)
The management of an asymptomatic types II-IV of hernia is controversial; most experts recommend against surgical treatment because the annual risk of developing acute symptoms requiring emergent surgery is 1.1 percent while the mortality rate from elective repair is 1.4 percent. (Ann Surg 2002;236:492.)
Surgical treatment is indicated in symptomatic types II-IV (paraesophageal) hernias. Elective repair is done in patients with GERD refractory to medical therapy, dysphagia, early satiety, postprandial chest or abdominal pain, anemia, and vomiting, and is associated with improved symptoms and better quality of life. (JAMA Surg 2015;150(5):424.) Emergent repair is done in patients with acute gastric volvulus, uncontrolled bleeding, obstruction, strangulation, perforation, or respiratory compromise, and is associated with high mortality. (Ann Surg 2016;264:854.)
Acute gastric volvulus occurs when the stomach rotates along its axis, and if rotated enough, it can lead to strangulation of the stomach, causing ischemia and potentially necrosis, perforation, and abdominal sepsis. Risk factors for gastric volvulus include age over 50, diaphragmatic abnormalities (paraesophageal hernia, hiatal hernia, or other diaphragm hernia), phrenic nerve paralysis, kyphoscoliosis, and other stomach or splenic abnormalities. (Int J Surg 2010;8:18.) Acute gastric volvulus is a surgical emergency associated with mortality rates estimated at 30 percent. (Surg Endosc 2016;30:1847.) Endoscopic derotation and percutaneous endoscopic gastrostomy tube placement are often attempted first to manage patients who are poor candidates for surgical repair. Operative decompression and derotation will be needed if the stomach is unable to be reduced endoscopically. (Gastrointest Endosc 1995;42:244.)
The patient underwent EGD in the OR, which showed healthy esophageal mucosa, but the surgeons were unable to pass the endoscope into the stomach. The surgery team then proceeded with exploratory laparotomy and left-sided thoracotomy, which revealed a large amount of gas and turbid fluid as well as a gastric volvulus with herniation into the thoracic cavity, which was reduced. The fundus and body along the greater curvature of the stomach were necrotic with perforation of the anterior wall of the fundus along the greater curvature, requiring transection of the distal esophagus and proximal gastrectomy. The remainder of the stomach showed patchy areas of necrosis. The esophagus and remaining stomach were left in discontinuity because of the unstable nature of the patient and the friability of the remaining tissue. The chest was washed out and closed, and a left tube thoracostomy was placed. The abdomen was washed out and left open with a wound vac.
Post-operatively, the patient's repeat lactate was 4.1 mmol/l, and she remained intubated, requiring vasopressor support with continued broad-spectrum antibiotics. She was transferred to surgical ICU for further management.