Life-threatening presentation of a parahiatal hernia after esophagectomy: a case report and review of the literature
- Camille Plourde and
- Émilie Comeau
- Département de chirurgie, Centre intégré universitaire de santé et de services sociaux de l'Estrie Centre hospitalier universitaire de Sherbrooke, Sherbrooke, Quebec, Canada
- Correspondence to Dr Émilie Comeau; emilie.m.comeau@usherbrooke.ca
Abstract
A woman presented to our hospital with acute abdominal pain 7 months following an oesophagectomy. A chest X-ray revealed a new elevation of the left diaphragm. CT demonstrated a large left diaphragmatic hernia incarcerated with non-enhancing transverse colon and loops of small bowel. She deteriorated rapidly into obstructive shock and was urgently brought to the operating room for a laparotomy. The diaphragmatic orifice was identified in a left parahiatal position, consistent with a parahiatal hernia. Incarcerated necrotic transverse colon and ischaemic loops of small bowel were resected, and the diaphragmatic defect was closed primarily. Because of haemodynamic instability, the abdomen was temporarily closed, and a second look was performed 24 hours later, allowing anastomosis and definitive closure. Parahiatal hernias are rare complications following surgical procedures and can lead to devastating life-threatening complications, such as an obstructive shock. Expeditious diagnosis and management are required in the acute setting.
Background
Diaphragmatic hernia can occur as a complication of oesophagectomy.1 Parahiatal hernia is an exceedingly rare type of postoperative diaphragmatic hernia and has seldom been reported following such type of surgery. Here, we report what is to our knowledge the first published case of incarcerated parahiatal hernia following oesophagectomy with an acute life-threatening presentation.
Case presentation
A woman presented to our hospital with acute abdominal pain and nausea, 7 months after oesophagectomy. On physical examination, she was in extreme pain but had normal vital signs. Her abdomen was diffusely tender, but soft without rebound nor guarding. She reported experiencing recurrent self-resolving episodes of nausea and abdominal pain since the surgery.
Her medical history was unremarkable except for a squamous cell carcinoma of the distal oesophagus for which the clinical diagnosis was T3N1M0 (stage III), according to the eighth edition of the American Joint Committee on Cancer TNM Classification. She underwent neoadjuvant chemoradiation therapy followed by minimally invasive Ivor-Lewis oesophagectomy. The perioperative course was uneventful, and the follow-up imaging was normal (figure 1). The final pathology was ypT0N1M0; therefore, no adjuvant therapy was given.
Post oesophagectomy chest X-ray (A). Chest X-ray at acute presentation 7 months later (B).
Investigations
A chest X-ray revealed a new elevation of the left diaphragm, a mild left pleural effusion and intrathoracic air (figure 1). Initial laboratory results were notably white blood cell 11.3, lactates 3.9, C-reactive protein 1.5 and creatinine 44. CT demonstrated a large left diaphragmatic hernia incarcerated with colon and small bowel. The herniated bowel had non-enhancing thickened walls, but no free air, suggesting ischaemia without perforation (figure 2).
CT of the chest and abdomen demonstrating a large left diaphragmatic hernia incarcerated with transverse colon, part of the left colon and multiple loops of small bowel (arrow). The herniated bowel has thickened walls with no enhancement (arrowhead). There is no pneumatosis or free air in the hernia sac, suggesting ischaemia but no perforation.
Treatment
After the patient underwent the CT, she became tachypneic and started desaturating. Her heart rate went up to 145 beats/min and blood pressure dropped to 96/78. While she partially responded to crystalloids, vasopressors and antibiotics were started and she was transferred urgently to the operating room.
A midline laparotomy was made, and the diaphragmatic orifice was identified in a left parahiatal position, measuring approximately 3.5 cm in diameter. It was located 1 cm from the intact oesophageal hiatus, therefore consistent with a parahiatal hernia. The collar had to be slightly enlarged radially to allow reduction in the abdominal cavity. From that moment, haemodynamics of the patient improved significantly. Although sepsis and hypovolaemia might have contributed to the shock, the marked haemodynamic improvement immediately following reduction was consistent with a significant obstructive component. The blood pressure went from 89–95/40 to 110–150/50 in less than a minute after the content of thoracic herniation was reduced. The hernia content was assessed, showing no perforation but a necrotic transverse colon and 170 cm of severely ischaemic small bowel, which were resected. The diaphragm was closed with a non-absorbable running suture, and a small bowel side-to-side stapled anastomosis was made. At that moment, the patient was still on vasopressors, had an intraoperative pH of 6.9, pCO2 53, pO2 167, HCO3− 11.2, lactates 9.0 and International Normalised Ratio (INR) 1.6. It seemed that the tissues were starting to bleed more easily. Fearing that coagulopathy was developing, it was decided to postpone the colonic anastomosis. The abdomen was closed with a temporary negative pressure closure system, and the patient was transferred to the intensive care unit.
Outcome and follow-up
The patient improved quickly and was brought back to the operating room the next morning, where a well vascularised small bowel anastomosis and colon with no signs of ischaemia or perforation were noted. A colo-colonic anastomosis was performed, and the abdomen was closed definitely.
On postoperative day 1, she was extubated. The nasogastric tube was removed on day 2, clear fluids were started, and she tolerated a regular diet the next day. She was discharged on postoperative day 5, after a 4-day course of intravenous antibiotics. A month later in clinic, she was doing well and felt relieved from the postprandial pain she had been experiencing since the oesophagectomy.
Discussion
Hiatal hernias are common with the majority consisting of sliding (>90%) or paraoesophageal type (5%). Parahiatal hernia is a rare form of hiatal hernia, characterised by the presence of a separate extrahiatal diaphragmatic defect with intervening normal crural muscle.2 The oesophageal hiatus is therefore intact. Although most congenital diaphragmatic hernia are either Morgagni or Bochdalek, congenital parahiatal hernias have been described. They seem to develop as a result of failure of closure of the pleuroperitoneal canal in embryonic development, which leads to a persistent pneumoenteric recess.3 The incidence in studies involving patients undergoing hiatal hernia repair is 0.2%–0.35%.4 5 The latest systematic review of the literature identified 14 cases of primary parahiatal hernias.6
On the other hand, secondary parahiatal hernias result from iatrogenic or traumatic injury to the diaphragm. We performed a search of the literature on PubMed using the keywords “parahiatal hernia” to identify all cases of acquired parahiatal hernias and found 29 articles. Of those, 20 were excluded: 4 did not report parahiatal hernias, 8 described primary types, 2 did not mention if the hernias were primary or secondary, two were paediatric studies, two were not in English or French and two were unavailable. Finally, we searched the bibliography of the articles to identify potentially missed cases. Full articles were reviewed only if deemed relevant according to titles or abstracts.
From the outset, we noticed that acquired parahiatal hernias seemed to be more common in the past. Effler described 16 patients requiring reoperation for complications attributable to a counterincision used for Allison transthoracic repair of hiatal hernia, which appears to be responsible for the majority of acquired parahiatal hernias in the prelaparoscopic era.7 However, one secondary parahiatal hernia following transthoracic hiatal hernia repair not explained by a planned counterincision was also reported.8
More recently, Palanivelu et al described 4 cases of acquired parahiatal hernias.5 Most patients presented with a chronic course of epigastric pain, nausea, vomiting, recurrent heartburn and/or postprandial bloating. None had clinical diagnosis of parahiatal hernia before surgery. Additionally, Takemura et al described a case of secondary parahiatal hernia with subacute incarceration of the stomach following treatment for a left malignant pleural mesothelioma.9 Similarly, Main reported a parahiatal hernia presenting with gastric herniation and necrosis 2 years following robotic Nissen fundoplication and hiatal hernia repair.10
Although uncommon, diaphragmatic hernia can occur after oesophagectomy, but they are generally hiatal hernias.11 In 2017, Akiyama et al described, for the first time, a true case of parahiatal hernia following oesophagectomy.1 Table 1 summarises our review, excluding 19 cases with planned counterincisions as the most likely cause of hernia.
Comparison of the clinical characteristics and surgical treatment of secondary parahiatal hernias
| Source | Age, gender | Symptoms | Previous surgery | Defect size (cm) | Treatment |
| Vallieres and Waters8 | 49, F | Transthoracic Heller myotomy, modified Belsey Mark IV antireflux repair (years later for residual symptoms) | 3-day course of epigastric pain and dysphagia; infarction and perforation of the gastric fundus. | 3 | Thoracotomy, partial gastrectomy and primary closure |
| Palanivelu et al 5 | 45, F | Epigastric pain (50%), nausea (15%), vomiting (10%), recurrent heartburn (80%) and/or post prandial bloating (25%) for 1–3 years | Laparoscopic fundoplication | 6 | Laparoscopic primary repair |
| 70, M | Laparoscopic transhiatal enucleation | 9 | Laparoscopic repair with mesh | ||
| 56, F | Laparoscopic fundoplication | 6 | Laparoscopic primary repair | ||
| 37, F | Laparoscopic fundoplication | 8 | Laparoscopic repair with mesh | ||
| Takemura et al 9 | 70, M | 4-day history of epigastric pain; gastric ulcer; gastric volvulus without ischemia | Treatment for left malignant pleural mesothelioma | 3 | Laparoscopic primary closure and proximal gastrectomy for iatrogenic gastric laceration |
| Main and Tymitz10 | 24, F | 1-day history of abdominal pain, nausea and vomiting, gastric herniation with areas of necrosis | Robotic hiatal hernia repair and Nissen fundoplication | NS | Laparoscopic repair with mesh, longitudinal gastrectomy |
| Akiyama et al 1 | 73, M | Abdominal pain and vomiting | Thoracoscopic oesophagectomy for oesophageal cancer with gastric tube reconstruction | 8 | Laparoscopic repair with mesh |
| Our case | 62, F | Abdominal pain, haemodynamic instability, necrosis of transverse colon and small bowel | Laparoscopic and thoracoscopic Ivor-Lewis oesophagectomy | 3.5 | Laparotomy, primary repair of diaphragm, transverse colectomy, small bowel resection, temporary closure of the abdomen followed by laparotomy, colo-colonic anastomosis and definitive closure 24 hours later |
-
NS, not specified.
To our knowledge, obstructive shock has never been reported with parahiatal hernias before. Although rare, haemodynamic compromise resulting from left atrial compression by a hiatal hernia has been reported.12–15 Compression of the left atrium leads to reduced ventricular filling, impaired systolic performance and increased pulmonary venous pressure, which can eventually progress to shock.12 In our case, we highly suspect compression of the heart was a significant factor explaining the patient’s haemodynamics because of the rapid improvement noted following hernia reduction. However, a cardiac echocardiogram was not performed, and sepsis and hypovolemia could also have been in cause. In most described cases of obstructive shock caused by a hiatal hernia, removal of gastric content with a nasogastric tube significantly improved haemodynamics of the patients. Here, the small bowel and colon were causing the obstruction in a parahiatal deficit. Decompression with a nasogastric tube was therefore not surprisingly unsuccessful.
This appears to be the first case of secondary parahiatal hernia after oesophagectomy with an obstructive shock as an acute life-threatening presentation. Following hiatal surgeries, care should be taken to inspect for any potential diaphragmatic injury. Given the severity of potential complications, repair of parahiatal hernias should be strongly considered in patients fit for surgery or if they develop symptoms.
Patient’s perspective
“I was diagnosed with esophageal tumor in 2019. A few weeks later, I needed a feeding tube because I couldn’t even swallow liquids anymore. As a treatment, I was offered 5 cycles of chemotherapy, 25 sessions of radiation therapy and, on top of that, a partial esophagectomy. Those weeks of therapy with a feeding tube were very difficult for me. The surgery was planned five weeks later by a renowned surgeon.
The surgery took place 4 months following the diagnosis and was considered a “success”. I was informed that without a stomach, I would need to relearn how to eat in smaller portions, but that with time I could almost be normal again. However, following the procedure I had recurring abdominal pain and nausea associated with 1 out of 3 meals. I had multiple discussions with the pivot nurse and was told to eat smaller meals and to contact my primary care physician. I came to the conclusion that I would only be able to eat rice and that I shouldn’t have had this surgery because my quality of life was harshly affected. I didn’t know what to eat and I spent a lot of time lying down waiting for symptoms to resolve. After 6 months, I was still weighting no more than 100 pounds, unable to gain weight. Sometimes I had episodes of uncontrollable retching without being able to throw up and those were very difficult since I was feeling like I was having a heart attack.
One night I had one of those major crises of violent spasms but this time it wouldn’t go away. I wanted to call an ambulance but since I lived in a secluded place deep in the woods, my partner suggested to drive me to the emergency room of one of the closest hospitals. I had multiple tests before they discovered the problem, and at that point I was in a critical condition.
I had to undergo two surgical procedures in two days, and I was cared for in the intensive care unit. My condition improved after 72h and I left the hospital 5 days later, which was truly a miracle.
It has been a long recovery but I was finally able to eat cautiously despite my esophagectomy and new intestinal condition. Although I lost about 40% of my bowel, I started gaining weight a few months later and am now stable at 112 pounds, which is good for me.”
Learning points
-
Parahiatal hernias are rare complications following surgical procedures involving dissection around the hiatus, such as oesophagectomy, and can lead to devastating life-threatening complications, like an obstructive shock.
-
Following these procedures, inspection for potential diaphragmatic injuries secondary to dissection should be done while reconstructing the hiatus.
-
Although the literature is scarce, parahiatal hernias might have a higher rate of complication compared with their regular hiatal counterpart. Regardless of symptoms, repair should be considered in patients who are fit for surgery. Unfortunately, the distinction between these types of hernias is hard to make and few are appropriately diagnosed preoperatively.6
Ethics statements
Footnotes
-
Contributors All authors were actively involved in direct patient care and have read and approved the manuscript. CP is the principal author; she did the review of the literature and wrote the first draft of the article. It was then reviewed and edited by EC and CP. EC is the treating surgeon of the patient and obtained her consent to the case report.
-
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
-
Competing interests None declared.
-
Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.
References
Use of this content is subject to our disclaimer