Gastric volvulus and tension gastrothorax secondary to spontaneous transdiaphragmatic intercostal hernia

Abstract

Spontaneous transdiaphragmatic intercostal hernia is an extremely rare clinical entity featuring dual defects in the diaphragm and chest wall. We report on the case of a 59-year-old man who developed a large left-sided hernia secondary to the minor trauma of a coughing fit. The hernia subsequently enlarged over the course of 3 years until it contained the stomach, leading to a gastric volvulus and tension gastrothorax with secondary pneumothorax. A subtotal gastrectomy was performed with Roux-en-Y reconstruction, and he made a full recovery.

Background

This case represents the first published example of gastric volvulus and tension gastrothorax secondary to transdiaphragmatic intercostal hernia. It is also the first published case of transdiaphragmatic intercostal hernia to demonstrate continued deterioration over a prolonged time course. Surgeons dealing with complex diaphragmatic and chest wall herniae should be aware of this potential complication. There is a growing understanding of the pathophysiology of transdiaphragmatic intercostal herniae, but clear management guidelines do not yet exist for this condition.

Case presentation

A 59-year-old man was brought in by ambulance, with the presenting complaint of coffee-ground vomiting for the past 12 hours and generalised malaise for the past 17 days. He lived at home with his wife. His comorbidities were non-alcoholic steatohepatitis (month 33 of a placebo-controlled trial to assess safety and efficacy of crenicriviroc in treating liver fibrosis on a background of no previous evidence of liver decompensation, with normal liver function tests), gastro-oesophageal reflux disease and chronic lower back pain. His weight at time of presentation was 106 kg, with a height of 175 cm and a body mass index (BMI) of 34.6 kg/m². He was diagnosed with a large hiatus hernia on gastroscopy ten months prior, and he had a 3-year history of a large left abdominal intercostal hernia (AIH) protruding between ribs 9 and 10, causing significant splaying of those ribs. His only surgical history was an uncomplicated open supraumbilical hernia repair 8 years previously.

The patient had reported experiencing sudden-onset severe pain at the site of the hernia 3 years previously, secondary to a vigorous coughing fit during a bout of pneumonia. This is presumed to be the initial onset of the hernia. He denied any prior trauma or surgery to the site. A CT scan performed at the time demonstrated a highly displaced costal margin fracture between the ninth and tenth rib costal cartilages, with the 10th and 11th ribs inferiorly deviated away from the normal costal margin. A broad-based intercostal hernia (20×8 cm at base) projected between the ninth and tenth ribs, containing spleen and the splenic flexure of the colon. Over the subsequent 3 years between this injury and the presentation above, multiple CT scans showed no evidence of diaphragmatic hernia. He had been reviewed as an outpatient by both thoracic and general surgeons and planned for a trial of non-operative management due to his mild symptoms and medical comorbidities.

In the emergency department, the patient appeared unwell, with a blood pressure of 80/50, a heart rate of 200 beats per minute (rapid atrial fibrillation), and requiring 4 L of oxygen to maintain saturations above 95%. His temperature was 34.3°C. Breath sounds were normal on the right, but no air entry was heard below the fourth intercostal space on the left. The chest wall hernia itself was clearly visible and tender, but soft (figure 1).

Figure 1.

Clinical photograph of the patient in the emergency department with a visible hernia in the left chest and flank after the insertion of an intercostal catheter.

Investigations

On initial point-of-care venous blood gas analysis, pH was 7.29 with an elevated carbon dioxide level consistent with moderate metabolic acidosis. Lactate was 11 mmol/L and base excess was −4.8 mmol/L. Formal blood samples showed a haemoglobin of 164 g/L, white cell count of 15.1×10⁹/L and platelet count of 427×10⁹/L. Creatinine was 133 μmol/L, an increase from 103 two weeks ago. Electrolytes, liver function tests and lipase were within normal limits. A chest radiograph revealed the presence of an intrathoracic viscus, likely being the stomach (figure 2).

Figure 2.

Preoperative erect chest radiograph showing deviated trachea and mediastinum towards the right chest, secondary to intrathoracic stomach and pneumothorax. Inferiorly deviated 10th rib can be seen posterior to stomach. AP, anteroposterior.

A contrast-enhanced CT demonstrated left tension gastrothorax with collapse of the left lung and rightward mediastinal shift, secondary to a dilated, herniated stomach (figure 3). The stomach had malrotated to form a secondary mesenteroaxial volvulus, with evidence of ischaemia, perforation and extraluminal gas (figure 4). The oesophago-gastric junction was intraperitoneal, with stomach projecting through the intercostal hernia and subsequently through a lateral diaphragmatic hernia to enter the chest.

Figure 3.

Coronal slice from CT scan on presentation. Intrathoracic stomach can be seen with intramural gas.

Figure 4.

Line diagram depicting gastric volvulus with relation to diaphragm and ribs. Author: Kateryna Burlak.

Differential diagnosis

This complex presentation featured imaging abnormalities in the respiratory, gastrointestinal and musculoskeletal systems, and it was not immediately obvious which was the instigating condition. In particular, the combination of tension physiology with a lack of air entry on the left led to the placement of a left intercostal catheter for presumed tension pneumothorax. The chest tube showed minimal swinging and the patient did not improve clinically. A repeat chest radiograph showed resolution of pneumothorax but persisting rightwards deviation of mediastinum, supporting the alternative diagnosis of tension gastrothorax (figure 5). The idea of tension pneumothorax was altogether abandoned once the ischaemic stomach with volvulus was identified on CT scan (figure 3). At this point, the on-call general surgeon was asked to review the patient, who arranged to transfer the patient to theatre for urgent surgery.

Figure 5.

Erect chest radiograph after insertion of intercostal catheter, showing persisting deviation of mediastinal structures to the right despite drainage of pneumothorax.

Treatment

Nasogastric tube insertion was attempted prior to gastroscopy but was unable to decompress the stomach, consistent with Borchardt’s triad. Gastroscopy revealed an extremely distended and necrotic-appearing stomach, necessitating the decision to proceed to laparotomy. The findings were a large left posterior diaphragmatic defect, containing a necrotic stomach. The cavity also contained omentum, spleen and transverse colon, but these appeared healthy. The cavity was contaminated secondary to a perforation in the lesser curvature of the stomach. A subtotal gastrectomy was performed, transecting the cardia and antrum with a linear Endo GIA ‘Tri-Staple’ stapler (Medtronic, USA). The abdomen was washed out with copious normal saline. The diaphragmatic defect was not closed, the stomach was left stapled off, and the abdomen was left open at this initial damage control surgery. A 28Fr chest drain was placed under vision. A post-operative chest radiograph confirmed resolution of the gastrothorax and normal position of the left hemidiaphragm (figure 6).

Figure 6.

Supine chest radiograph immediately after initial damage control surgery. Left lung reinflated with resolution of tension. Focal atelectasis at the left base.

At relook laparotomy the next day, following a period of stabilisation in the intensive care unit, there was minimal contamination in the abdomen. The diaphragmatic defect was closed using 2–0 PDS sutures and a Bio-A biologic mesh 10×15 cm (Gore, USA) (figure 7), and a Roux-en-Y gastric reconstruction was performed. A percutaneous enterogastrostomy (PEG) feeding tube was placed in the remnant gastric antrum. The intercostal hernia was not repaired, as the general contamination contra-indicated the mesh which would be necessary.

Figure 7.

An intraoperative photograph depicts the apposition of the diaphragmatic defect with interrupted sutures.

Postoperatively, the patient was given extended courses of broad-spectrum antimicrobials, including antifungals. Total parenteral nutrition was initiated early postoperatively, which was gradually downtitrated as the patient tolerated more enteric feeding through the PEG tube. An oral contrast study showed no evidence of anastomotic leak. Histopathology from the gastric specimen showed extensive full-thickness necrosis and perforation, but no evidence of underlying malignancy or dysplasia.

On day 5 postoperative, a CT pulmonary angiogram demonstrated extensive segmental and sub-segmental pulmonary emboli, despite ongoing venous thromboembolism chemoprophylaxis. He was commenced on weight-adjusted therapeutic dosages of enoxaparin, and eventually changed to apixaban. On day 21 postoperative, a sample of diarrhoea tested positive for Clostridium difficile, which was successfully treated with a course of oral vancomycin.

The patient was discharged on postoperative day 37.

Outcome and follow-up

Ten months postdischarge, the patient remains well, with no further hospital admissions. His PEG tube was removed six weeks post-operatively. He initially struggled with early satiety and poor appetite, which contributed to a weight loss of 15 kg. His overall weight has plateaued at 91 kg, with a new BMI of 29.7 kg/m². The intercostal hernia is still present, which causes occasional discomfort with extended duration of walking. He is planned for ongoing outpatient surgical follow-up and further consideration of definitive repair of intercostal hernia.

Discussion

The most commonly used terminology for intercostal herniae divides them into ‘transdiaphragmatic intercostal herniae’ (those that involve a diaphragmatic defect in addition to the intercostal defect—TDIH) and ‘AIH’ (those that do not involve a diaphragmatic defect).¹ Both types are much more common in the setting of blunt or penetrating trauma. When they occur secondary to violent coughing, a history of smoking or chronic obstructive pulmonary disease is likely to be present.¹

Spontaneous atraumatic AIHs remain a relatively rare phenomenon, with 49 cases reported in the professional literature.^2–20 Almost all occurred in the context of a coughing fit. 23 occurred on the left, and 26 on the right. All occurred at or below the level of the seventh intercostal space. Out of 49, 33 cases had a definite diaphragmatic defect. A variety of organs were noted to be present in the herniae—most commonly small bowel, large bowel and liver. Four herniae were noted to contain stomach, but none of these were strangulated.1 2 16 21 However, strangulation of other organs, including liver and small bowel, have been reported previously.^{13 22} Given the relative rarity of this disease process, there have been limited evidence-based guidelines published, however recent reviews have begun to offer clear recommendations.^{3 23}

Prior to this presentation, this patient had had three CT scans over 3 years showing an uncomplicated AIH, an intact diaphragm and an intra-abdominal stomach. He then rapidly deteriorated over the course of 10 days, which possibly corresponds to his stomach moving intrathoracically. It is thought that the initial injury ruptured the costal margin with detachment of (possibly attenuated) fibres between the diaphragm and the 10th rib, along with intercostal muscle fibres between the 9th and 10th ribs, thus weakening the diaphragm and creating a small defect. Over time, the increasing size of the intercostal hernia put further shear strain on the diaphragm, widening the defect. Increased intra-abdominal pressure, such as in the setting of chronic cough, can ‘push’ abdominal viscera through the defect and into the chest cavity.²⁴

This is the first reported case of AIH or TDIH to lead to total gastrothorax. Tension gastrothorax is an equally rare condition (a 2015 review found 25 reported cases) that occurs when herniation of the stomach into the chest cavity leads to a pronounced mass effect and subsequent obstructive shock.²⁴ According to Bunya et al, five steps are required: (1) existence of a diaphragmatic defect; (2) increased intra-abdominal pressure; (3) prolapse of the stomach into thoracic cavity; (4) a functional change in the gastro-oesophageal junction (by way of an abnormal angulation) to form a one-way valve and (5) reduction in cardiac output as a result of mediastinum shift.²⁴ The diaphragmatic defect can be acquired, as in this case, or congenital. Treatment should be aimed at immediate gastric decompression with nasogastric tube or endoscopy, followed by definitive management of the underlying cause. Pneumothorax is a much more common cause of tension physiology than gastrothorax, but early recognition of the latter is crucial, since placement of an intercostal catheter to treat pneumothorax could potentially lead to iatrogenic gastric injury.

Learning points.

• Abdominal intercostal herniae are frequently associated with diaphragmatic injury.

• Undiagnosed diaphragmatic herniae can enlarge over time, exposing patients to further risks such as tension gastrothorax.

• No consensus guidelines exist for optimal management of asymptomatic or mildly symptomatic intercostal herniae due to low overall case numbers, but recent reviews have begun to offer a more in-depth analysis of surgical options.

• Surgical repair of diaphragmatic and intercostal herniae should be considered early to prevent escalation in symptoms.

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