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A Life-Threatening Problem Hidden Within a Hiatal Hernia

— Condition resolved after clinicians used a novel oral combination

MedpageToday
A woman about to undergo a gastroscopy

A 61-year-old woman presents to a hospital in Austria for an outpatient gastroscopy due to an ongoing problem with black and tarry stools. She has no abdominal pain or problems with elimination. Her only comorbidity is psoriasis vulgaris, and she is a non-smoker.

Her medical history includes having had an open appendectomy, and she reports having suffered a stroke about 20 years previously, for which she took aspirin until about 11 years ago, although there are no medical records to verify the diagnosis.

The patient underwent two previous gastroscopies (the first at age 21 and the second about 15 years previously) to investigate a chronic intermittent iron-deficiency anemia first diagnosed when she was 40 years old. Both gastroscopies revealed no pathologies. She also had a colonoscopy, but no source of bleeding was detected.

Based on the unremarkable endoscopic findings, her physicians considered her to have chronic iron deficiency due to excessive menstrual bleeding, for which the medical team prescribed iron supplementations.

After the patient went through menopause, however, the anemia persisted, and she notes at this most recent medical appointment that she has not taken any iron supplements for the last 5 years.

Given her medical history, clinicians order blood tests; the results are all within normal range, including her iron level, which is 115 mcg/dL (normal is 50-170 mcg/dL).

Clinicians successfully intubate the patient and perform a gastroscopy, which detects an axial hiatal hernia spreading approximately 37 to 38 cm from the incisors, advancing aborally, with a diameter of 2.2 cm. Also noted are numerous small, flat hematin spots covering the gastric fundus and corpus, not evident in the antral area. The duodenum is macroscopically intact.

The medical team makes a diagnosis of Cameron lesion, due to inversion of the fibroelastic gastroscope showing a small macroscopically visible, linear erosion (which is not bleeding) within the hiatal hernia. Clinicians collect tissue samples, which reveal signs of carditis.

image
Cameron erosion at the time of diagnosis: During the initial gastroscopy, the erosion presented as a linear mucosal defect (white arrow). After partial gas desufflation, the volume and diameter of the sliding hiatal hernia were reduced, providing a better presentation of the lesion and to enable sampling. The left middle and lower part and lower right of the lesion show several hematin spots.

Clinicians start the patient on pantoprazole, a proton pump inhibitor (PPI), given 40 mg b.i.d. for 2 weeks, and afterwards once daily, along with sucralfate (four times daily) for 6 weeks.

When the patient returns for follow-up gastroscopy 6 weeks later, she indicates no problems and no melena, and results of laboratory tests are within reference ranges.

Endoscopic examination again reveals multiple flat hematin spots in the proximal two-thirds of the stomach. The erosion is still visible, although half was covered by fibrin. Clinicians take tissue samples from the erosion, and change the patient's treatment to pantoprazole 40 mg once daily, along with a gel containing hyaluronic acid, chondroitin sulfate, and poloxamer 407 (four times daily, 30 min postprandially, and at bedtime) for 3 months.

A subsequent appointment for another follow-up endoscopy is cancelled due to the COVID-19 pandemic; clinicians contact the patient by phone and she reports still having no complaints and no melena. After completing the above prescribed treatment for 3 months, she takes only pantoprazole 40 mg once daily.

Eventually, a second follow-up gastroscopy is performed 7 months after the initial examination, which shows no evidence of the erosion and an intact cardiac mucosa.

Discussion

Clinicians reporting this of a 61-year-old woman with late-diagnosed Cameron lesion that failed to improve with administration of a single dose of a PPI note that adding oral treatment with combined poloxamer 407 with hyaluronic acid and chondroitin sulfate successfully resolved the erosion.

The gastric mucosal defects localized within hiatal hernias are named for the American physician Alan Cameron, who with John Higgins between diaphragmatic hernias and occult gastrointestinal (GI) bleeding.

The case authors note that the prevalence of the lesions, which vary in size, is determined by two factors: the size of the hiatal hernia, and the size of the hernial sac.

Mucosal defects occur more commonly in the presence of larger hernias -- Cameron lesions are found in 5.2% of all patients with hiatal hernias who are undergoing gastroscopy, and more than 60% present as a single tissue defect. About half of all gastroscopic examinations performed for other reasons lead to diagnosis of a Cameron lesion.

Etiology and Pathophysiology

The case authors explain that the pathogenesis of Cameron lesions has not been clearly defined, but is thought to be caused by the combined effects of extra- and intra-luminal mechanical and chemical factors, including:

  • Presence of a diaphragmatic hernia (axial or paraesophageal)
  • Extraluminal rubbing of the upper stomach and lower esophagus against the diaphragmatic hiatus
  • Ratio between hernial opening and hernial size
  • The "dual-hit" hypothesis -- i.e., external compression on the gastric wall combined with an aggressive influence from inside such as gastric acid, gastro-esophageal reflux, or the oral intake of non-steroidal anti-inflammatory drugs (NSAIDs) for 3 days a week within a month
  • Helicobacter pylori
  • Gastric ischemia and stasis

Morphology

Cameron lesions are non-peptic non-gastroesophageal reflux disease-associated mucosal defects, which develop on the top of gastric folds over the distal ring of a hiatal hernia (rarely proximally), usually along the lesser gastric curvature.

In addition, there may be erosions that are superficial in nature, or deep-riding ulcers, generally longitudinal, but in some cases oblong or ellipsoid, but thought to never involve the entire circumference of the hiatal hernia.

Symptoms

Cameron lesions tend to affect older women who have long-standing anemia and large hiatal hernias. Because hiatal hernias rarely cause symptoms, signs of Cameron lesion-related GI bleeding may be overt (for example, melena, hematochezia, hematemesis) or occult (iron-deficiency anemia or a positive fecal occult blood test).

Some patients with Cameron lesions have a history of recurrent GI bleeding, and may have required blood transfusions; the use of oral NSAIDs or iron supplements is also commonly seen in those affected, and less often, patients may also have peptic ulcer or esophagitis.

Diagnosis

Gastroscopy is the diagnostic criterion standard for Cameron lesions, which often remain undetected during the initial assessment; thus, each hiatal hernia should be inspected ante- and retrograde with an additional perpendicular presentation of the hernial opening where the diaphragmatic hiatus exerts the highest pressure on the gastric wall (the predominant location of Cameron ulcers).

The case authors also urge examination of the mucosa above and beneath the hiatus esophagus, given the lesions' potential upward or downward migration, depending on how much the stomach is insufflated. Chromoendoscopy and optical magnification may also be helpful, and patients may have evidence of mucosal edema, erythematous stomach lining, and ecchymoses on gastric folds.

While no specific laboratory tests or imaging modalities have been identified to detect Cameron lesions, in some cases blood tests may reveal iron-deficiency anemia, and x-ray or computed tomography (CT) may show complications like perforation, volvulus, or gastric blood clots.

In addition, under certain conditions, CT angiogram or Tc-99m-labeled red blood cell scintigraphy will reveal an active bleeding source (i.e., bleeding speed at least 0.5 ml/min and 0.04 ml/min, respectively). The role of capsule endoscopy remains unclear.

Histopathology

The case authors note that they are aware of only one published report describing in Cameron lesions, including mucosal alterations related to mucosal vascular obstruction consistent with ischemic gastropathy, such as hemorrhagic infiltrates, fibrin thrombi, inflammatory response, sanguine micro-suffusions, sloughing of epithelial cells, atrophy of crypts, and coagulation necrosis.

Initial tissue sampling in that case revealed mucosal alterations consistent with minor chronic carditis.

Management

Little is known about the natural history of Cameron lesions; thus, in the absence of strict guidelines and algorithms to guide their , decisions about whether they should be managed conservatively or require surgical treatment must be individualized, the authors explain, adding that published reports range from cases of spontaneous healing to high-dose PPI treatment regimens.

Clinical presentation generally guides management, and usually involves initiation of PPI treatment upon diagnosis. In patients already receiving PPIs once daily, clinicians advise increasing the dosage or frequency of administration. Patients with iron deficiency require iron supplementation, or an increase in dosage for those already taking iron, the authors write, cautioning that a single iron supplementation is not sufficient.

These measures have been associated with adequate symptom control in up to half of patients with Cameron lesions; since up to one-third of patients with Cameron lesions have life-threatening gastrointestinal bleeding, stabilizing measures are required in cases of significant bleeding and hemodynamic shock.

Use of NSAID should be immediately discontinued, the authors warn, noting that some experts recommend the administration of prokinetic agents.

The criteria for progressing from conservative to surgical treatment have not been identified, although some specialists suggest a secondary surgical treatment in patients who have persistent complaints such as anemia, or frequent hospitalization due to GI bleeding or chronic blood loss, or complications such as refractory bleeding, perforation, volvulus, and gastric incarceration.

The main goal of surgery is to eliminate the associated hiatal hernia, with options including laparoscopic/open fundoplication with or without gastropexy and the recently developed single-incision transgastric underrunning.

If Cameron lesions are bleeding, endoscopic hemostasis is a relevant but rarely reported nonsurgical option, with band ligation and clipping shown to be effective. A follow-up gastroscopy was not performed in many studies, but could be indicated when complaints persist.

This patient's lack of response to initial PPI treatment after gastroscopy led the medical team to use the mixture of hyaluronic acid, chondroitin sulfate, and poloxamer 407, which has shown benefits in animal studies in healing scars after endoscopic mucosal resection or submucosal dissection, and the expectation was that it could provoke an early proliferation of collagen and elastic fibers in gastric mucosal defects, thus also contributing to ulcer healing.

As a low-cost alternative to existing treatments for Cameron lesions, hyaluronic acid and chondroitin sulfate -- both used primarily in wound treatment -- are being studied in the treatment of GI lesions.

Because of their skin regenerative properties, both hyaluronic acid and chondroitin sulfate are widely used in cosmetics, and have proven to be effective in the healing of acute and chronic wounds. Recent research has also investigated their role in the treatment of , and the case authors note that based on existing reports and their own experience with their patient, both agents may also be helpful in conservative treatment of Cameron lesions in combination with PPIs.

Still, the agents are not able to influence the most important factor of the hiatal hernia itself.

Conclusion

The authors conclude that their patient's case illustrates that Cameron lesions should be considered as a diagnosis in patients with hiatal hernia who also have iron-deficiency anemia. Diagnosis can be made on upper endoscopy, and further studies are required to determine the precise role of combined poloxamer 407 with hyaluronic acid and chondroitin sulfate in the management of Cameron lesions.

  • author['full_name']

    Kate Kneisel is a freelance medical journalist based in Belleville, Ontario.

Disclosures

The case authors noted no conflicts of interest.

Primary Source

American Journal of Case Reports

Iliev IE, Loidl A "A 61-Year-Old Woman with Chronic Iron-Deficiency Anemia Due to a Cameron Lesion and a Response to Oral Application of Combined Poloxamer 407 with Hyaluronic Acid and Chondroitin Sulfate Following Single Treatment with Pantoprazole: A Case Report" Am J Case Rep 2021; 22: e928021.