Cameron Lesions

Etiology

Various theories have been proposed for the possible pathogenesis of Cameron lesions, with mechanical trauma caused by constant rubbing of mucosal folds at the constriction level of the diaphragm being the most likely cause. It has been postulated that different forces act at the neck of the gastric sac during breathing, including the upward sliding movement of the stomach, negative intrathoracic pressure causing outward and upward force, and impinging force by the crura during gastric sliding through the diaphragm.[4] Other mechanisms include gastric acid injury, focal ischemia due to diaphragmatic pressure on the herniated sac, and gut wall edema due to venous obstruction leading to vascular stasis.[5] Cameron lesions have not been shown to have a positive correlation with nonsteroidal anti-inflammatory drug use, Helicobacter pylori infection, or the absence of protein pup inhibitor usage. However, binge drinking in some studies has been related to the Cameron lesions.[6]

Epidemiology

The prevalence rates of hiatal hernia range from 0.8 to 2.9 in all patients undergoing upper gastrointestinal endoscopy. Cameron lesions are seen in 5% of patients with known hiatal hernia discovered in upper endoscopic studies.[7][8] Cameron lesions may heal spontaneously within a couple of days, contributing to the underreporting of these lesions. The size of the hiatal hernia correlates directly with the prevalence of these lesions. Since the frequency of hiatal hernia increases with age, most cases are reported in the elderly population.[9] It is rare in the pediatric subgroup of patients, with the youngest patient being 3. The prevalence is higher in women as compared to men.[10] 

Most of the cases have been reported from developed countries, including North America and Western Europe. There are few cases reported from Africa, but this could be attributed to underreporting. There has been no correlation with any environmental factors or genetics. Studies have shown that inadequate fiber intake and high sitting position during defecation could be possible risk factors for developing Cameron lesions.[11]

Histopathology

Macroscopically, the lesions appear as white, linear, superficial erosions on the crests of inflamed mucosal folds at the site of diaphragmatic indentation. A biopsy is not recommended to make the diagnosis of Cameron lesions. Histopathological examination in the past has revealed gastric atrophy, fibrosis, necrosis, and infiltration by immune-related cells. Microscopic characteristics have shown changes in vascular obstruction indicative of ischemic gastropathy. These changes include inflammatory markers, hemorrhagic infiltrates, fibrin deposition, sloughing of the epithelium, cryptic atrophy, and coagulative necrosis.[12]

History and Physical

Cameron lesions can present with a broad range of clinical features. The patients can be completely asymptomatic in the beginning. Cameron lesions have been identified as an etiology for occult upper gastrointestinal blood loss, at times leading to iron deficiency anemia.[13] The usual presentation in these cases includes anemia symptoms like fatigue, pallor, melena, palpitations, or dyspnea on exertion.[14] The larger hernias (>3 cm) are associated more frequently with iron deficiency anemia than the small hernias (<3 cm).[9] This is because the size of the lesions increases with an increase in the hiatal hernia size. The patients may also present with gastroesophageal reflux disease-like symptoms with epigastric discomfort. Rarely, patients may experience life-threatening overt GI bleed.[5]

Evaluation

Cameron lesions pose a diagnostic challenge to physicians. The laboratory tests show iron deficiency with low hemoglobin levels and microcytic hypochromic anemia. Iron studies reveal low iron and ferritin levels with high total iron-binding capacity.[15] The chest x-ray may help visualize the hiatal hernia, showing a large posterior mediastinum structure. A barium swallow is also a non-invasive modality to diagnose hiatal hernia. The hernias are usually seen on radiographic films but not the Cameron lesions.[8]

Endoscopy is the gold standard for diagnosis, although it is not uncommon to overlook these lesions due to their unique location. They are often missed on the initial endoscopy and are usually discovered on subsequent endoscopies.[16] The endoscopic evaluation involves careful antegrade and retrograde visualization of the region of the hernia sac and adjacent mucosa. Endoscopic findings include mucosal erythema, edema, and ecchymotic bleeding, along with the Cameron lesions in the gastric mucosal folds. Cameron lesions usually present as linear erosions of the gastric mucosa, frequently with erythematous borders.[17] 

Newer diagnostic studies like magnification chromoendoscopy may be useful to demonstrate the mucosal defects. They have also been shown to be useful in situations where there are no well-defined lesions. The usefulness of capsule endoscopy has not been fully documented. Given the short transit time and the cost involved, it is unlikely to be a diagnostic modality of choice.[18]

Treatment / Management

The treatment options include medical management, surgery, and endoscopic intervention.[2] The medical management consists of acid suppression with a proton pump inhibitor to help hasten mucosal healing and iron supplementation for concurrent iron deficiency anemia. Most patients have some acid-related problems (reflux disease, esophagitis) and are usually on acid inhibitors. This combination therapy has shown good results, with a noticeable improvement in the ulcer’s healing and the correction of the hemoglobin levels.[6] Endoscopic band ligation of actively bleeding Cameron lesions has shown good results in a limited number of cases. Endoscopic hemostasis is difficult in such scenarios due to technical and anatomical reasons. Cauterization and Epinephrine injection have been tried in some cases with temporary improvement.[19] The surgical options include fundoplication. It is recommended in patients with uncontrolled bleeding from these lesions, medically refractory disease, and complicated hernia with incarceration, volvulus, or perforation. Considering the close relation of hiatal hernia and Cameron lesions, fundoplication has been considered to be a reasonable treatment.[20][21](A1)

Differential Diagnosis

Cameron lesion has several differential diagnoses based on the patient’s age, comorbidities, and overall health. Some conditions have similar clinical features and need to be differentiated from Cameron lesions based on the presenting signs and symptoms and endoscopy. These include telangiectasias, ulcers, esophagitis, Mallory-Weiss tear, diverticulosis, Dieulafoy lesion, and neoplasms (see Image. Cameron Lesion and Ulcer).[22]

Prognosis

The outcomes of medical therapy are excellent. There has been good healing with acid inhibitors and iron supplementation at 6 weeks. Some studies have shown complications in the form of persistent anemia and rebleeding. The majority of patients who have been unresponsive to medical therapy continue to lose blood and require multiple transfusions.[23] Surgical treatment with fundoplication remains the mainstay of treatment in non-responsive cases. The surgical treatment has shown good results, with no recurrent ulcer or hemorrhage seen in the follow-up period.[24]

Complications

The diagnosis is usually missed due to the non-specific findings. It may result in delayed treatment, eventually resulting in adverse complications. The fatal complication is spontaneous hemorrhage if left untreated. The other complication includes worsening iron deficiency anemia, which in severe cases may cause hemodynamic instability and worsening of pre-existing comorbidities.[13]