Severe achalasia with hiatal hernia

Clinical History

The patient suffered from dysphagia and regurgitation and found intake of food increasingly difficult. She was admitted due to severe dehydration and malnutrition. Upon admission her weight was 38kg and her general condition poor. She previously had a percutaneous endoscopic gastrostomy (PEG) tube to secure nutrition.

Imaging Findings

She was referred to a barium swallow for anatomic evaluation.
The examination showed end stage achalasia combined with a large hiatal hernia. There was an abnormal dilatation of oesophagus with air-filled sacculate ballooning (Fig. 1a+b)). The passage of barium contrast was severely delayed due to almost total loss of peristalsis, and a spastic lower oesophageal sphincter (LES) with has a lumen of only 2 mm (Fig. 2b). Additionally, the large hiatal hernia measuring 13x8cm in diameter prevented the passage of barium contrast (Fig. 2a+b). Trendelenburg/Valsalva manoeuvre in supine position showed that most of the hernia was sliding, but it also had an paraesophagal component, making it a Type III hiatal hernia. It comprised 2/3 of the stomach, including the fundus and the majority of the corpus.

Discussion

Achalasia is a motor disease of unknown cause in which there is a loss of peristalsis in the distal oesophagus and a failure of LES relaxation. It is a rare disorder with an prevalence of 10 cases per 100, 000 individuals [1]. Men and women are affected with equal frequency and the disease can occur at any age, however, onset before adolescence is rare.
Hiatal hernia refers to herniation of elements of the abdominal cavity through the oesophageal hiatus of the diaphragm. Contrary to achalasia it is a common radiological finding with a reported frequency of 20-50% in the general population. The wide reported frequency is partly dependent on the age of the population studied as the frequency increases with age. [2]
Although hiatal hernia is more common, its occurrence and potential implication in achalasia is less well-known. There are few articles on the subject, and no greater study has been published in English the last 15 years. When reviewing the literature, the great majority of the reports on hiatal hernia in achalasia have emphasized its rarity with a prevalence ranging from 1.4%-14% [3-5]. Factors responsible for the rarity are not known.
However, based on the literature published so far the most common symptoms in patients with hiatal hernia and achalasia are dysphagia and/or regurgitation. The sex, age distribution, type and frequency of symptoms, and LES pressures have not been significantly different in patients having hiatal hernia compared to those without hernia. Furthermore, patients with achalasia and hiatal hernia where successfully treated with pneumatic balloon dilatation without complications, suggesting that such treatment can be performed successfully. [2, 6, 7]
The patient in this clinical case was treated with pneumatic balloon dilatation.

Differential Diagnosis List

Final Diagnosis

Sliding hiatal hernia in end stage achalasia.

URL:	https://www.eurorad.org/case/13855
DOI:	10.1594/EURORAD/CASE.13855
ISSN:	1563-4086