CASE 16549 Published on 31.10.2019

Corkscrew oesophagus – A rare cause of dysphagia

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

Mariana Chaves, Rafaela Sousa, Sofia Amante, Alexandra Mesquita, Rodrigo Cordeiro, David Silva

Hospital do Divino Espírito Santo de Ponta Delgada, Azores, Portugal

Patient

80 years, male

Categories
Area of Interest Gastrointestinal tract ; Imaging Technique Fluoroscopy
Clinical History

An 80-year-old male patient presented with chest pain, dysphagia, and progressive weight loss. He underwent a cardiac and otorhinolaryngological examination and a cervico-thoracic CT scan, that showed no evidence of malignancy or other relevant findings. As the symptoms persisted, an upper gastrointestinal endoscopy and a videofluorographic swallowing study were requested.

Imaging Findings

The upper gastrointestinal endoscopy revealed oesophageal tortuosity without other relevant findings (Fig. 1).

Videofluoroscopy was performed using barium contrast with typical consistency. Oral and pharyngeal phases showed no major defects (Fig. 2 a-d). However, a posterior oesophageal indentation at the level of C5-C6 was detected, apparently caused by the cricopharyngeus muscle hypertrophy. This finding is called the cricopharyngeal bar (Fig. 3).

The oesophageal phase was observed on the lateral view (Fig. 4), showing peristaltic oesophageal contractions, interrupted by many tertiary (non-propulsive) contractions, seen in the middle and distal oesophagus, which formed a "corkscrew" morphology.

Discussion

Diffuse oesophageal spasm (DES) is a rare oesophageal motility disorder associated with dysphagia. [1] It is characterised by multiple spontaneous and uncoordinated oesophageal contractions [2,3] and can occur at any age, although it is predominantly seen in patients over 50 years of age. [1] DES typically causes substernal chest pain with dysphagia for both liquids and solids, but symptoms may occur in different locations and range in severity and duration. [4] 

As in this case, many patients start the investigation with an upper gastrointestinal endoscopy, as it allows to search for structural abnormalities like oesophageal benign or malignant stenosis or oesophagitis. In patients with DES, it does not reveal any specific abnormality, but oesophageal tortuosity (Fig. 1) or disordered contractions could be found during the exam. [1]

Videofluoroscopy findings are variable. The majority of barium swallows will be abnormal, but just some patients show disruption of peristalsis with tertiary contractions, which can obliterate the oesophageal lumen causing a "corkscrew" or "rosary bead” oesophagus, the classic appearance of DES (Fig. 4). [1,3]

A cricopharyngeal bar is a radiologic descriptor of a posterior impression at the pharyngoesophageal segment, that can occur as a response to oesophageal disease (e.g., DES) and may contribute to the patient's symptoms if the indentation from the cricopharyngeal muscle occupies more than 50% of the expected luminal oesophagus diameter, as in this case (Figure 3). [2]

The oesophageal motility disorders are confirmed by manometry studies, which evaluate lower oesophageal sphincter function, the oesophageal body peristalsis, and the characteristics of contraction waves. [3] DES is defined manometrically as simultaneous contractions in the smooth muscle of the oesophagus alternating with normal peristalsis in over 20% of wet swallows with amplitude contractions of greater than 30 mmHg. [5]

Based on radiologic findings, DES was suspected, and the manometry confirmed the diagnosis, allowing to guide the patient to a specific therapy.

DES is a chronic condition, and its treatment is inconsistent in the literature. Dietary modification may be helpful because liquids and soft foods are better tolerated than solids. [4] Medical management includes the use of muscle relaxants, anxiolytic agents with antireflux therapy. Some patients could require surgical treatment with myotomy. [6] 

The prognosis of DES is a moderate one, due to the reduction of quality of life and a high rate of morbidity associated with the nutritional status of these patients, which can be considerably affected. [2]

Written informed patient consent for publication has been obtained.

Differential Diagnosis List
Diffuse oesophageal spasm with corkscrew oesophagus and cricopharyngeal bar
Diffuse oesophageal spasm
Oesophageal cancer
Oesophageal diverticula
Oesophagitis
Achalasia
Final Diagnosis
Diffuse oesophageal spasm with corkscrew oesophagus and cricopharyngeal bar
Case information
URL: https://www.eurorad.org/case/16549
ISSN: 1563-4086