Introduction
Medical problems related to oesophagus are frequently encountered in clinical practice. Oesophageal disorders can range from common problems like gastro-oesophageal reflux disease (GORD) to rarer but serious problems like oesophageal cancer. Among the oesophageal-related complaints, dysphagia is the most alarming red-flag that warrants urgent assessment with upper GI endoscopy, with or without barium swallow. In this series of case reports, we selected four different cases of dysphagia where routine oesophago-gastrodudenoscopy (OGDS) has failed to confirm the diagnosis. In these cases, barium swallow, although simple and inexpensive, was useful to reach a diagnosis.
Case 1: Cardiac or oesophageal?
Brief clinical history
A 79-year-old lady complained of dysphagia, retrosternal burning pain and chest tightness for 2 years duration. Her symptoms were triggered by swallowing but not by exertion. She’s had type 2 diabetes for 20 years. She had her OGDS done last year and was diagnosed as reflux esophagitis with GORD. But her symptoms were not relieved with treatment for GORD. Her vital signs were normal, HbA1c was 7.5%, and serum Creatinine was 76 microgram/dL. Other tests were also normal. ECG showed ST and T changes in anterior leads (V1-4). Chest x ray was also normal.
Medications
She was on metformin 1 bd and Gliclazide 80mg bd, amlodipine 5 mg od, Rabeprazole plus domperidone od, atorvastatin 20 mg OD and aspirin 75mg od.
Clinical reasoning and problem-solving
As her symptoms were more related to swallowing, it is unlikely to be of cardiac origin. Since oesophageal pathology was still suspected with no significant abnormality on OGDS, barium swallow was performed. The test revealed abnormal spiral appearance of mid and lower esophagus, diagnosed as Corkscrew oesophagus (Diffuse oesophageal spasm). (See the Figure 1)
Fig 1: Barium swallow showing abnormal spiral appearance due to diffuse esophageal spasm
Case2: Organic or psychosomatic?
Brief clinical history
A 23-year-old lady presented with the complaint of difficulty in swallowing, feeling that the food was stuck at lower end of her throat. She said there was no difference in swallowing of solids and liquids. She had to do self-induced vomiting so that she could swallow food afterwards, leading to weight loss despite good appetite, and insomnia due to anxiety from inability to eat anything. Two years ago, she had upper GI Endoscopy (OGDS) which revealed only mild gastritis, without features of esophageal cancer or stricture. Other health check-ups were all within normal limits.
Medication history
She was taking esomeprazole, domperidone, antacids, alprazolam and escitalopram. Her symptoms were not relieved by these medications.
Clinical reasoning and problem solving
The main differential diagnoses of this young lady were (1) anorexia nervosa as she had marked anxiety and induced vomiting and (2) globus hystericus due to anxiety neurosis as she had a sense of obstruction in throat together with the history of normal OGDS finding.
But she said she was concerned about her weight loss. This contrasted with the intense fear of weight gain, a typical finding in aneroxia nervosa. Again, insomnia and anxiety occurred only after her dysphagia as she became worried about her disease (secondary but not primary). Significant organic cause of dysphagia that can be easily missed by OGDS in young patients is achalasia cardia. So, barium swallow X-ray was done. Her barium esophagogram showed esophageal dilation with poor emptying, absent peristalsis, and narrowing of the distal esophagus, referred to as “bird’s beak appearance”. In figure 2, we could see her esophagus was almost full. She might have been inducing vomiting before meals to relieve the oesphagus loaded with old foods.
Fig 2: Mega-oesophagus with air-fluid level and terminal bird-beak’s appearance
Case 3: Pale and dysphagic
Brief clinical history
A 34-year-old lady complained of difficulty in swallowing and felt something stuck in her throat on swallowing. It was particularly worse with solid food. It was not severe but persistent. She said she had no significant weight loss, but she was slightly breathless on exertion. She had no anorexia nor change of bowel habits, but she did have a significant history of menorrhagia. Barium Swallow test and some blood tests were ordered on this patient.
Fig 3: Barium swallow showing esophageal web formation in proximal esophagus
Fig 4: conjunctival pallor of the above patient (with consent from the patient)
Clinical reasoning and problem solving
A young lady with history of long-standing menorrhagia with confirmed iron-deficiency anemia now presented with dysphagia due to esophageal web formation. So, this is a case of Plummer-Vinson syndrome (PVS) (also called Paterson-Kelly syndrome and sideropenic dysphagia)
Case 4: “Something in my throat”
Brief clinical history
A 72-year-old man presented with difficulty in swallowing food. He felt the food stuck at his throat when he swallowed. Sometimes he noticed regurgitation of old foods He denied sore throat, nor pain on swallowing (odynophagia). There were no weight loss nor loss of appetite. These symptoms had been persistent for two years.
Recently he consulted with an ENT surgeon and routine throat examination was found to be normal, so laryngoscopy was planned for further assessment. While he was waiting for laryngoscopy, he was advised by our medical team to do barium swallow and some blood tests first. The barium swallow revealed a diverticulum at the upper esophagus (see Figure 5). The diagnosis of Zenker’s diverticulum was made and he was referred to a gastroenterologist for further management.
Fig 5: small barium-filled out pouching in cervical esophagus in red circle.
Clinical reasoning and problem-solving
This patient presented with long-standing dysphagia of two-year duration without other alarm features such as weight loss, anemia or anorexia. So, malignant obstruction was less likely. Although his dysphagia was mild (no weight loss even with 2 years’ duration), he did have some regurgitation of food, which increased the possibility of an out pouching in pharyngo-esophageal region.
Literature review
Approach to dysphagia
Dysphagia has a range of differential diagnoses, from the most feared esophageal cancer to benign causes. It must first be differentiated from odynophagia (painful swallowing) and psychogenic globus sensation in throat. True or organic dysphagia can be divided into two groups: oropharyngeal and oesophageal.1
Oesophageal dysphagia is subdivided into mechanical and dysmotility disorders.1 Mechanical obstruction is characterized by persistent and progressive dysphagia that usually gets worse with solid than liquid foods. Benign strictures tend to take longer clinical course without other red-flag symptoms, while malignant strictures progress rapidly in a matter of weeks or several months.
In dysmotility problems, dysphagia is intermittent and difficulty in swallowing of both solids and liquids is usually seen. The clinical course may span over years, and some patients develop specific techniques like standing up while eating to help the food down. Most motility disorders and some mechanical dysphagia (like small esophageal webs) can result in normal OGDS, but the diagnosis is usually apparent on barium study.1
Fig 7: Diagnostic flow-chart of dysphagia1
Corkscrew oesophagus
Corkscrew esophagus is a rare esophageal motility disorder characterized by high amplitude peristaltic contractions in the distal esophagus.2 Barium swallow can reveal the corkscrew appearance in distal esophagus like a winding staircase. (Figure 1)
The typical clinical symptoms include chest pain, dysphagia or gastroesophageal reflux disease (GORD). To date, the pathogenesis of corkscrew esophagus is still obscure. This rare condition occurs predominately in the aged; therefore, some researchers propose that aging may result in physiological deterioration of esophageal motility. Recent studies revealed the contributing role of GORD and psychiatric comorbidity may induce this disease.
Manometry of esophagus is the gold standard to confirm esophageal spasm. However, it is not an initial test because of its sophisticated technical needs, so usually reserved as a pre-procedure assessment tool.
There are no established guidelines for the management of diffuse esophageal spasm. Treatment should be individualized depending on the patient’s symptoms. Calcium channel blockers and sublingual nitroglycerin or longer-acting agents, such as isosorbide dinitrate taken orally before meals are used commonly. Trazodone and imipramine have been effective in controlled clinical trials in relieving chest pain. Some preliminary studies showed some benefits with botulinum toxin injection, phosphodiesterase inhibitors (e.g., sildenafil) and bethanechol.
Achalasia cardia
Achalasia cardia is esophageal dysmotility disorder of unknown etiology with hypertonic lower esophageal sphincter (LES), which fails to relax on swallowing and failure of normal esophageal peristalsis, leading to progressive dilatation or mega-esophagus.3 Failure of LES relaxation is thought to be due to defective nitric oxide release by inhibitory neurons in LES.
Endoscopic pneumatic dilatation or injection of barium toxin into LES is usually first choice of intervention. Surgical myotomy (Heller’s operation) is usually reserved for resistant cases. Peroral endoscopic myotomy (POEM) is a newer alternative approach to open operation. All the above invasive procedures can lead to post-operative GORD, so long-term PPI therapy is usually indicated after such procedures.
It is estimated that there are 36%–50% false negative detection rate for achalasia in community hospitals.4 The diagnosis of achalasia is suggested by clinical features and confirmed by further diagnostic tests, such as esophagogastroduodenoscopy, barium swallow and manometry. Recent advances in diagnostic methods, including high resolution manometry might even help predicting outcome or selected more appropriate procedures to treat the disease.5 The barium x-rays have an accuracy over 90% in diagnosing achalasia cardia.6 However, oesophageal manometry is the gold standard for the diagnosis of achalasia cardia.
Plummer-Vinson syndrome (PVS)
Plummer-Vinson syndrome9 (PVS) is a rare condition characterized by the classic triad of dysphagia, iron-deficiency anemia, and esophageal webbing. Although it’s a benign condition by itself, having long-standing Plummer-Vinson syndrome may increase the risk of developing esophageal cancer. Main management is to treat IDA with iron supplements and treat the cause of blood loss because dysphagia resolves with iron supplementation in many patients.
OGDS is less likely to detect esophageal webs compared with barium swallow. Role of OGDS here is to exclude esophageal cancer and for endoscopic dilatation for rapid symptomatic relief. So, in this case OGDS should follow barium swallow to exclude the must-not-miss oesophageal cancer and for therapeutic dilatation.
Zenker’s diverticulum
Zenker’s diverticulum, also known as pharyngoesophageal diverticulum, is a pharyngeal pouch that forms where the lower part of the throat and the upper part of the esophagus meet.7 It is the most common type of diverticula of the esophagus.
A person with Zenker’s diverticulum may experience difficulty swallowing, bad breath, chronic cough, a feeling of excessive phlegm or mucus in the throat, sensation of a lump in the throat, gurgling sound in throat immediately after swallowing, regurgitation of food (often hours after a meal) and weight loss (gradual over long duration- unlike rapid and profound weight loss of oesophageal cancer).
The most effective method for diagnosing esophageal diverticula is a barium swallow. As the barium solution moves down the esophagus, it fills the pouches so they can be easily seen on an X-ray.
Cases of esophageal diverticulum that cause minor symptoms can be treated through lifestyle changes, such as eating a bland diet, chewing food thoroughly, and drinking plenty of water after meals. Surgical treatment is recommended for highly symptomatic patients. Since outpouchings are herniation through crico-pharyngeus muscle, definitive treatment usually involves myotomy of the cricopharyngeal muscle. Myotomy may be pursued through either open surgical or endoscopic techniques. The choice between the different approaches depends on local expertise and preferences.
Conclusion
In this case series, we highlighted the important role of barium swallow study for the diagnosis of dysphagia. Most of the time, clinicians tend to do endoscopy as the first test in the evaluation of dysphagia due to its advantage of being able to obtain tissue biopsy if required. On the other hand, barium swallow is usually regarded as an old man’s method and easily overlooked. In a patient with organic dysphagia, if endoscopy reveals no pathology or only minor changes which could not by itself explain patient’s symptoms (such as reflux oesophagitis in case 1 or gastritis in case 2), we should not forget motility disorders of esophagus. While endoscopy is the gold standard for intra-luminal visualization, it can easily miss the muscular mural /motility disorders of esophagus. Simple and easy-to-perform barium swallow study is highly useful in this respect.
References
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- Bell D. Corkscrew oesophagus. In: Radiopaedia.org. Radiopaedia.org; 2021.
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- Booy JD, Takata J, Tomlinson G, Urbach DR. The prevalence of autoimmune disease in patients with esophageal achalasia. Diseases of the Esophagus. 2012 Apr;25(3):209–13.
- Laurino-Neto RM, Herbella F, Schlottmann F, Patti M. Evaluation of esophageal achalasia: From symptoms to the chicago classification. Vol. 31, Arquivos Brasileiros de Cirurgia Digestiva. Colegio Brasileiro de Cirurgia Digestiva; 2018.
- Ott D, Richter J, Chen Y, Wu W, Gelfand D, Castell D. Esophageal radiography and manometry: correlation in 172 patients with dysphagia. American Journal of Roentgenology. 1987 Aug 1;149(2):307–11.
- Bizzotto A, Iacopini F, Landi R, Costamagna G. Zenker’s diverticulum: exploring treatment options. Acta Otorhinolaryngol Ital. 2013 Aug;33(4):219–29.
Author Information
Than Than Aye1, Hpone Pyae Tun2, Hsu Yee Hnin3
- Dr.Med.Sc. (General Medicine), Consultant Endocrinologist and Professor Emeritus at University of Medicine 2, Yangon
- M.Med.Sc. (Internal Medicine), General Physician
- M.B.,B.S., MRCP, Postgraduate Student
