Corkscrew Esophagus

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Image Source: The New England Journal of Medicine©

An 83-year-old woman came to the gastroenterology clinic with complaints of difficulty swallowing (dysphagia) and regurgitation with each meal. The patient also complained of chest pain after food intake (postprandial chest pain).

The patient had a history of dysphagia for both solids and liquids for the past couple of years. The symptoms had exaggerated in the previous year with a weight loss of 9 kgs in a year.

A corkscrew pattern was seen on barium esophagram (Panel A). Esophagoscopy revealed tight gastroesophageal junction and nonperistaltic spastic contractions (Panel B).

Esophageal manometry showed an increased relaxation pressure of the lower esophageal sphincter, absence of peristalsis, and spastic contractions consistent with spastic achalasia.

The patient was offered endoscopic myotomy, but she declined. Instead, her achalasia was managed with endoscopic injection of botulinum toxin. At a 5-month follow-up, her symptoms had reduced substantially with no regurgitation and only intermittent dysphagia.

Achalasia is an esophageal motility disorder.

In simpler words, when the food pipe fails to push the food to the stomach due to defective contractions and relaxations (peristalsis) of the esophagus and due to impaired relaxation of the lower esophageal sphincter. These abnormalities create a functional blockage in the passage of the food, thus dysphagia.

Achalasia is a rare disorder that can, though, affect any age, but it is mostly seen in individuals between 20 and 60 years. Children are usually spared.
Less than 5% of cases have been reported in children.

Symptoms of achalasia develop insidiously and include gradually progressive dysphagia to both solids and liquids. Other clinical manifestations include regurgitation of undigested food, mild to moderate weight loss, chest pain, indigestion, etc.

Chest pain may occur either postprandially or spontaneously. Regurgitation is a potential health hazard, particularly if nocturnal, as it may lead to pulmonary aspiration. Another important concern is weight loss. Severe weight loss is a red flag, especially in the elderly, and when coupled with rapidly progressive dysphagia, as it can be a sign of cancerous growth presenting as achalasia.

Esophageal manometry is the gold standard investigation for achalasia.

The classical features of achalasia seen on manometry are

  • Increased resting lower esophageal sphincter pressure
  • Impaired relaxation of the LES in response to swallowing
  • Absence of peristalsis

Other investigation options include a barium swallow and endoscopy.
Barium swallow shows characteristic bird beak appearance of the lower esophagus. Also, it reveals a dilated esophagus, and the contrast material takes more time than usual to pass through the esophagus because of impaired LES relaxation.

Image Source: ResearchGate

Management options include

  • Graded Pneumatic dilation
  • Laparoscopic myotomy
  • Intrasphincteric botulinum toxin injection to block acetylcholine at LES
  • Medical treatment with calcium channel blockers or nitrates to relax the LES. This option is the least helpful and only suitable for elderly patients who can’t undergo any other treatment methods.

The bottom line to diagnose achalasia in time and select an individualized management approach as directed by the patient’s age, symptomatology, and comorbidities.

References

Kristle Lee Lynch, M. P. (2019, July). Achalasia. Retrieved from MSD Manual: https://www.msdmanuals.com/professional/gastrointestinal-disorders/esophageal-and-swallowing-disorders/achalasia

Samuel Han, M. a. (2020, April 30). Corkscrew Esophagus in Achalasia. Retrieved from The New England Journal of Medicine: https://www.nejm.org/doi/full/10.1056/NEJMicm1911516

Vaezi MF, Pandolfino JE, Vela MF. ACG clinical guideline: diagnosis and management of achalasia. Am J Gastroenterol. 2013 Aug. 108(8):1238-49; quiz 1250.

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Dr. Arsia Parekh
Dr. Arsia Hanif has been a meritorious Healthcare professional with a proven track record throughout her academic life securing first position in her MCAT examination and then, in 2017, she successfully completed her Bachelors of Medicine and Surgery from Dow University of Health Sciences. She has had the opportunity to apply her theoretical knowledge to the real-life scenarios, as a House Officer (HO) serving at Civil Hospital. Whilst working at the Civil Hospital, she discovered that nothing satisfies her more than helping other humans in need and since then has made a commitment to implement her expertise in the field of medicine to cure the sick and regain the state of health and well-being. Being a Doctor is exactly what you’d think it’s like. She is the colleague at work that everyone wants to know but nobody wants to be. If you want to get something done, you approach her – everyone knows that! She is currently studying with Medical Council of Canada and aspires to be a leading Neurologist someday. Alongside, she has taken up medical writing to exercise her skills of delivering comprehensible version of the otherwise difficult medical literature. Her breaks comprise either of swimming, volunteering services at a Medical Camp or spending time with family.

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