Nutcracker Esophagus
Nutcracker Esophagus
Nutcracker Esophagus
Pathophysiology
Pathology specimens of the esophagus in patients with nutcracker esophagus show no significant
abnormality, unlike patients with achalasia, where destruction of the Auerbach's plexus is seen. The
pathophysiology of nutcracker esophagus may be related to abnormalities in neurotransmitters or other
mediators in the distal esophagus. Abnormalities in nitric oxide levels, which have been seen in achalasia,
are postulated as the primary abnormality.[2][8] As GERD is associated with nutcracker esophagus, the
alterations in nitric oxide and other released chemicals may be in response to reflux.[9]
Diagnosis
In patients who have dysphagia, testing may first be done to exclude an anatomical cause of dysphagia,
such as distortion of the anatomy of the esophagus. This usually includes visualization of the esophagus
with an endoscope, and can also include barium swallow X-rays of the esophagus. Endoscopy is typically
normal in patients with nutcracker esophagus; however, abnormalities associated with gastroesophageal
reflux disease, or GERD, which associates with nutcracker esophagus, may be seen.[10] Barium swallow in
nutcracker esophagus is also typically normal,[2] but may provide a definitive diagnosis if contrast is given
in tablet or granule form. Studies on endoscopic ultrasound show slight trends toward thickening of the
muscularis propria of the esophagus in nutcracker esophagus, but this is not useful in making the
diagnosis.[11]
Three other criteria for the definition of the nutcracker esophagus have been defined. The Gothenburg
criterion consists of the presence of peristaltic contractions, with an amplitude of 180 mm Hg at any place
in the esophagus.[10][9] The Richter criterion involves the presence of peristaltic contractions with an
amplitude of greater than 180 mm Hg from an average of measurements taken 3 and 8 cm above the lower
esophageal sphincter. It has been incorporated into a number of clinical guidelines for the evaluation of
dysphagia.[9] The Achem criteria are more stringent, and are an extension of the study of 93 patients used
by Richter and Castell in the development of their criteria, and require amplitudes of greater than 199 mm
Hg at 3 cm above the lower esophageal sphincter (LES), greater than 172 mm Hg at 8 cm above the LES,
or greater than 102 mm Hg at 13 cm above the LES.[9][14]
Treatment
People are usually reassured that the disease is unlikely to worsen.
However, the symptoms of chest pain and trouble swallowing may
be severe enough to require treatment with medications, and rarely,
surgery.
Medications
Procedures
Endoscopic therapy with botulinum toxin can also be used to improve dysphagia which stabilizes
unintentional weight loss, but the effect has limited effect on other symptoms, including pain, while also
being a temporary treatment lasting a few weeks.[20] Finally, pneumatic dilatation of the esophagus, which
is an endoscopic technique where a high-pressure balloon is used to stretch the muscles of the LES, can be
performed to improve symptoms, but again no clinical improvement is seen in regards to motility.[21]
In people who have no response to medical or endoscopic therapy, surgery can be performed. A Heller
myotomy involves an incision to disrupt the LES and the myenteric plexus that innervates it. The Heller
myotomy is used as a final treatment option in patients who do not respond to other therapies.[22]
Prognosis
Nutcracker esophagus is a benign, nonprogressive condition, meaning it is not associated with significant
complications.
See also
Esophageal spasm
References
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External links