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ISPUB.COM The Internet Journal of Gastroenterology
Volume 7 Number 2
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Nutcracker Esophagus With Epiphrenic Diverticulae: Case
Report
S Arulprakash, P Rathnakar Kini, T Pugazhendhi, S Jeevankumar
Citation
S Arulprakash, P Rathnakar Kini, T Pugazhendhi, S Jeevankumar. Nutcracker Esophagus With Epiphrenic Diverticulae:
Case Report. The Internet Journal of Gastroenterology. 2008 Volume 7 Number 2.
Abstract
Nutcracker esophagus (NE) is a manometric pattern that is commonly seen in patients with noncardiac chest pain and/or
dysphagia with normal esophageal peristalsis. We report a rare association of multiple epiphrenic esophageal diverticulae of
distal esophagus with NE.
INTRODUCTION
Primary esophageal motility disorders comprise various
abnormal manometric patterns which usually present with
dysphagia or chest pain. Unlike achalasia other disorders
like diffuse esophageal spasm (DES) and NE , have no well
defined pathology and could represent a range of motility
abnormalities associated with subtle neuropathic changes,
gastresophageal reflux and anxiety states. Manometric
patterns of NE poorly correlate with symptoms and response
to medical or surgical therapy. Epiphrenic diverticulae of
distal esophagus are said to occur with motility disorders
like DES, it's rarely reported with NE.
CASE REPORT
A 62-year-old male presented to our department because of
intermittent non progressive dysphagia for the past 2 years.
The patient reported an increasing frequency and duration of
the symptoms which were independent from food intake,
medication. There was no history of recurrent heart burn,
nasal regurgitation, nocturnal cough or stale food vomiting.
Barium swallow was performed which showed multiple
diverticulae in lower end of esophagus. (Fig.1)
Figure 1
Figure 1: Endoscopic view of the diverticulae of distal
esophagus above the OG Jn.
We performed an upper GI endoscopy revealed multiple
outpouchings in lower third of esophagus. (Fig.2)
Nutcracker Esophagus With Epiphrenic Diverticulae: Case Report
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Figure 2
Figure 2: Barium esophagogram showing epiphreinic
diverticulum
Esophageal manometry confirmed the presence of a
nutcracker esophagus, defined by high-amplitude
contractions of the distal esophagus (Fig.3). The patient was
treated with nifedipine 10mg before each meal. The
symptoms severity decreased but manometric pattern
persisted on 6 month follow up.
Figure 3
Figure 3: Manometry showing high amplitude contractions
(> 180 mm Hg)
DISCUSSION
Nutcracker esophagus (NE) was introduced by Benjamin
and Castell in 1980. Other terms used are Supersqueezer and
hypertensive peristalsis. The manometric feature proposed
for a diagnosis of nutcracker esophagus (1) is a mean distal
esophageal peristaltic wave amplitude greater than 2
standard deviations above normal (i.e., greater than 180 mm
Hg) (2) in a symptomatic patient (measured as the average
amplitude of 10 swallows at two recording sites positioned 3
and 8 cm above the LOS). Peristaltic contractions of long
duration (>6 sec) are found commonly but are not required
for manometric diagnosis of nutcracker esophagus (3).
NE has been described in 27%-48% of patients with
noncardiac chest pain (3). This manometric pattern is often
unassociated with pain. The underlying mechanism for chest
pain is obscure. In some patients there is coexisting acid
reflux, in others, an underlying psychological problem has
been observed (4). Transition of NE to other motility
disorders raises speculation that it lies in the beginning of
spectrum of motility disorder that ends in achalasia.
Chest pain is the predominant symptom (90%) and
dysphagia is less common. (3) Symptoms intensity,
frequency, and location vary. Most patients are evaluated for
chest pain syndrome and referred to gastroenterologist after
ruling out cardiac disease. Associated symptoms include
depression, anxiety, and somatization; but not apparent on
routine evaluation. Physical examination is invariably
Nutcracker Esophagus With Epiphrenic Diverticulae: Case Report
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normal.
All patients have normal peristalsis hence barium studies and
radionuclide transit studies are normal (5). Association of
epiphrenic diverticulae has not been reported with NE which
was present in our case. Epiphrenic diverticula are rare and
occur in the terminal 10- 15 cm of the esophagus these
pulsion diverticula often occur secondary to motility
disorders of the esophagus, DES (24%) or achalasia (15%).
It's rarely reported along with NE. (6). Symptoms are often
due to motor disorder, rather than the diverticulum itself.
Most patients with diverticulum require no specific therapy.
Surgery is usually reserved for people with significant
progressive dysphagia or recurrent aspiration pneumonia. (7)
Diagnosis of NE requires manometry. Various criteria for
diagnosis was used by various authors, most experts use the
above mentioned criteria. Long term manometric follow up
failed of show consistent findings. Evolution of NE to
diffuse esophageal spasm and achalasia has been reported.
Management includes reassurance psychological
intervention. Suggested approach is a trial of anti-reflux
treatment followed by nitrates (ISDN 5-10 mg SL), calcium
channel blockers (Nifedipine 10-30 mg QID), visceral
analgesics (Imipramine 50 mg HS), sedatives (Trazadone,
Alprazolam). Diltiazem 60-90- mg TID has been tried with
some success. Nonresponder occasionally respond to
botulinum toxin injection and myotomy (8).
We conclude that nutcraker esophagus is a manometric
abnormality unusually associated with pulsion diverticulum
of esophagus. Management of NE and epiphrenic
diverticulum must be individualized based on severity of
symptoms.
CORRESPONDENCE TO
Dr. Arulprakash.SPlot. No: 119 A, First main road, Second
cross street, Lakshmi nagar extension, Porur, Chennai:
600116. Ph: 09962033234 E-mail: drarulaash@yahoo.co.in
References
1. Spechler et al. Classification of esophageal motility
abnormalities. Gut 2001; 49; 145-151.
2. Ritcher JE, et al. Esophageal manometry in 95 healthy
adult volunteers. Dig Dis Sci 1987; 32:583.
3. Katz PO, Castell JA. Nonachalasia motor disorders. In:
Castell DO, Richter JE, eds. The esophagus, 3rd ed.
Philadelphia: Lippincott Williams & Wilkins, 1999:215-34.
4. Clouse RE, Lustman PJ. Psychiatric illnesses and
contraction abnormalities of the esophagus. N Engl J Med
1983; 309: 1337-92.
5. de Caestecker J S, Blackwell J N et al. Clinical value of
radionuclide esophageal transit measurement. Gut 1986; 27;
659-666
6. Dhiren Nehra, Reginald V. et al. Physiologic Basis for the
Treatment of epiphrenic diverticulum. Ann. Surg. 2002; 235;
3: 346-354.
7. Clark S. C, Norton S. A et al.Esophageal epiphrenic
diverticulum: an unusual presentation and review. Ann R
Coll Surg Engl. 1995; 77(5): 342-345.
8. Tutuian R, Castell DO. Esophageal motility disorders:
modern management. Curr Treat Options Gastroenterol.
2006 Jul; 9(4):283-94.
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Author Information
S. Arulprakash
Resident, Department of Digestive Health and Diseases, Kilpauk Medical College Hospital
P. Rathnakar Kini
Resident, Department of Digestive Health and Diseases, Kilpauk Medical College Hospital
T. Pugazhendhi
Department of Digestive Health and Diseases, Kilpauk Medical College Hospital
S. Jeevankumar
Department of Digestive Health and Diseases, Kilpauk Medical College Hospital
