Icd Case No. 2

ICD CASE No.
2
OUTLINE
• History & PE
• Radiographic image
• Differential diagnosis
• Diagnosis
• Achalasia Discussion
• Prognosis
• Treatment
• Ethical Issues
CC: progressive dysphagia
(solid)
HISTORY
• Ankylosing Spondylitis
1999
• dull chest pain (5/10) that goes up to throat

1 year PTC • Tx: PPI
• episode of vomiting of previously ingested grapes

2 weeks PTC without the characteristic gastric juice (yellow fluid).
PE
• VS: BP 120/80 HR 78 RR 18 T 36.7
• Conscious, coherent, not in distress
• Symmetrical chest expansion, clear breath
sounds
• No palpitations, S1>S2 (apex), S2>S1 (base),
no heaves/lifts/thrills, no murmurs
• No abdominal tenderness
Differential Diagnosis
• Achalasia
– Bird’s beak appearance
• Reflux esophagitis with stricture
– Narrowing is usually higher than the EG junction
– Normal esophageal peristalsis
• Carcinoma
– Only minimal dilation with normal peristalsis
• Diffuse Esophageal Spasm
– "corkscrew" or "rosary bead" appearance
CARCINOMA
STRICTURES DES
ACHALASIA
FINAL DIAGNOSIS
ACHALASIA
DIAGNOSIS
• Barium study
• Esophageal manometry
• Endoscopy with biopsy
• Chest radiograph
• CT scan
• Trans-abdominal ultrasound
DIAGNOSIS
Lateral view: dilated esophagus
Barium study
DIAGNOSIS
• Esophageal manometry
– Usually required for confirmation of diagnosis
• Elevated resting LES pressure
• Incomplete LES relaxation
– Absence of peristalsis
DIAGNOSIS
• Endoscopy
– Must rule out malignancy
– Reveals dilated esophagus with normal mucosa
– Retained fluid/food
DIAGNOSIS
• Chest radiograph
– With severe disease, may readily see the large,
dilated esophagus with air fluid level at the
aortic arch or above
– Stomach bubble frequently absent
DIAGNOSIS
• CT Scan
– Not typically used for diagnosis
– nonspecific and insensitive in the early stages of
achalasia
– Seen as dilated luminal structure with retained
debris and narrowing at level where it enters the
stomach
DIAGNOSIS
• Trans-abdominal Ultrasonography
– homogenous esophageal wall thickening and dilat
ation of the esophagus)
– technique is not yet prevalent in clinical practice.
DISCUSSION
• A rare disease
• loss of ganglion cells w/in myenteric plexus
(1:100,000)
• Idiopathic origin (autoimmune, inflammatory
or hereditary)
• 25-60 y/o
• M=F
DISCUSSION
• Progressive dilatation and sigmoid deformity
of the esophagus w/ hypertrophy of LES
• Increased incidence of esophageal cancer
• S/sx
– dysphagia (solid and liquid) -85-95%
– Regurgitation
– chest pain
– weight loss
– Difficulty belching
DISCUSSION
• PRIMARY achalasia (Idiopathic)
– Unknown cause of inflammatory neuronal
degeneration
• SECONDARY achalasia (pseudoachalasia)
– Recognized pathologic causes of esophageal
motility disorders
• Malignancy (especially gastric cancer) • Sarcoidosis
• Pseudo-obstruction
• MEN, Type 2B
• Neurofibromatosis
• Chagas’ disease • Eosinophilic gastroenteritis
• Juvenile Sjögren’s • Fabry’s disease
• Amyloidosis • Scleroderma
• Chronic idiopathic intestinal
DISCUSSION
• association of the IL23R Arg381Gln polymorphism with

susceptibility to idiopathic achalasia and inflammatory bowel
disease, psoriasis, and ankylosing spondylitis.
De Leon, A. R., et. al., Association between idiopathic achalasia and IL23R gene. 31 MAR 2010
TREATMENT
• Medical management
• Mechanical dilation
• Chemical paralysis (Botulinum toxin)
• Surgical myotomy
TREATMENT
• Medical therapy
– Nitrates, calcium channel blockers (nifedipine)
• Cause smooth muscle relaxation but with limited
success
TREATMENT
• Pneumatic dilation of the LES
– Tears muscle fibers of LES, thus weakening it
• type and diameter of dilator,
• balloon inflation pressure and rate
• duration of inflation
• number of inflations per session
– Good short-term results
– Potential complications of esophageal
perforation (2-6%) and GERD
TREATMENT
• Botulinum toxin injection
– Inhibits release of excitatory acetylcholine from
nerve endings (thus causing lower LES pressures)
– Good short-term results, but long term efficacy
unknown
TREATMENT
• Surgical myotomy
– LES muscle fibers cut
– Laparoscopy becoming more popular
– Good relief of symptoms
– Superior method for achieving better long term
results
– Debate: fundoplication is necessary to prevent
longstanding GERD
PROGNOSIS
• prevalence of treated achalasia is
10.82/100,000
• stable incidence but a rising prevalence
• Survival of achalasia cases is significantly less
than age-matched healthy controls.
ETHICAL ISSUES
• DOUBLE EFFECTS
– An act is seen to have good and bad effects
– In order for the act to be permissible:
• Action must be good itself or at least morally indifferent
ETHICAL ISSUES
ETHICAL ISSUES
ETHICAL ISSUES
ETHICAL ISSUES
THANK YOU 

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ICD CASE No.

• dull chest pain (5/10) that goes up to throat

• episode of vomiting of previously ingested grapes

Lateral view: dilated esophagus

• association of the IL23R Arg381Gln polymorphism with

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