The normal-IRP side of the Chicago algorithm covers everything that is not achalasia or EGJOO. The
sphincter relaxes; the question shifts to what the body is doing. Distal esophageal spasm is a
timing problem (premature contraction). Hypercontractile esophagus is a vigor problem (DCI above
8000). Ineffective motility and absent contractility sit at the failed end. Scleroderma is the
one disorder that breaks the rule, presenting as absent contractility plus a hypotensive LES that
lets reflux through. And EGJOO is the elevated-IRP-with-preserved-peristalsis bucket that v4.0 made
harder to call on purpose, because v3.0 was overcalling it.
The case. A patient with chest pain and intermittent dysphagia. Cardiac workup is negative. Manometry shows
a normal IRP, 30% of swallows with a distal latency of 3.8 seconds, and a normal DCI on the rest.
What is the diagnosis, and what is the first treatment move?
Topics covered
- IRP as the sorter: elevated IRP routes to achalasia or EGJOO; normal IRP opens the body branch
- Distal esophageal spasm: ≥20% premature swallows (DL under 4.5s) on a normal IRP, in a symptomatic patient
- Hypercontractile (jackhammer) esophagus: ≥20% of swallows with DCI above 8000, in a symptomatic patient
- Why distal latency captures spasm and contraction front velocity does not: deglutitive inhibition vs raw wave speed
- Spasm treatment ladder: address contributors first (opioids, GERD, EoE), then calcium channel blockers, nitrates, sildenafil, peppermint oil, neuromodulators
- Type 3 achalasia vs spasm: same body pattern, different IRP, and POEM vs medical therapy follows
- Scleroderma esophagus: absent contractility plus hypotensive LES as a dual hit; Nissen contraindicated, partial wrap or no wrap
- Ineffective esophageal motility (IEM): more than 70% ineffective or at least 50% failed swallows; surgical implications for fundoplication planning
- EGJ outflow obstruction: v4.0 requires both supine and upright IRP elevation, intrabolus pressurization, symptoms, and a confirmatory test
- FLIP distensibility index below 2.0 mm²/mmHg or maximum diameter below 12 mm confirms reduced EGJ opening
- Opioid-induced esophageal dysfunction: mimics type 3 achalasia, EGJOO, and spasm; reverses with taper before any therapy
Key decisions
- Type 3 achalasia and distal esophageal spasm share the body pattern; the IRP separates them. The therapy follows: tailored myotomy for type 3, medical ladder for spasm
- Distal latency under 4.5 seconds identifies spasm; DCI above 8000 identifies jackhammer. Both require symptoms before they become a clinical diagnosis
- Severe IEM and absent contractility separate at 70% vs 100% failed swallows; absent contractility plus hypotensive LES is the scleroderma fingerprint
- EGJOO requires confirmatory imaging, FLIP or timed barium, before invasive intervention. v4.0 deliberately raised the bar
- Any tracing that mimics type 3 achalasia in an opioid-using patient triggers an opioid taper and a repeat manometry before any procedure
- Scleroderma esophagus contraindicates a full 360-degree Nissen; partial wrap or no wrap is the rule because the dysmotility cannot overcome a tight wrap
Related episodes
For the full chapter with MCQs, tables, and primary-guideline references, visit www.boardpearls.com.
Questions or feedback: hello@boardpearls.com.
- (00:00) - Opening: IRP sorts everything
- (02:00) - Spasm and jackhammer share preserved relaxation
- (05:00) - Distal latency and why CFV was retired
- (08:30) - Spasm clinical picture and workup
- (11:30) - Spasm treatment ladder
- (16:00) - Hypercontractile esophagus and the symptom rule
- (19:30) - Scleroderma esophagus and the partial-wrap rule
- (23:00) - Ineffective and absent contractility
- (26:00) - EGJOO and the v4.0 bar
- (30:00) - FLIP and timed barium as the rescue
- (33:00) - Synthesis and what's next
