DDSEP Motility VII 2024

Question 1

What are the most typical findings of MEN IIB?

Answer 1

Megacolon and constipation are most common GI manifestation and typically present during early childhood.

Question 1

What disease process will cause intestinal neuronal dysplasia characterized by hyperganglionosis?

Answer 1

MEN IIB- in this you see GI complications long before other complications (such as medullary carcinoma of thyroid 100% and pheo’s 60%)

Question 2

What is another disease which may cause chronic constipation and megacolon which has intestinal neuronal dysplasia?

Answer 2

Neurofibromatosis. Can cause mass like submucosal neurofibromas which can even obstruct.

Question 3

What type of Achalasia is this?

Answer 3

Type III

This is “spastic” achalasia/distal esophageal spasm in which you have a reduced distal latency < 4.5 seconds without peristalsis and DCI > 450 in >20% of swallows.

May be mixed with panesophageal pressurization

Question 4

What achalasia subtype is this?

Answer 4

Type II

Here you have pan esophageal pressurization with 20% or more of swallows with esophageal pressurization and DCI should not be calculated. 100% failed peristalsis.