Esophageal Motor Disorders

Question 1

Swallow Physiology

Answer 1

• Food bolus in pharynx stimulates swallow center in the medulla. 0.15 seconds.
• Vagal reflexes kick in: vocal cord closure, larynx rises, epiglottis close; also UES relaxes. 0.6 seconds.
• Pharyngeal clearance with swallowing. <1.0 seconds
• Respiration resumes

Question 2

Prenatal Sucking/Swallowing

Answer 2

• Palate complete 12th week
• Pharyngeal swallow: 10-12 weeks.
• True suckling: 18-24 weeks
• Sustain nutrition orally: 34-37 weeks

Question 3

Preterm infant feeding

Answer 3

• Immature oral sensorimotor skills: less efficient than term infants
• Poor endurance: cannot maintain or continue to feed for long periods of time.
• Frequent state changes.

Question 4

Vagally evoked peristalsis in straited muscle of the esophagus

Answer 4

• upper third of the esophagus is striated muscle. Innervated by nucleus ambiguous.
• middle third of the esophagus is a mixture of striated and smooth muscle.
• lower third of the esophagus is smooth muscle.
• smooth muscle is innervated by dorsal motor nucleus of the vagus nerve. it is the postganglionic neuron which releases NO/VIP/ATP (inhibitory) or Ach/SP (excitatory) to innervate smooth muscle.

Question 5

vagal efferents to esophageal smooth muscles

Answer 5

-more inhibitory neurons in proximal esophagus compared to distal esophagus

Question 6

Cricopharyngeal achalasia

Answer 6

• Feeding problems with nasal regurgitation and aspiration: at birth.
• Barium swallow shows a bar in the region of the cricopharyngeal muscle
  -Treatment: transcervical CP myotomy, endoscopic myotomy, botox injection or balloon distension
• some patients are asymptomatic.

Question 7

Cricopharyngeal Achalasia Manometry

Answer 7

Question 8

Normal Esophageal Manometry

Answer 8

Question 9

Normal Esophageal Manometry

Answer 9

Question 10

Achalasia Manometry

Answer 10

Question 11

Achalasia diagnosis

Answer 11

Type 1 Achalasia: IRP greater than or equal to upper limit or normal AND absent peristalsis, no relaxation of LES.
Type 2: no relaxation of LES, and green column: pan esophageal pressurization. -usually better outcomes with either pneumatic dilation or Heller myotomy.
- Achalasia variant: EGJ outflow obstruction: IRP greater than or equal to upper limit or normal AND some instances of intact or weak peristalsis.

Question 12

Achalasia pathogenesis

Answer 12

Infection or toxic insult induces inflammation –> Interferon gamma release –> induces class I antigen expression. draws cytotoxic T cells, leads to destruction of the neurons.

Question 13

Achalasia early disease

Answer 13

• minimal esophageal dilation
• inflammation of the myenteric plexus, without a decrease in ganglion cells (type II). Correct answer is early achalasia.
  Can progress to late disease: esophageal dilation, reduction in the ganglion cell # and a decrease in varicose nerve fibers in the myenteric plexus (progression to type 1)

Question 14

Triple A or ALlgrove syndrome

Answer 14

-Alacrima: cries but no tears, this is present from birth.
- Adrenocorticotrophic hormone insensitivity (hypoglycemia)~5 years of age
-Gene for Tripe A syndrome: 12q13.

Question 15

Rozycki Syndrome

Answer 15

• Achalasia associated with AR deafness, short stature, vitiligo, muscle wasting.

Question 16

Other associations of Achalasia

Answer 16

Chagas’ disease, Down syndrome, Sarcoidosis, Hirschsprung’s disease, Pyloric stenosis, Paraneoplastic syndromes, Hodgkin’s disease

Question 17

Trick question re Scleroderma

Answer 17

Question: patient has typical skin manifestations of scleroderma and patient has dysphagia, the patient does NOT have achalasia, what they have is weak LES and weak peristalsis.

Question 18

Scleroderma manometry

Answer 18

Question 19

Achalasia Treatment

Answer 19

• Laparoscopic Heller Myotomy
• Pneumatic dilation
• Botox injection of GEJ (temporizing)
• medication: isosorbide dinitrate or nifedipine.

Question 20

Dilation vs Surgery

Answer 20

Results of surgical and pneumatoic dilation are similar.
Complications: post operative GERD.

Question 21

Esophagram in Achalasia

Answer 21

Question 22

POEM

Answer 22

Per Oral Endoscopic Myotomy -new surgical technique.

Question 23

Hiatal hernia: two bands of pressurization

Answer 23

Question 24

Jackhammer Esophagus

Answer 24