Esophageal Motor Disorders Flashcards
Swallow Physiology
- 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
Prenatal Sucking/Swallowing
- Palate complete 12th week
- Pharyngeal swallow: 10-12 weeks.
- True suckling: 18-24 weeks
- Sustain nutrition orally: 34-37 weeks
Preterm infant feeding
- 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.
Vagally evoked peristalsis in straited muscle of the esophagus
- 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.
vagal efferents to esophageal smooth muscles
-more inhibitory neurons in proximal esophagus compared to distal esophagus
Cricopharyngeal achalasia
- 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.
Cricopharyngeal Achalasia Manometry
Normal Esophageal Manometry
Normal Esophageal Manometry
Achalasia Manometry
Achalasia diagnosis
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.
Achalasia pathogenesis
Infection or toxic insult induces inflammation –> Interferon gamma release –> induces class I antigen expression. draws cytotoxic T cells, leads to destruction of the neurons.
Achalasia early disease
- 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)
Triple A or ALlgrove syndrome
-Alacrima: cries but no tears, this is present from birth.
- Adrenocorticotrophic hormone insensitivity (hypoglycemia)~5 years of age
-Gene for Tripe A syndrome: 12q13.
Rozycki Syndrome
- Achalasia associated with AR deafness, short stature, vitiligo, muscle wasting.
Other associations of Achalasia
Chagas’ disease, Down syndrome, Sarcoidosis, Hirschsprung’s disease, Pyloric stenosis, Paraneoplastic syndromes, Hodgkin’s disease
Trick question re Scleroderma
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.
Scleroderma manometry
Achalasia Treatment
- Laparoscopic Heller Myotomy
- Pneumatic dilation
- Botox injection of GEJ (temporizing)
- medication: isosorbide dinitrate or nifedipine.
Dilation vs Surgery
Results of surgical and pneumatoic dilation are similar.
Complications: post operative GERD.
Esophagram in Achalasia
POEM
Per Oral Endoscopic Myotomy -new surgical technique.
Hiatal hernia: two bands of pressurization
Jackhammer Esophagus
