Case Study
PART 1 Oral cavity, pharynx and esophagus
GI Motility online (2006) doi:10.1038/gimo82
Published 16 May 2006
A patient with chronic severe oropharyngeal dysphagia
Gary D. Gramigna, M.S., C.C.C.
and Raj K. Goyal, M.D.
About the contributors
Case description
The patient is a young man who suffered a traumatic brain injury one and a half years ago while skiing, and was found to have contusions of the cerebellum, brainstem, and medulla, resulting in quadriplegia and multiple cranial nerve palsies, including right lower motor neuron facial paralysis as well as a right vocal cord paralysis.
The patient was unable to swallow, and was a severe aspiration risk. He received a tracheostomy and a gastrostomy tube for nutritional support. The tracheostomy tube was removed 4 months later. He also received a right vocal cord implant for the paralyzed right vocal cord. Other surgery included a right upper lid silicone implant to facilitate blink. The patient suffered no cognitive impairment.
Over a period of 1 year after the accident the patient had three videofluoroscopic swallow studies (VFSS), all of which reported severe dysphagia, no swallow response, and minimal to no soft palate elevation, cough ability, or opening of the upper esophageal sphincter (UES).
The patient had esophageal dilation of the UES with rubber dilators on two occasions but without any improvement in swallowing or handling secretions. He remains dependent on gastrostomy tube (G-tube) feeding. The patient also reported receiving swallow treatments of electrical stimulation (Vital Stim) and Deep Pharyngeal Neuromuscular Stimulation (DPNS) also without any benefit.
A repeat VFSS, 6 months ago, had shown increased movements of soft palate and pharyngeal wall, with attempted swallows as well as reflexive cough in response to aspiration. The patient was deemed to be recovering the swallow reflex. A behavioral home program of facilitating techniques to strengthen the swallow muscles (Shaker exercise, lingual resistance exercise); heighten swallow sensitivity using tactile-thermal application, sour bolus, capsaicin; and enhancing swallow movements (Mendelsohn maneuver) was undertaken. These treatments had limited affect on the patient's swallowing difficulty.
He now presented for reevaluation and consideration of further therapeutic measures and Botox injections in the UES. At this time, neurology evaluation revealed deficit of cranial nerves V, VII (lower motor neuron), IX, X, and XII, with mild ataxia but no spasticity. Esophagogastroduodenoscopy showed easy passage of the endoscope through the UES and grade 4 esophagitis with no strictures, no ulcerations or mass.
A repeat VFSS was performed (Video 1).
Q1. What is wrong with the oral preparatory phase?
Q2. What muscles and cranial nerves are involved in oral preparatory phase of swallowing?
Q3. Does the patient have premature spill of barium into the pharynx?
Q4. Is a swallow response present in this patient?
Q5. Does the patient have normal velopharyngeal closure or nasal regurgitation?
Q6. What nerves and muscles are involved in velopharyngeal closure?
Q7. Does this study show tracheal aspiration?
Q8. What mechanisms normally prevent aspiration?
Q9. Enumerate the mechanistic abnormalities that may be predisposing to aspiration in this patient.
Q10. What is the evidence of pharyngeal paresis?
Q11. What muscles and nerves are responsible for pharyngeal propulsion?
Q12. Does the UES open in this study?
Q13. What are the unique features of the upper esophageal sphincter (UES)? How does this differ from the closure of the lower esophageal sphincter (LES)?
Q14. What is the difference between opening and relaxation? What is the mechanism of UES opening? How do opening and relaxing of the upper esophageal sphincter (UES) differ from that in the lower esophageal sphincter (LES)?
Q15. What muscles and nerves are responsible for UES opening?
Q16. What is the neural deficit that is responsible for the swallowing difficulty in this patient?
Q17. What diagnostic and therapeutic options would you consider?
Q18. What is Botox? Is it likely to be useful in this case?