Struck Dysphagic
Article Outline
- Answer to the Clinical Challenges and Images in GI Question: Image 5: Struck Dysphagic
- References
- Copyright
Question: An 85-year-old woman presented with a 9-month history of cramping chest pain and dysphagia for liquids and solids. Initial weight loss from 65 to 51 kg stabilized on a self-imposed diet of nutritious soup. The patient was otherwise in good health except for regular episodes of dizziness on standing. She was not taking medications. Medical help had not been sought earlier because the patient was the primary caregiver for a disabled husband in their isolated farmhouse. The patient reported that her symptoms commenced after she had been struck by lightening during a fierce electrical storm. A shining sphere of light floated into the house as she opened the front door, planning to close the farm gates that were rattling in the wind. A moment later, the ball lightening exploded and she was thrown across the room and left temporarily unconscious. From that moment on, she was unable to swallow normally.
Routine hematology and biochemistry blood tests were normal. During the examination, orthostatic hypotension was documented with a >20 mmHg drop of blood pressure accompanied by near syncope on 2 occasions. An electrocardiogram revealed sinus rhythm without reactive tachycardia, suggesting autonomic dysfunction as a cause of her symptoms. Upper gastrointestinal endoscopy revealed no obstructive lesion and no mucosal disease. Uncoordinated contractions with mild delay of bolus transport were observed on barium esophagram (Figure A). High-resolution manometry was performed to establish the diagnosis. In a vivid case of nature imitating art, the unusual appearance of the spatiotemporal plot was reminiscent of an electrical storm (Figure B). What is the diagnosis? What is the most likely etiology?
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Answer to the Clinical Challenges and Images in GI Question: Image 5: Struck Dysphagic
High-resolution manometry shows rapid anterograde and retrograde contractions in the distal esophagus with incomplete relaxation of the lower esophageal sphincter. Prolonged spasm with dramatic shortening of the esophagus was observed on occasion. Multiple, repeated swallows failed to inhibit contractile activity completely and the intraesophageal pressure was elevated, indicating bolus retention (Figure C). The diagnosis of vigorous achalasia was made.1
Ball lightening is among nature's rarest and most mysterious spectacles. The formation of stable spheres of electrical energy during storms that move about and can even enter buildings before discharging has been reported on many occasions (Figure D), although its existence has often been questioned. Support for the reality of this phenomenon has been provided recently by production of ‘ball lightening’ under laboratory conditions by the Max Plank Institute in München, Germany.
Lightening injuries include burns, cardiac dysrhythmia, and neurologic injuries. Autonomic nervous system dysfunction may occur, including keraunoparalysis, a frightening condition caused by massive sympathetic stimulation, which consists of temporary paralysis, deathly pallor, and absent peripheral pulses. Orthostatic hypotension without reactive tachycardia provides indirect support that autonomic dysfunction may underlie the highly unusual esophageal dysfunction observed in this case. Parasympathetic injury related to lightening strike has not been reported; however, intact vagal function is required for reflex inhibition of esophageal tone with coordinated peristalsis and relaxation of the lower esophageal sphincter.2 Vagal injury impairs this process and has been reported to produce manometric findings and symptoms similar to achalasia.3
The patient was treated by repeated endoscopic dilation of the lower esophageal sphincter. Swallowing function improved, body weight increased, and she continues to live independently on her farm.
References
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Conflicts of interst The authors disclose no conflicts.
PII: S0016-5085(09)00186-3
doi:10.1053/j.gastro.2009.02.007
© 2009 AGA Institute. Published by Elsevier Inc. All rights reserved.
