ELECTRONIC IMAGE OF THE MONTH Eosinophilic Infiltration of the Muscularis Propria in a Patient with Jackhammer Esophagus Treated With Per-Oral Endoscopic Myotomy Q9
Hiroki Sato, Manabu Takeuchi, and Kazuya Takahashi Division of Gastroenterology, Niigata University Medical and Dental Hospital, Niigata, Japan
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43-year-old man visited our hospital because of a 25-year history of untreated dysphagia and chest pain. He had sleep apnea that was treated with continuous positive airway pressure, but had no allergies and was taking no medication. Physical and laboratory examinations showed no specific findings (peripheral blood eosinophils, 388.1/mL [6.6%]). Endoscopy showed an edematous esophagus wall with a compressed lumen with adequate air supply (Figure A). Six biopsy samples were obtained from different esophageal regions. The biopsy specimen of the mucosa appeared normal, with slight epithelial hyperplasia, no eosinophils, and showed no signs of gastroesophageal reflux such as papillary elongation or spongiosis (Figure B). A timed barium esophagogram showed spastic contraction and some barium stagnant in the mid/distal esophagus, but appropriate emptying at 1, 2, and 5 minutes (Figure C). Endoscopic ultrasonography (EUS) showed circumferential hypertrophy of the muscle layer in the middle/lower
A
esophagus (4.1-mm thick) (Figure D). On high-resolution manometry (HRM) (Starmedical, Tokyo, Japan), the maximum distal contractile integral (DCI) was extremely high (48,664.9 mm Hg s-1 cm-1), with a DCI greater than 8000 in 8 swallows, although the integrated relaxation pressure was normal at 11.4 mm Hg. The esophageal segment with high-amplitude contraction on HRM corresponded to the hypertrophied segment documented by EUS. The patient was diagnosed with jackhammer esophagus (Figure E). After 2 months of medical therapy (calcium blockers [nifedipine 10 mg 1/day] and proton pump inhibitors [esomeprazole 10 mg 1/day]), the patient showed no improvement. Per-oral endoscopic myotomy was performed to treat esophageal spastic contraction and for histopathologic assessment.1 A 14-cm esophageal myotomy was performed longitudinally from the midesophagus to the esophagogastric junction (Figure F). During myotomy, 6 full-thickness muscle layer biopsies
Clinical Gastroenterology and Hepatology 2015;-:-–SCO 5.2.0 DTD YJCGH54060_proof 30 January 2015 10:53 pm ce DVC
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were performed by placing the full-muscle layer between both ends of a biopsy forceps (Radial Jaw 4; Boston Scientific, Marlborough, MA), and showed eosinophilic infiltration in the perivascular and perimysial areas (Figure G). We propose the term eosinophilic esophageal myositis to describe these findings, based on histologic characteristics. Although dysphagia markedly improved after 1 month, chest pain persisted. Therefore, treatment with oral prednisone (0.5 mg/kg/d) was initiated, resulting in complete alleviation of chest pain. Jackhammer esophagus is a rare esophageal motility disorder characterized by hypercontractile peristalsis, with a DCI greater than 8000 mm Hg s-1 cm-1 in at least one swallow, with normal integrated relaxation pressure in HRM (Chicago classification).2 One of the reasons for our limited understanding of the pathophysiology of this esophageal motor disorder is the inability to sample muscle tissue during endoscopy, and the invasiveness of surgical biopsy for this purpose. Currently used strategies, such as medication, Botox injections, and laparoscopic/ endoscopic myotomy, are all symptomatic treatments. We have shown a patient with a full-thickness biopsy specimen in jackhammer esophagus. In our patient, EUS showed muscle hypertrophy, and full-thickness biopsy specimens showed increased eosinophils in the muscularis propria. This condition, if confirmed in larger
populations, could be termed eosinophilic esophageal myositis. Additional studies also should be performed to clarify whether per-oral endoscopic myotomy adequately treats jackhammer esophagus. The potential role of eosinophilic infiltration of the muscularis propria in the pathophysiology of jackhammer esophagus deserves further study.
References 1.
Inoue H, Minami H, Kobayashi Y, et al. Peroral endoscopic myotomy (POEM) for esophageal achalasia. Endoscopy 2010; 42:265–271.
2.
Bredenoord AJ, Fox M, Kahrilas PJ, et al. Chicago classification criteria of esophageal motility disorders defined in high resolution esophageal pressure topography. Neurogastroenterol Motil 2012;24(Suppl 1):57–65.
Q1 Acknowledgments The authors thank Masaaki Kobayashi, Yuichi Sato, Satoru Hashimoto, and Kenichi Mizuno (Division of Gastroenterology, Niigata University Medical and Dental Hospital) for their technical support. The authors also thank Takeshi Koizumi (Department of Medicine II, Niigata University Medical and Dental Hospital) for referring the patient. Conflicts of interest The authors disclose no conflicts. © 2015 by the AGA Institute 1542-3565/$36.00 http://dx.doi.org/10.1016/j.cgh.2014.11.005
e2 SCO 5.2.0 DTD YJCGH54060_proof 30 January 2015 10:53 pm ce DVC
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Hiroki Sato, Manabu Takeuchi, and Kazuya Takahashi Division of Gastroenterology, Niigata University Medical and Dental Hospital, Niigata, Japan
web 4C/FPO
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52
Q3 Q4
Q5
43-year-old man visited our hospital because of a 25-year history of untreated dysphagia and chest pain. He had sleep apnea that was treated with continuous positive airway pressure, but had no allergies and was taking no medication. Physical and laboratory examinations showed no specific findings (peripheral blood eosinophils, 388.1/mL [6.6%]). Endoscopy showed an edematous esophagus wall with a compressed lumen with adequate air supply (Figure A). Six biopsy samples were obtained from different esophageal regions. The biopsy specimen of the mucosa appeared normal, with slight epithelial hyperplasia, no eosinophils, and showed no signs of gastroesophageal reflux such as papillary elongation or spongiosis (Figure B). A timed barium esophagogram showed spastic contraction and some barium stagnant in the mid/distal esophagus, but appropriate emptying at 1, 2, and 5 minutes (Figure C). Endoscopic ultrasonography (EUS) showed circumferential hypertrophy of the muscle layer in the middle/lower
A
esophagus (4.1-mm thick) (Figure D). On high-resolution manometry (HRM) (Starmedical, Tokyo, Japan), the maximum distal contractile integral (DCI) was extremely high (48,664.9 mm Hg s-1 cm-1), with a DCI greater than 8000 in 8 swallows, although the integrated relaxation pressure was normal at 11.4 mm Hg. The esophageal segment with high-amplitude contraction on HRM corresponded to the hypertrophied segment documented by EUS. The patient was diagnosed with jackhammer esophagus (Figure E). After 2 months of medical therapy (calcium blockers [nifedipine 10 mg 1/day] and proton pump inhibitors [esomeprazole 10 mg 1/day]), the patient showed no improvement. Per-oral endoscopic myotomy was performed to treat esophageal spastic contraction and for histopathologic assessment.1 A 14-cm esophageal myotomy was performed longitudinally from the midesophagus to the esophagogastric junction (Figure F). During myotomy, 6 full-thickness muscle layer biopsies
Clinical Gastroenterology and Hepatology 2015;-:-–SCO 5.2.0 DTD YJCGH54060_proof 30 January 2015 10:53 pm ce DVC
53 54 55 56 57 58 59 60 61 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99 100 101 102 103 104
ELECTRONIC IMAGE OF THE MONTH, continued 105 106 107 108 109 110 111 112 113 114 115 116 117 118 119 120 121 122 123 124 125 126 127 128 129 130 131 132 133 134 135 136 137 138 139 140 141 142 143 144 145 146
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were performed by placing the full-muscle layer between both ends of a biopsy forceps (Radial Jaw 4; Boston Scientific, Marlborough, MA), and showed eosinophilic infiltration in the perivascular and perimysial areas (Figure G). We propose the term eosinophilic esophageal myositis to describe these findings, based on histologic characteristics. Although dysphagia markedly improved after 1 month, chest pain persisted. Therefore, treatment with oral prednisone (0.5 mg/kg/d) was initiated, resulting in complete alleviation of chest pain. Jackhammer esophagus is a rare esophageal motility disorder characterized by hypercontractile peristalsis, with a DCI greater than 8000 mm Hg s-1 cm-1 in at least one swallow, with normal integrated relaxation pressure in HRM (Chicago classification).2 One of the reasons for our limited understanding of the pathophysiology of this esophageal motor disorder is the inability to sample muscle tissue during endoscopy, and the invasiveness of surgical biopsy for this purpose. Currently used strategies, such as medication, Botox injections, and laparoscopic/ endoscopic myotomy, are all symptomatic treatments. We have shown a patient with a full-thickness biopsy specimen in jackhammer esophagus. In our patient, EUS showed muscle hypertrophy, and full-thickness biopsy specimens showed increased eosinophils in the muscularis propria. This condition, if confirmed in larger
populations, could be termed eosinophilic esophageal myositis. Additional studies also should be performed to clarify whether per-oral endoscopic myotomy adequately treats jackhammer esophagus. The potential role of eosinophilic infiltration of the muscularis propria in the pathophysiology of jackhammer esophagus deserves further study.
References 1.
Inoue H, Minami H, Kobayashi Y, et al. Peroral endoscopic myotomy (POEM) for esophageal achalasia. Endoscopy 2010; 42:265–271.
2.
Bredenoord AJ, Fox M, Kahrilas PJ, et al. Chicago classification criteria of esophageal motility disorders defined in high resolution esophageal pressure topography. Neurogastroenterol Motil 2012;24(Suppl 1):57–65.
Q1 Acknowledgments The authors thank Masaaki Kobayashi, Yuichi Sato, Satoru Hashimoto, and Kenichi Mizuno (Division of Gastroenterology, Niigata University Medical and Dental Hospital) for their technical support. The authors also thank Takeshi Koizumi (Department of Medicine II, Niigata University Medical and Dental Hospital) for referring the patient. Conflicts of interest The authors disclose no conflicts. © 2015 by the AGA Institute 1542-3565/$36.00 http://dx.doi.org/10.1016/j.cgh.2014.11.005
e2 SCO 5.2.0 DTD YJCGH54060_proof 30 January 2015 10:53 pm ce DVC
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