esophageal intramural pseudodiverticulosis ( eipd ) is a rare disease of unknown origin which was first described by mendl in 1960 . in the majority of cases dysphagia , which sometimes slowly progresses ,
eipd is often associated with gastroesophageal reflux , chronic alcohol abuse , diabetes and candida infection , but its etiology and pathogenesis remain obscure [ 2 , 3 ] .
it often complicates severe stricture , which makes double contrast esophagogram and computed tomography more useful than esophagogastroduodenoscopy ( egd ) in diagnosing eipd .
the former two procedures reveal multiple , small , flask - shaped outpouchings in the wall of the esophagus , which are the characteristics of eipd .
almost all cases of eipd are benign and responds well to medical treatment for inflammation and to endoscopic dilatation of the esophagus [ 2 , 3 ] .
we here report a suggestive case of a severe form of eipd whose dysphagia was ameliorated not by endoscopic dilatation therapy but by oral administration of anti - fungal medicine .
a 59-year - old man suffered from dysphagia since 1995 , but he did not consult any hospital until the symptom worsened in 2008 .
when he consulted a nearby hospital , egd revealed severe constriction in the thoracic esophagus , and he was referred to our hospital for treatment .
he had undergone subtotal gastrectomy with billroth ii reconstruction for gastric ulcer when 40 years old .
physical examination showed no unusual findings , but laboratory data showed anemia ( hb : 10.1 g / dl ) , low serum albumin ( 3.5 g / dl ) and slight increase of tumor marker scc ( 1.9 ng / ml ; normal limit 01.5 ng / ml ) .
the esophageal lumen was slightly dilated on the oral side of the narrowing , where several sac - like barium collections ( up to 12 cm in diameter ) protruded .
the barium barely thread through a 5- to 6-cm - long segment of severe narrowing , and the anal side of the narrowing only slightly appeared on the radiography ( fig .
esophagoscopy revealed a severe , fibrotic stenosis 26 cm from the incisors . on the oral side of the stenosis
a thin - caliber endoscope ( 6-mm outside diameter ; olympus xp260n ) barely passed through this narrowed region where the esophageal mucosa was not ulcerated ( fig .
then a large number of tiny orifices corresponding to pseudodiverticula were identified along the anal side of the stenotic region ( fig .
computed tomography revealed wall thickening and small gas collection , which corresponded to pseudodiverticula , in the wall of the upper to middle thoracic esophagus . as endoscopic examination revealed a severe fibrotic stenosis ,
endoscopic mechanical dilatation therapy was applied to this patient rather than medical treatment in expectation of prompt resolution of symptoms .
we repeatedly performed endoscopic balloon dilatation ( ebd ) for the esophageal constriction , but the effect was very limited , and the dysphagia relapsed with esophageal re - stenosis shortly after the treatments .
we then tried making an incision in the esophageal mucosa endoscopically using an it knife .
this treatment improved his symptom and radiography also showed improvement of the constriction , but the dysphagia gradually worsened , and 6 months after the treatment , egd revealed re - stenosis of the esophagus .
he showed progressed malnutrition due to severe dysphagia and had lost 5 kg of body weight during 6 months . since
a white , thick , creamy liquid suggestive of pus emerging from the orifices was sometimes detected endoscopically ( fig .
3a , b ) and candida albicans was detected in pas staining of the biopsy specimen , we began giving this patient an anti - fungal medicine in addition to performing ebd therapy , and these treatments greatly improved his dysphagia .
egd revealed significant improvement of the esophageal constriction and disappearance of the pus , although multiple small depressions remained in the entire esophagus ( fig .
radiography showed significant improvement in the flow of swallowed barium , although there still remained a clumped filling defect in the upper thoracic esophagus ( fig .
it was also revealed by repeated radiography that very small ( 13 mm ) , multiple , flask - shaped outpouchings were diffusely distributed in the esophagus on the anal side of the narrowing ( fig .
the patient required continuous anti - fungal medicine because his symptom quickly worsened after temporary discontinuation of the medication .
it is now 24 months since we began anti - fungal treatment , and he is still symptom - free with no severe constriction detected on egd .
eipd is characterized by the development of multiple , epithelium - lined cysts within the esophageal wall ; each cyst connects to the esophageal lumen via a narrow ostium .
the cysts connected to the esophageal lumen result from ductal dilatation of the esophageal submucosal glands and excretory ducts , displaying multiple pseudodiverticula radiologically [ 2 , 3 ] . in the present case ,
characteristic , multiple , tiny , flask - shaped outpouchings were identified in the esophagus along the anal side of the narrowing . on the other hand ,
the esophageal mucosa on the oral side of the narrowing showed greater barium collection as occurs in fistula formation , which is somewhat unusual for eipd and could have resulted from the branching or bridging between adjacent diverticula .
the long period before receiving medical care and concomitant candida infection could be responsible for the unusual shape of eipd in this case . since the first case report of eipd in 1960
, there have numerous case reports and reviews concerning this rare esophageal disorder [ 2 , 3 , 5 ] , but the present case could represent a most advanced condition of eipd , considering that the swallowed barium barely thread through the stricture and esophagoscopy revealed a stiff , fibrotic stenosis .
previous reports documented that , while oral administration of medication is employed for patients with eipd accompanied by esophagitis or esophageal candidiasis , ebd has been carried out for patients with severe constriction [ 2 , 3 ] .
our case was resistant to endoscopic mechanical dilatation therapy , although previous reports consistently described that endoscopic dilatation of the stricture usually gives immediate and long - lasting relief of dysphagia in eipd [ 2 , 3 , 6 , 7 ] .
in addition , this was the first attempt to treat severe stenosis of eipd with endoscopic incision therapy , which has been successfully applied for the dilatation of benign strictures of the esophagus due to other causes , but this therapy also showed only a limited effect for resolution of the dysphagia in our case .
remarkably , in spite of failures with endoscopic mechanic dilatation treatments for the severe stricture , oral administration of anti - fungal medication turned out to be effective in the treatment of eipd , leading to long - lasting resolution of the dysphagia in the present case .
re - examination by radiography and egd during the anti - fungal medication showed that the constriction of the esophagus had also improved , although the tiny diverticula did not resolve after the treatment .
subsequently , the patient required continuous anti - fungal medication for sustained improvement of the dysphagia .
the first is an obstruction of the ducts due to inflammatory cells or desquamated epithelium , and the second is compression of the ductal orifices due to periductal infiltrates and/or fibrosis [ 2 , 3 , 9 ] .
previous reports showed that c. albicans infection is frequently present in eipd , and such infection may be involved in the etiology of eipd in either mechanism [ 2 , 3 ] .
the clinical course of our case also suggests a significant contribution of candida infection in the exacerbation of the eipd . in the present case , in addition to pre - existing chronic fibrosis of the esophageal wall , concomitant inflammatory cellular infiltration triggered by candida infection
may have worsened the stricture of eipd , possibly by enhanced intramural pressure in the narrowed esophagus . in conclusion , we experienced a case of an advanced stage of esophageal stricture derived from eipd , which was resistant to endoscopic mechanical dilatation therapy .
even in such a severe case , anti - fungal medication was still effective in relieving the persistent dysphagia .
an important lesson of this case is that anti - fungal medication , rather than endoscopic dilatation therapy , could be considered as a first - line therapy against dysphagia and constriction in eipd regardless of the severity of the esophageal constriction .
 esophageal intramural pseudodiverticulosis ( eipd ) is a rare disease of unknown etiology that displays multiple pseudodiverticula radiologically , leading to benign esophageal stricture . 
 dysphagia , which sometimes slowly progresses , is the main symptom in the majority of cases . 
 we here report a 59-year - old male eipd patient who suffered from severe dysphagia . 
 radiography and endoscopy of this patient disclosed a severe constriction in the upper thoracic esophagus . 
 although we tried several endoscopic procedures including frequent endoscopic balloon dilatation ( ebd ) , the effect was very limited and his dysphagia relapsed shortly after the treatments . during the procedures , we noticed some white , thick , creamy liquid emerging from the orifices of eipd , and pas staining of biopsy specimens revealed infection with candida albicans . 
 hence , the patient was given anti - fungal medicine in addition to ebd . 
 the additional treatment with anti - fungal medicine dramatically improved his symptoms and the esophageal constriction . 
 this case suggests that anti - fungal treatment is an effective first - line therapy even against a severe form of esophageal constriction in eipd . 
Introduction
Case Report
Discussion