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Case Reports
. 2008 Feb;22(2):187-9.
doi: 10.1155/2008/603105.

Esophageal leiomyomatosis -- an unusual cause of pseudoachalasia

S Ray  1 , S Singh Saluja, R Gupta, T Kanti Chattopadhyay
Affiliations
Case Reports

Esophageal leiomyomatosis -- an unusual cause of pseudoachalasia

S Ray et al. Can J Gastroenterol. 2008 Feb.

Abstract
in English, French

Esophageal leiomyomatosis is a rare hamartomatous disorder with varied presentation. In the literature, it is described mostly in children, and is associated with Alport's syndrome. A case of leiomyomatosis that presented as achalasia not associated with Alport's syndrome is described in a 35-year-old woman with a 16-year history of dysphagia. Barium swallow showed a smooth narrowing at the lower end of the esophagus with a longer than usual stricture length. Endoscopy showed a dilated esophagus with a submucosal nodule in the region of the cardia. A computed tomography scan revealed circumferential thickening of the esophagus involving the gastroesophageal junction, with fat planes maintained with the adjacent structure. Endoscopic ultrasound demonstrated a lesion arising from the muscularis propria. The manometry findings were suggestive of achalasia. She underwent transhiatal esophagectomy with gastric pull-up. Leiomyomatosis should be considered as a cause of psuedoachalasia in patients with symptoms suggestive of achalasia and atypical barium findings. Attempts should be made to confirm the diagnosis preoperatively using computed tomography and/or endoscopic ultrasound. Esophagectomy is the treatment of choice.

La léiomyomatose œsophagienne est un trouble hamartomateux rare dont la présentation est variée. Dans les publications, on la décrit surtout chez les enfants, et elle s’associe au syndrome d’Alport. Un cas de léiomyomatose prenant la forme d’une achalasie non associée au syndrome d’Alport est décrit chez une femme de 35 ans souffrant de dysphagie depuis 16 ans. Le repas baryté a révélé un rétrécissement régulier à l’extrémité inférieure de l’œsophage, plus long qu’à l’habitude. L’endoscopie a démontré un œsophage dilaté avec nodule sous-muqueux dans la région du cardia. Une tomodensitométrie a révélé un épaississement circonférentiel de l’œsophage à la jonction gastro-œsophagienne, les plans adipeux étant maintenus avec la structure adjacente. L’échoendoscopie a révélé une lésion provenant de la couche longitudinale. Les résultats de la manométrie étaient indicateurs d’une achalasie. La patiente a subi une œsophagectomie transhiatale avec rétablissement gastrique.

La léiomyomatose devrait être considérée comme une cause de pseudoachalasie chez les patients présentant des symptômes indicateurs d’une achalasie et dont les résultats du repas baryté sont atypiques. Il faudrait tenter de confirmer le diagnostic avant l’opération par tomodensitométrie ou échoendoscopie. L’œsophagectomie est le traitement de choix.

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Figures

Figure 1)
Figure 1)
A Barium swallow study, showing a dilated esophagus with a smooth narrowing (arrow) at the gastroesophageal junction. B Computed tomography scan, showing a circumferential wall thickening (arrow) in the region of the gastroesophageal junction with a proximal dilated esophagus
Figure 2)
Figure 2)
A Microphotograph, showing esophageal mucosa on the right with an unencapsulated spindle cell lesion in the submucosa (hematoxylin and eosin stain, original magnification × 40). B Higher-power microphotograph, demonstrating intersecting fascicles of spindle cells possessing eosinophilic cytoplasm and normochromatic elongated nuclei. Note the absence of pleomorphism, mitotic activity or necrosis (hematoxylin and eosin stain, original magnificaion ×200)
Figure 3)
Figure 3)
Photomicrographs from the esophagectomy specimen, showing leiomyomatous nodules (N) beneath the mucosa (M) (A and B); a higher-power view, showing spindle cells with eosinophilic cytoplasm and elongated nuclei (C); and diffuse infiltration of the leiomyomatous proliferation between the normal muscle fibres (arrow in D)
See this image and copyright information in PMC

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