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Preoperative coronal CT-scan showing enlargement of mid and lower esophageal body.
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The diagnosis of diffuse leiomyomatosis associated with an Alport syndrome was made with extensive involvement of the esophagus, rectum and clitoris Fig. 1.
(A) Endoscopic ultrasound showed a 5.4 mm thickening of the internal circular muscular layer in the lower part of esophagus, at 35 cm from teeth (arrow). (B) Endoscopic ultrasound showed the level of transition with normal muscular layer at 17 cm from teeth (neck level).
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The EUS indicated that the level of transition between normal and thickened muscular layers was at the level of the neck, just below Killian's triangle Fig. 2.
(A) External placement of metallic clip combined with EUS shows transition point between normal and enlarged circular muscle of esophagus to be in the neck (B) 3D-GCT delineates gastric remnant morphology after previous surgery and shows voluminous leiomyoma of lower esophagus.
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The 3D-GCT allowed to measure the volume and possible dilation of the residual stomach and showed that a gastric conduit could probably reach the neck Fig. 3.
(A) Operative specimen showing completely thickened esophagus (B) Voluminous leiomyoma of the lower esophagus (long arrow) and enlarged circular muscle fibers of the mid and upper esophagus (short arrow).
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Because of the intensity of the abdominal pain, she had to be discharged with home parenteral nutrition Fig. 4.