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Extensive preoperative workup in diffuse esophageal leiomyomatosis associated with Alport syndrome influences surgical treatment: A case report.

Dagbert F, Pelascini E, Pasquer A, Gincul R, Mion F, Poncet G, Robert M - Int J Surg Case Rep (2015)

Bottom Line: Misdiagnosis is frequent and previous surgeries can complicate surgical management.We describe a case of diffuse esophageal leiomyomatosis associated with Alport syndrome in a 21 year-old female where endoscopic ultrasonography (EUS) with concomitant fluoroscopy and 3D-gastric computed tomography (3D-GCT) modified surgical management.EUS and 3D-GCT should strongly be considered as part of routine preoperative workup in these patients.

View Article: PubMed Central - PubMed

Affiliation: Service de Chirurgie Digestive, Hôpital Edouard-Herriot, Lyon, France. Electronic address: francois.dagbert@umontreal.ca.

No MeSH data available.


Related in: MedlinePlus

(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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fig0010: (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).

Mentions: Because of severe painful dysphagia, total esophagectomy was elected with formation of a gastric conduit.. Due to the previous surgery on the stomach, possible difficulties with gastric conduit length were evoked. Additional diagnostic procedures with endoscopic ultrasound (EUS) combined with fluoroscopy as well as 3D gastric computed tomography with air (3D-GCT) using multidetector CT scanner after absorption of effervescent salt diluted in 10 ml of water and IV injection of butylscopolamine were realized. 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. 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.


Extensive preoperative workup in diffuse esophageal leiomyomatosis associated with Alport syndrome influences surgical treatment: A case report.

Dagbert F, Pelascini E, Pasquer A, Gincul R, Mion F, Poncet G, Robert M - Int J Surg Case Rep (2015)

(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).
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4430222&req=5

fig0010: (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).
Mentions: Because of severe painful dysphagia, total esophagectomy was elected with formation of a gastric conduit.. Due to the previous surgery on the stomach, possible difficulties with gastric conduit length were evoked. Additional diagnostic procedures with endoscopic ultrasound (EUS) combined with fluoroscopy as well as 3D gastric computed tomography with air (3D-GCT) using multidetector CT scanner after absorption of effervescent salt diluted in 10 ml of water and IV injection of butylscopolamine were realized. 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. 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.

Bottom Line: Misdiagnosis is frequent and previous surgeries can complicate surgical management.We describe a case of diffuse esophageal leiomyomatosis associated with Alport syndrome in a 21 year-old female where endoscopic ultrasonography (EUS) with concomitant fluoroscopy and 3D-gastric computed tomography (3D-GCT) modified surgical management.EUS and 3D-GCT should strongly be considered as part of routine preoperative workup in these patients.

View Article: PubMed Central - PubMed

Affiliation: Service de Chirurgie Digestive, Hôpital Edouard-Herriot, Lyon, France. Electronic address: francois.dagbert@umontreal.ca.

No MeSH data available.


Related in: MedlinePlus