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Endoscopic appearance of diffuse cavernous hemangioma of the rectosigmoid
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Clinics and Research in Hepatology and Gastroenterology (2015) xxx, xxx—xxx
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Endoscopic appearance of diffuse cavernous hemangioma of the rectosigmoid Vincent Zimmer a,∗, Jochen Schuld b, Carsten Schmidt c, Alexander Massmann d, Frank Lammert a a
Department of Medicine II, Saarland University Medical Center, Homburg, Germany Department of General, Visceral, Vascular and Pediatric Surgery, Saarland University Medical Center, Homburg, Germany c Department of Medicine II, Friedrich-Schiller-University, Jena, Germany d Department of Diagnostic and Interventional Radiology, Saarland University Medical Center, Homburg, Germany b
A 55-year-old man presented with lower gastrointestinal bleeding. Medical history included mechanical aortic valve replacement and recurrent painless hematochezia since early childhood. Prior evaluations suspected a vascular rectal malformation, and multiple transanal procedures, including sclerosant injections and surgical ligations, had been performed. Digital rectal examination revealed fresh blood, spongy circumferential masses, and a reduced sphincter tone. At colonoscopy, the mucosa was focally discolored with areas of intramucosal hemorrhages and dilated vessels in the sigmoid (A, top). On further retraction, some small polyps (A, middle) and multiple soft protrusions collapsing on insufflation and indistinguishable
∗
Corresponding author. Department of Medicine II, Saarland University Medical Center, Kirrberger Strasse 100, 66421 Homburg, Germany. Tel.: +49 6841 1623 210; fax: +49 6841 1623 264. E-mail addresses: [email protected] (V. Zimmer), [email protected] (J. Schuld), [email protected] (C. Schmidt), [email protected] (A. Massmann), [email protected] (F. Lammert).
from (idiopathic) rectal varices were noted extending from the anal verge up to 20 cm proximal (A, lower). Because of continuous diffuse bleedings transfemoral selective angiography of the inferior mesenteric, internal iliac and pudendal artery was performed demonstrating early pooling and arterial blood supply predominantly by the superior rectal artery (B, asterisk) and through small feeding vessels of the sigmoid artery (B, arrowheads). Transarterial embolization was not taken into account to prevent the associated high risk of ischemia with necrosis of the rectosigmoid and anal sphincter. Thus, the patient underwent a sphinctersaving rectosigmoid resection with colostomy. At surgery, multiple venectasias extending into the perirectal fat tissue were noted. Surgical pathology demonstrated multiple submucosal lakes of dilated thin-walled vascular structures interspersed by smooth muscle fibres and connective tissue stroma fibrosis (C), supporting the final diagnosis of diffuse cavernous hemangioma of the rectosigmoid (DCHRS). As for the lesions with a polypoid aspect on endoscopy, histopathology revealed intact colonic mucosa with inconspicous crypt architecture, focal hemorrhages, and submucosal fibrosis in these areas (not shown).
http://dx.doi.org/10.1016/j.clinre.2015.03.003 2210-7401/© 2015 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Zimmer V, et al. Endoscopic appearance of diffuse cavernous hemangioma of the rectosigmoid. Clin Res Hepatol Gastroenterol (2015), http://dx.doi.org/10.1016/j.clinre.2015.03.003
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V. Zimmer et al.
Figure 1 A. Colonoscopic view of the sigmoid with focally discolored mucosa, areas of intramucosal hemorrhage and dilated vessel (top). After withdrawal into the rectum, some small polyps (middle) and multiple soft protrusions collapsing on insufflation indistinguishable from (idiopathic) rectal varices were detected (lower). B. Transfemoral selective angiography of the inferior mesenteric, internal iliac and pudendal artery with early pooling and predominant arterial blood supply by the superior rectal artery (asterisk) through small feeding vessels of the sigmoid artery (arrowheads). C. Surgical pathology specimen with submucosal lakes of dilated thin-walled vascular structures interspersed by smooth muscle fibres and connective tissue stroma fibrosis (H&E stain).
DCHRS is an uncommon vascular malformation presenting with recurrent lower gastrointestinal bleeding beginning in infancy or early adolescence. Hamartomatous in nature, it is thought to arise from embryonic sequestration of mesodermal tissue [1]. Misclassification as inflammatory and/or ischemic conditions, extended hemorrhoids or rectal varicosis due to portal hypertension is frequent, and, indeed, a portocaval shunt procedere has been reported [2,3]. Thus,
endoscopists should be aware of this entity and consider it as a differential diagnosis of presumed idiopathic rectal varices [4]. Features of anorectal endosonography (EUS) of rectal hemangioma, which was not performed in the patient at that time, have only recently been reported indicating sponge-like hypoechogenic lesions within the rectal wall with pulsatile arterial feeders and large venous outflow vessels [4—6].
Please cite this article in press as: Zimmer V, et al. Endoscopic appearance of diffuse cavernous hemangioma of the rectosigmoid. Clin Res Hepatol Gastroenterol (2015), http://dx.doi.org/10.1016/j.clinre.2015.03.003
+Model CLINRE-731; No. of Pages 3
ARTICLE IN PRESS
Diffuse cavernous hemangioma of the rectosigmoid
Disclosure of interest The authors declare that they have no conflicts of interest concerning this article.
Acknowledgment Special thanks to Bertin Walter, Institute of Pathology, Saarland University Medical Center, for providing the histopathology photograph.
References
3 [2] Wang HT, Gao XH, Fu CG, et al. Diagnosis and treatment of diffuse cavernous hemangioma of the rectum: report of 17 cases. World J Surg 2010;34:2477—86. [3] Kaiser AM, Spanos C. An unusual cause of rectal bleeding. Br J Radiol 2005;78:373—5. [4] Veloso N, Silva JD, Pinto-Marques P. A rare cause of rectal bleeding. Gastroenterology 2012;143:e8—9. [5] Gottlieb K, Coff P, Preiksaitis H, et al. Massive hemorrhage in pregnancy caused by a diffuse cavernous hemangioma of the rectum — EUS as imaging modality of choice. Medscape J Med 2008;10:206. [6] Sharma M, Adulqader A, Shifa R. Endoscopic ultrasound for cavernous hemangioma of rectum. Endosc Ultrasound 2014;3: 63—5.
[1] Lyon DT, Mantia AG. Large-bowel hemangiomas. Dis Colon Rectum 1984;27:404—14.
Please cite this article in press as: Zimmer V, et al. Endoscopic appearance of diffuse cavernous hemangioma of the rectosigmoid. Clin Res Hepatol Gastroenterol (2015), http://dx.doi.org/10.1016/j.clinre.2015.03.003
ARTICLE IN PRESS
CLINRE-731; No. of Pages 3
Clinics and Research in Hepatology and Gastroenterology (2015) xxx, xxx—xxx
Available online at
ScienceDirect www.sciencedirect.com
IMAGE OF THE MONTH
Endoscopic appearance of diffuse cavernous hemangioma of the rectosigmoid Vincent Zimmer a,∗, Jochen Schuld b, Carsten Schmidt c, Alexander Massmann d, Frank Lammert a a
Department of Medicine II, Saarland University Medical Center, Homburg, Germany Department of General, Visceral, Vascular and Pediatric Surgery, Saarland University Medical Center, Homburg, Germany c Department of Medicine II, Friedrich-Schiller-University, Jena, Germany d Department of Diagnostic and Interventional Radiology, Saarland University Medical Center, Homburg, Germany b
A 55-year-old man presented with lower gastrointestinal bleeding. Medical history included mechanical aortic valve replacement and recurrent painless hematochezia since early childhood. Prior evaluations suspected a vascular rectal malformation, and multiple transanal procedures, including sclerosant injections and surgical ligations, had been performed. Digital rectal examination revealed fresh blood, spongy circumferential masses, and a reduced sphincter tone. At colonoscopy, the mucosa was focally discolored with areas of intramucosal hemorrhages and dilated vessels in the sigmoid (A, top). On further retraction, some small polyps (A, middle) and multiple soft protrusions collapsing on insufflation and indistinguishable
∗
Corresponding author. Department of Medicine II, Saarland University Medical Center, Kirrberger Strasse 100, 66421 Homburg, Germany. Tel.: +49 6841 1623 210; fax: +49 6841 1623 264. E-mail addresses: [email protected] (V. Zimmer), [email protected] (J. Schuld), [email protected] (C. Schmidt), [email protected] (A. Massmann), [email protected] (F. Lammert).
from (idiopathic) rectal varices were noted extending from the anal verge up to 20 cm proximal (A, lower). Because of continuous diffuse bleedings transfemoral selective angiography of the inferior mesenteric, internal iliac and pudendal artery was performed demonstrating early pooling and arterial blood supply predominantly by the superior rectal artery (B, asterisk) and through small feeding vessels of the sigmoid artery (B, arrowheads). Transarterial embolization was not taken into account to prevent the associated high risk of ischemia with necrosis of the rectosigmoid and anal sphincter. Thus, the patient underwent a sphinctersaving rectosigmoid resection with colostomy. At surgery, multiple venectasias extending into the perirectal fat tissue were noted. Surgical pathology demonstrated multiple submucosal lakes of dilated thin-walled vascular structures interspersed by smooth muscle fibres and connective tissue stroma fibrosis (C), supporting the final diagnosis of diffuse cavernous hemangioma of the rectosigmoid (DCHRS). As for the lesions with a polypoid aspect on endoscopy, histopathology revealed intact colonic mucosa with inconspicous crypt architecture, focal hemorrhages, and submucosal fibrosis in these areas (not shown).
http://dx.doi.org/10.1016/j.clinre.2015.03.003 2210-7401/© 2015 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Zimmer V, et al. Endoscopic appearance of diffuse cavernous hemangioma of the rectosigmoid. Clin Res Hepatol Gastroenterol (2015), http://dx.doi.org/10.1016/j.clinre.2015.03.003
+Model CLINRE-731; No. of Pages 3
ARTICLE IN PRESS
2
V. Zimmer et al.
Figure 1 A. Colonoscopic view of the sigmoid with focally discolored mucosa, areas of intramucosal hemorrhage and dilated vessel (top). After withdrawal into the rectum, some small polyps (middle) and multiple soft protrusions collapsing on insufflation indistinguishable from (idiopathic) rectal varices were detected (lower). B. Transfemoral selective angiography of the inferior mesenteric, internal iliac and pudendal artery with early pooling and predominant arterial blood supply by the superior rectal artery (asterisk) through small feeding vessels of the sigmoid artery (arrowheads). C. Surgical pathology specimen with submucosal lakes of dilated thin-walled vascular structures interspersed by smooth muscle fibres and connective tissue stroma fibrosis (H&E stain).
DCHRS is an uncommon vascular malformation presenting with recurrent lower gastrointestinal bleeding beginning in infancy or early adolescence. Hamartomatous in nature, it is thought to arise from embryonic sequestration of mesodermal tissue [1]. Misclassification as inflammatory and/or ischemic conditions, extended hemorrhoids or rectal varicosis due to portal hypertension is frequent, and, indeed, a portocaval shunt procedere has been reported [2,3]. Thus,
endoscopists should be aware of this entity and consider it as a differential diagnosis of presumed idiopathic rectal varices [4]. Features of anorectal endosonography (EUS) of rectal hemangioma, which was not performed in the patient at that time, have only recently been reported indicating sponge-like hypoechogenic lesions within the rectal wall with pulsatile arterial feeders and large venous outflow vessels [4—6].
Please cite this article in press as: Zimmer V, et al. Endoscopic appearance of diffuse cavernous hemangioma of the rectosigmoid. Clin Res Hepatol Gastroenterol (2015), http://dx.doi.org/10.1016/j.clinre.2015.03.003
+Model CLINRE-731; No. of Pages 3
ARTICLE IN PRESS
Diffuse cavernous hemangioma of the rectosigmoid
Disclosure of interest The authors declare that they have no conflicts of interest concerning this article.
Acknowledgment Special thanks to Bertin Walter, Institute of Pathology, Saarland University Medical Center, for providing the histopathology photograph.
References
3 [2] Wang HT, Gao XH, Fu CG, et al. Diagnosis and treatment of diffuse cavernous hemangioma of the rectum: report of 17 cases. World J Surg 2010;34:2477—86. [3] Kaiser AM, Spanos C. An unusual cause of rectal bleeding. Br J Radiol 2005;78:373—5. [4] Veloso N, Silva JD, Pinto-Marques P. A rare cause of rectal bleeding. Gastroenterology 2012;143:e8—9. [5] Gottlieb K, Coff P, Preiksaitis H, et al. Massive hemorrhage in pregnancy caused by a diffuse cavernous hemangioma of the rectum — EUS as imaging modality of choice. Medscape J Med 2008;10:206. [6] Sharma M, Adulqader A, Shifa R. Endoscopic ultrasound for cavernous hemangioma of rectum. Endosc Ultrasound 2014;3: 63—5.
[1] Lyon DT, Mantia AG. Large-bowel hemangiomas. Dis Colon Rectum 1984;27:404—14.
Please cite this article in press as: Zimmer V, et al. Endoscopic appearance of diffuse cavernous hemangioma of the rectosigmoid. Clin Res Hepatol Gastroenterol (2015), http://dx.doi.org/10.1016/j.clinre.2015.03.003