J Clin Gastroenterol
Volume 49, Number 7, August 2015
7. Whiston E, Taylor JW. Genomics in Coccidioides: insights into evolution, ecology and pathogenesis. Med Mycol. 2014;52:149–155. 8. Wisman IM, Moreno AJ, Parker AL, et al. Gastrointestinal dissemination of coccidioidomycosis. Am J Gastroenterol. 1986;81:589–593. 9. Blair JE, Balan V, Douglas DD, et al. Incidence and prevalence of coccidioidomycosis in patients with end-stage liver disease. Liver Transpl. 2003;9:843–850. 10. Albillos A, Lario M, Alvarez-Mon M. Cirrhosis-associated immune dysfunction: distinctive features and clinical relevance. J Hepatol. 2014;61:1385–1396.
Congenital Absence of Portal Vein With Cecal Varices To the Editor: The first case of portal vein absence was reported by John Abernethy in 1793, and these malformations are eponymously known as Abernethy malformations.1 Cases of Abernethy malformation with giant inferior mesenteric (IM) varices are exceedingly rare.2 In this paper we describe a case of this variety with the presence of cecal varices—a unique and unusual finding that we report for the first time. A 5-year-old boy was brought by his parents to the outpatient department of our institution for evaluation of recurrent blood-stained stools. The child was first noticed to have bright red blood in stool at the age of 1 year. Gradually, these episodes increased in frequency and the quantity of blood loss also increased. He was also noted to have black, tarry stools on occasions. He did not report any abdominal pain or discomfort along with these complaints. Systematic inquiry was negative for any fever, rash, oral ulcers, or joint pains. He was born through cesarean section and achieved all developmental milestones at appropriate ages. His family history was not significant for any bleeding disorders, and 2 older siblings were healthy. His diet consisted of cereals, rice, fruits, vegetables, meat, and dairy products. On physical examination, he appeared pale and had some degree of failure to thrive. His weight was 11 kg and his The authors declare that they have nothing to disclose.
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height was 85 cm; both parameters were below the fifth centile for age. Abdominal examination did not reveal any tenderness or visceromegaly. A digital rectal examination revealed a small amount of blood-stained stool. The child had undergone extensive laboratory workup in the past, which did not reveal any abnormalities other than microcytic hypochromic anemia. He had undergone upper and lower gastrointestinal endoscopies in the past, but apart from a few rectal varices no other abnormalities were detected. Band ligation for these rectal varices had been performed twice in the past. However, the child continued to have blood in his stool. A tagged-red cell scintigraphy scan was performed, which revealed a large tortuous vascular structure within the abdomen. This was followed by a CT angiography of the abdomen, which revealed congenital absence of the portal vein. A giant IM varix was also seen, which arose from near the confluence of splenic and superior mesenteric veins and extended down into the pelvis to drain into the right common iliac vein. A diagnosis of Abernethy malformation, type II, was made, and the patient underwent creation of a surgical shunt between the IM vein and the inferior vena cava. Postoperatively, the patient had 1 episode of lower gastrointestinal bleeding for which sclerotherapy was performed. Following this, the patient remained asymptomatic at the 6-month follow-up visit. Lower gastrointestinal bleeding as a presentation of Abernethy malformation is exceedingly rare. In previously published reports, this has been mostly attributed to the presence of rectal varices, which result in bright red blood per rectum.3,4 However, in the present case, a dilated ileocecal vein with cecal varices was also seen (in addition to rectal varices), which was a possible source of lower gastrointestinal bleeding. The presence of cecal varices in conjunction with Abernethy malformation is being reported here for the first time. Although this anomaly is very rare, it can present with blood in stool—a fairly common symptom in children and adults. Therefore, general physicians and pediatricians must be aware of this clinical entity to avoid delay in diagnosis and treatment. Interestingly, most patients with this malformation do not exhibit the typical signs of portal hypertension (splenomegaly, esophageal varices, or caput medusae)
2015 Wolters Kluwer Health, Inc. All rights reserved.
Letters to the Editor
or hepatic encephalopathy (despite having hyperammonemia), which can potentially delay the diagnosis.5 Muhammad Awais, MB, BS* Abdul Rehman, MB, BSw Noor Ul-Ain Baloch, MB, BSw *Department of Radiology, Aga Khan University Hospital wDepartment of Biological & Biomedical Sciences, Aga Khan University, Karachi, Sindh, Pakistan
REFERENCES 1. Mistinova J, Valacsai F, Varga I. Congenital absence of the portal vein—case report and a review of literature. Clin Anat. 2010;23:750–758. 2. Arana E, Marti-Bonmati L, Martinez V, et al. Portal vein absence and nodular regenerative hyperplasia of the liver with giant inferior mesenteric vein. Abdom Imaging. 1997;22:506–508. 3. Grazioli L, Alberti D, Olivetti L, et al. Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol. 2000;10:820–825. 4. Corness JAG, McHugh K, Roebuck DJ, et al. The portal vein in children: radiological review of congenital anomalies and acquired abnormalities. Pediatr Radiol. 2006;36:87–96. 5. Hu GH, Shen LG, Yang J, et al. Insight into congenital absence of the portal vein: is it rare? World J Gastroenterol. 2008;14:5969–5979.
Could a Transient Deficiency in Von Willebrand Factor Be a Cause of Gastrointestinal Angiodysplasia? To the Editor: We read with interest a recent article by Holleran et al1 where it was shown that the serum ratio of angiopoietin-1 (Ang-1) to angiopoietin-2 (Ang-2) is lower in patients with sporadic small bowel gastrointestinal angiodysplasia (GIAD) compared with controls. The same study further demonstrated a significant difference in C.S.J. is a speaker for Given Imaging. N.K.S. declares that there is nothing to disclose.
www.jcge.com |
Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
629
Volume 49, Number 7, August 2015
7. Whiston E, Taylor JW. Genomics in Coccidioides: insights into evolution, ecology and pathogenesis. Med Mycol. 2014;52:149–155. 8. Wisman IM, Moreno AJ, Parker AL, et al. Gastrointestinal dissemination of coccidioidomycosis. Am J Gastroenterol. 1986;81:589–593. 9. Blair JE, Balan V, Douglas DD, et al. Incidence and prevalence of coccidioidomycosis in patients with end-stage liver disease. Liver Transpl. 2003;9:843–850. 10. Albillos A, Lario M, Alvarez-Mon M. Cirrhosis-associated immune dysfunction: distinctive features and clinical relevance. J Hepatol. 2014;61:1385–1396.
Congenital Absence of Portal Vein With Cecal Varices To the Editor: The first case of portal vein absence was reported by John Abernethy in 1793, and these malformations are eponymously known as Abernethy malformations.1 Cases of Abernethy malformation with giant inferior mesenteric (IM) varices are exceedingly rare.2 In this paper we describe a case of this variety with the presence of cecal varices—a unique and unusual finding that we report for the first time. A 5-year-old boy was brought by his parents to the outpatient department of our institution for evaluation of recurrent blood-stained stools. The child was first noticed to have bright red blood in stool at the age of 1 year. Gradually, these episodes increased in frequency and the quantity of blood loss also increased. He was also noted to have black, tarry stools on occasions. He did not report any abdominal pain or discomfort along with these complaints. Systematic inquiry was negative for any fever, rash, oral ulcers, or joint pains. He was born through cesarean section and achieved all developmental milestones at appropriate ages. His family history was not significant for any bleeding disorders, and 2 older siblings were healthy. His diet consisted of cereals, rice, fruits, vegetables, meat, and dairy products. On physical examination, he appeared pale and had some degree of failure to thrive. His weight was 11 kg and his The authors declare that they have nothing to disclose.
Copyright
r
height was 85 cm; both parameters were below the fifth centile for age. Abdominal examination did not reveal any tenderness or visceromegaly. A digital rectal examination revealed a small amount of blood-stained stool. The child had undergone extensive laboratory workup in the past, which did not reveal any abnormalities other than microcytic hypochromic anemia. He had undergone upper and lower gastrointestinal endoscopies in the past, but apart from a few rectal varices no other abnormalities were detected. Band ligation for these rectal varices had been performed twice in the past. However, the child continued to have blood in his stool. A tagged-red cell scintigraphy scan was performed, which revealed a large tortuous vascular structure within the abdomen. This was followed by a CT angiography of the abdomen, which revealed congenital absence of the portal vein. A giant IM varix was also seen, which arose from near the confluence of splenic and superior mesenteric veins and extended down into the pelvis to drain into the right common iliac vein. A diagnosis of Abernethy malformation, type II, was made, and the patient underwent creation of a surgical shunt between the IM vein and the inferior vena cava. Postoperatively, the patient had 1 episode of lower gastrointestinal bleeding for which sclerotherapy was performed. Following this, the patient remained asymptomatic at the 6-month follow-up visit. Lower gastrointestinal bleeding as a presentation of Abernethy malformation is exceedingly rare. In previously published reports, this has been mostly attributed to the presence of rectal varices, which result in bright red blood per rectum.3,4 However, in the present case, a dilated ileocecal vein with cecal varices was also seen (in addition to rectal varices), which was a possible source of lower gastrointestinal bleeding. The presence of cecal varices in conjunction with Abernethy malformation is being reported here for the first time. Although this anomaly is very rare, it can present with blood in stool—a fairly common symptom in children and adults. Therefore, general physicians and pediatricians must be aware of this clinical entity to avoid delay in diagnosis and treatment. Interestingly, most patients with this malformation do not exhibit the typical signs of portal hypertension (splenomegaly, esophageal varices, or caput medusae)
2015 Wolters Kluwer Health, Inc. All rights reserved.
Letters to the Editor
or hepatic encephalopathy (despite having hyperammonemia), which can potentially delay the diagnosis.5 Muhammad Awais, MB, BS* Abdul Rehman, MB, BSw Noor Ul-Ain Baloch, MB, BSw *Department of Radiology, Aga Khan University Hospital wDepartment of Biological & Biomedical Sciences, Aga Khan University, Karachi, Sindh, Pakistan
REFERENCES 1. Mistinova J, Valacsai F, Varga I. Congenital absence of the portal vein—case report and a review of literature. Clin Anat. 2010;23:750–758. 2. Arana E, Marti-Bonmati L, Martinez V, et al. Portal vein absence and nodular regenerative hyperplasia of the liver with giant inferior mesenteric vein. Abdom Imaging. 1997;22:506–508. 3. Grazioli L, Alberti D, Olivetti L, et al. Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol. 2000;10:820–825. 4. Corness JAG, McHugh K, Roebuck DJ, et al. The portal vein in children: radiological review of congenital anomalies and acquired abnormalities. Pediatr Radiol. 2006;36:87–96. 5. Hu GH, Shen LG, Yang J, et al. Insight into congenital absence of the portal vein: is it rare? World J Gastroenterol. 2008;14:5969–5979.
Could a Transient Deficiency in Von Willebrand Factor Be a Cause of Gastrointestinal Angiodysplasia? To the Editor: We read with interest a recent article by Holleran et al1 where it was shown that the serum ratio of angiopoietin-1 (Ang-1) to angiopoietin-2 (Ang-2) is lower in patients with sporadic small bowel gastrointestinal angiodysplasia (GIAD) compared with controls. The same study further demonstrated a significant difference in C.S.J. is a speaker for Given Imaging. N.K.S. declares that there is nothing to disclose.
www.jcge.com |
Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
629
