Clinical Challenges

The diagnosis

Based on the clinical and imaging findings, a diagnosis of gallbladder adenomyomatosis was made. GA is a benign and usually asymptomatic condition that occurs mainly beyond the age of 50-60 years and is very rare in childhood.¹ Symptomatic gallbladder adenomyomatosis indicates cholecystectomy, considering the presence of inflammation or gallbladder stones.² Therefore, a laparoscopic cholecystectomy was performed on our patient. Rokitansky-Aschoff sinuses were seen in the entire thickened gallbladder wall on gross pathologic examination (Figure D). Histopathologic examination confirmed the diagnosis of GA with cholecystitis. The patient was eventually diagnosed with diffuse GA. She was successfully discharged from the hospital 4 days after surgery, and 3 months of follow-up were uneventful.

gr1d_lrg_web.jpg

References

Eroglu N et al. Diffuse adenomyomatosis of the gallbladder in a child. J Pediatr Hematol Oncol. 2016;38:e307-9.

Bonatti M. et al. Gallbladder adenomyomatosis: imaging findings, tricks and pitfalls. Insights Imaging. 2017;8:243-53.

Hammad AY et al. A literature review of radiological findings to guide the diagnosis of gallbladder adenomyomatosis. HPB (Oxford). 2016;18:129-35.

The diagnosis

Based on the clinical and imaging findings, a diagnosis of gallbladder adenomyomatosis was made. GA is a benign and usually asymptomatic condition that occurs mainly beyond the age of 50-60 years and is very rare in childhood.¹ Symptomatic gallbladder adenomyomatosis indicates cholecystectomy, considering the presence of inflammation or gallbladder stones.² Therefore, a laparoscopic cholecystectomy was performed on our patient. Rokitansky-Aschoff sinuses were seen in the entire thickened gallbladder wall on gross pathologic examination (Figure D). Histopathologic examination confirmed the diagnosis of GA with cholecystitis. The patient was eventually diagnosed with diffuse GA. She was successfully discharged from the hospital 4 days after surgery, and 3 months of follow-up were uneventful.

gr1d_lrg_web.jpg

References

Eroglu N et al. Diffuse adenomyomatosis of the gallbladder in a child. J Pediatr Hematol Oncol. 2016;38:e307-9.

Bonatti M. et al. Gallbladder adenomyomatosis: imaging findings, tricks and pitfalls. Insights Imaging. 2017;8:243-53.

Hammad AY et al. A literature review of radiological findings to guide the diagnosis of gallbladder adenomyomatosis. HPB (Oxford). 2016;18:129-35.

The diagnosis

Based on the clinical and imaging findings, a diagnosis of gallbladder adenomyomatosis was made. GA is a benign and usually asymptomatic condition that occurs mainly beyond the age of 50-60 years and is very rare in childhood.¹ Symptomatic gallbladder adenomyomatosis indicates cholecystectomy, considering the presence of inflammation or gallbladder stones.² Therefore, a laparoscopic cholecystectomy was performed on our patient. Rokitansky-Aschoff sinuses were seen in the entire thickened gallbladder wall on gross pathologic examination (Figure D). Histopathologic examination confirmed the diagnosis of GA with cholecystitis. The patient was eventually diagnosed with diffuse GA. She was successfully discharged from the hospital 4 days after surgery, and 3 months of follow-up were uneventful.

gr1d_lrg_web.jpg

References

Eroglu N et al. Diffuse adenomyomatosis of the gallbladder in a child. J Pediatr Hematol Oncol. 2016;38:e307-9.

Bonatti M. et al. Gallbladder adenomyomatosis: imaging findings, tricks and pitfalls. Insights Imaging. 2017;8:243-53.

Hammad AY et al. A literature review of radiological findings to guide the diagnosis of gallbladder adenomyomatosis. HPB (Oxford). 2016;18:129-35.

Answer to ‘What’s your diagnosis?’: Gastric adenocarcinoma and proximal polyposis of the stomach syndrome.

Fundic gland polyps (FGPs) are the most common gastric polyps and when occurring in the sporadic setting are typically benign; however, FGPs that occur in gastrointestinal polyposis syndromes such as familial adenomatosis polyposis can progress to adenocarcinoma and require surveillance. Therefore, it is important to distinguish sporadic versus syndromic fundic gland polyposis. Gastric adenocarcinoma and proximal polyposis of the stomach is a recently described condition that significantly increases the risk of developing invasive gastric adenocarcinoma from FGPs. Diagnostic criteria include (1) gastric polyposis restricted to the body and fundus with no small bowel or colonic involvement, (2) >100 gastric polyps or >30 polyps in a first-degree relative, (3) histology consistent with FGP with areas of dysplasia, (4) a family history consistent with an autosomal-dominant pattern of inheritance, and (5) exclusion of other syndromes and proton pump inhibitor use.¹ Unlike familial adenomatosis polyposis, the polyposis is restricted to the oxyntic mucosa of the gastric body and fundus with sparing of the gastric antrum, small bowel, and colon. The genetic basis of the disease has been attributed to a point mutation in the APC gene promotor IB region leading to a loss of tumor suppressor function.² Typical histology shows large FGPs with areas of low-grade and high-grade dysplasia, as seen in our patient.

There are few data on the natural history of gastric adenocarcinoma and proximal polyposis of the stomach, but effective surveillance is limited by the degree of polyposis. There are multiple reports of hidden adenocarcinoma on surgically resected specimens, as well as rapid progression to metastatic adenocarcinoma despite adequate diagnosis and surveillance.^1,3 Therefore, total gastrectomy should be offered to patients who are surgical candidates. Our patient underwent genetic testing that revealed a point mutation in the APC promotor IB. He declined surgical intervention and opted for surveillance endoscopy every 6 months.
 

References

1. Worthley D.L. et al. Gut. 2012;61:774-9

2. Li J et al. Am J Hum Genet. 2016;98:830-42

3. Rudloff U. Clin Exp Gastroenterol. 2018;11:447-59

Answer to ‘What’s your diagnosis?’: Gastric adenocarcinoma and proximal polyposis of the stomach syndrome.

Fundic gland polyps (FGPs) are the most common gastric polyps and when occurring in the sporadic setting are typically benign; however, FGPs that occur in gastrointestinal polyposis syndromes such as familial adenomatosis polyposis can progress to adenocarcinoma and require surveillance. Therefore, it is important to distinguish sporadic versus syndromic fundic gland polyposis. Gastric adenocarcinoma and proximal polyposis of the stomach is a recently described condition that significantly increases the risk of developing invasive gastric adenocarcinoma from FGPs. Diagnostic criteria include (1) gastric polyposis restricted to the body and fundus with no small bowel or colonic involvement, (2) >100 gastric polyps or >30 polyps in a first-degree relative, (3) histology consistent with FGP with areas of dysplasia, (4) a family history consistent with an autosomal-dominant pattern of inheritance, and (5) exclusion of other syndromes and proton pump inhibitor use.¹ Unlike familial adenomatosis polyposis, the polyposis is restricted to the oxyntic mucosa of the gastric body and fundus with sparing of the gastric antrum, small bowel, and colon. The genetic basis of the disease has been attributed to a point mutation in the APC gene promotor IB region leading to a loss of tumor suppressor function.² Typical histology shows large FGPs with areas of low-grade and high-grade dysplasia, as seen in our patient.

There are few data on the natural history of gastric adenocarcinoma and proximal polyposis of the stomach, but effective surveillance is limited by the degree of polyposis. There are multiple reports of hidden adenocarcinoma on surgically resected specimens, as well as rapid progression to metastatic adenocarcinoma despite adequate diagnosis and surveillance.^1,3 Therefore, total gastrectomy should be offered to patients who are surgical candidates. Our patient underwent genetic testing that revealed a point mutation in the APC promotor IB. He declined surgical intervention and opted for surveillance endoscopy every 6 months.
 

References

1. Worthley D.L. et al. Gut. 2012;61:774-9

2. Li J et al. Am J Hum Genet. 2016;98:830-42

3. Rudloff U. Clin Exp Gastroenterol. 2018;11:447-59

Answer to ‘What’s your diagnosis?’: Gastric adenocarcinoma and proximal polyposis of the stomach syndrome.

Fundic gland polyps (FGPs) are the most common gastric polyps and when occurring in the sporadic setting are typically benign; however, FGPs that occur in gastrointestinal polyposis syndromes such as familial adenomatosis polyposis can progress to adenocarcinoma and require surveillance. Therefore, it is important to distinguish sporadic versus syndromic fundic gland polyposis. Gastric adenocarcinoma and proximal polyposis of the stomach is a recently described condition that significantly increases the risk of developing invasive gastric adenocarcinoma from FGPs. Diagnostic criteria include (1) gastric polyposis restricted to the body and fundus with no small bowel or colonic involvement, (2) >100 gastric polyps or >30 polyps in a first-degree relative, (3) histology consistent with FGP with areas of dysplasia, (4) a family history consistent with an autosomal-dominant pattern of inheritance, and (5) exclusion of other syndromes and proton pump inhibitor use.¹ Unlike familial adenomatosis polyposis, the polyposis is restricted to the oxyntic mucosa of the gastric body and fundus with sparing of the gastric antrum, small bowel, and colon. The genetic basis of the disease has been attributed to a point mutation in the APC gene promotor IB region leading to a loss of tumor suppressor function.² Typical histology shows large FGPs with areas of low-grade and high-grade dysplasia, as seen in our patient.

There are few data on the natural history of gastric adenocarcinoma and proximal polyposis of the stomach, but effective surveillance is limited by the degree of polyposis. There are multiple reports of hidden adenocarcinoma on surgically resected specimens, as well as rapid progression to metastatic adenocarcinoma despite adequate diagnosis and surveillance.^1,3 Therefore, total gastrectomy should be offered to patients who are surgical candidates. Our patient underwent genetic testing that revealed a point mutation in the APC promotor IB. He declined surgical intervention and opted for surveillance endoscopy every 6 months.
 

References

1. Worthley D.L. et al. Gut. 2012;61:774-9

2. Li J et al. Am J Hum Genet. 2016;98:830-42

3. Rudloff U. Clin Exp Gastroenterol. 2018;11:447-59

Answer: Blue rubber bleb nevus syndrome.

Based on the history of hemangioma resection and vascular lesions in the small intestine, along with typical manifestations of chronic gastrointestinal bleeding, diagnosis of blue rubber bleb nevus syndrome (BRBNS) was made. According to an American College of Gastroenterology Clinical Guideline,¹ for patients with recurrence of small bowel bleeding, endoscopic management could be considered depending on the patient’s clinical course and response to prior therapy. Consequently, injections of lauromacrogol with SBE (single-balloon enteroscopy) were given (Figure D). Lesions that ranged from 1 to 2 cm were injected with 1-2 mL lauromacrogol until the mucosa turned white. Three SBEs had been performed in a 5-month period. A total of 20 lesions were successfully treated with lauromacrogol. The treated hemangiomas became small, and the site healed 5 months after treatment (Figures E and F). The patient has been followed for 1 year, and he remains in good clinical condition with his latest hemoglobin level at 110 g/L. No further blood transfusion is needed.

161701_FigsDEF_web.jpg

BRBNS is a rare disorder characterized by discrete venous malformations of varying size and appearance that are present on the skin and within the gastrointestinal tract.²With wider application of video capsule endoscopy (VCE) and the increase of image resolution, the detection rate and diagnostic accuracy of BRBNS are significantly improved. Treatment of BRBNS varies depending on the site, size, and number of lesions. Medication, surgery, and endoscopic therapy are currently clinically applied. The successful use of sirolimus was recently reported in the treatment of vascular lesions.³Sirolimus has potential adverse effects on renal function, bone marrow, and cholesterol metabolism, however. In consideration of the patient’s young age, we did not adopt this method. Surgical resection is more suitable for limited or life-threatening lesions. The lesions in this patient were mild and sporadic. Consequently, in this case, endoscopic injection of lauromacrogol was performed. This was the most complicated case of endoscopic treatment of BRBNS in our center and proved lauromacrogol injection was a feasible approach. According to a literature review, lauromacrogol has been used to treat vascular lesions for decades, but there is still no standard instruction for the dosage of lauromacrogol. We hope that our experience can be a reference for the endoscopic treatment of BRBNS.

References (add links)

1. Gerson LB et al. ACG clinical guideline: Diagnosis and management of small bowel bleeding. Am J Gastroenterol.

2. Felton SJ and Ferguson JE. Multiple cutaneous swellings associated with sudden collapse. JAMA.

3. Yuksekkaya H et al. Blue rubber bleb nevus syndrome: Successful treatment with sirolimus. Pediatrics.

Answer: Blue rubber bleb nevus syndrome.

Based on the history of hemangioma resection and vascular lesions in the small intestine, along with typical manifestations of chronic gastrointestinal bleeding, diagnosis of blue rubber bleb nevus syndrome (BRBNS) was made. According to an American College of Gastroenterology Clinical Guideline,¹ for patients with recurrence of small bowel bleeding, endoscopic management could be considered depending on the patient’s clinical course and response to prior therapy. Consequently, injections of lauromacrogol with SBE (single-balloon enteroscopy) were given (Figure D). Lesions that ranged from 1 to 2 cm were injected with 1-2 mL lauromacrogol until the mucosa turned white. Three SBEs had been performed in a 5-month period. A total of 20 lesions were successfully treated with lauromacrogol. The treated hemangiomas became small, and the site healed 5 months after treatment (Figures E and F). The patient has been followed for 1 year, and he remains in good clinical condition with his latest hemoglobin level at 110 g/L. No further blood transfusion is needed.

161701_FigsDEF_web.jpg

BRBNS is a rare disorder characterized by discrete venous malformations of varying size and appearance that are present on the skin and within the gastrointestinal tract.²With wider application of video capsule endoscopy (VCE) and the increase of image resolution, the detection rate and diagnostic accuracy of BRBNS are significantly improved. Treatment of BRBNS varies depending on the site, size, and number of lesions. Medication, surgery, and endoscopic therapy are currently clinically applied. The successful use of sirolimus was recently reported in the treatment of vascular lesions.³Sirolimus has potential adverse effects on renal function, bone marrow, and cholesterol metabolism, however. In consideration of the patient’s young age, we did not adopt this method. Surgical resection is more suitable for limited or life-threatening lesions. The lesions in this patient were mild and sporadic. Consequently, in this case, endoscopic injection of lauromacrogol was performed. This was the most complicated case of endoscopic treatment of BRBNS in our center and proved lauromacrogol injection was a feasible approach. According to a literature review, lauromacrogol has been used to treat vascular lesions for decades, but there is still no standard instruction for the dosage of lauromacrogol. We hope that our experience can be a reference for the endoscopic treatment of BRBNS.

References (add links)

1. Gerson LB et al. ACG clinical guideline: Diagnosis and management of small bowel bleeding. Am J Gastroenterol.

2. Felton SJ and Ferguson JE. Multiple cutaneous swellings associated with sudden collapse. JAMA.

3. Yuksekkaya H et al. Blue rubber bleb nevus syndrome: Successful treatment with sirolimus. Pediatrics.

Answer: Blue rubber bleb nevus syndrome.

Based on the history of hemangioma resection and vascular lesions in the small intestine, along with typical manifestations of chronic gastrointestinal bleeding, diagnosis of blue rubber bleb nevus syndrome (BRBNS) was made. According to an American College of Gastroenterology Clinical Guideline,¹ for patients with recurrence of small bowel bleeding, endoscopic management could be considered depending on the patient’s clinical course and response to prior therapy. Consequently, injections of lauromacrogol with SBE (single-balloon enteroscopy) were given (Figure D). Lesions that ranged from 1 to 2 cm were injected with 1-2 mL lauromacrogol until the mucosa turned white. Three SBEs had been performed in a 5-month period. A total of 20 lesions were successfully treated with lauromacrogol. The treated hemangiomas became small, and the site healed 5 months after treatment (Figures E and F). The patient has been followed for 1 year, and he remains in good clinical condition with his latest hemoglobin level at 110 g/L. No further blood transfusion is needed.

161701_FigsDEF_web.jpg

BRBNS is a rare disorder characterized by discrete venous malformations of varying size and appearance that are present on the skin and within the gastrointestinal tract.²With wider application of video capsule endoscopy (VCE) and the increase of image resolution, the detection rate and diagnostic accuracy of BRBNS are significantly improved. Treatment of BRBNS varies depending on the site, size, and number of lesions. Medication, surgery, and endoscopic therapy are currently clinically applied. The successful use of sirolimus was recently reported in the treatment of vascular lesions.³Sirolimus has potential adverse effects on renal function, bone marrow, and cholesterol metabolism, however. In consideration of the patient’s young age, we did not adopt this method. Surgical resection is more suitable for limited or life-threatening lesions. The lesions in this patient were mild and sporadic. Consequently, in this case, endoscopic injection of lauromacrogol was performed. This was the most complicated case of endoscopic treatment of BRBNS in our center and proved lauromacrogol injection was a feasible approach. According to a literature review, lauromacrogol has been used to treat vascular lesions for decades, but there is still no standard instruction for the dosage of lauromacrogol. We hope that our experience can be a reference for the endoscopic treatment of BRBNS.

References (add links)

1. Gerson LB et al. ACG clinical guideline: Diagnosis and management of small bowel bleeding. Am J Gastroenterol.

2. Felton SJ and Ferguson JE. Multiple cutaneous swellings associated with sudden collapse. JAMA.

3. Yuksekkaya H et al. Blue rubber bleb nevus syndrome: Successful treatment with sirolimus. Pediatrics.

Syphilis 

Although extremely rare, we checked a Venereal Disease Research Laboratory PCR, the results of which came back positive. A Treponema pallidum hemagglutination assay also returned positive with titers 1:5120 (ULN, < 1:80), confirming the diagnosis of syphilis. During his hospitalization, the patient developed a syphilitic skin rash on his back, chest, palms, feet, and soles (Figure C). The patient was started on penicillin G, 4 million units intravenously every 4 hours. The fever broke 36 hours after antibiotic initiation and 48 hours later, his bilirubin started to downtrend, followed by the alkaline phosphatase and GGT 3 days later. His rash completely disappeared 5 days after antibiotic initiation. He received a total of 2 weeks of penicillin G intravenously at 24 million units a day and his liver enzymes normalized 7 weeks later. 

160898_Fig_C_web.jpg

In our case, the patient had several other possible reasons for his liver enzyme elevation, including drug-induced liver injury, cocaine, and alcohol use, which could have contributed to his disturbed liver enzymes. The steady improvement in his cholestatic liver enzymes, fever, and rash, shortly after the initiation of penicillin G indicates that syphilis was the cause of his hepatitis. Given the improvement in his symptoms and biochemical markers, we refrained from obtaining a liver biopsy.

References 

1. Lee M., Wang C., Dorer R. et al. A great masquerader: Acute syphilitic hepatitis. Dig Dis Sci. 2013;58:923-5. 
2. Mullick CJ. Liappis A.P. Benator D.A. et al. Syphilitic hepatitis in HIV-infected patients: A report of 7 cases and review of the literature. Clin Infect Dis. 2004;39:e100-e105. 
3. German MN. Matkowskyj K.A. Hoffman R.J. et al. A case of syphilitic hepatitis in an HIV-infected patient. Hum Pathol. 2018;79:184-7.

Syphilis 

Although extremely rare, we checked a Venereal Disease Research Laboratory PCR, the results of which came back positive. A Treponema pallidum hemagglutination assay also returned positive with titers 1:5120 (ULN, < 1:80), confirming the diagnosis of syphilis. During his hospitalization, the patient developed a syphilitic skin rash on his back, chest, palms, feet, and soles (Figure C). The patient was started on penicillin G, 4 million units intravenously every 4 hours. The fever broke 36 hours after antibiotic initiation and 48 hours later, his bilirubin started to downtrend, followed by the alkaline phosphatase and GGT 3 days later. His rash completely disappeared 5 days after antibiotic initiation. He received a total of 2 weeks of penicillin G intravenously at 24 million units a day and his liver enzymes normalized 7 weeks later. 

160898_Fig_C_web.jpg

In our case, the patient had several other possible reasons for his liver enzyme elevation, including drug-induced liver injury, cocaine, and alcohol use, which could have contributed to his disturbed liver enzymes. The steady improvement in his cholestatic liver enzymes, fever, and rash, shortly after the initiation of penicillin G indicates that syphilis was the cause of his hepatitis. Given the improvement in his symptoms and biochemical markers, we refrained from obtaining a liver biopsy.

References 

1. Lee M., Wang C., Dorer R. et al. A great masquerader: Acute syphilitic hepatitis. Dig Dis Sci. 2013;58:923-5. 
2. Mullick CJ. Liappis A.P. Benator D.A. et al. Syphilitic hepatitis in HIV-infected patients: A report of 7 cases and review of the literature. Clin Infect Dis. 2004;39:e100-e105. 
3. German MN. Matkowskyj K.A. Hoffman R.J. et al. A case of syphilitic hepatitis in an HIV-infected patient. Hum Pathol. 2018;79:184-7.

Syphilis 

Although extremely rare, we checked a Venereal Disease Research Laboratory PCR, the results of which came back positive. A Treponema pallidum hemagglutination assay also returned positive with titers 1:5120 (ULN, < 1:80), confirming the diagnosis of syphilis. During his hospitalization, the patient developed a syphilitic skin rash on his back, chest, palms, feet, and soles (Figure C). The patient was started on penicillin G, 4 million units intravenously every 4 hours. The fever broke 36 hours after antibiotic initiation and 48 hours later, his bilirubin started to downtrend, followed by the alkaline phosphatase and GGT 3 days later. His rash completely disappeared 5 days after antibiotic initiation. He received a total of 2 weeks of penicillin G intravenously at 24 million units a day and his liver enzymes normalized 7 weeks later. 

160898_Fig_C_web.jpg

In our case, the patient had several other possible reasons for his liver enzyme elevation, including drug-induced liver injury, cocaine, and alcohol use, which could have contributed to his disturbed liver enzymes. The steady improvement in his cholestatic liver enzymes, fever, and rash, shortly after the initiation of penicillin G indicates that syphilis was the cause of his hepatitis. Given the improvement in his symptoms and biochemical markers, we refrained from obtaining a liver biopsy.

References 

1. Lee M., Wang C., Dorer R. et al. A great masquerader: Acute syphilitic hepatitis. Dig Dis Sci. 2013;58:923-5. 
2. Mullick CJ. Liappis A.P. Benator D.A. et al. Syphilitic hepatitis in HIV-infected patients: A report of 7 cases and review of the literature. Clin Infect Dis. 2004;39:e100-e105. 
3. German MN. Matkowskyj K.A. Hoffman R.J. et al. A case of syphilitic hepatitis in an HIV-infected patient. Hum Pathol. 2018;79:184-7.

Whipple's disease

The ultrasound features were highly suggestive of malabsorption, a hypothesis that was supported by the laboratory findings. Celiac disease, one of the most common causes of malabsorption, was excluded by serology tests. Esophagogastroduodenoscopy was therefore repeated: The mucosa of the distal first part and second part of the duodenum appeared completely covered with tiny white spots (Figure C). Histologic examination revealed that the mucosal architecture of the villi was altered by the presence of infiltrates of macrophages with wide cytoplasm filled with round periodic acid-Schiff (PAS)-positive inclusions, associated to aggregates of neutrophils attacking the epithelium (Figure D). These histologic findings are consistent with Whipple's disease.

159852_Fig C_and_D_web.jpg

In the literature, few case reports describe the ultrasound findings in patients with Whipple's disease. The most frequent sonographic features include small-bowel dilatation with wall thickening, the presence of peri-intestinal fluid effusion and mesenteric and retroperitoneal lymphadenopathy.2,3 

The final diagnosis relies on intestinal biopsy and the histologic finding of foamy macrophages containing large amounts of diastase-resistant PAS-positive particles in the lamina propria of the duodenum, jejunum, ileum, or gastric antral region.

The diagnosis, particularly in cases of extraintestinal involvement, can be confirmed by polymerase chain reaction positivity for T. whipplei in the examined tissue. 

Therapy consists of the administration of ceftriaxone (2 g IV once daily) for 2 weeks followed by oral therapy with trimethoprim-sulfamethoxazole for 1 year. 
 

References 

1. Schneider T et al. Whipple's disease: New aspects of pathogenesis and treatment. Lancet Infect Dis. 2008;8:179-90. 
2. Brindicci D et al. Ultrasonic findings in Whipple's disease. J Clin Ultrasound. 1984;12:286-8. 
3. Neye H et al. Der Morbus Whipple's Disease - A rare intestinal disease and its sonographic characteristics. Ultraschall Med. 2012;33(04):314-5.

Whipple's disease

The ultrasound features were highly suggestive of malabsorption, a hypothesis that was supported by the laboratory findings. Celiac disease, one of the most common causes of malabsorption, was excluded by serology tests. Esophagogastroduodenoscopy was therefore repeated: The mucosa of the distal first part and second part of the duodenum appeared completely covered with tiny white spots (Figure C). Histologic examination revealed that the mucosal architecture of the villi was altered by the presence of infiltrates of macrophages with wide cytoplasm filled with round periodic acid-Schiff (PAS)-positive inclusions, associated to aggregates of neutrophils attacking the epithelium (Figure D). These histologic findings are consistent with Whipple's disease.

159852_Fig C_and_D_web.jpg

In the literature, few case reports describe the ultrasound findings in patients with Whipple's disease. The most frequent sonographic features include small-bowel dilatation with wall thickening, the presence of peri-intestinal fluid effusion and mesenteric and retroperitoneal lymphadenopathy.2,3 

The final diagnosis relies on intestinal biopsy and the histologic finding of foamy macrophages containing large amounts of diastase-resistant PAS-positive particles in the lamina propria of the duodenum, jejunum, ileum, or gastric antral region.

The diagnosis, particularly in cases of extraintestinal involvement, can be confirmed by polymerase chain reaction positivity for T. whipplei in the examined tissue. 

Therapy consists of the administration of ceftriaxone (2 g IV once daily) for 2 weeks followed by oral therapy with trimethoprim-sulfamethoxazole for 1 year. 
 

References 

1. Schneider T et al. Whipple's disease: New aspects of pathogenesis and treatment. Lancet Infect Dis. 2008;8:179-90. 
2. Brindicci D et al. Ultrasonic findings in Whipple's disease. J Clin Ultrasound. 1984;12:286-8. 
3. Neye H et al. Der Morbus Whipple's Disease - A rare intestinal disease and its sonographic characteristics. Ultraschall Med. 2012;33(04):314-5.

Whipple's disease

The ultrasound features were highly suggestive of malabsorption, a hypothesis that was supported by the laboratory findings. Celiac disease, one of the most common causes of malabsorption, was excluded by serology tests. Esophagogastroduodenoscopy was therefore repeated: The mucosa of the distal first part and second part of the duodenum appeared completely covered with tiny white spots (Figure C). Histologic examination revealed that the mucosal architecture of the villi was altered by the presence of infiltrates of macrophages with wide cytoplasm filled with round periodic acid-Schiff (PAS)-positive inclusions, associated to aggregates of neutrophils attacking the epithelium (Figure D). These histologic findings are consistent with Whipple's disease.

159852_Fig C_and_D_web.jpg

In the literature, few case reports describe the ultrasound findings in patients with Whipple's disease. The most frequent sonographic features include small-bowel dilatation with wall thickening, the presence of peri-intestinal fluid effusion and mesenteric and retroperitoneal lymphadenopathy.2,3 

The final diagnosis relies on intestinal biopsy and the histologic finding of foamy macrophages containing large amounts of diastase-resistant PAS-positive particles in the lamina propria of the duodenum, jejunum, ileum, or gastric antral region.

The diagnosis, particularly in cases of extraintestinal involvement, can be confirmed by polymerase chain reaction positivity for T. whipplei in the examined tissue. 

Therapy consists of the administration of ceftriaxone (2 g IV once daily) for 2 weeks followed by oral therapy with trimethoprim-sulfamethoxazole for 1 year. 
 

References 

1. Schneider T et al. Whipple's disease: New aspects of pathogenesis and treatment. Lancet Infect Dis. 2008;8:179-90. 
2. Brindicci D et al. Ultrasonic findings in Whipple's disease. J Clin Ultrasound. 1984;12:286-8. 
3. Neye H et al. Der Morbus Whipple's Disease - A rare intestinal disease and its sonographic characteristics. Ultraschall Med. 2012;33(04):314-5.

Pancreatic adenocarcinoma arising from main duct intraductal papillary mucinous neoplasm with inadvertent main pancreatic duct stenting. 
The FNA was positive for carcinoma with abundant mucin, which, taken together with the imaging findings, was indicative of pancreatic adenocarcinoma arising from main duct intraductal papillary mucinous neoplasm (M-IPMN).  
The post-endoscopic retrograde cholangiopancreatography (ERCP) CT revealed inadvertent placement of the fully covered self-expanding metallic stent (fcSEMS) within the main pancreatic duct (MPD) stricture and persistent common bile duct (CBD) obstruction. On post hoc review of the fluoroscopic and cross-sectional imaging, it became evident that the massively dilated MPD was mistaken during ERCP for the CBD and left hepatic duct (Figure F). In addition, the patient also had several cysts within the liver (compatible with incidental polycystic liver disease), which further complicated real-time image interpretation.  

159010_answer_pho_web.jpg

Pancreatic adenocarcinoma arising from main duct intraductal papillary mucinous neoplasm with inadvertent main pancreatic duct stenting. 
The FNA was positive for carcinoma with abundant mucin, which, taken together with the imaging findings, was indicative of pancreatic adenocarcinoma arising from main duct intraductal papillary mucinous neoplasm (M-IPMN).  
The post-endoscopic retrograde cholangiopancreatography (ERCP) CT revealed inadvertent placement of the fully covered self-expanding metallic stent (fcSEMS) within the main pancreatic duct (MPD) stricture and persistent common bile duct (CBD) obstruction. On post hoc review of the fluoroscopic and cross-sectional imaging, it became evident that the massively dilated MPD was mistaken during ERCP for the CBD and left hepatic duct (Figure F). In addition, the patient also had several cysts within the liver (compatible with incidental polycystic liver disease), which further complicated real-time image interpretation.  

159010_answer_pho_web.jpg

Pancreatic adenocarcinoma arising from main duct intraductal papillary mucinous neoplasm with inadvertent main pancreatic duct stenting. 
The FNA was positive for carcinoma with abundant mucin, which, taken together with the imaging findings, was indicative of pancreatic adenocarcinoma arising from main duct intraductal papillary mucinous neoplasm (M-IPMN).  
The post-endoscopic retrograde cholangiopancreatography (ERCP) CT revealed inadvertent placement of the fully covered self-expanding metallic stent (fcSEMS) within the main pancreatic duct (MPD) stricture and persistent common bile duct (CBD) obstruction. On post hoc review of the fluoroscopic and cross-sectional imaging, it became evident that the massively dilated MPD was mistaken during ERCP for the CBD and left hepatic duct (Figure F). In addition, the patient also had several cysts within the liver (compatible with incidental polycystic liver disease), which further complicated real-time image interpretation.  

159010_answer_pho_web.jpg

Answer: Colonic Malakoplakia.

Histopathologic examination of the biopsy specimens revealed nodular mixed inflammatory cells and infiltration of the epithelioid histiocytes in lamina propria (Figure D; stain: hematoxylin and eosin; original magnification 40×). The histiocytes showed foamy and eosinophilic cytoplasm (Figure E, arrow) and some of them had a targetoid appearance (Figure E, arrow head; stain: hematoxylin and eosin; original magnification 200×). Von Kossa stains highlighted the targetoid structures in the histiocytes (Figure F, Michaelis-Gutmann bodies). The granular cytoplasm of the histiocytes was positive on periodic acid-Schiff stain (Figure G). Based on these findings, the patient was diagnosed with colonic malakoplakia.

157447_DEFG_web.jpg

Malakoplakia is an uncommon, chronic, granulomatous inflammatory disease. It most commonly affects the urinary tract and gastrointestinal tract, but may occur at any anatomic site. Malakoplakia of the gastrointestinal tract are seen most frequently in the rectum, sigmoid, and right colon.¹ It is diagnosed by the characteristic histologic feature of accumulated histiocytes with abundant eosinophilic granular cytoplasm containing basophilic inclusions, consistent with Michaelis-Gutmann bodies. Although the exact etiology and pathogenesis of malakoplakia are unclear, it seems to originate from an acquired defect in the intracellular destruction of phagocytosed bacteria, usually associated with Escherichia coli, Klebsiella, and Mycobacterium.² It can have various causes, such as immunosuppression, malignant neoplasms, systemic diseases, and genetic diseases. Clinical manifestation of colonic malakoplakia is diverse, ranging from asymptomatic to malaise, fever, abdominal pain, diarrhea, hematochezia, and intestinal obstruction. Granulomatous reaction of malakoplakia generates the endoscopic appearance of lesions, which ranges from plaques to nodules and yellow-brown masses. In the early stage, malakoplakia commonly presents as soft yellow to tan mucosal plaques endoscopically, as seen in our case (Figure A). As the disease progresses in the later stage, malakoplakia presents as raised, grey to tan polypoid lesions of various sizes with peripheral hyperemia and a central depressed area, as seen in our case (Figure B).³ Owing to this endoscopic morphology, colonic malakoplakia may be misdiagnosed as atypical lymphoma, familial adenomatous polyposis, and metastatic carcinoma. To date, the natural course of malakoplakia of the colon is unclear, and no guidelines for treatment, treatment methods, duration of treatment, or surveillance are currently available. However, treatment of malakoplakia is essential to reduce immunosuppression and includes antibiotics with intracellular action and choline agonists that replenish the decreased cyclic 3’, 5’-guanosine monophosphate levels. In summary, although malakoplakia of the colon is very rare, it should be considered in the differential diagnosis of polypoid colonic lesions, especially in immunocompromised or malnourished patients.
 

References

1. Cipolletta L et al. Gastrointest Endosc. 1995 Mar;41(3):255-8.

2. Berney T et al. Transpl Int. 1999;12(4):293-6.

3. Weinrach DM et al. Arch Pathol Lab Med. 2004 Oct;128(10):e133-4.

Answer: Colonic Malakoplakia.

Histopathologic examination of the biopsy specimens revealed nodular mixed inflammatory cells and infiltration of the epithelioid histiocytes in lamina propria (Figure D; stain: hematoxylin and eosin; original magnification 40×). The histiocytes showed foamy and eosinophilic cytoplasm (Figure E, arrow) and some of them had a targetoid appearance (Figure E, arrow head; stain: hematoxylin and eosin; original magnification 200×). Von Kossa stains highlighted the targetoid structures in the histiocytes (Figure F, Michaelis-Gutmann bodies). The granular cytoplasm of the histiocytes was positive on periodic acid-Schiff stain (Figure G). Based on these findings, the patient was diagnosed with colonic malakoplakia.

157447_DEFG_web.jpg

Malakoplakia is an uncommon, chronic, granulomatous inflammatory disease. It most commonly affects the urinary tract and gastrointestinal tract, but may occur at any anatomic site. Malakoplakia of the gastrointestinal tract are seen most frequently in the rectum, sigmoid, and right colon.¹ It is diagnosed by the characteristic histologic feature of accumulated histiocytes with abundant eosinophilic granular cytoplasm containing basophilic inclusions, consistent with Michaelis-Gutmann bodies. Although the exact etiology and pathogenesis of malakoplakia are unclear, it seems to originate from an acquired defect in the intracellular destruction of phagocytosed bacteria, usually associated with Escherichia coli, Klebsiella, and Mycobacterium.² It can have various causes, such as immunosuppression, malignant neoplasms, systemic diseases, and genetic diseases. Clinical manifestation of colonic malakoplakia is diverse, ranging from asymptomatic to malaise, fever, abdominal pain, diarrhea, hematochezia, and intestinal obstruction. Granulomatous reaction of malakoplakia generates the endoscopic appearance of lesions, which ranges from plaques to nodules and yellow-brown masses. In the early stage, malakoplakia commonly presents as soft yellow to tan mucosal plaques endoscopically, as seen in our case (Figure A). As the disease progresses in the later stage, malakoplakia presents as raised, grey to tan polypoid lesions of various sizes with peripheral hyperemia and a central depressed area, as seen in our case (Figure B).³ Owing to this endoscopic morphology, colonic malakoplakia may be misdiagnosed as atypical lymphoma, familial adenomatous polyposis, and metastatic carcinoma. To date, the natural course of malakoplakia of the colon is unclear, and no guidelines for treatment, treatment methods, duration of treatment, or surveillance are currently available. However, treatment of malakoplakia is essential to reduce immunosuppression and includes antibiotics with intracellular action and choline agonists that replenish the decreased cyclic 3’, 5’-guanosine monophosphate levels. In summary, although malakoplakia of the colon is very rare, it should be considered in the differential diagnosis of polypoid colonic lesions, especially in immunocompromised or malnourished patients.
 

References

1. Cipolletta L et al. Gastrointest Endosc. 1995 Mar;41(3):255-8.

2. Berney T et al. Transpl Int. 1999;12(4):293-6.

3. Weinrach DM et al. Arch Pathol Lab Med. 2004 Oct;128(10):e133-4.

Answer: Colonic Malakoplakia.

Histopathologic examination of the biopsy specimens revealed nodular mixed inflammatory cells and infiltration of the epithelioid histiocytes in lamina propria (Figure D; stain: hematoxylin and eosin; original magnification 40×). The histiocytes showed foamy and eosinophilic cytoplasm (Figure E, arrow) and some of them had a targetoid appearance (Figure E, arrow head; stain: hematoxylin and eosin; original magnification 200×). Von Kossa stains highlighted the targetoid structures in the histiocytes (Figure F, Michaelis-Gutmann bodies). The granular cytoplasm of the histiocytes was positive on periodic acid-Schiff stain (Figure G). Based on these findings, the patient was diagnosed with colonic malakoplakia.

157447_DEFG_web.jpg

Malakoplakia is an uncommon, chronic, granulomatous inflammatory disease. It most commonly affects the urinary tract and gastrointestinal tract, but may occur at any anatomic site. Malakoplakia of the gastrointestinal tract are seen most frequently in the rectum, sigmoid, and right colon.¹ It is diagnosed by the characteristic histologic feature of accumulated histiocytes with abundant eosinophilic granular cytoplasm containing basophilic inclusions, consistent with Michaelis-Gutmann bodies. Although the exact etiology and pathogenesis of malakoplakia are unclear, it seems to originate from an acquired defect in the intracellular destruction of phagocytosed bacteria, usually associated with Escherichia coli, Klebsiella, and Mycobacterium.² It can have various causes, such as immunosuppression, malignant neoplasms, systemic diseases, and genetic diseases. Clinical manifestation of colonic malakoplakia is diverse, ranging from asymptomatic to malaise, fever, abdominal pain, diarrhea, hematochezia, and intestinal obstruction. Granulomatous reaction of malakoplakia generates the endoscopic appearance of lesions, which ranges from plaques to nodules and yellow-brown masses. In the early stage, malakoplakia commonly presents as soft yellow to tan mucosal plaques endoscopically, as seen in our case (Figure A). As the disease progresses in the later stage, malakoplakia presents as raised, grey to tan polypoid lesions of various sizes with peripheral hyperemia and a central depressed area, as seen in our case (Figure B).³ Owing to this endoscopic morphology, colonic malakoplakia may be misdiagnosed as atypical lymphoma, familial adenomatous polyposis, and metastatic carcinoma. To date, the natural course of malakoplakia of the colon is unclear, and no guidelines for treatment, treatment methods, duration of treatment, or surveillance are currently available. However, treatment of malakoplakia is essential to reduce immunosuppression and includes antibiotics with intracellular action and choline agonists that replenish the decreased cyclic 3’, 5’-guanosine monophosphate levels. In summary, although malakoplakia of the colon is very rare, it should be considered in the differential diagnosis of polypoid colonic lesions, especially in immunocompromised or malnourished patients.
 

References

1. Cipolletta L et al. Gastrointest Endosc. 1995 Mar;41(3):255-8.

2. Berney T et al. Transpl Int. 1999;12(4):293-6.

3. Weinrach DM et al. Arch Pathol Lab Med. 2004 Oct;128(10):e133-4.

Mucous membrane pemphigoid with esophageal web stricture. 

Additional laboratory examination showed that his serum anti-BP180 antibody level was high (11.7 U/mL; normal range, <9.0 U/mL). Biopsy specimens taken from the laryngopharyngeal erosion showed subepithelial blister formation and it was consistent with pemphigoid pathologically (Figure D). He did not have cutaneous lesions and was diagnosed with mucous membrane pemphigoid (MMP). After performing endoscopic dilation, prednisolone (20 mg/d) was administered orally. Three months after starting the prednisolone treatment, follow-up endoscopy showed improvements of the laryngopharyngeal erosions (Figure E) and esophageal blister on the web. However, esophageal narrowing remained, and thus endoscopic balloon dilation was performed (Figure F-H). Three months after the dilation, the narrowing improved (Figure I). 

156356_d_to_i_web.jpg

Mucous membrane pemphigoid with esophageal web stricture. 

Additional laboratory examination showed that his serum anti-BP180 antibody level was high (11.7 U/mL; normal range, <9.0 U/mL). Biopsy specimens taken from the laryngopharyngeal erosion showed subepithelial blister formation and it was consistent with pemphigoid pathologically (Figure D). He did not have cutaneous lesions and was diagnosed with mucous membrane pemphigoid (MMP). After performing endoscopic dilation, prednisolone (20 mg/d) was administered orally. Three months after starting the prednisolone treatment, follow-up endoscopy showed improvements of the laryngopharyngeal erosions (Figure E) and esophageal blister on the web. However, esophageal narrowing remained, and thus endoscopic balloon dilation was performed (Figure F-H). Three months after the dilation, the narrowing improved (Figure I). 

156356_d_to_i_web.jpg

Mucous membrane pemphigoid with esophageal web stricture. 

Additional laboratory examination showed that his serum anti-BP180 antibody level was high (11.7 U/mL; normal range, <9.0 U/mL). Biopsy specimens taken from the laryngopharyngeal erosion showed subepithelial blister formation and it was consistent with pemphigoid pathologically (Figure D). He did not have cutaneous lesions and was diagnosed with mucous membrane pemphigoid (MMP). After performing endoscopic dilation, prednisolone (20 mg/d) was administered orally. Three months after starting the prednisolone treatment, follow-up endoscopy showed improvements of the laryngopharyngeal erosions (Figure E) and esophageal blister on the web. However, esophageal narrowing remained, and thus endoscopic balloon dilation was performed (Figure F-H). Three months after the dilation, the narrowing improved (Figure I). 

156356_d_to_i_web.jpg

Answer: Infectious gastroparesis secondary to acute hepatitis A infection.

A computed tomography scan of the abdomen/pelvis demonstrated marked gastric distention without obvious obstructing mass and normal caliber small bowel and colon. Additional laboratory workup revealed a positive hepatitis A IgM antibody. Hepatitis B surface antigen and core IgM antibody were negative, as was the hepatitis C virus antibody. Human immunodeficiency virus antigen and antibody were negative. An esophagogastroduodenoscopy was performed that showed a large amount of food in a dilated and atonic stomach.

With conservative treatment, the patient’s liver enzymes trended down over the next 2 days to alanine aminotransferase 993 U/L, aspartate aminotransferase 244 U/L, and direct bilirubin 3.8 mg/dL. At the time of discharge, she was tolerating soft foods without any difficulty. She was educated on taking appropriate precautions to avoid transmitting the hepatitis A infection to others. Her risk factor for hepatitis A was recent incarceration.

Here we highlight a rare case of infectious gastroparesis secondary to hepatitis A infection. Hepatitis A virus is a small, nonenveloped, RNA-containing virus.¹ It typically presents with a self-limited illness with liver failure occurring in rare cases. Common presenting symptoms including nausea, vomiting, jaundice, fever, diarrhea, and abdominal pain.Laboratory abnormalities include elevations in the serum aminotransferases, alkaline phosphatase, and total bilirubin.² The diagnosis is confirmed with a positive hepatitis A IgM antibody. The most common route of transmission is the fecal-oral route such as through consumption of contaminated water and food or from person-to-person contact.¹ Individuals can develop immunity to the virus either from prior infection or vaccination.

Gastroparesis refers to delayed emptying of gastric contents when mechanical obstruction has been ruled out. Common causes of gastroparesis include diabetes mellitus, medications, postoperative complications, and infections. Infectious gastroparesis may present acutely after a viral prodrome and symptoms may be severe and slow to resolve.³

References

1. Lemon SM. N Engl J Med. 1985 Oct 24;313(17):1059-67.

2. Tong MJ et al. J Infect Dis. 1995 Mar;171 Suppl 1:S15-8.

3. Bityutskiy LP. Am J Gastroenterol. 1997 Sep;92(9):1501-4.

Answer: Infectious gastroparesis secondary to acute hepatitis A infection.

A computed tomography scan of the abdomen/pelvis demonstrated marked gastric distention without obvious obstructing mass and normal caliber small bowel and colon. Additional laboratory workup revealed a positive hepatitis A IgM antibody. Hepatitis B surface antigen and core IgM antibody were negative, as was the hepatitis C virus antibody. Human immunodeficiency virus antigen and antibody were negative. An esophagogastroduodenoscopy was performed that showed a large amount of food in a dilated and atonic stomach.

With conservative treatment, the patient’s liver enzymes trended down over the next 2 days to alanine aminotransferase 993 U/L, aspartate aminotransferase 244 U/L, and direct bilirubin 3.8 mg/dL. At the time of discharge, she was tolerating soft foods without any difficulty. She was educated on taking appropriate precautions to avoid transmitting the hepatitis A infection to others. Her risk factor for hepatitis A was recent incarceration.

Here we highlight a rare case of infectious gastroparesis secondary to hepatitis A infection. Hepatitis A virus is a small, nonenveloped, RNA-containing virus.¹ It typically presents with a self-limited illness with liver failure occurring in rare cases. Common presenting symptoms including nausea, vomiting, jaundice, fever, diarrhea, and abdominal pain.Laboratory abnormalities include elevations in the serum aminotransferases, alkaline phosphatase, and total bilirubin.² The diagnosis is confirmed with a positive hepatitis A IgM antibody. The most common route of transmission is the fecal-oral route such as through consumption of contaminated water and food or from person-to-person contact.¹ Individuals can develop immunity to the virus either from prior infection or vaccination.

Gastroparesis refers to delayed emptying of gastric contents when mechanical obstruction has been ruled out. Common causes of gastroparesis include diabetes mellitus, medications, postoperative complications, and infections. Infectious gastroparesis may present acutely after a viral prodrome and symptoms may be severe and slow to resolve.³

References

1. Lemon SM. N Engl J Med. 1985 Oct 24;313(17):1059-67.

2. Tong MJ et al. J Infect Dis. 1995 Mar;171 Suppl 1:S15-8.

3. Bityutskiy LP. Am J Gastroenterol. 1997 Sep;92(9):1501-4.

Answer: Infectious gastroparesis secondary to acute hepatitis A infection.

A computed tomography scan of the abdomen/pelvis demonstrated marked gastric distention without obvious obstructing mass and normal caliber small bowel and colon. Additional laboratory workup revealed a positive hepatitis A IgM antibody. Hepatitis B surface antigen and core IgM antibody were negative, as was the hepatitis C virus antibody. Human immunodeficiency virus antigen and antibody were negative. An esophagogastroduodenoscopy was performed that showed a large amount of food in a dilated and atonic stomach.

With conservative treatment, the patient’s liver enzymes trended down over the next 2 days to alanine aminotransferase 993 U/L, aspartate aminotransferase 244 U/L, and direct bilirubin 3.8 mg/dL. At the time of discharge, she was tolerating soft foods without any difficulty. She was educated on taking appropriate precautions to avoid transmitting the hepatitis A infection to others. Her risk factor for hepatitis A was recent incarceration.

Here we highlight a rare case of infectious gastroparesis secondary to hepatitis A infection. Hepatitis A virus is a small, nonenveloped, RNA-containing virus.¹ It typically presents with a self-limited illness with liver failure occurring in rare cases. Common presenting symptoms including nausea, vomiting, jaundice, fever, diarrhea, and abdominal pain.Laboratory abnormalities include elevations in the serum aminotransferases, alkaline phosphatase, and total bilirubin.² The diagnosis is confirmed with a positive hepatitis A IgM antibody. The most common route of transmission is the fecal-oral route such as through consumption of contaminated water and food or from person-to-person contact.¹ Individuals can develop immunity to the virus either from prior infection or vaccination.

Gastroparesis refers to delayed emptying of gastric contents when mechanical obstruction has been ruled out. Common causes of gastroparesis include diabetes mellitus, medications, postoperative complications, and infections. Infectious gastroparesis may present acutely after a viral prodrome and symptoms may be severe and slow to resolve.³

References

1. Lemon SM. N Engl J Med. 1985 Oct 24;313(17):1059-67.

2. Tong MJ et al. J Infect Dis. 1995 Mar;171 Suppl 1:S15-8.

3. Bityutskiy LP. Am J Gastroenterol. 1997 Sep;92(9):1501-4.

Sigmoid colon perforation secondary to transcutaneous epicardial pacer wires.

A plain film image (Figure A) shows diffusely dilated loops of bowel with subdiaphragmatic air concerning for GI viscous perforation. Dedicated cross-sectional imaging confirms intra-abdominal free air, and in representative cross section, the epicardial pacing wires can be visualized within the gastrointestinal lumen (Figure D, arrows). At the time of surgical consultation, the radiology report was notable for concern regarding possible disruption of peritoneum secondary to the difficult surgical chest tube placement in a patient with a high-riding left hemidiaphragm. Urgent laparoscopic exploration secondary to these findings unexpectedly revealed that the transcutaneous epicardial pacing wires had been inadvertently placed through the sigmoid colon (Figure C). The pacer wires were cut and removed intraoperatively. Unfortunately, 4 days after removal of pacer wires, the patient continued to have ongoing distension and was found to have sigmoid volvulus necessitating endoscopic decompression. After a prolonged hospitalization and recovery, he was discharged with a normal bowel pattern and tolerating oral intake to a skilled nursing facility.

154439_figureCD_web.jpg

Temporary transcutaneous epicardial pacing wires are often placed after complex cardiovascular surgical procedures. Complications from wire placement are thought to be relatively rare and are typically associated with migration into local structures after wire placement and infectious complications secondary to retained wires.^1,2 Perforation of local structures during placement is less common, and GI viscous perforation in particular is not a well-characterized cause of associated morbidity.³

Our case demonstrates that, in patients with hemidiaphragm elevation, epicardial wire placement risks GI viscous perforation. Furthermore, given the frequency of concomitant surgical hardware in this patient population, identification of malpositioned epicardial wires on plain film and even cross-sectional imaging can be difficult and can delay diagnosis.

References

1. Del Nido P, Goldman BS. J Card Surg. 1989 Mar;4(1):99-103.

2. Kapoor A et al. Interact Cardiovasc Thorac Surg. 2011 Jul;13(1):104-6.

3. Haba J et al. Can Assoc Radiol J. 2013 Feb;64(1):77-80.

Sigmoid colon perforation secondary to transcutaneous epicardial pacer wires.

A plain film image (Figure A) shows diffusely dilated loops of bowel with subdiaphragmatic air concerning for GI viscous perforation. Dedicated cross-sectional imaging confirms intra-abdominal free air, and in representative cross section, the epicardial pacing wires can be visualized within the gastrointestinal lumen (Figure D, arrows). At the time of surgical consultation, the radiology report was notable for concern regarding possible disruption of peritoneum secondary to the difficult surgical chest tube placement in a patient with a high-riding left hemidiaphragm. Urgent laparoscopic exploration secondary to these findings unexpectedly revealed that the transcutaneous epicardial pacing wires had been inadvertently placed through the sigmoid colon (Figure C). The pacer wires were cut and removed intraoperatively. Unfortunately, 4 days after removal of pacer wires, the patient continued to have ongoing distension and was found to have sigmoid volvulus necessitating endoscopic decompression. After a prolonged hospitalization and recovery, he was discharged with a normal bowel pattern and tolerating oral intake to a skilled nursing facility.

154439_figureCD_web.jpg

Temporary transcutaneous epicardial pacing wires are often placed after complex cardiovascular surgical procedures. Complications from wire placement are thought to be relatively rare and are typically associated with migration into local structures after wire placement and infectious complications secondary to retained wires.^1,2 Perforation of local structures during placement is less common, and GI viscous perforation in particular is not a well-characterized cause of associated morbidity.³

Our case demonstrates that, in patients with hemidiaphragm elevation, epicardial wire placement risks GI viscous perforation. Furthermore, given the frequency of concomitant surgical hardware in this patient population, identification of malpositioned epicardial wires on plain film and even cross-sectional imaging can be difficult and can delay diagnosis.

References

1. Del Nido P, Goldman BS. J Card Surg. 1989 Mar;4(1):99-103.

2. Kapoor A et al. Interact Cardiovasc Thorac Surg. 2011 Jul;13(1):104-6.

3. Haba J et al. Can Assoc Radiol J. 2013 Feb;64(1):77-80.

Sigmoid colon perforation secondary to transcutaneous epicardial pacer wires.

A plain film image (Figure A) shows diffusely dilated loops of bowel with subdiaphragmatic air concerning for GI viscous perforation. Dedicated cross-sectional imaging confirms intra-abdominal free air, and in representative cross section, the epicardial pacing wires can be visualized within the gastrointestinal lumen (Figure D, arrows). At the time of surgical consultation, the radiology report was notable for concern regarding possible disruption of peritoneum secondary to the difficult surgical chest tube placement in a patient with a high-riding left hemidiaphragm. Urgent laparoscopic exploration secondary to these findings unexpectedly revealed that the transcutaneous epicardial pacing wires had been inadvertently placed through the sigmoid colon (Figure C). The pacer wires were cut and removed intraoperatively. Unfortunately, 4 days after removal of pacer wires, the patient continued to have ongoing distension and was found to have sigmoid volvulus necessitating endoscopic decompression. After a prolonged hospitalization and recovery, he was discharged with a normal bowel pattern and tolerating oral intake to a skilled nursing facility.

154439_figureCD_web.jpg

Temporary transcutaneous epicardial pacing wires are often placed after complex cardiovascular surgical procedures. Complications from wire placement are thought to be relatively rare and are typically associated with migration into local structures after wire placement and infectious complications secondary to retained wires.^1,2 Perforation of local structures during placement is less common, and GI viscous perforation in particular is not a well-characterized cause of associated morbidity.³

Our case demonstrates that, in patients with hemidiaphragm elevation, epicardial wire placement risks GI viscous perforation. Furthermore, given the frequency of concomitant surgical hardware in this patient population, identification of malpositioned epicardial wires on plain film and even cross-sectional imaging can be difficult and can delay diagnosis.

References

1. Del Nido P, Goldman BS. J Card Surg. 1989 Mar;4(1):99-103.

2. Kapoor A et al. Interact Cardiovasc Thorac Surg. 2011 Jul;13(1):104-6.

3. Haba J et al. Can Assoc Radiol J. 2013 Feb;64(1):77-80.