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Pancreatic vascular tumor or AVM? Utility of EUS in the diagnosis of hereditary hemorrhagic telangiectasia
At the Focal Point
Commentary Mucin-producing gallbladder carcinoma (MPGBC) is a rare biliary malignancy first described in the Japanese literature nearly 30 years ago. Both histologic variants, well-differentiated adenocarcinoma (intracystic papillary adenocarcinoma) and mucinous carcinoma, can produce massive amounts of mucus that make great radiologic photo ops. Although traditional gallbladder carcinoma is an assuredly aggressive lesion, what little is known about the natural history of MPGBC suggests a variable course. A familiar theme in GI oncology is the notion that the lethality of a cancer may relate more to residing in a symptom-evading location rather than having intrinsically sinister pathobiology. A tumor at the head of the pancreas that impinges on biliary drainage is likely to present earlier (with jaundice) than a similarly sized cancer situated further out along the tail (although the comparative prognosis of one location over another has not been well studied, patients with pancreatic cancers of the body and tail appear to have a poorer survival rate than those with head lesions). The majority of reported MPGBC patients present with symptoms attributed to distention of the gallbladder with characteristically viscous mucin: abdominal pain, fever, and jaundice. This case generates some interesting clinical queries beyond the utility of the extensive preoperative workup–the serendipitous transabdominal US would have been reason enough for surgical resection at our institution. I have never understood the rationale for nasogallbladder drainage in patients with acute cholecystitis, much less for comparatively “chronic” cancer, although this seems to be all the rage among the academic biliary endoscopists in Japan. And I doubt it is as popular among the unfortunate patients who are its beneficiaries. The efforts to preoperatively image and characterize this rare tumor, with a dizzying array of redundant and, in my opinion, superfluous but slick imagery, would, however, do the NSA (http://en.wikipedia.org/wiki/National_Security_Agency) proud. The SpyGlass platform has a cultlike following in the United States that even Snowden would covet. The option to perform a Spybiopsy (not that a biopsy would change management here) can be limited by poor visualization (see Video) compared with conventional peroral “baby scope” cholangioscopy. And stay tuned for improvements in the coming iteration. That said, we are indebted to our authors for this cinematic submission and look forward to the sequel. David Robbins, MD, MSc Assistant Editor for Focal Points
Pancreatic vascular tumor or AVM? Utility of EUS in the diagnosis of hereditary hemorrhagic telangiectasia A 57-year-old man was admitted for abdominal pain. CT showed acute cholecystitis and an incidental lesion in the pancreas. The cholecystitis was managed conservatively with antibiotics and resolved. A second CT scan with thin cuts revealed multiple pancreatic enhancing lesions (0.31 cm) suspicious for neuroendocrine lesions or vascular malformations (A). EUS was used to examine the pancreas with a plan to perform FNA. Anechoic lesions with positive Doppler flow were seen in the pancreas, suggestive of vascular malformations (B), and an uncinate lesion was supplied by the superior mesenteric artery (C). A gastric vascular malformation was incidentally seen (D). The patient subsequently provided a history of recurrent oral bleeding and epistaxis. Clinical examination revealed lingular and digital telangiectases. His father also had episodes of epistaxis. The patient was given a diagnosis of hereditary
hemorrhagic telangiectasia (HHT), and the pancreatic lesions were managed conservatively. EUS is useful in characterizing enhancing pancreatic lesions, and HHT should be considered when one is identified. DISCLOSURE All authors disclosed no financial relationships relevant to this publication. Eric Wee, MBBS, MRCP, MMed, Sushmitha Pearl Fernandes, MBBS, Gastroenterology, Department of General Medicine, Khoo Teck Puat Hospital, Singapore http://dx.doi.org/10.1016/j.gie.2013.10.046
Commentary The gray-haired GIE readership among us may shriek when hallowed clinical principles (such as taking a history before ordering a diagnostic test) are replaced by a “new school” strategy of upfront high-tech imagery and (maybe) asking questions later. At first blush, this Focal Point is another cool twist on the epidemic of incidentalomas that we face as the www.giejournal.org
Volume 79, No. 3 : 2014 GASTROINTESTINAL ENDOSCOPY 517
At the Focal Point
threshold to obtain high-resolution, cross-sectional imaging continues to drop lower than a limbo bar at a GI fellowship reunion party (the world record for lowest limbo dance is held by an 18-year-old Trinidadian woman who successfully traversed one only 8.5 inches off the ground). Also known as Osler-Weber-Rendu syndrome, hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant, board question favorite characterized by telangiectasia of the skin and mucous membranes and recurrent upper GI bleeding. If anyone asks, it is not associated with cholecystitis (certainly not the kind that is managed nonoperatively!). Genetic mutations in the endoglin and ALK1 genes, which code for proteins involved in normal blood vessel development and integrity, are responsible for the clinical hallmarks of recurrent childhood epistaxis and, later in life, melena. The lack of an elastic lamina normally responsible for vessel contractility leads to dilation of its end terminus and may explain the proclivity of these lesions to bleed, sometimes massively. The Curaçao criteria (named after an idyllic island in the southern
518 GASTROINTESTINAL ENDOSCOPY Volume 79, No. 3 : 2014
www.giejournal.org
At the Focal Point
Caribbean Sea, off the Venezuelan coast, that happens to be ideally situated for coming up with diagnostic criteria) include spontaneous, recurrent epistaxis; characteristic oral, nasal, or digital telangiectases; visceral vascular malformation lesions; and a similarly affected first-degree relative. Pancreatic involvement is common and asymptomatic in adult patients and is optimally imaged during arterial phase multi-detector CT. Telangiectases appear as highly enhancing round or irregular lesions; the differential should include pancreatic neuroendocrine tumors or hypervascular metastases (such as from hypernephroma). EUS with Doppler interrogation alone is ideally situated to distinguish among these possibilities (only a vessel is anechoic and Doppler positive) and EUS with FNA can be used to safely sample lesions (even vascular ones) with any degree of echogenicity. Turning back to our case, whether taking that history would have obviated the need for EUS is speculative but less fun than being reminded of one of my favorite self-deprecating Oslerisms: “Common sense in matters medical is rare, and is usually in inverse ratio to the degree of education.” That surely takes the pressure off all of us. David Robbins, MD, MSc Assistant Editor for Focal Points
Retrieval of a large foreign body from the ileum with double-balloon enteroscopy (with videos)
A 31-year-old, institutionalized man with a psychiatric disorder presented after he swallowed a plastic fork. Initial CT demonstrated the fork in the second portion of the duodenum, with the tines oriented distally. The patient was followed expectantly. A subsequent CT scan demonstrated the fork, now in the distal small bowel, still with tines directed distally (A). Thickening of the small-bowel wall with adjacent stranding was identified, without evidence of free air or fluid collection. A lower double-balloon enteroscopy was performed. The fork was located 30 cm from the ileocecal valve. One
of the outside prongs appeared embedded in the bowel wall. The fork was pushed back and out of the wall by use of a rat-tooth forceps. A large snare was threaded over the handle of the fork and brought back over the tines. The snare was closed, bunching the tines together (B). The fork was slowly withdrawn to the colon and removed (C) (Video 1; available online at www. giejournal.org). No significant trauma was seen. The patient was treated with intravenous antibiotics and admitted for 24-hour observation. Repeat abdominal plain films revealed no evidence of an adverse event.
www.giejournal.org
Volume 79, No. 3 : 2014 GASTROINTESTINAL ENDOSCOPY 519
Commentary Mucin-producing gallbladder carcinoma (MPGBC) is a rare biliary malignancy first described in the Japanese literature nearly 30 years ago. Both histologic variants, well-differentiated adenocarcinoma (intracystic papillary adenocarcinoma) and mucinous carcinoma, can produce massive amounts of mucus that make great radiologic photo ops. Although traditional gallbladder carcinoma is an assuredly aggressive lesion, what little is known about the natural history of MPGBC suggests a variable course. A familiar theme in GI oncology is the notion that the lethality of a cancer may relate more to residing in a symptom-evading location rather than having intrinsically sinister pathobiology. A tumor at the head of the pancreas that impinges on biliary drainage is likely to present earlier (with jaundice) than a similarly sized cancer situated further out along the tail (although the comparative prognosis of one location over another has not been well studied, patients with pancreatic cancers of the body and tail appear to have a poorer survival rate than those with head lesions). The majority of reported MPGBC patients present with symptoms attributed to distention of the gallbladder with characteristically viscous mucin: abdominal pain, fever, and jaundice. This case generates some interesting clinical queries beyond the utility of the extensive preoperative workup–the serendipitous transabdominal US would have been reason enough for surgical resection at our institution. I have never understood the rationale for nasogallbladder drainage in patients with acute cholecystitis, much less for comparatively “chronic” cancer, although this seems to be all the rage among the academic biliary endoscopists in Japan. And I doubt it is as popular among the unfortunate patients who are its beneficiaries. The efforts to preoperatively image and characterize this rare tumor, with a dizzying array of redundant and, in my opinion, superfluous but slick imagery, would, however, do the NSA (http://en.wikipedia.org/wiki/National_Security_Agency) proud. The SpyGlass platform has a cultlike following in the United States that even Snowden would covet. The option to perform a Spybiopsy (not that a biopsy would change management here) can be limited by poor visualization (see Video) compared with conventional peroral “baby scope” cholangioscopy. And stay tuned for improvements in the coming iteration. That said, we are indebted to our authors for this cinematic submission and look forward to the sequel. David Robbins, MD, MSc Assistant Editor for Focal Points
Pancreatic vascular tumor or AVM? Utility of EUS in the diagnosis of hereditary hemorrhagic telangiectasia A 57-year-old man was admitted for abdominal pain. CT showed acute cholecystitis and an incidental lesion in the pancreas. The cholecystitis was managed conservatively with antibiotics and resolved. A second CT scan with thin cuts revealed multiple pancreatic enhancing lesions (0.31 cm) suspicious for neuroendocrine lesions or vascular malformations (A). EUS was used to examine the pancreas with a plan to perform FNA. Anechoic lesions with positive Doppler flow were seen in the pancreas, suggestive of vascular malformations (B), and an uncinate lesion was supplied by the superior mesenteric artery (C). A gastric vascular malformation was incidentally seen (D). The patient subsequently provided a history of recurrent oral bleeding and epistaxis. Clinical examination revealed lingular and digital telangiectases. His father also had episodes of epistaxis. The patient was given a diagnosis of hereditary
hemorrhagic telangiectasia (HHT), and the pancreatic lesions were managed conservatively. EUS is useful in characterizing enhancing pancreatic lesions, and HHT should be considered when one is identified. DISCLOSURE All authors disclosed no financial relationships relevant to this publication. Eric Wee, MBBS, MRCP, MMed, Sushmitha Pearl Fernandes, MBBS, Gastroenterology, Department of General Medicine, Khoo Teck Puat Hospital, Singapore http://dx.doi.org/10.1016/j.gie.2013.10.046
Commentary The gray-haired GIE readership among us may shriek when hallowed clinical principles (such as taking a history before ordering a diagnostic test) are replaced by a “new school” strategy of upfront high-tech imagery and (maybe) asking questions later. At first blush, this Focal Point is another cool twist on the epidemic of incidentalomas that we face as the www.giejournal.org
Volume 79, No. 3 : 2014 GASTROINTESTINAL ENDOSCOPY 517
At the Focal Point
threshold to obtain high-resolution, cross-sectional imaging continues to drop lower than a limbo bar at a GI fellowship reunion party (the world record for lowest limbo dance is held by an 18-year-old Trinidadian woman who successfully traversed one only 8.5 inches off the ground). Also known as Osler-Weber-Rendu syndrome, hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant, board question favorite characterized by telangiectasia of the skin and mucous membranes and recurrent upper GI bleeding. If anyone asks, it is not associated with cholecystitis (certainly not the kind that is managed nonoperatively!). Genetic mutations in the endoglin and ALK1 genes, which code for proteins involved in normal blood vessel development and integrity, are responsible for the clinical hallmarks of recurrent childhood epistaxis and, later in life, melena. The lack of an elastic lamina normally responsible for vessel contractility leads to dilation of its end terminus and may explain the proclivity of these lesions to bleed, sometimes massively. The Curaçao criteria (named after an idyllic island in the southern
518 GASTROINTESTINAL ENDOSCOPY Volume 79, No. 3 : 2014
www.giejournal.org
At the Focal Point
Caribbean Sea, off the Venezuelan coast, that happens to be ideally situated for coming up with diagnostic criteria) include spontaneous, recurrent epistaxis; characteristic oral, nasal, or digital telangiectases; visceral vascular malformation lesions; and a similarly affected first-degree relative. Pancreatic involvement is common and asymptomatic in adult patients and is optimally imaged during arterial phase multi-detector CT. Telangiectases appear as highly enhancing round or irregular lesions; the differential should include pancreatic neuroendocrine tumors or hypervascular metastases (such as from hypernephroma). EUS with Doppler interrogation alone is ideally situated to distinguish among these possibilities (only a vessel is anechoic and Doppler positive) and EUS with FNA can be used to safely sample lesions (even vascular ones) with any degree of echogenicity. Turning back to our case, whether taking that history would have obviated the need for EUS is speculative but less fun than being reminded of one of my favorite self-deprecating Oslerisms: “Common sense in matters medical is rare, and is usually in inverse ratio to the degree of education.” That surely takes the pressure off all of us. David Robbins, MD, MSc Assistant Editor for Focal Points
Retrieval of a large foreign body from the ileum with double-balloon enteroscopy (with videos)
A 31-year-old, institutionalized man with a psychiatric disorder presented after he swallowed a plastic fork. Initial CT demonstrated the fork in the second portion of the duodenum, with the tines oriented distally. The patient was followed expectantly. A subsequent CT scan demonstrated the fork, now in the distal small bowel, still with tines directed distally (A). Thickening of the small-bowel wall with adjacent stranding was identified, without evidence of free air or fluid collection. A lower double-balloon enteroscopy was performed. The fork was located 30 cm from the ileocecal valve. One
of the outside prongs appeared embedded in the bowel wall. The fork was pushed back and out of the wall by use of a rat-tooth forceps. A large snare was threaded over the handle of the fork and brought back over the tines. The snare was closed, bunching the tines together (B). The fork was slowly withdrawn to the colon and removed (C) (Video 1; available online at www. giejournal.org). No significant trauma was seen. The patient was treated with intravenous antibiotics and admitted for 24-hour observation. Repeat abdominal plain films revealed no evidence of an adverse event.
www.giejournal.org
Volume 79, No. 3 : 2014 GASTROINTESTINAL ENDOSCOPY 519