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Sideropenic Dysphagia
Electronic Clinical Challenges and Images in GI David A. Katzka and David L. Jaffe, Section Editors
Sideropenic Dysphagia
See related article, Lenz P et al, on page e72 in CGH. Question: A 43-year-old woman with a history of recurrent median cervical cyst, treated operatively 2 years earlier by thyroglossal duct excision with partial hyoid resection, presented to our otolaryngology clinic due to progressive cervical dysphagia for solid foods and a weight loss of 10 kg. Videofluoroscopy revealed a postcricoid web arising from the anterior wall and passing over into a tubular stricture (Figure A). On admission to our department, the patient complained of fatigue, burning sensation in the mouth, and loss of taste. She reported increased menstrual blood loss and uterine fibroids, but there was no history of gastrointestinal bleeding. Physical examination revealed a tachycardic patient with pale skin, cheilitis angularis (Figure B), mild anterior atrophic glossitis (Figure C) as well as dystrophic nail changes (Figure D). Laboratory studies showed profound microcytary hypochromic anemia (hemoglobin, 5.9 g/dL; mean corpuscular volume, 52 fl; mean corpuscular hemoglobin, 13 pg) and depleted iron stores (serum iron, 13 g/dL [normal, 37–145]; transferrin saturation, 3% [normal, 16 – 45]; ferritin, ⬍3 g/L [normal, 15–150]). Celiac serology (anti-gliadin; anti-transglutaminase IgA) was negative, and duodenal biopsies were normal. After difficult intubation despite conscious sedation, upper endoscopy revealed a proximal stricture (7– 8 mm in
diameter and 2 cm in length), but no web nor other mucosal irregularities. Otherwise, upper endoscopy and colonoscopy were unremarkable. Interestingly, the patient experienced marked symptomatic improvement after this “diagnostic” intervention. Iron replacement and adjunctive folic acid therapy were started, and she was scheduled for hysterectomy. What is the diagnosis? See the GASTROENTEROLOGY web site (www.gastrojournal. org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. VINCENT ZIMMER, MD Department of Medicine II Saarland University Hospital Homburg, Germany ARNO BUECKER, MD Department of Diagnostic and Interventional Radiology Saarland University Hospital Homburg, Germany FRANK LAMMERT, MD Department of Medicine II Saarland University Hospital Homburg, Germany Conflicts of interest The authors disclose no conflicts. © 2009 by the AGA Institute 0016-5085/09/$36.00 doi:10.1053/j.gastro.2009.03.051
GASTROENTEROLOGY 2009;137:e1– e2
e2
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI
GASTROENTEROLOGY Vol. 137, No. 6
Answer to the Clinical Challenges and Images in GI Question: Image 1: PlummerVinson Syndrome The patient illustrates the typical clinical features of Plummer–Vinson syndrome (PVS; or Paterson–Brown–Kelly syndrome), which is characterized by the triad of cervical dysphagia, iron-deficiency anemia, and postcricoid esophageal webs.1 Often encountered additional mucocutaneous changes encompass atrophic glossitis, cheilitis angularis, and dystrophic nail changes up to the classic koilonychie (spoon-shaped nails). These findings, however, are very common in iron-deficient states and are neither specific for PVS nor required for the diagnosis. Although its pathogenesis remains obscure, iron deficiency appears to be the single most important factor. Because PVS is virtually nonexistent in certain regions where iron deficiency is endemic, for example, Africa, it is reasonable to assume that other genetic and/or environmental factors may be critical. At the beginning of the last century, PVS used to be common particularly in Scandinavia, but it has become an altogether rare condition nowadays, maybe owing to improvements in nutrition. Middle-aged woman are at highest risk. Of interest, in recent years a comparably high proportion of PVS patients were found to have underlying celiac disease as specific cause of iron deficiency.1 Apart from classical postcricoid webs, functional strictures possibly due to iron deficiency-related alterations of esophageal motility may occur, as in our patient.2 Iron supplementation and mechanical dilatation are the therapeutic mainstay in PVS. Because dysphagia improved markedly in our patient after the endoscopic session, accidental web disruption has to be assumed. Because of her continuous clinical improvement on iron therapy, we abstained form further endoscopic interventions. The overall clinical relevance of PVS is in the differential diagnosis of dysphagia and the increased risk of squamous cell carcinoma in the esophagus.1,3 Although not evidence based, we recommend endoscopic surveillance at yearly intervals for patients presenting with PVS. References 1. Novacek G. Plummer-Vinson syndrome. Orphanet J Rare Dis 2006;15:36. 2. Uygur-Bayramicli O, Tuncer K, Dolapcioglu C. Plummer-Vinson syndrome presenting with an esophageal stricture. J Clin Gastroenterol 1999;29:291–292. 3. Wynder EL, Hultberg S, Jacobsson F, et al. Environmental factors in cancer of the upper alimentary tract. A Swedish study with special reference to Plummer-Vinson (Patterson-Kelly) syndrome. Cancer 1957;10:470 – 482. For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).
Sideropenic Dysphagia
See related article, Lenz P et al, on page e72 in CGH. Question: A 43-year-old woman with a history of recurrent median cervical cyst, treated operatively 2 years earlier by thyroglossal duct excision with partial hyoid resection, presented to our otolaryngology clinic due to progressive cervical dysphagia for solid foods and a weight loss of 10 kg. Videofluoroscopy revealed a postcricoid web arising from the anterior wall and passing over into a tubular stricture (Figure A). On admission to our department, the patient complained of fatigue, burning sensation in the mouth, and loss of taste. She reported increased menstrual blood loss and uterine fibroids, but there was no history of gastrointestinal bleeding. Physical examination revealed a tachycardic patient with pale skin, cheilitis angularis (Figure B), mild anterior atrophic glossitis (Figure C) as well as dystrophic nail changes (Figure D). Laboratory studies showed profound microcytary hypochromic anemia (hemoglobin, 5.9 g/dL; mean corpuscular volume, 52 fl; mean corpuscular hemoglobin, 13 pg) and depleted iron stores (serum iron, 13 g/dL [normal, 37–145]; transferrin saturation, 3% [normal, 16 – 45]; ferritin, ⬍3 g/L [normal, 15–150]). Celiac serology (anti-gliadin; anti-transglutaminase IgA) was negative, and duodenal biopsies were normal. After difficult intubation despite conscious sedation, upper endoscopy revealed a proximal stricture (7– 8 mm in
diameter and 2 cm in length), but no web nor other mucosal irregularities. Otherwise, upper endoscopy and colonoscopy were unremarkable. Interestingly, the patient experienced marked symptomatic improvement after this “diagnostic” intervention. Iron replacement and adjunctive folic acid therapy were started, and she was scheduled for hysterectomy. What is the diagnosis? See the GASTROENTEROLOGY web site (www.gastrojournal. org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. VINCENT ZIMMER, MD Department of Medicine II Saarland University Hospital Homburg, Germany ARNO BUECKER, MD Department of Diagnostic and Interventional Radiology Saarland University Hospital Homburg, Germany FRANK LAMMERT, MD Department of Medicine II Saarland University Hospital Homburg, Germany Conflicts of interest The authors disclose no conflicts. © 2009 by the AGA Institute 0016-5085/09/$36.00 doi:10.1053/j.gastro.2009.03.051
GASTROENTEROLOGY 2009;137:e1– e2
e2
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI
GASTROENTEROLOGY Vol. 137, No. 6
Answer to the Clinical Challenges and Images in GI Question: Image 1: PlummerVinson Syndrome The patient illustrates the typical clinical features of Plummer–Vinson syndrome (PVS; or Paterson–Brown–Kelly syndrome), which is characterized by the triad of cervical dysphagia, iron-deficiency anemia, and postcricoid esophageal webs.1 Often encountered additional mucocutaneous changes encompass atrophic glossitis, cheilitis angularis, and dystrophic nail changes up to the classic koilonychie (spoon-shaped nails). These findings, however, are very common in iron-deficient states and are neither specific for PVS nor required for the diagnosis. Although its pathogenesis remains obscure, iron deficiency appears to be the single most important factor. Because PVS is virtually nonexistent in certain regions where iron deficiency is endemic, for example, Africa, it is reasonable to assume that other genetic and/or environmental factors may be critical. At the beginning of the last century, PVS used to be common particularly in Scandinavia, but it has become an altogether rare condition nowadays, maybe owing to improvements in nutrition. Middle-aged woman are at highest risk. Of interest, in recent years a comparably high proportion of PVS patients were found to have underlying celiac disease as specific cause of iron deficiency.1 Apart from classical postcricoid webs, functional strictures possibly due to iron deficiency-related alterations of esophageal motility may occur, as in our patient.2 Iron supplementation and mechanical dilatation are the therapeutic mainstay in PVS. Because dysphagia improved markedly in our patient after the endoscopic session, accidental web disruption has to be assumed. Because of her continuous clinical improvement on iron therapy, we abstained form further endoscopic interventions. The overall clinical relevance of PVS is in the differential diagnosis of dysphagia and the increased risk of squamous cell carcinoma in the esophagus.1,3 Although not evidence based, we recommend endoscopic surveillance at yearly intervals for patients presenting with PVS. References 1. Novacek G. Plummer-Vinson syndrome. Orphanet J Rare Dis 2006;15:36. 2. Uygur-Bayramicli O, Tuncer K, Dolapcioglu C. Plummer-Vinson syndrome presenting with an esophageal stricture. J Clin Gastroenterol 1999;29:291–292. 3. Wynder EL, Hultberg S, Jacobsson F, et al. Environmental factors in cancer of the upper alimentary tract. A Swedish study with special reference to Plummer-Vinson (Patterson-Kelly) syndrome. Cancer 1957;10:470 – 482. For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).