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Ann Thorac Surg 2006;81:1138-1140
© 2006 The Society of Thoracic Surgeons

Case report

Neurofibromatosis of the Esophagus

Giuseppe S. Sica, MD, PhD a , * , Vejay Sujendran, MD a , Brian Warren, MD b , Nicholas D. Maynard, MD, MS a

a Department of Surgery, John Radcliffe Hospital, Headington, Oxford, United Kingdom
b Department of Pathology, John Radcliffe Hospital, Headington, Oxford, United Kingdom

Accepted for publication February 1, 2005.

* Address correspondence to Dr Sica, Department of Surgery, Tor Vergata School of Medicine, University of Tor Vergata, Viale Oxford 81, Rome, 00133 Italy (Email: sica.giuseppe{at}fastwebnet.it).

There have been previous reports suggesting an association between von Recklinghausen's neurofibromatosis and esophageal dysmotility. We report the first case of true Recklinghausen's neurofibromatosis of the esophagus leading to end-stage pseudoachalasia. The diagnosis and management of this condition is discussed together with the pathogenesis and pathology of this rare entity.






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