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From question on page 1282
The diagnosis was pseudoachalasia due to neurofibromatosis of the oesophagus. Computerised tomograpy showed circumferentially thickened and dilated oesophagus. Neurofibromas were visible infiltrating the muscularis propria at endoscopic ultrasonography.
A diagnostic laparoscopy was performed and showed multiple nodules around the gastro-oesophageal junction (GOJ) and also throughout the small bowel mesentery. Histology of the nodules confirmed neurofibromatosis.
Subsequently, the patient underwent pneumatic dilatations of up to 40 mm but his dysphagia recurred rapidly. Because of refractory symptoms and after discussion with the patient, oesophagectomy through a left thoracoabdominal incision and a left cervical incision was performed. Reconstruction was via a gastric tube anastomosed to the cervical oesophagus. The patient has made an uneventful recovery with no significant complications over the past 12 months of follow up.
The specimen consisted of a dilated thick walled oesophagus with an obvious narrowing just above the GOJ where multiple neurofibromas were present. Microscopic examination confirmed plexiform neurofibromatosis.
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