A 37-year-old white female presented with a three-month history of dysphagia that began suddenly with solids and progressed to include also liquids. Associated with the dysphagia were retrosternal chest pain, regurgitation of food, and a 30-pound weight loss. During this period of time, she had multiple admissions for dehydration due to her inability to swallow and had diagnostic tests performed, including a chest radiograph, upper GI series, endoscopy, and manometry.
The patient's past medical history was significant for cervical cancer Stage 1B four years prior to this presentation. Pathologic analysis at that time revealed an invasive squamous cell carcinoma with a depth of invasion to one-half the thickness of the cervical wall. The left and right parametrial nodes were positive for metastatic disease; however, the vagina was not involved. The patient underwent a radical hysterectomy and eight cycles of radiation therapy. Annual surveillance, including an evaluation within six months of this presentation, included a PAP smear, chest radiograph, pelvic MRI, and bone scan, which revealed no evidence of recurrent disease.
The patient's physical exam was remarkable for a thin white female in no acute distress. Her abdomen was scaphoid with no organomegally or palpable masses. Her rectal exam was Guaiac negative. No adenopathy was noted. A barium esophagram revealed the classic “bird-beak” deformity, a dilated esophageal body with distal tapering to a smooth stricture at the level of the lower esophageal sphincter (). No mucosal irregularities or mass effects were noted. At endoscopy, the body of the esophagus was mildly dilated and particulate matter was present within its lumen. No mucosal irregularities were present (). During the initial endoscopic procedures, the endoscope was reportedly passed into the stomach without difficulty. No abnormalities in the stomach or duodenum were noted. Manometry was performed using the station pull-through technique (). A lower esophageal (LES) pressure of 45 mm Hg was obtained with no relaxation of the LES during swallowing. No effective peristalsis was seen in the body of the esophagus.
The patient's barium swallow, revealing a mildly dilated esophagus with distal tapering to a smooth stricture at the level of the lower esophageal sphincter.
The patient's lower esophageal sphincter, revealing normal mucosa and no evidence of the tumor
Preoperative Esophageal Manometry
A treatment regimen consisting of injection of the LES with 100 U of botulinum toxin was initiated. The patient described minimal improvement in the symptoms lasting about two weeks. Additionally, the patient was started on oral nifedipine and sublingual nitroglycerin therapy with no improvement. As the patient's symptoms progressed, treatment progressed to esophageal dilation. Endoscopy was repeated and difficulty was reported in passing the endoscope through the gastroesophageal junction. Wire-guided Savary dilation to 54 French was performed under endoscopic guidance. Although initial relief was noted, the patient's symptoms recurred within three days of the endoscopy.
The patient was referred for surgical intervention, and she was considered to be a candidate for a laparoscopic Heller myotomy. At surgery, the right crus of the diaphragm was noted to be calcified, with the esophagus adherent to it. The esophagus was bluntly mobilized off of the right crus, revealing the tumor invading both the esophagus and the diaphragm (). The right vagus nerve was encased by the tumor () and extension of the tumor into the aorta was suspected. Multiple biopsies of the tumor were taken, and because the frozen section analysis was positive for malignancy, the planned procedure was aborted.
(A) Intraoperative picture showing the tumor invading the esophagus and right crus of the diaphragm. (B) A line drawing representation of the intraoperative findings.
A close up of the patient's esophagus, revealing encasement of the vagus nerve by the tumor.
The pathology report was that of a poorly differentiated squamous cell carcinoma (). Immunohisto-chemical stains for cytokeratin (CAM 5.2), chromogranin, and synaptophysin were positive for keratin only. These findings are consistent with metastatic cervical carcinoma. A postoperative CAT scan revealed periaortic adenopathy, invasion of the aorta, and invasion of the pericardium ().
Biopsy of the patient's tumor, revealing a squamous cell carcinoma.
The patient's CT of the abdomen obtained postoperatively revealing (A) periaortic adenopathy, (B) infiltration of the aorta by the tumor, and (C) periaortic mass with invasion of the aorta and pericardium by the tumor.