Abstract
Background
Pseudoachalasia is often caused by malignant involvement at the gastroesophageal junction (GEJ) leading to dysphagia.
Aims
We describe a case of type 3 achalasia presenting in a woman with metastatic pancreatic cancer with no direct involvement at the GEJ, fundus or cardia.
Methods
A case report and literature review were performed.
Results
A 53-year-old woman presented with a 2-month-history of progressive abdominal pain, nausea and vomiting with a 30-pound weight loss. She had a remote history of breast cancer in remission after surgery and chemoradiation. On presentation, she denied chest pain, reflux, dysphagia or odynophagia. Abdominal exam revealed focal epigastric tenderness and jaundice. Abdominal CT showed a 6.7 x 5.8 cm conglomerate mass involving the hepatic hilum, pancreatic head, duodenum, common bile duct, and portal vein with gastric outlet and biliary obstruction. This mass was confirmed to be a pancreatic adenocarcinoma on pathology. She then underwent nasogastric tube decompression. Initial esophagogastroduodenoscopy (EGD) confirmed a stenotic area at the distal duodenal cap. A duodenal stent and common bile duct stent were placed during a second EGD. The esophagus and GEJ were unremarkable on both endoscopic exams. She was started on chemotherapy with gemcitabine and abraxane. Two weeks after her stent placement, she rapidly developed severe retrosternal squeezing discomfort and choking occurring with swallowing. CT chest and abdomen were negative for any intrathoracic and diaphragmatic involvement with stability of the mass. A barium swallow study demonstrated tertiary contractions in the thoracic esophagus with marshmallow hold-up in the distal esophagus. She then underwent a high-resolution esophageal manometry study that demonstrated an elevation of integrated residual pressure (IRP) of the lower esophageal sphincter (LES) and absence of peristalsis, with the distal 2/3rds of the esophagus showing a simultaneous and prolonged pressure front consistent with type 3 achalasia, Chicago classification v3.0 [Figure 1]. All contractions had a distal contractile integral (DCI) of >8000 mmHg-cm-s. She experienced significant symptom improvement with pinaverium bromide, a gut-specific calcium channel antagonist.
A review of the literature revealed that there have been 4 English-language cases published on pseudoachalasia associated with pancreatic cancer, with all cases describing direct infiltration of pancreatic cancer in the GEJ, cardia or fundus with manometric features of type I achalasia.
Conclusions
We report the first case of type 3 achalasia with no evidence of direct malignant infiltration at the GEJ on radiographic and endoscopic evaluations. Possible mechanisms to explain this phenomenon include paraneoplastic antibody-mediated impairment of enteric neurons that decrease nitric oxide availability, or microscopic disease involvement at the GEJ.
Funding Agencies
None
Adenomyoma of common bile duct arising in a type I choledochal cyst
