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[Biliary obstruction in von Recklinghausen disease]
G Hernández Alvarez1, R Ayala Landa, G Hernández Adrian
1Servicio de Gastroenterología y Cirugía del Hospital Carlos J. Bello de la Cruz Roja Venezolana, Caracas.
G.E.N
|January 1, 1991
Summary
Enchondromatosis with hereditary visceral neurofibromas (EVR) is a rare condition causing tumors along nerves. These can obstruct the bile duct, mimicking pancreatitis and requiring surgical intervention.
Area of Science:
- Genetics
- Gastroenterology
- Pathology
Background:
- Hereditary Visceral Neurofibromas (EVR) is an uncommon autosomal dominant disorder.
- It characteristically presents with neurofibromas along spinal, cranial, and autonomic nerves.
Observation:
- Neurofibromas can occur in the gastrointestinal tract, primarily the jejunum, stomach, and ileum.
- Rarely, duodenal neurofibromas may ulcerate, causing bleeding and severe pain, mimicking acute pancreatitis.
Findings:
- Neurofibromas originating from the minor omentum's nerve network can affect the extrahepatic biliary system.
- Enlarged neurofibromas can lead to biliary obstruction, necessitating surgical treatment.
Implications:
- Accurate diagnosis requires excluding other causes of biliary obstruction like gallstones or neoplasms.
- Diagnostic procedures include ultrasonography, ERCP, and CT scans.