Malka D, Hammel P, Vilgrain V, Fléjou J F, Belghiti J, Bernades P
Medical-Surgical Federation of Hepatogastroenterology, Hôpital Beaujon, Clichy, France.
Gut. 1998 Jan;42(1):131-4. doi: 10.1136/gut.42.1.131.
Autosomal dominant polycystic kidney disease, the most frequent inherited polycystic disease, is a systemic disorder characterised by the development of numerous and bilateral kidney cysts leading to chronic renal failure. Extrarenal cysts are located mainly in the liver but also in various organs including the pancreas. To our knowledge, complications of pancreatic cysts in this disease have never been reported.
The first case of painful chronic obstructive pancreatitis due to a true pancreatic cyst in a patient with autosomal dominant polycystic kidney disease is reported. Abdominal transparietal and endoscopic ultrasonography, computed tomography, and endoscopic retrograde cholangiopancreatography showed a cystic lesion in the body of the pancreas associated with upstream dilatation of the main pancreatic duct. Intraoperative ultrasonography before and after cyst fluid aspiration, and pancreatography and pathological examination of the resected distal pancreas confirmed that both main pancreatic duct enlargement and chronic pancreatitis were caused by a benign cyst.
Chronic obstructive pancreatitis should be added to the extrarenal complications of autosomal dominant polycystic kidney disease.
常染色体显性遗传性多囊肾病是最常见的遗传性多囊疾病，是一种全身性疾病，其特征是双侧出现大量肾囊肿，最终导致慢性肾衰竭。肾外囊肿主要位于肝脏，但也可见于包括胰腺在内的各种器官。据我们所知，尚未有关于该疾病胰腺囊肿并发症的报道。
本文报道了首例常染色体显性遗传性多囊肾病患者因真性胰腺囊肿导致疼痛性慢性阻塞性胰腺炎的病例。经腹部经壁超声、内镜超声、计算机断层扫描以及内镜逆行胰胆管造影检查发现，胰腺体部有一囊性病变，伴有主胰管上游扩张。在囊肿抽液前后进行的术中超声检查，以及对切除的胰腺远端进行的胰管造影和病理检查证实，主胰管扩张和慢性胰腺炎均由良性囊肿所致。
慢性阻塞性胰腺炎应被纳入常染色体显性遗传性多囊肾病的肾外并发症。
