Khuri Safi, Kluger Yoram
Department of General Surgery, HepatoPancreatoBiliary Surgical Unit, Rambam Medical Care Center, Haifa, Israel.
J Med Cases. 2022 Oct;13(10):509-512. doi: 10.14740/jmc4012. Epub 2022 Oct 31.
Anomalies of the pancreas organ, especially congenital anomalies, are very uncommon, and can involve the pancreatic ductal as well as the pancreatic extra-ductal systems. While these anomalies usually present as an incidental radiological finding during adulthood, sometimes, they can present as recurrent upper abdominal pain as a presentation of recurrent episodes of acute idiopathic pancreatitis. Thus, these anomalies should be regarded in the differential diagnosis list, as a cause, for recurrent idiopathic pancreatitis, especially in the adult age group of patients. Multiple different variations, mainly in duct course and configuration of the pancreatic ductal system have been reported. In addition, duplication anomalies and cystic dilatation of the pancreatic duct are well known anomalies as well. A combined anomaly of the aforementioned anomalies is even rarer. Herein, we present the case of a male patient, 47 years old, with recurrent admissions to different hospitals due to upper abdominal pain and episodes of severe idiopathic acute pancreatitis. Imaging tests, mainly abdomino-pelvic computed tomography (CT) scan and magnetic resonance imaging (MRI)/magnetic resonance cholangiopancreatography (MRCP) showed a dominant duct of Santorini without divisum along with cystic dilation of the proximal portion of the Santorini duct. Being a very rare pancreatic duct anomaly, the patient was treated by a multidisciplinary team (MDT) of physicians, including pancreas surgeons, gastroenterologists and radiologists. A surgical resection in the form of total pancreatectomy with Roux-en-Y gastrointestinal reconstruction was contemplated. Perioperative and postoperative periods were uneventful. The previously mentioned anomaly is unknown in the English literature and is introduced as new anomaly known as "Kluri".
胰腺器官的异常,尤其是先天性异常,非常罕见,可累及胰腺导管系统和胰腺导管外系统。虽然这些异常通常在成年期作为偶然的影像学发现出现,但有时也可能表现为反复上腹部疼痛,作为复发性急性特发性胰腺炎发作的表现。因此,在鉴别诊断清单中应将这些异常视为复发性特发性胰腺炎的一个病因,尤其是在成年患者群体中。已经报道了多种不同的变异,主要是胰腺导管系统的导管走行和形态。此外,胰腺导管的重复异常和囊性扩张也是众所周知的异常。上述异常的合并异常更为罕见。在此,我们报告一例47岁男性患者,因上腹部疼痛和严重特发性急性胰腺炎发作多次入住不同医院。影像学检查,主要是腹盆腔计算机断层扫描(CT)和磁共振成像(MRI)/磁共振胰胆管造影(MRCP)显示一条无分支的桑托里尼主胰管,同时桑托里尼导管近端囊性扩张。作为一种非常罕见的胰腺导管异常,该患者由包括胰腺外科医生、胃肠病学家和放射科医生在内的多学科团队(MDT)进行治疗。考虑采用全胰切除术加Roux-en-Y胃肠道重建的手术切除方式。围手术期和术后过程顺利。上述异常在英文文献中未见报道,现作为一种新发现的异常引入,称为“Kluri”。