Pôle d'hépato-digestive, Hôpitaux Universitaires de Strasbourg, Université de Strasbourg, Faculté de Médecine, IRCAD/EITS, Institut Hospitalo-Universitaire (IHU) Mix Surg, Strasbourg, France.
Ann Surg Oncol. 2012 Nov;19(12):3753-4. doi: 10.1245/s10434-012-2502-4. Epub 2012 Jul 26.
Duodenal duplication cysts constitute a rare congenital anomaly of the gastrointestinal tract. A recent meta-analysis of the literature between 1999 and 2009 reported a total of 47 cases of duodenal duplication cysts.1 These abnormalities are mostly diagnosed in infancy and childhood. In rare cases, they can remain asymptomatic until adulthood, and 38 % of patients are diagnosed after age 20 years.1 (,) 2 Duodenal duplication cysts are generally benign lesions; nevertheless, three cases of malignant tumours arising inside have been reported.3 (-) 5
In this multimedia article, we illustrated the case of an 18 year-old female patient presenting with recurrent episodes of mild pancreatitis. MRI revealed a cystic structure measuring 2.5 cm in diameter located in the duodenal wall next to the papilla of Vater. Endoscopic ultrasound showed a cystic lesion cephalad to the papilla, protruding into the duodenal lumen. Endoscopic retrograde cholangiopancreatography was not feasible due to the dislocation of the papilla, whose macroscopic aspect was normal. To further elucidate the anatomical relations, 3D reconstruction of the MRI images was performed. There was neither dilatation of the biliary tract nor a visible communication between the common bile duct and the cystic structure. The pancreatic duct also was at distance. Those findings were suggestive of a duodenal duplication. Nevertheless, the differential diagnosis6 of a choledochocele (Todani III) could not be formally excluded. Indication for surgical resection was symptomatic disease in a context of potential malignancy.
By right subcostal incision (video), surgical exploration revealed a soft tissue mass palpable at the second portion of the duodenum. Following duodenotomy, the mucosa was incised cephalad to the papilla of Vater, which could previously be localized by methylene blue injection by a catheter inserted into the cystic duct. The cystic structure was dissected and no communication between the cyst and the biliary tract was individualized. The final diagnosis was made by histological examination showing duodenal duplication. There was neither heterotopic gastric mucosa nor excreto-biliary epithelial layer. There were no signs of malignancy. The postoperative course was marked by hematemesis externalised by the nasogastric tube. We reintervened at postoperative day 2 to ensure hemostasis. A clot was removed from the area of duodenal mucosa without any visible active bleeding. Further recovery was uneventful; the patient was discharged at postoperative day 10 and is actually asymptomatic.
The ideal treatment of duodenal duplication cysts is complete surgical resection.7 Due to proximity to the bilio-pancreatic duct, total resection sometimes requires pancreaticoduodenectomy. This major surgical procedure entails the disadvantages of high morbidity and mortality with poor quality of life. In our opinion, this procedure should remain an ultimate option. Less invasive approaches have been proposed, including partial resection or internal derivation.7 Marsupialization is a surgical approach that has been accomplished even endoscopically.1 Nevertheless, these techniques do not provide total resection and leave the risk of degenerescence. As cases of malignancy are reported, we decided to realize a complete surgical excision of the lesion. Three-dimensional reconstruction of the biliary anatomy is an innovative procedure, which allowed us to show the absence of any communication between the cyst and either the common bile duct or the pancreatic duct.8 So, the surgical approach could be specified preoperatively ensuring the integrity of the common bile duct. Duplication cysts could be connected to the pancreaticobiliary ducts in about 29 %.1 Subsequent realization of a total surgical excision combined the advantages of complete resection with minimal invasiveness.
For relieving symptoms and preventing further complications, such as pancreatitis or malignant transformation, surgical resection of duodenal duplication cysts is indicated. In cases of difficulties to individualize the neighboring anatomical structures preoperatively, 3D reconstruction is a helpful approach to determine the surgical strategy. Enucleation allows a total excision while minimizing the adverse effects and therefore it is our treatment of choice for duodenal duplication cysts without communication.
十二指肠重复囊肿是一种罕见的胃肠道先天性异常。最近一项对 1999 年至 2009 年文献的荟萃分析报告了总共 47 例十二指肠重复囊肿病例。这些异常大多在婴儿和儿童期诊断出来。在极少数情况下，它们可能直到成年无症状，38%的患者在 20 岁后诊断。十二指肠重复囊肿一般为良性病变，但有 3 例报告为内部恶性肿瘤。
在这个多媒体文章中，我们描述了一名 18 岁女性患者反复发作轻度胰腺炎的病例。MRI 显示直径 2.5 厘米的囊性结构位于靠近 Vater 乳头的十二指肠壁上。内镜超声显示乳头上方的囊性病变向十二指肠腔内突出。由于乳头脱位，无法进行内镜逆行胰胆管造影。为了进一步阐明解剖关系，对 MRI 图像进行了 3D 重建。胆道无扩张，也没有可见的胆总管与囊性结构之间的沟通。胰管也相距较远。这些发现提示为十二指肠重复囊肿。然而，仍不能正式排除胆总管囊肿（Todani III 型）的鉴别诊断。手术切除的指征是存在潜在恶性病变的症状性疾病。
通过右肋缘下切口（视频），手术探查发现第二部分十二指肠可触及软组织肿块。十二指肠切开后，在 Vater 乳头上方切开黏膜，通过插入到囊性胆管的导管向囊性导管内注射亚甲蓝可定位乳头。分离囊性结构，未发现囊性与胆道之间的沟通。组织学检查诊断为十二指肠重复囊肿。无异位胃黏膜或外分泌胆管上皮层。无恶性肿瘤迹象。术后出现经鼻胃管排出的呕血。术后第 2 天我们再次干预以确保止血。从没有明显活动性出血的十二指肠黏膜区域清除了一个血块。进一步恢复顺利；患者术后第 10 天出院，目前无症状。
十二指肠重复囊肿的理想治疗方法是完全手术切除。由于靠近胆胰管，完全切除有时需要胰十二指肠切除术。这种主要的手术方法具有高发病率和死亡率以及较差的生活质量的缺点。在我们看来，这种方法应该仍然是最终的选择。已经提出了一些微创方法，包括部分切除或内部引流。囊肿造口术是一种甚至可以通过内镜完成的手术方法。然而，这些技术不能提供完全切除，并留下退行性变的风险。由于有恶性肿瘤的报道，我们决定对病变进行完全手术切除。胆道的三维重建是一种创新的方法，使我们能够显示囊性结构与胆总管或胰管之间没有任何沟通。因此，可以在术前确定手术方法，确保胆总管的完整性。囊肿与胰胆管相连的比例约为 29%。随后进行的完全手术切除结合了完全切除的优点和微创的优势。
为了缓解症状并预防胰腺炎或恶性转化等进一步并发症，应考虑手术切除十二指肠重复囊肿。在术前难以确定相邻解剖结构的情况下，3D 重建是确定手术策略的一种有用方法。囊肿剜除术可以进行完全切除，同时最大限度地减少不良反应，因此对于没有沟通的十二指肠重复囊肿，我们选择剜除术作为首选治疗方法。
