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伴有胰管相通的胰腺旁肠重复畸形:儿童胰腺炎和复发性腹痛的一个病因

Juxtapancreatic intestinal duplications with pancreatic ductal communication: a cause of pancreatitis and recurrent abdominal pain in childhood.

作者信息

Black P R, Welch K J, Eraklis A J

出版信息

J Pediatr Surg. 1986 Mar;21(3):257-61. doi: 10.1016/s0022-3468(86)80849-1.

Abstract

Pancreatic duplications with ductal communications should be included in the differential diagnosis of any child presenting with recurrent abdominal pain of unknown etiology and should be considered as a possible cause of pancreatitis in childhood. Such duplications most likely arise from nonregressing diverticula of the pancreatic bud during embryologic development. Their clinical presentation is unique from other duplications because of their anatomic association with the pancreatic duct. Pain and weight loss are the major presenting complaints, although many patients have nausea and vomiting. Serum chemistries, in particular the serum amylase, are usually normal and are of little help in the differential diagnosis. Radiographic evaluation has not been particularly helpful in the past. ERCP, ultrasonic examination, and CT scan show great promise, however. Operative intervention should be tailored for the individual patient. The operation performed will depend upon operative findings. Intraoperative pancreatograms or cystograms are very helpful in differentiating these cysts from others at the time of operation. Pathologically, most of the duplications have a thickened muscular coat that usually has some evidence of inflammation. They are usually lined with gastric mucosa. Except in the most severe cases, the pancreas is histologically normal, suggesting that most of the pain experienced by these patients is secondary to inflammation within the duplication. The inflammatory response may completely destroy the mucosal lining and cause fibrosis within the muscular coat of the duplication. In those instances, these lesions cannot be differentiated from pancreatic pseudocyst. This may account for some of the "idiopathic" pseudocysts reported in the literature.

摘要

伴有导管相通的胰腺重复畸形应纳入任何病因不明的反复腹痛儿童的鉴别诊断中,并应被视为儿童胰腺炎的可能病因。这种重复畸形很可能源于胚胎发育过程中胰腺芽的憩室未退化。由于其与胰管的解剖学关联,它们的临床表现与其他重复畸形不同。疼痛和体重减轻是主要的就诊主诉,尽管许多患者伴有恶心和呕吐。血清化学指标,尤其是血清淀粉酶,通常正常,对鉴别诊断帮助不大。过去,影像学评估也没有特别大的帮助。然而,内镜逆行胰胆管造影(ERCP)、超声检查和CT扫描显示出很大的前景。手术干预应根据个体患者进行调整。所实施的手术将取决于手术所见。术中胰管造影或囊肿造影在手术时将这些囊肿与其他囊肿区分开来非常有帮助。病理上,大多数重复畸形有增厚的肌层,通常有一些炎症迹象。它们通常内衬胃黏膜。除了最严重的病例外,胰腺组织学上正常,这表明这些患者经历的大多数疼痛继发于重复畸形内的炎症。炎症反应可能会完全破坏黏膜内衬,并导致重复畸形肌层内的纤维化。在这些情况下,这些病变无法与胰腺假性囊肿区分开来。这可能解释了文献中报道的一些“特发性”假性囊肿。

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