Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Baylor College of Medicine.
J Pediatr Gastroenterol Nutr. 2019 Dec;69(6):704-709. doi: 10.1097/MPG.0000000000002515.
Functional pancreatic sphincter dysfunction (FPSD), previously characterized as pancreatic sphincter of Oddi dysfunction, is a rarely described cause of pancreatitis. Most studies are reported in adults with alcohol or smoking as confounders, which are uncommon risk factors in children. There are no tests to reliably diagnose FPSD in pediatrics and it is unclear to what degree this disorder contributes to childhood pancreatitis.
We conducted a literature review of the diagnostic and treatment approaches for FPSD, including unique challenges applicable to pediatrics. We identified best practices in the management of children with suspected FPSD and formed a consensus expert opinion.
In children with acute recurrent pancreatitis (ARP) or chronic pancreatitis (CP), we recommend that other risk factors, specifically obstructive factors, be ruled out before considering FPSD as the underlying etiology. In children with ARP/CP, FPSD may be the etiology behind a persistently dilated pancreatic duct in the absence of an alternative obstructive process. Endoscopic retrograde cholangiopancreatography with sphincterotomy should be considered in a select group of children with ARP/CP when FPSD is highly suspected and other etiologies have been effectively ruled out. The family and patient should be thoroughly counseled regarding the risks and advantages of endoscopic intervention. Endoscopic retrograde cholangiopancreatography for suspected FPSD should be considered with caution in children with ARP/CP when pancreatic ductal dilatation is absent.
Our consensus expert guidelines provide a uniform approach to the diagnosis and treatment of pediatric FPSD. Further research is necessary to determine the full contribution of FPSD to pediatric pancreatitis.
功能性胰腺括约肌功能障碍（FPSD），以前被描述为Oddi 胰管括约肌功能障碍，是胰腺炎的一个罕见病因。大多数研究报告都涉及到成年人，其混杂因素是酒精或吸烟，这在儿童中是不常见的危险因素。目前还没有可靠的测试方法来诊断儿科中的 FPSD，也不清楚该疾病在多大程度上导致了儿童胰腺炎。
我们对 FPSD 的诊断和治疗方法进行了文献回顾，包括适用于儿科的独特挑战。我们确定了疑似 FPSD 患儿管理的最佳实践，并形成了共识专家意见。
在患有急性复发性胰腺炎（ARP）或慢性胰腺炎（CP）的儿童中，我们建议在考虑 FPSD 为潜在病因之前，排除其他风险因素，特别是梗阻性因素。在患有 ARP/CP 的儿童中，当没有其他梗阻性病变时，FPSD 可能是胰腺导管持续扩张的病因。当高度怀疑 FPSD 且已有效排除其他病因时，应考虑在一组选择的 ARP/CP 儿童中进行选择性内镜逆行胰胆管造影术（ERCP）加括约肌切开术。应向家属和患者详细说明内镜干预的风险和优势。当不存在胰管扩张时，对于患有 ARP/CP 的儿童，应谨慎考虑疑似 FPSD 的 ERCP。
我们的共识专家指南为儿科 FPSD 的诊断和治疗提供了统一的方法。需要进一步研究以确定 FPSD 对儿童胰腺炎的全部贡献。
