Endoscopic Management of Pancreaticopleural Fistula in a Child with Hereditary Pancreatitis

Lee, Da Hye; Lee, Eun Joo; Kim, Ju Whi; Moon, Jin Soo; Kim, Yong-Tae; Ko, Jae Sung

doi:10.5223/pghn.2019.22.6.601

Cited by 8 publications

(9 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…These four PRSS1 mutations often cause autosomal dominant hereditary pancreatitis. However, judging from the reports of children with hereditary pancreatitis caused by mutations in the PRSS1 gene, R122H and R122C mutations are predominant [10,11,12], and reports of children with hereditary pancreatitis caused by R116C mutations are rare [13,14,15]. We found hereditary pancreatitis caused by R116C mutation in children with recurrent pancreatitis.…”

Section: Discussionmentioning

confidence: 69%

R116C mutation in PRSS1 gene causes hereditary pancreatitis and elevated creatine kinase in one child：a case report

Cheng¹,

Zhou²,

Dong³

2021

Preprint

View full text Add to dashboard Cite

Background Functionally acquired mutations in the PRSS1 gene can lead to autosomal dominant hereditary pancreatitis (Hereditary Pancreatitis, HP). The most frequently reported mutation sites are R122H, N29I, A16V, and R122C. R116C mutation was less frequently reported to be associated with HP. Moreover, there are few reports about association of hereditary pancreatitis with elevated creatine kinase in children. Case presentation: We reported a patient presented with repeated abdominal pain and recurrent acute pancreatitis accompanied by elevated creatine kinase. The genomic DNA of lymphocytes from peripheral blood was extracted for whole exon gene analysis. The patient had a heterozygous mutation in exon 3 c.346C > T, resulting in substitution of cysteine at position 116 with arginine (p.R116C). Her father had the same mutation in exon 3 c.346C > T. The diagnosis of hereditary pancreatitis due to R116C mutation in PRSS1 gene was confirmed. Conclusions The patient's hereditary pancreatitis is caused by the mutation of PRSS1 gene R116C, characterized by elevated creatine kinase in patient.

show abstract

Section: Discussionmentioning

confidence: 69%

R116C mutation in PRSS1 gene causes hereditary pancreatitis and elevated creatine kinase in one child：a case report

Cheng¹,

Zhou²,

Dong³

2021

Preprint

View full text Add to dashboard Cite

show abstract

“…The diagnosis of pancreatic fistula is suspected when pancreatic enzymes are elevated in the pleural fluid [1] , [2] , [3] , [4] . Yang et al.…”

Section: Discussionmentioning

confidence: 99%

“…The reported number of cases presenting with this complication is minimal, and no consensus regarding the best course of management for this condition exists. Reported treatments include conservative treatments, such as pleural drainage, and interventional treatments, such as endoscopic retrograde cholangiopancreatography (ERCP) sphincter stenting or surgery [ 3 , 4 ]. In this report, we present 2 cases of this rare entity and discuss aspects of diagnosis and treatment.…”

Section: Introductionmentioning

confidence: 99%

Pancreaticopleural fistula in children: Report of 2 cases

Tri

Thach

Duy

et al. 2022

Radiology Case Reports

View full text Add to dashboard Cite

Pancreaticopleural fistula is an extremely rare complication of pancreatic duct injury. The reported treatments include conservative approaches, such as pleural drainage, and interventional approaches, such as sphincter stenting via endoscopic retrograde cholangiopancreatography and surgery. However, no specific consensus treatment has been defined. We present 2 cases of pediatric patients with pancreaticopleural fistulas due to pancreatic trauma and pancreatitis that were successfully treated surgically. The most prominent symptom in both cases was dyspnea caused by pleural effusion. Thoracoabdominal computed tomography scans showed large pleural effusions and visible fistulas from the pancreatic duct to the thoracic cavity through the esophageal hiatus and aortic hiatus. Following unsuccessful conservative treatment using pleural drainage, the 2 patients underwent surgical fistulo-jejunostomy and cystojejunostomy. Both patients were stable and were discharged on postoperative days 10 and 12. Conservative treatment for pancreaticopleural fistula often fails, and a surgical approach, such as fistulo-jejunostomy and cystojejunostomy, can serve as an efficacious management strategy when conservative treatment fails.

show abstract

“…If the patient does not respond well after two or three weeks of treatment, or if symptoms progress, interventional therapies such as ERP and surgical management should be considered. Endoscopic retrograde pancreatography with stent placement is preferred in adults because it is less invasive, but only a few successful cases in children have been reported [ 7 , 11 , 24 ]. Decreasing the ductal pressure and sealing the leakage site are the main benefits of the ductal stent.…”

Section: Discussionmentioning

confidence: 99%

Pancreaticopleural fistula in a child with chronic pancreatitis harboring compound SPINK1 variants

et al. 2021

View full text Add to dashboard Cite

Background Pancreaticopleural fistula (PPF) is a rare complication of chronic pancreatitis (CP) that requires a high index of clinical suspicion in the patient who presents with a pleural effusion. Visualizing the fistula tract from the pancreatic duct to the pleural space by radiological imaging provides confirmation of this complication. Case presentation A 9-year-old boy who presented with massive right pleural effusion secondary to PPF, a complication of CP from a genetic mutation involving two mutations of SPINK1. We successfully managed the case with by endoscopic pancreatic duct stent placement after failure of conservative treatment approaches. Conclusions PPF is a rare but serious complication of CP in all ages. The diagnosis of PPF in children requires a high index of clinical suspicion and should be considered in the differential diagnosis of massive pleural effusion where pancreatic pathology is present. A high level of pleural fluid amylase and the results from radiological imaging when the patients have symptoms play essential roles in the diagnosis of PPF. Currently, Magnetic resonance cholangiopancreatigraphy (MRCP) is the imaging modality of choice. Endoscopic therapy and surgery are treatment options for patients who do not respond to conservative therapy.

show abstract

Endoscopic Management of Pancreaticopleural Fistula in a Child with Hereditary Pancreatitis

Cited by 8 publications

References 18 publications

R116C mutation in PRSS1 gene causes hereditary pancreatitis and elevated creatine kinase in one child：a case report

R116C mutation in PRSS1 gene causes hereditary pancreatitis and elevated creatine kinase in one child：a case report

Pancreaticopleural fistula in children: Report of 2 cases

Pancreaticopleural fistula in a child with chronic pancreatitis harboring compound SPINK1 variants

Contact Info

Product

Resources

About