Therapeutic step-up strategy for management of hereditary pancreatitis in children

doi:10.1016/j.jpedsurg.2014.05.016

Journal of Pediatric Surgery

Volume 50, Issue 4, April 2015, Pages 511-514

https://doi.org/10.1016/j.jpedsurg.2014.05.016 Get rights and content

Abstract

Background/Purpose

Various different regimes exist for the treatment of hereditary pancreatitis in childhood. Here, we propose a therapeutic pathway with emphasis on endoscopic and surgical procedures.

Methods

From 2006 to 2013, 12 patients with a diagnosis of hereditary pancreatitis were prospectively included in a therapeutic step-up schema. The treatment outcome was evaluated and correlated to aetiological factors and pathoanatomic findings.

Results

After diagnostic work-up (laboratory data, ultrasound examination, magnetic resonance cholangiopancreatography and genetic testing), all 12 patients underwent early endoscopic retrograde cholangiopancreatography (ERCP), which was successfully performed in ten children. Obstructive pancreatitis was found in eight children, and required sphincterotomy, dilation and stenting for 12 months. In two children with unsuccessful ERCP, open surgical drainage procedures were performed. After a mean follow-up of 32 months all children are free of recurrence of pancreatitis without any impairment of everyday activities.

Conclusions

For children with hereditary pancreatitis, a therapeutic step plan with early ERCP and open surgical drainage procedures in case of impossible or insufficient endoscopic treatment prevents recurring pancreatitis and offers a normal quality of life without any major complications.

Section snippets

Patients and methods

From 2006 to 2013, we treated 12 patients with a diagnosis of hereditary pancreatitis at our Department of Pediatric surgery. All 12 patients were prospectively included in a therapeutic step-up schema including conservative management, endoscopic retrograde cholangiopancreatography (ERCP) and surgery (Fig. 1). We evaluated the success of treatment and correlated the initial findings and pathoanatomic variations with the available treatment options.

Results

From 2006 to 2013, 12 patients with hereditary pancreatitis were treated at our Department of Pediatric Surgery (Table 1). All children were younger than 16 years. Seven out of 12 patients had a family history of pancreatitis or pancreatic adenocarcinoma. In addition, our series included three siblings.

All patients presented with a history of diffuse abdominal complaints, until elevated amylase and lipase (at least three times over the limit) led to the diagnosis of pancreatitis. The average age

Discussion

Pancreatitis is a rare disease in children and, in contrast to pancreatitis in adults, exogenic factors seem to play a minor role [9]. In up to one third of all cases of childhood pancreatitis, the pathogenesis remains unclear; therefore, these cases are classified as “idiopathic pancreatitis” [10].

A series of genetic anomalies associated with chronic pancreatitis in childhood have been identified in recent years. It is known that mutations in the PRSS1 and SPINK1 genes cause an imbalance

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Citation Excerpt :
Third, sphincterotomies and other endoscopic interventions were frequently performed in both patient cohorts. In 2015 a small study showed encouraging results in 12 children with hereditary pancreatitis who improved after endoscopic therapy or surgery using a step-up approach (mean follow-up 32 months). [15] However a year later, a larger multicenter study showed that endoscopic sphincterotomy does not improve the clinical course of IRAP (median follow-up 84 months). [16]
Idiopathic recurrent acute pancreatitis (IRAP) describes frequent episodes of pancreatitis without an etiology found using current testing. We compared the natural history of IRAP with recurrent acute pancreatitis with genetic mutations.
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IRAP and pancreatitis in mutation carriers is associated with chronic pancreatitis. Important differences in natural history were observed, but no association was found with cancer or pancreas-related mortality. Efforts to understand the genetic contributions to IRAP, minimize opioids and unnecessary ERCPs are encouraged.
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Original ArticleTherapeutic step-up strategy for management of hereditary pancreatitis in children

Abstract

Background/Purpose

Methods

Results

Conclusions

Section snippets

Patients and methods

Results

Discussion

Clin Gastroenterol Hepatol

Pancreatology

Gastroenterology

Pancreatology

Pancreatology

Gastrointest Endosc

Gastrointest Endosc

J Pediatr Surg

J Pediatr Surg

Hereditary pancreatitis is caused by a mutation in the cationic trypsinogen gene

Nat Genet

Mutations in the gene encoding the serine protease inhibitor, Kazal type 1 are associated with chronic pancreatitis

Nat Genet

Variants in CPA1 are strongly associated with early onset chronic pancreatitis

Nat Genet

Mutations of the cystic fibrosis gene in patients with chronic pancreatitis

N Engl J Med

Analysis of CFTR, SPINK1, PRSS1 and AAT mutations in children with acute or chronic pancreatitis

J Pediatr Gastroenterol Nutr

Hereditary pancreatitis: dilemmas in differential diagnosis and therapeutic approach

J Gastrointest Cancer

Original Article
Therapeutic step-up strategy for management of hereditary pancreatitis in children