Transcript Chronic Pancreatitis in Children

CHRONIC PANCREATITIS IN
CHILDREN
Shannon Cannon
December 5, 2013
Presentation and Surgical Management
Case Presentation
A.D. is an 11 yo F with diagnosis of chronic pancreatitis,
referred from GI Clinic. She first presented with pancreatitis in
2006 and has had multiple subsequent episodes of
progressively worsening length/severity. Her symptoms have
worsened in last 3 months. Her main presenting symptom is
abdominal pain.
PMH: Choledocholithiasis
PSH:
 Appendectomy 2009
 Cholecystectomy 8/2012
 ERCP with sphincterotomy x3, most recently on 9/2013
Case Presentation (continued)

Chronic abdominal pain
 Taking
amitriptyline, oxycodone, and ibuprofen
regularly
 Seen in pain medicine clinic for chronic pain associated
with pancreatitis

SPINK1 positive
ERCP
Pediatric Pancreatitis

Classifications: Acute Pancreatitis, Acute Recurrent
Pancreatitis, Chronic Pancreatitis
 Subset

of chronic – genetic/hereditary pancreatitis
Acute – similar presentation to adult
 Epigastric

pain radiating to back, N/V
Chronic – irreversible inflammatory process with
histologic changes in pancreatic tissue and
subsequent dysfunction
Definitions
INSPPIRE consortium (International Study Group of Pediatric Pancreatitis: In Search of a
Cure)
J Pediatr Gastroenterol Nutr 2012;55: 261 – 265
Epidemiology

Incidence of all pancreatitis in children: 1/50,000
 Chronic
pancreatitis is rare hereditary pancreatitis
more rare

Causes of chronic pancreatitis in Children
 Higher
percentage idiopathic (30-50%) compared to
adult population
 Obstructive: pancreas divisum, choledochal cysts,
duplication of pancreatic duct, trauma, idiopathic
fibrosing pancreatitis
Microscopic findings: Inflammatory cell infiltrate (predominantly chronic),
fibrosis, loss of acinar tissue, relative sparing of the islets of Langerhans
Gastrointestinal and Liver Pathology, Ch.17, 514-556
Presentation

Abdominal Pain
 Neuropathy,
Interstitial and ductal hypertension, Chronic
inflammation, or Pseudocyst

Less common presentations:
 Duodenal/colonic
obstruction
 Splenic vein occlusion
 Suspicion of a malignant neoplasm
Diagnosis

Labs
 Exocrine:
fecal elastase,
amylase, lipase
 Endocrine: blood glucose
 Genetic mutations

Imaging: “chain of lakes,”
dilated pancreatic duct
 CT
 ERCP
 MRCP
Semin Pediatr Surg. 2012
Aug;21(3):266-71.
Hereditary Factors

Associated gene mutations
 CFTR,

Rare…but common among those with chronic
pancreatitis
 79%

PRSS1, SPINK1
of patients in a single-site retrospective study
Increased risk of pancreatic adenocarcinoma
J Pediatr Gastroenterol Nutr. 2012
May;54(5):645-50
Puestow Procedure (Lateral
Pancreaticojejunostomy)


Drainage procedure (as opposed to resection
procedure)
Major steps
Transverse supraumbilical or a Chevron incision.
 Duodenum mobilization, exposure of the anterior pancreas
 Identification of the dilated pancreatic duct, on palpation or
ultrasound, confirmed with needle aspiration.
 Incise dilated pancreatic duct, removing stones if present
 35 cm Roux-en-Y jejunal limb creation, passing roux limb
retrocolic to the pancreas

J Gastrointest Surg 2009 May;13(5):1004-8
Semin Pediatr Surg. 2012
Aug;21(3):266-71.
Patient Selection


Refractory abdominal pain
Attempts at treatment with ERCP & pancreatic duct
stent placement have failed

Retrospectively selected 87 patients with hereditary
pancreatitis (1998-2012)




52% women, median age 16.9±1.7
68% confirmed hereditary with genetic testing (16% SPINK1)
32% went on to surgical intervention, 12.7% with Puestow
Conclusions


21% of patients treated endoscopically first required a
subsequent surgery for recurrent symptoms, 64 % of patients
treated with surgery required a subsequent endoscopic
procedure (p < 0.01).
Time between interventions: 2-3 years with endoscopy, 5-10
years with surgery
www.elsevier.com/locate/jpedsurg
Surgical t reat ment of childhood recurrent pancreat it is
Mat t hew S. Clif t on, Juan C. Pelayo, Raul A. Cort es, Erich J. Gret hel,
Amy J. Wagner, Hanmin Lee, Michael R. Harrison,
Diana L. Farmer, Kerilyn K. Nobuhara*
Division of Pediatric Surgery, Department of Surgery, University of California San Francisco, Box 0570,
San Francisco, CA 94143-0570, USA
Index words:


Abstract
Background/ Purpose: Surgical intervention that improves pancreatic ductal drainage is a reasonable
treatment strategy for recurrent pancreatitis in children.
Methods: This study was approved by the Committee on Human Research (San Francisco, CA). A
retrospective chart review was performed on children aged 0 to 17 years given the International
Classification of Diseases, Ninth Revision coding diagnosis of chronic pancreatitis who underwent
surgical intervention from 1981 to 2005.
Results: From 1981 to 2005, 32 children were treated for the diagnosis of chronic pancreatitis. The
etiologies were obstructive (n = 13), idiopathic (n = 10), hereditary (n = 6), medications (n = 2), and
infection (n = 1). Fifteen patients underwent 17 operations for chronic pancreatitis, including Puestow (n =
9), cystenterostomy (n = 2), Whipple (n = 1), distal pancreatectomy (n = 1), Frey (n = 1), DuVal (n = 1),
excision of enteric duplication cyst (n = 1), and pancreatic ductal dilation (n = 1). The mean age at
presentation of patients undergoing surgery was 6.0 F 4.1 years (mean F SD). The mean time from
presentation to operation was 3.3 F 3.3 years.
There were no deaths after surgical intervention. Of 15 patients, 2 (13%) required rehospitalization within
90 days of surgery, one for bowel obstruction, the other for splenic infarction. The median length of stay
postoperatively was 8 days (range, 5-66 days).
Conclusions: Chronic pancreatitis in children differs markedly in etiology when compared with adults. In
most cases seen in our institution, chronic pancreatitis resulted from ineffective ductal drainage. These
disorders are amenable to surgical decompression, which, ultimately, can prevent disease recurrence.
D 2007 Elsevier Inc. All rights reserved.
Retrospective study of 32 pediatric patients with
chronic pancreatitis (1981-2005)
15 patients with surgical intervention, 9 with
Puestow procedure (47%, 28%)
Chronic pancreatitis;
Puestow procedure
 Mean
age at time of surgery 6.0±4.1 years
 Mean time presentation to operation 3.3±3.3 years

Childhood pancreatitis in the United States is a relatively
rare clinical entity with an incidence of approximately 1 in
50,000. The clinical presentation is similar to that in
adulthood, with the cardinal symptom being epigastri c pain
radiating to the back. Nausea and vomiting are commonly
associated symptoms [1,2]. Abnormal laboratory values
include elevations of serum amylase and lipase; however, in
the setting of long-standing disease, elevations may not be
present because of severe fibrotic changes in the pancreas.
Traditionally, pancreatitis is categorized as either acute or
chronic. Acute pancreatitis is an inflammatory process of the
pancreas, with or without multiorgan system involvement of
defined duration, followed by normal restoration of pancreatic function and morphology [3]. Chronic pancreatitis is a
7 patients with follow-up, 5 completely pain-free
* Corresponding author. Tel.: +1 415 476 0449; fax: +1 415 476 2314.
E-mail address: [email protected] (K.K. Nobuhara).
0022-3468/$ – see front matter D 2007 Elsevier Inc. All rights reserved.
doi:10.1016/j.jpedsurg.2007.02.009
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Modified Puestow procedure for the management of chronic
pancreatitis in children
Pablo Laje, N. Scott Adzick
Department of Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA, USA
Received 18 January 2013; revised 5 March 2013; accepted 6 March 2013

Retrospective series 2003-2013 of 6 patients with chronic pancreatitis with
Key words:
Puestow
procedure Abstract
Modified Puestow
Purpose: To present our experience with the modified Puestow procedure in the management of





5 patients previouslyMedian
failed
ERCP
time between
onset of treatment
pain and surgery was 4 years (range: 1–9). Median age at surgery was
7.5 years (range: 5–15). Median hospital stay was 12 days (range: 9–28). Median follow up was
years (range: 5 months
9 years). All patients
had temporary postoperative
improvement of their
Improved pain in all4.5
patients
buttovariable
long-term
results
abdominal pain. In two patients the pain recurred at 6 months and 2 years postoperatively and
pancreatitis. The pancreatic duct was dilated in all cases, with a maximum diameter of 5 to 10 mm.




procedure;
children with chronic pancreatitis.
3/6
had SPINK1
mutation
Chronicpatients
pancreatitis
Methods: Retrospective chart review of patients treated between 2003 and 2012.
Results: Six patients underwent a modified Puestow procedure (lateral pancreaticojejunostomy) for the
Median age 7.5 years
management of chronic pancreatitis, three females and three males. Four patients had hereditary
pancreatitis (three with confirmed N34S mutation in the SPINK1 gene), one patient had chronic
Median time presentation
to operation
years
pancreatitis of unknown
etiology, and one4patient
with annular pancreas developed obstructive chronic
required total pancreatectomy to treat intractable pain, 3 and 8 years after surgery. Two
Recurrence of pain,eventually
eventual
pancreatectomy (n=2, both hereditary)
patients were pain free for two years and subsequently developed occasional episodes of pain. The two
most recent patients are pain free at 1 year (obstructive chronic pancreatitis) and 5 months (hereditary
Recurrence of acute
pancreatitis episodes, overall improved pain (n=2, 1
pancreatitis) follow-up. Two patients developed type I diabetes mellitus 10 and 12 months
postoperatively (one with hereditary and one with idiopathic chronic pancreatitis).
hereditary)
Conclusion: We conclude that the modified Puestow procedure in children is feasible and safe. It seems
provide
definitive pain control
and prevent further damage to the pancreas in patients with obstructive
Pain free, too soontochronic
for
follow-up
(n=2)
pancreatitis. However, in patients with hereditary pancreatitis, pain control outcomes are
variable and the operation may not abrogate the progression of disease to pancreatic insufficiency.
Conclusion: Safe to perform,
variable pain outcomes with hereditary
© 2013 Elsevier Inc. All rights reserved.
pancreatitis
Case Recap



11 yo F with hereditary, chronic pancreatitis
presenting with severe pain, refractory to
endoscopic interventions. SPINK1 mutation positive.
Implication of SPINK1 mutation in outcome for this
patient?
Need for further interventions in the future?
Conclusions



Chronic pancreatitis is rare in children (hereditary
pancreatitis even rarer)
Puestow procedure is a drainage procedure used to
treat refractory cases of chronic pancreatitis
Low-powered studies are not strongly conclusive in
demonstrating lasting outcomes or long-term
efficacy of Puestow.
Future Directions

Robotic Puestow



Meehan J & Sawin RR, Robotic lateral pancreaticojejunostomy (Puestow).
J Pediatr Surg. 2011 Jun;46(6):e5-8
Eid GM et al. Robotic-assisted laparoscopic side-to-side lateral
pancreaticojejunostomy. J Gastrointest Surg. 2011 Jul;15(7):1243
Further identifying genetic causes of chronic pancreatitis

Masson E et al. A conservative assessment of the major genetic causes of
idiopathic chronic pancreatitis: data from a comprehensive analysis of
PRSS1, SPINK1, CTRC and CFTR genes in 253 young French patients.
PLoS One. 2013 Aug 8;8(8):e73522
Questions?
References
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9.
Morinville VD, et al. Definitions of Pediatric Pancreatitis and Survey of Present Clinical
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Dzakovic A & Superina R. Acute and chronic pancreatitis: surgical management. Semin
Pediatr Surg. 2012 Aug;21(3):266-71.
Ceppa EP, et al. Hereditary pancreatitis: endoscopic and surgical management. J
Gastrointest. Surg. 2013 May;17(5):847-56; discussion 856-7
Sultan M, et al. Genetic prevalence and characteristics in children with recurrent
pancreatitis. J Pediatr Gastroenterol Nutr. 2012 May;54(5):645-50
Ceppa EP and Pappas TN. Modified puestow lateral pancreaticojejunostomy. J
Gastrointest Surg. 2009 May;13(5):1004-8
Clifton MS, et al. Surgical treatment of childhood recurrent pancreatitis. J Pediatr Surg.
2007 Jul;42(7):1203-7.
Miqdady M and Kitagawa S. Causes of chronic pancreatitis in children and adolescents. Up
To Date, accessed 12/4/2013
DuBay D, et al. The modified Puestow procedure for complicated hereditary pancreatitis in
children. J Pediatr Surg. 2000 Feb;35(2):343-8
Iacobuzio-Donahue CA & Montgomery E. (2012) Gastrointestinal and Liver Pathology.
“Non-neoplastic and neoplastic pathology of the pancreas” 2nd Ed. Ch. 17: 514-556