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Indian Pediatr 2013;50:
1016-1019 |
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Clinical Profile and Outcome of Chronic
Pancreatitis in Children
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SD Chowdhury, A Chacko, BS Ramakrishna, AK Dutta, J Augustine, AK
Koshy, EG Simon and AJ Joseph
From Department of GI Sciences, Christian Medical College, Vellore,
Tamil Nadu, India.
Correspondence to: Professor Ashok Chacko, Department of G.I.
Sciences, Christian Medical College, Vellore,
Tamil Nadu, India.
Email: [email protected]
Received: April 03, 2013;
Initial review: May 28, 2013;
Accepted: June 03, 2013.
Published online: May 5, 2013.
PII: S097475591200371
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Objective: To evaluate the etiology, presentation, complications and
management of chronic pancreatitis in children.
Design: Retrospective chart review.
Setting: Gastroenterology department at Christian
Medical College and Hospital, Vellore, India between January 2005 and
December 2010.
Participants: 99 Children (<18 yrs) diagnosed
with chronic pancreatitis based on clinical and imaging features.
Main outcome measures: Etiology, clinical
presentation, complications and management of chronic pancreatitis in
children.
Results: Of 3887 children who attended the
Gastroenterology department, 99(2.5%) had chronic pancreatitis, of which
60 (60.6%) were males. In 95(95.9%) patients no definite cause was
detected and they were labeled as Idiopathic chronic pancreatitis. All
patients had abdominal pain, while 9(9.1%) had diabetes mellitus. Of the
22 children tested for stool fat, 10(45.5%) had steatorrhea. Pancreatic
calcification was seen in 69 (69.7%). 68 (71.6%) patients with
idiopathic chronic pancreatitis had calcification. Calcific idiopathic
chronic pancreatitis was more frequent in males (67.6% vs. 48.1%,
P=0.07), and was more commonly associated with diabetes mellitus
(13.2% vs. none, P=0.047) and steatorrhea (61.5% vs.
16.7%, P=0.069). Pseudocyst (17.1%) and ascites (9.1%) were the
most common complications. All children were treated with pancreatic
enzyme supplements for pain relief. 57 patients were followed up. With
enzyme supplementation, pain relief was present in 32 (56.1%) patients.
Of those who did not improve, 10 underwent endotherapy and 15 underwent
surgery. Follow up of 8 patients who underwent endotherapy, showed that
5 (62.5%) had relief. Follow up of 11 patients who underwent surgery
showed that only 3 (27 %) had pain relief. There was no death.
Conclusions: Idiopathic chronic pancreatitis is
the predominant form of chronic pancreatitis in children and
adolescents. It can present with or without calcification. The calcific
variety is an aggressive disease characterized by early morphological
and functional damage to the pancreas.
Key words: Chronic pancreatitis, Diabetes
mellitus, Pancreatic calcification, Steatorrhea, Tropical chronic
pancreatitis.
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Chronic pancreatitis is a progressive inflammatory
disease leading to irreversible damage of the pancreas with resultant
exocrine and endocrine insufficiency. Abdominal pain is one of the most
distressing symptoms of chronic pancreatitis and leads to significant
impairment of quality of life [1]. Steatorrhea and diabetes mellitus
develop in the long term [2].
Though the disease is well characterized in adults,
there is limited data in children and adolescents; and most of the
reported series include small number of subjects [3-5]. We conducted
this study to evaluate the etiology, clinical presentation,
complications and management of chronic pancreatitis in children.
Methods
Medical charts of patients attending the
Gastroenterology department at the Christian Medical College and
Hospital, Vellore, India between January 2005 to December 2010 were
retrospectively reviewed. Children diagnosed with chronic pancreatitis,
with age of onset of symptoms below 18 years were included.
Chronic pancreatitis was diagnosed on the basis of
clinical features (abdominal pain, steatorrhea or diabetes mellitus) and
the identification of pancreatic ductal and/or parenchymal changes
(calcification, atrophy, ductal dilatation) on imaging [6]. Clinical
information, complications and laboratory data were collected by a
standardized review of medical charts and the data recorded in data
forms. Nutritional status was assessed using WHO growth charts for
height for age and body mass index (BMI) for age [7].
Pancreatic exocrine insufficiency was assessed by
estimation of 72-hour stool fat using van de Kamer method [8].
Steatorrhea was diagnosed if the stool fat was >18 g in a 72-hour stool
collection [9]. Blood sugar was checked in all the children. A fasting
plasma glucose of ³
126 mg/dL, 2 hr post-prandial plasma glucose >200 mg/dL or HbA1C >6.5%
was used to diagnose diabetes mellitus [10]. Morphological changes in
the pancreas were identified by imaging studies of abdomen viz.
ultrasound abdomen, contrast enhanced computed tomography (CECT), MRI
with maganetic resonance cholangio-pancreatography (MRCP), endoscopic
retrograde cholangio-pancreatography (ERCP) and / or endoscopic
ultrasound (EUS). Main pancreatic duct was considered to be dilated if
the duct diameter was more than 3 mm in the head and 2 mm in the body or
tail of the pancreas [11].
Etiology of chronic pancreatitis was defined as
follows: Metabolic chronic pancreatitis: Presence of
hypercalcemia (>10.5 mg/dL) or hypertriglyceridemia (> 1000 mg/dL);
Hereditary chronic pancreatitis:
³2 first degree
relatives diagnosed to have chronic pancreatitis with an autosomal
dominant pattern of inheritance; Traumatic chronic pancreatitis:
History of significant abdominal trauma preceding the onset of symptoms;
and Idiopathic chronic pancreatitis: No definite cause detected
[12].
Statistical analysis: Baseline data were
presented as mean (SD) for continuous variables and as proportions for
categorical variables. For comparison between two groups, chi square
test or Fisher’s exact test was used for discrete variables and
independent sample t test was used for continuous variables.
Ethical clearance for the study was obtained from the Institutional
review board of Christian Medical College, Vellore, India.
Results
A total of 3887 children were seen in the
Gastroenterology department between January 2005 and December 2010. Of
these children, 99 (2.5%) were diagnosed to have chronic pancreatitis.
No definite etiology was detected in 95 (95.9%)
patients and they were labeled as idiopathic chronic pancreatitis.
History of blunt abdominal trauma prior to onset of symptoms was present
in 4 (4.04%) patients and they were diagnosed as post traumatic chronic
pancreatitis. Pancreas divisum was detected in 8 (8.42%) of these
patients and family history of chronic pancreatitis was present in 3
patients.
The mean (SD) age at presentation and onset of
desease was 15.2 (3.6) and 11 (4) years, respectively. Sixty (60.6%)
were males. All patients had abdominal pain requiring an average of two
hospitalizations for pain. Diabetes mellitus was detected in 9 (9.1%)
children. The blood sugar control in these children was poor as
evidenced by mean (SD) HbA1c of 9.5 (2.7). Seven children with diabetes
mellitus were treated with insulin for blood sugar control, one child
was started on oral hypoglycemic agent and another was on diet control
alone.
Elevated stool fat was detected in 10 (45.5%) of 22
children evaluated for the same. Two of these children had symptomatic
steatorrhea while 8 had subclinical steatorrhea. Height and weight were
recorded in 32 children; height for age was <3 rd
percentile in 9 (28.1%) and BMI for age was <3rd
percentile in 16 (50%) children. Pancreatic morphological abnormalities
as identified by imaging studies is shown in Table I.
Dilatation of the main pancreatic duct was seen in 88 (88.9%),
pancreatic atrophy in 73 (73.7%) and pancreatic calcification in 69
(69.7%) patients. Intraductal calcification was seen in 16 (23.2%)
patients, isolated parenchymal calcification in 30 (43.5%) patients and
combined ductal and parenchymal calcification was seen in 23 (33.3%)
patients. No pancreatic mass was seen in any patient.
TABLE I Morphological Changes of the Pancreas Identified by Imaging
Morphological Changes
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n (%) |
Pancreatic calcification, duct dilatation and parenchymal
atrophy |
43 (43.4%) |
Pancreatic calcification and duct dilatation |
17 (17.2%) |
Duct dilatation and parenchymal atrophy
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24 (24.2%) |
Pancreatic calcification and parenchymal atrophy |
6 (6.1%) |
Pancreatic calcification |
3 (3.0%) |
Duct dilatation |
6 (6.1%) |
Complications were noted in 30 (30.3%) children. Five
children had more than one complication. Pseudocyst 17(17.1%) and
ascites 9(9.1%) were the most common complications. In fifteen children,
the pseudocyst was managed conservatively with nasojejunal feeds and
pancreatic enzyme supplementation. In two children, pseudocysts were
drained at surgery. Ascitic fluid examination was done in five children.
All had elevated ascitic fluid amylase. Splenic vein thrombosis was
detected in 4 (4.04%) cases. Three children had gastrointestinal
bleeding in the form of hematemesis and / or melena. In one patient
fundal varix was identified as the cause of bleeding and cyanoacrylate
glue injection was performed. In another child a pseudoaneurysm
involving the splenic artery measuring approximately 5.7 × 5 cm was
detected using CT angiogram, and treated by coil embolization. In the
third child no definite cause was identified. None of the children had
biliary obstruction or fistula formations. Malignancy was not seen in
any patient.
Amongst the children with idiopathic chronic
pancreatitis, two morphological types were identified, one group with
calcification (Calcific idiopathic chronic pancreatitis -CICP) and the
other without calcification (Non-Calcific idiopathic chronic
pancreatitis-NCICP). Comparison of the clinical profile of the two
groups is shown in Table II.
TABLE II Clinical Profile of Calcific and Non Calcific Idiopathic Chronic Pancreatitis
Clinical |
Calcific Idiopathic |
Non -Calcific Idiopathic
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P
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Chracteristics |
Chronic Pancreatitis
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Chronic Pancreatitis
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Value
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(n =68) |
(n =27) |
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Age of onset
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11 ± 4.6
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11 ± 4.03
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1.00 |
Sex (M:F)
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46:22
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13:14
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0.07
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Pain
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68 (100%)
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27 (100%)
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- |
Diabetes mellitus
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9 (13.23%)
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0
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0.047 |
Steatorrhea (n = 19) |
8/13 (61.5%)
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1/6 (16.7%)
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0.069 |
All patients with abdominal pain were given a trial
of enteric coated pancreatic enzyme supplements along with proton pump
inhibitors or H2 receptor
antagonist. Follow-up data on 57 patients (median 18 months (2-72
months)) showed that 32 (56.1%) patients had subjective relief of
abdominal pain with pancreatic enzymes. Endotherapy or surgery was
performed in patients who did not have pain relief with enzyme
supplements. Ten patients underwent endotherapy (Extracorporeal shock
wave lithotripsy (ESWL) and stone clearance: 2, ERCP with sphincterotomy
and stenting: 8). Follow-up data available for 8 patients, (median
follow up 36 months (6- 102 months) showed that 5 (62.5%) patients
(1–ESWL and stone clearance; and 4 – ERCP with sphincterotomy and
stenting) had relief of pain following endotherapy. Fifteen patients
underwent surgery for management of abdominal pain, (lateral pancreatic
jejunostomy: 10, Frey’s procedure: 5). Follow up available for 11
patients (median 24 months (12 -60), showed that only 3 (27 %) had pain
relief post surgery.
Discussion
The predominant cause of chronic pancreatitis in our
study was Idiopathic chronic pancreatitis. Three children with family
history of chronic pancreatitis and 8 children with pancreas divisum
were included in the idiopathic chronic pancreatitis group as the 3
children did not have a dominant inheritance pattern and association of
pancreas divisum with chronic pancreatitis is suspect [13].
Complications were seen in one-third cases, the most common being
pseudocyst and ascites. Pain was the presenting symptom in all patients.
Studies from China have also reported Idiopathic
chronic pancreatitis as the predominant cause of chronic pancreatitis
among children [5]. Data on prevalence of exocrine and endocrine
insufficiency amongst children with chronic pancreatitis are limited
[14]. The prevalence of symptomatic steatorrhea was lower (2.02% vs.
9.5%) and diabetes mellitus higher (9.1% vs. 0%) in the present
study as compared to the study by Wang, et al. [5]. The study
from China found that BMI of children with chronic pancreatitis was
similar to that of healthy children and adolescents [5]. In contrast, we
identified nutritional impairment in a significant proportion of Indian
children with chronic pancreatitis. Possible reasons could be high
prevalence of sub-clinical steatorrhea and diabetes mellitus as well as
fat restricted diet [15]. The rate of complications in the present study
was similar to that reported in a large prospective nationwide study of
adult chronic pancreatitis from India [16].
An interesting observation was failure of surgery to
provide sustained relief of pain as opposed to studies on adults with
chronic pancreatitis where surgery was effective for short and long term
relief of pain [17]. The reason for discrepancy in pain relief with
surgery and endotherapy is not clear. A possible reason could be that
patients selected for surgery had more aggressive disease where relief
of duct obstruction alone did not provide adequate relief of pain.
Two subsets of idiopathic chronic pancreatitis were
identified a large group with pancreatic calcification (CICP) and a
smaller group without pancreatic calcification (NCICP). Patients with
Calcific idiopathic chronic pancreatitis had higher prevalence of
diabetes mellitus. Calcific idiopathic chronic pancreatitis is
phenotypically similar to ‘Tropical Calcific pancreatitis’ and differs
from ‘early onset (juvenile) idiopathic chronic pancreatitis’ of the
West as it is characterized by male predominance, early calcification
and a high frequency of endocrine insufficiency. Non-calcific idiopathic
chronic pancreatitis on the other hand has an equal sex distribution, no
calcification and low incidence of endocrine insufficiency. This group
needs to be followed up for a longer period of time to ascertain whether
it is phenotypically similar to ‘Early onset (juvenile) idiopathic
chronic ‘pancreatitis’ of the West.
The present study has several limitations: a
retrospective study in which assessment of pain was subjective and
evaluation and management were not protocol based; data on growth
parameters and steatorrhoea were not available for all patients; and
genetic mutation analysis was not performed for etiology evaluation.
Contributors: AC and SDC: conceived and designed
the study, and along with BSR and AJJ: revised the manuscript for
important intellectual content; AC: will act as the guarantor of the
article; SDC, AKK and JA: collected the data and drafted the paper; EGS
and AKD: analyzed the data and helped in manuscript writing.
Funding: None; Competing interests: None
stated.
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