CHRONIC
FIBROSING
PANCREATITIS
IN
CHILDHOOD:
A
CAUSE
OF
RECURRENT
ABDOMiNAL
PAIN
Thomas E. Williams, Jr., M.D., Neil J. Sherman, M.D.,
and H. William Clatworthy, Jr., M.D.
Front the Departments of Surgery, The Ohio State University College of Medicine,
and The Children’s Hospital, Columbus, Ohio
(ReceivedJtine23; acceptetl for publication August 15, 1967.)
-
-Supported in ptrt l)\’ a graduate training grant, 1TL GM 15.39-01, from the Ihsstitthte of General
NIe(liCal Sciences, National Institutes of Health, Bethesda, ‘i-1arvlahld.
T.E.’\V., Jr., is resi(ient ill surgery, The Ohio State University, and National Institutes of Health Aca-(lemic Trainee in Research Surgery; N.J.S. is resident in surgery, The Ohio State Universth’.
ADDRESS FOR REPRINTS: (H.W.C.,Jr.) Children’s Hospital, Columbus, Ohio 43205.
PEDIATRICS, Vol. 40, No. 6, December 1967
D
ISOIIDEIIS of tile pancreas have received little attention as a cause of recurrent abdominal pain in children. Standard texts of pediatrics, pediatric surgery, andpathol-ogy make virtually no mention of acute or
chronic pancreatitis in childhood. Although
acute and traumatic pancreatitis are more
frequently described entities in the younger
age groups, the chronic forms are rarely
recognized. Since there is no characteristic clinical picture, only five cases14 of one
chronic form, idiopathic fibrosing pancrea-titis, have been reported in children.
Al-tilough tilese patients have no distinctive
symptoms or laboratory findings, the micro-scopic examination of the pancreas charac-teristically shows an interstitial fibrosis, loss of acini, sparing of the islets of Langerhans,
and a variable amount of inflammatory reaction.
An additional case in a young girl with severe disabling abdominal pain is reported here. A Ilumber of findings in this carefully studied patient suggest that the develop-ment of this fibrotic process is not a
conse-quence of an obstructive phenomenon in
the pancreatic ducts.
CASE REPORT
K. 1-I., ail 1 1-year-old white female, entered the
Columbus Children’s Hospital in February 1966
for evaluation of recurrent abdominal pain. For
the 3 ears prior to admission the patient had multiple episodes of epigastric tramping or aching pain, which would come on suddenly and last from
1 to 6 days. The pain had no relationship to meals.
There were no nausea, vomiting, diarrhea, jaun-dice, or foul sillehliilg stools. Tile patient had not
lost weight (Fig. 1). ShIe hla(I obtained partial
relief from antichohinergics, paregoric, and aspirin,
but nlisse(l a total of 83 days of the school year.
The patient had measles at age 5 ‘ears. A paternal
grandfather had episodes of recurrent abdominal pain.
On examination there vas moderate tenderness
in both lower quadrants, somewhat more marked
on the right.
The hemoglobin was 1 1.4 gm/100 ml,
hemato-crit was 34%, leucocvte count was 7,400 cells per
cubic mm, and the erythrocyte sedimentation rate was 22 mm per hour. The urinalysis was normal. The serum iipase was 0.6 Cherry-Crandall units, and the serum amyiase ranged from 106 to 150 Somogi units. The serum calcium was 9.7 mgI
100 ml, and a fasting blood sugar was 86 mg/100 ml. The blood urea nitrogen and serum electrolytes
vere normal. The serum cholesterol was 145 mg/
100 ml, the phospholipids were 167 mg/100 ml, and the free fatty acids were 0.7 tg/1O0 ml. Re-peated stool fat determinations were negative, and trvpsin was present in the stools. No ova or
para-sites were demonstrated. The heterophil titer anti
lupus erythematosus cell preparations were
nega-tive, but tile latex fixation was 2 + positive. Boentgen examinations of the chest and abdo-men were normal; no calcifications were seen in the abdomen. An upper gastrointestinal
examina-tion showed some delay of passage of the contrast
material through the duodenum and a slight
en-hargement of the C-loop. The barium enema, oral
cholecystogram, and intravenous urogram were
nor-111,11. A selective celiac angiogram showed normal
filling of this vascular bed with no stenosis. An
dee-troencephalogram was normal.
F’ollowing her initial evaluation, the patient was
reatimitted to the hospital for exploratory lapa-rotomy. The liver and gallbladder were grossly normal; the stomach and the mesentery of the colon and small bowel appeared somewhat
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AGE-YEARS
ilL. 1. Growth chart, K.II. Arrows lhldicate tIle age at Wilich the patient ilad iler first clinical
svmp-tonis. There is no apparent ill effect OIl 11cr
rOwtll.
and firnl iiodular masses vere present in the Ilead
of tilt’ dhlCC5. A I)0rt11 pr(sstire of 18 cm of
saline Was Oi)tained and a portovenogram vas nor-mal. A biopsy sas taken from tile head of the
pancreas. lollowing tllis a tube ciloiecvstostomv
\Tds 1)erforllled in order to obtain a I)0st0l)erltie
cholangiogram. ‘Iwo w(eks after tile operation a
biliai’ perfusion pr’sstire of 1 1 em of hvpaque
was fouiid \hehl a chloiecvstochoiangiogranl %aS
perforflled. The p’ig resstire at this time as
20 cm of hvpaque. A common channel of the
corn-mon i)ii(’ (ilict dhl(l pancreatic duct s’as demon-strated (Fig. 2). Amviase (leterminations on the (iriiilage from tile cilolec\stostorny tulle were
per-sisteti ti zero. 11w l)dtiellt progressed satisfactorily
dhl(l ulultiple strum ani\lasc detenniiiations in tue
postoperative I)ri01 \%(‘r( not elevated.
Nonetile-less, at tilt.’ time of discharge tile pati(nt was hay-ing intermittent (‘I)isO(ies of pain simiiar to that
Vhlicil sile 11:1(1 at the time of admission. \Vhien tile
Iiti(hit \ids (hiscilirge(l fronl tile h051)ital, ill April 1966, she was taking tincture of belladonna four times (iailV (mid occasionally sup)ierneIltiIlg thliS
vith I)I1(l):irl)it:ii. Two v(’eks after leaving tile
llospitil the patient had a severe attack of epi-gastric pain after (irinkihig iced tea. The pltient l)ec:ihlle asvmptomatic ail(l (liscofltinUed belladonna
nl(dication in .\ngust, 1966. She hlad had few
cohfll)ldillts ill tll( sIli)se(1tlellt ear ill(i has returned
to school and full activity.
of chronic pa1lcre1titis associated with
aminoaciduria. Subsequently, five otiler
families have been reported. Gerber op-erated on two cilildren with the hereditary form of chronic pancreatitis; upon finding
FIG. 2. Perfusion cholecvstocholangiogranl. Arrows
in(hicate dive ill the proxilmil Il:1ncre1tic duct; a
Fic. .3. Biopsy specimen, head of pancreas. Note the dense interstitial fi-brosis, loss of acinar tissue, sparing of the islets of Langerhans, and the
focal complex branching ducts. Inflammatory cells are absent.
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multiple ductal strictures and a stenosed proximal pancreatic duct, he provided re-lief by performing a pancreatic drainage
procedure as was done by Cornet and his
associates. Poulsen in 1950 reported the
association of hyperhpidemia and
pancrea-titis; more of tllese cilildren are now being recognized. Hendren, et al.1#{176}have recently
collected several cases which presented
with symptoms similar to those in our
pa-tient. In some an area of obstruction in the
pancreatobiliary system was demonstrated and corrected with an appropriate surgical procedure. Tile gross appearance of the pancreas at surgery and tile absence of a demonstrable obstructive mechanism in their tilird patient is similar to our case.
Chronic relapsing pancreatitis is charac-terized by exacerbations of pain and tipper abdominal discomfort witil intervals of seeming well-being. In the cases reviewed and in our patient, pain located anywhere
in the tipper abdomen is the primary and
most constant symptom. Tile serum
amy-lase is usuallv normal. Adults frequently
lose weight; children often have grown
nor-mally. Symptoms at first are transitory and
mild; but, diabetes, steatorrllea, aIld pail-creatic calcification may ensue over a num-ber of years. This clinical picture must be contrasted with recurrent acute episodes, where the patient presents \Vitll an acute abdomen and an elevated serum amylase. Although many writers believe that recur-ring attacks oP acute pancreatitis may lead
!:o tile chronic form tilis is nt always tile case. Certainly, most believe, tilat ductal
obstruction is a primary factorin producing
pancreatitis witil a subsequent necrosis aild fibrosis.
The etiologic diagnosis of idiopathic
cllronic fibrosing pancreatitis is one of cx-clusion. The patients with the familial and hereditary forms demonstrate a positive family history, aminoaciduria, or
hyperli-pidemia. Pancreattis secondary to
hvper-parathyroidism, infectious disease, or due-tai obstruction by helminths must be exclud-ed by appropriate studies. A conclusive evaluation of obstruction in the
bili-,i
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,FIG. 4. Higher magnification of an area of complex ducts in Figure 3.
Eosinophlilic material is present in the iurnina and there are multishaped
configurations of the ductuies.
cause, it must be intrapancreatic. \Vain-wright’s autopsy studies’ have suggested
that tilis condition of scarring in tile ab-sence of a severe clinical picture is most iikely due to multiple subclinical episodes
of focal acinar rupture and subsequent fibrosis with an associated squamous
meta-Piasia of tile smaller ducts.
In our I)atiellt tile sections of tile
pan-creas contained large fibrous septa corn-posed of a dense hyalinized coliagenous material which separated the remaining
iso-lated pancreatic tissues. The ducts were of normal size. Complex branching was seen in one area (Fig. 3 and 4). No squamous
metaplasia of the ducts was noted but eosin-ophilic secretions were present in tile lti-mina. The islet cells appeared normal. The marked fibrosis seen in this patient is similar to that seen in specimens of end
These microscopic findings, tIle normal hiliary perfusion presstlr(’s, and the normal cilolangiogram suggest tilat major pancreat-ic ductal obstruction is not Iresent. Since there was ilO apparent element of
obstruc-tion in the smaller ducts, tile pathogenesis
of tile diffuse fibrosis remains unexplained.
SUMMARY
An unusual instance of chronic fibrosing pancreatitis in a young girl is presented. This represents tile sixth case reported in
tile literature. This diagnosis should be
sus-pected in cases of lOilg-term, unexplained, recurring, disabling, abdominal pain.
Com-plete evaluation of the etiologic factors and anatomy before laparotomy is essential. A thorough examination of the pancreatobili-ary tree during surgery and a careful biop-sy will prove the diagnosis. The etiology of
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-ARTICLES
this fibrosing process cannot be explained
for obstruction does not appear to be a pre-dominant factor.
REFERENCES
1. Comfort, is-I. \V., Garnbiii, E. E., and Bagen-stoss, A. H. : Chronic relapsing pancreatitis: a study of 29 cases without associated
disease of the bihiary or gastrointestinal tract. Gastroenterologv, 6:239 and 376,
1946.
2. Davis, M. L., and Keisey, W. M. : Chronic
pancreatitis in childhood. Amer. J. Dis.
Chiid., 81:687, 1951.
3. Warwick, W. J., and Leavitt, S. R. : Chronic
relapsing pancreatitis in childhood. J. Dis.
Child., 99:648, 1960.
4. Ingomar, C. J., and Terslev, E. : A case of chronic pancreatitis in early childhood.
Danish Med. Bull., 12:91, 1965.
5. Comfort, M. W., and Steinberg, A. C.:
Pedi-gree of a family with hereditary chronic
re-lapsing pancreatitis. Gastroenterologv, 21:
54, 1952.
6. Gross, J. B., Gambill, E. E., and Ulricil, J. A.:
Hereditary pancreatitis: description of a fifth kindred and summary of clinical features. Amer.
J.
Med., 33:358, 1962.7. Cornet, E., Dupon, hi., hardy, M., and Gordeef, A.: Pancreatite chronique famiiiale
primitive avec ectasies canaiaires. (6 cas operes. ) J. Chir., 84:527, 1962.
8. Gerber, B. C. : Hereditary pancreatitis. Arch. Surg., 87:70, 196:3.
9. Poulsen, H. M. : Famiiial lipemia. Acta Med.
Scand., 138:413, 1950.
10. Hendren, W. H., Creep, J. M., and Patton,
A. S.: Pancreatitis in childhood: exierence
with fifteen cases. Arch. Dis. Child., 40:132,
1965.
1 1. Wainwright, C. W. : Intrapancreatic
obstruc-tion. New Eng. J. Med., 244:161, 1951.
12. Blumenthal, H., and Probstein, J. C. : A con-cept of cirrhosis of the pancreas. Arch. Surg.,
81:396, 1960.
Acknowledgment
Drs. Charles Reiner and Jacob \V. Old both
