The Unusual

Presentation

of Pancreatitis

By Steven 2. Rubin and Sigmund

in Infancy

H. Ein

Toronto, Ontario. Canada l Two infants with acute pancreatitis are described. It appears that acute pancreatitis in infancy presents in one of two ways: part of a severe generalized illness, or as ascites. The outlook for the former depends on the prognosis of the generalized illness. If pancreatitis can be recognized as the cause of the ascites. then adequate treatment will result in survival. Although the cause in some infants is said to be idiopathic, exploration should be performed, the lesser sac investigated and if the child is in reasonable condition, appropriate study of the biliary tree and pancreatic ductile system should be considered. If a pseudocyst is present, adequate drainage is mandatory. Early diagnosis and appropriate treatment will be possible only if this rare disorder is suspected. The eventual prognosis is excellent and the chance of recurrent attacks is minimal.

INDEX WORD: Pancreatitis.

T HEtis PAUCITY in infancy

of descriptions of pancreatiand the resultant difficulty in diagnosis and delay in treatment play a significant role in the morbidity and mortality. Within the past 5 yr, two infants of 3 mo presented with ascites of unknown etiology. The similarity of both in reference to their presentation, treatment, complications and outcome warrants their being reported. CLINICAL

MATERIAL

Case I (#802921) J.H. presented at the age of 3 mo with a 2-wk history of girth. green, “mushy” diarrhea and increasing abdominal Following a normal atraumatic pregnancy and delivery he thrived for the first 2% mo of life. There was t-t? history of trauma and no familial illnesses were noted. Examination on admission revealed mild dyspnea with grunting respirations. The abdomen was distended by ascites and bilateral pitting ankle edema was elicited. There was

From The Division of General Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada. Presented at Canadian Association of Pediatric Surgeons Meeting Toronto, Oniario. Canada, January 1977. Address reprint requests to Sigmund H. Ein. M.D., F.R.C.S.(C). Division of General Surgery, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario. Canada MSG 1X8. 01979 by Grune & Stratton, Inc. 0022-3468/79/l 402-0006$01.00/0

146

nothing to suggest any trauma. Investigation included a complete blood count, BUN, electrolytes, calcium, amylase, proteins, urinalysis, bone marrow, and urine VMA; all values were within the normal range. Paracentesis of the ascitic fluid revealed an inflammatory exudate. It was not tested for amylase. Chest x-rays showed an increasing pleural effusion that was eventually controlled by the insertion of an intercostal drain removing amber colored inflammatory exudative fluid. Intravenous pyelography and barium enema were normal, but a barium meal suggested a retrogastric mass. Despite supportive care with intravenous nutrition, he deteriorated rapidly with an increasingly tense and swollen abdomen associated with compromised respirations. On June 26, 1970 laparotomy was performed. The preoperative diagnosis of malignancy was excluded but a postoperative differential diagnosis of a hard retrogastric mass remained. Although “chronic pancreatitis” was considered, a gastric duplication was thought more likely. Postoperatively, nasojejunal feeding was unsuccessful, and intravenous nutrition was complicated by sepsis. A suspected left subphrenic abscess required re-exploration on August 12, 1970. The abscess proved to be an infected pseudocyst of the tail of the pancreas. The fluid contained amylase at a concentration of 2500 Somogyi units/liter. External drainage of the cyst was instituted. Immediate postoperative improvement was followed by a recrudescence of the presenting symptoms. Despite the moribund state reoperation was carried out on September 28, 1970. Distal pancreatectomy and Roux-en-Y pancreatojejunostomy was performed. A steady convalescence followed this procedure. At the age of 2 yr, his weight (I 3.8 kg) and height (89 cm) were at the 75th percentile, and he has remained well for the last 6 yr. Comment. If an amylase had been determined in the ascitic fluid, pancreatitis would have been suspected and more attention would have been directed toward the pancreas during exploration. At the second operation, when an infected pseudocyst was found, the decision to drain externally was made because the child was very sick and the pseudocyst wall was too thin for internal drainage. External drainage in this case was faster, easier and technically more feasible. For the same reasons a distal pancreatectomy and Roux-en-Y pancreato-jejunostomy was decided upon when the third operation was performed 6 wk later.

Case 2 (#994783) J.G. was first admitted for investigation of ascites at 3 mo of age. The pregnancy and delivery were uneventful. The baby gained 5% lb in the first 21/, mo of life, but after weaning she ceased to thrive and an increasing abdominal girth was noted. There was nothing to suggest trauma. Investigations included a complete blood count, BUN, electrolytes, proteins, liver function tests, routine urinalysis, chest x-ray, intravenous pyelography, liver spleen scan,

Journal of Pediatric Surgery, Vol. 14, No. 2 (April), 1979

147

PANCREATITIS

abdominal angiography, and inferior vena cavagram. All were normal. On July 22, 1975 exploratory laparotomy demonstrated an inflammatory process along the lesser curve of the stomach and around the head of the pancreas. There was no evidence of fat necrosis. Histology of specimens of oment m and exudate showed acute inflammation. A serum amylase postoperatively was 83 Somogyi U/l. Amylase content of the amber colored clear ascitic fluid was 200 Somogyi U/l. She was discharged improved on the tenth postoperative day, but recurrent ascites associated with a septic fever and anemia required rehospitalization. A cystic mass was palpable in the left upper quadrant; this finding was confirmed by abdominal x-ray, liver spleen scan, and echography. Serum amylase was 62 Somogyi U/l. Severe respiratory distress, high fever, and a hemoglobin of 6 g/100 ml accompanied the increasingly distended and tense abdomen. A blood transfusion and emergency re-exploration were performed on August 30, 1975 and a large pseudocyst of the pancreas was drained externally. It contained a murky fluid with an amylase of 2000 Somogyi U/l. Because oral diet was contraindicated, on the third postoperative day when her general condition had stabilized, a nasojejunal feeding tube (size 5F) was easily passed under fluroscopy and a gradually increasing concentration and volume of “Flexical” (Mead Johnson Laboratories, Canada) was administered by a slow drip. A mild bleed heralded a major hemorrhage from the cyst lumen on September 6, 1975. This abated spontaneously, but blood transfusion was required. Four days later, a spontaneous cyst-jejunostomy was demonstrated by a sinugram, external drainage ceased, and the abdominal wound closed. No mass was palpable and radiography and abdominal echography did not show a cyst. During the seventh postoperative week a normal oral diet was commenced and nasojejunal feeding was discontinued. She has remained well for almost 2 yr. Comment. Again, retrospectively, testing the ascitic fluid for amylase would have been helpful in this case. At the initial exploration, greater attention should have been made to exclude the diagnosis of pancreatitis. In this patient, who was not as sick as the previous infant, it may have been worthwhile pursuing investigation at the operating table to exclude an anomaly causing pancreatitis i.e., stenosis of the ampulla of Vater or a pancreatic duct anomaly. At the second operation, it was elected to perform external drainage rather than an internal drainage procedure because the pseudocyst was just beneath the abdominal wall incision and therefore most suitable for this simple treatment.

DISCUSSION

Pancreatitis in infancy is a rare disease with diverse etiologies. In childhood, the major causes are traumatic and idiopathic.’ Less common causes include congenital pancreatitis or an inherited metabolic defect especially of amino acid or lipid metabolism. Mild pancreatitis with a raised serum amylase may be recognized during the course of diabetes mellitus, mumps, collagen disease, burns, and sepsis. Drugs, espe-

cially steroids and hydrochlorothiazides, have an association with acute pancreatitis. Among reports of pancreatitis in childhood, a few scattered cases in the first year of life are described.‘” In early infancy, the single most important step in the diagnosis is the actual consideration of the possibility that one is dealing with pancreatitis. The striking absence of the adult clinical pattern of severe abdominal pain, vomiting, and signs of peritonitis is evident. Two distinct clinical syndromes of pancreatitis in early infancy emerge. The first is the frequent post mortem finding of pancreatic inflammation in the neonatal infant who is severely ill and usually septic.2 Pancreatitis in this instance is not the primary cause of the child’s poor condition but an incidental finding. The second mode of presentation is exemplified by the two cases in this paper. There is a gradual onset of malaise, irritability, weight loss, and fever associated later with vomiting and increasing abdominal distension due to ascites. Serum amylase, proteins, and bilirubin are characteristically normal, and x-ray investigations are similarly noncontributory. The only diagnostic test is the increased amylase content of the ascitic fluid. The pancreatic ascites are often associated with a pseudocyst. However, in contradistinction to the pseudocyst occurring in later childhood, there is no history of preceding trauma.5 In addition to our two infants described, four similar cases have been variously reported.” The difficulty in diagnosis becomes obvious when one considers the number of operations performed on these six children (Table I). In cases 5 and 6, even the initial laparotomy did not give a definitive diagnosis and the postoperative diagnoses were gastric duplication and healed perforated gastric ulcer respectively. The only slightly increased amylase content of the ascitic fluid in case 6 might have alerted the surgeons to the possibility of acute pancreatitis. Once a pseudocyst has developed, barium meal, echography and computed tomography x-ray scanning are useful. The operative procedure is often complicated by the lack of a preoperative diagnosis in a moribund child. Ascitic fluid should be sent for amylase, and the surgeon should look for fat necrosis. The lesser sac and pancreas must be carefully inspected. It has been often assumed

148

RUBIN AND

Table 1. Pancreatitis

EIN

(With Ascites) in Infancy

Number of Operations

Hendren et al.

I

2

Degnosis

Preoperative

Definitive Procedure

Pancreatitis

Laparotomy

Pericarditis

Thcracotomy-negative Roux-en-Y cystjejunostomy

Hendren et al.

3

Pancreatitis

Laparotomy i- external drainage

Stenosis of ampulla

Transduodenal sphincterotomy

Recurrent pancreatitis Hartley

2

duodenal duplication

Pancreatoduodenectomy

‘Hemorrhagic ascites’

Negative laparotomy

Chronic intussusception

Laparotomy + external drainage

Coupland

1

Ascites

Laparotomy and external drainage

Rubin and Ein

3

Retroperitoneal malignancy

Laparotomy

Subphrenic abscess

External drainage of infected pseudocyst

Severe pancreatitis

Distal pancreatectomy and Roux-en-Y

Ascites

Laparotomy

Severe ascites i- respiratory distress

External drainage of pseudocyst

pancreatojejunostomy Rubin and Ein

VI

2

that the cause of this infantile pancreatitis was idiopathic, but in most cases seen during infancy, no attempt has been made to track down the anatomic cause, if such a definitive lesion in fact exists.‘” Most of these small children have been treated by simple drainage. Only one of the six cases (Table 1) had recurrent acute pancreatitis and was shown to have a duodenal duplication obstructing the pancreatic duct requiring a pancreatoduodenectomy.3 No disease of the biliary system was noted in any of these infants, although Hendren4 Moossa,’ Blumenthal,* and others have reported stenosis of the ampulla of Vater as a cause of pancreatitis. It would seem entirely reasonable to exclude that pathology by cholangiography and possibly also transduodenal exploration of the ampulla, unless the infant were moribund at the time. It has also been emphasized that pancreatography can be of help in disclosing an anomaly of the ductile system of the pancreas as a cause for pancreatitis. Again this should be done only in a patient in reasonable condition at the time of operation.

Pseudocysts are said to be best drained internally but there should be no hesitation to drain these cysts externally if the situation so warrants it. Once the diagnosis of pancreatitis is made, external drainage of the lesser sac should be considered. External drainage has been used as a life-saving procedure in the management of pancreatic abscess and the occasional immature pseudocyst although a long standing pancreatic fistula has been described complicating this form of drainage.’ The preoperative and immediate postoperative support of the patient with intravenous infusions, nasogastric drainage, and adequate monitoring in an intensive care unit are necessities. While the disease is still active it is important to avoid stimulation of the pancreas while maintaining nutrition. This can be accomplished by total parenteral nutrition, but equally well and less hazardously, by a continuous jejunal elemental diet while maintaining nasogastric is not necessary as drainage.8S9 A gastrostomy the tube can be passed transnasally.

REFERENCES 1. Moosa AR: Acute pancreatitis in childhood. Prog PediatrSurg4:111-127, 1972 2. Blumenthal HT, Probstein JG: Acute pancreatitis in the newborn in infancy and in childhood. Am Surg 27:533539, 1961 3. Coupland G: Pancreatic ascites in childhood. J Pediatr Surg 5570, 1970 4. Hendren WH, Greep JM, Patton AS: Pancreatitis in childhood: Experience with IS cases. Arch Dis Child 40: 132145,1965 5. Hartley RC: Pancreatitis Under the age of 5 years: A report of 3 cases. J Pediatr Surg 2:419-423, 1967

6. Williams WH, Hendren WH: lntrapancreatic duodenal duplication causing pancreatitis in a child. Surgery 69:708-715, 1971 7. Welch KJ: In Mustard WT. et al: Pediatric Surgery Vols. 1 and 2 (ed 2). Chicago, Year Book Publications, 1969, p 750 8. Voitk AJ, Brown RA, McArdle AH, et al: Clinical uses of an elemental diet: Preliminary studies. CMA Journal. 107:123-129,1972 9. Ragins H, Levenson SM. Signer R, et al: Intrajejunal administration of elemental diet at neutral pH avoids pancreatic stimulation. Am J Surg 126:606-614, 1973