- Email: [email protected]
Chronic relapsing pancreatitis in childhood
Chronic Relapsing
Pancreatitis
By C. Festen, R. Severijnen, Nijmegen,
Pancreatitis,
chronic relapsing; pancreatitis,
A
LTHOUGH CHRONIC relapsing pancreatitis is a rare disease in childhood, it may be an important cause of recurrent abdominal pain. Diagnosis is frequently difficult, because signs and symptoms are often nonspecific. In infants, pancreatic amylase may not be present in the first year of life, and later peak levels are reached within the first 48 hours, which then rapidly return to normal.’ Chronic relapsing pancreatitis may be due to cystic fibrosis, hyperparathyroidism, hyperlipidemia, and aminoaciduria. Of the idiopathic variety, the hereditary form is the most frequent, This is an autosomal dominant disease of variable penetrance, nearly exclusively in Caucasians. There is no sex preference. Signs and symptoms frequently start early, with a mean age of 10 years and a peak at the age of 5’1~(youngest patient, 11 months).4 Frequency and duration of the attacks are variable. There is no specific cause. Sometimes a family history is difficult to elict and should be specifically questioned. Pancreatic calcifications are observed in 50% to 70% on plain x-rays of the abdomen.2c5 On ultrasonography, the pancreatic duct is frequently dilated, and by endoscopic retrograde cholangiopancreaticography (ERCP) one or more obstructive lesions may be demonstrated. The diagnosis of hereditary chronic relapsing pancreatitis should only be made when the onset of the illness dates back to early childhood, several members of the family are affected, and no specific cause can be detected. Similar clinical and radiographic findings are encountered without a family history. Btirger,3 in a collective review, found the following complications. Ongoing attacks of pancreatitis may lead to diabetes mellitus (22.5%) after a mean dura-
From the Pediatric Surgical Center, Catholic University and St-Radboud Hospital, Nijmegen, The Netherlands. Date accepted: October 6, 1989. Address reprint requests to Professor Dr C. Festen, St-Radboud Hospital, PO Box 9101, Nijmegen, The Netherlands. Copyright 8 1991 by W. B. Saunders Company 0022-3468/91/2602-0016$03.00/0
182
F. v.d. Staak, and P. Rieu
The Netherlands
@As a result of our own experience and a review of the literature, early investigation by endoscopic retrograde cholangiopancreaticography (ERCP) and surgical intervention is recommended in chronic relapsing pancreatitis in childhood. Copyright o 1991 by W.B. Saunders Company INDEX WORDS: childhood.
in Childhood
tion of 33.4 years (youngest patient, 12 years), and steatorrhoea (13%) after a mean duration of 28.5 years (youngest patient, 12 years). Pancreatic stones are seen after an average time of 31.7 years (youngest patient, 7 years). Pseudocyst is seen in 10% to 30%.‘” Cavernous transformation of the portal vein may ensue,’ and there may be an increased risk of pancreatic carcinoma later in life.6 As a special form, fibrosing pancreatitis is described with signs of obstructive jaundice.’ Treatment is usually conservative, and operative treatment is only considered later in life when serious symptoms persist. CASE REPORTS Case 1 A 16-year-old white girl was referred for severe intermittent abdominal pain, which started at the age of 4 years. Four years previously, an ERCP had been performed, which showed a cystic dilatation of the pancreatic duct with several strictures. Amylase and trypsine levels in the duodenal aspirate were lowered. Glucose tolerance test was normal. Her older brother also was treated for chronic relapsing pancreatitis elsewhere. Physical examination was unremarkable. Serum amylase was normal. X-ray studies showed pancreatic calcifications and gallstones. Ultrasonography demonstrated a dilated pancreatic duct and two pseudocysts in the body and the tail of the pancreas. Gallstones were confirmed. ERCP showed the ductal pathology. The choledochal duct was normal. The glucose tolerance test was then abnormal. At celiotomy both pseudocysts were resected, the dilated duct was longitudinally opened, all stones were removed, and a pancreaticojejunostomy was performed. Cholecystectorny was also performed. Postoperative recovery was uneventful. During follow-up (6 years) she remained free of symptoms of pancreatitis. She developed frank diabetes mellitus and had to be treated for anorexia nervosa. There was no steatorrhoea.
Case
2
A 16-year-old white girl was admitted because of attacks of abdominal pain with elevated serum amylase levels of increasing frequency and seriousness, which started at the age of 4 years. Her younger sister, her father, and an aunt had been treated for chronic relapsing pancreatitis. Exploratory celiotomy had already been performed twice, and the appendix was removed. Two years previously diabetes mellitus had been diagnosed. She seemed in good health, and physical examination was unrewarding. Ultrasound showed a dilated pancreatic duct. ERCP confirmed the dilatation and distorsion of the duct. Fat absorption was slightly disturbed. At celiotomy the pancreatic duct was longitudinally opened, the stones were removed, and a pancreaticojejunostomy was performed. The postoperative course was uneventful. During 15year follow-up, she remained free of abdominal symptoms. The diabetes mellitus persisted. She showed normal growth and development. JournalofPediatric
Surgery, Vol26, No 2 (February), 1991:
pp 182-193
183
CHRONIC PANCREATITIS IN CHILDHOOD
Case 3 An B-year-old white girl had recurrent attacks of abdominal pain dating from early childhood. During the attacks the serum amylase was elevated. Glucose tolerance test was slightly disturbed. On plain abdominal radiograph pancreatic calcifications were seen. Ultrasound and ERCP proved cystic dilatation of the pancreatic duct with multiple strictures. Also, the choledocal duct was dilated with a stricture at the ampulla. Celiotomy was performed. The pancreatic duct was filleted for the whole distance and concrements were removed, followed by pancreaticojejunostomy. Cholecystectomy and choledochoduodenostomy were performed. She made an uneventful recovery and remained free of symptoms during 1 year of follow-up. Blood glucose became normal and there was normal growth and development.
DISCUSSION
The most striking experience was that all these patients were free of symptoms immediately after operation and stayed so during the follow-up period. Further destruction of the pancreas seems to be prevented. The same experience is confirmed by several other studies.‘-” The cause of ideopathic chronic relapsing pancreatitis, isolated or familial (hereditary), is not known. The stricture of the pancreatic duct, isolated but frequently multiple, with prestenotic dilatation, may be the cause but also may simply represent the result of recurrent inflammation. Experimentally complete duct obstruction leads to pancreatic atrophy” and not to pancreatitis, but narrowing of the duct can cause chronic and recurrent inflammation.” Obstruction by a tumor or duplication may give chronic relapsing pancreatitis3
The onset of symptoms in early childhood and demonstration of stricture of the pancreatic duct in children as young as 4 years of age has been suggested as proof for a congenital anomaly.8,9 Uniformly good results after surgical decompression of the obstructed duct strongly suggest a causitive relationship. Improved techniques make ERCP possible even in small infants (youngest, 4 months).13 Demonstration of one or more strictures with prestenotic dilatation favor decompression, even at an early age, to relieve pain and prevent further destruction of the pancreas. Several authors noted resolution of pancreatic exocrine and endocrine dysfunction after operation. In experimental pancreatitis, normalization of the histological picture has been described after surgical decompression.‘4 In isolated stricture at the ductal entrance, papillotomy may be considered. When the stenosis is more proximal or the duct is narrowed in several locations, it is filleted longitudinally and all calculi are extracted. Drainage should be accomplished according to the technic of Du Va115or Puestow and Gillesby.16 An eventual pseudocyst should be resected or drained. As a rule, results are excellent. In conclusion, chronic relapsing pancreatitis should be considered in the differential diagnosis of recurrent abdominal pain in childhood. After confirming diagnosis and demonstration of dilatation of the pancreatic duct by ultrasound, ERCP should be performed, even in young infants. After identification of ductal anomaly, early operation should be seriously considered to relieve symptoms and preclude pancreatic destruction.
REFERENCES 1. Rothstein FC, Wyllie R, Gauderer MWL: Hereditary pancreatitis and recurrent abdominal pain of childhood. J Pediatr Surg 20:535-537, 1985 2. Scott HW, Neblett WW, O’Neili JA, et al: Longitudinal pancreaticojejunostomy in chronic relapsing pancreatitis with onset in childhood. Ann Surg 199:610-622, 1984 3. Burger D: Pankreaserkrankungen im Kindesalter. Stuttgart, Germany, Hippocrates, 1982 4. Gross JB, Comfort MW: Hereditary pancreatitis: Report on two additional families. Gastroenterology 32:829-854, 1957 5. Dean RH, Scott HW, Law DH: Chronic relapsing pancreatitis in childhood: Case report and review of the literature. Ann Surg 173:443-449. 1971 6. Kattwinkel J, Lapey A, di Sant’Agnese PA, et al: Hereditary pancreatitis: Three new kindreds and a critical review of the literature. Pediatrics 51:55-69,1973 7. Ghishan FK, Greene HL, Avant G, et al: Chronic relapsing pancreatitis in childhood. J Pediatr 102:514-518, 1983 8. Bell DA, Greco MA, Mitnick JS, et al: Chronic relapsing pancreatitis in childhood. J Pediatr Surg 16:741-743,198l
9. O’Neill JA, Greene H, Ghishan FK: Surgical implications of chronic pancreatitis. J Pediatr Surg 17:920-926, 1982 10. Alwmark A, Jonson G, Mattsson K: Chronic relapsing pancreatitis in a child-Endoscopic diagnosis: A case report. Acta Chir Stand 143:253-255,1977 11. Gebhardt C, Stolte M: Pankreasgang-Okklusion durch Injektion einer schnellhartenden Aminosaurelosung: Experimentelle Studie. Arch Chir 346:149-166,1978 12. Hermann RE, Davis JH: The role of incomplete pancreatic duct obstruction in the etiology of pancreatitis. Surgery 48:318-329.1960 13. Cotton PB, Laage NJ: Endoscopic retrograde cholangiopancreatography in children. Arch Dis Child 57:131-136, 1982 14. Camevali JF, ReMine WH, Dockerty MB, et al: An experimental study of side-to-side pancreaticojejunostomy after ductal obstruction. Arch Surg 80:774-787,196O 15. Du Val MK: Caudal pancreatico-jejunostomy for chronic relapsing pancreatitis. Ann Surg 140:775-785, 1954 16. Puestow CB, Gillesby WJ: Retrograde surgical drainage of pancreas for chronic relapsing pancreatitis. Arch Surg 76898907, 1958
Pancreatitis
By C. Festen, R. Severijnen, Nijmegen,
Pancreatitis,
chronic relapsing; pancreatitis,
A
LTHOUGH CHRONIC relapsing pancreatitis is a rare disease in childhood, it may be an important cause of recurrent abdominal pain. Diagnosis is frequently difficult, because signs and symptoms are often nonspecific. In infants, pancreatic amylase may not be present in the first year of life, and later peak levels are reached within the first 48 hours, which then rapidly return to normal.’ Chronic relapsing pancreatitis may be due to cystic fibrosis, hyperparathyroidism, hyperlipidemia, and aminoaciduria. Of the idiopathic variety, the hereditary form is the most frequent, This is an autosomal dominant disease of variable penetrance, nearly exclusively in Caucasians. There is no sex preference. Signs and symptoms frequently start early, with a mean age of 10 years and a peak at the age of 5’1~(youngest patient, 11 months).4 Frequency and duration of the attacks are variable. There is no specific cause. Sometimes a family history is difficult to elict and should be specifically questioned. Pancreatic calcifications are observed in 50% to 70% on plain x-rays of the abdomen.2c5 On ultrasonography, the pancreatic duct is frequently dilated, and by endoscopic retrograde cholangiopancreaticography (ERCP) one or more obstructive lesions may be demonstrated. The diagnosis of hereditary chronic relapsing pancreatitis should only be made when the onset of the illness dates back to early childhood, several members of the family are affected, and no specific cause can be detected. Similar clinical and radiographic findings are encountered without a family history. Btirger,3 in a collective review, found the following complications. Ongoing attacks of pancreatitis may lead to diabetes mellitus (22.5%) after a mean dura-
From the Pediatric Surgical Center, Catholic University and St-Radboud Hospital, Nijmegen, The Netherlands. Date accepted: October 6, 1989. Address reprint requests to Professor Dr C. Festen, St-Radboud Hospital, PO Box 9101, Nijmegen, The Netherlands. Copyright 8 1991 by W. B. Saunders Company 0022-3468/91/2602-0016$03.00/0
182
F. v.d. Staak, and P. Rieu
The Netherlands
@As a result of our own experience and a review of the literature, early investigation by endoscopic retrograde cholangiopancreaticography (ERCP) and surgical intervention is recommended in chronic relapsing pancreatitis in childhood. Copyright o 1991 by W.B. Saunders Company INDEX WORDS: childhood.
in Childhood
tion of 33.4 years (youngest patient, 12 years), and steatorrhoea (13%) after a mean duration of 28.5 years (youngest patient, 12 years). Pancreatic stones are seen after an average time of 31.7 years (youngest patient, 7 years). Pseudocyst is seen in 10% to 30%.‘” Cavernous transformation of the portal vein may ensue,’ and there may be an increased risk of pancreatic carcinoma later in life.6 As a special form, fibrosing pancreatitis is described with signs of obstructive jaundice.’ Treatment is usually conservative, and operative treatment is only considered later in life when serious symptoms persist. CASE REPORTS Case 1 A 16-year-old white girl was referred for severe intermittent abdominal pain, which started at the age of 4 years. Four years previously, an ERCP had been performed, which showed a cystic dilatation of the pancreatic duct with several strictures. Amylase and trypsine levels in the duodenal aspirate were lowered. Glucose tolerance test was normal. Her older brother also was treated for chronic relapsing pancreatitis elsewhere. Physical examination was unremarkable. Serum amylase was normal. X-ray studies showed pancreatic calcifications and gallstones. Ultrasonography demonstrated a dilated pancreatic duct and two pseudocysts in the body and the tail of the pancreas. Gallstones were confirmed. ERCP showed the ductal pathology. The choledochal duct was normal. The glucose tolerance test was then abnormal. At celiotomy both pseudocysts were resected, the dilated duct was longitudinally opened, all stones were removed, and a pancreaticojejunostomy was performed. Cholecystectorny was also performed. Postoperative recovery was uneventful. During follow-up (6 years) she remained free of symptoms of pancreatitis. She developed frank diabetes mellitus and had to be treated for anorexia nervosa. There was no steatorrhoea.
Case
2
A 16-year-old white girl was admitted because of attacks of abdominal pain with elevated serum amylase levels of increasing frequency and seriousness, which started at the age of 4 years. Her younger sister, her father, and an aunt had been treated for chronic relapsing pancreatitis. Exploratory celiotomy had already been performed twice, and the appendix was removed. Two years previously diabetes mellitus had been diagnosed. She seemed in good health, and physical examination was unrewarding. Ultrasound showed a dilated pancreatic duct. ERCP confirmed the dilatation and distorsion of the duct. Fat absorption was slightly disturbed. At celiotomy the pancreatic duct was longitudinally opened, the stones were removed, and a pancreaticojejunostomy was performed. The postoperative course was uneventful. During 15year follow-up, she remained free of abdominal symptoms. The diabetes mellitus persisted. She showed normal growth and development. JournalofPediatric
Surgery, Vol26, No 2 (February), 1991:
pp 182-193
183
CHRONIC PANCREATITIS IN CHILDHOOD
Case 3 An B-year-old white girl had recurrent attacks of abdominal pain dating from early childhood. During the attacks the serum amylase was elevated. Glucose tolerance test was slightly disturbed. On plain abdominal radiograph pancreatic calcifications were seen. Ultrasound and ERCP proved cystic dilatation of the pancreatic duct with multiple strictures. Also, the choledocal duct was dilated with a stricture at the ampulla. Celiotomy was performed. The pancreatic duct was filleted for the whole distance and concrements were removed, followed by pancreaticojejunostomy. Cholecystectomy and choledochoduodenostomy were performed. She made an uneventful recovery and remained free of symptoms during 1 year of follow-up. Blood glucose became normal and there was normal growth and development.
DISCUSSION
The most striking experience was that all these patients were free of symptoms immediately after operation and stayed so during the follow-up period. Further destruction of the pancreas seems to be prevented. The same experience is confirmed by several other studies.‘-” The cause of ideopathic chronic relapsing pancreatitis, isolated or familial (hereditary), is not known. The stricture of the pancreatic duct, isolated but frequently multiple, with prestenotic dilatation, may be the cause but also may simply represent the result of recurrent inflammation. Experimentally complete duct obstruction leads to pancreatic atrophy” and not to pancreatitis, but narrowing of the duct can cause chronic and recurrent inflammation.” Obstruction by a tumor or duplication may give chronic relapsing pancreatitis3
The onset of symptoms in early childhood and demonstration of stricture of the pancreatic duct in children as young as 4 years of age has been suggested as proof for a congenital anomaly.8,9 Uniformly good results after surgical decompression of the obstructed duct strongly suggest a causitive relationship. Improved techniques make ERCP possible even in small infants (youngest, 4 months).13 Demonstration of one or more strictures with prestenotic dilatation favor decompression, even at an early age, to relieve pain and prevent further destruction of the pancreas. Several authors noted resolution of pancreatic exocrine and endocrine dysfunction after operation. In experimental pancreatitis, normalization of the histological picture has been described after surgical decompression.‘4 In isolated stricture at the ductal entrance, papillotomy may be considered. When the stenosis is more proximal or the duct is narrowed in several locations, it is filleted longitudinally and all calculi are extracted. Drainage should be accomplished according to the technic of Du Va115or Puestow and Gillesby.16 An eventual pseudocyst should be resected or drained. As a rule, results are excellent. In conclusion, chronic relapsing pancreatitis should be considered in the differential diagnosis of recurrent abdominal pain in childhood. After confirming diagnosis and demonstration of dilatation of the pancreatic duct by ultrasound, ERCP should be performed, even in young infants. After identification of ductal anomaly, early operation should be seriously considered to relieve symptoms and preclude pancreatic destruction.
REFERENCES 1. Rothstein FC, Wyllie R, Gauderer MWL: Hereditary pancreatitis and recurrent abdominal pain of childhood. J Pediatr Surg 20:535-537, 1985 2. Scott HW, Neblett WW, O’Neili JA, et al: Longitudinal pancreaticojejunostomy in chronic relapsing pancreatitis with onset in childhood. Ann Surg 199:610-622, 1984 3. Burger D: Pankreaserkrankungen im Kindesalter. Stuttgart, Germany, Hippocrates, 1982 4. Gross JB, Comfort MW: Hereditary pancreatitis: Report on two additional families. Gastroenterology 32:829-854, 1957 5. Dean RH, Scott HW, Law DH: Chronic relapsing pancreatitis in childhood: Case report and review of the literature. Ann Surg 173:443-449. 1971 6. Kattwinkel J, Lapey A, di Sant’Agnese PA, et al: Hereditary pancreatitis: Three new kindreds and a critical review of the literature. Pediatrics 51:55-69,1973 7. Ghishan FK, Greene HL, Avant G, et al: Chronic relapsing pancreatitis in childhood. J Pediatr 102:514-518, 1983 8. Bell DA, Greco MA, Mitnick JS, et al: Chronic relapsing pancreatitis in childhood. J Pediatr Surg 16:741-743,198l
9. O’Neill JA, Greene H, Ghishan FK: Surgical implications of chronic pancreatitis. J Pediatr Surg 17:920-926, 1982 10. Alwmark A, Jonson G, Mattsson K: Chronic relapsing pancreatitis in a child-Endoscopic diagnosis: A case report. Acta Chir Stand 143:253-255,1977 11. Gebhardt C, Stolte M: Pankreasgang-Okklusion durch Injektion einer schnellhartenden Aminosaurelosung: Experimentelle Studie. Arch Chir 346:149-166,1978 12. Hermann RE, Davis JH: The role of incomplete pancreatic duct obstruction in the etiology of pancreatitis. Surgery 48:318-329.1960 13. Cotton PB, Laage NJ: Endoscopic retrograde cholangiopancreatography in children. Arch Dis Child 57:131-136, 1982 14. Camevali JF, ReMine WH, Dockerty MB, et al: An experimental study of side-to-side pancreaticojejunostomy after ductal obstruction. Arch Surg 80:774-787,196O 15. Du Val MK: Caudal pancreatico-jejunostomy for chronic relapsing pancreatitis. Ann Surg 140:775-785, 1954 16. Puestow CB, Gillesby WJ: Retrograde surgical drainage of pancreas for chronic relapsing pancreatitis. Arch Surg 76898907, 1958