Impact of Age at Diagnosis on Clinical Features in Children with Anomalous Arrangement of the Pancreaticobiliary Duct

Abstract

Aims: In patients with an anomalous arrangement of the pancreaticobiliary duct (AAPBD), clinical presentations may differ between infants and older children. The optimal timing of surgery remains controversial, particularly in early infancy. The aim of this study was to evaluate the clinicopathological features and clinical outcomes using comparative methods between infants and older cases.

Materials ad Methods: From 1983 to 2007, a total of 85 consecutive children with AAPBD were treated at our institute. They included 46 with the cystic type, 33 with the fusiform type, and 6 with the non-dilatation type. These patients were divided into 2 age groups: “infant” (n=9), <12 months old; and “older”, >1 year old (n=76). A retrospective study was performed.

Results: Mean age was 5.2 months (range, 8 days–11 months) in the infant group and 5.2 years (range, 1.2–17.3 years) in the older group. Jaundice was significantly more frequent in the infant group (p<0.05), whereas abdominal pain was more common in the older group (p<0.001). Bleeding tendencies such as cranial hemorrhage or bloody stools were noted in only 3 infants. In terms of liver histology, liver cirrhosis was observed in 2 infants, one of whom was a 3-month-old girl with severe jaundice resulting in living-donor liver transplantation, despite bile drainage. A single postoperative death occurred due to an adenocarcinoma arising in a choledochal cyst in a 12-year-old girl.

Conclusions: Problems characteristic of infantile AAPBD were a severe bleeding tendency and irreversible liver cirrhosis, which could develop as young as 3 months old. The surgical recommendation for infantile AAPBD is thus early surgery before the age of 3 months to prevent liver failure.

Key words

anomalous arrangement of the pancreaticobiliary duct - infant - choledochal cyst - bleeding tendency - liver failure

References

• 1 Babbitt DP, Starshak RJ, Clemett AR. Choledochal cyst: a concept of etiology.  Am J Roentgenol Radium Ther Nucl Med. 1973;  119 57-62
• 2 Castaldo P, Grazi GL, Wood RP. et al . An unusual case of choledochal cyst causing hepatic failure requiring orthotopic liver transplantation.  J Pediatr Surg. 1992;  27 1557-1559
• 3 Chaudhary A, Dhar P, Sachdev A. et al . Choledochal cysts – differences in children and adults.  Br J Surg. 1996;  83 186-188
• 4 Chen CJ. Clinical and operative findings of choledochal cysts in neonates and infants differ from those in older children.  Asian J Surg. 2003;  26 213-217
• 5 de Vries JS, de Vries S, Aronson DC. et al . Choledochal cysts: age of presentation, symptoms, and late complications related to Todani's classification.  J Pediatr Surg. 2002;  37 1568-1573
• 6 Fumino S, Iwai N, Deguchi E. et al . Bleeding tendency as a first symptom in children with congenital biliary dilatation.  Eur J Pediatr Surg. 2007;  17 2-5
• 7 Fumino S, Iwai N, Deguchi E. et al . Spontaneous rupture of choledochal cyst with pseudocyst formation – report on 2 cases and literature review.  J Pediatr Surg. 2006;  41 e19-e21
• 8 Iwai N, Deguchi E, Yanagihara J. et al . Cancer arising in a choledochal cyst in a 12-year-old girl.  J Pediatr Surg. 1990;  25 1261-1263
• 9 Iwai N, Fumino S, Tsuda T. et al . Surgical treatment for anomalous arrangement of the pancreaticobiliary duct with nondilatation of the common bile duct.  J Pediatr Surg. 2004;  39 1794-1796
• 10 Iwai N, Yanagihara J, Tokiwa K. et al . Congenital choledochal dilatation with emphasis on pathophysiology of the biliary tract.  Ann Surg. 1992;  215 27-30
• 11 Jackson CC, Wu Y, Chenren S. et al . Bile decompression in children with histopathological evidence of pre-existing liver cirrhosis.  Am Surg. 2002;  68 816-819
• 12 Jona JZ, Babbitt DP, Starshak RJ. et al . Anatomic observations and etiologic and surgical considerations in choledochal cyst.  J Pediatr Surg. 1979;  14 315-320
• 13 Jordan Jr PH, Goss Jr JA, Rosenberg WR. et al . Some considerations for management of choledochal cysts.  Am J Surg. 2004;  187 790-795
• 14 Kimura K, Ohto M, Ono T. et al . Congenital cystic dilatation of the common bile duct: Relationship to anomalous pancreaticobiliary ductal union.  Am J Roentogenol. 1977;  128 571-577
• 15 Lebenthal E, Lee PC. Development of functional responses in human exocrine pancreas.  Pediatrics. 1980;  66 556-560
• 16 Ono S, Sakai K, Kimura O. et al . Development of bile duct cancer in a 26-year-old man after resection of infantile choledochal cyst.  J Pediatr Surg. 2008;  43 E17-E19
• 17 Ono S, Tokiwa K, Aoi S. et al . A bleeding tendency as the first symptom of a choledochal cyst.  Pediatr Surg Int. 2000;  16 111-112
• 18 Suita S, Shono K, Kinugasa Y. et al . Influence of age on the presentation and outcome of choledochal cyst.  J Pediatr Surg. 1999;  34 1765-1768
• 19 Todani T, Akita E, Eto T. (The Japanese Study Group on Pancreaticobiliary Maljunction): Diagnostic criteria of pancreaticobiliary maljunction.  J Hep Bil Pancr Surg. 1994;  1 219-221
• 20 Todani T, Urushihara N, Morotomi Y. et al . Characteristics of choledochal cysts in neonates and early infants.  Eur J Pediatr Surg. 1995;  5 143-145
• 21 Tsai MS, Lin WH, Hsu WM. et al . Clinicopathological feature and surgical outcome of choledochal cyst in different age groups: the implication of surgical timing.  J Gastrointest Surg. 2008;  12 2191-2195
• 22 Vijayaraghavan P, Lal R, Sikora SS. et al . Experience with choledochal cysts in infants.  Pediatr Surg Int. 2006;  22 803-807

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