Biliary Manometry in Choledochal Cyst With Abnormal Choledochopancreatico Ductal Junction By Naomi Iwai, Kazuaki Tokiwa, Toshiaki Tsuto, Jun Yanagihara, and Toshio Takahashi Kyoto, Japan 9 Intraoperative m a n o m e t r y of the biliary tract and measurement of amylase levels in choledochal cysts w e r e performed in seven patients, aged 14 months to 6 years, with choledochal cysts, in an investigation of the pathophysiology of the biliary tract. An abnormal choledochopancreatico ductal junction was observed in these seven patients by preoperative endoscopic retrograde cholangiopancreaticography (ERCP) or intraoperative cholangiograms. All six patients examined showed a high amylase level in the choledochal cyst (5,450 to 4 6 , 6 0 0 Somogyi Units). The intraoperative m a n o m e t r y of the biliary tract showed that a remarkable high pressure zone as was found in the area of sphincter of Oddi was not found in the area of abnormal choledochopancreatico ductal junction. The pressure recordings also demonstrated that the sphincter of Oddi pressure in the patient with choledochal cyst was increased by gastrin stimulation. On the contrary, no pressure reaction to gastrin or secretin was found in the area of abnormal choledochopancreatic ductal junction. From these results it seems that free reflux of pancreatic juice into the biliary system occurs, and the reflux stream depends upon the pressure gradient b e t w e e n pancreatic ductal pressure and common bile duct pressure because of the lack of a sphincter function at the choledochopancreatico ductal junction. 9 1986 by Grune & Stratton, Inc. INDEX W O R D S : anomalies.
Choledochal
cyst;
pancreaticobiliary
S T H E E T I O L O G Y of choledochal cyst, Babbitt' proposed an abnormal relationship between the common bile duct and the pancreatic duct. Since then, attention has been drawn mainly to the morphologic abnormality of the common bile duct and the pancreatic duct. 2' 3.4 To determine the etiology of choledochal cysts, however, one must also study the pathophysiology of the biliary tract secondary to an abnormal choledochopancreatico ductal junction.
A
MATERIALS AND METHODS From 1962 to 1985, 27 children with congenital choledochal cyst were treated in the Division of Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine. Seven of the 27 patients have recently undergone intraoperative manometry of the biliary tract, and an abnormal junction of the pancreaticobiliary ductal system was found in all seven by preoperative endoscopic retrograde cholangiopancreaticography (ERCP) or intraoperative cholangiograms. Their ages were 14 months to 5 years. Five were female and two were male. Four had fusiform and three had cystic dilatation of the common bile duct. The length of the common channel, measured by E R C P or intraoperative cholangiography, ranged from 1.3 to 2.0 cm. The manometric studies were performed intraoperatively. Pressure recordings were obtained with a polyvinyl catheter with an
Journal of Pediatric Surgery, Vol 21, No 10 (October), 1986: pp 873-876
internal diameter of 0.8 m m and an outer diameter of 1.0 mm. The probe contained an end hole orifice measuring 0.8 m m in diameter. The probe was filled with sterile saline, and perfusion took place at a constant rate of 30 m L / h . This apparatus was connected to a transducer (Gould Inc, P231D, Oxnard, Calif), and the pressures were recorded on a Nippon-Sanei thermal pen recorder (NipponSanei 360, 8 channel polygraph, Tokyo). Zero pressure, used throughout this study, was determined by recording atmospheric pressure at the distal end of the common bile duct. To obtain pressure recordings from the biliary duct system the probe was inserted into the sphincter of Oddi through the distal end of the common bile duct and the abnormal choledochopancreatico ductal junction. The probe was then drawn from the duodenum to the biliary tract at a constant speed of 0.8 m m / s . The pressure profile of the biliary tract was recorded in centimeters as the probe was withdrawn. Tetragastrin (4 3,/kg) was injected intravenously, and manometric studies of the biliary tract were performed before and three minutes after the injection. Secretin (1 U / k g ) was then administered as a single intravenous injection after the resting pressure of the biliary tract had returned to the base line. The manometric study was performed five minutes after the injection of secretin. Pressure measurements were recorded with the distal end of the common bile duct as zero. Sphincter of Oddi peak pressure was determined as sphincter of Oddi pressure, and the length of the high pressure zone was measured in centimeters. An abnormal choledochopancreatico ductal pressure is defined as a pressure at the distal end of the common channel. Results were expressed as mean _+ SE, and Student"s t-test was used for statistical analysis. A p value < .05 was considered to be significant.
RESULTS
A m y l a s e Levels in the Choledochal Cyst
As shown in Table 1, all of the six patients, who were examined, showed high amylase levels in the choledochal cyst (5,450 to 46,500 Somogyi Units).
Biliary Manometry Before Enteric Hormone Stimulation The seven patients examined before gastrin or secretin stimulation exhibited a characteristic biliary pressure profile (Fig 1). As the probe was drawn into the sphincter of Oddi from the duodenum, a sharp rise
From the First Department of Surgery, and the Division of Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Japan. Address reprints requests to Naomi lwai, MD, Division of Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kamigyo-ku, Kyoto, 602, Japan. 9 1986 by Grune & Stratton, Inc. 0022-3468/2110-0010503.00/0 873
874
IWAI El- AL
Table 1. Cases of Choledochal Cyst With Abnormal Choledochopancreatico Ductal Junction in Which We Performed Intraoperative Biliary Manometry Patient No.
Age
Sex
Symptom
Form of Choledochal Dilatation
Length of Common Channel (cm)
Amylase Level in the Cyst
1
12/3yr
F
Abdominal pain, jaundice, fever
Fusiform
2.0
46,500
2
3 yr
M
Abdominal pain, vomiting
Cystic
1.5
27,850
3
5 yr
M
Abdominal pain, vomiting
Fusiform
1.3
7,290
4 5
14 mo 3 yr
F F
Fever Abdominal pain, vomiting
Cystic Fusiform
1.4 1.5
5,450 18,400
6
3 yr
F
Vomiting
Fusiform
2.0
12,300
7
16 mo
F
Fever, icterus
Cystic
1.5
Not examined
4
3
2
1
0 cm
0.3 cm. The choledochopancreatico ductal junction pressure was 12.0 _+ 1.2 cmH20.
Biliary Manometry After Gastrin Stimulation abnormal cho ledochopancreatico ductal junction
Fig 1. Biliary resting pressure profile of case 1. The record shows an intraluminal pressure as the probe is drawn from the duodenum to the distal end of the common bile duct. A marked high pressure zone is observed in the area of the sphincter of Oddi, and a gradient decline of pressure is found in the common channel and abnormal choledochopancreatico ductal junction.
in pressure occurred and a gradient decline of pressure was observed in the common channel and the abnormal choledochopancreatico ductal junction. The pressure then dropped to zero at the distal end of the common bile duct. The average values of the duodenum and sphincter of Oddi pressure were 8.0 _+ 2.1 c m H 2 0 and 35.0 • 6.7 cmH20, respectively. Thus, the pressure difference between the sphincter of Oddi pressure and the duodenal pressure was 27.0 _+ 2.7 cmH20. The length of the high pressure zone in the sphincter of Oddi was 1.7 • 4
3
2
1
As shown in Table 2, the duodenal pressure after gastrin stimulation was 8.5 _+ 2.3 cmH20. The mean sphincter of Oddi pressure increased from 35.0 • 6.7 c m H 2 0 before gastrin stimulation, to 46.5 _+ 5.4 c m H 2 0 after stimulation (Fig 2); not a significant change. However, the change in pressure difference before and after gastrin stimulation was significant (P < .05). The mean length ~)f the high pressure zone after gastrin stimulation was 1.8 _+ 0.1 cm. The choledochopancreatico ductal junction pressure after gastrin stimulation was 12.3 • 1.3 cmH20. Thus, tetragastrin stimulation caused no significant change.
Biliary Manometry After Secretin Stimulation As shown in Table 3, secretin stimulation lowered duodenal pressure to 4.5 + 0.9 cmH20, but this was not a significant change. The mean values of sphincter of Oddi pressure and the pressure difference after secretin stimulation were 17.5 _+ 2.5 c m H 2 0 and 15.0 + 2.2 cmH20, respectively. These values were
0 cm
abnormal choledochopancreatico ductal Junction
Fig 2. Biliary pressure profile a f t e r gastrin stimulation of case 1. An increase of sphincter of Oddi pressure is found after gastrin administration. However, no reaction to gastrin stimulation is found in the area of abnormal choledochopancreatico ductal junction.
Table 2. Pressure Measurements of the Biliary Tract Before and After Tetragastrin Stimulation (Mean _+ SE)
Before stimulation (n = 7) After gastrin stimulation (n = 7) *P < .05.
Duodenal Pressure (cmH20)
Sphincter of Oddi Pressure (cmH20)
Pressure Difference Between Sphincter of Oddi Pressureand Duodenal Pressure (cmH20)
8.0 _+ 2.1 8.5 -+ 2.3
35.0 + 6.7 46.5 _+ 5.4
27.0 _+ 2.7* 38.0 -+ 3.8*
Length of High Pressure Zone (cm) 1.7 _+ 0.3 1.8 _+ 0.1
Choledochopancreatico Ductal Junction Pressure (cmH20) 12.0 _+ 1.2 12.3 _+ 1.3
875
BILIARY MANOMETRY IN CHOLEDOCHAL CYST
Table 3. Pressure Measurements of the Biliary Tract Before and After Secretin Stimulation (Mean _+ SE)
Duodenal Pressure (cmH20)
Sphincter of Oddi Pressure (cmH20)
Pressure Difference Between Sphincter of Oddi Pressure and Duodenal Pressure (cmH20)
Before stimulation (n - 7)
8.0 -+ 2.1
35.0 + 6.7*
2 7 . 0 _+ 2.7 t
1.7 + 0.3
12.O + 1.2
After secretin stimulation (n - 7)
4.5 _+ 0 . 9
17.5 + 2 . 5 *
15.O + 2.2~-
1.3 _+ 0.1
9.3 _+ 1.2
Length of High Pressure Zone (cm)
Choledochopancreatico Ouctal Junction Pressure (cmH20)
* P < .05. t P < .02.
significantly lower than the prestimulation values (Fig 3). The length of the high pressure zone after secretin stimulation was 1.3 + 0.1 cm, which is not significantly different from the prestimulation length. Secretin stimulation decreased the pressure of the choledochopancreatico ductal junction to 9.3 + 1.2 c m H 2 0 , n o t a significant change. DISCUSSION
The amylase level of the cystic contents in this series was elevated, as noted in previous reports, 5'6 indicating reflux of the pancreatic juice into the bile duct. Manometric studies of the biliary tract in human adults have recently been performed with E R C P manometry. %8 In our study, however, intraoperative manometry was done on younger children to obtain pressure recordings from the biliary tract. In the present study, a high pressure zone in the area of the sphincter of Oddi was found in patients with choledochal cyst as in normal human adults. 7'8 As one of the etiologic factors in choledochal cyst formation, an abnormally high pressure on the distal side of the common bile duct, including the sphincter of Oddi, has been suggested by Okada et al. 9 However, it was impossible to judge from the present study whether the sphincter of Oddi pressure in patients with choledochal cyst was higher than normal. 4
1
-T--L
L
~
~
2
~
_
1
0 cm
i
!
1
abnormal choledocho~ [,ancrea tico ductal junction
Fig 3. Biliary pressure profile after secretin stimulation of case 1. A decrease of sphincter of Oddi pressure is observed after secretin administration. However, no reaction to secretin s t i m u l a tion is found in the area of abnormal choledochopancreatico ductal junction.
In the nonstimulated biliary tract, no high pressure zone was found in the area of the common channel or the choledochopancreatico ductal junction. This result indicates that there is no sphincter function in these regions as there is in the area of the sphincter of Oddi and that the influence of the sphincter of Oddi pressure does not extend to the area of the abnormal choledochopancreatico ductal junction. The action of gastrin on the sphincter of Oddi was studied by Lin ~~and Geenen et al, 7 who found that it increased the sphincter of Oddi pressure in normal adults. In the present study, gastrin also increased the sphincter of Oddi pressure in patients with choledochal cyst. However, no increase of pressure was found in the area of the common channel or the choledochopancreatico ductal junction after the administration of tetragastrin. This result indicates that the change in sphincter function caused by gastrin administration does not extend to the area of abnormal choledochopancreatico ductal junction. Geenen et al reported that secretin caused an initial increase in human sphincter of Oddi pressure within three minutes after intravenous administration, but at six minues the sphincter of Oddi pressure frequently fell below control levels. In our present study, secretin decreased the sphincter of Oddi pressure five minutes after administration. On the other hand, secretin did not decrease the pressure in the area of the common channel or the abnormal choledochopancreatico ductal junction. This result indicates that secretin's effect on sphincter function does not extend to the area of abnormal choledochopancreatico ductal junction. The amylase levels in choledochal cysts and pressure changes in the biliary tract suggest that a free reflux of pancreatic juice into the biliary system is allowed, and the reflux stream depends on the gradient between pancreatic ductal pressure and common bile duct pressure. This free reflux might be explained by the lack of sphincter function at the junction of the common and pancreatic ducts, which was demonstrated by our pressure measurements.
REFERENCES
1. Babbitt DP: Congcnital choledochal cyst: New etiological concept based on anomalous relationship of the common bile duct and pancreatic bulb. Ann Radiol 12:231-240, 1969
2. Komi K, Kuwashima T, Kuramoto M, et al: Anomalous arrangementof the pancreaticobiliaryductal system in choledochal cyst. TokushimaJ. Exp Med 23:37 48, 1976
876
3. Jona JD, Babbitt DP, Starshak R J, et al: Anatomic observations and etiologic and surgical considerations in choledochal cyst. J Pediatr Surg 14:315-320, 1979 4. Arima E, Akita H: Congenital biliary tract dilatation and anomalous junction of the pancreatico-biliary ductal system. J Pediatr Surg 14:9-15, 1979 5. Kimura K, Tsugawa C, Ogawa K, et al: Choledochal cystetiological considerations and surgical management in 22 cases. Arch Surg 113:159-163, 1978 6. Miyano T, Suruga K, Suda K: Abnormal choledocho-pancreatico ductal junction related to the etiology of infantile obstructive jaundice diseases. J Pediatr Surg 14:16-26, 1979
IWAI ET AL
7. Geenen JE, Hogan W J, Dodds W J, et al: Intraluminal pressure recording from the human sphincter of Oddi. Gastroenterology 78:317-324, 1980 8. Guelrud M, Bettarello A, Cecconello I, et al: Sphincter of Oddi pressure in chagasic patients with megaesophagus. Gastroenterology 85:584-588, 1983 9. Okada A, Ooguchi Y, Kamata S, et al: Anomalous junction of pancreatico-biliary ductal system. Jap J Pediatr Surg 14:53-60, 1980 10. Lin TM: Actions of gastrointestinal hormones and related peptides on the motor function of the biliary tract. Gastroenterology 69:1006-1022, 1975