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 manometry of the biliary tract and measurement of amylase levels in choledochal cysts were performed in seven patients, aged 14 months to 6 years, with choledochal cysts, in an investigation of the patho- physiology of the biliary tract. An abnormal choledocho- pancreatico ductal junction was observed in these seven patients by preoperative endoscopic retrograde cholangio- pancreaticography (ERCP) or intraoperative cholangio- grams. All six patients examined showed a high amylase level in the choledochal cyst (5,450 to 46,600 Somogyi Units). The intraoperative manometry 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 between pancreatic ductal pressure and common bile duct pressure because of the lack of a sphincter function at the choledochopancre- atico ductal junction. �9 1986 by Grune & Stratton, Inc.

INDEX WORDS: Choledochal cyst; pancreaticobiliary anomalies.

A S THE ETIOLOGY 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 pan- creatic duct. 2' 3.4 To determine the etiology of chole- dochal cysts, however, one must also study the patho- physiology of the biliary tract secondary to an abnormal choledochopancreatico ductal junction.

MATERIALS AND METHODS

From 1962 to 1985, 27 children with congenital choledochal cyst were treated in the Division of Surgery, Children's Research Hospi- tal, 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 ERCP or intraoperative cholangiography, ranged from 1.3 to 2.0 cm.

The manometric studies were performed intraoperatively. Pres- sure recordings were obtained with a polyvinyl catheter with an

internal diameter of 0.8 mm and an outer diameter of 1.0 mm. The probe contained an end hole orifice measuring 0.8 mm in diameter. The probe was filled with sterile saline, and perfusion took place at a constant rate of 30 mL/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 (Nippon- Sanei 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 mm/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/kg) 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 chole- dochopancreatico 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 chole- dochal 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 Univer- sity of Medicine, Japan.

Address reprints requests to Naomi lwai, MD, Division of Surgery, Children's Research Hospital, Kyoto Prefectural Univer- sity of Medicine, Kamigyo-ku, Kyoto, 602, Japan.

�9 1986 by Grune & Stratton, Inc. 0022-3468/2110-0010503.00/0

Journal of Pediatric Surgery, Vol 21, No 10 (October), 1986: pp 873-876 873

874 IWAI El- AL

Table 1. Cases of Choledochal Cyst With Abnormal Choledochopancreatico Ductal Junction

in Which We Performed Intraoperative Biliary Manometry

Form of Patient Choledochal Length of Common Amylase Level

No. Age Sex Symptom Dilatation Channel (cm) in the Cyst

1 12/3 yr 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 14 mo F Fever Cystic 1.4 5,450

5 3 yr F Abdominal pain, vomiting Fusiform 1.5 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

abnormal cho ledocho- pancreatico 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 cmH20 and 35.0 • 6.7 cmH20, respectively. Thus, the pressure difference between the sphincter of Oddi pressure and the duode- nal 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 0 cm

abnormal choledocho- pancreatico ductal Junction

0.3 cm. The choledochopancreatico ductal junction pressure was 12.0 _+ 1.2 cmH20.

Biliary Manometry After Gastrin Stimulation

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 cmH20 before gastrin stimulation, to 46.5 _+ 5.4 cmH20 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 cmH20 and 15.0 + 2.2 cmH20, respectively. These values were

Fig 2. Biliary pressure profile af ter 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 choledochopan- creatico ductal junction.

Table 2. Pressure Measurements of the Biliary Tract Before and After Tetragastrin Stimulation (Mean _+ SE)

Pressure Difference Between Sphincter of

Duodenal Sphincter of Oddi Pressure and Length of Choledochopancreatico Pressure Oddi Pressure Duodenal Pressure High Pressure Ductal Junction Pressure (cmH20) (cmH20) (cmH20) Zone (cm) (cmH20)

Before stimulation (n = 7) 8.0 _+ 2.1 35.0 + 6.7 27.0 _+ 2.7* 1.7 _+ 0.3 12.0 _+ 1.2

After gastrin stimulation (n = 7) 8.5 -+ 2.3 46.5 _+ 5.4 38.0 -+ 3.8* 1.8 _+ 0.1 12.3 _+ 1.3

*P < .05.

BILIARY MANOMETRY IN CHOLEDOCHAL CYST

Table 3. Pressure Measurements of the Biliary Tract Before and After Secretin Stimulation (Mean _+ SE)

875

Pressure Difference Between Sphincter of

Duodenal Sphincter of Oddi Pressure and Length of Choledochopancreatico Pressure Oddi Pressure Duodenal Pressure High Pressure Ouctal Junction Pressure (cmH20) (cmH20) (cmH20) Zone (cm) (cmH20)

Before stimulation (n - 7) 8.0 -+ 2.1 35.0 + 6 .7 * 27.0 _+ 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

*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 choledocho- pancreatico 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 ERCP 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 ~ 2 1 0 cm

1 -T--L L ~ ~ _ 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 st imula-

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 chole- dochopancreatico ductal junction. The action of gas- trin 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 com- mon and pancreatic ducts, which was demonstrated by our pressure measurements.

REFERENCES

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876 IWAI ET AL

3. Jona JD, Babbitt DP, Starshak R J, et al: Anatomic observa- tions 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 cyst- etiological considerations and surgical management in 22 cases. Arch Surg 113:159-163, 1978

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9. Okada A, Ooguchi Y, Kamata S, et al: Anomalous junction of pancreatico-biliary ductal system. Jap J Pediatr Surg 14:53-60, 1980

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