Saudi Journal of Gastroenterology
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Year : 1997  |  Volume : 3  |  Issue : 2  |  Page : 78-83
Hepatobiliary scintigraphy in the diagnosis of choledochal cysts in children


1 Department of Nuclear Medicine, King Khalid University Hospital, Riyadh, Saudi Arabia
2 Department of Pediatric Surgery, King Khalid University Hospital, Riyadh, Saudi Arabia
3 Department of Pediatrics, King Khalid University Hospital, Riyadh, Saudi Arabia
4 Department of Pediatric Surgery, Security Forces Hospital, Riyadh, Saudi Arabia
5 Department of Radiology, King Khalid University Hospital, Riyadh, Saudi Arabia

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Date of Submission11-Nov-1996
Date of Acceptance10-Apr-1997
 

   Abstract 

The objective is to present the usefulness of hepatobiliary scintigraphy in the investigation of children with suspected choledochal cysts through our experience in King Khalid University Hospital at King Saud University, Riyadh. Seven patients aged between I and 10 years (average 4.8 yrs) comprising six females and one male were investigated. Laboratory tests, abdominal Ultrasound and/or CT, and cholangiography were performed whenever indicated. Persistent activity in a dilated common bile duct, with or without dilatation of intrahepatic bile ducts, was considered a positive indicator for choledochal cyst disease. Four children with cystic dilatation (type I) were diagnosed by hepatobiliary scintigraphy, one saccular (type II), and two cases of Caroli's disease (type V). The diagnosis of choledochal cyst was proven by surgery with histological confirmation. Visualization of the gallbladder occurred in one case only. The common bile duct was seen in four cases. Late activity in the bowel was noted in two cases.

How to cite this article:
El Desouki M, Mohamadiyeh M, Al Rabeaah A, Othman S, Al Jurayyan N, Asaad A, Skiff Z, Malabarey T, Al Samarrai A. Hepatobiliary scintigraphy in the diagnosis of choledochal cysts in children. Saudi J Gastroenterol 1997;3:78-83

How to cite this URL:
El Desouki M, Mohamadiyeh M, Al Rabeaah A, Othman S, Al Jurayyan N, Asaad A, Skiff Z, Malabarey T, Al Samarrai A. Hepatobiliary scintigraphy in the diagnosis of choledochal cysts in children. Saudi J Gastroenterol [serial online] 1997 [cited 2019 Oct 23];3:78-83. Available from: http://www.saudijgastro.com/text.asp?1997/3/2/78/33930


Choledochal cyst is a clinical entity with low incidence and prevalence rates [1] . It was first reported in 1852 long after its pathology was described by Vatero and Ezler in 1723 [2],[3] . It is more commonly seen in females than in males and usually in the first two decades of life [4],[5],[6],[7],[8] . It has also been diagnosed antenatally [9],[10],[11],[12] . There is prediliction for Orientals, mostly Japanese [4],[13] . The typical presentation of the disease has been a child with the triad of jaundice, abdominal pain and palpable mass. The currently accepted classification of choledochal cyst is that introduced by Alonso-Lej et al., [14] which is a modification of the original work by Todani et al [15] . However, the most frequent types seen in practice are types I and IV [7],[15],[16],[17] . Choledochal cyst can also present in adults [16] . The diagnosis of choledochal cyst is made by explorative cholangiogram. Hepatobiliary scintigraphy can uniquely demonstrate the relation of the cyst to the biliary system. In this work we present our experience at the King Khalid University Hospital, Riyadh, investigating children with suspected choledochal cysts by hepatobiliary scintigraphy using a Tc-99m-iminodiacetic acid derivative, characterizing the findings on CT and ultrasound.


   Patients and methods Top


The medical records of all children with the final diagnosis of choledochal cyst between 1986 and 1995 were reviewed. Seven patients between the ages of 1 and 10 years (average 4.8 yrs.) were diagnosed during this period. There were six females and one male, six of the patients were Saudis. None of the children presented with the triad of abdominal pain, right upper quadrant mass and jaundice; classical of choledocal cyst disease. Five children had two of the triad symptoms and two had only jaundice. Jaundice was the most frequent presenting symptom and, thus, all our patients have had phenobarbital (5mg/kg/day) administered to them.


   Hepatobiliary scintigraphy Top


Seventy-four to 185 MBq (2-5 mCi) of Tc-99m-Dis­isopropyl (Disofenin) were administered intravenoulsly to the patients after 3-4 hours of fasting. Imaging of the abdomen started immediately after injection with flow (16 frame / 2 sec each) on a 64 matrix. This was followed by blood pool and sequential static images of 3-5 minutes each for 60 minutes in the anterior projection until visualization of gallbladder and /or bile ducts was observed. Other views in the right anterior oblique, right lateral, and posterior projections were then taken. If a photopoenic area over the liver or below it was identified or a filling-in pattern was observed, delayed images of the abdomen were obtained at 20­24 hours post injection.

The scintigraphic images were reinterpreted by three nuclear medicine physicians (MD, MM, SO) and the visualization time of the lesion was recorded as well as the shape of it for classification purposes. Conclusions were reached by consensus or by the most experienced physician.


   Diagnostic laboratory tests Top


Serial liver function tests were routinely done including total bilirubin with the percentage of direct fraction and serum amylase determined as well.


   Ultrasound and computerized tomography Top


All patients underwent abdominal ultrasound and/or CT. Ultrasound images were obtained using a variety of real-time ultrasound machines with 5.0­7.5 MHz sector transducers. All studies were performed after an adequate time of fasting (in relation to expected time of feeding). Diagnostic evaluation included size, shape, and wall thickness of the gallbladder and dilatation of the intra- and extrahepatic biliary trees.


   Cholangiogram Top


Percutaneous or intraoperative cholangiography was performed as the definitive diagnostic test. The lesion, gallbladder and both exta- and intrahepatic bile ducts were evaluated. Except for the hepatobiliary scintigraphy, all the diagnostic tests, imaging and nonimaging, were reviewed but not specifically analyzed in this report.


   Results Top


The diagnosis of choledochal cyst by hepatobiliary scintigraphy was proven by surgery with histological confirmation of the cystic lesion. [Figure - 1] shows all types of choledochal cysts.


   Hepatobiliary scintigraphy Top


Four choledochal cysts were cystic dilatation (Type I) [Figure - 2]A, B, and C and [Figure - 3], one was saccular (Type II) [Figure - 4], and two were cases of Caroli's Disease (Type V) [Figure - 5]. [Table - 1] summerizes the types of the choledochal cysts as found on HBS. The most frequent type is the cystic dilatation (57%). The time of visualization of the choledochal cysts was variable since some of them were visualized within the first 24 hours [Figure - 2] whereas another one was visualized earlier at 4h p.i [Figure - 3]. Three cysts showed immediate activity that increased gradually over time [Figure - 3]. One cyst showed initial photopoenia that filled in subsequently [Figure - 2]. The gallbladder was visualized in one case only at 6h p.i. The common bile duct was seen in four cases. Activity in the bowel was seen in two cases after 12h p.i. [Table - 2] summerizes all the results of the HBS.

Ultrasonography and Computerized Tomography and Cholangiography detected all cysts and dilatation of the bile ducts.


   Discussion Top


Choledochal cyst (CC) is an uncommon clinical entity with a reported incidence between one in 13,000 and one in two million [1] . The first clinical description of CC was reported by Douglas in 1852 [2] . The first surgical excision was performed by Worter in 1924 [18] . Females are more commonly affected than males [4],[5],[6],[7],[8] . Our patients match the ones reported in the literature in age and sex.

The currently accepted classification of CC is that introduced by Alonso-Lej et al., [14] which is a modification to the original work by Todani et al [15] [Figure - 1]. However, the most frequent types seen in practice are type I and IV [7],[15],[16],[17] . Four of our cases were type I, representing cystic dilatation of the common bile duct. The characteristics of the CC in our patients were consistent with those reported in the literature [17] . None of our patients had a high level of serum amylase or symptoms suggesting acute pancreatitis. The most frequent presentation of our patients was that of jaundice with abdonminal mass and none had the classical triad of CC.

The etiology of CC is still largely unknown. It has been suggested that embryologic biliary obstruction and subsequent common bile duct wall weakening might be the underlying causative pathology [19],[20] . Definitive diagnosis of CC is based on quite invasive procedures such as percutaneous or intraoperative cholangiography. The use of US and CT has helped in decreasing the frequency of invasive approaches to finalize the diagnostic process. Advances in the use of US has also led to the diagnosis of CC antenatally [9],[10],[11],[12] . However, the option of complete reliance on morphology-based diagnostic technology is not free of difficult situations. These tests may not be able to differentiate CC from other benign cysts (i.e. hepatic or pancreatic cysts) [7] . Some CC can be missed by US because of the technical quality of the test or failure to recognize unusual pathology and thus US may underestimate this diagnosis [16] . Characterization of their findings with a physiologic approach may be needed and can be provided by hepatobiliary scintigraphy (HBS). Taplin GY et al. [21] introduced rose bengal labeled with 131-Iodine in 1955. This halogenated fluoroscein radioactive tracer has high hepatobiliary specificity but the high energy and the 8.1-day physical half-life of 131-­Iodine restricted the dose to a very small range. This yielded poor quality images and limited the clinical utility of this radioactive tracer. In 1975, Harvey E et al. [22] introduced the first generation of 99m-Tc­-Dimethyliminodiacetic acid (HIDA). It is an organic anion that is loosely bound to protein in the blood and may be displaced by bilirubin since both are handeled in the liver by the same mechanism. Other HIDA derivatives have been introduced. Of all the HIDA agents currently availbale, 99m-Tc-DISIDA (disofenin) appears to be the superior when the bilirubin level is between 15 and 30 mg/100 ml [23],[24]. Every diagnostic technology has limitations and HBS is no different. What appears to be a limitation in one technique can become an advantage to the other. In the diagnosis of CC, HBS may fail to visualize one cyst, evaluate intrahepatic bile ducts for possible association, or provide clear anatomic details. Nonetheless, HBS certainly complements the other diagnostic tests in the diagnosis of CC disease.


   Conclusion Top


Hepatobiliary scintigraphy is a valuable and quite useful diagnostic test as part of the work up plan for investigating children with choledochal cysts, mainly to characterize findings on computerized tomography and ultrasound in these children.

 
   References Top

1.Olbume NA. Choledochal cysts: A review of the cystic anomalies of the biliary tree. Ann R Coll Surg Engl 1975; 56:26-32.  Back to cited text no. 1    
2.Douglas AH. Case of dilation of the common bile duct. Monthly J Med Sci 1852; 14:97.  Back to cited text no. 2    
3.Vater A, Ezler CS. Dissertatio de scirrhis viserum occasione sections viri typanite defunte. Wittenburgae 1723, 4 Pamphlers 881, p22.  Back to cited text no. 3    
4.Chen WJ, Chang CH, Hung WT. Congenital choledochal cyst with observations on rupture of the cysts and intrahepatic ductal dilatation. J Pediatr Surg 1973; 8:529-38.  Back to cited text no. 4    
5.Barlow B, Tabor E, Blanc WA, et al. Choledochal cyst: a review of 19 cases. J Pediatr 1976; 89:934-40.  Back to cited text no. 5  [PUBMED]  
6.Bass EM, Cremin BJ. Choledochal cysts: A clinical and radiological evaluation of 21 cases. Pediatr Radiol 1976; 5:81-5.  Back to cited text no. 6  [PUBMED]  
7.Camponovo E, Buck JL, Drane WE. Scintigraphic features of choledochal cyst. J Nucl Med 1989; 30:622-8.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Park CH, Garafola JH, O'Hara AE. Preoperative diagnosis of asymptomatic choledochal cyst by rose bengal liver scan. J Nucl Med 1973; 15:310-11.  Back to cited text no. 8    
9.Lugo-Vicente HR. Prenatally diagnosed choledochal cysts: Observation or early surgery ? J Pediatr Surg 1955; 30:1288-­90.  Back to cited text no. 9    
10.Bancroft JD, Bucuvalas JC, Ryckman FC, Dudgeon DL, Saunders RC, Schwarz KB. Antenatal diagnosis of choledochal cyst. J Pediatr Gastroenterol Nutr 1994; 18:142-5.  Back to cited text no. 10  [PUBMED]  
11.Tsuchida Y, Kawarasaki H, Iwanaka T, Uchida H, Nakanishi H, Uno K. Antenatal diagnosis of biliary atresia type I cyst at 19 weeks' gestation: Differential diagnosis and etiologic implications. J Pediatr Surg 1995; 30:697-9.  Back to cited text no. 11  [PUBMED]  [FULLTEXT]
12.Wiedman MA, Tan A. Martinez CJ. Fetal sonography and neonatal scintigraphy of a choledochal cyst. J Nucl Med 1985; 26:893-6.  Back to cited text no. 12    
13.Miyano T, Suruga K, Chen SC. A clinicopathologic study of choledochal cyst. World J Surg 1980; 4:231-8.  Back to cited text no. 13  [PUBMED]  
14.Alonso-Lej F, Revers WB, Pessagno DJ. Congenital choledochal cyst with a report of two, and an analysis of 94 cases. Int Abst Surg 1959; 108:1.  Back to cited text no. 14    
15.Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: classification, operative procedures, and review of 37 cases including cancer arising from choledochal cyst. Am J Surg 1977; 134:263-9.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]
16.Lipsett PA, Pitt HA, Colombani PM, Boitnott JK, Cameron JL. Choledochal cyst disease: a changing pattern of presenttion. Ann Surg 1994; 220:644-52.  Back to cited text no. 16  [PUBMED]  [FULLTEXT]
17.Todani T, Urushihara N, Morotomi Y, et al. Characteristics of choledochal cysts in neonates and early infants. Eur J Pediatr Surg 1995; 5:143-5.  Back to cited text no. 17  [PUBMED]  
18.Worter GL. Congenital cystic dilatation of the common bile duct. Ann Surg 1924; 8:604-26.  Back to cited text no. 18    
19.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.  Back to cited text no. 19  [PUBMED]  [FULLTEXT]
20.Wong KC, Lister J. Human fetal development of the hepato­pancreatic duct junction: a possible explanation of congenital dilatation of the bilairy tract. J Pediatr Surg 1981; 16:139-45.  Back to cited text no. 20  [PUBMED]  [FULLTEXT]
21.Taplin GY, Meredith OM, Kade H. The radioactive (131-I-tagged) rose bengal uptake: Excretion test for liver function using external gama ray scintillation counting techniques. J Lab Med 1955; 45:665.  Back to cited text no. 21    
22.Harvey E, Loberg M, Cooper M. 99m-Tc-HIDA-A new radiopharmaceutical for hepatobiliary imaging (abstract). J Nucl Med 1975; 16:533.  Back to cited text no. 22    
23.Hernandez M, Rosenthall L. A cross-over study comparing the kinetics of 99m-Tc-labeled diisopropyl and p-butyl IDA analogues in patients. Clin Nucl Med 1980; 5:159.  Back to cited text no. 23  [PUBMED]  
24.Hernandez M, Rosenthall L. A cross-over study comparing the kinetics of 99m-Tc-labeled diethyl- and diisopropyl-­IDA analogues in patients. Clin Nucl Med 1980: 5:352.  Back to cited text no. 24    

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Correspondence Address:
Mahmoud El Desouki
Associate Professor and Head of Nuclear Medicine, King Khalid University Hospital, P.O. Box 7805, Riyadh 11472
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


PMID: 19864798

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