|Year : 2013 | Volume
| Issue : 1 | Page : 33-36
Ultrasound diagnosis of a delayed case of choledochocele with acalculous cholecystitis
Suleiman Idris Kazaure1, Anas Ismail1, Abdurrahman Abba Sheshe2
1 Department of Radiology, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria
2 Department of Surgery, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria
|Date of Web Publication||29-Aug-2013|
Suleiman Idris Kazaure
Department of Radiology, Bayero University/Aminu Kano Teaching Hospital, Kano
Source of Support: None, Conflict of Interest: None
Choledochocele is an uncommon malformation of the external billiary ducts that usually presents early in life. However, these are increasingly diagnosed in the adult population. This patient presented was a 27-year-old male with upper abdominal symptoms first diagnosed on ultrasonography. The value of ultrasound in both diagnosis, detection of complications, and management is highlighted in the review.
Keywords: Cholecystitis, choledochocele, ultrasound
|How to cite this article:|
Kazaure SI, Ismail A, Sheshe AA. Ultrasound diagnosis of a delayed case of choledochocele with acalculous cholecystitis. Niger J Basic Clin Sci 2013;10:33-6
|How to cite this URL:|
Kazaure SI, Ismail A, Sheshe AA. Ultrasound diagnosis of a delayed case of choledochocele with acalculous cholecystitis. Niger J Basic Clin Sci [serial online] 2013 [cited 2021 Dec 2];10:33-6. Available from: https://www.njbcs.net/text.asp?2013/10/1/33/117244
| Introduction|| |
Choledochocele is a cystic dilatation of the distal/intramural duodenal portion of the common bile duct (CBD) with herniation of CBD into duodenum.  It usually arise from a diverticulum of distal CBD with stenosis of ductal orifice or weakness of ductal wall.  Choledochoceles are a rare forms of choledochal cysts.  It commonly presents with cholecystitis, however, it may remain asymptomatic until adulthood. 
Choledochocoele is uncommon among adults compared with children, and are thought to be congenital in etiology.  Clinically, it presents with a triad of abdominal pain, jaundice, and abdominal mass. However, it could be complicated by cholangitis, pancreatitis, and cholecystitis.  Duodenal obstruction may present with severe nonbilious vomiting and electrolyte derangements.  The diagnosis is often clinical, but imaging is required for confirmation and investigation of associated anomalies. This involves the use of ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI) endoscopic retrograde cholangiography (ERCP), and sometimes, contrast studies. , Aside diagnostic purposes, imaging may guide management decision and the evaluation of success or complications of surgical treatments. Treatment options are becoming refined with advances in endoscopic procedures via sphincterotomy and sphincteroplasty. We present an unusual case of an adult male patient diagnosed with choledochocele on ultrasonography.
| Case Report|| |
The patient was a 27-year-old male who presented with a 3-day history of dull right upper quadrant abdominal pain, fever, and jaundice. The abdominal pain was intermittent and associated with occasional nonbilious vomiting. No abdominal distension or change in bowel habit. There were no constitutional symptoms, abdominal distension, or itching. The abdominal symptoms were intermittent for about 7 years prior to presentation but were never severe enough to prevent him from normal activities. There was no history of alcohol consumption, hepato-billiary surgery, trauma to the upper abdomen, or use of traditional medications.
On examination, he was febrile (39°C), not pale, jaundiced, and acyanosed. There was no peripheral adenopathy or limb edema. The abdomen was full, moving with respiration. A 3 × 4 cm mildly tender swelling was felt over the right upper quadrant. It was difficult to palpate above the mass but was palpable from below. The liver and other organs were not palpably enlarged. The bowel sounds were normal.
The Results of complete blood count showed evidence of neutrophil leucocytosis. No malaria parasite was seen on blood film. The clotting profile was normal. The liver function showed mild elevation of unconjugated serum bilirubin. The serum electrolytes were within normal limits.
Abdominal ultrasound scan revealed a well defined sonolucent oval shaped mass lesion in the region of porta-hepatis that is separate from the gall bladder. This mass appeared to at the distal end of the CBD, which was moderately dilated up to 7.3 mm in diameter [Figure 1]a and b. This mass measures about 71 × 59 mm is size and demonstrates absent color flow with color Doppler interrogation [Figure 1]c. The outlined gall bladder is grossly distended and shows thickened mucosa, up to 6.21 mm with a double wall sign due to mural edema. However, no calculus was seen within it. The liver, intrahepatic biliary and vascular channels as well as the pancreas are within normal limits. The spleen, kidneys, urinary bladder, and prostate gland also appeared sonographically normal. There was no ascites. In view of these findings, sonographic diagnosis of choledochocele with acalculous cholecystitis was made. He was advised to do abdominal MRI or CT-cholangiogram scan to further delineate the mass.
The patient was admitted and commenced on intravenous antibiotics, fluids, and analgesics. He had a course of vitamin K injection for 5 days. He did well and was discharged on the sixth day of admission to enable him source funds for further investigations. Three weeks later, he was readmitted with worsening fever and abdominal pain. Repeat abdominal ultrasound examination revealed same findings as earlier documented in addition to mobile specks within the gall bladder. Following parenteral antibiotics, rehydration, and preliminary laboratory investigations, surgical option was considered.
He had an exploratory laparotomy; a cystic mass was found adherent to the duodenum with surrounding fibrosis and peripyloric lymphadenopathy. The gallbladder and CBD were both distended. A Roux-n-Y choledochojejunostomy, with cholecystectomy and partial excision of the choledochocele and mucosal stripping of the remaining adherent portion was done.  The patient did well postoperatively and was discharged 10 days later.
| Discussion|| |
Congenital cystic malformations of the biliary system are uncommon but increasingly diagnosed in adult population. ,, They have a female:male preponderance of 4:1, with an incidence in the West between 1 in 50,000 and 1 in 200,000 live births but are much more frequent in Asia.  Nearly 25% of choledochal cysts are detected in the first year of life and 60% in the first decade, but 20% are diagnosed after the age of 20 years.  This case is a male patient, presenting at 27 years; with intermittent subtle symptoms for the past 7 years. Based on the location of the cytis segment of the biliary system, congenital cystic diseases of the biliary system were classified as follows: 
- Type I cysts are the most common type, representing 80-90% of all lesions, and are characterized by dilatation of the entire common hepatic and CBDs, or of segments of each; subtypes a, b, and c represent differences in cystic appearance, such as saccular or fusiform.
- Type II cysts are characterized by isolated protrusions or diverticula projecting from the CBD wall; they may be further classified into sessile or pedunculated subtypes.
- Type III cysts, also known as choledochoceles, are characterized by existence of the cystic component within the intraduodenal portion of the CBD. Choledochoceles are further classified as type IIIa (CBD terminating in to a cyst, which drained in to the duodenum) and type IIIb (cyst draining in to the adjacent intramural portion of CBD).
- Type IV cysts are characterized by multiple dilatations of the intrahepatic and extra hepatic biliary tree, with type IVA involving both intrahepatic and extra hepatic dilations and type IVB involving only extra hepatic dilations.
- Type V cysts, otherwise known as Caroli Disease, are characterized by intrahepatic biliary dilation, without extra hepatic involvement. ,,,
This case presented with type-IIIa disease.
As mentioned previously, patients typically present with triad of abdominal pain, jaundice, and palpable abdominal mass. ,,,, However, these are seen in about 40% of cases.  This is partially responsible for delay in the diagnosis, as seen in this case.
Though choledochocoele can be diagnosed on ultrasonography as done in this case, some form of cholangiography, like ERCP or MR-cholangiography are the gold standard investigations prior to surgery to define the exact extent and configuration of the cyst and to detect any evidence of coexisting choledocholithiasis, stricture, or mass. The latter may suggest the development of malignancy. Cholangiography could also demonstrate the relation of the distal bile duct to the pancreatic duct and identify any anomalous pancreaticobiliary duct junction. ,, Nevertheless, ultrasound and CT, together with good clinical acumen, may help to clarify related lesions and complication. 
Complications of choledochal cysts are the result of stasis and may include cholangitis, stone formation, recurrent pancreatitis, persisting pressure by a stone may lead to choledocho-duodenal fistula. ,,,, Although most of these changes were not seen in the presented case, it showed sonographic features of cholecystitis. In contrast, cholangiocarcinoma is a dreaded complication and may result due to chronic mucosal irritation; it has an incidence of 10-30%.  Another rare complication (though not seen in this case) is portal hypertension. This is a rare complication of long-standing choledochal cyst.  Furthermore, surgery in the presence of portal hypertension is difficult due to the vascular collaterals in the region of the porta hepatis and is associated with significant morbidity and mortality. 
The presence of complications contributes to significant morbidity and mortality, and to a large extent determines the modality of treatment in addition to other factors. This in part, makes the endoscopic surgical excision the most appropriate treatment option. However, due limited investigations an open exploration was considered the most appropriate in this case.
| Conclusions|| |
Abdominal ultrasound is an important investigative armamentarium in the management of patients with suspected hepatobilliary or upper abdominal complains. Choledochocele is a rare defect that needs careful imaging to be diagnosed.
A 27-year-old male presented with recurrent episodes of upper abdominal pain, jaundice, and fever. He was found to have a palpable right upper abdominal mass. Abdominal ultrasound scan revealed a well defined sonolucent diverticulum that communicates with the CBD and duodenum. At surgery, choledochocele was confirmed and excised. The patient had 3 months of uncomplicated follow up. The relevant literature was reviewed.
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