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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 6  |  Issue : 3  |  Page : 152-154

Spontaneous perforation of cystic duct in a 3-month-old Nigerian boy: a case report


1 Department of Paediatrics, Faculty of Clinical Sciences, College of Medical Sciences Department of Surgery, Ahmadu Bello University/Teaching Hospital, Shika, Zaria, Nigeria
2 Division of Paediatrics Surgery, Department of surgery, Faculty of Clinical Sciences, College of Medical Sciences, Ahmadu Bello University/Teaching Hospital, Shika, Zaria, Nigeria

Date of Submission22-Feb-2019
Date of Decision08-Jun-2019
Date of Acceptance13-Oct-2019
Date of Web Publication04-Feb-2020

Correspondence Address:
Dr. Musa S
Department of Paediatrics, Faculty of Clinical Sciences, College of Medical Sciences, Ahmadu Bello University/Teaching Hospital, Shika, Zaria
Nigeria
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DOI: 10.4103/ssajm.ssajm_7_19

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  Abstract 


Introduction: Biliary ascites is a rare condition in infancy and is commonly caused by the perforation of the extrahepatic bile duct. The cystic duct is the part of extrahepatic biliary tract most rarely affected. This is likely to be the first case of biliary ascites of infancy, secondary to spontaneous cystic duct perforation in our centre. Methods: Consent was obtained from the parents of the child. Ethical approval was also obtained from the research and ethics committee of Ahmadu Bello University Teaching Hospital, Shika, Zaria. The case notes of the child were summarized and the relevant literature was reviewed to give the report a context. Results: A three-month-old boy presented with ten days history of jaundice, a week history of abdominal distention, and a day’s history of fever. There was associated bilateral inguinal hernia, excessive cry, refusal to feed, and weight loss. Pregnancy history was normal. He was acutely ill-looking, irritable, febrile with an axillary temperature of 37.7°C, mildly pale, jaundiced with a greenish tinge, had a weight of 4.7kg. He had gross ascites and bilateral reducible inguinal hernia. Bedside abdominal paracentesis reveals thick yellow and foamy fluid. Abdominal ultrasound scan revealed gross ascites, clotting profile was deranged, there was hyperbilirubinaemia of 228 umol/L, other laboratory results including liver function test were normal. He was commenced on medical management for presumed sepsis with obstructive jaundice. Five days into the admission, there was no significant improvement, and the diagnosis was reviewed to a ruptured choledochal cyst and the child was prepared for an explorative laparotomy. Intra-operatively, the cystic duct was perforated. Cholecystectomy and repair of the common bile duct were performed and the child did well postoperatively. Conclusion: Perforated cystic duct should be suspected in infants presenting with jaundice abdominal distension, acholic stools with or without fever, vomiting, and inguinal hernias. Early diagnosis and prompt surgical intervention greatly reduce morbidity and mortality from the disease.

Keywords: Biliary ascites, cystic duct, infants, perforation


How to cite this article:
Musa, OO O, TT S, SM M, HW I, SM A. Spontaneous perforation of cystic duct in a 3-month-old Nigerian boy: a case report. Sub-Saharan Afr J Med 2019;6:152-4

How to cite this URL:
Musa, OO O, TT S, SM M, HW I, SM A. Spontaneous perforation of cystic duct in a 3-month-old Nigerian boy: a case report. Sub-Saharan Afr J Med [serial online] 2019 [cited 2020 Jul 5];6:152-4. Available from: http://www.ssajm.org/text.asp?2019/6/3/152/277784




  Introduction Top


Biliary ascites is a rare condition in infancy and is commonly caused by the perforation of the extrahepatic bile duct.[1] Of the parts of the extrahepatic duct, the cystic duct perforation is the rarest. Since 1932 when the first case of a perforated cystic duct was described, very few cases had been reported in English literature.[2] This is most likely the first case of biliary ascites of infancy, secondary to spontaneous cystic duct perforation in our centre.


  Case summary Top


A three-month-old boy presented with ten days history of jaundice, a week history of abdominal distention, and a day’s history of fever. The jaundice was insidious in onset and progressive associated with a passage of pale stools and deep yellow urine. Three days later, he developed abdominal swelling which was insidious in onset and increasing, associated with bilateral inguinal hernia. Fever started a day before presentation was high grade and continuous. There was history of excessive cry, refusal to feed, and weight loss. However, there was no history of rashes in the mouth, vomiting, or constipation. No bleeding from any orifices. He had neonatal jaundice which started in the first week of life and cleared spontaneously during the second week. No history of blood transfusion or any bloodletting traditional procedure. No history of use of non-orthodox medications, hena, or camphor. Other than a history of fever in the first trimester which necessitated hospital admission, the pregnancy period was uneventful. He was predominantly breastfed, poorly vaccinated, and his family was of the low socio-economic class.

He was acutely ill-looking, irritable, febrile with an axillary temperature of 37.7°C, mildly pale, jaundiced with a greenish tinge, had a weight of 4.7 kg. No dysmorphic features.

He had gross ascites with an abdominal girth of 46 cm, 2 cm above the umbilicus and prominent anterior abdominal wall veins. There was hepatomegaly of 4 cm below the right costal margin. No other Organomegaly. There was a bilateral reducible inguinal hernia. Bedsides abdominal paracentesis reveals thick yellow and foamy fluid.

Laboratory findings included: Sterile Ascitic fluid which showed pus cells on microscopy. Ascitic fluid biochemistry revealed protein—83 mmol/L, albumin—32 mmol/L, and glucose—8.3 mmol/L. An abdominal ultrasound scan revealed gross ascites, other findings were normal. Random blood sugar at presentation was 8.6 mmol/L which returned to normal after normal saline infusion. Clotting profile was deranged. There was mild azotaemia (7.3 mmol/L) and hyponatremia (122 mmol/L). Complete blood count revealed a haemoglobin concentration of 10g/dl, normal white blood cell count with granulocyte predominance and toxic granulations as well as thrombocytosis (625 × 109/L). There was hyperbilirubinemia of 228 umol/L, with a conjugated fraction of 105 umol/L (46%). Urinalysis showed traces of protein and blood, two pluses of bilirubin and ascorbic acid.

He was commenced on medical management for presumed sepsis with obstructive jaundice. The management included antibiotics, Vitamin K, antimalarials, anti-ulcer medicines, a unit of blood transfusion, and maintenance of intravenous fluids.

Five days into the admission, there was no significant improvement and the diagnosis was reviewed to a ruptured choledochal cyst and the child was prepared for an explorative laparotomy.

Intra-operatively, about 700 ml of bilious ascitic fluid was evacuated from the peritoneal cavity. There was a perforation on the anterior aspect of the cystic duct just proximal to the common bile duct. Cholecystectomy and repair of the common bile duct were performed. The child did well postoperatively, the serum bilirubin gradually normalized over a period of one month. He was subsequently discharged to follow-up, a month after the surgery.


  Discussion Top


Biliary ascites in infancy is a rare condition which is usually diagnosed at laparotomy. This is particularly so in resource-limited settings where facilities such as those for laparoscopy and Tc hepatoiminodiacetic acid (HIDA) scan are lacking.[1]

The condition commonly occurs between 1 and 12 weeks of life and has equal sex predilection.[3] It presents acutely or insidiously with ascites, jaundice, and alcoholic stools. The acute form may in addition be present with fever, inguinal hernias or hydroceles, and fulminant biliary peritonitis.[4] This case was acute and presented with worsening abdominal distention, jaundice, fever, vomiting, and bilateral inguinal hernias. These features mimic many other surgical conditions in the age group, and with limited facilities, the diagnosis could only be confirmed intra-operatively as with some previously reported cases.[5] The serum bilirubin in this case (228 umol/L, conjugated-105 umol/L) was higher than the commonly cited values (135–144 umol/L).[6] The liver enzymes were within normal limits as in most previously reported cases.[7]

The exact cause of spontaneous perforation of the cystic duct is unknown, however, associated conditions include congenital ductal stenosis, biliary stones, congenital weakness, and ischemia.[8],[9],[10] The most commonly cited predisposing factor is a localised embryogenic mural malformation of the common-cystic duct. The localised malformations lead to cystic duct perforation, while diffusedmalformation results in a choledochal cyst.[11] In resource-constraint settings, it is difficult to make a definitive diagnosis before surgery. However, with the classical symptoms, normal liver function, abdominal ultrasound, and Tc hepatoiminodiacetic acid (HIDA) scan, the diagnosis could be made before surgery. [5]

The definitive treatment is surgery as soon as the diagnosis is made during which the patency of the distal bile duct must be confirmed to exclude distal obstruction.[12] The outcome was very good as in some previously reported cases.[6],[11],[13] In a minority of cases, however, early complications such as portal vein thrombosis have been reported. Further, long-term complications such as hepatic cirrhosis could occur.[14]


  Conclusion Top


A perforated cystic duct should be suspected in infants presenting with jaundice abdominal distension, acholic stools with or without fever, vomiting, and inguinal hernias. HIDA scan, abdominal ultrasound, and liver function test could be used to diagnose the condition pre-operatively.

However, in resource-poor settings, explorative laparotomy should usually be undertaken to diagnose and treat the condition. This should be carried out as soon as possible, once the condition is suspected. Awareness of the condition amongst clinicians will lead to early diagnosis and prompt surgical intervention which in turn improve the outcome of the disease.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sharma SB, Sharma SC, Gupta V. Spontaneousbiliary perforation: a rare entity in late infancy and childhood. Indian J Pediatr 2003;70:829-31  Back to cited text no. 1
    
2.
Hammer B, Sander E. Spontaneous biliary peritonitis in infancy. J Pediatr Surg 1968;3:84-8.  Back to cited text no. 2
    
3.
Ando K, Miyano T, Kohno S, Takamizawa S, Lane G. Spontaneous perforation of choledochal cyst: A studyof 13 cases. Eur J Pediatr Surg 1998;8:23-5.  Back to cited text no. 3
    
4.
Kanojia RP, Sinha SK, Rawat J, Wakhlu A, Kureel S, Tandon R. Spontaneous biliary perforation in infancy and childhood: clues to diagnosis. Indian J Pediatr 2007;74:509-10.  Back to cited text no. 4
    
5.
Sahnoun L, Belghith M, Jallouli M, Maazoun K, Mekki M, Ben Brahim M et al. Spontaneous perforation of the extra hepatic bile duct in infancy: report of two cases and literature review. Eur J Pediatr 2007;166:173-5.  Back to cited text no. 5
    
6.
Banani SA, Bahador A, Nezakatgoo N. Idiopathic perforation of the extrahepatic bile duct in infancy: pathogenesis, diagnosis and management. J Pediatr Surg 1993;28:950-2.  Back to cited text no. 6
    
7.
Stringal G, Mercer S. Idiopathic perforation of the biliary tract in infancy. J Pediatr 1983;18:546-50.  Back to cited text no. 7
    
8.
Donohoe PK, Hendren WH. Bile duct perforation in a newborn with stenosis of the ampulla of Vater. J PediatrSurg 1976;11:823-5.  Back to cited text no. 8
    
9.
Colver HD. Perforation of biliary tract due to gallstones in infancy. Am J Surg 1964;160:226-31.  Back to cited text no. 9
    
10.
Northover JMA, Terblanche J. A new look at the arterial supply of the bile duct in man and its surgical implications. Br J Surg 1979;66:379-84.  Back to cited text no. 10
    
11.
Lily JR, Weintraub WH, Altman PR. Spontaneous perforation of the extrahepatic bile ducts and bile peritonitis in infancy. Surgery 1974;75:664-73.  Back to cited text no. 11
    
12.
Lees W, Mitchell JE. Bile peritonitis in infancy. Arch Dis Child 1966;41:188-92.  Back to cited text no. 12
    
13.
Prevat J, Babut J. Spontaneous perforation of biliary tract in infancy. Progr Pediatr Surg 1971;3:187-9.  Back to cited text no. 13
    
14.
Chardot C, Iskandarani F, DeDreuzy O, Duquesne B et al. Spontaneous perforation of biliary tract in infancy: a series of 11 cases. Eur J Pediatr Surg 1996;6:341-46.  Back to cited text no. 14
    




 

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