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Year : 2020  |  Volume : 8  |  Issue : 3  |  Page : 156-160

Management of congenital giant liver cyst with postoperative bile leak: A case report and literature review

1 Department of Academic Clinical, College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Jeddah, Makkah, Saudi Arabia
2 Department of Surgery, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia
3 Department of Academic Clinical, College of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
4 Department of Surgery, Maternity and Children Hospital, Makkah, Saudi Arabia

Date of Submission06-Mar-2021
Date of Acceptance22-Jun-2021
Date of Web Publication19-Jul-2021

Correspondence Address:
Dr. Abdullah Osama Bawazir
King Saud bin Abdulaziz University for Health Sciences, College of Medicine, P.O. Box 14, Jeddah 23544, Makkah
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ssj.ssj_65_21

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Congenital liver cyst is an uncommon disease in infancy and is usually asymptomatic. However, even rarer are congenital giant liver cysts which by virtue of their size are problematic. We discuss a case of a newborn with a giant hepatic cyst who was successfully treated surgically but experienced a postoperative bile leak as a complication. We review the management of this entity and propose an algorithm of care.

Keywords: Giant hepatic cyst in pediatric, giant hepatic cyst, management of giant hepatic cyst

How to cite this article:
Bawazir AO, Mahomed A, Kausar N, Bawazir R, Halabi R, Mamoun I, Maimani M. Management of congenital giant liver cyst with postoperative bile leak: A case report and literature review. Saudi Surg J 2020;8:156-60

How to cite this URL:
Bawazir AO, Mahomed A, Kausar N, Bawazir R, Halabi R, Mamoun I, Maimani M. Management of congenital giant liver cyst with postoperative bile leak: A case report and literature review. Saudi Surg J [serial online] 2020 [cited 2023 Feb 6];8:156-60. Available from: https://www.saudisurgj.org/text.asp?2020/8/3/156/321730

  Introduction Top

The congenital liver cyst (CLC) is an infrequent disease in childhood and is principally asymptomatic.[1],[2] The detection of these cysts may be coincidental during routine radiological imaging.[1] Most will be small in size, but a few are massive or will increase in size and might manifest as an abdominal mass.[3] Surgical intervention is sometimes necessary for these cysts.[3] A consequence might be postoperative bile leakage which may lead to cholangitis and intra-abdominal abscess.[2] Immediate treatment of these leaks with percutaneous transhepatic cholangiography (PTC) and external drainage will avert these complications.[4]

  Case Report Top

A 3-month-old girl was referred to our hospital for the management of a giant hepatic cyst initially diagnosed on fetal ultrasonography at 20 weeks of gestation. Abdominal examination at 1 month of age revealed a large hepatic mass with generalized distention [Figure 1]. Investigations which included an ultrasound and magnetic resonance imaging (MRI) scan revealed a solitary cystic lesion in the liver, measuring 11.9 cm × 9 cm × 7.1 cm. It was localized in the anterior right hepatic lobe displacing the rest of the hepatic lobes, including segment VI. There was no sign of biliary duct or gall bladder dilation.
Figure 1: One-month baby with massive abdominal distention

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However, the symptoms of early satiety and occasional respiratory distress appeared at 2 months of age. Laboratory investigations confirmed liver function tests, clotting, and biochemical profile to be within normal range. Tumor markers (AFP 989, CA19-9 10, and human chorionic gonadotropin <2) were also within the normal range. However, repeat MRI and computed tomography at this point confirmed that the cyst had expanded and measured 14.3 cm × 8.9 cm × 11.0 cm [Figure 2].
Figure 2: One-month baby with massive abdominal distention

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The infant was taken to the surgery which was performed through a right subcostal incision. A large cyst replacing all the right hepatic lobe was confirmed with gall bladder stretched over the wall. The solitary, unilocular cyst was drained comprehensively of a lightly bile stained liquid. Cytology, culture, and chemistry of the fluid did not yield much to aid in the care of this patient. About 60%–70% of the cystic wall was then removed using a LigaSure device. The gall bladder was excised as part of the deroofing, preserving the rest of the extrahepatic biliary tree which was visualised. An intraoperative cholangiogram was consequently not performed. A suction drain was inserted to the right upper quadrant, in relation to the dissection, at the end of the procedure.

The patient made a rapid recovery postoperatively but continued to drain between 60 and 100 ml of bile daily from the subhepatic drain [Figure 3]. A PTC was requested 3 weeks later and showed the filling of several liver cysts connected to the biliary tract [Figure 4]. A pig-tail catheter was placed in the cyst in the same sitting. Drainage from the subcostal drain ceased immediately, and both the pig tail and subcostal drains were removed 3 weeks later. The patient was discharged clinically well.
Figure 3: Postoperative magnetic resonance imaging demonstrating a residual cyst with a bile leak

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Figure 4: (a-d) Percutaneous transhepatic cholangiography shows multiple cystic intrahepatic dilatations communicating with the extrahepatic biliary tract which drains into the duodenum

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Histopathology of the wall showed the features suggestive of a benign congenital hepatic cyst. Some limited fibrosis was noted in the adjacent liver parenchyma, and the gall bladder itself was unremarkable.

At follow-up 2 years later, the patient remains very well, gaining in weight and free of jaundice. Serial ultrasound scans demonstrate no cyst recurrence.

  Discussion Top

CLC does not have a standardized nomenclature. Various labels including congenital solitary nonparasitic cyst of the liver, congenital solitary liver cyst, or simple liver cyst, have been used to describe the same entity.[1] CLC is rare at birth with a reported incidence of 2.5%.[2] Most of the patients are female and may have associated conditions such as type 1 diabetes, cardiac myoma, and sickle cell anemia appearing later in life.[5] These cysts commonly affect the right lobe of the liver.[6]

As a rule, CLC do not cause any manifestations after birth unless it expands dramatically. In this event, feeding difficulties, respiratory distress, duodenal impediment, and abdominal distension, might arise.[2],[7]] These symptoms occur from the secondary compressive effects of a large cyst on surrounding structurers. Furthermore, described is postnatal cholestasis as a result of pressure on the hepatic parenchyma and biliary system.[2] The cyst may also appear as a visible and palpable mass.[8]

Most of these congenital cysts are diagnosed during routine prenatal ultrasonography.[5] Characteristically, these appear as simple, single, anechoic cyst filled with fluid, and showing a posterior enhancement.[2] A study has suggested that an MRI should be performed postnatally to rule out biliary tree involvement. Furthermore, it also suggests that if the cyst decreases or remains stable in size then a yearly ultrasound is performed until the age of three. However, if the cyst expands or changes its configuration then a repeat of axial imaging should be performed as a prelude to intervention.[6]

The differential diagnosis includes solitary hepatic and choledochal cyst both of which may have bile content indicating a connection to the biliary tree.[3],[9] Choledochal cysts (type I-IV) are cystic abnormalities that arise form extrahepatic bile ducts as a result of pancreaticobiliary abnormalities.[10] Caroli disease, which is the part of the spectrum of choledochal cysts (type V), is a cystic lesion that arise from ductal plate abnormalities within a large intrahepatic duct.[10] It appears as several cystic dilations of the intrahepatic ducts, and it is usually confined to one lobe of the liver.[10] A radioisotope scan maybe useful in distinguishing between a simple and a choledochal cyst.[8] The possibility of the presented case being a variant of Caroli's disease has been raised.

In CLC, malignant transformation is exceptionally infrequent. Only a cyst >12 cm has been identified as a risk factor for malignancy.[2] Dysplastic alterations in the epithelial lining on histology might indicate a greater risk of malignant transformation, and therefore, an intraoperative frozen section of the cystic wall, as was our practice with this case, should be done.[9] Malignant transformations are usually into squamous cell or adenocarcinoma. This likelihood, although rare, supports the necessity of follow-up and where possible, a comprehensive removal of the hepatic cyst.[3]

Indicated for surgery includes severe respiratory or abdominal symptoms from pressure effects or giant cysts where malignant transformation later in life, poses a risk. The options for the management include internal drainage, enucleation, laparoscopic fenestration, and partial or complete resection.[10] A high recurrence rate has been associated with exclusively an ultrasound-guided percutaneous drainage of a cyst.[3] Total excision of the cystic epithelia lining along with a little margin of the liver tissue results in complete cure of the cyst.[9] Incomplete resection of the cyst with even a little epithelial remnants can cause a relapse.[10] Open surgery is preferred for giant cysts, but laparoscopic intervention may be an option for certain parts of the liver, specifically the anterior and lateral segments.[9] Liver cyst resection in new-borns is efficient and safe and associated with lower surgical and postsurgical complication.[7] Extensive resection generally has a high morbidity from bile leak and hemorrhage due to the exposed liver parenchyma.[10]

A cyst that connects with the biliary tree necessities an internal drainage procedure such as (cystjunostomy or cystgastrostomy), but if there is no communication then free drainage into the peritoneum, with a secondary external drain, is appropriate.[9]

PTC and percutaneous transhepatic biliary drainage are effective in the treatment of postoperative bile leaks and with low complications.[4] Although effective, complications of bacteremia and sepsis may still arise requiring a course of antibiotics and catheter change.[5]

We evaluated reports of patients with congenital hepatic cysts which were managed surgically and had postsurgical bile leaks as a complication [Table 1]. For postoperative bile leakage, we suggest a management algorithm [Figure 5] which encompasses two options for those with and without postoperative drains.
Figure 5: Algorithm for postoperative management of a bile leak

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Table 1: Cases of congenital hepatic cysts with postoperative biliary leakage

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Histologically, CLCs are usually well encapsulated and lined with simple cuboidal or columnar, secretory, epithelium.[3] In neonates, a study demonstrated biliary-type columnar epithelium, in contrast to older children where atrophic changes in the lining epithelium maybe observed.[3]

  Conclusion Top

The CLC is a rare condition in new-borns and infants that is usually asymptomatic. However, it can cause severe respiratory and gastrointestinal manifestations due to an increase in size. It is essential to diagnose this cyst accurately and as early as possible to aid the management. Large cysts usually require complete resection if possible to relieve symptoms. Where this is not possible a partial resection is reasonable but would require long-term follow. PTC is a useful tool to manage bile leaks consequent to surgery.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Rygl M, Snajdauf J, Petrů O, Kodet R, Kodetová D, Mixa V. Congenital solitary liver cysts. Eur J Pediatr Surg 2006;16:443-8.  Back to cited text no. 1
Popat B, Thakkar D, Deshmukh H, Rathod K. Percutaneous transhepatic biliary drainage in the management of post-surgical biliary leaks. Indian J Surg 2017;79:24-8.  Back to cited text no. 2
Recinos A, Zahouani T, Guillen J, Rajegowda B. Congenital Hepatic Cyst. Clin Med Insights Pediatr 2017;11:1179556517702853.  Back to cited text no. 3
Uller W, Wohlgemuth WA, Hammer S, Knoppke B, Goessmann H, Loss M, et al. Percutaneous treatment of biliary complications in pediatric patients after liver transplantation. Rofo 2014;186:1127-33.  Back to cited text no. 4
Allan M, Asimakidou M, Davenport M. Antenatally-detected liver cysts: Causes and characteristics, indications for intervention. J Pediatr Surg 2020;55:441-5.  Back to cited text no. 5
Nordin AB, Fallon SC, Carter BA, Brandt ML. Congenital hepatic cyst with antenatal diagnosis: A case report and literature review. Pediatr Surg Int 2013;29:847-50.  Back to cited text no. 6
Rogers TN, Woodley H, Ramsden W, Wyatt JI, Stringer MD. Solitary liver cysts in children: Not always so simple. J Pediatr Surg 2007;42:333-9.  Back to cited text no. 7
Raboei E, Luoma R. Definitive treatment of congenital liver cyst with alcohol. J Pediatr Surg 2000;35:1138-9.  Back to cited text no. 8
Linden AF, Pulcrano ME, Duffy BJ, Lange PA, Tsung DY, Hartman GE, et al. Laparoscopic excision of congenital hepatic cysts in the pediatric population: A case series and literature review. J Laparoendosc Adv Surg Tech A 2016;26:493-7.  Back to cited text no. 9
Kozlov Y, Kovalkov K, Kapuller V. Laparoscopic treatment of congenital nonparasitic liver cysts in newborns. J Laparoendosc Adv Surg Tech A 2019;29:1325-9.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1]


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