Calinescu et al. BMC Pediatrics(2024) 24:580
https://doi.org/10.1186/s12887-024-05043-z

BMC Pediatrics

CASE REPORTOpen Access

A case series of prenatal hepatic hilar cyst
in the presence of a gallbladder - navigating
the dilemma between biliary atresia
and choledochal cyst
Ana M. Calinescu1,2*, Anne-Laure Rougemont1,3, Valérie A. McLin1,4, Nathalie M. Rock1,4, Céline Habre5 and Barbara
E. Wildhaber1,2
Abstract
Background Prenatally diagnosed hepatic hilar cysts are a challenging finding for the clinician. They can either be
a sign of cystic biliary atresia (BA) or a choledochal cyst (CC), two diagnoses with different postnatal management
and prognosis. Based on a case report of four patients, we aim to propose a management algorithm for prenatallydiagnosed “hepatic hilar cysts”.
Case presentation A hepatic hilar cyst, ranging from 5 to 25 mm, was detected prenatally in all four girls confirmed
postnatally along with the presence of a gallbladder. Stool color was normal until two weeks of life at which time thestool color became lighter, and the patients developed cholestasis. All were operated before seven weeks of life: Case
1 had a CC with patent but irregular intrahepatic bile ducts at intraoperative cholangiogram, and no communication
with the duodenum. A Roux-en-Y bilioenteric anastomosis was performed. The cyst showed complete epithelial lining
loss, and liver pathology showed BA features. Case 2 had the final diagnosis of cystic BA with patent but abnormal
intrahepatic bile ducts. She underwent two operations: the first operation at four weeks as described for case 1, since
intraoperative findings were similar, as was histology. As cholestasis increased postoperatively, she underwent a Kasai
hepato-porto-enterostomy six weeks later, where distinct BA findings were found with complete scarring of the hilar
plate. Case 3 had a cystic BA with the cyst located within the common bile duct and atretic bile ducts proximal to theporta hepatis. It exhibited no communication with the liver or duodenum. A Kasai operation was performed, withhistology showing complete epithelial loss within the cyst wall and scarring of the hilar plate. Case 4 had a cystic BA
presenting a completely obliterated hepatic duct with the cyst lying within the common bile duct. A Kasai procedure
was performed. Histology showed a common bile duct with a residual lumen of 0.1 mm.
Conclusions The spectrum of disease from CC to BA in the setting of a prenatally discovered hepatic hilar cyst isemphasized. Even if cholangiogram differentiates most patients with BA from those with CC, caution is advised fortransitional types.

*Correspondence:
Ana M. Calinescu
ana-maria.calinescu@hug.ch
Full list of author information is available at the end of the article© The Author(s) 2024. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use,sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) andthe source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in thisarticle are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not includedin the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you willneed to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

Calinescu et al. BMC Pediatrics(2024) 24:580

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Keywords Cystic biliary atresia, Choledochal cyst, Prenatal hilar hepatic cyst, Hepaticojejunostomy, Case report

BackgroundToday it is generally acknowledged that prenatally diagnosed hepatic hilar cysts in the presence of a gallbladder have two distinct differential diagnoses, choledochal
cyst (CC) versus biliary atresia (BA) [1] with a differential
diagnosis of solitary liver cysts and gallbladder duplication. A hepatic hilar cyst is present in up to 16% of BA
patients [2–5]. Two patterns of cystic BA can occur:
most frequently they correspond to BA with patent but
abnormal intrahepatic bile ducts and an extrahepatic cyst
located within the hepatic duct or, more rarely, to BA
with atretic bile ducts proximal to the porta hepatis andan extrahepatic cyst in the fibrous choledochal remnant[6–9]. CC also have distinct subtypes, whereby the typical form with the presence of a cyst in the common bile
duct (Todani classification type I) accounts for up to 90%
of CC [10].
In the setting of a prenatal hepatic hilar cyst, postnatal ultrasound (US) with Doppler is the first imaging
examination to differentiate between a BA and a CC [11].
A hepatic hilar cyst with dilated intrahepatic bile ducts
is diagnostic of CC and rules out BA. Nevertheless, in
unclear presentations, i.e. a hilar cyst with abnormal gallbladder and/or triangular cord sign, an intraoperative
cholangiogram (IOC) is warranted [12].
Patients with CC classically undergo cyst excision andhepatico-jejunostomy, BA patients undergo Kasai hepato-porto-enterostomy (HPE). While Kasai HPE shouldbe performed as soon as possible, hepatico-jejunostomy
for perinatally diagnosed CC can be differed in the firstmonths of life, with the need for swift intervention incase of neonatal cholestasis [13]. Identifying the correct surgical approach and timing is essential for optimal
patient outcome. While CC patients usually are definitely
managed by hepatico-jejunostomy, cystic BA patients,
even if endowed with higher native liver survival rates,
still might need, at some time point, a liver transplantation [14].
The present case report illustrates the step-by-step process of clinical decision-making for patients prenatallydiagnosed with hepatic hilar cysts in the presence of avisible gallbladder in the unique setting of four patientswith transitional types of disease.
Case presentation
Preoperative, operative, pathology and postoperative
characteristics of four challenging female patients witha prenatally diagnosed hepatic hilar cyst diagnosed
between 2020 and 2022 with a follow-up of 15.5–38
months were reviewed. The present study follows theguidelines of the revised UN Declaration of Helsinki andcomplies with the local regulations in the Geneva University Hospitals, Swiss Ethics (Ethics Committee no.
AO_2022-00073). Informed consent was obtained fromeach patient’s legal guardian.
Pre- and immediate postnatal evaluation

All four girls presented a hepatic hilar cyst with a prenatal
size from 5 to 25 mm, measured at different time-points
with US and magnetic resonance cholangiopancreatography (MRCP), not evolving over time. Liver stiffness wasevaluated using acoustic radiation force impulse imaging(normal range 0.73–1.45 m/sec) [15]. Post-natal findings
are summarized in Table 1; Fig. 1a, b, c, d. Overall, stool
color was normal until two weeks of life when they progressively started to discolor, and the patients graduallybecame cholestatic, prompting surgery, all before sevenweeks of life.
Operative findings

In all four cases an intraoperative cholangiogram (IOC)
via the gallbladder was performed (Fig. 2a, b, c): retrograde opacification of patent but thin and irregular
intrahepatic bile ducts, with no opacification of the duodenum, was present in Cases 1 and 2 (Fig. 2). The fluid
collected from the cyst was nearly transparent and contained no pigment. Amylase and lipase values in the cyst
were normal. Inflammatory adhesions were mild aroundthe cyst in Case 1 and moderate in Case 2. The liver
surface was irregular in Case 1 and the liver soft, while
it was smooth and soft in Case 2. Given the presenceof a clear communication of the cyst at the level of thehepatic duct with the intrahepatic bile ducts, a Roux-enY bilio-jejunal anastomosis was performed. Cases 3 and4 showed absence of retrograde filling of contrast in theintrahepatic bile ducts reflecting their complete obliteration (Fig. 2), with the hilar cyst lying within the common
bile duct, corresponding to a cystic BA with atretic bileducts proximal to the porta hepatis, consequently a Kasai
HPE was performed. The liver surface was smooth andthe liver firm for Cases 3 and 4.
Pathology findings

In all 4 cases, liver biopsy findings were consistent withBA, showing mild portal tract edema and fibrosis, ductular reaction, and major cholestasis with giant cell transformation. In cases 2, 3 and 4, atretic biliary remnants
at the porta hepatis resected at portoenterostomy weresurrounded by a fibro-edematous and inflammatory
cuff. Bile duct and cyst characteristics are summarized inTable 2.

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Table 1 Summary of the post-natal findings in the four patients
Case 1
Todani I Choledochal
cyst

Case 2
Cystic BAwith patent but abnormal intrahepatic bile ductsF
41 3/7
10

Case 3
Cystic BAwith atretic bile ducts
proximal to the porta
hepatisF
40 4/7
11

Case 4
Cystic BAwith atretic bile ducts
proximal to the porta
hepatisF
37 3/75Sex
Birth (weeks)
Cyst size (mm) at 1st post-natal
US
Icterus (days)
Preoperative maximum conjugated bilirubin (µmol/l)Preoperative maximum GGT (U/l)
US gallbladderF
38 3/720
From birth
98.9

21
81.3

From birth
101

None
63

86
Normal91
Irregular wall

None
None
1.64

None
None
2.01

99
Small size
Irregular wall
None
None
n.a.

195
Small size
Irregular wall
Present
None
1.26

Hyperechoic triangular cord sign
US bile duct dilatation
Liver stiffness
m/sec
MRCP

Non-communicating hilar cyst
No dilated intrahepatic bile ducts

n.a.

n.a.

Patent intrahepatic bile ducts
No duodenal drainage

No intrahepatic bileducts
No duodenal drainage
Transparent
Kasai procedure

No intrahepatic bileducts
No duodenal drainage
Transparent
Kasai procedure

Age at 1st surgery (days)
2nd Surgery

Hilar cyst communicating with right bile ductyet dilated
Patent intrahepatic bileducts
No duodenal drainage
Transparent
Cyst excision,
hepatico-jejunostomy
42
n.a.

55
n.a.

42
n.a.

Age at 2nd surgery (days)
Follow up (months)

n.a.
38

n.a.
15.5

n.a.
32

Final diagnosis

Intraoperative cholangiogram

Bile colour
1st Surgery

Transparent
Cyst excision,
hepatico- jejunostomy
28
Kasai
procedure
69
28

Fig. 1 Ultrasound and magnetic resonance cholangiopancreatography coronal maximum intensity projection reformat. In Case 1, a large cyst of the liver
hilum (stars) communicating with an intrahepatic bile duct (white arrow) at both ultrasound (a) and MRCP (b). In case 2, a smaller hilar cyst (stars) without
any visible communication with intra- or extrahepatic bile ducts at both ultrasound (c) and MRCP (d). HA, hepatic artery; PV, portal vein; ST, stomach;
MRCP, Magnetic resonance cholangiopancreatography

Histological evaluation of the cyst wall showed, inCases 1, 2 and 3, a striking dense hyalinised and paucicellular layer. This hyaline membrane was seen on theluminal, inner aspect of the cyst (Fig. 3a, Case 1, and waspartially detached in cases 2 and 3 (Fig. 3b, c and d). No
residual epithelial lining was seen. In addition, the cyst
wall in Case 3 showed intense vascular congestion withhemorrhagic suffusion.

The supposed cyst of the porta hepatis in Case 4 laid
within the ductus choledochus. Histology showed a choledochus with a residual lumen of 0.1 mm, with subtotal
epithelial loss. The common hepatic duct and the choledochus showed loose fibro-edematous changes, without well-defined hyalinized layer or membrane. Instead,
focally increased collagen deposition was seen (Fig. 3e
and f ).

Calinescu et al. BMC Pediatrics(2024) 24:580

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Fig. 2 Intraoperative cholangiogram. Case 1 (a) and 2 (b) showed patent but irregular intrahepatic bile ducts (arrow) and no communication with theduodenum (star). Case 4 (c) showed no communication with either intrahepatic bile ducts or duodenum

Fig. 3 The cysts from Cases 1 to 3 (a to d) showed similar histological features, whereas findings were different in Case 4 (e and f). a. In Case 1, the 3.5 cm
long cyst showed no biliary epithelium; instead, the luminal portion of the cyst consisted of a dense hyaline layer overlying a congestive outer cyst wall
(Hematoxylin& Eosin, H&E, original magnification x60). b. In Case 2, a similar collagenized and detached layer is best seen with a Masson trichrome stain;
there is no biliary epithelium remnant (x10). c and d. Again, in Case 3, a hyaline membrane appears semi-detached in the cyst lumen, and overlying a cyst
wall with vascular congestion and hemorrhagic suffusion, but no epithelium (C, H&E, x100). On higher magnification, the membrane is dense and paucicellular (D, H&E, x200). e and f. Case 4 showed no obvious cystic dilatation. The common hepatic duct showed a lack of epithelium, the luminal portionof the duct showing fibro-edematous changes (E, Masson trichrome, x100). The inner portion of the ductus choledochus showed heterogeneous, focallyincreased collagen deposition (arrows), but no hyaline membrane (F, Masson trichrome, x200)

Table 2 Main histological findings
Size of proximal common hepatic duct (mm)Size of hepatic hilar biliary structures (µm)
Cyst size (cm)Hyaline membrane

Case 15n.a.
3.5 × 2.5
Yes

Epithelial lining

None

Case 2
3
75
4
Yes
Partially detached
None

Postoperative follow-up

Case 1and 4 cleared jaundice within the first two postoperative weeks. Because of persistent postoperativecholestasis without bile duct dilatation and pathology
suggestive of BA, Case 2 underwent a Kasai HPE
six weeks after the initial surgery without further

Case 3Atrophic50
2.5
Yes
Partially detached
None

Case 4
1.5
15–40
n.a.
No
Focal collagen deposition
Subtotal loss

supplementary investigations such as hepatobiliary iminodiacetic acid (HIDA) scan and MRCP given the highsuspicion of BA based on the pathological findings. We
thus decided against additional imaging to avoid delaysin the necessary surgical intervention. It is a matter of
debate if Case 1 is not a choledochal cyst but a cystic BA.

Calinescu et al. BMC Pediatrics(2024) 24:580

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Fig. 4 Timeline for final postnatal diagnosis of four patients diagnosed with prenatal hilar liver cysts.

Cases 1and 2 cleared jaundice and are well and thriving
at the current age of 32 and 22 months.
While Case 4 initially followed a similar course, shethen developed bile lakes and intermittent cholestaticjaundice. She actually cleared jaundice.
Case 3 presented an early episode of cholangitis at day
seven with persistent jaundice and underwent liver transplantation at the age of 8 months (Table 1).

Discussion
Pre- and postnatal evaluation

A prenatally discovered hepatic hilar cyst, in the presence of a visible gallbladder, has two distinct differential
diagnoses, BA versus CC. There are two widely acceptedindicators which may predict diagnosis: (1) the size of thecyst in BA is reported to be smaller than in CC, probably
due to the smaller volume of excreted bile [7, 16], and (2)in the case of CC the size of the cyst usually increases
continuously before birth [17]. Nonetheless, postnatal
diagnosis cannot be readily defined prenatally and postnatal imaging can be equivocal when not able to rule out
BA. As the two diagnoses have quite different management and, importantly, a distinctly different prognosis,
prenatal counselling of parents is challenging. A further
indicator could be abnormal levels of amniotic fluid
digestive enzymes measured before 22 weeks of gestation, which have been shown to predict BA in up to 90%
of patients with prenatal non-visualization of the gallbladder [18]. However, to the best of our knowledge, theprofile of amniotic fluid digestive enzymes has not been

analyzed in the presence of prenatal hepatic hilar cysts toconfirm or exclude BA.
After birth, the hepatic hilar cyst and associatedabdominal features should be investigated by US. In thepresence of a hepatic hilar cyst, US features highly suggestive of BA include the triangular cord sign, a small andirregular gallbladder, and abdominal heterotaxia with
polysplenia [19]. Further and understandably, the presence of dilated intrahepatic bile ducts is associated withCC and thus rules out BA [20]. Of note, multiple cut-off
values on the post-natal US have been identified to differentiate between CC and BA, ranging from 1 cm to 2.5 cm[21, 22]. Consequently, in the 90ies Redkar et al. proposedan algorithm for the management of prenatally diagnosed
hepatic hilar cysts based on dilated or non-dilated intrahepatic bile ducts [23]. However, this management algorithm proved to be difficult to apply, since the majority ofthe patients will not have dilated intrahepatic bile ducts.
Last but not least, hepatobiliary scintigraphy used in thisparticular situation has proven to be misleading becauseof numerous false-positive findings [24].
As for biological markers, it appears that newborns
with BA present with higher serum aminotransferases,
higher total bile acids and increased levels of bilirubin,when compared to babies with CC [16, 20]. Nevertheless,gamma-glutamyl transferase levels were not different in aclinically controlled study comparing CCs with BA [20].
Regular blood work-up might help distinguish between
BA and CC patients.
Patients diagnosed with prenatal hepatic hilar cystswith highly suggestive features of BA on US need an earlyCalinescu et al. BMC Pediatrics(2024) 24:580

Kasai operation. For a CC not developing jaundice anddiscolored stool in the postnatal evaluation, the optimal
timing for its treatment has not been definitely established yet [7]. That said, it is now accepted that patients
have to be monitored closely by US and liver function
tests. MRCP is usually only performed around threemonths to better delineate the anatomy of the cyst andbile ducts before elective surgery, scheduled between 3
and 6 months to avoid complications [25]. A meta-analysis revealed 68% positive predictive value and 94.3% negative predictive value of the MRI in diagnosing BA [26].
The effectiveness of MRCP in reliably excluding BA at
the optimal time of surgical intervention is limited by thenon-visualisation of the extrahepatic bile ducts in 37.5%
of infants less than 3 months of age under normal physiologic conditions and 50% of infants less than one month[27]. A HIDA scan can be proposed for the pre-operative
work-up; absent excretion of radiolabeled contrast into
the duodenum raises the suspicion of biliary atresia [28,29]. The same study identified 64.5% positive predictive
value and 97.2% negative predictive value for the HIDA
scan in diagnosing BA; thus, besides its irradiation, false
positive results might occur in patients with medical cholestasis [26]. The “intermediate cases” who develop jaundice should undergo prompt IOC [7] to differentiate an“obstructive” CC from cystic BA [30]. It is of the utmost
importance that the patient be transferred to a center
specialized in the management of BA.
Summary and recommendation for pre- and postnatal
evaluation (Fig. 5)

Foetuses with a prenatally diagnosed hepatic hilar cyst in
the presence of a visible gallbladder need early postnatal
evaluation with US. Cases where the diagnosis remains

Page 6 of 10

unclear and CC or BA cannot be confirmed by postnatal US, need close observation. Cholestasis must lead toearly IOC for a timely Kasai.
Intraoperative cholangiogram and surgery

IOC is the current gold standard for diagnosing BA.
Cholangiography patterns (evaluation of the intrahepatic
bile ducts and duodenal communication) can differentiate classical CC and BA patients [31].
During cholangiography, even in patients with a CC,
the duodenal communication is not always visible andbiliary fluid coming from patent intrahepatic bile ducts
can be clear due to longstanding extrahepatic biliary
obstruction by a blind-ending CC [32]. Similar cases to
our Case 1 were reported with a lack of distal communication with the duodenum, a finding usually associatedwith BA [9, 32].
A description of the IOC patterns of the proximal bileducts for cystic BA includes (i) cloudy, (ii) tree-like or (iii)
mixed pattern. These three findings have been shown
to be associated with outcomes for native liver survivalof 50%, 100% and 78%, respectively [3]. It has been proposed to perform a hepatico-jejunostomy for patientswith a hepatic duct diameter ≥ 1 mm [4], or whenever
the duct seemed large enough to accommodate an anastomosis [33]. Jaundice clearance rates were significantly
improved also whenever the hepatic radicles have adiameter of ≥ 1 mm when compared to < 1 mm diameter(93% vs. 77%) [4].
Cyst amylase and lipase concentration is usually analyzed, with high values speaking in favor of a pancreaticobiliary malunion and thus a CC [34]. However, it has
been stipulated that pancreaticobiliary malunion explains
CC in older patients [35], while neonatal forms of CC are

Fig. 5 Algorithm for the management of prenatal hepatic hilar cysts in the presence of a visible gallbladder. IOC, intraoperative cholangiogram; HPE,
hepato-porto-enterostomy; IHBD, intrahepatic bile ducts; HJ, hepaticojejunostomy; MRI, magnetic resonance imaging; US, ultrasoundCalinescu et al. BMC Pediatrics(2024) 24:580

Page 7 of 10

believed to be “congenital”. They rather seem to present
a weakness of the common bile duct in its early developmental stages, allowing its dilatation when intraductalpressure increases due to obstruction of the distal common duct [36]. Furthermore, pancreatic enzyme activity
seems to increase only after one month of extrauterine
life [22]. It is thus not surprising that Case 1 did not show
increased values of pancreatic enzymes in the cyst fluid.
While the management of CC is unequivocal withcyst excision and hepatico-jejunostomy, the best surgical strategy for cystic BA is still debated. Historically, aKasai HPE was proposed for cystic BA [37]. Native liver
survival for patients with cystic BA and Kasai has beenshown to be 68% with a patient survival of 76% (Table 3).
Later, hepatico-jejunostomy has been described for cystic
BA [4, 33, 38]. Excellent results have been reported forboth open and laparoscopic hepatico-jejunostomy performed for cystic BA [5], [39], with 98% patient survival
and 92% native liver survival (Table 4). The reported literature consists mainly of case reports with very different follow-up periods. Our findings suggest a possible
benefit in native liver and patient survival from performing a hepatico-jejunostomy for cystic BA, although theseoutcomes are influenced by multiple factors including thetiming of the surgery, bile duct diameter, postoperative
care, etc., factors that are not always well documented in
the below mentioned studies.
Summary and recommendation for cholangiography andsurgery (Fig. 5)

IOC is the key step in determining surgical treatment.
In the case of an obvious CC an hepatico-jejunostomy isperformed. If no intrahepatic bile ducts are seen, a standard Kasai must be performed. In the case of irregular
intrahepatic bile ducts and/or lack of communication

with the duodenum, if macroscopic intrahepatic bileducts are seen, it is reasonable to perform a hepaticojejunostomy for jaundice clearance.
Pathology findings

A common physiopathology of infantile obstructive cholangiopathy in BA and CC was suggested for the first timeby Landing in 1974 [48]. The recent demonstration of theloss of primary cilia from cholangiocytes in both BA andCC, together with the presence in a subset of cases of aductal plate malformation or of laterality defects, have
led to the proposal to include these conditions in the
spectrum of ciliopathies [49].
A complete or near complete lack of a biliary type
epithelial lining has been reported in the cysts of BA as
opposed to CC [2, 50, 51]. Accordingly, all four cases
reported here showed total or subtotal absence of an epithelial lining.
The more recent descriptions highlight the presence ofa striking grossly visible whitish membrane corresponding upon histological evaluation to an inner densely collagenized layer that tended to separate from the outerportion of the cyst wall [50, 51]. It is speculated that
this membrane may be due to subepithelial degenerative changes, perhaps related to the accumulation of fluid[51]. In the series by Lobeck et al. [50]. no subluminal
hyaline layer was seen in 18 of the 21 CC reported cases.
In the remaining patients, a layer of sclerotic collagen wasobserved. These three infants had liver biopsy featuresof obstructive cholangiopathy; however a diagnosis of
BA was not retained at surgery and follow-up [50]. Thishighlights the potential morphological overlap between
BA and CC. A similar paucicellular hyalinized membrane
was observed in Cases 1 to 3. Both the hepatic duct andthe choledochus in case 4 showed only focal areas of

Table 3 Outcome of Kasai hepato-porto-enterostomy for cystic biliary atresia
ArticleKomuro H [5]# ofpatient2

Age at Kasai HPE (days)

Outcome

54
57
11
30
45

Jaundice freeJaundice freeJaundice freeJaundice freeDead(cholangitis)
Jaundice freeJaundice freeJaundice free

MacKenzie T [40]
Masumoto K [41]Sinha S [42]111Suzuki T [12]Fatahi N [28]Tanaka H [7]11
3

Nio M [4]

26

203
n.a.
14
50
48
n.a.

Koshinaga T [43]Rahamtalla D [44]

1
1

120
56

Kasai HPE, Kasai hepato-porto-enterostomy; LT, liver transplantation

8 Dead3 LT
Jaundice freeJaundice free

Follow-up(years)
20
7.7
0.8
n.a.
0.25
17
0.8
2.1

n.a.
3
n.a.

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Table 4 Outcome of hepatico-jejunostomy for cystic biliary atresia
Article# ofpatient

1st surgery

1st surgery age(days)

Nr. (%) Kasai
HPE

Outcome

Tsuchida Y [45]Matsubara H [46]
Komuro H [5]

Nio M [3]Caponcelli E [2]Takahashi Y [33]

1
1
1
1
1
1
22
1
12

HJ
HJ
HJ
HJ
HJ
HJ
HJ
HJ
HJ

23
22
30
25
152
84
n.a.
n.a.
71.6 (24–136)

n.a.
n.a.
n.a.
n.a.
n.a.
n.a.
3/22 (13.6%)
1
1/12 (8.3%)

Faure A [47]3

Laparoscopic HJ

n.a.

n.a.

Nio M [4]

14

HJ

n.a.

3/14 (21.4%)

Lal R [38]

2

HJ

n.a.

Ji Y [39]Calinescu AM, 2024
(actual series)27
1

Laparoscopic HJ
HJ

32
45
67.9+/- 35.6
28

Jaundice freeJaundice freeJaundice freeJaundice freeLT
Jaundice freen.a.
Jaundice free
9/12 (75%) Jaundice free
1 Recurrent jaundice
2 LT
2 Jaundice free
1 Recurrent jaundice
10/14 (71.4%) Jaundice free
2 LT
2 DeadJaundice freeJaundice free
25/27 (92.6%) Jaundice freeJaundice freen.a.
1

Followup(years)
1.5
0.5
17
2.5
0.8
0.3
n.a.
n.a.
1.1–22

5.1
n.a.

n.a.
6+/-2.9
1.8

HJ, hepatico-jejunostomy; Kasai HPE, Kasai hepato-porto-enterostomy; LT, liver transplantation

increased collagen deposition. The resection specimen
showed no obvious cystic dilatation, and this case may
represent a more frustre form of cystic BA.
Liver histology may also reveal differences between
the two entities, with portal tract fibrosis and featuresof chronic cholangiopathy tending to be more severein BA than in CC [51], although significant overlap canbe seen [31, 49]. It can be argued if a needle liver biopsy
should be obtained prior to IOC for these patients. Nevertheless, if patients present with jaundice and discoloredstool a prompt intervention is warranted. If the pathology
results can influence the further course of action, the firstintervention performed for these children is not influenced by the pathology results.
Summary and recommendation for pathology findings

Pathology is essential to guide follow-up in patients with
hepatic hilar cysts and irregular intrahepatic bile ducts
who develop obstructive jaundice. In case of histology
compatible with BA, patients previously identified as presenting CC, should be monitored closely.
Postoperative follow-up

The chronology of neonates with prenatal hepatic hilar
cyst has shown that a subset of patients will evolvefrom a cystic BA with patent but abnormal intrahepatic
bile ducts, to BA with atretic bile ducts proximal to theporta hepatis [41]. Further, another physiopathologicalcorrelation was found between an extrahepatic biliary
cyst perforation and a cystic BA with atretic bile ducts
proximal to the porta hepatis [43]. Two explanations
were proposed: first, overlapping features of CCs and BA
might be due to the young age of the patients, more than
to the BA per se; second, the early presentation of some
cystic BA patients might be the same as CC [50]. It wasalso hypothesized that patients with CCs or cystic BA
might progress to type III BA [2, 6, 12, 41, 43]. The courseof Case 2 supports the hypothesis of an evolutive process
between the CC and cystic BA. One might postulate thata continuum exists in the development of CCs, cystic
BA with patent but abnormal intrahepatic bile ducts, toBA with atretic bile ducts proximal to the porta hepatis(Fig. 4). Therefore, it is not surprising that the Kasai procedure has shown excellent results in patients after anunsuccessful hepatico-jejunostomy performed for cystic
BA (Table 2).
Summary and recommendation for postoperative
follow-up (Fig. 5)

In patients with an antenatal history of a hepatic hilar
cyst who develop obstructive jaundice and presented
with irregular bile ducts on IOC at the time of hepaticojejunostomy, careful follow-up is essential to convert to
Kasai HPE in a timely fashion.

Calinescu et al. BMC Pediatrics(2024) 24:580

Strengths and limitations

While this case series provides valuable insights into thechallenging scenario of prenatally diagnosed hepatic hilar
cysts, its findings should be interpreted with consideration of the small sample size, retrospective nature, andthe need for caution in managing transitional types. Further research is warranted to validate the proposed management algorithm and enhance the understanding of
this complex clinical scenario.

Conclusions
The management of prenatally diagnosed hepatic hilar
cysts in the presence of a visible gallbladder is challenging. A management strategy was developed to help clinicians to navigate this situation (Fig. 5). Even if IOC
differentiates most patients with BA from those with aCC, for hybrid indeterminate forms with irregular intrahepatic bile ducts, caution is advised. A hepatico-jejunostomy can be performed for these forms, but the patientneeds a close, specialized follow-up, as a secondary Kasai
HPE might be required.
Abbreviations
BA	Biliary atresia
CC	Choledochal cyst
US	UltrasoundIOC	Intraoperative cholangiogram
HPE	Hepato-porto-enterostomy
HIDA	Hepatobiliary iminodiacetic acid scan
MRCP	Magnetic resonance cholangiopancreatography
Acknowledgements
The authors would like to thank all colleagues in the Geneva University
Hospitals involved in the management of infants with neonatal cholestasis.
Author contributions
Conceptualization, A. M. C. and B. E. W.; methodology, A.M. C., B.E. W.;
validation, all; formal analysis, all; investigation, A. M. C.; resources, A. M. C.;data curation, A. M. C.; writing—original draft preparation, all; writing—review
and editing, all; visualization, all; supervision, B. E. W.; project administration,
A. M. C. All authors have read and agreed to the published version of themanuscript. All authors are accountable for all aspects of the work in ensuringthat questions related to the accuracy or integrity of any part of the work areappropriately investigated and resolved.
Funding
This research did not receive any specific grant from public, commercial, or
not-for-profit funding agencies.
Open access funding provided by University of Geneva
Data availability
No datasets were generated or analysed during the current study.

Declarations
Ethical approval and consent to participate
The present study follows the guidelines of the revised UN Declaration of
Helsinki and complies with the local regulations in the Geneva University
Hospitals, Swiss Ethics (Ethics Committee no. AO_2022-00073).
Consent for publication
Informed consent was obtained from each patient’s legal guardian.

Page 9 of 10

Competing interests
The authors declare no competing interests.
Author details
1
Swiss Pediatric Liver Center, Department of Pediatrics, Gynecology and
Obstetrics, University of Geneva, Geneva, Switzerland
2
Division of Child and Adolescent Surgery, Department of Pediatrics,
Gynecology, and Obstetrics, Geneva University Hospitals, University ofGeneva, 6, Rue Willy Donzé, Geneva 1205, Switzerland
3
Division of Clinical Pathology, Diagnostic Department, Geneva University
Hospitals, University of Geneva, Geneva, Switzerland
4
Gastroenterology, Hepatology and Nutrition Unit, Division of PediatricSpecialties, Department of Pediatrics, Gynecology, and Obstetrics, Geneva
University Hospitals, University of Geneva, Geneva, Switzerland
5
Division of Pediatric Radiology, Diagnostic Department, Geneva
University Hospitals, University of Geneva, Geneva, Switzerland
Received: 26 November 2023 / Accepted: 30 August 2024

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