The anatomical relationship with the biliary tree. SLCs are

The term
hepatic cyst usually refers to solitary nonparasitic liver cysts (SLCs), also
called as simple cysts.(1, 2)
However, several other cystic lesions must be differenciated from true simple
cysts. Cystic lesions of the liver include Simple cysts, multiple cysts arising
in the setting of polycystic liver disease (PCLD), parasitic or hydatid
(echinococcal) cysts, cystic tumors and abscesses. These conditions can usually
be distinguished on the basis of the patient’s symptoms, clinical history, and
the radiographic appearance of the lesion. Ductal cysts, choledochal cysts, and
Caroli disease are differentiated from hepatic cysts by involvement of the bile
ducts and are not reviewed in this article.

patients with solitary liver cysts (SLCs), it is usually agreed that
laparoscopic unroofing offers the best balance between efficacy and safety. Liver
resection is more effective but carries higher risks. The cause of solitary
liver cyst is not known, but they are believed to be congenital in origin. The
cyst is lined with biliary-type epithelium, and perhaps results from
progressive dilatation of biliary microhamartomas. Because these cysts never
contain bile, the current hypothesis is that the microhamartomas fail to
develop normal connections with the biliary tree. Typically, the fluid within
the cyst has an electrolyte composition that mimics plasma. Bile, amylase, and
white blood cells are absent. The cyst fluid is continually secreted by the
epithelial lining of the cyst. For this reason, needle aspiration of simple
cysts is not curative, and recurrence is the norm.

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liver cysts (SLCs) rarely manifest in infancy. They are unilocular, benign
lesions of the liver and can be defined as a simple cyst or a biliary cyst
according to the composition of the cyst fluid and the cyst’s anatomical
relationship with the biliary tree. SLCs are generally not symptomatic,
especially in the postnatal period.(3, 4) Most require no intervention if found
prenatally.(5) However, large cysts (>3 cm) may obstruct
adjacent tissues and organs, leading to atrophy and complications like
spontaneous hemorrhage, bacterial infection, biliary obstruction, rupture, and
neoplastic degeneration into squamous cell carcinoma.(5-12) There
is a reported neonatal case of IVC obstruction from a SLC treated with surgical
intervention.(5, 13) Giant cysts of the liver are uncommon.(14) Simple hepatic cysts rarely cause symptoms,
however they become symptomatic due to mass effect, rupture, haemorrhage, and
infection. Large cysts can produce atrophy of the adjacent hepatic tissue while
huge cysts can cause complete atrophy of a hepatic lobe with compensatory
hypertrophy of the other side.(15) Congenital hepatic cysts are comparatively
rare, but the rate of diagnosis during pregnancy is increasing due to the
routine use of prenatal ultrasound.(16, 17) Congenital solitary liver cysts (CSLC) are
usually asymptomatic lesions, most frequently discovered during imaging
studies. The majority of the cysts that are discovered incidentally remain
small, but a few continue to grow and may present as an abdominal mass. Unlike
polycystic disease, these CSLC are not associated with renal, splenic,
pancreatic, or lung cysts.(18, 19) Their incidence is reported to be 2.8% during
routine abdominal ultrasound examinations in adults.(19, 20) The vast majority of cases involve patients in
their fifth and sixth decades and there are only few reports of these cysts in
children.(18, 19, 21, 22) Simple
cysts accounted for most of the prenatally detected and postnatally diagnosed
solitary lesions. They may be underrepresented in this series because some
asymptomatic simple cysts would not have been referred to our unit. Simple
cysts are generally thought to develop from aberrant bile ducts,(23-26) but an
origin from intrahepatic peribiliary glands has also been suggested.(26, 27) The pathogenesis of liver cysts is unclear.(18) Solitary benign cysts are believed to be
congenital and are thought to origin from pluripotent foregut tissue (19, 28) or to be caused by the abnormal development of
intrahepatic bile ducts (19, 23) or intrahepatic peribiliary glands.(18, 19) These cysts do not invade biliary or vascular
elements and they do not contain bile. CSLC are lined with simple cuboideal or
columnar epithelium secreting serous fluid and have a connective tissue capsule.
Most patients are asymptomatic, but complications related to the size of the
cyst.(19, 21) The differential diagnosis of a solitary
hepatic cyst includes simple cyst, choledochal cyst, biliary atresia,
mesenchymal hamartoma, cystic tumors (including embryonal sarcoma, dermoid and
teratoma), ciliated foregut cyst, and mesenchymal cysts.(16, 26) Since most congenital hepatic cysts are
parenchymal in origin and simple in nature (consisting of a basic cuboidal
epithelium with peripheral loose connective tissue), many cases do not require
specific treatment as they spontaneously regress over time.(16, 29) Although there is a broad differential
diagnosis of congenital hepatic hilar cysts, it is imperative to differentiate
these from associated cases of choledochal cysts with or without biliary
atresia.(16, 30, 31)
Perioperative cholangiography should be performed in all cases to detect
connections between the SLC and the biliary tree.(4, 32) Management options include percutaneous
aspiration, injection of sclerosing agents, laparoscopic or open fenestration,
and surgical cystectomy or Roux-en-Y hepaticojejunostomy.(4, 32, 33)


The aim
of this study was to describe the clinical and histopathological findings in
infants with congenital solitary intahepatic cyst.