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M. K. Atahan and B. Özçınar
31. Wong SM, Freedman RA, Sagara Y, Aydogan F,
Barry WT, Golshan M.Growing use of contralateral
prophylactic mastectomy despite no improvement in
long-term survival for invasive breast cancer. Ann
Surg. 2017;265:581–9.
32. Molina-Montes E, Pérez-Nevot B, Pollán M, SánchezCantalejo E, Espín J, Sánchez MJ.Cumulative risk of
second primary contralateral breast cancer in BRCA1/
BRCA2 mutation carriers with a rst breast cancer: a systematic review and meta-analysis. Breast.
2014;23:721–42.
33. Reiner AS, John EM, Brooks JD, Lynch CF, Bernstein
L, Mellemkjaer L, etal. Risk of asynchronous contralateral breast cancer in noncarriers of BRCA1 and
BRCA2 mutations with a family history of breast cancer: a report from the Women’s Environmental Cancer
and Radiation Epidemiology Study. J Clin Oncol.
2013;31:433–9.
34. Peralta EA, Ellenhorn JD, Wagman LD, Dagis A,
Andersen JS, Chu DZ. Contralateral prophylactic mastectomy improves the outcome of selected
patients undergoing mastectomy for breast cancer.
Am J Surg. 2000;180:439–45.
35. Herrinton LJ, Barlow WE, Yu O, Geiger AM, Elmore
JG, Barton MB, etal. Efcacy of prophylactic mastectomy in women with unilateral breast cancer:
a cancer research network project. J Clin Oncol.
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36. Kruper L, Kauffmann RM, Smith DD, Nelson
RA. Survival analysis of contralateral prophylactic
mastectomy: a question of selection bias. Ann Surg
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37. Fayanju OM, Stoll CR, Fowler S, Colditz GA,
Margenthaler JA.Contralateral prophylactic mastec-
tomy after unilateral breast cancer: a systematic review
and meta-analysis. Ann Surg. 2014;260:1000–10.
38. NCCN breast cancer guideline version 4.2020. https://
www.nccn.org/professionals/physician_gls/pdf/
breast.pdf. Accessed 7 Jul 2020
39. Hartmann LC, Schaid DJ, Woods JE, Crotty TP,
Myers JL, Arnold PG, etal. Efcacy of bilateral prophylactic mastectomy in women with a family history
of breast cancer. N Engl J Med. 1999;340:77–84.
40. Boughey JC, Hoskin TL, Degnim AC, Sellers TA,
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breast cancer. Ann Surg Oncol. 2010;17:2702–9.
41. Honold F, Camus M.Prophylactic mastectomy versus surveillance for the prevention of breast cancer
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Ann Surg Oncol. 2015;22:3474–80.
Prophylactic Surgery forLiver
Pathologies
OsmanNuriDilek , FeyyazGüngör ,
andArifAtay
9
9.1 Introduction
The liver is an organ blessed with the fate of the
person in Babylon in 2000s before Christ. Glisson
has identied the liver capsule with cadaver studies and published it as a book in 1654. With the
denition of anesthesia and infection control in
the late nineteenth century, abdominal surgical
interventions entered the surgical practice. Partial
liver resection performed by Lius in a 67-year- old
woman with liver adenoma in 1886 was recorded
as the rst liver surgery [1]. This was followed by
resection with Pasquelin’s cautery, partial resections made by Bruns (1888), “V”-shaped (wedge)
resections made by Keen (1889), and hemangioma resections (1893) by von Eiselberg. Cantlie
dened the invisible anatomic border (1897)
between the right and left lobes. The maneuver
applied by Pringle in 1908 to prevent bleeding
in patients with liver trauma has been developed
as a method of Pringle maneuver, which is still
used today. Wendel performed the rst success-
O. N. Dilek (*)
Department of Surgery, Section of
Hepatopancreatobiliary Surgery, Izmir Kâtip Çelebi
University School of Medicine, İzmir, Turkey
e-mail: osmannuri.dilek@ikc.edu.tr
F. Güngör · A. Atay
Department of Surgery, Izmir Kâtip Çelebi University
School of Medicine, İzmir, Turkey
e-mail: feyyaz.gungor@saglik.com.tr;
arif.atay@ikc.edu.tr
ful right hepatectomy in 1911 due to hepatocellular carcinoma. Tinker successfully resected the
rst hemangioma rupture case in 1935. During
the world wars, shock physiology and liver anatomy and physiology were better understood [1].
These surgeries were followed by hepatectomies
by Ishiyama (1941), French Lortat-Jacob et al.
(1951), and Japanese Honjo etal. (1949, 1953).
Lin (1960) from Taiwan described the nger
fracture method for the separation of liver tissue in 1950 and published his series of 34 cases
in 1960 with 12.1% mortality and 19% 5-year
survival rate [1, 2]. In the 1950s, Hjortsjo and
Couinaud described segmental anatomy. Later,
Couinaud’s work led to the adoption of the segment classication concept, which was also taken
under his name. Knowing the segmental anatomy
also initiated the processes that could contribute
to preserving liver tissue [1, 3].
Following the rst liver transplantation in
1963, there has been a great change in liver
surgery in the last 50years. In the 1980s, subsegmental resection concept was developed by
Makuuchi etal. (1990) [3]. In a cirrhotic patient,
it was also important to remove the tumor, leaving sufcient liver tissue. Indocyanine green
(ICG) test developed by Makuuchi etal. played
an important role in determining liver functionality. Makuuchi criteria (includes ascites, bilirubin, and ICG-15 min retention rate) became an
important criterion for safe resection. The same
team later developed the portal vein embolization
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2021
O. N. Dilek et al. (eds.), Prophylactic Surgery, https://doi.org/10.1007/978-3-030-66853-2_9
85
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O. N. Dilek et al.
technique, which would enable hypertrophy of
the left lobe to prevent remnant liver failure. In
the last two decades, liver resections have started
to be performed safely with laparoscopic and
robotic surgery. Preservation of the liver reserve
is one of the most important factors affecting survival and success in liver surgery. New horizons
have been opened in front of tissue-preserving
resections with three-dimensional imaging and
navigation systems.
In this section, the conditions related to prophylactic liver surgery will be discussed under
the heading of benign pathologies, malignant
pathologies and special conditions of the liver.
9.2 Benign Hepatobiliary
Pathologies
9.2.1 Hemangiomas
Hemangiomas are the most common benign liver
tumors. The vast majority of hemangiomas are
asymptomatic and are detected randomly. The
prevalence is reported to be 1–20% in the general
population [4–6]. It is 1–6 times more common in
women. They show multiple locations in 9–22%
of cases. Capillary hemangiomas are usually
small, peripherally located, and sometimes multiple. Cavernous-type hemangiomas appear less
and can reach larger diameters. Hemangiomas
larger than 5cm in diameter are called giant hemangiomas [4, 6–8]. Although most are asymptomatic, pain and abdominal discomfort are the most
common symptoms. Different degrees of thrombosis, calcication, and brosis can be observed
in large lesions (Fig.9.1) [6, 9]. Ultrasonography
is diagnostic but in suspicious cases, the diagnosis should be conrmed with contrast-enhanced
USG (CEUS), CT, and MRI.Contrast-enhanced
MRI is the most important determinant in differential diagnosis. Sensitivity and specicity are
more than 90% [10].
There is no direct relationship between the size
of hemangioma and complications. However,
there is a relationship between the characteristics
of the lesion and clinical symptoms. The size of
the hemangioma may increase during pregnancy
Fig. 9.1 The specimen and cross-sectional surface of our patient undergoing right hepatectomy due to giant hemangioma and thrombocytopenia
9 Prophylactic Surgery forLiver Pathologies
87
or with estrogen therapy. Conter and Longmire
(1988) stated that they believe that estrogen therapy contributes to the development and growth
of hemangioma. However, the exact mechanism
of hormonal effect has not been adequately claried [7, 8, 11]. This raises the question that prophylactic surgery should be performed in the
women population who want to become pregnant and have hemangioma. However, the literature data are limited in this regard. It is reported
that estrogen can cause an increase in the size
of the lesion, but spontaneous rupture rates are
similar for pregnant and nonpregnant women. In
the literature, patients who underwent enucleation due to lesion enlargement and pain that
reached 10cm in diameter 1year after birth were
reported [12].
In the follow-up of patients, it should be
decided by looking at the size and location of
hemangioma with clinical ndings and imaging [10]. Asymptomatic patients are monitored
and prophylactic surgery is not recommended.
Patients’ personal ndings, location, and features
of hemangioma should be evaluated together.
It is recommended to evaluate patients with
symptomatic (abdominal pain and compression symptoms) and lesion diameter >10cm by
a multidisciplinary team (hepatologist, hepatobiliary surgeon, interventional radiologist,
and pathologist) [6, 9]. According to the EASL
guide, diagnosis and follow-up with USG is sufcient for 3 cm lesions in healthy individuals.
They can use pregnancy or oral contraceptives.
In oncology or liver patients, differential diagnosis should be made with CT or MRI.In typical
cases, monitoring is sufcient. Patients with clinical or compressive symptoms and developing
Kasabach–Merritt syndrome should be evaluated
with a multidisciplinary approach [6].
Curry et al. (2020) recommend performing magnetic resonance imaging (MRI) within
6–12months when the diameter of the lesion is
>5cm. They reported that lesions with a growth
rate of ≤3mm per year were monitored for up
to 2 years, if the lesion was growing less than
3 mm per year, and there was no need for further imaging of MRI in 6–12months, if the lesion
appeared stable. If the lesion continues to grow
more than 3mm per year, the patient should be
evaluated by a multidisciplinary team for prophylactic surgery [10].
The rst complication that comes to mind
about hemangiomas was bleeding. There is no
clear information about the risk of hemangiomas
spontaneous bleeding. In a hundred-year literature review by Donati etal. (2011), they reported
that rupture was reported in 97 hemangioma
cases, 46 of which were spontaneous and 51 were
non-spontaneous [9]. Non-spontaneous ruptures
are more common in people under 40 years of
age. The bleeding risk of symptomatic hemangiomas is calculated as 1–5%. It is stated that
ruptures can be of any diameter (1–37 cm),
but hemangiomas with an average diameter of
11cm and above have a higher risk of bleeding.
Mortality risk due to rupture bleeding was calculated as 35–75% [9, 13, 14].
The preferred surgical methods for the treatment of hemangiomas are liver resection or
enucleation [15, 16]. There is no consensus on
the optimal treatment of large hemangiomas. The
rupture of hemangiomas appears to be a very
exceptional case, and prophylactic resection is
not recommended in asymptomatic cases [5].
Prophylactic surgery can be performed in cases
with clinical symptoms, leading to consumption coagulopathy (consumptive coagulopathyKasabach- Merritt syndrome), or patients with
pressure symptoms or larger than 10cm in diameter [9]. The procedure to be performed may vary
depending on the location and size of the lesion.
In appropriate cases, enucleation may be the rst
method of choice.
Preoperative transarterial embolization can
be used to reduce the lesion size in cases where
resection may be difcult by location and in
patients with a diameter of 10cm. Arterial embolization may reduce the risk of bleeding in surgery [9, 13, 14, 17, 18]. It has been also reported
that hemorrhage in the ruptured hemangioma
can be controlled with the same method [19,
20]. Although enucleation has been reported as
preserving more hepatic parenchyma and reducing postoperative complications than anatomical
liver resections, the appropriate approach to the
patient, the surgeon’s preference and experience,
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O. N. Dilek et al.
and the location of the lesion are also determinative in the form of treatment [15].
9.2.2 Hepatocellular Adenomas
Hepatocellular adenomas are rare, solid, and
benign liver lesions. They are seen most commonly
in women. The incidence is 1/1,000,000years old
and the risk increases 30–40 times in those using
long-term oral contraceptives. Adenomas are
usually unique and rarely can be multiple. The
use of estrogen-containing oral contraceptive
drugs in young women has an important place in
the etiology. Lesions regressed after discontinuation of the drug. In addition, patients with glycogen storage disease or metabolic syndrome have
a higher risk of developing adenoma [4, 21].
The greatest risk for adenomas is the possibility of rupture. However, the estimation of
rupture incidence has been reported from 25 to
64% in the literature, although it is difcult, as
it is mostly obtained from data of symptomatic
patients. Risk factors for bleeding include large
lesion (>5 cm), hormone use, pregnancy, exophytic and subcapsular location, and histopathological subtypes [22, 23].
The transformation risk of adenomas into
hepatocellular carcinoma has been reported
between 0 and 18% in the literature [21]. The
risk of malignancy is higher in male sex (10 times
greater), and in patients with lesions greater than
5 cm, height of AFP and activation of betacatenin histopathologically [21, 22, 24].
Asymptomatic patients can be followed, but
despite all precautions, considering spontaneous
rupture and malignant transformation in lesions
>5 cm in size, patients are candidates for prophylactic surgery and should be evaluated with a
multidisciplinary team [25].
9.2.3 Focal Nodular Hyperplasia
Focal nodular hyperplasia (FNH) is a benign
liver lesion caused by the proliferation of hyperplastic hepatocytes around a central star-like
scar. Focal nodular hyperplasia is the second
most common benign solid tumor of the liver,
with an estimated prevalence of 2.5–8% [
4, 26].
Typically, FNH is more common in women, and
female sex hormones are the biggest risk factors
for FNH [27, 28].
They are generally asymptomatic since they
are located peripherally. The prognosis for FNH
is generally excellent because the lesion is mostly
stable or may regress over time. Complications
such as bleeding and compression are rarely
reported, and malignant transformation has
not been reported [29–32]. In laparotomies
performed for other reasons, peripheral locations can be removed for differential diagnosis.
Prophylactic surgery is not recommended in
patients with denitive diagnosis.
9.2.4 Cystic Lesions
Cystic lesions of the liver can be simple,
tumoral, infectious, hemorrhagic, and traumatic.
Differential diagnosis is important for determining the treatment. Simple cysts of the liver are
clear uid-containing cystic formations that
are not related to the intrahepatic biliary tract.
Although simple cysts are present in about 1%
of the population, very few grow and even less
cause symptoms [33]. Simple cysts tend to occur
more commonly in the right lobe and are more
prevalent in women. The female-to-male ratio is
approximately 1.5:1 among those with asymptomatic simple cysts, while it is 9:1in those with
symptomatic or complicated simple cysts. Huge
cysts are found almost exclusively in women
over 50years of age [34]. Rarely, their size can
range from a few millimeters to massive lesions
occupying the upper abdomen [35]. It is sufcient to monitor most of the simple diagnosed
simple cysts. Simple and large volumes of simple
cysts can cause tension, pain, and pressure symptoms (Fig.9.2). Most simple cysts can be treated
with aspiration and sclerotherapy. Prophylactic
surgery is rarely required, and the most appropriate option is unroong. Prophylactic unroong
can be performed to reduce tension and pressure
in the presence of common cystic lesions in the
liver, kidney, and pancreas [36].
9 Prophylactic Surgery forLiver Pathologies
89
a bab
Fig. 9.2 Axillar CT section (a) with multiple liver cysts (stars) and the appearance of cysts in our patient who under-
went laparotomy for unroong (b)
Cystic lesions with malignant character (cystadenomas) can be confused with benign cysts.
Malignant degeneration should be suspected in the
presence of wall irregularities, solid components,
and septations [33]. Treatment of tumoral cystic
lesions is denitive surgery. When mucinous cystic
neoplasia or other rare cystic neoplasia is suspected,
it should be evaluated with a multidisciplinary team.
9.2.5 Caroli’s Disease
Caroli’s disease is a congenital anomaly of the
biliary tract of the liver, characterized by multifocal saccular dilatations in the biliary tract. Also
known as a type 5 biliary tract cyst. It is a disease characterized by cholangitis due to stasis
and stones formed in vesicles in the bile duct.
It has a hereditary transition feature. It can hold
the entire liver or be limited to the sector or lobe.
In medical treatment, ursodeoxycholic acid is
used. However, antibiotic and supportive therapy
and endoscopic interventions may be required
in recurrent cholangitis attacks [33]. Due to the
chronic inammatory process of the disease,
hypoproteinemia and developmental retardation
may occur. Cholangiocarcinoma develops in
7% of patients. Liver transplantation can be performed in diffuse lesions. In cases involving the
lobe or sector, lobectomy is sufcient [4].
9.2.6 Biliary Atresia
Biliary atresia is the most common cause of jaundice of the newborn requiring surgical treatment.
In patients with biliary atresia, which is usually
diagnosed in the months after birth, bile drainage
should be performed rapidly in order to prevent
liver damage and cirrhotic process (see Chap. 11).
Roux-en-Y type hepatoportoenterostomy (HPE)
is the standard treatment method in the treatment
of biliary atresia. HPE can be applied with laparoscopic and open surgery. However, complications such as stula and stenosis developing in the
early postoperative period are important causes
of morbidity and mortality [37]. In clinical studies, it is recommended to perform HPE primarily
in patients with biliary atresia, since prophylactic liver transplants to be performed in the early
period do not achieve the desired success due to
the small size of the baby and technical difculties. In the follow-up of HPE cases, liver tissue is
within normal limits in approximately one-third
of the cases after 4years. Approximately half of
the cases require liver transplantation due to stenosis in the biliary tract or liver failure [38]. It is
recommended that corrective restoration attempts
to be performed in cases with stenosis are not successful, and liver transplantation should be performed instead of corrective procedure due to the
growth of children [39].
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9.3 Malign Liver Pathologies
9.3.1 Hepatocellular Carcinoma
Hepatocellular carcinoma (HCC) is the second
most common cause of cancer-related deaths
worldwide [40]. In cases with HCC, the survival
time is shortened due to the rapid progression
of the disease or the cirrhosis to decompose.
Especially in patients with high risk of recurrence or early recurrence after resection, prophylactic liver transplantation can provide a
longer survival. Yang etal. (2016) reported that
longer survival can be achieved with prophylactic liver transplantation in selected cases with
gene expression, microvascular invasion, poor
differentiation, and presence of microsatellite
lesions [40, 41].
9.3.2 Simultaneous Lesions
About half of malignant tumors in the liver are
metastatic lesions. The most common metastases
are colorectal, breast, and bronchial origin. In
10–25% of cases with colorectal cancer, there is
liver metastasis when diagnosed [42, 43]. In the
follow-up of patients, more than half of the cases
(50–75%) develop synchronous or metachronous
liver metastasis. The chance of resection can be
obtained in 10–40% of patients with metastases
undergoing chemotherapy [43–45]. There are
different opinions about the timing and treatment
of metastatic liver tumors. In the conventional
approach, resection of the metastases is recommended. Some of the resections can be synchronized with the colorectal process, as well as before
or after the lesion location, number, and size. In
the literature, studies report that there is no statistically signicant difference between simultaneous and staged resections and it can be performed
in appropriate cases [46]. In a multicentric study
conducted in Korea, 3-year survival was reported
to be better in the two-staged surgery group than
in the simultaneous surgery group [47]. In the
clinical series of 226 cases of Nanji etal. (2017),
they reported that the operation performed in the
liver was more limited (number, size, and width
of the resection) in the patient group undergoing
synchronous resection [48].
Besides neoadjuvant chemotherapy increases
the chance of surgery, there are opinions that
the damage caused by the liver (steatohepatitis,
sinusoidal obstruction syndrome, remnant liver
insufciency, etc.) negatively affects morbidity
and mortality [49]. However, there are also studies indicating that it contributes to the reduction
of the number and diameter of metastatic lesions
with neoadjuvant chemotherapy, increases operability, and does not have a negative effect on
morbidity and mortality [50, 51]. In recent years,
better results have been achieved with effective
chemotherapy protocols. Pathological complete
recovery (CPR) is very low (4–11%) in patients
undergoing neoadjuvant chemotherapy protocols, but this rate rises to 35–50% in patients who
are considered to have complete clinical recovery (CCR) [43]. Gustavsson (2012) reported that
he applied oxaliplatin or irinotecan protocols
with uorouracil, recommended in the NCCN
guidelines, and did not recommend performing
prophylactic resection in patients with colorectal cancer (if there is no perforation, obstruction,
and bleeding). He also states that the diameter
and number of liver metastases have decreased in
patients receiving chemotherapy and their incidence decreased to 10% [42]. However, it should
be kept in mind that as a result of the examination of the lesions that are regressed or thought
to be lost after chemotherapy, complete resection
can be achieved in 20% of the cases, live tumor
cells are not completely eliminated, and this may
pose a signicant risk for relapse, survival, and
follow- up [44].
There are different results regarding tumorfree margin in the resection of metastases. While
it has been advocated to remove metastases with
1cm of normal liver tissue in the past years, it
is now reported that metastases can be removed
with 1 mm tumor-free margin. Besides, it has
been reported that metastasectomies performed
by separating the metastatic mass from vascular
structures adjacent to its anatomical border also
have a positive effect on survival [52–54]. There
are also studies reporting that there is no difference between new chemotherapy regimens and
9 Prophylactic Surgery forLiver Pathologies
91
R0 and R1 resections [52, 55, 56]. On the other
hand, the presence of tumor-specic mutant DNA
up to 4mm in liver tissue around metastasis has
been demonstrated [57]. Resections with wider
normal liver tissue should be preferred in appropriate cases to reduce the risk of local recurrence
and protection.
9.3.3 Incidental Solitary Lesions
Incidental solitary liver lesions are detected more
with increasing use of radiological imaging methods. Most of them are asymptomatic and benign,
and they are detected incidentally in radiological
scans. Denitive diagnoses are usually made and
further investigations are rarely needed. In cases
where incidental lesions are detected, whether
the patient has a history of cirrhosis, hepatitis,
and malignancy should be questioned rst [58].
Surgical resection is rarely required to diagnose
incidental solid liver lesions, but it can be performed for lesions of uncertain etiology, which
are symptomatic. If the possibility of HCC cannot be excluded despite the absence of risk factors
(cirrhosis, chronic liver disease, etc.), prophylactic surgical resection can be performed for riskreduction method and histological verication
[59]. It is recommended to evaluate the patients
by a multidisciplinary team (hepatologist, hepatobiliary surgeon, interventional radiologist, and
pathologist).
9.4 Miscellaneous Conditions
9.4.1 Portal Hypertension
Portal hypertension is a pathology that usually
develops as a result of cirrhosis, schistosomiasis, or
extrahepatic portal vein thrombosis. Portal hypertension is a result of increased resistance to portal
blood ow and can lead to complications such as
variceal bleeding and ascites. One-third of patients
with cirrhosis develop variceal bleeding, which is a
major cause of morbidity and mortality [60].
Numerous prophylactic procedures have been
described to prevent cirrhosis-related compli-
cations [61, 62]. In addition to beta blockers,
endoscopic band ligation and sclerotherapy are
used in emergency, elective, or prophylactic
treatment of esophageal varices (see Chap. 13).
AGA guidelines are used today to prevent bleeding of esophageal varices [60]. More than 90%
of cases are successful with a medical and endoscopic approach. However, the bleeding recurs in
60–70% of cases within 2years after the index
bleeding [63, 64].
Randomized studies comparing surgical portocaval shunts with medical therapy in cirrhotic
patients were conducted in the 1970s. Although
surgery is highly effective in preventing variceal
bleeding and ascites production, new problems
such as high risk of complications and encephalopathy are encountered after surgery [60, 65,
66]. For this reason, it has been determined that
prophylactic portocaval shunts do not show the
expected benet, and deaths due to liver failure
are higher than esophageal variceal hemorrhages
and these techniques have started to be applied
in selected cases. Apart from the shunts, there
are other options (non-shunt) such as terminal
esophagoproximal gastrectomies, esophageal
transections, and Sugiura procedure to prevent
esophageal variceal bleeding. These procedures
can be performed for therapeutic purposes as
well as for prophylactic purposes [67].
In patients with non-cirrhotic portal hypertension, it has been shown to be the opposite of the
situation, and deaths due to esophageal variceal
bleeding are higher [68]. Pal etal. (2005) showed
that prophylactic distal splenorenal shunts to be
performed in patients with non-cirrhotic portal
brosis are successful in preventing bleeding,
and the symptoms of splenomegaly, ascit, and
hypersplenism regressed in most patients [68].
Transjugular intrahepatic portosystemic stent
shunting (TIPS) is another method used in bleeding prophylaxis in patients with portal hypertension [69]. Sinusoidal and portal decompression
can be achieved with TIPS without the risk of
general anesthesia. In the AGA guideline, TIPS
is recommended as the rst option when medical and endoscopic approaches fail [60]. This
technique has made mechanical portal decompression popularized again in the treatment of
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O. N. Dilek et al.
portal hypertension. However, there are no data
supporting the use of TIPS for primary prophylaxis of variceal bleeding [60]. Considering the
insufcient data and TIPS-related complications
(high risk for hepatic encephalopathy), it is not
recommended for primary prophylaxis of variceal bleeding [60, 70].
Extrahepatic portal vein occlusion (EHPO)
is a disease characterized by portal hypertension,
severe esophageal variceal bleeding, and splenomegaly. Liver functions are usually within normal
limits. Non-cirrhotic portal is the most important
cause of hypertension in children. There is very
little data on medical and endoscopic approaches
in the treatment of extra venous obstructions
in children, and surgical prophylaxis is recommended [61]. For this purpose, Meso-Rex bypass
is recommended [62]. Pal etal. (2013) performed
prophylactic splenorenal shunt in 98 cases and
esophagogastric devascularization in 16 cases in
their 114 case series.
Cirrhosis is the most important cause (80%)
of ectopic varicose veins detected in the small
intestines, colon, and rectum, other than the
esophagus. The rst treatment option to be used
for prophylactic or denitive treatment is endoscopic approaches such as band ligation and
sclerotherapy [71].
9.4.2 Locally Invasive Gallbladder
Tumors
In 0.3–3% of cases undergoing laparoscopic
cholecystectomy, gall bladder carcinoma is
detected. In T1a cases, laparoscopic cholecystectomy is sufcient (see Chap. 11). The risk
of vascular and perineural invasion increases
in the lesions reaching the subserosis, and
lymph node involvement is detected in approximately half of the cases [72]. In T1b cases,
radical cholecystectomy should be performed.
In T2 cases, segment 4B and ve parts are
included in the resection. Kwon etal. (2020)
stated that there was no signicant difference in survival between segment 4–5 resection and wedge resections in the T2 series. In
the same series, survival in T2a (peritoneal
side) lesions was found to be better than T2b
(hepatic side) lesions [
73]. The cystic canal
stump sampling should be done with frozen
section. Extrahepatic biliary tract resection
and regional lymph node dissection are also
performed in cases with tumor-positive results.
Combination therapies with multidisciplinary
approach should be applied in T3 and T4
cases. In T3 cases, Caudate lobe resection and
lymph dissection and resection in the affected
tissues should be performed in addition to the
extended right or left hepatectomies [74].
9.4.3 Hydatid Cyst
Echinococcus granulosus and Echinococcus
multilocularis (alveolaris) cause hydatid
cysts and alveolar hydatid cysts in humans.
Perforation of the cyst into the biliary tract is
the most common complication in patients
with hydatid cyst. Cysts that are stulized to
the biliary tract change the treatment algorithm. In these patients, the results obtained
after sphincterotomy and stenting with ERCP
will determine the extent of the operation to be
performed. Another important cause of morbidity and mortality encountered after hydatid
cyst surgery is the development of a bile stula
(leak). In cases where bile stula is detected,
endoscopic sphincterotomy, stent application,
or nasobiliary drainage can prevent the pressure
in the biliary tract and ow of bile into the cyst
cavity. In patients who underwent prophylactic
endoscopic sphincterotomy, stula incidence
decreases, and the duration of hospitalization is
shortened [75, 76]. Çiçek etal. (2007), in a study
involving 69 patients who underwent hydatid
cyst surgery and developed bile stula, reported
that all patients were successfully treated with
endoscopic sphincterotomy and stenting [75].
Alveolar hydatid cysts form a lesion that covers the liver in a period of approximately 20years
and disrupt functions with signs of compression
[77]. Prophylactic resections can be performed in
cases where partial control can be achieved with
the use of albendazole or liver reserve is sufcient (Fig.9.3).
9 Prophylactic Surgery forLiver Pathologies
93
ab c
Fig. 9.3 In this picture, CT image (a) and surgical specimen (b, c) of our patient who had a right hepatectomy 23years
ago due to an alveolar hydatid cyst are still living healthy
9.4.4 Liver Traumas
The liver is the most frequently injured intraabdominal organ. In hemodynamically stable injuries, most of the cases will recover with close
follow-up of the patient (Laboratory, USGFAST, and CT). In 50–85% of cases with liver
trauma, bleeding stops spontaneously [78–80].
Complications such as hemobilia, hematoma, and
biloma that may be encountered in the healing
process can be successfully treated with interventional radiological procedures. Angiography and
embolization can also contribute to diagnosis and
treatment in liver injuries.
Approximately 14% of patients with liver
injuries require surgical intervention [80, 81].
Operative management of liver injuries in severe
injuries can be difcult even for experienced surgeons due to the complex nature of the liver, its
size, vascularity, blood supply, and hard-to-reach
venous drainage. The aim of the surgeon should
primarily be to provide hemodynamic stabilization of the patient. A denitive procedure can be
applied in experienced centers. However, shortterm Pringle maneuver can be performed to control bleeding in severe liver injuries detected in
patients who are hemodynamically unstable and
laparotomized. In case of lack of experience,
bleeding can be temporarily taken under control
by packing. At the time gained, the patient can be
transferred to an experienced center or depacking
after 24–48h.
After liver injury and other intra-abdominal
injuries are managed, the abdomen can be closed,
but open abdominal management should be kept
in mind as a risk-reducing method due to the risk
of abdominal compartment syndrome and the
need for a second look.
9.4.5 Hepatolithiasis
Hepatolithiasis is an endemic entity in many
countries, especially in the Far East. Its incidence
varies between 4 and 52% [82]. In some countries, it is a serious public health problem due to
its causes and consequences. Stone formation
in the liver can occur in many cases where bile
ow is blocked or slowed. Chronic inammation develops as a result of recurrent cholangitis
attacks in patients who develop bile duct stenosis and cholestasis, and this causes hyperplasic
changes in the mucosa. With the prolonged
inammatory process (10–20 years), cholangiocarcinoma develops in 10% of cases [83]. In
the treatment of hepatolithiasis, rst endoscopic
methods and medical approaches should be
applied. Prophylactic liver resections (Fig.9.4)
or biliary drainage procedures should be performed when treatment is inadequate [82].
9.5 Miscellaneous Procedures
9.5.1 Transplantation
Liver transplantation is one of the procedures
that have been standardized today and successfully performed by transplantation surgeons in
hepatobiliary centers. Liver transplantation can
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