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O. N. Dilek and N. Acar
Since both entities are quite rare, PC for their asymptomatic onset is not well established. Nevertheless, the removal of the additional section during the index surgery should not be forgotten in order to avoid recurrence [119]. Hourglass shaped can be seen with adenomyomatosis of gallbladder, and PC is recommended due to the high cancer risk of adenomyomatosis [120].
11.2.11 Carrier forBacterial Agents
Salmonella Typhi (S. Typhi) and Salmonella Paratyphi A (S.Paratyphi A) which are the bac-
terial agents of enteric fever can stay asymp­tomatic in gallbladder in 2–5% of the infected individuals [121]. These patients, which are dened as carriers, may not manifest any signs of disease, but still spread the bacteria by its fecal–oral route. Chronic carriage for S.Typhi is also related with gallbladder cancer due to chronic inammation [122]. PC is a feasible approach when carriage for S. Typhi is con­rmed in order to prevent both the spread of the infectious disease and the malignant trans­formation. However, it was also reported that cholecystectomy may not certainly eradicate the disease since the bacterial colonization can persist even in biliary tract, mesenteric lymph nodes, and liver [123].
11.3 Biliary Tract
Conditions of the biliary tract requiring pro­phylactic surgery are mostly related with the conditions requiring PC. Various preneoplas­tic pathologies may occur as a result of chronic inammation and mucosal damage resulting from some congenital or acquired pathologies of the biliary tract. In this part, some preneoplastic and non-neoplastic conditions are going to be briey re-mentioned as well as the conditions specic to biliary tract are going to be explained in detail.
11.3.1 Pancreaticobiliary
Maljunction
Pancreaticobiliary maljunction leads to biliary duct cancer, with the previously mentioned mech­anism, in 3.1–4% and 6.9–11% of the patients without and with biliary dilatation, respectively [84, 85]. Therefore, bile duct dilatation is the key point to determine the treatment approach. In cases with dilated bile duct, complete excision of the dilated extrahepatic bile ducts in addition to PC is recommended. Roux- en- Y hepaticojeju­nostomy or end-to-side hepaticoduodenostomy can be performed as the reconstructive tech­niques [124].
On the other hand, treatment approach in cases without biliary dilatation is controversial. Although majority of the studies have advocated that sole PC is adequate for the cases without biliary dilatation since bile duct cancer is not as frequent as gallbladder cancer, some studies have proposed an opposing opinion. Precancerous lesions, which were often not detected with pre­operative imaging, were found in 73% of non­dilated bile duct cancers with pancreaticobiliary maljunction [125]. Additionally, recent studies showed that histological changes on the epithe­lium of non-dilated bile ducts were similar to dilated bile ducts [124, 126]. For these reasons, prophylactic excision of the extrahepatic bile duct is introduced as a reasonable approach by some experts regarding the prevention of car­cinogenesis [87, 124]. However, prophylactic surgery for non-dilated bile duct in pancreatico­biliary maljunction should be evaluated carefully by considering the short- and long-term compli­cations of biliary surgery.
11.3.2 Choledochal Cyst
Choledochal cyst is a rare congenital condition which usually manifests during childhood and rarely in adults. Its incidence was reported as 1:100,000–150,000 in Western populations and 1:1000 in Asian populations. Pancreaticobiliary
11 Prophylactic Surgery forGallbladder andBiliary Tract Pathologies
maljunction is responsible for 50%–80% of the cases [127]. Besides causing cholangitis and pancreatitis episodes, it also carries a risk of malignant transformation just like pancreatico­biliary maljunction. Previous studies have shown that 10–30% of adults with choledochal cysts develop cholangiocarcinoma, and choledochal cysts increase the risk for cholangiocarcinoma 20–30 times higher than general population [128]. A meta-analysis of 18 studies by ten Hove etal. (2018) showed that malignancies may develop in up to 11% of patients with choledochal malfor­mation, and treating choledochal malformation
Fig. 11.4 This picture shows the specimen of a patient with a type 4 choledochal cyst
may prevent developing malignancy. In addition, no differences in the prevalence of malignancy between the different types of choledochal mal­formation were found [129].
ried on in patients with choledochal cysts even after surgical interventions.
Treatment choices vary according to the clas­sication introduced by Todani et al. (1977) [130]. Type I and type IV cysts have higher inci-
11.3.3 Biliary Intraepithelial
dence for cancer, while type II and type III have a lower risk [128, 131]. 68% of cholangiocarci­noma are reported to be associated with type I cysts and 21% with type IV [132]. Therefore, less invasive techniques such as simple excision and endoscopic sphincterotomy are usually adequate for the treatment of type II and type III cysts, respectively, when the possibility of a concurrent cancer can be excluded.
Management of type IVA and V (Caroli’s disease) cysts has been controversial due to the involvement of intrahepatic ducts. Cyst excision and an additional wide hilar hepati­coenterostomy can be performed in type IVA (Fig.11.4). Cancer development is reported in 7–15% of patients with type V cysts (Caroli’s disease) [132]. Hepatic lobectomy inlocalized type V cysts (Caroli’s disease) can be preferred. However, since prophylactic liver transplant is not a feasible approach in asymptomatic cases, close surveillance for cancer development is recommended instead [133].
Despite all these preventive efforts, there is 1% risk of cancer development following the cyst excision [134]. Lifelong follow-up should be car-
Biliary intraepithelial neoplasia (BilIN) develops as a result of chronic inammatory processes such as primary sclerosing cholangitis (PSC), hepatolithiasis, choledoc cyst, chronic hepatitis B and C, and alcoholic cirrhosis. Patients with PSC have a high risk (>160 times) of developing cholangiocellular carcinoma. Cancer develops in
0.5–1.5% of patients with PSC each year, and the risk of developing lifelong cancer is calculated as 15–20% [135]. It was determined that BilIN developed in 10% of cases with hepatolithiasis. BilIN is a precursor lesion of cholangiocarcinoma and represents three different degrees of dysplas­tic changes of the epithelium. Although its prog­nosis is not clear, prophylactic surgical resection with PC should be performed when detected, in order to remove the risk of cholangiocarcinoma at an early stage. Detection of BilIN in surgi­cal margins is an important problem in patients undergoing resection [132, 136].
Patients with Lynch syndrome have an increased risk of developing cholangiocarci­noma. Multiple primary papillomatosis is another
125
Neoplasia (BilIN)
126
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O. N. Dilek and N. Acar
genetic disease, characterized by precancerous papillomatosis of the mucosa [132].
11.3.4 Intraductal Papillary Neoplasia oftheBile Duct (IPN-B)
Intraductal papillary neoplasia of the bile duct (IPN-B) is another rare entity that constitutes 10–15% of bile duct tumors [137]. It has three subtypes which are thought to be premalignant lesion of cholangiocarcinoma: adenoma, border­line tumor, and carcinoma in situ [87]. Complete surgical excision is the best treatment modality in cases without distant metastasis [138]. Surgical technique depends on the location of the tumor. Since the main goal is to achieve R0 resection, additional hepatectomy or pancreaticoduodenec­tomy should be considered in proximal and dis­tant tumors, respectively.
11.3.5 Locally Invasive Gallbladder Cancer
Malignant cells in gallbladder cancer can spread through lymph vessels in the submucosal layer of the common bile duct, in addition to the large lymph vessels in subserosal layer [139]. With this concept, efcacy of the resection of extra­hepatic bile duct in gallbladder cancer has been researched in many studies. In general, routine resection of the extrahepatic bile duct is not rec­ommended in patients who have no involvement and have a negative cystic duct margin since major hepatic and biliary resections increase morbidity [140, 141]. Sakamoto etal. (2006) pro­posed resection of extrahepatic bile duct when perineural invasion exists, even in the absence of biliary inltration [142]. Chikamoto etal. (2009), on the other hand, advocated en bloc resection of the extrahepatic bile duct in curative resection even for T2 gallbladder cancer due to the lym­phatic spread through submucosal layer [139]. In several different studies, prophylactic extrahe­patic bile duct resection in patients without mac-
roscopic bile duct invasion has shown to have no survival impact [143, 144].
Laparoscopic cholecystectomy is the rst treatment method in patients with cholelithia­sis. However, in cases that are thought to have gallbladder carcinoma; it is recommended to perform surgery with laparotomy in terms of increased risk of port site metastasis (11–16%), gallbladder perforation risk (20%) and associ­ated tumor spread, and presence of invasion and regional lymph tissue dissection [73, 145, 146]. Because of the risk of tumor, specimens should be removed in a special bag.
In 1% of cases undergoing laparoscopic cho­lecystectomy, gall bladder carcinoma is detected. In T1a cases, laparoscopic cholecystectomy is sufcient. 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 [147]. In T1b cases, radical cholecystectomy should be performed. For this purpose, partial liver resec­tion and port site resection should be performed, including a tissue 3cm deep from the liver seg­ments 4B and 5. On the contrary, NCCN does not recommend prophylactic port site resection, since it is not associated with improved survival or recurrence [76]. In T2 cases, segment 4B and 5 parts are included in the resection. From the cystic canal stump, sampling is done with fro­zen section. Extrahepatic biliary tract resection and regional lymph node dissection are also per­formed in cases with tumor-positive results [73].
Conditions that require liver resection and vas­cular resection in order to increase survival and success due to biliary tract tumors are described in the relevant section (See Chap. 9; Liver).
Prophylactic surgery of the gallbladder and biliary tract targets to eliminate various risks which eventually impair the quality of life. Since there are still many controversial issues in terms of indications, it is vital to follow actual literature and guidelines on this topic. Biliary tract opera­tions should be performed in centers with high volumes, sufcient technical equipment, and experienced surgeons due to high morbidity and mortality risks.
11 Prophylactic Surgery forGallbladder andBiliary Tract Pathologies
127
11.3.6 Biliary Atresia
It is an idiopathic, progressive, brous obstruc­tive neonatal disease of the biliary tract. The incidence is one in 10–20 thousand births. It is the most common cause of the yellowness of the newborn requiring surgical treatment. It may be accompanied by other organ anomalies and mal­rotations. In the etiology of the disease, viruses, toxins, genetic mutations (CFC1 gene, PKD1L1 gene), and immunological disorders have been blamed [148]. In patients generally diagnosed in the months after birth, bile drainage should be performed rapidly in order to prevent liver damage and cirrhotic process. 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 [149]. In studies conducted, it is recommended to perform HPE primarily in patients with biliary atresia, since the pre­prophylactic liver transplants to be performed in the early period cannot achieve the desired suc­cess due to technical difculties (See Chap. 9; Liver). Following HPE cases, liver tissue is within normal limits in approximately one-third of the cases after 4years. Approximately half of the cases require liver transplantation due to ste­nosis in the biliary tract or liver failure [150]. It is recommended that corrective restoration attempts in cases with stenosis are not successful, and liver transplantation should be performed due to the growth of children [148].
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Prophylactic Splenectomy
NuruYusifogluBayramov, RuslanAydınogluMammadov, andFarahAlqızıGahramanova
12
12.1 Introduction
Splenectomy is performed for the palliative and radical treatment of primary splenic diseases, extra­splenic diseases, and splenomegaly. The rst sple­nectomy was performed by Andirano Zaccarello in 1549 on a woman with massive splenomegaly. The rst successful splenectomy for hematological disorder was performed by Quittenbaum in 1826 [1]. In 1893, Reigner reported the rst successful splenectomy for splenic rupture [2]. Since the rst report of laparoscopic splenectomy by Delaitre and Maignen in 1991, laparoscopic approach has become a standard procedure for elective splenec­tomy [3]. The rst laparoscopic splenectomy in children was performed in 1993 by Tulman [4].
12.2 Splenectomy
Classically, splenectomy is performed for ve main purposes: to stop bleeding, to extend the lifespan of the pathologic blood cells, in the treatment of the complications of splenomegaly, to remove the splenic masses and sources of dis­eases located in the spleen, and to establish the diagnosis. Indications for splenectomy can be divided into three groups: primary, secondary,
N. Y. Bayramov · R. A. Mammadov (*) F. A. Gahramanova Department of General Surgery and Transplantology, Azerbaijan Medical University, Baku, Azerbaijan
and controversial (Table12.1). The primary indi­cations consist of the cases, in which there is no other alternative; splenectomy is the only effec­tive treatment method. In secondary indications, splenectomy is an effective treatment method, but there are also other alternatives. In these cases, splenectomy should be selectively performed when other treatment methods are ineffective. Controversial group indicates the cases in which the effectiveness of splenectomy is not always high and splenectomy is considered as last resort.
The prophylactic splenectomy is to remove healthy or uncomplicated enlarged spleen to alleviate the clinical course of splenic and extra­splenic diseases and to prevent disease recur­rence and complications of splenomegaly.
Indications for prophylactic splenectomy are not dened precisely and quite controversial. The splenectomy indications are as follows:
– Splenic echinococcosis. – Gastric cancer. – Cytoreductive surgery for ovarian cancer. – HCC treatment. – LDLT (living donor liver transplantation). – Distal pancreatectomy. – Chronic pancreatitis. – Wandering spleen (mobile spleen). – Splenic vein thrombosis (Banti syndrome). – Hematological diseases. – Sickle cell anemia. – Splenic artery aneurysm. – Cirrhosis.
© 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_12
133
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