Figure 10.1 Evolution of cholecystectomy.

10.3.1  Haemolytic disorders

Patients with haematologic disorders are at increased risk of gallstone formation. These disorders include sickle cell anaemia, thalassaemia and hereditary spherocytosis [11]. The individual incidence of gallstones varies from each disorder. Patients with sickle cell disease (SCD) have an incidence of gallstones ranging from 12% to 42%, with the higher values typically seen in older patients [12,13]. In addition to gallstones, 16%–23% of SCD patients have the presence of biliary sludge [12,14]. Patients with hereditary spherocytosis also have a high incidence of concomitant gallstones, ranging from 5% to 40% [15,16] and with coexisting Gilbert syndrome further increasing the likelihood of gallstone formation, and presentation at a younger age [16].

Table 10.1 Operational care of the child with SCD

Source:  Tobin JR, Butterworth J, Anesthesia and Analgesia 2004, 98: 283–284; Ohene-Frempong K, Seminars in Hematology 2001, 38: 5–13; Koshy M, et al., Blood 1995, 86: 3676–3684; Howard J, et al., Lancet 2013, 381: 930–938; Vichinsky EP, et al., New England Journal of Medicine 1995, 333: 206–213; Platt OS, et al., New England Journal of Medicine 1994, 330: 1639–1644; Buck J, Davies SC, Hematology & Oncology Clinics of North America 2005, 19: 897–902; Marchant WA, Walker I, Paediatric Anaesthesia 2003, 13: 473–489.

SCD children presenting for evaluation for cholecystectomy require special mention. A thorough preoperative evaluation remains a critical step in the evaluation and treatment of these patients. SCD patients are at risk of acute vaso-occlusive pain crises, acute chest syndrome and seizures, occurring in up to 20% [17–19]. Surgical stress further increases the risk of these events. Additionally, these children often have a history of multiple blood transfusions, leading to alloimmunisation and potential difficulties with finding compatible blood for these patients [20–22]. Lastly, many of these patients are immunocompromised due to clinical asplenism, a direct result of chronic splenic infarction, leaving them immunosuppressed [23,24]. The main risks are the development of acute chest syndrome, a significant cause of mortality in this group [17]. Table 10.1 illustrates a suggested perioperative preparation for a child with SCD [25–31].

Patients with SCD and repeat acute splenic sequestration crises may be primarily referred to a paediatric surgeon for a splenectomy [32,33]. Other haemoglobinopathies, such as thalassaemia or hereditary spherocytosis, may also necessitate a splenectomy in the treatment of the disorder [33–37]. Given the increased risk of cholelithiasis in all patients with chronic haemolysis, screening of the gallbladder and biliary tree is recommended prior to performance of the splenectomy [32,36,38]. If gallstones are present, a cholecystectomy is typically performed in conjunction with the splenectomy, irrespective of the absence or presence of symptoms. Several studies have shown that cholecystectomy can be performed concurrently with splenectomy safely, with low risk of complications in patients with haemolytic disease [39–41]. Given an increased risk of complications associated with a delay in cholecystectomy for symptomatic gallstones in patients with haemolytic disease, it is recommended that these patients should have their gallbladder removed at the detection of gallstones, irrespective of whether a splenectomy is indicated [42–45].

10.3.2  Nonhaemolytic disorders

TPN has been implicated in multiple studies as a risk factor for bile stasis and cholelithiasis in adults as well as children [46–48]. According to Roslyn et al., 43% of children receiving long-term TPN ultimately develop gallstones [47]. In this study, the average time on TPN for children with gallstone disease was 36 months, but stone formation was seen in patients on TPN as short as 8 months. However, in a separate study of children who received parenteral nutrition, the investigators found no statistical significant association with the duration of TPN and the development of cholelithiasis [49]. Rather, the statistically significant differences between those patients who developed cholelithiasis and those who did not were [1] increased number of abdominal operations, [2] loss of the ileocecal valve and [3] a diagnosis of short bowel syndrome. A recent review suggests that cholecystectomy should be performed in children with TPN-associated cholelithiasis, as these stones are unlikely to resolve spontaneously [10].

Cholelithiasis in neonates and infants is a rare phenomenon. Given the limited experience with this disorder, randomised studies are lacking, but some knowledge can be gleaned from the prior case report experiences and retrospective reviews. According to two case series, more than half of neonates and infants with gallstones experience spontaneous resolution of their gallstones or remain asymptomatic [50,51]. Only those infants that develop biliary colic or infectious complications require a cholecystectomy. Another retrospective review revealed that 2 infants of a total of 13 necessitated cholecystectomy [52]. of the remaining 11 infants, 1 had choledocholithiasis that resolved spontaneously and 10 remained asymptomatic. Interestingly, five of the asymptomatic patients experienced resolution of their gallstones after an average follow-up of 1.8 years. Given the lack of randomised controlled trials, we are left to rely upon these case reports and retrospective reviews on the topic. From such data, we see that some cases of neonatal and infantile cholelithiasis can be managed expectantly unless infectious complications or other symptoms develop.

Acalculous cholecystitis is a disorder that should be considered in any child with critical illness, especially those patients with severe sepsis, trauma or burns that subsequently develop pain in the epigastrium or right upper quadrant. These will not have gallstones demonstrated on ultrasound (US) but may have evidence of gallbladder wall thickening or distention, with possible sludge. Acalculous cholecystitis is associated with a high mortality, up to 50% in some series [53]. The preferred treatment for acalculous cholecystitis is bowel rest and intravenous antibiotics, in combination with decompression and drainage with a cholecystotomy tube [54–57]. Interventional radiologists can place an US-guided percutaneous cholecystostomy tube at the bedside with few complications [55–58]. Finally, interval cholecystectomy after percutaneous cholecystostomy has been indicated in those treated for calculous cholecystitis whose illness was too severe to undergo primary cholecystectomy. However, an interval cholecystectomy does not seem to be indicated in those patients treated for acalculous cholecystitis [57,59].

Finally, biliary dyskinesia is a disease that is increasingly recognised as an indication for cholecystectomy in the paediatric and adolescent populations. It is theorised that biliary dyskinesia results from a lack of coordination between gallbladder contractions and the relaxation of the sphincter of Oddi [60]. Biliary dyskinesia often presents with non-specific epigastric or right upper quadrant abdominal pain associated with nausea and vomiting, normal laboratory results and a normal right upper quadrant Doppler US. If a subsequent gallbladder emptying study reveals a gall-bladder ejection fraction of <35%, biliary dyskinesia can be entertained as the diagnosis [4,61]. Unfortunately, up to 40% of those with gallbladder emptying fractions of <35% do not experience relief of symptoms after a cholecystectomy [4,62]. Long-term follow-up of patients after cholecystectomy for biliary dyskinesia has shown that those with an ejection fraction of <15% are the most likely to achieve resolution of their symptoms [4].

10.4  EVALUATION OF THE GALLBLADDER AND BILIARY TREE

Evaluation of a patient with possible gallbladder or biliary disease begins with a thorough history from the child or their parents and a thorough physical examination. An appropriate history should document the onset, location, radiation and evolution of the abdominal pain. Questions should be directed at exacerbating or relieving factors, as well as any history of fevers or jaundice. Risk factors such as concomitant haemolytic diseases, a history of TPN administration, travel history and other past medical and surgical history can prove valuable. A full physical examination should include all quadrants of the abdomen. The examiner should document the presence or absence of a Murphy’s sign,^* as well as signs of jaundice. Blood tests should be directed towards elucidating the origin of the abdominal pain. Abnormalities such as leucocytosis, as well as elevation of the liver function tests, should be noted. The amylase and lipase levels should also be examined, looking for possible concurrent pancreatitis. Finally, guided imaging studies should be obtained to evaluate the gallbladder and biliary tree.

Ultrasonography is the key imaging study for evaluation of the biliary tree. It is effective, quick and noninvasive and can evaluate the entire biliary system, as well as other solid organs in the abdominal cavity. Standard signs of cholecystitis include gallbladder wall thickening, pericholecystic fluid and cholelithiasis. Choledocholithiasis should be suspected in patients with elevated CBD diameter measurements. The difficulty of evaluating the gallbladder and biliary tree anatomy in the paediatric population is that many of the structures, such as the gallbladder wall thickness and the CBD diameter, vary depending upon the age of the patient. Several studies have been performed to determine the normal radiographic dimensions of various biliary structures and the appropriate limits of normal biliary measurements, depending upon age of the patient being evaluated. One such study examining the sonographic dimensions of the normal gallbladder and biliary tree in 51 paediatric patients (ages ranging from 1 month to 16 years old) found that uniform gallbladder wall thickness never exceeded 3 mm [63]. Additionally, the common hepatic duct (CHD) gradually increased with age but was never more than 4 mm in diameter. Another study evaluating normal biliary anatomy in 173 infants and children ranging in age from 1 day to 13 years showed that the average diameter of the CBD in these patients was 1.27 ± 0.67 mm [64]. No differences were found between male and females. Furthermore, the CBD diameter was <3.3 mm in all patients. In those £3 months of age, the CBD was <1.2 mm in diameter. Finally, the growth of diameter of the CBD was linear and progressed at 0.002 cm per week [64].

10.5  CHOLEDOCHOLITHIASIS AND GALLSTONE PANCREATITIS

Choledocholithiasis, while thought to be rare in children, actually occurs with a reported incidence ranging from 7% to 20% among all children undergoing cholecystectomy [65–67]. Unfortunately, despite being relatively common, recommendations vary as to the optimal treatment for choledocholithiasis.

Several surgical options, with or without endoscopic retrograde cholangiopancreatography (ERCP), have been employed in the treatment of choledocholithiasis. Options include open cholecystectomy with CBD exploration, laparoscopic cholecystectomy with laparoscopic CBD exploration and, finally, laparoscopic cholecystectomy with either preoperative or postoperative ERCP and endoscopic stone extraction [68–70]. Realistically, not every centre has the capability to perform ERCP in infants and children [71], and similarly, surgical experience with laparoscopic CBD exploration varies among surgeons and institutions, and in many cases, the appropriate training is lacking [72]. Certainly, preoperative ERCP with stone extraction is an effective tool for children with choledocholithiasis [69,73]. The benefit of a preoperative ERCP with stone extraction is that if the ERCP is not successful, then the surgeon knows that stone extraction must occur during the operation. Conversely, the benefit of primary laparoscopic cholecystectomy with laparoscopic CBD exploration is that the sphincter of Oddi^† mechanism is not destroyed. Furthermore, the single-staged procedure is more cost-effective [70]. A Cochrane review of the adult literature on CBD stone clearance found that open exploration was superior to ERCP, but laparoscopic common duct exploration and ERCP were comparable in both success and complications rates [74]. The management of CBD stones varies from institution to institution, and factors such as access to skilled paediatric endoscopic services, as well as the skill of the surgeons for CBD exploration, guide management of these difficult paediatric patients.

Acute pancreatitis is increasingly being recognised in the children, with an estimated incidence of 13.2 cases per 100,000 patients [75]. of all causes of paediatric pancreatitis, biliary tract disease accounts for up to 30% of all cases [76–80]. Different than adults, in whom stones or tumours account for the majority of cases with obstructive pancreatitis, 30% of all obstructive pancreatitis cases in children are secondary to biliary sludge [78–80]. Currently, there is no indication to perform a cholecystectomy for biliary sludge-induced pancreatitis after a single episode. However, recurrent sludge-induced pancreatitis may benefit from a cholecystectomy [81]. In the setting of mild pancreatitis with few or no systemic manifestations, a cholecystectomy should be performed during the index admission, as interval cholecystectomy has been associated with high rates of recurrent biliary pancreatitis [82]. By contrast, cholecystectomy for severe pancreatitis and associated peripancreatic collections should be delayed for at least 6 weeks or until resolution of the collections occurs [82].

10.6  CHOLECYSTECTOMY (BOX 10.1)

Open cholecystectomy was the standard of care for treatment of gallbladder disease for many years. The standard right upper quadrant oblique incision allowed direct visualisation of critical structures and access to the gallbladder and biliary tree. As surgery has advanced, laparoscopic cholecystectomy has become the standard of care for treatment of gallbladder disease [83]. Additional advances in minimally invasive techniques means that there are now two laparoscopic approaches that can be used for cholecystectomy:

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