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The characteristics of biliary tract disease have been well documented in adults. However, many pediatric providers do not readily consider the possibility of cholecystitis or cholelithiasis in young patients who present with vague upper abdominal pain. However, gallbladder disease is being identified more frequently in children than in the past. The etiology of this increase is multifactorial and includes the increased and prolonged use of total parenteral nutrition (TPN), biliary dyskinesia, more frequent and liberal use of diagnostic modalities (abdominal ultrasound [US], abdominal computed tomography scans), and diseases of anemia (especially sickle cell disease and hereditary spherocytosis). Most importantly, the increasing frequency of metabolic syndromes in children is leading to a higher incidence of gallstone disease, which had previously been an etiology linked to the adult population. This has led to an increase in the number of cholecystectomies being performed in children and the teenage population. As with adults, the gold standard approach for cholecystectomy in children is via laparoscopy. One should expect success and complication rates to be similar between the different population ages.
Indications for Workup and Operation
The primary disease processes that lead to consideration for cholecystectomy include cholelithiasis, cholecystitis (acute, acalculous, and chronic), acute hydrops of the gallbladder, and biliary dyskinesia. Although the patient’s history may differ, usually the signs and symptoms are similar for all these conditions. Epigastric and right upper abdominal pain, with or without associated fever or jaundice, should immediately prompt the child’s healthcare provider to consider a gallbladder condition as the possible diagnosis.
Symptomatic cholelithiasis is often but not always the result of impaired gallbladder emptying related to gallstones. Gallstones are generally classified as being either nonhemolytic or hemolytic (secondary to sickle cell disease, spherocytosis, and thalassemia) in composition. Nonhemolytic gallstones are nearly always cholesterolic, whereas hemolytic gallstones are usually composed primarily of calcium bilirubinate. Regardless, the signs and symptoms are identical, related to biliary colic, and sometimes seen in association with excessive fat intake. An abdominal US will reveal the presence of gallstones in the gallbladder. Should cholecystitis occur, the patient may develop fever and possibly jaundice, and the US may show gallbladder wall thickening or pericholecystic fluid (or both). Leukocytosis and right upper abdominal pain frequently occur. Patients with symptomatic cholelithiasis should undergo elective laparoscopic cholecystectomy. Those with acute, severe cholecystitis are usually treated with intravenous antibiotics and urgent cholecystectomy. Rarely, in a patient with severe comorbidities or with a presentation of septic shock, a cholecystostomy tube may be required as a temporizing measure.
In the subset of patients with the presentation of gallstone pancreatitis, the standard algorithm for treatment is to allow the pancreatitis to slowly resolve by observing the patient’s symptoms and following the patient’s lipase and amylase levels. The cholecystectomy should be done in the same admission due to the risk for recurrence if the cholecystectomy is delayed.
The finding of biliary ductal dilation with US or the development of jaundice raises the possibility of choledocholithiasis. A number of options exist at this point. We prefer to proceed with preoperative endoscopic retrograde cholangiopancreatography (ERCP) with sphincterotomy and stone removal, if indicated, because the surgeon is then better able to plan the subsequent laparoscopic operation. 2 ERCP has been shown to be a safe procedure in the pediatric population, although the availability of an experienced endoscopist depends on local expertise in one’s community. If the common duct stone is extracted at ERCP and sphincterotomy, the surgeon can proceed with routine laparoscopic cholecystectomy. However, if the stone cannot be removed, the surgeon will know that laparoscopic choledochal exploration and stone removal should be planned. If that is not successful, then open exploration may be needed under the same anesthesia.
When the symptoms of epigastric and right upper abdominal pain, with or without associated fever or jaundice, are associated with a severe illness, trauma, burn, or sepsis, acalculous cholecystitis should be considered. The etiology of acalculous cholecystitis is, as its name implies, not due to gallstones. It is believed that this development is related to the association of events surrounding the disease under treatment. This includes gallbladder quiescence, excessive red cell hemolysis, TPN use with the absence of food intake, dehydration, and an adynamic ileus. An abdominal US may reveal gallbladder distention, gallbladder wall thickening, and pericholecystic fluid. Occasionally, sludge or debris will be visualized in the gallbladder. Failure of medical management (nothing by mouth, parenteral antibiotics, and intravenous fluids) may result in the need for laparoscopic cholecystectomy.
Biliary dyskinesia is rarely seen in infants. However, this condition is being encountered more often in children and adolescents, and it is considered by many gastrointestinal providers to be a valid reason for cholecystectomy in this age group. These patients often present with nonspecific abdominal pain that may be located in the right upper abdomen or epigastrium. The pain may be associated with meals and may be related to an excessively fatty diet. An abdominal US is invariably not diagnostic. In this instance, a technetium-99m radionuclide scan is diagnostic. In healthy individuals, the gallbladder ejection fraction should be greater than 35%. If a patient has symptoms consistent with biliary colic and a radionuclide gallbladder-emptying scan documenting an ejection fraction of less than 35%, laparoscopic cholecystectomy should be considered. Unfortunately, not all symptoms in these patients will be relieved. An ejection fraction of less than 15% usually results in relief of symptoms. Similarly, there is a small subset of patients who suffer abdominal pain with biliary hyperkinesia. This is defined as a gallbladder ejection fraction greater than 65%. The number of pediatric patients with this specific pathology is small so there are still further studies to be done as to whether a cholecystectomy is the treatment of choice, but there is some preliminary evidence to suggest that cholecystectomy can provide symptom relief.
Operative Technique
Four-Port Technique
Unless the patient is hospitalized for gallstone pancreatitis or acute cholecystitis, admission to the hospital is on the day of operation. The patient is asked to urinate just before proceeding to the operating room. In an elective cholecystectomy without ongoing infection or patient risk factors with decreased immunity, preoperative antibiotics are not indicated. If antibiotics are indicated, a cephalosporin is usually administered before beginning the operation. The patient is endotracheally intubated, and gastric decompression is accomplished via an orogastric tube. The patient is placed in the supine position and the abdomen is prepped from the nipple line to the symphysis pubis. The operating surgeon stands to the patient’s left and the assisting surgeon to the patient’s right. The scrub nurse/camera holder is to the left of the surgeon. Viewing monitors are positioned at the head of the table on both the right and left sides ( Fig. 18-1 ).
A vertically placed 10- to 12-mm incision is made directly through the umbilicus. Through this incision, a 10- to 12-mm cannula is inserted and the abdomen is insufflated. We generally use a 10-mm 45-degree operating telescope for visualization during the operation. The 10-mm telescope is used because a 10- to 12-mm umbilical incision is usually needed for removal of the gallbladder. A second 5-mm cannula is inserted under direct vision in the epigastric region. In older patients, this cannula can be placed through or to the right of the falciform ligament and well above the liver edge. In younger patients, it should be to the left of the linea alba. Depending on the size and amount of subcutaneous fat, the remaining operating instruments are introduced directly through the abdominal wall via a stab incision (if the patient is thin) or through 5-mm cannulas. One instrument is placed in the right lower quadrant (RLQ) and the other is located in the right midabdomen (RMA), inferior to the right epigastric port and near the liver edge. These working sites need to be more widely separated in smaller patients ( Fig. 18-2 ). In children younger than 10 years of age, instruments inserted in these latter two sites may be 3 mm in size ( Fig. 18-3 ).
Grasping the dome of the gallbladder with a locking grasper inserted through the RLQ incision and retracting the dome toward the right shoulder reveals the gallbladder infundibulum and porta hepatis. The infundibulum of the gallbladder (just distal to the cystic duct) is grasped with the surgeon’s left-handed instrument using a nonlocking grasper. This grasping forceps is then retracted inferior and laterally, creating a 90-degree angle between the cystic duct and the common bile duct ( Fig. 18-4A ). This helps avoid misidentification of these two structures. Using either a Maryland dissector or cautery-tip instrument in the surgeon’s right hand, the peritoneum overlying the confluence of the cystic duct and infundibulum is opened. The cystic duct is then circumferentially dissected using the Maryland dissector in the surgeon’s right hand. Occasionally, a right-angled dissector is useful in completing this dissection. To avoid injury to the common duct, it is important to clearly identify the cystic duct entering the gallbladder at the completion of this dissection (the critical view of safety; see Fig. 18-4A ). At this point, the cystic duct is ligated with two clips placed proximally and a single clip at the cystic duct–infundibulum junction, and it is then divided ( Fig. 18-4B ). If an intraoperative cholangiogram is to be performed, a number of options are possible. The distal cystic duct near the infundibulum can be ligated with an endoscopic clip, and the cystic duct proximal to the clip is partially transected to expose its lumen. A cholangiocatheter is then inserted into the cystic duct to perform the study ( Fig. 18-5A ). Another option is to use the Kumar clamp technique, which involves an atraumatic clamp positioned across the infundibulum of the gallbladder. Through a side port in the clamp, a sclerotherapy needle is advanced into the infundibulum ( Fig. 18-5B ). The advantage of this technique is that a small cystic duct is not cannulated, which can sometimes be difficult. Thus, this technique is especially useful in smaller patients ( Fig. 18-6 ).