Pediatric Gallbladder Disease Surgery Treatment & Management

In the event of congenital anomalies, most surgeons recommend a liver biopsy and cholangiography if the diagnosis or anatomy cannot be clearly discerned by means of preoperative imaging.

Indications in patients with acquired biliary disease can be slightly more complex. Some patients, such as those with total parenteral nutrition (TPN)-induced cholestasis and those with associated noncalcified cholelithiasis, can be treated medically; however, most gallstone disease in pediatric patients is treated in much the same way as similar disease in adults.

Cholecystectomy is the treatment of choice for symptomatic gallbladder disease. Preoperatively, on the basis of laboratory and radiology study findings, the surgeon should determine whether the patient is at high risk for choledocholithiasis. Elevation of liver function test results or dilation of the common bile duct (CBD) should raise clinical suspicion.

These patients should undergo preoperative endoscopic retrograde cholangiopancreatography (ERCP) to evaluate and clear the CBD, if needed. ^[23] In centers where this procedure is not available for pediatric patients or in pregnant patients (in whom radiation is not feasible), magnetic resonance cholangiopancreatography (MRCP) can be performed to confirm the presence of choledocholithiasis before a more invasive procedure is initiated.

Once the patient has had the CBD cleared (if possible) or has been deemed at low risk for choledocholithiasis, a cholecystectomy is performed. This procedure is usually performed laparoscopically and may be safely performed in infants and children. ^{[4, 24, 25, 26]} Intraoperative procedures such as cholangiography and CBD exploration may be necessary if a stone persists in the biliary tract or if preoperative ERCP was unavailable. ^{[27, 28]}

In healthy, asymptomatic patients with gallstones, cholecystectomy is not necessary; however, it may be performed at the same time as another procedure if an associated underlying disorder is present. Patients with hereditary spherocytosis and asymptomatic gallstones may benefit from a combined cholecystectomy and splenectomy. If no stones are present, a cholecystectomy is unnecessary because the risk of developing biliary disease after a splenectomy is low.

Similarly, in patients with sickle cell disease or diabetes mellitus, emergency cholecystectomy and cholecystitis are associated with high morbidity and mortality; therefore, elective cholecystectomy is recommended upon recognition of gallstone disease, regardless of symptoms. ^[29] Choledocholithiasis is reported in more than 10% of patients with sickle cell disease and should be considered during the preoperative evaluation.

In otherwise healthy pediatric patients with symptomatic gallstones, conservation therapy with diet control should be initiated, followed by elective cholecystectomy. Repeated episodes of biliary colic or admission for cholecystitis should prompt more urgent therapy.

Open cholecystectomy is reserved for patients in whom laparoscopic cholecystectomy failed (most commonly because of either anatomic variation or adhesive disease due to a prior operation) or for those rare patients with preoperative contraindications to laparoscopy (eg, a hostile abdomen due to severe adhesive disease, severe cardiopulmonary compromise, or known aberrant anatomy deemed by the surgeon to render laparoscopic intervention unsafe).

Rarely, instead of cholecystectomy, a cholecystostomy is performed as a bridge to cholecystectomy in critically ill children with sepsis. Indications for cholecystostomy include critical illness due to cholecystitis in an unstable child who is unsuitable for operative intervention. ^[30]

Very few contraindications for surgical intervention are recognized in patients with gallstone disease. Patients with medical comorbidities should be preoperatively optimized to ensure safe operative intervention. For example, patients with sickle cell disease should be transfused to a preoperative hemoglobin level of 10 g/dL. Acute gallstone pancreatitis should delay surgery until clinically resolved. Additionally, patients should be screened for a family history of anesthetic complications (eg, malignant hyperthermia).

Some patients are not optimal candidates for laparoscopic surgery; however, in those patients, open cholecystectomy can usually be safely performed.

Medical management of pediatric patients with gallbladder disease consists of adequate hydration and pain control. Antibiotics are also generally prescribed for acute cholecystitis, choledocholithiasis, and gallstone pancreatitis with pancreatic necrosis, as well as just prior to a cholecystectomy. Second-generation cephalosporins or penicillin-based compounds with a beta-lactamase inhibitor are generally acceptable.

Alternatives to surgical treatment have not been particularly successful. Oral administration of chenodeoxycholic acid (ursodiol) has been used in the management of gallstones but has been successful in fewer than 15% of patients after 2 years. Shock-wave lithotripsy has also been used, but with a success rate of only 20%. These treatments have not been extensively studied in pediatric populations.

Spontaneous resolution of gallstones has been demonstrated in infants, and recurrence is rare in this population. In this group, treatment is generally reserved to those at risk for cholangitis. Likewise, pediatric patients with acalculous cholecystitis may not require surgery. This is especially true for critically ill patients in whom anesthesia and an operation would be detrimental. In these patients, antibiotic administration, nasogastric suction, NPO (nil per os) status, and hyperalimentation are warranted.

In adult patients, some success with oral medications in the treatment of gallstones has been reported. Hydroxymethylglutaryl coenzyme A (HMG-CoA) reductase inhibitors (eg, atorvastatin) have been shown to dissolve cholesterol gallstones completely in 15% of adult patients and partially in 28%. However, these medications have not shown any value in the treatment of pigment stones in pediatric patients. Unless coupled with successful management of the underlying cause, the gallstones persist.

If acute pancreatitis is present secondary to gallstone obstruction of the pancreatic duct, treatment consists of cessation of oral intake, intravenous (IV) administration of fluids, antibiotic therapy, and pain control; although symptoms are occasionally severe, they generally remit in 3-4 days, and laboratory values return to normal.

Patients should undergo laparoscopic cholecystectomy with intraoperative cholangiography within 1 month. They should not undergo preoperative ERCP without definitive evidence of choledocholithiasis, because this procedure may exacerbate the pancreatitis. An exception is persistent, unrelenting pancreatitis with clinical or radiographic evidence of choledocholithiasis.

In otherwise healthy patients with cholesterol stones, gallstone disease in the pediatric period should be considered a marker of poor cholesterol and triglyceride control. These patients often report frequent fast food or fatty food ingestion, may be obese, and are at risk for other serious diseases such as vascular disease, type 2 diabetes mellitus, hypertension, and morbid obesity. Counseling should be provided by both a physician and a dietician to provide education and a means to reverse any potential health risks.

Elective cholecystectomy has a 0.17% mortality and a 14% complication rate; it remains the best therapy for symptomatic gallstone disease. Laparoscopic cholecystectomy is the preferred approach for most patients. ^[5] It is associated with less pain, fewer complications, and faster recovery than open procedures (1.6 vs 4.3 days). Only 2-5% of laparoscopic cholecystectomies convert to open procedures. A study by Babb et al using data from the Kids' Inpatient Database found that open cholecystectomy continues to be offered as the initial approach in a significant minority of children with gallbladder disease. ^[6]

Single-incision laparoscopic cholecystectomy may also be performed, ^[11] as well as robotic laparoscopic cholecystectomy. ^[31] Although these offer improved cosmesis with a similar risk/benefit profile, they have typically been associated with a longer operating time. A single-center study by Nolan et al, aimed at comparing various minimally invasive approaches to cholecystectomy—laparoscopic multiport (LMP), laparoscopic single-incision (LSI), robotic multiport (RMP), and robotic single-incision (RSI)—found that RSI cholecystectomy had a significantly shorter postoperative length of stay than the others and had an operating time equivalent to that of LMP cholecystectomy. ^[32]

A higher incidence of bile duct injury is noted with laparoscopic techniques; thus, mortality is also increased. In the case of acute cholecystitis, higher success rates are reported when the cholecystectomy is performed within 3 days of symptom onset. ^{[33, 34, 35]}  Steps for enhancing the safety of laparoscopic cholecystectomy in patients with acute cholecystitis were published in the Tokyo Guidelines 2018. ^[36]

Preparation for surgery

Patients with cholecystitis who are acutely ill should be treated with IV fluids, analgesics, and broad-spectrum antibiotics. Once the fluid deficits are corrected and any infection is controlled, the patient should receive a cholecystectomy as soon as possible. Preoperatively, ultrasonography (US) is performed to confirm the diagnosis of cholelithiasis and to evaluate for the presence or absence of CBD involvement.

If jaundice, severe pain, or pancreatitis is present, choledocholithiasis may be suspected, and ERCP and sphincterotomy are recommended before the laparoscopic operation. MRCP may be performed as an alternative screening test to avoid a second invasive procedure if no stones are found. This is an important step in preparing for surgery because it allows the surgeon to determine if intraoperative removal of stones from the CBD will be necessary; this may be more difficult to perform laparoscopically.

Patients with sickle cell disease who undergo laparoscopic cholecystectomy may require a transfusion before the procedure. The preoperative hemoglobin level should be 10 g/dL to avoid acute chest syndrome. Patients with sickle cell disease are susceptible to vaso-occlusive crises, pneumonia, sepsis, and pulmonary infarcts during surgery. Adequate preoperative, intraoperative, and postoperative hydration is necessary. In addition, adequate analgesia and supplemental oxygen therapy are essential to avoid sickle cell crisis. Transfusion management of these patients must be individualized.

All patients should have an active type and screen prior to surgery. Additionally, patients should receive antibiotic prophylaxis preoperatively and should be assessed for risk of deep venous thrombosis (DVT), with implementation of prophylaxis when needed.

Operative details

Upon arrival in the operating room, the patient is positioned, sequential compression devices are activated (when indicated), and the patient is given anesthesia. The patient is then prepared and widely draped, with the entire abdomen exposed.

In laparoscopic cholecystectomy, an open technique is used to place a 5 mm or 10 mm trocar within the umbilicus, and a pneumoperitoneum is established. Additional trocars are inserted under direct vision. Most surgeons use a second 5 mm or 10 mm trocar placed at a subxiphoid site, with two more 3 mm or 5 mm trocars placed subcostally in the right upper quadrant (RUQ) in the midclavicular and anterior axillary line. In younger patients, the ports must be placed widely because the intra-abdominal working area is reduced.

The smaller ports are used for grasping the gallbladder. The lateral port is used to retract the fundus of the gallbladder cephalad. The medial cannula is used to grasp the gallbladder infundibulum and to retract it inferiorly and laterally for further exposure of the triangle of Calot. Once the triangle of Calot is cleared, only the cystic artery and cystic duct should be seen entering the gallbladder. At this time, intraoperative cholangiography may be performed if clinically indicated (eg, in patients with unclear anatomy or suspected choledocholithiasis). (See the images below.)

Intraoperative cholangiography is performed by clipping the cystic duct at the neck of the gallbladder, making an incision in the lateral wall of the cystic duct, and passing the cholangiocatheter through one of the right-side ports (or through a transabdominally placed angiocatheter) and into the cystic duct. Fluoroscopy is used with infusion of contrast to assess the anatomy. ^[37] Once this is complete, the cystic duct is clipped and divided. Similarly, the cystic artery is clipped and divided. The peritoneum overlying the gallbladder is placed on tension with the two grasping forceps, and a cautery device is used to divide the gallbladder from the liver.

Before removing the gallbladder, confirm adequate placement of the clips, and ensure that all bleeding is controlled. The gallbladder is then removed through the umbilical port, which may have to be enlarged to accommodate the gallbladder and stones. This is sometimes done with a specimen retrieval bag. The pneumoperitoneum is then released, the ports are removed under visualization to ensure adequate hemostasis at each port site, and the port incisions are closed.

An open cholecystectomy can be accomplished via either an upper midline or a right subcostal (Kocher) incision. The entire gallbladder, from the fundus downward, is dissected until the attachments to the cystic artery and cystic duct are visualized. These structures are then suture-ligated and divided. If there is concern about the security of the cystic duct closure or if severe infection is present, a closed-suction drain may be placed.

Many centers have shown that elective laparoscopic cholecystectomy can be performed as an outpatient day procedure. This practice has not been as common in children, because of concerns about postoperative pain control, but it has been reported. In an analysis of 2050 patients from the 2012 and 2013 National Surgical Quality Improvement Program-Pediatric (NSQIP-P) databases, Sacco Casamassima et al found outpatient laparoscopic cholecystectomy to be safe in pediatric patients who had no significant associated comorbid conditions. ^[38]

The average hospital stay after a laparoscopic cholecystectomy is 36 hours; the average stay after an open cholecystectomy is 3 days. The patient should continue to receive hydration until he or she is able to tolerate a diet. DVT prophylaxis should be continued until the patient is ambulating well. The patient should be observed for postoperative fever, jaundice, ileus, pancreatitis, bile leak, and urinary retention.

Early postoperative ambulation is recommended, but strenuous activity should be limited for 1 week or until pain is controlled after laparoscopic cholecystectomy and for 4 weeks after open cholecystectomy.

Complications associated with cholecystectomy include the following:

• Hemorrhage
• Infection
• Bile duct injury
• Hepatic artery injury
• Ileus
• Pancreatitis - In children with gallstone pancreatitis, performing cholecystectomy during the index admission rather than after a delay appears to reduce the recurrence of pancreatitis ^[39]
• Bile leakage
• Postcholecystectomy syndrome

Anesthesia is associated with separate risks.

Patients should be seen for routine follow-up 2 weeks after surgery. At the follow-up visit, patients are evaluated for incision healing, pain, any signs of infection or jaundice, return of appetite, and bowel and bladder function.