Pediatric Gallbladder Disease Surgery 

Updated: Sep 21, 2018
Author: Holly L Neville, MD; Chief Editor: Harsh Grewal, MD, FACS, FAAP 

Overview

Background

Gallbladder disease in the pediatric population causes pain and results in significant morbidity. The diagnosis is frequently delayed in this population because of its relative infrequency compared with disease in adults; this results in additional illness, pain, and missed school days for the child and missed work for the parent. More serious complications may develop, such as acute cholecystitis, choledocholithiasis, cholangitis, and pancreatitis.

Even though gallbladder disease is relatively uncommon in the pediatric population, pediatric patients account for a disproportionate number of cholecystectomies; this rate has been rising in recent years. Pediatric gallbladder disease is most commonly associated with hemolytic diseases or hemoglobinopathies; however, other risk factors are recognized.

Extended administration of total parenteral nutrition (TPN) or prior extensive bowel resection increases the risk of gallbladder disease, a cause that will likely continue to increase as survival rates improve in extremely low birth weight infants. Sadly, as childhood obesity reaches near epidemic proportions in the United States, gallbladder disease related to dietary factors is becoming more prominent.[1, 2]

Gallbladder and biliary tract disease should be in the differential diagnosis of any pediatric patient who presents with right-upper-quadrant pain, jaundice, or unremitting dyspepsia with normal endoscopic gastric findings. Asymptomatic gallstones and symptomatic pigment gallstones in children are common indications for surgery. Noncalcified gallstones due to long-term cholestasis or TPN may respond to medical therapy such as ursodeoxycholic acid.[3] As in adults, laparoscopic cholecystectomy is the most feasible option for most patients.[4]

Aside from gallstones, cholestasis, and biliary dyskinesia, the pediatric population can experience congenital abnormalities of the gallbladder, including gallbladder perforation, hydrops of the gallbladder, gallbladder atresia, and choledochal cysts.

The introduction of laparoscopy revolutionized the practice of surgery. Trocars have been described since 25 BCE, when these devices were used to drain "bad humors" from the abdomen. The first endoscopic examination was performed in 1901 by a German gynecologist. This technique was further developed in the early part of the 20th century but remained prohibitively dangerous as a consequence of uncontrolled increases in abdominal pressure and an inability to maintain internal temperature.

By 1970, laparoscopy was commonly used by gynecologists. In 1987, the French physician Mouret performed the first human laparoscopic cholecystectomy. While performing laparoscopy for a gynecologic procedure on a woman known to have biliary colic, he tilted his camera upward and found that he was able to remove the gallbladder without making additional incisions. This forever changed the treatment of gallbladder disease. In 1992, a National Institutes of Health (NIH) consensus conference concluded that laparoscopy was the approach of choice for cholecystectomy.[5]

With the development of laparoscopic cholecystectomy, the annual number of cholecystectomies increased substantially.[6] Few true contraindications for laparoscopic cholecystectomy are recognized; the leading contraindication is unclear anatomy. Open cholecystectomy remains a safe and viable alternative when laparoscopy is not feasible and is commonly performed in infants and children with more uncommon diseases of the biliary tree.

Surgical intervention has evolved to single-incision laparoscopic cholecystectomy (SILC) and needle port–assisted SILC. These approaches, in adults, have been shown to have equal efficacy, with longer operating times and improved cosmesis.[7]

Anatomy

Bile secretion begins in the bile canaliculus in the liver. From here, bile enters the terminal channels (the canals of Hering), which gradually enlarge as they approach the portal canal. Bile flows from the centrilobular cells in zone 3 toward the portal triads in zone 1. These ducts anastomose to form hilar intrahepatic ducts, which, in turn, become the main hepatic ducts.

The porta hepatis and the right and left hepatic ducts join to form the common hepatic duct (CHD). Generally, the right and left hepatic ducts join outside the liver; however, in 5% of the population, this occurs inside the liver or at the location where the cystic duct joins the right hepatic duct. In 70% of the population, the cystic duct directly enters the CHD. Other possibilities include a cystic duct that runs parallel to the CHD or a cystic duct that runs anterior or posterior to the bile duct before medially joining the bile duct.

The common bile duct (CBD) lies inside layers of the lesser omentum and is anterior to the portal vein and to the right of the hepatic artery. It passes retroperitoneally behind the first portion of the duodenum, behind the head of the pancreas, and enters into the second part of the duodenum. The CBD then passes through the duodenal wall to join the main pancreatic duct, thus forming the ampulla of Vater and resulting in the duodenal papilla on the duodenal side. The sphincter of Oddi surrounds the bile and pancreatic ducts while they are inside the duodenal wall.

The arterial supply of the bile ducts is mostly from the right hepatic artery. The blood supply of the CBD arises from branches of the hepatic and gastroduodenal arteries. Injury to these vessels may lead to stricture of the bile ducts.

Lymphatics from the lower portion of the CBD drain into glands near the head of the pancreas. Lymphatic drainage from the rest of the biliary tree empties into the hilum of the liver.

The gallbladder stores bile created by the liver. This system allows controlled release of bile into the duodenum as needed for lipid solubilization. The gallbladder sits below the right hemiliver. It is divided into a fundus that lies between the transverse colon and the rectus abdominis and ninth costal cartilage, a body that lies close to the duodenum, an infundibulum, and a neck. The Hartmann pouch is an outpouching of the infundibulum that lies close to the neck of the gallbladder. When gallstones are impacted in this area, they obstruct the cystic duct and produce cholecystitis or obstruction of the adjacent CHD, which may lead to Mirizzi syndrome.

The neck of the gallbladder is connected to the cystic duct, which then empties into the CBD. The spiral valve of Heister, formed by the mucous membrane of the neck, regulates the flow of bile.

The arterial supply to the gallbladder comes from the cystic artery, which is usually a branch of the right hepatic artery. Venous drainage is provided by the cystic vein, which usually empties into the portal vein or directly into the hepatic sinusoids. Lymphatic drainage empties into a lymph gland near the neck of the gallbladder. The celiac axis supplies sympathetic innervation of the gallbladder; visceral pain is conducted through this and is frequently referred to the right subcostal, epigastric, and scapular regions. Parasympathetic innervation arises from both branches of the vagus nerve.

Tubuloalveolar glands aid in the production of mucus in the neck of the gallbladder. Rokitansky-Aschoff sinuses are invaginations of the surface epithelium that may extend through the muscularis and may be a source of inflammation secondary to bacterial stasis and proliferation within the sinuses. The ducts of Luschka sit along the hepatic surface of the gallbladder and directly open into the intrahepatic bile ducts. These may be a source of bile leak after cholecystectomy.

Anatomic variations are common and are likely related to injury to the CBD. Care must be taken to properly identify the anatomy of each individual patient; when the anatomy is unclear, intraoperative cholangiography should be performed to avoid potential serious duct injury.

Pathophysiology

Acquired disorders of the gallbladder include the following:

  • Hydrops of the gallbladder
  • Acalculous cholecystitis
  • Cholestasis
  • Cholelithiasis
  • Acute and chronic cholecystitis
  • Choledocholithiasis
  • Cholangitis

Hydrops or acute distention of the gallbladder with edema but without inflammation of the gallbladder wall may be a symptom of severe sepsis or shocklike states. Acute hydrops has been associated with Kawasaki disease and Henoch-Schönlein purpura.

Acalculous cholecystitis is uncommon in children but may arise after successful resuscitation from sepsis or shock when a previously unrecognized hydrops of the gallbladder becomes infected. This is confirmed by ultrasonographic findings that reveal a nonfunctioning, distended gallbladder without gallstones.

Cholestasis is defined as a failure of bile to move through the biliary system and may lead to liver disease. Symptoms and treatment depend on the primary disease process but generally include jaundice, pruritus, xanthomata, hepatomegaly, dark urine, hypopigmented stools, and, possibly, splenomegaly. In neonates, this disorder is also known as neonatal direct hyperbilirubinemia or neonatal hepatitis. Causes of cholestasis in pediatric patients include congenital anomalies, prolonged dependence on parenteral nutrition, cholangitis, hepatitis, pregnancy, and prolonged illness.[8]

In the pediatric population, cholelithiasis most commonly presents at puberty but can occur at any point in development. Underlying medical causes of gallstones are present in more than 50% of patients with calculous cholecystitis. In infants, the presence of gallstones is generally related to an extended period of fasting, required parenteral nutrition, or abdominal surgery. These gallstones are generally mixed cholesterol-calcium bilirubinate stones. Cholesterol stones (see the image below) have surpassed hemolytic stones as the principal type of gallstones in pediatric patients. The etiology and risk factors remain similar to those seen in adults; a high-fat diet is the primary predisposing factor.

Gallbladder and contents. Note yellow-green choles Gallbladder and contents. Note yellow-green cholesterol stones.

Hemolytic processes that result in gallstones in pediatric patients include sickle cell anemia, hereditary spherocytosis, and thalassemia. Stones found in these diseases are black-pigment stones predominantly composed of calcium bilirubinate. Medications such as ceftriaxone, furosemide, octreotide, ceftriaxone, and cyclosporin have been linked to gallstone formation.

Cholecystitis refers to inflammation of the gallbladder. Acute cholecystitis occurs secondary to an obstructing stone in the cystic duct that results in bile stasis and bacterial overgrowth. Chronic cholecystitis occurs as a result of several attacks of acute cholecystitis and results in an ulcerated and scarred gallbladder epithelium.

Although gallstones are the most common cause of cholecystitis in adults and children, acalculous cholecystitis may occur following local inflammation or infection. Typhoid fever, scarlet fever, measles, and AIDS have been associated with acalculous cholecystitis, as have Mycoplasma, Streptococcus group A and B, Shigella, and Escherichia coli infections. Shock, sepsis, hyperalimentation, fasting, intravenous narcotics, and transfusions are risk factors in the development of acute acalculous cholecystitis following surgery.

Cholangitis is caused by an ascending infection of the biliary tract and usually occurs after a gallstone blocks the CBD. The most commonly involved organisms include E coli and Klebsiella, Pseudomonas, and Enterococcus species.

Choledocholithiasis occurs in 11% of children with cholelithiasis and almost 20% of pediatric patients with gallstone pancreatitis. This condition is caused by the passage of stones through the cystic duct with entrapment at the papilla of Vater.

Etiology

Pediatric gallbladder disease stems from numerous causes. The following are some of the best-described causes[9] :

  • Partial or complete obstruction of the biliary ductal system
  • Hemolytic disease
  • Estrogen effect
  • Increased triglycerides
  • Obesity
  • Familial predisposition
  • Metabolic-related and stress-related causes (eg, short bowel syndrome with reduced bile salt absorption)

However, numerous less defined causes have been recognized, including the association between previous abdominal or renal surgery and gallstones or the development of acalculous cholecystitis.

Epidemiology

Congenital abnormalities of the gallbladder are rare. On the other hand, cholelithiasis has an incidence rate of 0.15-0.22% in the pediatric population. Although the incidence of pediatric cholelithiasis has risen over the past decades, the number of children with cholelithiasis who have hemolytic disease has decreased, reflecting either an increase in the incidence of cholesterol cholelithiasis and biliary dyskinesia or an increase in the recognition and willingness to treat these diseases in children.[8] The increased willingness to treat these diseases may be due to increasingly widespread use of laparoscopy in children.

Possible reasons for a rise in gallbladder disease incidence include the following:

  • Improved availability of ultrasonography (US) for assessment of abdominal pain
  • High teenage pregnancy rates
  • Increased prevalence of childhood obesity

Although gallbladder disease in pediatric populations historically was a sign of an underlying hemolytic disease, cholesterol stones in children with obesity are currently the most common cause of gallbladder disease. Female adolescents are 11-22 times more likely to have gallbladder disease than male adolescents are.

One study reported that in 224 pediatric patients who underwent cholecystectomy, the mean weight was 58 kg.[10] Surgery was performed secondary to symptomatic cholelithiasis in 166 patients, secondary to gallbladder dyskinesia in 35, secondary to pancreatitis in seven, in combination with splenectomy in six, secondary to cholecystitis in five, secondary to choledocholithiasis in one, and secondary to acalculous cholecystitis or polyp in the remaining two. Gallstone formation is seen in 20% of patients with sickle cell disease prior to adolescence.[11]

Prognosis

When pediatric gallbladder disease is treated appropriately and in a timely fashion, the prognosis is excellent. Most patients recover from a cholecystectomy and return to regular activities within a week. If left untreated, cholecystitis can lead to significant illness. Some patients have diarrhea, gastritis, esophagitis, and colicky abdominal pain after cholecystectomy, particularly after the ingestion of foods high in sugar. This is termed postcholecystectomy syndrome and may occur in as many as 30% of patients.

Patient Education

The epidemic of childhood obesity and poor diet in the United States will likely result in higher rates of gallbladder disease. Any child who presents with symptoms of gallbladder disease should be evaluated and counseled regarding appropriate dietary management.[2]

Before consenting to surgical treatment of gallbladder disease, both the patient and the family should be educated regarding the risks of both laparoscopic and open cholecystectomy. Described risks should included the following:

  • Retained stone in the CBD
  • Bile leak
  • Bleeding
  • Infection
  • CBD injury [12]
  • CBD stricture
  • Hepatic artery injury
  • Conversion from a laparoscopic procedure to an open one
  • Damage to adjacent structures
  • Postcholecystectomy syndrome, which may include gastritis, irritable bowel symptoms, and postprandial pain [13, 14]
 

Presentation

History and Physical Examination

A variety of clinical findings may be noted in pediatric patients with gallbladder disease, as follows.[15]

Hydrops

Hydrops or acute distention of the gallbladder generally presents following severe sepsis or shocklike states. Acute hydrops has been associated with Kawasaki disease and Henoch-Schönlein purpura.

Acalculous cholecystitis

This is uncommon in children but may arise following successful resuscitation from sepsis or shock when previously unrecognized hydrops of the gallbladder becomes infected. Presentation includes abdominal pain, vomiting, fever, and laboratory findings that may mimic acute cholecystitis.

Cholestasis

Cholestasis generally presents with jaundice, pruritus, xanthomata, hepatomegaly, dark urine, hypopigmented stools, and, possibly, splenomegaly.

Cholelithiasis

Cholelithiasis symptoms usually include vague abdominal pain in a child with obesity. Fatty food intolerance less common in pediatric patients than in adults. Some children may present with classic symptoms of biliary colic, including severe, intermittent, colicky right upper quadrant or epigastric pain following ingestion of fatty foods.

Cholecystitis

Unlike the other processes, cholecystitis presents with persistent pain (generally >8 h) in the right upper quadrant or epigastric region, nausea and vomiting, fever, anorexia, and mild elevation of liver function test findings, particularly alkaline phosphatase (ALP), alanine aminotransferase (ALT), and aspartate aminotransferase (AST) levels. When associated with cholesterol stones, the presentation is generally mild. In patients with sickle cell disease or diabetes, cholecystitis may present as a much more severe illness, resulting in sickle cell crisis, sepsis, or diabetic ketoacidosis.

Cholangitis

The presentation of cholangitis in the pediatric population is similar to that seen in adults. The classic triad of symptoms (ie, the Charcot triad) includes fever, right-upper-quadrant pain, and jaundice. Without treatment, these symptoms advance to include confusion, hypotension, and sepsis. This condition presents with symptoms that mimic cholecystitis, only with the additional symptoms of jaundice and pain that radiates through to the back.

Complications

Gallstone disease may lead to choledocholithiasis, acute or chronic cholecystitis, cholangitis, gallbladder perforation, or pancreatitis. Pancreatitis is reported in as many as 8% of pediatric patients with gallstones. These potential complications should be discussed with patients with biliary colic due to gallstone disease.

 

Workup

Laboratory Studies

In any patient with suspected biliary or gallbladder disease, obtain blood type and screen, a complete blood count (CBC), and a complete metabolic panel, including alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), total bilirubin, direct and indirect bilirubin, amylase, and lipase levels. In patients who are obese, screen for cholesterol and triglyceride abnormalities, and perform glucose screening to evaluate for diabetes.

Leukocytosis with a left shift may be observed in cholecystitis. ALT and AST levels are used to evaluate the presence of hepatitis and may be mildly elevated in cholecystitis or with common bile duct (CBD) obstruction. Significant elevation is usually indicative of hepatitis and should prompt a hepatitis serology profile.

Bilirubin and ALP assays are used to evaluate evidence of CBD obstruction. Bilirubin should be broken down into direct and indirect bilirubin. This is of particular use in patients with hemolysis or hemolytic diseases (eg, sickle cell disease). These patients may have a chronically elevated indirect bilirubin level that is not indicative of hepatobiliary disease. Choledocholithiasis with CBD obstruction results in elevation of total bilirubin and direct bilirubin levels. The indirect bilirubin levels remain within the reference range in this case.

Amylase and lipase assays are used to evaluate for the presence of pancreatitis. Amylase levels may also be mildly elevated in cholecystitis. An elevated ALP level is observed in 25% of patients with cholecystitis.

Urinalysis is used to rule out pyelonephritis and renal calculi, which are included in the differential diagnosis of right-upper-quadrant pain.

All females of childbearing age should undergo a pregnancy test.

Imaging Studies

Ultrasonography

In all children and infants, ultrasonography (US) can reveal the size and echogenicity of the liver as well as detect stones and sludge in the bile ducts and gallbladder and cystic or obstructive dilatation of the biliary system. Its accuracy may be limited by morbid obesity and lack of patient cooperation. US is the best imaging study for initial evaluation of a pediatric patient with suspected biliary and gallbladder disease.

Findings in patients with biliary atresia may include triangular cord or periportal echogenicity representing a cone-shaped fibrotic mass cranial to the portal vein or a “ghost” triad, which includes a gallbladder length less than 1.9 cm, lack of smooth mucosa lining, and an indistinct wall. The triangular cord sign and the presence of gallbladder abnormalities are probably the most accurate US findings for diagnosing or excluding biliary atresia.[16]

In patients with cholecystitis, US reveals pericholecystic fluid and gallbladder-wall thickening of more than 4 mm. It can be used to reveal a sonographic Murphy sign. US is 90-95% sensitive and 78-80% specific in these patients.

In patients with choledocholithiasis, US reveals a dilated CBD (defined as ≥8 mm).

Polypoid lesions of the gallbladder, though rare in children, may be detected and followed by means of high-quality US.[17]

Plain radiography

Plain radiography reveals calcified gallstones in only 10-25% of cases but can be useful in ruling out pneumonia, which may have a similar presentation. This study can also be used to evaluate for calcification of stones in patients who may be treatable by medical means.

Computed tomography

Computed tomography (CT) can provide information similar to that provided by US; however, it involves radiation and may require reconstructions in order to gain detailed information. CT may be useful in exploring the surrounding anatomy if the diagnosis is uncertain. It is also useful in assessing the pancreas in cases of severe gallstone pancreatitis.

Magnetic resonance imaging

A study by Kim et al involving 208 infants with jaundice found magnetic resonance imaging (MRI) to have performance comparable that of US for diagnosis of biliary atresia.[18]

Magnetic resonance cholangiopancreatography

Magnetic resonance cholangiopancreatography (MRCP) is used to assess the biliary tract in all age groups. It is 90% sensitive and 77% specific in revealing extrahepatic biliary atresia. MRCP is particularly useful in patients with congenital anomalies and in evaluating suspected choledocholithiasis. It has the added benefit of avoiding radiation exposure; however, it may require sedation or anesthesia in order to obtain an optimal study, particularly in younger patients.

Nuclear medicine studies

Hepatobiliary scintigraphic imaging agents (eg, technetium-99m iminodiacetic acid derivatives), may be helpful in assessing cystic duct patency in a patient with a hydrops of the gallbladder or cholelithiasis. The most sensitive test for acute cholecystitis involves intravenous (IV) injection of radiolabeled hepatobiliary iminodiacetic acid (HIDA) secreted into the biliary tree. In acute cholecystitis, HIDA enters the CBD but does not enter the gallbladder; thus, the gallbladder is not filled at completion of the study.

If the gallbladder is not visualized, IV morphine administration can improve the accuracy of HIDA scanning by increasing resistance to flow through the sphincter of Oddi, resulting in filling of the gallbladder if the cystic duct is patent.

In a newborn, if a hepatobiliary scintiscan fails to reveal tracer movement from the biliary tree into the small bowel and liver biopsy findings reveal interlobular bile duct proliferation, exploration with intraoperative cholangiography (see More Invasive Procedures) is necessary to rule out biliary atresia.

More Invasive Procedures

Other diagnostic options to be considered in this setting include more invasive procedures such as liver biopsy, intraoperative cholangiography, and endoscopic retrograde cholangiopancreatography (ERCP).

Liver biopsy may be performed percutaneously, under US guidance, or via an open technique. This procedure is not usually required for isolated diseases of the gallbladder but may be necessary in some patients.

Intraoperative cholangiography may be required in patients with gallbladder disease who have suspected anomalous anatomy, choledocholithiasis, or masses in the CBD. This procedure is often performed in conjunction with laparoscopic or open cholecystectomy. In newborns, this procedure is performed to determine the biliary ductal anatomy and to differentiate among biliary atresia, Alagille syndrome, and cholestasis.

ERCP is an endoscopic procedure commonly performed by either surgeons or gastroenterologists who specialize in the procedure. Its signal feature is that it is both diagnostic and therapeutic. In this procedure, the CBD is cannulated, and stone retrieval or biopsy may be performed. Papillotomy is often performed to permit release of entrapped stones and debris and to prevent impaction of stones in the future. Additionally, stents may be placed for obstructive disease or to assist in resolution of duct injuries. This test requires fluoroscopy.

Histologic Findings

The histology of gallbladder disease may range from acute inflammation to chronic inflammation with lymphocytic and/or neutrophil infiltration, depending on the chronicity of illness. When stones are present, they are either cholesterol or pigmented stones; pigmented stones are indicative of hemolytic disease.

 

Treatment

Approach Considerations

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. 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 performing a more invasive procedure.

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.[19, 20, 21] 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.[22, 23]

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.[24] 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.[25]

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 Therapy

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 is 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.

Surgical Therapy

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. 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.

Single-incision laparoscopic cholecystectomy may also be performed,[7] as well as robotic laparoscopic cholecystectomy.[26] 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.[27]

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.[28, 29, 30]  Steps for enhancing the safety of laparoscopic cholecystectomy in patients with acute cholecystitis are available in the Tokyo Guidelines 2018.[31]

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 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.)

Gallbladder as seen at time of laparoscopy. Gallbladder as seen at time of laparoscopy.
Gallbladder dissection with visualized cystic duct Gallbladder dissection with visualized cystic duct and cystic artery.

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. 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.

Postoperative Care

Many centers have shown that elective laparoscopic cholecystectomy can be performed as an outpatient day procedure. This practice is not 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.[32]

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

Complications associated with cholecystectomy include the following:

Anesthesia is associated with separate risks.

Long-Term Monitoring

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.