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bile duct cyst specimen

Image: “These gross images of the bile duct cyst specimen. (A) The image of the bile duct cyst in operation. (B) The specimen of the bile duct cyst. ” by Wang et al. License: CC BY 2.0


Definition of Pediatric cholecystitis

Cholecystitis

“Cholecystitis” Image created by Lecturio

Cholecystitis refers to inflammation of the gallbladder. Pediatric cholecystitis is rare. There are subtle differences in the anatomic and pathologic features of the disease between adults and children.

Extensive, dedicated research has not yet been conducted to discern the clinicopathologic features of the disease and establish the best treatment options. The differences between adult and pediatric cholecystitis can be tabulated as below:

Feature Adult cholecystitis Pediatric cholecystitis
Incidence Very common Rare (~5% of cases)
Sex ratio More common in females Neutral susceptibility or greater in males
Presentation Most patients are asymptomatic. Very few children are asymptomatic.
Pathology A higher proportion of calculus disease secondary to cholesterol A higher proportion of pigment stones
Association Not often associated with a systemic disease Significant association with few systemic disorders
Operative techniques Laparoscopic cholecystectomy is the gold standard; usually with minimal complications. There is no consensus on the best treatment option. Surgery in the pediatric population is more challenging and difficult.

Epidemiology of Pediatric cholecystitis

The incidence of pediatric cholelithiasis and cholecystitis is on the rise. The estimated current incidence is about 1.9%.

Anatomy

Few anatomical characteristic eponyms should be familiar to one while studying cholecystitis.

Calot´s triangle

Variously known as the hepatobiliary triangle or the cystohepatic triangle, this potential space at the porta hepatis contains the cystic artery and the cystic duct. Its boundaries are comprised of the cystic duct joining the:

  • Common bile duct
  • Common hepatic duct
  • Superior inferior margin of the liver

Optimum dissection in the Calot’s triangle, and perfect ligation of the cystic artery and the cystic duct, are essential elements of surgical cholecystectomy.

Classification of Pediatric cholecystitis

Cholecystitis denotes inflammation of the gallbladder. There are several types of cholecystitis based on the inciting pathology. This taxonomical segregation can be summarized as follows:

  • Chronic cholecystitis
  • Acute calculus cholecystitis
  • Acute acalculus cholecystitis
  • Emphysematous cholecystitis
  • Xanthogranulomatous cholecystitis
  • Suppurative cholecystitis

Pathogenesis of Pediatric cholecystitis

The key factors in gallstone formation in the pediatric population can be summarized as follows:

  • Endocrine influence
  • Temporary hepatic function dysregulation
  • Dietary changes
  • Dehydration
  • Inflammation
  • Genetic and hereditary predisposition

Risk Factors of Pediatric cholecystitis

Unlike adult cholelithiasis, pediatric cholelithiasis and cholecystitis are often associated with certain systemic disorders that make the child susceptible to gallstone disease. In 90% of cases, obstruction of the cystic duct neck by a stone is the most common reason for emergency cholecystectomy in children, especially when one or more factors render them susceptible to the disease. These risk factors can be summarized as follows:

  • Obesity
  • Abdominal surgery
  • Cystic fibrosis
  • Red blood cell membrane fragility
  • Hemolytic disorders
  • Total parenteral nutrition
  • Gilbert’s disease
  • IgA deficiency
  • Family history of gallstones
  • Ileal disease and resection
  • Sepsis
  • Ceftriaxone treatment
  • Adolescence
  • Female gender
  • Hemolytic uremic syndrome
  • Hemoglobinopathies

Clinical Features of Pediatric cholecystitis

Vignette

A girl with fever and severe abdominal pain. She had a fever and has been losing weight despite abdominal bloating. She is rushed to the OR. Her BMI is 30 kg/m2. The pain is of an acute nature with a sharp character, located in the right upper quadrant and radiating to the back. Ultrasonography reflects gallstones and a thickened gallbladder wall.

What do her providers suspect she has?

Clinical signs and symptoms

The example above is a classical presentation of acute cholecystitis. It is often a clinical diagnosis. The chief complaints can thus be summarized as follows:

  • Abdominal pain
  • Fever
  • Nausea
  • Vomiting
  • Anorexia
  • Bloating

The abdominal pain commonly associated with cholecystitis is very characteristic and has the following unique properties:

  • Acute in nature
  • Sharp, colicky in character
  • Epigastric or right upper quadrant location
  • Radiates to the back or right scapular area

Physical examination findings can be listed as follows:

  • Fever and dehydration
  • Right upper quadrant (RUQ) tenderness with guarding
  • May have Murphy’s sign

There are a few well-known specific signs that appear during the clinical assessment of a patient with acute cholecystitis. They have been summarized below.

Murphy´s sign

Inhalation while the examiner is palpating in the right hypochondriac region along the bottom of the rib cage results in sharp pain in patients with acute cholecystitis.

Boas´s sign

Escalated sensitivity below the right scapula, with hyperesthesia, is encountered in the clinical examination of patients with acute cholecystitis.

Diagnosis of Pediatric cholecystitis

The diagnosis of acute cholecystitis in the pediatric population is often based on clinical findings and few confirmatory non-invasive tests, such as ultrasonography. When used judiciously, ultrasonography has high sensitivity and specificity.

The key features of acute cholecystitis on ultrasonography, which help clinch the diagnosis, are:

  • Thickened gallbladder wall (> 3mm)
  • Fluid collection around the gallbladder
  • Acoustic shadows
  • Any visible mass
  • Sonographic Murphy sign: Increased abdominal tenderness on the application of pressure of the ultrasonography probe is equivalent to the clinical Murphy’s sign. It has diagnostic implications.
  • Gallbladder distension
  • Sludge
  • Obstructing stone demonstration in the gallbladder neck or the cystic duct is confirmatory
  • Cholelithiasis

The beneficiary role of imaging studies in the diagnosis of cholecystitis can be mentioned briefly as follows:

CT scan

A CT scan is not as sensitive as ultrasonography. Its specific use is to document the presence of gallstones with similar density to bile, which is potentially undiagnosed on ultrasonography. Reactive liver hypervascularity with enhanced parenchymal changes can be appreciated. The tensile gallbladder fundus sign is a CT-based sign of cholecystitis. It is about 75% sensitive and 95% specific, with a confirmed role in establishing an early diagnosis.

MRI

MRI sensitivity parallels that of ultrasound; however, in the pediatric population, MRI is not user-friendly and has many caveats, such as its expensive nature, discomfort during the procedure, and requiring great co-operation from the patient.

ERCP

ERCP stands for endoscopic retrograde cholangiopancreatography. It is an invasive test and has a key diagnostic and simultaneous therapeutic role in managing common bile duct stones. Common bile duct stones are encountered in patients with predisposing systemic diseases, such as hemolytic disorders, sickle cell anemia, and other hemoglobinopathies.

MRCP

MRCP is the MRI-based, non-invasive equivalent of ERCP for diagnostic use only. It can demonstrate affected stones as filling defects. Co-operation from the pediatric population for such procedures is very difficult to procure; hence, these investigations are used quite infrequently compared to the adult patient population.

Technitium HIDA scan

The hepatobiliary iminodiacetic acid (HIDA) scan is the most sensitive test for diagnosing cholecystitis. The radioactive tracer is injected and, subsequently, drainage of the same is documented. HIDA use is discouraged in children because of the radioactive material.

Management of Pediatric cholecystitis

Managing pediatric acute cholecystitis involves short-term and long-term goals. The short-term goals revolve around managing pain, controlling inflammation, and removing the inciting factor if any. The treatment is surgical cholecystectomy.

Long-term goals include ruling out and treating the presence of underlying predisposing factors, such as obesity, control of hemolytic disorders, evasion of a hemolytic crisis in patients with sickle cell disease, and other hemoglobinopathies. Preventing repeated bouts of subacute cholecystitis in patients with hemolytic disorders and curbing progression to chronic cholecystitis is also essential. One needs to mitigate risk factors in individuals who are at risk. Weight loss is often a simple, yet effective, solution in such pediatric patients.

More data and research are required to determine conclusively when to operate and when to conserve a child with acute cholecystitis.

Laparoscopic cholecystectomy, albeit being arduous in the pediatric population, is standard management. It involves a faster recovery, shorter hospital stay, and a lower overall cost of management. The other advantages of laparoscopic cholecystectomy can be listed as follows:

  • Minimally invasive nature.
  • Cosmetic scar.
  • Evasion from muscle cutting incisions and its complications.
  • Enhanced post-operative recovery.
  • Diminished post-operative pain.
  • Early recovery and functional rehabilitation.
  • Easy and quick mobilization.

The most relevant pivotal aspects of laparoscopic cholecystectomy which are unique to the pediatric population can be mentioned as follows:

  • Respect the space constraints.
  • Epigastric cannula placement in the left upper quadrant.
  • Right-sided working and retracting ports are, respectively, inserted in the lumbar or iliac regions.
  • The controversial role of intraoperative cholangiography and common bile duct exploration.
  • Easier dissection at the Calot’s triangle due to smaller fat deposits and flimsy peritoneal covering.

Prognosis

Prognosis of pediatric acalculus cholecystitis is often concerning, and the overall mortality rate is estimated to be around 30% of cases. The presence of shock and a low fibrinogen value are predictable factors for mortality in pediatric acalculus cholecystitis.

Summary

Cholecystitis is inflammation of the gallbladder. The incidence of pediatric cholecystitis is increasing. It is associated with systemic diseases and is potentially curable.

Cholecystitis in the pediatric population is different from that in adults. Most children suffer from idiopathic cholelithiasis.

Acute cholecystitis is often a clinical diagnosis with classic signs and symptoms. In acute cholecystitis, Murphy’s sign and Boas’s sign can be elicited.

Ultrasonography is a simple diagnostic test with high sensitivity and specificity. Sonographic Murphy’s sign is increased abdominal tenderness on the application of pressure of the ultrasonography probe. Other imaging studies, such as CT scan, MRI, and HIDA scan are seldom used in the pediatric population.

Laparoscopic cholecystectomy is the standard treatment. There are various benefits of laparoscopic cholecystectomy over open surgical cholecystectomy; the most significant are early recovery and mobilization.

Other important management features include the prevention of bouts of cholecystitis in patients with systemic diseases, control of risk factors, and avoidance of developing chronic cholecystitis.

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