Pediatric cholecystitis is an infrequent but significant pathology in children because of its association with systemic diseases and the availability of a potential cure in the form of surgery. This article envisages laying out the basics of pediatric cholecystitis with the clinical aspect being the prime focus.
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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. Compared to adults; pediatric cholecystitis is rather seldom encountered. There are subtle differences in the anatomical and pathological features of this disease between adults and children; once again reiterating the fact that children are not small adults.

Extensive, dedicated research is yet to be carried out to discern confidently the clinicopathological features and to establish the best treatment options. The differences thus noticed can be tabulated as below:

Feature Adult cholecystitis Pediatric cholecystitis
Incidence It is a very common disease. It is an infrequent diagnosis.
Sex ratio It is more common in females. This justifies the famous statement expounding the typical patient of gallstones; “Fat, fertile, female of forty”. There is no female predisposition. Evidence attests to the fact that the sex ratio of affected children reflects neutral behavior or even more susceptibility in males.
Presentation Majority are asymptomatic. Very few children suffer from an asymptomatic disease.
Pathology Higher proportion of stone disease secondary to cholesterol. Escalated incidence of pigment stones.
Association Sporadic quite common. Significant association with few specific systemic disorders noticeably present.
Operative techniques Laparoscopic cholecystectomy is the gold standard and is usually a relatively standard procedure 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

Incidence of pediatric cholelithiasis and cholecystitis is on the rise. The best estimate of 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 comprise of the cystic duct joining the:

  • Common bile duct
  • Common hepatic duct
  • Superiorly the 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 calculous cholecystitis
  • Acute acalculous cholecystitis
  • Emphysematous cholecystitis
  • Xanthogranulomatous cholecystitis
  • Suppurative cholecystitis

Pathogenesis of Pediatric cholecystitis

The key factors in the formation of gallstones 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 is often associated with certain systemic disorders. These conditions make the child susceptible to the gallstone disease. Obstruction of the neck of the cystic duct by a stone in 90% of cases is the most common reason for emergency cholecystectomy in children; especially with one or more factors rendering 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 sex
  • hemolytic uremic syndrome
  • hemoglobinopathies

Clinical Features of Pediatric cholecystitis

Vignette

A girl with fever and severe abdominal pain. She has a fever and has been losing weight despite noticing abdominal bloating. She is rushed to the OR. Her BMI is 30. Pain is of acute nature, sharp character; located in the right upper quadrant. This abdominal pain radiates to the back. Ultrasonography reflects gallstones and thickened gallbladder wall.
What do her providers suspect she has?

Clinical signs and symptoms

The example above beautifully illustrates the classical presentation of acute cholecystitis. Acute cholecystitis 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 possesses the following unique properties:

  • Acute in nature
  • Sharp, colicky in character
  • Epigastric or right upper quadrant location
  • Radiation to the back or the 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 famous specific signs expatiated during clinical assessment of a patient with acute cholecystitis. They have been summarized below for easy memorization and recall:

Murphy´s sigh

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

Boas´s sign

Escalated sensitivity below the right scapula with hyperaesthesia is encountered in 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 can be tabulated as follows:

  • 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 role of imaging studies with a beneficiary role in the diagnosis of cholecystitis can be briefly mentioned 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. 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 necessity of great co-operation from the patient.

ERCP

ERCP stands for Endoscopic Retrograde Cholangio Pancreatography. It is an invasive test and has a key diagnostic and simultaneous therapeutic role in the management of 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 with diagnostic use only. It can demonstrate impacted stones as filling defects. Co-operation from the pediatric population for such procedures is very difficult to procure and hence these investigations are quite infrequently used as compared to the adult community.

Technitium HIDA scan

Hepatobiliary Iminodiacetic Acid Scan is the most sensitive test for diagnosis of cholecystitis. The radioactive tracer is injected and subsequently, drainage of the same is documented. With radioactive material involved; HIDA usage is discouraged in children.

Management of Pediatric cholecystitis

Management of a child with acute cholecystitis involves short-term and long-term goals. The short-term goals evolve around pain management, inflammation control and removal of 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. Prevention of 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 conclusively determine when to operate and when to conserve a child with acute cholecystitis.

Laparoscopic cholecystectomy, albeit being arduous in the pediatric population, is the standard management. It involves a faster recovery, a diminished hospital stay and a decreased 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 lesser fat deposit and flimsy peritoneal covering.

Prognosis

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

Summary

Cholecystitis is inflammation of the gallbladder. The incidence of pediatric cholecystitis is on an increase. It is associated with system diseases and is potentially curable.

Cholecystitis in the pediatric population is different as compared to adults. The majority of 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 being 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 the development of chronic cholecystitis.

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