What is the diagnosis and treatment for congenital gallbladder hypoplasia (underdeveloped gallbladder)?

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Last updated: April 28, 2025View editorial policy

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From the Guidelines

Gallbladder hypoplasia is a rare congenital condition that should be managed symptomatically, with surgical intervention reserved for severe cases or recurrent cholecystitis. This condition is characterized by an underdeveloped gallbladder that is smaller than normal size, often going undiagnosed until adulthood when patients present with symptoms similar to those of gallbladder disease, such as right upper quadrant pain, nausea, vomiting, and intolerance to fatty foods. Management typically involves symptomatic treatment rather than surgical intervention unless symptoms are severe. Patients should follow a low-fat diet, take small frequent meals, and may benefit from bile acid supplements like ursodeoxycholic acid (typical dose 8-10 mg/kg/day divided into 2-3 doses) to improve bile flow and reduce symptoms, as seen in the management of other hepatobiliary diseases 1. Pain management with NSAIDs or acetaminophen can be used for acute episodes. Cholecystectomy is generally not recommended as the primary treatment since the gallbladder is already underdeveloped, but may be considered in cases of recurrent cholecystitis or severe symptoms unresponsive to medical management, with laparoscopic cholecystectomy being the preferred approach when surgery is necessary 1. The condition results from incomplete development during embryogenesis, where the gallbladder bud fails to fully develop. Regular follow-up with a gastroenterologist is important to monitor symptoms and adjust treatment as needed. Key considerations in management include:

  • Symptomatic treatment as the primary approach
  • Dietary modifications to reduce symptoms
  • Use of bile acid supplements to improve bile flow
  • Reserved use of cholecystectomy for severe or recurrent cases
  • Preference for laparoscopic cholecystectomy when surgical intervention is necessary, as recommended for other conditions like acute calculus cholecystitis 1.

From the Research

Definition and Diagnosis of Gallbladder Hypoplasia

  • Gallbladder hypoplasia is a congenital abnormality of the gallbladder, which is rare and difficult to diagnose with imaging studies 2.
  • Patients with gallbladder hypoplasia are usually asymptomatic or have symptoms that mimic gallstone disease.
  • The diagnosis is often made intraoperatively and is established by histopathological examination.

Clinical Presentation and Treatment

  • A case report of a 62-year-old male with cholelithiasis symptoms, who was falsely diagnosed with gallstone disease by abdominal ultrasound scan, revealed a rudimentary gallbladder with hypoplastic gallbladder tissue 2.
  • Surgeons need to consider congenital anomalies of the gallbladder intraoperatively to avoid iatrogenic injury to the biliary tract during routine laparoscopic cholecystectomy.
  • Intraoperative cholangiography can be a useful tool to avoid unnecessary surgical risky interventions.

Related Conditions and Treatment Options

  • Cholesterol gallstones are a common gastrointestinal disorder, and individuals with gallstones may experience various gastrointestinal symptoms 3.
  • Ursodeoxycholic acid (UDCA) is a bile acid that dissolves gallstones, and there is increasing interest in UDCA for symptomatic gallstones, particularly in those unfit for surgery 4.
  • Cholecystectomy is indicated for patients with symptomatic gallstones or sludge, and should be performed laparoscopically with a four-trocar technique, if possible 5.

Incidental Findings and Follow-up

  • Incidental dysplasia in the gallbladder mucosa can be found on occasional cholecystectomies, and additional sections should be taken to rule out underlying adenocarcinoma 6.
  • The incidence of subsequent identification of invasive adenocarcinoma on additional sections is low, and cholecystectomy is sufficient treatment for gallbladder dysplasia.
  • However, patients with incidental dysplasia in the gallbladder may have an associated pancreatobiliary carcinoma, and serial imaging follow-up may be necessary to assess for subsequent development of neoplasia elsewhere in the pancreaticobiliary tree.

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Nutritional approaches to prevention and treatment of gallstones.

Alternative medicine review : a journal of clinical therapeutic, 2009

Research

The Treatment of Gallstone Disease.

Deutsches Arzteblatt international, 2020

Research

Dysplasia in Gallbladder: What Should We Do?

Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract, 2019

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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