Clinical Challenges

What's your diagnosis?

Publish date: August 1, 2023
By
Jiayu Yan, MD
Wei Chen, MD
Yajun Chen, MD, PHD

A 15-year-old girl presented with an 18-month history of intermittent right upper quadrant pain that appeared after meals and was relieved after rest. She denied any nausea, vomiting, chills, diarrhea, or constipation. The patient reported no trauma. At admission, physical examination showed tenderness in the right upper abdomen without rebound or guarding. Murphy's sign was also present. The laboratory tests were unremarkable.


Ultrasound examination indicated gallbladder wall thickening. Furthermore, a contrast-enhanced computed tomographic scan showed marked gallbladder wall thickening with an annular unenhanced proliferative muscularis layer surrounding enhanced proliferative mucosal epithelium (Figure A), and magnetic resonance imaging showed multiple cyst-like spaces in the gallbladder wall (Figures B and C).
What is the diagnosis, and how should it be managed?


 

Previously published in Gastroenterology

The diagnosis

Based on the clinical and imaging findings, a diagnosis of gallbladder adenomyomatosis was made. GA is a benign and usually asymptomatic condition that occurs mainly beyond the age of 50-60 years and is very rare in childhood.1 Symptomatic gallbladder adenomyomatosis indicates cholecystectomy, considering the presence of inflammation or gallbladder stones.2 Therefore, a laparoscopic cholecystectomy was performed on our patient. Rokitansky-Aschoff sinuses were seen in the entire thickened gallbladder wall on gross pathologic examination (Figure D). Histopathologic examination confirmed the diagnosis of GA with cholecystitis. The patient was eventually diagnosed with diffuse GA. She was successfully discharged from the hospital 4 days after surgery, and 3 months of follow-up were uneventful.

Clinical challenge (Gastro Hep Advances/AGA)
According to the gross features and areas affected, GA is classified into four types: localized, segmental, annular, and diffuse.2 To our knowledge, this case presents the most distinguished imaging findings of diffuse GA in the English literature, including the “rosary sign” on contrast-enhanced CT and the “pearl necklace sign” on T2-weighted MRI.3 Given the problem of difficult visualization of coexisting malignancy, cholecystectomy should be routinely considered for patients with diffuse GA.2

References

Eroglu N et al. Diffuse adenomyomatosis of the gallbladder in a child. J Pediatr Hematol Oncol. 2016;38:e307-9.

Bonatti M. et al. Gallbladder adenomyomatosis: imaging findings, tricks and pitfalls. Insights Imaging. 2017;8:243-53.

Hammad AY et al. A literature review of radiological findings to guide the diagnosis of gallbladder adenomyomatosis. HPB (Oxford). 2016;18:129-35.

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