Rosary and Pearl Necklace

Question: 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 (CT) scan showed marked gallbladder wall thickening with an annular unenhanced proliferative muscularis layer surrounding enhanced proliferative mucosal epithelium (Figure A), and magnetic resonance imaging (MRI) showed multiple cyst-like spaces in the gallbladder wall (Figure B and C). What is the diagnosis, and how should it be managed? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Based on the clinical and imaging findings, a diagnosis of gallbladder adenomyomatosis (GA) was made. GA is a benign and usually asymptomatic condition that occurs mainly beyond the age of 50 to 60 years and is very rare in childhood.1Eroğlu N. Erduran E. İmamoğlu M. et al.Diffuse adenomyomatosis of the gallbladder in a child.J Pediatr Hematol Oncol. 2016; 38: e307-e309Crossref PubMed Scopus (4) Google Scholar Symptomatic gallbladder adenomyomatosis indicates cholecystectomy, considering the presence of inflammation or gallbladder stones.2Bonatti M. Vezzali N. Lombardo F. et al.Gallbladder adenomyomatosis: imaging findings, tricks and pitfalls.Insights Imaging. 2017; 8: 243-253Crossref PubMed Scopus (35) Google Scholar 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. According to the gross features and areas affected, GA is classified into 4 types: localized, segmental, annular, and diffuse.2Bonatti M. Vezzali N. Lombardo F. et al.Gallbladder adenomyomatosis: imaging findings, tricks and pitfalls.Insights Imaging. 2017; 8: 243-253Crossref PubMed Scopus (35) Google Scholar 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.3Hammad A.Y. Miura J.T. Turaga K.K. et al.A literature review of radiological findings to guide the diagnosis of gallbladder adenomyomatosis.HPB (Oxford). 2016; 18: 129-135Abstract Full Text Full Text PDF PubMed Scopus (24) Google Scholar Given the problem of difficult visualization of coexisting malignancy, cholecystectomy should be routinely considered for patients with diffuse GA.2Bonatti M. Vezzali N. Lombardo F. et al.Gallbladder adenomyomatosis: imaging findings, tricks and pitfalls.Insights Imaging. 2017; 8: 243-253Crossref PubMed Scopus (35) Google Scholar