|Year : 2021 | Volume
| Issue : 3 | Page : 107-110
Adenomyomatosis of the gall bladder in an 8-year-old girl
Tune-Yie Shih1, Wei-Yun Hsu2, Hong-Lin He3
1 Division of Pediatric Surgery, Department of Surgery, Chi-Mei Medical Center, Tainan, Taiwan
2 Department of Pediatrics, Chi-Mei Medical Center, Tainan, Taiwan
3 Department of Pathology, Chi-Mei Medical Center, Tainan, Taiwan
|Date of Submission||12-May-2020|
|Date of Decision||17-Dec-2020|
|Date of Acceptance||25-Jan-2021|
|Date of Web Publication||12-Jun-2021|
Division of Pediatric Surgery, Department of Surgery, Chi-Mei Medical Center, No. 901, Tzong-Huang Road, Yongkang Dist., Tainan 71004
Source of Support: None, Conflict of Interest: None
Adenomyomatosis of the gall bladder (AMG), with its characteristic Rokitansky-Aschoff sinuses, is a rare disease in children, and only 13 pediatric cases have been reported in the literature. An 8-year-old girl was diagnosed in our institute with symptomatic gall bladder adenomyomatosis and received cholecystectomy with uneventful postoperative course. The pediatric AMG should be regarded as a special type of AMG with different pathophysiology and natural course from those of the adults. Thus, the surgical indications of AMG in children should be considered meticulously to avoid unnecessary cholecystectomy..
Keywords: Adenomyomatosis of the gall bladder, cholecystectomy, Rokitansky-Aschoff sinuses
|How to cite this article:|
Shih TY, Hsu WY, He HL. Adenomyomatosis of the gall bladder in an 8-year-old girl. Formos J Surg 2021;54:107-10
| Introduction|| |
Adenomyomatosis of the gall bladder (AMG) is an unique entity in gall bladder diseases and is characterized by proliferation of the epithelial mucosa and hypertrophy of the smooth muscle layer with invagination of the mucosa into the muscular wall, forming intramural diverticula termed Rokitansky-Aschoff sinuses (RAS).,,,,,,,,, AMG is relatively common in adults and is present in about 5% of all cholecystectomy specimens.,, On the contrary, AMG is very rare in children. We report a pediatric case of AMG, review the literature, and explore the differences in the clinical presentations and management strategies between the children and the adults.
| Case Report|| |
An 8-year-old female was referred to the pediatric surgery clinic for the surgical evaluation of thickening of the gall bladder wall. She sought medical attention because of bilateral breast enlargement. As part of the precocious puberty workups, an abdominal ultrasonography to check the adrenal glands was performed and incidentally revealed diffused thickening of the gall bladder wall. Other laboratory tests data were normal.
She gave a history of repeated attacks of chest tightness, epigastralgia with nausea during exercise in the recent 2 years. These symptoms were self-limited but occurred frequently. On physical examination, the right breast measured 3 cm × 2.5 cm and the left 3 cm × 3 cm. The abdomen was soft with mild tenderness at the right upper guardant and periumbilical area. A repeated ultrasound of the hepatobiliary system revealed echogenic foci in diffusely thickened gall bladder wall with ring-down artifacts [Figure 1]. The clinical impression was AMG. Magnetic resonance cholangiopancreatography (MRCP) was done subsequently and showed focal wall-thickening of gall bladder fundus and no other biliary system abnormalities. With the diagnosis of symptomatic AMG, a laparoscopic cholecystectomy was performed. The resected gall bladder revealed mural thickening involving the distal half, with the thickest (up to 1.5 cm) at the distal part of the body [Figure 2]. The pathological examination revealed hyperplastic mucosal glands encircled by thick smooth muscle bundles with the formation of RAS within the underlying smooth muscle layer, consistent with adenomyomatosis [Figure 3]. The postoperative recovery was smooth, and the patient had been free of the exercise associated discomforts in the following 6 months.
|Figure 2: The opened specimen showing hyperplasia of mucosa and hypertrophy of muscular wall involving the distal half of the gall bladder|
Click here to view
|Figure 3: Rokitansky-Aschoff sinuses in the smooth muscle layer of the gall bladder (H and E stain, ×100)|
Click here to view
| Discussion|| |
The first report of pediatric AMG was in a 5-year-old boy in 1998. To the best of our knowledge, there were only 13 cases of pediatric AMG reported in the literature,,,,,, and this girl may be the first case of pediatric AMG reported in Taiwan.
Although AMG is believed to be a benign, acquired disease, its etiology, pathophysiology, natural course, and optimal therapeutic protocol remain unclear.,,,,, A frequently cited theory is that changes in intracystic pressure (caused by abnormal neuromuscular activities) may lead to proliferation of the mucosa and hyperplasia of the muscle layer, causing invagination of the epithelium into the muscular wall, with the formation of RAS.,, AMG in adults is associated with gall stones in up to 91.7% of the cases. The nature of AMG is thought to be benign but was found in 6.4-25% of cases of gall bladder cancer., AMG was also known as acquired, but it had been reported in infants and showed clear evidences of spontaneous resolution.,,
The symptoms of AMG are usually vague, nonspecific, recurrent abdominal discomforts but some cases required emergency services because of acute right upper quadrant pain.,,,,,, Most cases of adult AMG were thought to be asymptomatic, and this may in part account for its rarity in the pediatric population, since it may remained undiscovered to the adulthood till the onsets of pain related to stone formation or inflammation.,,, However, the symptoms of AMG may also occur without gall stones or cholecystitis.
AMG is classified into three types according to the extent of involvement: generalized (diffuse), segmental (annular), and localized (fundal).,,,,, The opinion to which type being the most common varied according to different authors. The segmental type is annular thickening in the body of the gall bladder which was separated into two communicating compartments. The localized or fundal type is limited to the fundus and usually presents as a nodule protruding from the gall bladder fundus. Our case could be classified as combined segmental and fundal types.
The diagnosis of AMG is based on the visualization of the pathognomonic lesions – RAS in the thickened muscular wall.,,,,,,,,, Ultrasonography is the first-line choice to make the image diagnosis because it is noninvasive and easily repeatable. Sonographic findings include gall bladder wall thickening, intramural anechogenic (bile-filled) cysts or echogenic (stone or sludge-filled) foci, and V-shaped ring down (comet tail) artifacts. These artifacts have been proposed to result from reverberation between the components or contents of RAS.,,,,, The reported sensitivity of ultrasound is from 43 to 80%, the specificity from 54 to 96%, and the accuracy from 66 to 89%. In the absence of typical ultrasound findings of AMG, other diagnostic modalities may be helpful. In some cases, a “rosary sign” is found on computed tomography (CT) and is due to enhancement of the invaginated epithelium surrounded by the unenhanced muscle layer.,, Similarly, the “pearl necklace” sign on T2-weighted phase of magnetic resonance imaging (MRI) has been described as high-intensity cavities in the gall bladder wall, representative of RAS.,,, Some authors have claimed that MRCP should be performed for detailed preoperative evaluation because major variations and anomalies in the pancreatobiliary system may accompany up to 18% of cases of AMG.,,
The optimal therapeutic protocol for AMG has not been established. This is mainly due to the lack of data regarding the long-term consequences of this condition in the general population. However, it is generally accepted that symptomatic cases should be operated on.,,,,,, Cholecystectomy is a reasonable choice since all reported patients who have undergone cholecystectomy have been free of symptoms postoperatively. For the asymptomatic cases in whom AMG is diagnosed incidentally, the management strategy remains controversial. Although many authors have recommended conservative treatment with regular ultrasonographic monitoring for asymptomatic AMG,,,, some have claimed that the conservative strategy should be reserved only for patients with clear contraindications to surgery., The latter group consider that AMG predisposes the patients to stone formation and chronic inflammation, which may lead to dysplasia and cancer. Furthermore, it is sometimes impossible to distinguish AMG from gall bladder carcinoma before surgery., The reports of complete resolutions of AMG in infants have not been found in other age groups, and whether a symptomatic patient has the chance to recover spontaneously is never investigated. In fact, the diagnosis of AMG in unoperated patients may be doubtful since false positive results do exist using ultrasonography, CT, or MRI except when pathological confirmation is available. From these points of view, the operation for AMG is both therapeutic and diagnostic.
Including our case, there are 14 cases of pediatric AMG to our knowledge.,,,,,, The ages at diagnosis range from 12 hours to 17 years with a median age of 8 years. The numbers of cases in each sex are equal. The classifications include diffuse type in four cases, segmental types in three, localized type in three, combined fundal and segmental types in two, and unclear type in two. Except for the three infants (aged 12 hours, 6 weeks and 4 months), all patients were symptomatic with acute pain in three and chronic pain in eight. The laboratory studies were not contributory to the diagnosis. The imaging methods included ultrasonography (n = 11), CT (n = 2), and MRI/MRCP (n = 7). No association with stone or malignancy was found. All symptomatic cases received cholecystectomy (open in five and laparoscopic in six) with subsequent relief of their symptoms. The asymptomatic infants were monitored with regular ultrasound and complete resolution of the lesions were observed in the aforementioned two infants at 8 and 15 months of age, respectively., The infant diagnosed at 4 months of age had no change of her gall bladder lesion at 7 months of age, and no information was available thereafter.
Obviously, the present therapeutic guidelines of AMG for pediatric patients follow those for the adults. However, in our opinion, the pediatric AMG should be regarded as a special type of AMG with different pathophysiology and natural course from those of the adults. Thus, the surgical indications of AMG in children should be considered meticulously to avoid unnecessary cholecystectomy. More experiences and data are needed to establish the optimal therapeutic protocol for pediatric AMG.
Declaration of patient consent
The authors certify that they have obtained appropriate patient's guardian consent form. In the form, the guardian has given the consent for the child's images and other clinical information to be reported in the journal. The guardian understands that the child's name and initial will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Zarate YA, Bosanko KA, Jarasvaraparn C, Vengoechea J, McDonough EM. Description of the first case of adenomyomatosis of the gallbladder in an infant. Case Rep Pediatr 2014;2014:248369.
Parolini F, Indolfi G, Magne MG, Salemme M, Cheli M, Boroni G, et al
. Adenomyomatosis of the gallbladder in childhood: A systematic review of the literature and an additional case report. World J Clin Pediatr 2016;5:223-7.
Chen CB, Kabbany MN. Gallbladder adenomyomatosis in an infant. ACG Case Rep J 2020;7:e00433.
Kinoshita H, Ariga H, Shirota J, Sasaki K, Shibukawa Y, Fukuda Y, et al
. Combined fundal and segmental adenomyomatosis of the gallbladder in a child: A rare case report. Case Rep Pediatr 2019;2019:2659089.
Alapati S, Braswell LE. Neonatal adenomyomatosis of the gallbladder: An incidental finding at 12 h of life. Radiol Case Rep 2015;9:859.
Alberti D, Callea F, Camoni G, Falchetti D, Rigamonti W, Caccia G. Adenomyomatosis of the gallbladder in childhood. J Pediatr Surg 1998;33:1411-2.
Pasierbek M, Korlacki W, Grabowski A. Adenomyomatosis of the gallbladder in an adolescent, a very rare condition. Arch Argent Pediatr 2020;118:e43-7.
Bevan G. Acalculous adenomyomatosis of the gallbladder. Gut 1970;11:1029-34.
Moon J, Shin YC, Heo TG, Choi PW, Kim JI, Jung SW, et al
. Differentiation of gallbladder adenomyomatosis from early-stage gallbladder cancer before surgery. Ann Hepatobiliary Pancreat Surg 2019;23:334-8.
Hammad AY, Miura JT, Turaga KK, Johnston FM, Hohenwalter MD, Gamblin TC. A literature review of radiological findings to guide the diagnosis of gallbladder adenomyomatosis. HPB (Oxford) 2016;18:129-35.
[Figure 1], [Figure 2], [Figure 3]