Adenomyomatosis of the gallbladder (AMG) is one of the proliferative cholesteroses characterized by epithe- lial proliferation and the formation of mucosal ...
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CASE REPORT
Adenomyomatosis of the Gallbladder in a Child SALIH CETINKURSUN, MD,* ILHAMI SURER, MD,* SALIH DEVECI, MD,† SUZI DEMIRBAG, MD,* ¨ MUTLU SAGLAM, MD,‡ CUNEYT ATABEK, MD,* and HALUK OZTURK, MD* KEY WORDS: adenomyomatosis; gallbladder; cholecystectomy.
Adenomyomatosis of the gallbladder (AMG) is one of the proliferative cholesteroses characterized by epithelial proliferation and the formation of mucosal pouches into and often beyond thickened muscular layers. These pouches were first described by Rokitansky in 1842 and later by Aschoff in 1905. Today they are frequently called the Rokitansky–Aschoff sinuses and are the classical and diagnostic feature of AMG, whose incidence varies from 2.8% to 5% in gallbladder examinations (1–3). This entity was diagnosed chiefly by radiologists, but the pathogenesis, pathology, and indications for surgery was not well understood for years. Although gallbladder diseases in children are reported with increasing frequency because of the widespread use of ultrasonography as a diagnostic tool (4), the occurence of AMG in children has been reported to date only in one case (5). Here we report the another case of symptomatic AMG that was successfully treated by open cholecystectomy. CASE REPORT A 6-year-old boy was referred to our department for recurrent abdominal pain. His previous in-patient history was as follows: the patient had admitted to another center 1.5 years ago with abdominal pain, fever, and bile vomiting. Hepatitis A, B, C; Epstein-Barr virus infection; and infectious, metabolic, and autoimmune diseases had been ruled out previously, and he had been treated empirically. Recurrent abdominal pain was the unique symptom since that time. Hydatid disease of liver had been diagnosed in boy’s mother 1 year ago and the animal contact history of the child was positive. On admission, the child had normal height and weight for his age and sex (50th percentile). Physical examination findings were normal except for a faintly positive Murphy’s sign. Results of laboratory investigations, including hemograms, determination of transaminases, GGT, alkaline phosphatase, Manuscript received February 26, 2002; accepted June 2, 2002. From the Departments of *Pediatric Surgery, †Pathology, and ‡Radiology, G¨ulhane Military Medical Academy, Ankara, Turkey. Address for reprint requests: Dr. Ilhami Surer, Department of Pediatric Surgery, G¨ulhane Military Medical Academy 06018, Etlik-Ankara, Turkey.
bilirubin, and amylase, were negative but ELISA test for hydatid disease was positive. An ultrasound scan of the gallbladder and abdomen confirmed a small and multiseptated gallbladder with thickened wall (Figure 1). The remaining biliary tree and abdominal organs were normal. Further investigations included abdominopelvic computerized tomography, which showed normal parenchymal structure of abdominal organs and no primary or satellite cyst to confirm dissemination of hydatid disease; and magnetic resonance cholangiopancreotography (MRCP), which showed a small gallbladder with lobulated mucosa and multiple heterogenous hyperintense cysts but no stone in the gallbladder (Figure 2). Preoperatively, isolated hydatid disease of the gallbladder was mentioned in the differential diagnosis of this pathology due to positive animal contact history, positive ELISA test result, and several radioimaging examination results. Open cholecystectomy was performed. The postoperative course was uneventful and the child was discharged from the hospital on day 5. After 1 year of follow-up the patient is symptomfree with normal laboratory test results. Pathological Findings. The excised gallbladder measured 3 × 1.5 cm. It displayed a thickened wall (6 mm) and diffuse diverticulosis of the mucosa beyond the thickened gallbladder muscular layer with normal serosal surface. Microscopic findings of the specimen showed features of adenomyomatosis made up of a mixture of glands, smooth muscle bundles, and dilated Rokitansky–Aschoff sinuses (Figure 3).
DISCUSSION Adenomyomatosis was defined by Jutras and Levesque (6) in 1960 as a pathological condition characterized by hyperplasia of the gallbladder wall due to excessive proliferation of the epithelium and thickening of the muscular layer. The normal gallbladder wall is no more than 1 mm thick and does not contain mucosal folds. However, when the gallbladder becomes thick enough, it may show a foldlike appearance due to projections of the markedly thickened wall into the lumen. These intraluminal projections can be seen as thick radiolucent lines, which produce an irregular outline of the gallbladder wall (7). As shown in our case, these folds can mimic septation and even modern imaging techniques may not be
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Fig 1. Ultrasonographic view: small, multiseptated gallbladder with thickened wall.
Fig 2. MRCP: small gallbladder and intact biliary tree; lobulated gallbladder mucosa (arrows).
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Fig 3. Generalize type adenomyomatosis; intramural dilated glands and Rokitansky–Aschoff sinuses (H&E, 50)
helpful to determine definitive pathology. The pathogenetic mechanism resulting in mucosal pseudodiverticula formation (Rokitansky–Aschoff sinuses) is possibly related to an increased intraluminal pressure, primarily caused by abnormalities of muscle contraction, similar to diverticular disease of the colon (8). Although Rokitansky–Aschoff sinuses are seen to a certain degree in chronic cholecystitis, they are not as prominent and dilated as in AMG and the sinuses seldom penetrate the muscular layer (9). According to the morphological classification, AMG is currently divided into three types: generalized, localized, and segmental (3, 10). The incidence of AMG in the adult population is unknown; the disease has been reported to account for over 40% of all benign gallbladder lesions (11). However, to the best of our knowledge, AMG in the pediatric age group has been reported only in one case (5). In recent years, attention has been drawn its malignant potential. Different adult series have described an incidence of up to 6.4% of gallbladder cancer developing in patients with segmental AMG (12–14), and AMG is associated with gallstones in up to 91.7% of the cases (13). Due to the rarity of this entity in the pediatric population, not enough information and experience has been obtained yet for the epidemiology and treatment principles of the disease. Clinical diagnosis of this entity is almost impossible. The nature of pain in this group of patients was not different from that caused by gallstones, cholecystitis, or biliary colic (3, 15, 16). The several studies pointed out Digestive Diseases and Sciences, Vol. 48, No. 4 (April 2003)
marked a correlation of ultrasonographic and pathological findings; therefore ultrasonography is an easy and noninvasive diagnostic tool for the AMG (13, 17). The most characteristic sonographic appearance common to all cases was the presence of anechogenic (bile filled) or echogenic (biliary sludge or gallstones filled) foci within the thickened gallbladder wall. In more complicated cases with biliary symptoms but no evidence of gallstones and infection, more sophisticated investigations such as Tc99 HIDA radionuclide scan, percutaneous transhepatic cholecystocholangiography, endoscopic retrograde pancreoticocholangiography (5), or MRCP (as performed in our case) may be indicated for the surgical decision. However, all these techniques and laboratory tests may fail to determine a definitive pathology, and a high index of suspicion should be always the essential step in the preoperative assessment period. We have reported on a child with AMG, requiring cholecystectomy to determine the exact pathology and obtain symptomatic relief. However, AMG in childhood still is a surgical challenge due to the lack of clear answers to questions such as whether the incidental radiological finding of adenomyomatosis in a child who has no biliary symptoms warrants surgery. Although there are several reports that delay in treatment carries malignancy risk, the authors believe that before one can suggest cholecystectomy in every child with symptomatic or asymptomatic AMG, there is a need for better understanding of the natural course of this entity. When surgical treatment is chosen, type of the operation should be arranged
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according to the patient’s clinical situation and the surgeon’s experience. REFERENCES 1. Fotopoulos JP, Crampton AR: Adenomyomatosis of the gallbladder. Med Clini North Am 48:9–36, 1964 2. Colquhoun J: Adenomyomatosis of the gallbladder (intramural diverticulosis). Br J Radiol 34:101–112, 1961 3. Jutras JA: Hyperplastic cholecystoses. Am J Roentgenol 83:795– 827, 1960 4. Debray D, Pariente D, Gauthier F, Myara A, Bernard O: Cholelithiasis in infancy: a study of 40 cases. J Pediatr 122:385–391, 1993 5. Alberti D, Callea F, Camoni D, Falchetti D, Rigamonti W, Caccia G: Adenomyomatosis of the gallbladder in childhood. J Pediatr Surg 33:1411–1412, 1998 6. Jutras JA, Levesque HP: Adenomyoma and adenomyomatosis of the gallbladder. Radiologic and pathologic correlation. Radiol Clin North Am 4:483–500, 1966 7. Cynn WS, Forbes T, Schreiber M: Unusual radiographic manifestations of adenomyomatosis of the gallbladder. Radiology 113:577– 579, 1974 8. Williams J, Slavin G, Simpson P, Cox A, de Lacey G: Diverticular disease (adenomyomatosis) of the gallbladder: A radiological– pathological survey. Br J Radiol 59:29–34, 1986
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9. Ram MD, Midha D: Adenomyomatosis of the gallbladder. Surgery 78:224–229, 1975 10. Weedon D: Disease of the gallbladder. In Pathology of the Liver. RNM MacSween, PP Antony, PJ Scheur, AD Burt, KG Ishak, B Portmann (eds). Edinburgh, Scotland Churchill Livingstone, 1994, pp 525–526 11. Christiensen AH, Ishak KG: Benign tumours and pseudotumours of the gallbladder. Arch Pathol Lab Med 90:423–432, 1979 12. Katoh T, Nakai T, Hayshy S, Satake T: Non invasive carcionoma of the gallbladder arising in localized type adenomyomatosis. Am J Gastroenterol 83:670–674, 1988 13. Ootani T, Shirai Y, Tsskada K, Muto T: Relationship between gallbladder carcinoma and the segmental type of adenomyomatosis of the gallbladder. Cancer 69:2647–2652, 1992 14. Aldrige MC, Bismuth H: Gallbladder cancer. The polyp–cancer sequence. Br J Surg 77:363–364, 1990 15. Heald RJ: Adenomyomatosis as a source of error in the diagnosis of gallbladder disease. Br J Surg 57:353–356, 1970 16. LeQuesne LP, Ranger I: Cholecystitis glandularis proliferans. Br J Surg 44:447–448, 1957 17. Fowler RC, Reid WA: Ultrasound diagnosis of adenomyomatosis of the gallbladder: Ultrasonic and pathologic correlation. Clin Radiol 39:402–406, 1988
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