Porcelain

Gallbladder in a Child: A Case Report and Review

By Helen B. Casteel,

Susan L. Williamson, Little

0 Calcification of the gallbladder wall (porcelain gallbladder; PGB) is a rare form of gallbladder disease not previously described in a child. A IO-year-old girl is presented with PGB that was discovered incidentally during intravenous urography. Computed tomography localized the calcification to the gallbladder wall. Cholecystectomy was performed due to the associated increased incidence of biliary tract carcinoma reported in adult patients. The etiology, diagnosis, and management of PGB and its significance in a pediatric patient are discussed. 0 7990 by W.B. Saunders Company. INDEX WORDS: der.

Porcelain gallbladder; calcification,

gallblad-

P

ORCELAIN gallbladder (calcification of the gallbladder wall; PGB) is rare in adults, occurring in 0.06% to 0.8% of cholecystectomy specimens.’ Although unusual, the finding of a calcified gallbladder has clinical significance consequent to the associated incidence of primary biliary cancer. This report describes a lo-year-old child with calcification of the gallbladder of unknown etiology. Although numerous cases of PGB have been reported, only adults have previously been noted to have PGB. CASE

REPORT

A lo-year-old white girl was referred for evaluation of PGB noted on intravenous urography, which was performed for evaluation of recurrent urinary tract infections (Fig 1). The patient denied gastrointestinal symptoms such as abdominal pain, vomiting, diarrhea, or food intolerances. She had been healthy and growing well. There was no history of abdominal trauma, hematologic disease, or use of total parenteral nutrition. The family history was unremarkable for gallbladder disease. Her physical examination was normal, and showed a well-developed, well-nourished child. The patient was observed for 6 months without the development of symptoms suggestive of gallbladder disease and the calcification remained unchanged on the abdominal radiograph. Ultrasound of the gallbladder failed to detect the thin layer of calcification and no gallstones were observed. Normal gallbladder contraction was demonstrated by serial measurements of gallbladder size by ultrasound after intravenous injection of a synthetic cholecystokinin component (Kinevac, 0.2 rg/kg; E.R. Squibb and Sons, Inc, Princeton, NJ). Computed tomography (CT) of the abdomen showed calcification of the medial wall of the gallbladder (Fig 2). An elective cholecystectomy was performed; intraoperative cholan-

From the Departments of Pediatrics, Radiology, and Surgery, University of Arkansas for Medical Sciences and Arkansas Children’s Hospital, Little Rock, AR. Address reprint requests to Helen Butler Casteel, MD. Division of Pediatric Gastroenterology, Arkansas ChildrenS Hospital. 800 Marshall, Little Rock, AR 72202. o 1990 by W.B. Saunders Company. 0022-3468/90/2512-0036$03.00/0 1302

E. Stevers Golladay,

Rock,

and Stephen

C. Fiedorek

Arkansas

giography was normal. The child had an uneventful postoperative recovery. Changes of cholecystitis were present in the gallbladder. There was a brittle, bluish area on one wall and no gallstones or tumor tissue were found. DISCUSSION

PGB is a rare form of chronic gallbladder disease in adults. The etiology of the disease is unclear, but is likely related to chronic inflammation. Gallstones are present in most patients with PGB and act as a chronic irritant, or obstruct the cystic duct and produce hydrops of the gallbladder.* Other possibilities are that the calcification may be subsequent to intramural hemorrhage from chronic inflammation or due to an imbalance in calcium metabolism.3y4 Pathologically, deposition of calcium occurs in the muscular layer of the gallbladder and forms a rim of calcification on or in multiple scattered areas in the glandular spaces of the mucosa.’ Most patients are asymptomatic, but biliary signs and symptoms such as jaundice, weight loss, abdominal pain, vomiting, or the presence of an epigastric or right upper quadrant mass have been reported.‘*‘-’ The disease is five times more common in women than men.3 Patients’ ages range from 38 to 84 years, with a mean age at diagnosis of 54 years.3v7This patient is the first pediatric patient to be reported in the literature. The diagnosis is typically made, as in this patient, by detection of the calcification on an abdominal radiograph. Ultrasonography or CT are useful for localization of the calcification within the wall of the gallbladder. Small calcified stones may appear similar on CT scan to a PGB due to dependent layering and adherence of the stones to the gallbladder wall.’ Most calcified gallbladders are dysfunctional, with nonvisualization on oral cholecystography. There is no previous report in the literature of assessment of gallbladder function in PGB by ultrasound and Kinevac stimulation.8 PGB has clinical importance because of the disproportionately high frequency of associated biliary carcinoma. Etala4 found 16 (20.5%) patients with PGB in 78 cases of carcinoma of the gallbladder. Twenty-six (1.5%) patients from the total series of 1,786 cholecystectomies had PGB; therefore, 61.5% of the patients with PGB had carcinoma.4 Other investigators have found lower but significant incidences. Cornell and Clarke3 found 16 (0.4%) calcified gallbladders in 4,27 1 cholecystectomies performed over a 45-year period. Two (12.5%) of these contained carcinoma. In most series, the carcinoma is usually well advanced and the

Journal of Pediatric Surgery, Vol 25, No 12 (December), 1990: pp 1302-l

303

PORCELAIN GALLBLADDER IN A CHILD

Fig 2. Abdominal the gallbladder.

Calcification Fig 1. intravenous urography.

in the right upper abdomen

noted during

prognosis is poor. In Etala’s patients, the 5-year survival rate was 3’S4 The association of PGB and biliary cancer has not been explained. Gallbladder disease is unusual in infancy and childhood. Predisposing factors for the development of chronic cholecystitis or cholelithiasis include anatomic abnormalities of the biliary tract, hemolytic disease, or diseases that affect the composition of bile. Gallstones have also been found in infants treated with total parenteral nutrition, particularly with the concomitant use of furosemide. Genetic factors and obesity may play a causal role.’ Cholecystitis in children is usually associated with

CT scan showing calcification

in the wall of

an underlying disease. However, the etiology of the present patient’s cholecystitis remains obscure, because she had no condition predisposing her to the development of gallbladder disease. Furthermore, this patient’s clinical findings differed from the reported adult cases of PGB. Her gallbladder showed signs of cholecystitis but no evidence of gallstones, although a small stone could have previously passed. Also, her gallbladder retained function and was able to contract on stimulation with Kinevac. It is possible that the PGB was detected at an early stage. Additional calcification could have occurred with cholecystitis of longer duration, and resulted in loss of gallbladder function. Although the natural history of PGB in a child is unknown, the high incidence of primary biliary cancer associated with PGB in adults suggests that cholecystectomy should be performed on diagnosis regardless of the patient’s symtomatology or age.

REFERENCES 1. Polk HC: Carcinoma terology 50:582-585, 1966

and the calcified

gallbladder.

Gastroen-

2. Phemister DB, Rewbridge AG, Rudisill H Jr: Calcium carbonate gallstones and calcification of the gallbladder following cystic duct obstruction. Ann Surg 94:493-516, 193 1 3. Cornell CM, Clarke R: Vicarious calcification gallbladder. Ann Surg 149:267-272, 1959

involving

4. Etala E: Cancer 49:2283-2299, 1962

Med Argent

de la vesicula

5. Berk RN: Radiology

biliar.

of the gallbladder,

Prensa

the

in Schiff L, Schiff ER

(eds): Diseases of the Liver (ed 5). Philadelphia, PA, Lippincott, 1982, pp 1398-1399 6. Berk RN, Arbuster TG, Saltzstein SL: Carcinoma in the porcelain gallbladder. Radiology 106:29-3 1, 1973 7. Kane RA, Jacobs R, Katz J, et al: Porcelain gallbladder: Ultrasound and CT appearance. Radiology 152:137-141, 1984 8. Davis GB, Berk RN, Scheible FW, et al: Cholecystokinin, cholecystography, sonography and scintigraphy: Detection of chronic acalculous cholecystitis. AJR 139:1117-l 121, 1982 9. Silverman A, Roy CC: Pediatric Clinical Gastroenterology (ed 3). St Louis, MO, Mosby, 1983, pp 800-801

Porcelain gallbladder in a child: a case report and review.

Calcification of the gallbladder wall (porcelain gallbladder; PGB) is a rare form of gallbladder disease not previously described in a child. A 10-yea...
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