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Acalculous Cholecystitis And Malignant Ileal Carcinoid Tumor EZEKIEL 0. 0. ODELOWO, M.B., B.S., Chief Resident, SUBHI D. ALI, M.D., Instrllctor of Surgerv, THOMAS CALHOUN, M.D., FA.C.S., Assistant Professor ol Surgerv, and LEWIS H. KURTZ, M.D., EA.C.S., Associate Professor of Suirgerv and Chief Medical Officer, Howard University Division, District of Columbia General Hospital, Washington, D.C.

Acute and chronic inflammatory gallbladder disease, occurring without stones, has become a well documented, but poorly explained entity. This article illustrates the case of a 65-year-old male who presented with septic shock secondary to acalculous choIceystitis. He was treated successfully with cholecystostomy and subsequent cholecystectomy. An incidental finding was a malignant carcinoid of the ileum. Current literature on the subject is briefly presented. CASE HISTORY

On his second admission to the Howard University Surgical Service in D.C. General Hospital, Washington, D.C., A.Y., a 65-year-old retired construction worker presented in the Emergency Room with a three day history of nausea and five hours of generalized colicky abdominal pain. The pain followed consumption of a,pint of whiskey and became progressively worse. Vomiting occurred the following morning, as did constipation. Two similar episodes two days earlier had been relieved by laxatives. Past history revealed a urethreal stricture for which he had been dilated 2'/2 years ago. His mother died of carcinoma of the stomach as did a nephew of the same malignancy. PHYSICAL EXAMINATION

Temperature, 100°F; BP, 130/90; Pulse, 90; Respiration, 20. He was alert and well oriented but with acute abdominal distress on admission. The abdomen was moderately distended and diffusely tender to deep palpation, but without rebound. The liver was enlarged, but was firm and smooth. An easily reducible, small, left direct inguinal hernia was also detected. The remainder of the examination was essentially normal.

Admission impressions were partial intestinal obstruction, acute pancreatitis with paralytic ileus, urethreal stricture and direct left inguinal hernia. LABORATORY DATA Initial WBC count was 11,100/cu mm, RBC count 4,430,000/cc mm, Hgb. 14.3 gm and Hct. 43.1%. The urine was cloudy and amber colored with a specific gravity of 1.010, pH of 6.0, WBC of 12-36/HPF and 2 bacteria. Glucose, acetone, protein and occult blood were absent. Prothrombin time was 12.4 secs. for a control of 12 secs. PTT was 21.5 secs. for a control of 28.9 secs. Serum calcium was 10.4 mg%, phosphate 2.0 mg%, total protein 7.6 gm., albumin 3.8 gm., alkaline phosphatase 350 KA units, acid phosphatase 0.9 Bodansky units, serum creatinine 1.4 mg'Yo and uric acid 6.6 mg'/o. The BUN was 30, blood glucose 165, CO2 20, Cl 99, Na 140, K 4.4, and serum amylase less than 180 units. Chest x-rays were within normal limits and upright and reclining abdominal films revealed only lumbar osteoarthritic changes.

HOSPITAL COURSE Initial management consisted of nasogastric tube decompression, IV fluids and thiamine. On the second hospital day, decreased bowel sounds were noted. His temperature ranged from 102-104°. Intravenous ampicillin was started. The next day he became agitated and had increased abdominal tenderness. The total bilirubin was 3.0 and direct bilirubin 0.4. A C.V.P catheter was inserted and acidosis was corrected. Antibiotic was changed to keflin. On his third hospital day a suprapubic catheter was inserted, and an intravenous cholangiogram showed no visualization of the biliary tree. Temperature ranged between 102 and 104° and the patient became hypotensive and stuporous with absent to weak peripheral pulses. There was marked guarding in the R.U.Q. associated with rebound tenderness. Emergency cholecystostomy was done through a right Kocher incision under N20/02 and Inovar anesthesia

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after awake intubation. Purulent bile from a markedly intlamed, distended gallbladder was aspirated and cultured. Subsequently, yellowish-green bile egressed from the cystic duct but no stones were visible or palpable in the gallbladder or extrahepatic biliary tract. Prior to terminaition of the procedure, umbilicated nodules were seen in the small bowel mesentery close to the site of incomplete walling off of the inflammatory process by the omentum. These were not fully evaluated, however, because of his tenuous condition. The post operative course was marked by prolonged azotemia and hyperchloremic acidosis. Urine cultures were repeatedly negative, but both the gailbiladder "bile" and blood specimens grew E. coli, sensitive to all appropriate antibiotics.

Fig. 1. Carcinoid ileum-high power lOX.

Postoperative upper gastric and smaill bowel series were normal, as was IVP A barium colon enema was also normal and a cholangiogram done through the cholecystostomy tube revealed a patent and normal biliary tree. Ten weeks after cholecystostomy and six weeks after the cholecystostomy tube was removed, he was reexplored, with subsequent cholecystectomy and wide resection of the mid-ileum with its contiguous mesentery and nodes. Histologically this area proved to be a malignant carcinoid with nodal metastases1 (Fig. 1.) No stones were found. His postoperative course was marked by "high output" renal failure and pre-renal azotemia which were corrected with intensive management. He was discharged three months after hospitalization with followup by the surgical and urological divisions. No obstruction was associated with the carcinoid tumor, thus, we do not attribute any of the patient's symptoms to it. He did not manifest the carcinoid syndrome. DISCUSSION

Usually the absence of gallstones and visualization of the gallbladder via a cholecystogram

MAY, 1975

excludes acute or chronic cholecystitis. There are those instances, however, where inflammation of the gallbladder occurs without associated stones in any part of the biliary tree i.e. acalculous or non-calculous cholecystitis. The incidence of acalculous cholecystitis varies according to age, race and geographical location2. Estimates in reported series range between 2.2 and 47% of all cases of cholecystitis3, and as high as 32% in children4. The high incidence of 12 to 47% is quoted from European literature where no clear operative documentation is available in all cases5. Parnis reported an incidence of 35% among Nigerians with gallbladder disease6,. Among population groups in the U.S., including American Indians who have a high incidence of gallbladder disease,' a 5-10% incidence is generally accepted. The condition is reported more frequently in children than in adults7-9. The pathogenesis of non-calculous cholecystitis is not always well defined. Many associated pre-disposing or precipitating conditions have been reported5 " as causative. Trauma has also been implicated"'6, as well as prolonged parental alimentation' and sickle cell disease'. In children and adolescents, acalculous cholecystitis is usually a complication of anatomical anomalies of the cystic duct, cystic artery or mesentery of the gallbladder, whereas, in the adult, E. coli and clostridial infections predominate5. Theories about pathogenesis include "biliary dyskenesia"' 8, overwork syndrome called "hyperplastic cholecystosis"3 '819, toxicity of concentrated bile to the gallbladder mucosa20 and bile stasis from various causes21,22. Unfortunately, not all cases reported in the literature have operative or autopsy documentation and some cases of cholesterolosis have been regarded, we believe erroneously, as acalculous cholecystitis. The exact pathophysiology is unknown. Symptoms and signs are often identical with those of calculous cholecystitis. The noncalculous variety is, however, commoner in children than adults and does not exhibit a predilection for females7-9. A high index of suspicion is a prerequisite to making a diagnosis. In spite of this, it is

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sometimes unusual to make a correct preoperative diagnosis9. Where indicated an attempt should be made to exclude associated conditions5 through the following steps:

LITERATURE CITED

1. WILSON, H. and R.C. CHEC, T SHERMAN, and E.H. STORER. Carcinoid Tumors. Current Problems in Surgery, Nov. 1970. 2. GLENN, F and H{. MANNIX. The Acalculous 1.Intraoperative evalultionl of the upper gastrointestinal Gallbladder, Ann. Surg., 144:670, 1956. 3. JACOBS, L.A. et al. Hyperplastic Cholecystosis. traict and pancreas. Arch. Surg., 104:193, 1972. 2. Liver biopsy in jaundiced patients without galilstones. 4. HOPKINS, J.W. et al. Acute Noncalculous 3. Catretul alssessment of the commoni bile duct (including Cholecystitis in a Three Year Old Child. Clin. Pediat., operative cholangiography) and slphincter of Oddi. 1:105, 1962. 4. Culture of gallbl.adder w.;ll aind bile. 5. MUNSTER, A.M. aind J.R. BROWN. Acalculous 5. Serial stool cultures aind agglutination tests for SatlmCholecystitis. Amer. Jour. Surg., 113:730-734, 1967. onella. 6. PARNIS, R.O. Gallbladder Disease in Nigeria. Tr. 6. Seriail serum amylase, gluCose tolerance tests aind L.E. Roy. Soc. Trop. Med. aind Hyg., 58:437, 1964. 7. ANSPIGER, L.A. et al. Acute Noncalculous tests. Cholecystitis in Children. Amer. Jour. Surg., 7. Postoperative surveillance to ascertain cure. 100:103-106, 1960. The main controversy surrounding 8. CRICHLOW, R.W. et ail. Cholecystitis in Adolescents. Amer. Jour. Digestive Dis., 17, 68-72. acalculous cholecystitis has been the unGRAIVIER, L. et ail. Gallbladder Diseaise in Int'ants satisfactory results of cholecystectomy for a 9. aind Children. Surgery, 63:690-696, 1968. syndrome suggesting gallbladder disease 10. HtOERR, S.T. and TB. HAZARD. Acute Cholecystitis Without Gallbliadder Stones. Amer. Jour. Surg., without gallstones. For example, Mackey re111:47-55, 1966. ported such poor results in 36.2%. Only those II. GOODWIN, M.N. aind B.A. PRUITT, JR. Acalculous patients whose symptoms arise from Cholecystitis in Burned Patients. Amer. Jour. Surg., 122:591-593, 1971. cholecystitis, whether calculous or acalculous, 12. HOWARD, R.J. and J.P DELANEY. Post Traumatic are apt to derive benefit from cholecystectomy. Cholecystitis., J.A.M.A., 218:1006-1007, 1971. And in spite of the usually low morbidity and 13. SCHWARTZ, S.E. Principles of Surgery, McGrawHill Book Company, 1969, p. 1096. mortality from this procedure, a "diagnostic SHAW, R.C. Post Traumatic Acute Acalculous cholecystectomy" is unacceptable to most 14. Cholecystitis in Young Miales, Milit. Med., surgeons. 135:210-214, 1970 Cholecystostomy may be life-saving until 15. THOMSON, J.W III et al. Acute Cholecystitis Complicating Operations for Other Diseases. Ann. the patient is fit for cholecystectomy or a more Surg., 155:489-494, 1962. formidable procedure where indicated. The 16. WEEDER, R.S. et al. Acute Noncalculous Cholecystitis Associated with Severe Injury. Amer. cholecystostomy tube provides a ready route Jour. Surg., 119:729-732, 1970. for postoperative cholangiography after control 17. ANDERSON, D.L. Acalculous Cholecystitis-A Posof infection. sible Complication of' Parenteral Hlyperalimentation. Med. Ann., District of Columbia, 41:448-450, 1972. 18. CRONIN, K. and M.D. MIDDLETON, Symptoms of SUMMARY Gallbladder Diseaise With a Negative Cholecystogram. Lancet, 1:1392-3, l96,. A case of a 65-year-old male with endotoxic J.A. Hyperplastic Cholecystoses. Amer. J. shock from acalculous cholecystitis and inci- 19. JUTRAS, Roentgen., 83:795-827, 1960. dental malignant carcinoid of the ileum has 20. Case Records of the Massachusetts General Hospital Weekly. Clinico-pathological Exercises, Case 37-1973. been presented. We have outlined the successNew Eng. Jour. Med., 289, 578-585, 1973. ful management of this patient with 21. BLATT, J.M. and Y. FLOMAN. Post Traiumaitic Acute cholecystostomy followed, after 10 weeks, Acalculous Cholecystitis. Int. Surg. (Jerusalem), 57:982-3, 1972. with cholecystectomy and resection of a malig22. GLENN, F and G. WANTZ. Acute Cholecystitis nant small bowel carcinoid tumor. Following the Surgical Treatment of' Unrelated Disease. Surv. Gvnecol. Obstet., 102:145-153. 1956. (Henry et al., from page 222) ment 2, Mai, 1973. Normal and Pathological Pituitary. Clin. Neurosurg., 8. HARDY, J. Personal Communication. 16:185-217, 1969. 9. CIRIC, I.S. and J. TARKINGTON, Transsphenoidal 6. HARDY, J. Transsphenoidal Hypophysectomy. J. Microsurgery. Surgical Neurol., 2:207-211, 1974. Neurosurg., 34:581-594, 1971. 10. HARDY, J. and 1. CIRCI. Selective Anterior Hypo7. HARDY, J. and F ROBERT, M. SOMMA, and J.L. physectomy for Treatment of Diabetic Retinopathy: A VEZINA. Acromegalie-gigantisme-Traitement chiTranssphenoidal Microsurgical Technique. J. A.M.A., rurgical par exerese transsphenoidale de I'adenome 203:73-78, 1968. hypophysaire, Neuro-Chirurgie, Tome 19, supple-

Acalculous cholecystitis and malignant ileal carcinoid tumor.

205 Vol. 67, No. 3 Acalculous Cholecystitis And Malignant Ileal Carcinoid Tumor EZEKIEL 0. 0. ODELOWO, M.B., B.S., Chief Resident, SUBHI D. ALI, M.D...
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