0022-5347 /92/14 73-0681$03.00/0
Vol. 147, 681-682, March 1992 Printed in U.S. A.
THE JOURNAL OF UROLOGY
Copyright© 1992 by AMERICAN UROLOGICAL ASSOCIATION, INC.
TOXIC SHOCK SYNDROME: A COMPLICATION OF CONTINENT URINARY DIVERSION PADRAIC D. McCAHILL, DONNA I. WHITTLE AND STEPHEN C. JACOBS From the Division of Urology, Department of Surgery, and Division of Infectious Diseases, Department of Medicine, University of Maryland, School of Medicine, Baltimore, Maryland
ABSTRACT
Toxic shock syndrome, a potentially lethal multisystem illness that usually affects menstruating women, is characterized by the acute onset of fever, hypotension, skin and mucous membrane changes, nausea, vomiting, diarrhea, myalgias, capillary leak, vascular collapse and multiorgan dysfunction. The disease is mediated by toxin produced by distinct strains of Staphylococcus aureus. We describe a case in which a toxin producing strain growing in a continent urinary diversion produced toxic shock syndrome. KEY WORDS:
Staphylococcus aureus; shock, septic; urinary diversion
Late complications of continent urinary diversion chiefly comprise incontinence, difficulty of efferent limb catheterization, pyelonephritis, reflux, hydronephrosis and stone formation.1 We describe a new potential complication, toxic shock syndrome, which was first observed in 1978 and has been noted mainly in women using tampons. In most cases the syndrome is caused by a Staphylococcus aureus toxin called toxic shock syndrome toxin-1. 2 A relatively minor infection can lead to toxemia with devastating effects. The toxin appears to mediate damage in almost every organ through a direct effect on host cells and by induction of endogenous mediators. 3 Approximately 12% of reported cases of toxic shock syndrome have been nonmenstrual-related, many of which were apparently due to alternate toxins, such as enterotoxin. 4 Sites of infection in these cases have included various surgical and nonsurgical wounds, empyema and osteomyelitis. A review of the literature discloses only 1 case of urinary tract infection causing toxic shock syndrome, which involved pyonephrosis of an upper segment of a duplicated system in an infant. 5 We describe a case of toxic shock syndrome with S. aureus infection in a continent diversion. CASE REPORT
An acutely ill 18-year-old white woman was transferred to our hospital with a history of bladder exstrophy that had been managed with an ilea! loop since infancy. The loop had been removed 6 months previously because of stricture, and an Indiana right colon pouch was created. Convalescence was uneventful, the patient was continent and serum creatinine was 1.0 mg./dl. Although she had been compliant with the catheterization schedule at home, 2 weeks before presentation she had moved to college and reportedly may have performed catheterization less often in that setting. The patient was seen initially for a sore throat, headache, myalgias, nausea, vomiting and fever. Physical examination revealed a hyperemic, erythematous pharynx, and she was treated with acetaminophen and prochlorperazine for a presumed viral infection. The next day diarrhea, increased fever, and pronounced weakness and fatigue developed. That night the paramedics found the patient unconscious with a systolic blood pressure of 45 mm. Hg, pulse 140 and temperature 104.7F. Shortly after arrival to a local hospital she was intubated for respiratory distress. Rapid intravenous fluid resuscitation and dopamine improved the blood pressure. A diffuse sunburn-like rash and bilateral conjunctival hemorrhage were noted. A serous vaginal discharge was present but there was no evidence of
menstruation and no tampon. Shortly after intubation right hemiplegia developed. A catheter was placed in the pouch and 600 cc purulent urine were obtained. Laboratory values included white blood count 41,900/ml. 3 (normal 4,300 to 10,800), hematocrit 35.7% (normal 39.0 to 44.0), platelets 240,000 (normal 140,000 to 340,000), sodium 119 mEq./1. (normal 136 to 145), potassium 5. 7 mEq./1. (normal 3.5 to 5.0), chloride 89 mEq./1. (normal 100 to 106), total carbon dioxide 10 mEq./1. (normal 24 to 30), blood urea nitrogen 47 mg./dl. (normal 10 to 20), creatinine 4.8 mg./dl. (normal 0. 7 to 1.5), calcium 6.6 mg./dl. (normal 9.1 to 10.6), albumin 2.3 gm./ dl. (normal 3.5 to 5.0), serum transaminase 114 IU/1. (normal 50 or less) and serum lactate dehydrogenase 1,810 units/1. (normal 297 to 537). The patient was transferred to our hospital, and cranial computerized tomography (CT) demonstrated brain edema and suggested a middle cerebral artery infarct. Abdominal CT showed mild bilateral hydronephrosis and a small amount of fluid posterior to the pouch, which could not be aspirated. A diethylenetriaminepentaacetic acid renal scan demonstrated decreased function but no evidence of obstruction, and a roentgenogram of the pouch showed no extravasation and no ureter al reflux. A urine culture was positive for S. aureus, blood cultures were negative and vaginal cultures yielded normal flora. The patient was treated initially with ceftriaxone, gentamicin, nafcillin and metronidazole. As would be expected with toxic shock syndrome, large amounts of intravenous fluid and dopamine were required to maintain the blood pressure despite antistaphylococcal antibiotics. The patient became systemically edematous and adult respiratory distress syndrome developed. Severe brain edema secondary to a middle cerebral infarct and capillary leakage led to uncal herniation, which was treated with mechanical hyperventilation. Ten days later diffuse fine desquamation of the skin developed, with gross peeling of the skin on the feet and hands. The condition slowly improved during the next 3 weeks, and creatinine and liver function tests returned to normal. The patient was extubated and she was transferred to a rehabilitation hospital. She remains permanently disabled, with right hemiplegia and aphasia. A cerebral angiogram 3 weeks after the acute event showed evidence of a left middle cerebral artery infarction. Radial immunodiffusion of the S. aureus from the urine revealed it to be a toxic shock syndrome toxin-1 producer. 6 Assays for enterotoxins A, B, C and D were negative. DISCUSSION
Toxic shock syndrome is potentially lethal, and requires prompt diagnosis and intensive integrated therapy. It is similar
Accepted for publication June 21, 1991.
681
682
MCCAHILL, WHITTLE AND JACOBS
to botulism in that local production of a toxin produces severe systemic effects. Blood cultures are usually negative and the S. aureus infection will be present in the vagina or in other localized sites. 4 Diagnosis of toxic shock syndrome is based on meeting established criteria set forth by the Centers for Disease Control. Our patient had classic symptoms and signs, that is fever, hypotension, rash, myalgia, weakness, confusion, vomiting, diarrhea, renal insufficiency and hepatic dysfunction. Classic skin changes include erythroderma, a sunburn-like rash that appears in the early hours of toxic shock syndrome, and desquamation of the palms and soles 7 to 12 days later. Erythematous mucous membranes, mouth ulcers and petechiae are also noted, and in severe cases liver and renal dysfunction may be present. Adult respiratory distress syndrome, cardiac dysfunction and disseminated intravascular coagulation may also occur. A cereoraTinfarctis not typical in toxic shock s:yndronie, although 3 cases of fatal cerebral edema have been reported. 7 An angiogram in our patient ruled out any predisposing lesions. Fortunately, toxic shock syndrome is not likely to become a common problem in patients with urinary diversion. Only about 10 to 15% of healthy subjects carry S. aureus either in the nose, throat or vagina, and only 10% of these strains are capable of toxic shock syndrome toxin-1 production. 8• 9 In the intact urinary tract S. aureus is less common, accounting for only 3.3% of positive urine cultures, of which only 8.3% lead to bacteremia.10 Although urine culture from intestinal loop urinary diversions is frequently positive, only 2.3% of these cultures grow S. aureus. 11 To date, S. aureus has not been a significant uropathogen in patients with continent urinary diversions. 12 Since intact bladder mucosa is relatively impermeable, it is not surprising that there have been no reported cases of toxic shock syndrome from cystitis. Conversely, bowel mucosa exposed to urine can absorb electrolytes, 13 oligopeptides and small molecules, such as methotrexate, particularly if there is urinary stasis. 14 Inflamed bowel is apparently permeable to toxic shock syndrome toxin-1, which is a 193-amino acid peptide, 15 since toxic shock syndrome has been associated with S. aureus enterocolitis.16 Infrequent catheterization of a continent urinary diversion will lead to bacterial overgrowth, high pressure, inflamed bowel mucosa and possible leakage. If a toxin producing bacteria is present, then toxic shock syndrome can rapidly develop. Urologists have encountered toxic shock syndrome from infected surgical wounds following ureterolithotomy, orchiectomy and vesicourethral suspension. 17 Clinicians treating patients with urinary diversion need to be aware of this new setting for a life-threatening complication. Prompt, aggressive and integrated therapy can be life-saving. Patient education should stress the importance of the regularity of the catheterization regimen. Dr. Jay A. Jacobson, LDS Hospital, Salt Lake City, Utah, performed the toxin analysis.
REFERENCES
1. Lieskovsky, G., Boyd, S. D. and Skinner, D. G.: Management of late complications of the Kock pouch form of urinary diversion. J. Urol., 137: 1146, 1987. 2. Garbe, P. L., Arko, R. J., Reingold, A. L., Graves, L. M., Hayes, P. S., Hightower, A. W., Chandler, F. W. and Broome, C. V.: Staphylococcus aureus isolates from patients with nonmenstrual toxic shock syndrome. Evidence for additional toxins. J.A.M.A., 253: 2538, 1985. 3. Parsonnet, J.: Mediators in the pathogenesis of toxic shock syndrome: overview. Rev. Infect. Dis., suppl. 1, 11: S263, 1989. 4. Reingold, A. L., Hargrett, N. T., Dan, B. B., Shands, K. N., Strickland, B. Y. and Broome, C. V.: Nonmenstrual toxic shock syndrome: a review of 130 cases. Ann. Intern. Med., 96: 871, 1982. 5. Atp-ert, G., eampos, .J. M. and- Snyder; H. M.;-IH: -Pyonephrosis presenting as toxic shock syndrome in infancy. Ped. Infect. Dis. J., 4: 561, 1985. 6. Ritz, H. L., Kirkland, J. J., Bond, G. G., Warner, E. K. and Petty, G. P.: Association of high levels of serum antibody to staphylococcal toxic shock antigen-producing strains of Staphylococcus aureus. Infect. lmmun., 43: 954, 1984. 7. Smith, D. B. and Gulinson, J.: Fatal cerebral edema complicating toxic shock syndrome. Neurosurgery, 22: 598, 1988. 8. Tuazon, C. U. and Sheagren, J. N.: Increased rate of Staphylococcus aureus among narcotic addicts. J. Infect. Dis., 129: 725, 1974. 9. Linnemann, C. C., Jr., Staneck, J. L., Hornstein, S., Barden, T. P., Rauh, J. L., Bonventre, P. F., Buncher, C. R. and Beiting, A.: The epidemiology of genital colonization with Staphylococcus aureus. Ann. Intern. Med., 96: 940, 1982. 10. Arpi, M. and Renneberg, J.: The clinical significance of Staphylococcus aureus bacteriuria. J. Urol., 132: 697, 1984. 11. Needham, R. N., Smith, M. M. and Matsen, J.M.: Differences in the bacteriology of intestinal loop urinary diversions. J. Urol., 104: 831, 1970. 12. Mansson, W., Colleen, S. and Mardh, P.-A.: The microbial flora of the continent cecal urinary reservoir, its stoma and the peristomal skin. J. Urol., 135: 247, 1986. 13. Kosko, J. W., Kursh, E. D. and Resnick, M. I.: Metabolic complications of urologic intestinal substitutes. Urol. Clin. N. Amer., 13: 193, 1986. 14. Fossa, S. D., Heilo, A. and B0rmer, 0.: Unexpectedly high serum methotrexate levels in cystectomized bladder cancer patients with an ileal conduit treated with intermediate doses of the drug. J. Urol., 143: 498, 1990. 15. Blomster-Hautamaa, D. A., Novick, R. P. and Schlievert, P. M.: Localization of biologic functions of toxic shock syndrome toxin1 by use of monoclonal antibodies and cyanogen bromide-generated toxin fragments. J. lmmunol., 137: 3572, 1986. 16. Gruber, W. C. and Pietsch, J. B.: Toxic shock syndrome associated with Staphylococcus aureus enterocolitis. Ped. Infect. Dis. J., 7: 71, 1988. 17. Petitti, D., D'Agostino, R. B. and Oldman, M. J.: Nonmenstrual toxic shock syndrome. Methodologic problems in estimating incidence and delineating risk factors. J. Reprod. Med., 32: 10, 1987.
Vol. 147, 681-682, March 1992 Printed in U.S. A.
THE JOURNAL OF UROLOGY
Copyright© 1992 by AMERICAN UROLOGICAL ASSOCIATION, INC.
TOXIC SHOCK SYNDROME: A COMPLICATION OF CONTINENT URINARY DIVERSION PADRAIC D. McCAHILL, DONNA I. WHITTLE AND STEPHEN C. JACOBS From the Division of Urology, Department of Surgery, and Division of Infectious Diseases, Department of Medicine, University of Maryland, School of Medicine, Baltimore, Maryland
ABSTRACT
Toxic shock syndrome, a potentially lethal multisystem illness that usually affects menstruating women, is characterized by the acute onset of fever, hypotension, skin and mucous membrane changes, nausea, vomiting, diarrhea, myalgias, capillary leak, vascular collapse and multiorgan dysfunction. The disease is mediated by toxin produced by distinct strains of Staphylococcus aureus. We describe a case in which a toxin producing strain growing in a continent urinary diversion produced toxic shock syndrome. KEY WORDS:
Staphylococcus aureus; shock, septic; urinary diversion
Late complications of continent urinary diversion chiefly comprise incontinence, difficulty of efferent limb catheterization, pyelonephritis, reflux, hydronephrosis and stone formation.1 We describe a new potential complication, toxic shock syndrome, which was first observed in 1978 and has been noted mainly in women using tampons. In most cases the syndrome is caused by a Staphylococcus aureus toxin called toxic shock syndrome toxin-1. 2 A relatively minor infection can lead to toxemia with devastating effects. The toxin appears to mediate damage in almost every organ through a direct effect on host cells and by induction of endogenous mediators. 3 Approximately 12% of reported cases of toxic shock syndrome have been nonmenstrual-related, many of which were apparently due to alternate toxins, such as enterotoxin. 4 Sites of infection in these cases have included various surgical and nonsurgical wounds, empyema and osteomyelitis. A review of the literature discloses only 1 case of urinary tract infection causing toxic shock syndrome, which involved pyonephrosis of an upper segment of a duplicated system in an infant. 5 We describe a case of toxic shock syndrome with S. aureus infection in a continent diversion. CASE REPORT
An acutely ill 18-year-old white woman was transferred to our hospital with a history of bladder exstrophy that had been managed with an ilea! loop since infancy. The loop had been removed 6 months previously because of stricture, and an Indiana right colon pouch was created. Convalescence was uneventful, the patient was continent and serum creatinine was 1.0 mg./dl. Although she had been compliant with the catheterization schedule at home, 2 weeks before presentation she had moved to college and reportedly may have performed catheterization less often in that setting. The patient was seen initially for a sore throat, headache, myalgias, nausea, vomiting and fever. Physical examination revealed a hyperemic, erythematous pharynx, and she was treated with acetaminophen and prochlorperazine for a presumed viral infection. The next day diarrhea, increased fever, and pronounced weakness and fatigue developed. That night the paramedics found the patient unconscious with a systolic blood pressure of 45 mm. Hg, pulse 140 and temperature 104.7F. Shortly after arrival to a local hospital she was intubated for respiratory distress. Rapid intravenous fluid resuscitation and dopamine improved the blood pressure. A diffuse sunburn-like rash and bilateral conjunctival hemorrhage were noted. A serous vaginal discharge was present but there was no evidence of
menstruation and no tampon. Shortly after intubation right hemiplegia developed. A catheter was placed in the pouch and 600 cc purulent urine were obtained. Laboratory values included white blood count 41,900/ml. 3 (normal 4,300 to 10,800), hematocrit 35.7% (normal 39.0 to 44.0), platelets 240,000 (normal 140,000 to 340,000), sodium 119 mEq./1. (normal 136 to 145), potassium 5. 7 mEq./1. (normal 3.5 to 5.0), chloride 89 mEq./1. (normal 100 to 106), total carbon dioxide 10 mEq./1. (normal 24 to 30), blood urea nitrogen 47 mg./dl. (normal 10 to 20), creatinine 4.8 mg./dl. (normal 0. 7 to 1.5), calcium 6.6 mg./dl. (normal 9.1 to 10.6), albumin 2.3 gm./ dl. (normal 3.5 to 5.0), serum transaminase 114 IU/1. (normal 50 or less) and serum lactate dehydrogenase 1,810 units/1. (normal 297 to 537). The patient was transferred to our hospital, and cranial computerized tomography (CT) demonstrated brain edema and suggested a middle cerebral artery infarct. Abdominal CT showed mild bilateral hydronephrosis and a small amount of fluid posterior to the pouch, which could not be aspirated. A diethylenetriaminepentaacetic acid renal scan demonstrated decreased function but no evidence of obstruction, and a roentgenogram of the pouch showed no extravasation and no ureter al reflux. A urine culture was positive for S. aureus, blood cultures were negative and vaginal cultures yielded normal flora. The patient was treated initially with ceftriaxone, gentamicin, nafcillin and metronidazole. As would be expected with toxic shock syndrome, large amounts of intravenous fluid and dopamine were required to maintain the blood pressure despite antistaphylococcal antibiotics. The patient became systemically edematous and adult respiratory distress syndrome developed. Severe brain edema secondary to a middle cerebral infarct and capillary leakage led to uncal herniation, which was treated with mechanical hyperventilation. Ten days later diffuse fine desquamation of the skin developed, with gross peeling of the skin on the feet and hands. The condition slowly improved during the next 3 weeks, and creatinine and liver function tests returned to normal. The patient was extubated and she was transferred to a rehabilitation hospital. She remains permanently disabled, with right hemiplegia and aphasia. A cerebral angiogram 3 weeks after the acute event showed evidence of a left middle cerebral artery infarction. Radial immunodiffusion of the S. aureus from the urine revealed it to be a toxic shock syndrome toxin-1 producer. 6 Assays for enterotoxins A, B, C and D were negative. DISCUSSION
Toxic shock syndrome is potentially lethal, and requires prompt diagnosis and intensive integrated therapy. It is similar
Accepted for publication June 21, 1991.
681
682
MCCAHILL, WHITTLE AND JACOBS
to botulism in that local production of a toxin produces severe systemic effects. Blood cultures are usually negative and the S. aureus infection will be present in the vagina or in other localized sites. 4 Diagnosis of toxic shock syndrome is based on meeting established criteria set forth by the Centers for Disease Control. Our patient had classic symptoms and signs, that is fever, hypotension, rash, myalgia, weakness, confusion, vomiting, diarrhea, renal insufficiency and hepatic dysfunction. Classic skin changes include erythroderma, a sunburn-like rash that appears in the early hours of toxic shock syndrome, and desquamation of the palms and soles 7 to 12 days later. Erythematous mucous membranes, mouth ulcers and petechiae are also noted, and in severe cases liver and renal dysfunction may be present. Adult respiratory distress syndrome, cardiac dysfunction and disseminated intravascular coagulation may also occur. A cereoraTinfarctis not typical in toxic shock s:yndronie, although 3 cases of fatal cerebral edema have been reported. 7 An angiogram in our patient ruled out any predisposing lesions. Fortunately, toxic shock syndrome is not likely to become a common problem in patients with urinary diversion. Only about 10 to 15% of healthy subjects carry S. aureus either in the nose, throat or vagina, and only 10% of these strains are capable of toxic shock syndrome toxin-1 production. 8• 9 In the intact urinary tract S. aureus is less common, accounting for only 3.3% of positive urine cultures, of which only 8.3% lead to bacteremia.10 Although urine culture from intestinal loop urinary diversions is frequently positive, only 2.3% of these cultures grow S. aureus. 11 To date, S. aureus has not been a significant uropathogen in patients with continent urinary diversions. 12 Since intact bladder mucosa is relatively impermeable, it is not surprising that there have been no reported cases of toxic shock syndrome from cystitis. Conversely, bowel mucosa exposed to urine can absorb electrolytes, 13 oligopeptides and small molecules, such as methotrexate, particularly if there is urinary stasis. 14 Inflamed bowel is apparently permeable to toxic shock syndrome toxin-1, which is a 193-amino acid peptide, 15 since toxic shock syndrome has been associated with S. aureus enterocolitis.16 Infrequent catheterization of a continent urinary diversion will lead to bacterial overgrowth, high pressure, inflamed bowel mucosa and possible leakage. If a toxin producing bacteria is present, then toxic shock syndrome can rapidly develop. Urologists have encountered toxic shock syndrome from infected surgical wounds following ureterolithotomy, orchiectomy and vesicourethral suspension. 17 Clinicians treating patients with urinary diversion need to be aware of this new setting for a life-threatening complication. Prompt, aggressive and integrated therapy can be life-saving. Patient education should stress the importance of the regularity of the catheterization regimen. Dr. Jay A. Jacobson, LDS Hospital, Salt Lake City, Utah, performed the toxin analysis.
REFERENCES
1. Lieskovsky, G., Boyd, S. D. and Skinner, D. G.: Management of late complications of the Kock pouch form of urinary diversion. J. Urol., 137: 1146, 1987. 2. Garbe, P. L., Arko, R. J., Reingold, A. L., Graves, L. M., Hayes, P. S., Hightower, A. W., Chandler, F. W. and Broome, C. V.: Staphylococcus aureus isolates from patients with nonmenstrual toxic shock syndrome. Evidence for additional toxins. J.A.M.A., 253: 2538, 1985. 3. Parsonnet, J.: Mediators in the pathogenesis of toxic shock syndrome: overview. Rev. Infect. Dis., suppl. 1, 11: S263, 1989. 4. Reingold, A. L., Hargrett, N. T., Dan, B. B., Shands, K. N., Strickland, B. Y. and Broome, C. V.: Nonmenstrual toxic shock syndrome: a review of 130 cases. Ann. Intern. Med., 96: 871, 1982. 5. Atp-ert, G., eampos, .J. M. and- Snyder; H. M.;-IH: -Pyonephrosis presenting as toxic shock syndrome in infancy. Ped. Infect. Dis. J., 4: 561, 1985. 6. Ritz, H. L., Kirkland, J. J., Bond, G. G., Warner, E. K. and Petty, G. P.: Association of high levels of serum antibody to staphylococcal toxic shock antigen-producing strains of Staphylococcus aureus. Infect. lmmun., 43: 954, 1984. 7. Smith, D. B. and Gulinson, J.: Fatal cerebral edema complicating toxic shock syndrome. Neurosurgery, 22: 598, 1988. 8. Tuazon, C. U. and Sheagren, J. N.: Increased rate of Staphylococcus aureus among narcotic addicts. J. Infect. Dis., 129: 725, 1974. 9. Linnemann, C. C., Jr., Staneck, J. L., Hornstein, S., Barden, T. P., Rauh, J. L., Bonventre, P. F., Buncher, C. R. and Beiting, A.: The epidemiology of genital colonization with Staphylococcus aureus. Ann. Intern. Med., 96: 940, 1982. 10. Arpi, M. and Renneberg, J.: The clinical significance of Staphylococcus aureus bacteriuria. J. Urol., 132: 697, 1984. 11. Needham, R. N., Smith, M. M. and Matsen, J.M.: Differences in the bacteriology of intestinal loop urinary diversions. J. Urol., 104: 831, 1970. 12. Mansson, W., Colleen, S. and Mardh, P.-A.: The microbial flora of the continent cecal urinary reservoir, its stoma and the peristomal skin. J. Urol., 135: 247, 1986. 13. Kosko, J. W., Kursh, E. D. and Resnick, M. I.: Metabolic complications of urologic intestinal substitutes. Urol. Clin. N. Amer., 13: 193, 1986. 14. Fossa, S. D., Heilo, A. and B0rmer, 0.: Unexpectedly high serum methotrexate levels in cystectomized bladder cancer patients with an ileal conduit treated with intermediate doses of the drug. J. Urol., 143: 498, 1990. 15. Blomster-Hautamaa, D. A., Novick, R. P. and Schlievert, P. M.: Localization of biologic functions of toxic shock syndrome toxin1 by use of monoclonal antibodies and cyanogen bromide-generated toxin fragments. J. lmmunol., 137: 3572, 1986. 16. Gruber, W. C. and Pietsch, J. B.: Toxic shock syndrome associated with Staphylococcus aureus enterocolitis. Ped. Infect. Dis. J., 7: 71, 1988. 17. Petitti, D., D'Agostino, R. B. and Oldman, M. J.: Nonmenstrual toxic shock syndrome. Methodologic problems in estimating incidence and delineating risk factors. J. Reprod. Med., 32: 10, 1987.
