780 DEMEDIUK ET AL.

9. Wiedemann B, Peter K. Induction of beta-lactamases in Gram- negative bacteria. Diagn. Microbiol. Infect. Dis. 1989; 12

10. Thomson KS. Weber DA, Sanders CC, Sanders WE. B- lactamase production in members of the family Enterobac- teriaceae and resistance to B-lactam-enzyme inhibitor combinations. Antimicrob. Agents Chemother. 1990; 34:

1 1. Korfmann G, Wiedemann B. Genetic control of beta-lactamase production in E. cloacae. Rev. Infect. Dis. 1988; 10: 793-7.

12. Wiedemann B. Selection of beta-lactamase producers during cephalosporin and penicillin therapy. Scand. J. Infect. Dis. 1986; 49 (Suppl.): 100.

13. Sanders CC. B-lactamases of Gram-negative bacteria: New chal- lenges for new drugs. Clin. Infect. Dis. 1992; 14: 1089-99.

14. Khan GA, Scott AJ. The place of rifamycin-B-diethylamide in the treatment of cholangitis complicating biliary obstruction. Br. J. Pharmacol. Chemother. 1967; 31: 506-12.

(SUPPI.): 131-7.

622-7.

15. Keighley MRB, Drysdale RB, Quoraishi AH, Burdon DW, Alexander-Williams J. Antibiotics in biliary disease: The rela- tive importance of antibiotic concentrations in the bile and serum. Gut 1976; 17: 495-500.

16. Mortimer PR, Mackie DB, Haynes S. Ampicillin levels in human bile in the presence of biliary tract disease. BMJ 1969;

17. McLeish AR, Strachan CJL, Powis SJA, Wise R, Bevan PG. The influence of biliary disease on the excretion of Cefazolin in human bile. Surgery 1977; 81: 426-30.

18. Koyama K, Takagi Y, Ito K, Sat0 T. Experimental and clinical studies on the effect of biliary drainage in obstructive jaundice. Am. J. Surg. 1981; 142: 293-9.

19. Holman JM, Rikkers LF. Biliary obstruction in host defense failure. J. Surg. Res. 1982; 32: 208-13.

20. Katz S, Grosfeld JL, Gross K et a f . Impaired bacterial clear- ance and trapping in obstructive jaundice. Ann. Surg. 1984;

3: 88-9.

199: 14-20.

Aust. N.Z. J. Surg. (1996) 66, 780-781

CASE REPORT

PROGRESSIVE ECTODERMAL CHANGES IN THE CRON KH ITE-CANADA SYNDROME

D. C. N. K. NYAM,* M. S . Hot AND H. S . GOH* *Department of Colorectal Surgery and tDepartment of Pathology, Singapore General Hospital,

Outram Road, Singapore

Key words: Cronkhite-Canada syndrome, ectoderm, polyps.

INTRODUCTION The Cronkhite-Canada syndrome is characterized by pan- gastrointestinal polyposis, alopecia, skin hyperpigmentation and onychodystrophia. It is a rare entity with less than 100 cases reported in the English literature. We present the case of a male patient initially presenting with mild diarrhoea, and highlight the progressive nature of his ectodermal changes which parallel the severity of his gastrointestinal symptoms and the course of his disease.

Both upper and lower gastrointestinal endoscopy revealed a polypoidal granular appearance of the mucosa. Multiple biop- sies were taken all along the gastrointestinal tract. These showed harmatomatous polyps with normal mucosa associated with oedema and inflammation in the lamina propria (Fig. 1). Blood investigations showed a haemoglobin level of 13.7 g/dL, serum potassium of 3.5 mmol/L, albumin of 29 g/L with a serum protein of 5 1 g/L. Serum zinc, selenium, magnesium and calcium were normal.

He was treated with a high-protein diet, protein supplements and supplementary enteral feeding. He was well for 3 months but subsequently deteriorated rapidly. He was admitted to hos- pital and started on enteral and parenteral hyperalimentation. CASE REPORT Anti-diarrhoea medications were prescribed to slow down the

This improved his stool frequency to three to four times a

alopecia and the pigmentation over the upper part of his body increased. His nails dropped off their beds. An implanted central venous port was used for prolonged parenteral nutrition.

His clinical status and biochemical parameters improved and

A 54-year-oId Chinese man WJ) presented with hypergeusia, bowel transit time and maximize his absorptive capabilities. diarrhoea and colicky abdominal pain associated with anorexia

increased to four or five times per day. This was associated with loss of hair, nail changes and hyperpigmentation over the upper limbs. There were no joint or chest pains. There was no family history of gastrointestinal polyps or carcinoma. He was thin but not cachexic.

and weight loss Over a 4-month period' His stools frequency day. At this tirne his ectodermal signs began to progress. His

he was discharged after about 2 weeks. He remained in fair

for further hyperalimentation. His hyperpigmentation was now

D. c. N. K. Nyam* Of Colorectal health for about another 8 weeks, after which he was readmitted Singapore General Hospital, Outram Road, Singapore 169608.

Accepted for publication 4 August 1994. generalized and the tone of his skin darkened considerably. All

CRONKHITEXANADA SYNDROME 78 1

Fig. 1. Colonic polyp consisting of cystically dilated glands, some of which have ruptured and are surrounded by an inflammatory infiltrate. The stroma is oedematous and shows chronic inflam- mation. No glandular dysplasia is seen. (H&E)

nails in his hands and feet had dropped off, being replaced by very soft pitting beds. His alopecia was now universal.

Although his condition improved transiently, it generally continued on a downward trend. He developed peripheral oedema requiring protein and albumin support and high potas- sium replacements. His serum trace element levels remained normal through this time. Despite full parenteral support his condition worsened. He developed septicaemia from a noso- comial pneumonia and died 6 months after presentation, 10 months after onset of symptoms, with multi-organ failure.

DISCUSSION The first association of diffuse gastrointestinal polyposis with ectodermal changes of alopecia, nail atrophy and hyperpigmen- tation of the skin was made by Cronkhite and Canada in 1955.' Since then, less than 100 cases have been reported in the English literature. The polyps were initially thought of as ad- enomas but subsequent review showed them to be inflammatory in nature.* There is no familial tendency in this condition. They are now classified as harmatomatous polyps of the juvenile retention type.

Clinical symptoms usually appear in the fourth and fifth

decade. The initial presentation of our patient revealed a rela- tively mild disease that rapidly progressed over the course of the next few months to an almost fulminant nature.

The nail dystrophy in these patients include thinning, split- ting and separation from the nailbeds. Hyperpigmentation has been most commonly reported in the extremities and face. Alo- pecia was noted to begin with the onset of diarrhoea, implying that malnutrition may have a role in its aetiology. A similar presentation was noted in our patient. However, the alopecia and pigmentation progressed in tandem with the severity of diarrhoea. This aspect of the ectodermal features of this syn- drome has not been emphasized in previous reports. Malnutri- tion is not the sole cause of these symptoms because patients on total parenteral nutrition who have no biochemical defi- ciency, like CSJ, still manifest these symptoms. It would seem that the ectodermal and the gastrointestinal inflammation may have similar underlying aetiologies leading to concomitant pro- gression in both aspects.

The diarrhoea and hypoproteinaemia seem to arise as a result of protein loss into the gastrointestinal lumen. This may be due to a defect in the differentiation of the endothelium, which sug- gests a functional disorder rather than a neoplastic process. Non-specific gastrointestinal tract malabsorption has been previously demonstrated. A disaccharidase deficiency with bac- teria overgrowth has been described.' Associations with sys- temic vasculitis4 and erosive arthritisS have also been described. Our patient did not have any of these symptoms.

The treatment of the Cronkhite-Canada syndrome has evolved from resection of the polyp-containing region to con- servative treatment with hyperalimentation. Our patient had total involvement of the gastrointestinal tract that precluded sur- gical intervention and was therefore supported with hyper- alimentation, anabolic steroids and antibiotics. These have only transient benefits?

Malnutrition inevitably contributed to the onset of septicae- mia from a nosocomially acquired pneumonia, leading to multi- organ failure. The prognosis of this condition is poor, with a reported survival rate of 45% for 6 months.

1.

2.

3.

4.

5.

6.

REFERENCES Cronkhite LW, Canada WJ. Generalised gastrointestinal poly- posis. An unusual syndrome of polyposis, pigmentation, alopecia and onchodystrophia. N. Engl. J. Med. 1955; 252: 101 1-15. Diner WC. The Cronkhite-Canada syndrome. Radiology 1972; 105: 715-16. Johnson GK, Soersel KH, Hensley GT et al. Cronkhite-Canada syndrome: Gastrointestinal pathophysiology and morphology. Gastroenterology 1972; 63: 140-52. Parsa C. Cronkhite-Canada syndrome associated with systemic vasculitis. An autopsy study. Hum. Pathol. 1982; 13: 758-60. Samders KM, Resnik CS. Owen DS. Erosive arthritis in Cronk- hite-Canada syndrome. Radiology 1985; 156: 309-10. Daniel ES, Ludwig ST, Lewin KJ et al. The Cronkhite-Canada syndrome. An analysis of clinical and pathologic features and therapy in 55 patients. Medicine 1982; 61: 293-309.