Is ADPKD associated with small-bowel diverticular disease?

José M. Peña, Raquel Pernaute and Carmen Vicente de Vera

Nephrology Service, Hospital de Barbastro (Huesca), and Internal Medicine Service, Hospital Universitario Arnau de, Vilanova, Lérida, Spain

Sir,

It has become evident to nephrologists that autosomal dominant polycystic kidney disease (ADPKD) is a systemic disease with multiple extrarenal manifestations. Among these, colonic diverticulosis may be a cause of morbidity and mortality when it presents as diverticulitis and perforation. We report the case of a patient on a dialysis programme because of end-stage renal disease (ESRD) secondary to ADPKD, who was diagnosed with colonic diverticulosis and multiple diverticuli in the small bowel in both the jejunum and ileum. No previous cases of this association have been reported.

Case.

A 75-year-old female chronic haemodialysis ADPKD patient was admitted to our department in August 1999 because of abdominal pain. Nine years earlier she had required an emergency admission because of abdominal pain. A laparotomy was performed and multiple diverticulosis was found in the second part of the jejunum with a perforation and a localized abscess. A partial resection of the jejunum was performed. Post-operative outcome was favourable.

Upon admission in August 1999 a barium intestinal transit and an enema were performed. In the former, numerous diverticuli were found diffusely distributed in the jejunum and ileum (the largest in the jejunum) (Figure 1Go). None of them had radiological signs of complication. The barium enema also showed multiple non-complicated diverticuli, mainly in the sigmoid colon but also in the ascending and transverse colon.



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Fig. 1 Barium-contrast intestinal transit with multiple non-complicated diverticuli of varying size in the jejunum.

 
Clinical problems were solved with diet and intravenous fluids and the final diagnosis was subocclusive pain secondary to peritoneal adhesions.

Discussion.

Among extrarenal manifestations of ADPKD, colonic diverticular disease is a remarkable one. Its prevalence and rate of complications seem to be increased in these patients [1], despite the contradictory results of some authors [2].

A thorough Medline search revealed no previous reports of similar cases of ADPKD associated with multiple diverticulosis in the small and large bowel. Involvement of the small bowel is particularly remarkable as colonic diverticular disease associated with ADPKD is well known. The only exception, to our knowledge, is the case of a patient in an Italian family with type 2 ADPKD who developed an oesophageal diverticulum [3].

It is possible, of course, that the ADPKD and the multiple diverticulosis of the small bowel were coincidental. If so, this would also be a rarity. In general, jejuno–ileal diverticular disease is a rare entity. In a survey of three major US institutions over 23 years, 208 cases of small-bowel diverticulosis were detected, only 18% of them being of jejuno–ileal location. [4]. The prevalence of jejuno–ileal diverticular disease in ESRD is unknown. In a survey of post-mortem examinations carried out on a series of 78 haemodialysis patients to evaluate gastrointestinal pathology, no cases were detected [5]. Because of this we think that the coexistence of the two pathologies in our patient was not a coincidence.

The absence of other reports of small-bowel diverticular disease may be partially due to a lack of screening radiological tests for the small bowel (different from the colon, for which barium enema is usually performed). Consequently, other patients with this manifestation may not have been detected.

Given the advanced age at the time of the clinical manifestations, a particular mutation (probably of type 2) in the ADPKD gene in our patient cannot be ruled out. A genetic study of the patient could not be performed at our institution.

In summary, we report the first observation of an ADPKD haemodialysis patient with multiple diverticulosis of the small bowel. More reports of similar cases will be necessary before a clear association between ADPKD and small-bowel diverticular disease can be made.

References

  1. Perrone RD. Extrarenal manifestations of ADPKD. Kidney Int1997; 51: 2022–2036[ISI][Medline]
  2. Sharp CK, Zeligman BE, Johnson AM, Duley I, Gabow PA. Evaluation of colonic diverticular disease in autosomal dominant polycystic kidney disease without end-stage renal disease. Am J Kidney Dis1999; 34: 863–868[ISI][Medline]
  3. Bozza A, Aguiari G, Scapoli C et al. Autosomal dominant polycystic kidney disease linked to PKD2 locus in a family with severe extrarenal manifestations. Am J Nephrol1997; 17: 458–461[ISI][Medline]
  4. Akhrass R, Yaffe MB, Fischer C, Ponsky J, Shuck JM. Small-bowel diverticulosis: perceptions and reality. J Am Coll Surg1997; 184: 383–388[ISI][Medline]
  5. Varizi ND, Dure-Smith B, Miller R, Mirahmadi MK. Pathology of gastrointestinal tract in chronic hemodialysis patients: an autopsy study of 78 cases. Am J Gastroenterol1985; 80: 608–611[ISI][Medline]




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