Cacchi-ricci and Caroli: Both Rare but Frequently Associated

Z. Benamara *

Nephrology, Dialysis and Renal Transplantation Unit, Chu Ibn Rochd, Casablanca, Morocco.

S. Hamine

Nephrology, Dialysis and Renal Transplantation Unit, Chu Ibn Rochd, Casablanca, Morocco.

S. Nahmed

Pediatric Radiology Unit, Chu el Harouchi, Casablanca, Morocco.

S. S. Elkhayat

Nephrology, Dialysis and Renal Transplantation Unit, Chu Ibn Rochd, Casablanca, Morocco.

G. Medkouri

Nephrology, Dialysis and Renal Transplantation Unit, Chu Ibn Rochd, Casablanca, Morocco.

M. Zamd

Nephrology, Dialysis and Renal Transplantation Unit, Chu Ibn Rochd, Casablanca, Morocco.

N. Mtioui

Nephrology, Dialysis and Renal Transplantation Unit, Chu Ibn Rochd, Casablanca, Morocco.

M. G. Benghanem

Nephrology, Dialysis and Renal Transplantation Unit, Chu Ibn Rochd, Casablanca, Morocco.

D. Laoudiyi

Pediatric Radiology Unit, Chu el Harouchi, Casablanca, Morocco.

K. Chbani

Pediatric Radiology Unit, Chu el Harouchi, Casablanca, Morocco.

S. Salam

Pediatric Radiology Unit, Chu el Harouchi, Casablanca, Morocco.

*Author to whom correspondence should be addressed.


Abstract

Aims: Caroli and Cacchi Ricci are two rare diseases but frequently associated. We report the case of a young girl who presented these exceptional entities.

Results: We report the case of a 14-year-old girl with no particular pathological history, who presented to the emergency room with rectal bleeding that had been evolving for 1 months. The association of Cacchi Ricci and Caroli diseases was diagnosed on imaging (abdominal ultrasound and computed tomogrphy, bili-MRI), following an assessment carried out in the face of signs of portal hypertension and gastrointestinal bleeding.

Conclusion: Although both rare, Cacchi Ricci disease and Caroli syndrome are frequently associated. This association is to be watched all the more since it increases overall morbidity and mortality and often requires consideration of double hepatorenal transplantation as the only curative therapeutic alternative.

Keywords: Caroli, Cacchi Ricci, sponge medullar kidney


How to Cite

Benamara , Z., Hamine , S., Nahmed , S., Elkhayat , S. S., Medkouri , G., Zamd , M., Mtioui , N., Benghanem , M. G., Laoudiyi , D., Chbani , K., & Salam , S. (2023). Cacchi-ricci and Caroli: Both Rare but Frequently Associated. Asian Journal of Research in Nephrology, 6(1), 88–91. Retrieved from https://journalajrn.com/index.php/AJRN/article/view/65

Downloads

Download data is not yet available.

References

K.Garfiels, SW.Leslie. Medullary sponde kidney. In: StatPearls. StatPearls Publishing; 2023 Jan. 2023 May 30.

Fabris A, Anglani F, Lupo A, Gambaro G. Medullary sponge kidney: state of the art. Nephrol Dial Transplant. 2013;28(5): 1111-9

Geavlete P, Nita G, Alexandrescu E, Geavlete B. The impact of modern endourological techniques in the treatment of a century old disease--medullary sponge kidney with associated nephrolithiasis. J Med Life. 2013;6(4): 482-5.

Umar J, Kudaravalli P, John S. Caroli disease. In: StatPearls. Stat Pearls Publishing; 2023 Jan. 2022 Aug 1.

Giacobbe C, Di Dato F, Palma D, Amitrano M, Iorio R, Fortunato G. Rare variants in PKHD1 associated with Caroli syndrome: two case reports. Mol Genet Genomic Med. 2022;10(8): e1998. DOI: 10.1002/mgg3.1998

Fahrner R, Dennler SGC, Dondorf F, Ardelt M, Rauchfuss F, Settmacher U. Liver resection and transplantation in Caroli disease and syndrome. J Visc Surg. 2019;156(2):91–95.

DOI: 10.1016/j.jviscsurg.2018.06.001.

Duranti E, Duranti D. Caroli's Syndrome in Hemodialysis: When Diagnostic and Therapeutic Difficulties can have Serious Consequences. Asp Biomed Clin Case Rep. 2023;6(2):64-68.