| Abstract|| |
Crossed renal ectopia is an unusual anomaly, 90% of which is fused to the ipsilateral kidney. Multicystic dysplasia in association with crossed non-fused renal ectopia is a very rare phenomenon and can produce a palpable abdominal mass. We report a 2-year old girl who presented with a mass in the abdomen. Detailed evaluation including laparotomy confirmed a diagnosis of multicystic dysplasia in a crossed non-fused ectopic kidney. This diagnostic entity should be considered, when a mass is found in the abdomen or pelvis, and when no kidney can be found in it's normal ipsilateral position.
Keywords: Crossed non-fused renal ectopia, Multicystic dysplasia.
|How to cite this article:|
Al Mugeiren MM. Association of Multicystic Dysplasia and Crossed Nonfused Renal Ectopia: A Case Report. Saudi J Kidney Dis Transpl 1997;8:148-51
|How to cite this URL:|
Al Mugeiren MM. Association of Multicystic Dysplasia and Crossed Nonfused Renal Ectopia: A Case Report. Saudi J Kidney Dis Transpl [serial online] 1997 [cited 2019 Oct 14];8:148-51. Available from: http://www.sjkdt.org/text.asp?1997/8/2/148/39389
| Introduction|| |
Crossed renal ectopia is an unusual congenital anomaly in which both the kidneys lie on one side and are fused  . However, in about 10-15% of cases crossed ectopia may be encountered without fusion  . Multicystic dysplasia in a crossed, fused or non-fused, ectopic kidney is rare  . Herein is reported a case of multicystic dysplasia in a crossed, non-fused, ectopic "right" kidney.
| Case Report|| |
HYM, a 2-year old girl, was referred for evaluation of distension of the left lower abdomen from the age of six months. There were no associated symptoms. A large, smooth, mobile mass was palpable in the left lower abdomen. There were no other abnormalities on physical examination. Abdominal ultrasonography (US) revealed a 6.5 cm x 6.5 cm cystic mass located below a normal looking left kidney [Figure - 1].The right kidney was not visualized.
Intravenous urography (IVU) revealed an enlarged malrotated left kidney and the left ureter was displaced laterally by the mass. The right kidney was not visualized Computerized axial tomography (CAT) of the abdomen showed a large cystic mass in the center of the lower abdomen and two small cystic masses on the left side [Figure - 2] as well as absent right kidney [Figure - 3]. It confirmed the US and IVU findings. A pre-operative diagnosis of mesenteric cyst was made.
At laparotomy, a large retroperitoneal cystic mass was found at the base of the sigmoid mesentery. The left kidney was normal and right kidney was absent. The cystic mass, which was not fused with the left kidney, was resected. Histopathological examination of the mass revealed multicystic dysplasia of the kidney [Figure - 4]a and b.
| Discussion|| |
Crossed renal ectopia is reported in 1:2,000 to 1:7,000 of autopsies  . Four types have been described  . They are: a) crossed renal ectopia with fusion; b) crossed renal ectopia without fusion; c) solitary crossed renal ectopia and; d) bilaterally crossed renal ectopia. The embryological explanation is that there is an abnormal development of the ureteral bud arising in close proximity to the metanephric blastema  .
Multicystic renal dysplasia is a common cause of abdominal mass in infancy. It is a histological diagnosis that describes the presence of primitive metanephric structures within the kidney  . Dysplasia may involve the kidney totally, segmentally or focally. It has also been subdivided into solid and cystic variations. It is thought that the dysplasia is the result of either a high or low origin of the ureteric bud from the Wolffian duct, which then grows into metanephric tissue lacking the potential for normal renal development, thereby leading to the formation of a dysplastic kidney  . To the best of our knowledge, there have been only 19 reported cases of crossed ectopic kidneys with multicystic dysplasia of the crossed unit ,,,,,,,, . Of these only one, a newborn, had crossed ectopic non fused mylticystic kidney  . Our patient is probably the second case of crossed ectopic non-fused kidney with multicystic dysplasia.
In conclusion, although crossed renal ectopia is uncommon, it must be considered when a cystic mass is found in the abdomen or pelvis even if it is not fused to the ipsilateral kidney particularly if no kidney can be found on the opposite side.
| Acknowledgement|| |
I would like to thank Professor. Asal Al Samarrai and Dr. Saeed Ahmed for their critical review of the manuscript and Gemma S. Gemaol for her secretarial assistance.
| References|| |
|1.||Marshall FF, Freedman MT. Crossed renal ectopia. J Urol 197S;119:188-91. |
|2.||Hertz M, Rubinstein ZJ, Shahin N, Melzer M. Crossed renal ectopia: clinical and radiological findings in 22 cases. Clin Radiol 1977;28:339-44. [PUBMED] |
|3.||Evans WP, Sumner TE, Lorentz WB Jr, Resnick MI. Association of crossed fused renal ectopia and multicystic kidney. J Urol 1979;122:821-22. [PUBMED] |
|4.||Bagentose AH. Congenital anomalies of the kidney. Med Clin North Am 1951;35:987-91. |
|5.||McDonald ZH, McClellan DS. Crossed renal ectopia. Am J Surg 1957;93:995-99. |
|6.||Bernstein I. Developmental abnormalities of the renal parenchyma, renal hypoplasia and dysplasia. Pathobiol Annu 1968;3:213-15. |
|7.||Maizels M, Simpson SB Jr. Primitive ducts of renal dysplasia induced by culturing ureteral buds denuded of condensed renal mesenchyme. Science 1983;219:509-10. [PUBMED] [FULLTEXT]|
|8.||Tanenbaum B, Sanford RS, Pochaczevsky R, Weinberg SR, Rosenkranz W. Crossed fused renal ectopia with calcific cystic dysplasia mimicking a malignant renal neoplasm. J Can Assoc Radiol 1972;23:142-4. [PUBMED] |
|9.||Abeshouses BS, Bhisitkul I. Crossed renal ectopia with and without fusion. Urol Int 1959;9:63. |
|10.||Herczeg T, Rutkai P, Deak J. Metanephrogenie cystic remnant of crossed dystopic kidney. J Urol 1956;76:488. |
|11.||Daniel WW, Datnow B. Crossed fused renal ectopia with renal dysplasia. Am J Roentogenol 1977;128:845-6. |
|12.||Rosenberg HK, Snyder HM 3d, Duckett J, Abdominal mass in a newborn: Multicystic dysplasia of crossed fused renal ectopiaultrasonic demonstration. J Urol 1984;131:1160-1. |
|13.||Nussbaum AR, Hartman DS, Whitley N, McCauley RG, Sanders RC. Multicystic dysplasia and crossed renal ectopia. Am J Roentgenol 1987;149:407-10. |
|14.||Hendren WH, Donahoe PK, Pfister RC. Crossed renal ectopia in children. Urology 1976;7:135-44. [PUBMED] |
|15.||Fishman M, Borden S. Crossed fused renal ectopia with single crossed ectopic ureterocele. J Urol 1982;127:117-8. [PUBMED] |
|16.||Farkas A, Earon J, Firstater M. Crossed renal ectopia with crossed single ectopic ureterocele. J Urol 1978;119:836-8. [PUBMED] |
Mohammad M Al Mugeiren
Department of Pediatrics, College of Medicine and King Khalid University Hospital, P.O. Box 2925, Riyadh 11461
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]