| 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 2020 Jun 6];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.
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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]