Cystic dysplasia in ectopic kidney associatedwith absent vas deferens and congenital inguinal hernia

CYSTIC DYSPLASIA. IN ECTOPIC KIDNEY ASSOCIATED WITH ABSENT VAS DI FERENS AND CONGENITAL INGUINAL HERNIA .

IIERSILMIAN, E.R .C .S .

i' M . DAWSON . ER .C .S . A . W L . I:EUNG . ER .C .R . M . R SINGI3 . ER .C .S . From the Department of Surgery and Hadiolo Hammersmith Hospital, London, England

report cc case of congenital cystic dysplasia of a kidney associated w 7RAC I-adefea'eus and cong enital ingainal hernia on thee sank side, The findings are e* plaine fc tern ; Congenital abnormalities of the kidney are often . associated with other abnormalities lie urogenital system and should he looked for when . ,ranging children with this condition,

t of the mesonephrie duct al absence of the vas defi esicle . or epididvmis, and nay sociated with abnormalities of the ureter "due .) .' Testicular ductal abnormalities m al forms from isolated segmental a and absent seminal e conditions may be bilateral .' t . dysplastic kidneys are almost sociated with a recognizable urinary rmality 3 L

Case Report ition of a thirty,regnant woman showed the presence of lo c the abdomen of the fetus intestinal obstruction (Fig . l) . s subsequently delivered by cesarthirty-seven weeks . Apgar scores ie minute and 9 at five minutes . On ininal examination, an irregular mobile mass was palpated in the right iliac fossa . mal x-ray films failed to demonstrate inobstruction, and the infant was diniavell . irogrant !iVUt showed an '} extending from vertebral

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level Dll to L3 with a no v icaliceal sysng system was tem . A second opacified eo The bladder seen in the right iliac fossa (Fig, . vt=as compressed by a soft tissue mass in the lower abdomen . Repeat ultrasound examination confirmed the cystic nature of the mass, and teratoma was considered an alternative diagnosis . A renogram demonstrated no appreciable renal function in the second kidney in th , right iliac fossa . A magnetic resonance imaging Mill} examination was performed using a Picker ogenie whole body scanner operating at tesla, Sagittal and coronal images of the abdomen were obtained using the saturation recovery (SR 1000) and the inversion recovery (IR 1400, /400) sequences . Mlii revealed multiple e}-stir lesions occupying the lower half of the abdomen (Fig . 3A), displacing the bowel loops posteriori y A normal right kidney was demonstrated (Fig . 313), but the left .kidney could net be confidently identified . It was believed the multicvstie mass could represent a multicvstic left kidney. At operation a large lobulated cystic mass was found in the signoid mesentery (Fig . 4) . Attached to this was a blind-endin

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ed to be ureter, The mass was out difficulty, and the infant -y. Histology of the lesion nign cystic dysplasia of the kidney, ks later the child presented to the cr ` far with a reducible left inal hers At exploration of the inguinal o vita deferens could be found . The epiis was found to be a vestigial remnant . A otonty was performed, and an apparently tic was placed in a dartos pouch . The ads a good recovery and remains cli 1

ical findings may be explained in ernterms . During normal development esonenhros and genital ridge on the mee develop into the testis, rete testis, and n stc The wolffian duct similarly phros and matures to ie the lower end of the , the ureteric bud develops in the or definitive kidneys This subsees the definitive collecting sysd vas deferens subsequently o aer xsition in the scrotam . 4

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cc by Cystic dysplasias are thou rl a failure of the collecting sys to slop properly. In this case, failure o> -,volffian the subduct to differentiate would accoun sequent failure of the ureteric bud t metanephrie cap . The congenital inguinal hernia developed due to abnormal descent and development of the testis .' Similarly, the absent vas deferens may be explained, although this condition may he bilateral ." Characteristically the cystic dysplastic kidney presents as a mass in the abdomen of the neonate . The condition is benign if unilateral, although fatal bllaterally . It is more commonly associated with congenital valvular obstruction .' In a review of dysplastic kidneys, Ridsont identified that in all cases there is a recognized ab formality of the urinary tract ' this case absent vas and rudimentary Retrospectively, the prenatal c testinal obstruction was clearly mistaken large cystic kidney, the appearance of w simulated dilated loops of bowel . Further investigation confirmed the diagnosis . This case, however, highlights that other abnormalities of the urinary system may be present in children with this condition . Careful exploration of the surrounding structures, particularly the rernaining ipsilateral genitourinary organs known to develop front the rtesonephrie ridge, may 'fy other Surgical y correctable conditions . 111 Middletoo Road Hackney London, E84 LN' Engla (ME . J1ERSFy1%M\

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Surq Enai . 17 : 159 !1955, . 3 . liirlsoa HA : Renal dv sulasia, J C i : Pathoi 24 : 5 7 (1 4 . L.an nsan J: The a ngenita. }wean, ;nr : ltdlical Psnbr,mogA 31,3 c, Baltimore, Villhams and wilkin:. Co l 1074, chap 11 0-200 .

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