Persistent Müllerian duct remnantin a newborn

'EBSISTENT I

F

UCT RE MNAONT

NEWBORN

I tIAIIKI,

l) .

roar the Departments of t ral Pediatric rgery, and t')trasound Unit :, Soroka Un e c Hospital and qulty of Health Sciences ._ Ben Curi n University of the Negev, Beer-Sheva . Israel

ABSTRACT . . 1uiie rian duct remnant is a newborn is ex rare x ing . Thejo eset cell the nitrasunir and -radrologic features of the entify.

product of an uncomplicated full-term pregnancy, was examined soon after birth hecau,se of bilateral undescended testes and lower abdominal mass . Phenotype was mascuine with a perfect dtfferenliation of the external genitalia Ultrasonic examination showed bilateral moderate hydronephrosis of the kidneys and a partially solid but dominantly cystic elongated mass which was located behind the bladder and ended below the level of bladder neck (Fig . 1) . Voiding evstourethrography was normal . Simultaneous barium enema revealed space-occupying lesion between bladder and rectum . On rectal examination, the mass was palpable anteriorly, and during massage

cervi tissue No vas-

ended by dense fz e structure was idc ntifiab [IN sterectom -terolycis were 1 formed preserving all the elements eonnectc both small testes . Thus bilateral orc hio cxt easily performed . Pepeated ultrasound of_ net's revealed normal kidneys and ureters. months later, while the right testis be severely atrophic and riot palpable at al left testis was palpable in the scrotwn_

bloody discharge coming from external meatus was suggestive of possible existence of iniilierian duct remnants . Betrograde; urethrography using "flush technique" demonstrated well! he vaina attached to the posterior urethra (Fig . 9) . _m exploratory iaparotomy was performed revealing a. Yshaped uterus and vagina . The whitish oval structures of 0 .5 x 0 .8 em in diameter on the right, 0 .9 x 1 .0 cm in diameter on the left at the end of the fallopian extensions were suggestive of testes (Fig . 3) . Frozen sections from them showed histology c pattern of immature testes with absence of germinal cells . The ureters were dilated above the level of the

33?

UROLOGY

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Fluuru: S .

bladder :

aS :

fallopian full€c ; 7' lion of ul. 1 'ian inhibiting factor ;.4 1C usually secreted by fetal testes . The karyo 46,33 and external genitalia is n u .
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d

gaaphy showing and vagina (V),

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coai men I. on was the firstt to describe this ale psendohermaphroditism con, n inullerian duct structures in the condition has been reported siblings suggesting an autosomal er

of inheritance . The. term "hernia Uteri inguinalis" is )classification of this -

)i condition is usually found in ird decade of life in patients iricocele, or inor . it diagloss r mulleritan duct orn is extremely rare . The to : synthesis or ac-

AF'RU . . uxl

VOLUME XXXL NUMBER 4

this syndrome should be rearec, vborns identification of the i ale 1t' av be very difficult in presence of fallopian-like structures . Sot,a authors2 ' recommend that the mulls i_an strtic tares should be left in place because their el proximity to the vas might load to their destruu _rtion during removal, and possible fertil : should he preserved . I n ow c ,se xernoval of t`tmullerian structures seems to be justifiable hcause of fibrotic tissue around the ureters aa .c lack of normal testicular morphology in frozen section .

Medical Cell ;ci' 'he, d)B, is erencrs ogrephv ill i crssxuai i say 3adfr .i ;:' 82 : i,f> t"'&4 2 . Shun by an \ sh PC:: Fa m ilial rers c r a:'Plcrfan duetamtnu :, :Tin 1a473Uo-'6j_ t . Scum : i, sad fi,n 9'de*et tV At,le mclle_laa hermphrodatiam it a ase report of r re eausec ie rt,h=t FW! Start 2s : 2^3 if977 : . Fam11ia1 t d:tant me c s . duo t;arv Beh shit ll, e . A u-ome F 7`0 1 131 : L68 (NS4' .