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Acute Urinary Retention Due to Didelphys Uterus Associated With an Obstructed Hemivagina in a 5-Month-Old Infant By L. Pansini, M. Torricelli, A. Gomarasca, C. Brambilla, S. Beolchi, and M. Sideri Milan, Italy 9 A double uterus associated with an obstructed hemi- vagina is a rare malformation syndrome that has been described in about one hundred cases in the world litera- ture. The malformation is diagnosed after menarche because of symptoms caused by hematocolpos or hema- tometra, and it is occasionally associated with ipsilateral renal agenesis. In the case described here, the malforma- tion was symptomatic when the patient was 5 months old, and was associated with ipsilateral renal hypoplasia. The clinical and etiologic implications are discussed. 9 1988 byGrune & Stratton. Inc. INDEX WORDS: Double uterus; didelphys uterus. CASE REPORT A 5-month-old girl was referred to our Department of Pediatric Surgery because of symptoms of acute urinary retention. Bladder catheterization produced 300 mL of sterile urine. Upon physical examination we found a solid round mass with a smooth surface, 5 cm in diameter, that occupied the pelvis. Rectal examination showed a distended posterior cul-de-sac, not painful, and confirmed the presence of a pelvic mass adhering to the pelvic floor. Renal echotomography showed a hypertrophic left kidney, whereas the right kidney was not visualized. However, an ectopic kidney was present in the presacral region. Urography confirmed the presence in the right presacral region of an irregular renal pelvis and of a distended right ureter (Fig 1). Voiding cystography showed the bladder displaced anteriorly and laterally to the left. Upon colpogra- phy, the vaginal cavity was displaced to the left and compressed by an extrinsic mass (Fig 2). Surgery was performed to evaluate the origin of the mass. After needle aspiration of 60 mL of jelly-like brownish fluid from the mass, a didelphys uterus with an obstructed right hemivagina was observed. A communication was performed between the right obstructed and left patent hemivaginas. Right ureteronephrectomy then completed the surgical procedure. Two years later the child was doing well. At vaginoscopy, the two vaginas were patent and two normal cervices were observed. DISCUSSION Didelphys uterus with an obstructed hemivagina is a malformation syndrome that usually becomes symp- tomatic after menarche. ] In the present case, the symptoms appeared at the age of 5 months. The From the Departments of Pediatric Surgery, and Obstetrics and Gynecology, "Vittore Buzzi" General Hospital; and the First Clinic of Obstetrics and Gynecology, University of Milan, Italy. Address reprint requests Dr M. Sideri, Prima Clinica Ostetrica e Ginecologica dell'Universith di Milano, v. Commenda, 12, 20122, Milano, Italy. 9 1988 by Grune Stratton, Inc. 0022-3468/88/2310-0025$03.00/0 Fig 1. Urography. An irregular right renal pelvis (arrows) and distended right ureter can be seen. 984 Journal of Pediatric Surgery, Vo123, No 10 (October), 1988: pp 984-985

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Page 1: Acute urinary retention due to didelphys uterus associated with an obstructed hemivagina in a 5-month-old infant

Acute Urinary Retention Due to Didelphys Uterus Associated With an Obstructed Hemivagina in a 5-Month-Old Infant

By L. Pansini, M. Torricelli, A. Gomarasca, C. Brambilla, S. Beolchi, and M. Sideri

Milan, I ta ly

�9 A double uterus associated wi th an obstructed hemi- vagina is a rare mal format ion syndrome that has been described in about one hundred cases in the wor ld l i tera- ture. The mal format ion is diagnosed af ter menarche because of symptoms caused by hematocolpos or hema- tometra , and it is occasionally associated wi th ipsilateral renal agenesis. In the case described here, the mal forma- t ion was symptomat ic when the pat ient was 5 months old, and was associated wi th ipsilateral renal hypoplasia. The clinical and etiologic implications are discussed. �9 1988 byGrune & Stratton. Inc.

INDEX W O R D S : Double uterus; didelphys uterus.

CASE REPORT

A 5-month-old girl was referred to our Department of Pediatric Surgery because of symptoms of acute urinary retention. Bladder catheterization produced 300 mL of sterile urine. Upon physical examination we found a solid round mass with a smooth surface, 5 cm in diameter, that occupied the pelvis. Rectal examination showed a distended posterior cul-de-sac, not painful, and confirmed the presence of a pelvic mass adhering to the pelvic floor. Renal echotomography showed a hypertrophic left kidney, whereas the right kidney was not visualized. However, an ectopic kidney was present in the presacral region. Urography confirmed the presence in the right presacral region of an irregular renal pelvis and of a distended right ureter (Fig 1). Voiding cystography showed the bladder displaced anteriorly and laterally to the left. Upon colpogra- phy, the vaginal cavity was displaced to the left and compressed by an extrinsic mass (Fig 2). Surgery was performed to evaluate the origin of the mass. After needle aspiration of 60 mL of jelly-like brownish fluid from the mass, a didelphys uterus with an obstructed right hemivagina was observed. A communication was performed between the right obstructed and left patent hemivaginas. Right ureteronephrectomy then completed the surgical procedure. Two years later the child was doing well. At vaginoscopy, the two vaginas were patent and two normal cervices were observed.

DISCUSSION

Didelphys uterus with an obstructed hemivagina is a malformation syndrome that usually becomes symp- tomatic after menarche. ] In the present case, the symptoms appeared at the age of 5 months. The

From the Departments of Pediatric Surgery, and Obstetrics and Gynecology, "Vittore Buzzi" General Hospital; and the First Clinic of Obstetrics and Gynecology, University of Milan, Italy.

Address reprint requests Dr M. Sideri, Prima Clinica Ostetrica e Ginecologica dell'Universith di Milano, v. Commenda, 12, 20122, Milano, Italy.

�9 1988 by Grune Stratton, Inc. 0022-3468/88/2310-0025$03.00/0

Fig 1. Urography. An irregular right renal pelvis (arrows) and distended right ureter can be seen.

984 Journal of Pediatric Surgery, Vo123, No 10 (October), 1988: pp 984-985

Page 2: Acute urinary retention due to didelphys uterus associated with an obstructed hemivagina in a 5-month-old infant

ACUTE URINARY RETENTION 985

Fig 2. Colpography. The vagina is evidently displaced to the left and compressed by an extrinsic mass,

symptomatology, consisting of acute urinary retention, was a consequence of the displacement of the urinary bladder, due to distension of the obstructed right hemivagina. Catheterization resolved the acute symp- toms, but to remove the cause of the bladder displace- ment, surgery was necessary, consisting of the drain- age of the pelvic mass with the creation of a communi- cation between the obstructed and patent hemivaginas. However, the question of the origin of the fluid that overdistended the right hemivagina remains unsolved.

A possible explanation is that the fluid was secreted by the epithelium lining the cervix and the walls of the obstructed hemivagina. I n fact, it has been demon- strated that the obstructed vaginal pockets accom- panying the didelphys uterus are covered by columnar epithelium. 2 This finding is not surprising, since also in cases of imperforate hymen the vaginal wall is covered by columnar epithelium) Moreover, it is well known that estrogen is present in the serum of the newborn for at least 6 months after the delivery and could therefore stimulate secretion in a target epithelium. It is even possible that in our patient, unlike other reported cases, hypersecretion occurred with the development of a distended obstructed hemivagina.

Didelphys uterus with imperforate hemivagina is usually associated with ipsilateral renal agenesis. 4 It has been proposed that a common pathogenetic event in the caudal region of the mesonephric duct may be responsible for the renal and Mullerian defects. 2'5 In fact, the development of the urinary and genital tracts occurs at the same embryonal age, between the sixth and ninth weeks of gestation. 6 In addition, from a phylogenetic point of view, the common evolution of the urinary and genital systems is recent, and for this reason, more prone to developmental anomalies. How- ever, the present finding of an ectopic although hypo- plastic kidney is unusual. We do not know if diagnos- ing the abnormality at an early age made the detection easier in this case. It is possible that in other reported cases with older subjects, an ectopic kidney was pres- ent but overlooked at urography or surgery because of a certain degree of involution. Therefore, a careful search for ectopic kidney is suggested in cases of didelphys uterus associated with an obstructed hemi- vagina. Finally, as observed in this case, the malforma- tion occurs more frequently on the right side; the reasons for this are still unclear.

REFERENCES

1. Rock JA, Jones HW: The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis Am J Obstet Gynecol 138:339-346, 1980

2. Candiani GB, Fedele L, Zamberletti D, et al: L'utero didelfo con emivagina imperforata. Ann Ostet Ginecol Med Perinat 102:233-243, 1981

3. Dei M, Marchionni M, Vannelli G, et al: Extensive diethylstil-

bestrol unrelated vaginal adenosis associated with imperforate hymen: A case report. Cervix and LFGT 1:161-166, 1983

4. Marshall FF, Beisel DS: The association of uterine and renal anomalies. Obstet Gynecol 51:559-565, 1978

5. Gruenwald P: Developmental basis of degenerative and patho- logic growth in the uterus. Arch Pathol 35:53-69, 1943

6. Crosby WM, Hill EC: Embryology of the Mullerian duct system. Obstet Gynecol 20:507-519, 1962