Transverse testicular ectopia with persistent müllerian duct syndrome

Transverse Testicular Ectopia With Persistent Miillerian Duct Syndrome By ibrahim

Karnak,

F. Cahit Tanyel, Ankara,

Transverse mtillerian testicular reported. discussed. presence dissection

testicular ectopia is rarely associated with persistent duct syndrome. The ninth pediatric case of transverse ectopia with persistent mijllerian duct syndrome is The clinical and operative findings and treatment are The importance of abdominal exploration in the oftwo gonads in one inguinal side and the avoidance of of mtillerian structures has been stressed.

T

RANSVERSE TESTICULAR ECTOPIA (TTE) is one of the rarest forms of testicular ectopia. In this condition, two testes are located in one inguinal side. One testis crosses the midline to meet its opposite mate at the internal inguinal ring, in the inguinal canal, or in the hemiscrotum. The entity has also been named as crossed testicular ectopia, testicular pseudoduplication, unilateral double testis, and transverse aberrant testicular maldescent.‘,’ TTE was first described by Lenhossek in 1886. Since then, about 100 cases have appeared in the English-language literature.1-4 Persistent mi.illerian duct syndrome (PMDS) is a rare form of male pseudohermafroditism characterized by the persistence of mtillerian duct structures in phenotypically normal boys.3 PMDS was first described by Nilson in a man with inguinal hernia in 1939 as hernia uteri inguinale. Subsequently, more than 100 caseshave appeared in the literature.3 Although TTE and PMDS association have been reported in adults, only eight pediatric cases have appeared in the English-language literature4-6 (Table 1). The ninth case of TTE associated with PMDS prompted us to do a comprehensive review of the literature with evaluation of the clinical features and the management. CASE REPORT A 9-month-old boy was admitted with the complaint of right inguinal swelling that had been presented since birth. On physical examination, a right-sided inguinal hernia and empty left scrotum was found. The right gonad of 1 X 1 cm was in the right scrotum. Other physical findings

From the Departments of Pediatric Surgery und Pediatric Hacettepe University Faculty of Medicine, Ankara, Turkey. Address reprint requests sity Faculty of Medicine, Slhhiye Ankara, Turkey.

to E Cahit Department

Copyright 0 1997 by WB. Saunders 0022-3468/97/3209-0025$03,00/O

1362

Pathology,

Tanyel, MD, Hacettepe of Pediatric Surgery, Company

Univer06100

Zuhal

AkCGren,

and Akgijn

Hipijnmez

Turkey

J Pediatr Company.

Surg

32: 1362- 1364. Copyright

INDEX WORDS: Ectopic lerian duct syndrome.

testis,

testicular

o 1997 by W.B. Saunders

ectopia,

persistent

mtil-

were normal. The family history was unremarkable. The diagnosis of right sided inguinal hernia and left nonpalpable testis was made. At the time of operation, the right inguinal region was explored initially. Hernia sac and cord structures traversing the internal inguinal ring were identified. During pulling of the right gonad, an additional gonad protruded through the internal ring (Fig 1). Both gonads were similar in size (1 X 1 cm) and macroscopically were testes. There were two vas deferenses and vascular structures accompanying each gonad. At left inguinal exploration, no other gonad was found in the canal. The abdomen was explored through a pfannensteel incision. Blood supplies to the gonads originated from both sides. Both vas deferenses were found to be in close relation with a tubular, uteruslike structure located in midline and posterior to the urinary bladder. Biopsy specimen was taken from this tubular structure. Frozen sections of both gonads showed them to be testes without evidence of ovarian tissue. Both the right and left testes were placed into subdartos pouches on their respective sides after limited mobilization of the cord structures and vessels. Histopathologic examination of the tubular structure showed infantile uterus lined with columnar epithelium (Fig 2). Postoperative course was uneventful. During the postoperative period, both abdominal ultrasonography and voiding cystourethrography were normal. Buccal smear showed no Barr body, and the karyotype based on peripheral blood was 46, XY.

DISCUSSION

Transverse testicular ectopia (TTE) is a rare form of testicular ectopia of uncertain embryological etiology. Adherence and fusion of developing wolffian ducts, aberrant gubernaculum, testicular adhesions, defective internal inguinal ring, and traction on a testis by persistent mtillerian structures are the examples of suggested embryological explanations.’ Persistent mtillerian duct syndrome (PMDS) may result from the failure of synthesis or releases of mtillerian duct inhibitory factor (MIF), the failure of end organs to respond to MIF, or defect in the timing of the release of MIE3 It seems possible that the mechanical effect of the persistent mtillerian duct structures prevents the testicular descent or leads to both testicles descending toward the same hemiscrotum, producing TTE, as in our case. Journaloffediatricsurgery,

Vol32,

No 9 (September),

1997: pp 1362-1364

TESTICULAR

ECTOPIA

Fig 1. Operative denotes uterus.

AND

view

MULLERIAN

during

right

DUCT

SYNDROME

inguinal

exploration.

1363

Arrow

It has been well recognized that inadequate mtillerian depression occurs in 20% of TTE cases.‘-3,7Additional genitourinary abnormalities such as hypospadias, penoscrotal transposition, seminal vesical cyst, common vas deferens, renal agenesis, horseshoe kidney, and ureteropelvic junction obstruction are reported among patients who have TTE.‘-i” Pseudohermafroditism exists in nearly one third of patients who have TTE.4 Therefore, complete radiological evaluation of the genitourinary system and karyotyping are required in all TTE cases. We did not meet with additional genitourinary abnormality in our case. Although preoperative diagnosis could have been made in some cases, the diagnosis is often made incidentally during herniorraphy or during exploration for undescended testis. When two gonads of the same size are encountered during inguinal operation, complete abdominal exploration is required. On the other hand, either before inguinal exploration or immediately after the diagnosis is established, laparoscopy may be considered if available. Cord structures should carefully be inspected for any abnormality, and the origin of the vessels should be noted. Both gonads undergo frozen section biopsy to Table

1. Clinical

Data of the Cases of lTE

Associated

With

Year

Age

Side

Hernia

2yr 9mo 1.5 yr

Right

Present

Uterus

and tubes

Stonham4 Link4

1913 1928 1930

Left Right

Present Present

Uterus Uterus

and tubes

Staube? StaubeP

1965 1965

lOmo* 3 mo*

Fourcroys Mahfo@ Mahfo&

1982 1990 1990

4yr

Right Left Right

Present Present Present

Uterus and tubes Uterus Mtillerian duct

3mo 27 d

Right Right

Present Present

Uterus Uterus

Karnak (present case)

1996

9mo

Right

Present

Uterus

Study

Pellegrin?

*Siblings.

Miillerian

PMDS Structure

and tubes and tubes

Fig 2. Infantile original magnification

uterus lined x131.

with

columnar

epithelium

(H&E,

exclude the possibility of mixed gonadal dysgenesis. If there is no obvious uterus and fallopian tubes, the tissue between the vas deferenses should undergo biopsy to recognize the mtillerian duct structures. The vas deferenses and vessels should not be dissected extensively because of the possibility of trauma. Various procedures have been described for the treatment of TTE. Transseptal fixation, also referred to as modified Ombredanne operation, has initially gained popularity. In this operation both cords and spermatic vessels reach at the respective testes, which are placed into the respective hemiscrotums through the same inguinal canal. If there is trauma or infection, both cords and vascular structures are prone to damage. These possibilities make this operation risky. Therefore, we recommend translocation of the ectopic testis through its inguinal canal into the newly formed hemiscrotum. Malignancy appears to be associated with cryptorchidism, and there have been reports of embryonal carcinoma, seminoma, choriocarcinoma, and teratoma in patients who have TTE or PMDS.” There is no report of malignancy arising from the retained mtillerian structures, and the absence of MIF does not appear to increase the relative risk of testicular malignancy.‘? Therefore, routine hysterectomy is not recommended in patients who have obvious uterus and fallopian tubes. TTE should be suspected preoperatively in patients who have unilateral inguinal hernia associated with contralateral nonpalpable testis. If two gonads come into view during exploration of one inguinal side, complete abdominal exploration must be performed. Both gonads and the tissue between cord structures must undergo biopsy. The ectopic testis should be replaced into its respective hemiscrotum through the ipsilateral inguinal canal. Extensive dissection of vas deferenses and excision of persistent mtillerian duct structures should be avoided to prevent the injury.

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ET AL

REFERENCES 1. Hammoudi S: Transverse testicular ectopia. J Pediatr Surg 24:223224, 1989 2. Gauderer MWL. Grisoni ER, Stellato TA, et al: Transverse testicular ectopia. J Pediatr Surg 17:43-47, 1982 3. Thompson ST, Grillis MA, Wolkoff LH, et al: Transverse testicular ectopia in a man with persistent miillerian duct syndrome. Arch Path01 Lab Med 118:752-755. 1994 4. Golladay ES, Redman JF: Transverse testicular ectopia. Urology 19:181-186. 1982 5. Fourcroy JL, Belman AB: Transverse testicular ectopia with persistent mtlllerian duct. Urology 19:536-538. 1982 6. Mahfouz ESH, Issa MA, Farag TI, et al: Persistent miillerian duct syndrome: Report of two boys with associated crossed testicular ectopia. J Pediatr Surg 25:692-693, 1990 7. Bloom DA, Wan J, Key D: Disorders of the male external genitalia and inguinal canal. in Kelalis PP. King LR, Belman AB (eds):

Clinical Pediatric Urology, chap 22. Philadelphia, PA. Saunders, 1992, pplOl51049 8. bzkardeg H, Genniyanoglu C, Gtlrdal M, et al: Transverse testicular ectopia with penoscrotal transposition. Pediatr Surg Int 9:532-533, 1994 9. Peterson NE: Association of transverse testicular ectopia and seminal vesicle cyst. J Urol 118:345-346, 1977 10. Miura T. Takdhashi G: Crossed ectopic testis with common vas deferens. J Urol 134: 1206- 1208. 1985 I I. Mouli K, McCarthy P Ray P, et al: Persistent mtlllerian duct syndrome in a man with transverse testicular ectopia. J Urol 139:373375, 198X 12. Martin EL, Bennett AH, Cromie WJ: Persistent mtlllerian duct syndrome with transverse testicular ectopia and spermatogenesis. J Ural 147:1615-1617, 1992