Testis sparing surgery for steroid unresponsive testicular tumors of the adrenogenital syndrome.

PURPOSE: Surgical management of steroid unresponsive testicular tumors of the adrenogenital syndrome has been orchiectomy. Magnetic resonance imaging (MRI) of these tumors accurately delineates the extent of disease. Testis sparing surgery is an important consideration, since male individuals with congenital adrenal hyperplasia are potentially fertile. We present our results of surgical management of this tumor based on MRI findings. MATERIALS AND METHODS: Four boys with steroid unresponsive testicular tumors of the adrenogenital syndrome were evaluated with MRI, testicular ultrasound and color flow Doppler examinations preoperatively and postoperatively. Three patients had 21-hydroxylase deficiency and 1 had 3-beta-hydroxysteroid dehydrogenase deficiency. Contralateral testicular abnormalities included a vanished testis, testicular atrophy due to trauma and bilateral tumors in 1 boy each. Bilateral orchiectomy and surgical enucleation were performed in 1 and 3 patients, respectively. Followup ranged from 8 to 18 months. RESULTS: Postoperative MRI of the testis in 2 of 3 patients showed no evidence of recurrent tumor. Postoperative testicular sonography revealed no tumor and vascular flow in 2 of 3 patients. All 3 patients who underwent testis sparing surgery have a viable testis in the scrotum without evidence of recurrent disease. CONCLUSIONS: MRI of the testis in patients with testicular tumors of the adrenogenital syndrome accurately defines the extent of disease. Surgical enucleation of this tumor has been performed successfully without recurrent disease. This surgical approach should be considered for any patient with a steroid unresponsive tumor and contralateral abnormalities. We believe that surgical enucleation is the procedure of choice for all patients with this tumor, since it maximizes future fertility potential.

Principles in repair of hypospadias.

Hypospadias, a common congenital deformity, is the result of incomplete formation of the urethra during the 10th- to 14th weeks of gestational life. Surgery to repair the foreshortened urethra and penile curvature in hypospadias has a history of more than 150 years. To date, more than 300 surgical procedures with accompanying variations have been proposed for the repair of hypospadias. With modern techniques of tissue transfer, even the most severe case of hypospadias is amenable to repair. The modern era of hypospadias surgery has allowed the application of skin grafts and flap techniques to add significantly to urethral length. At the same time, a variety of new techniques have been proposed to "minimize" hypospadias surgery. In this article the authors argue that for the worst cases of hypospadias, complete dissection of the abnormal anatomy and modern tissue transfer techniques should be used to reconstruct the deformity.

Scrotal recurrence of paratesticular rhabdomyosarcoma in a previously undescended testicle 5 years after orchiectomy.

A case of scrotal recurrence of a paratesticular rhabdomyosarcoma 5 years after orchiectomy and chemotherapy is described. To our knowledge local recurrences have been reported previously only in the inguinal region. The patient had a history of orchiopexy, which may have contributed to the scrotal recurrence.

Laparoscopic single stage and staged orchiopexy.

The initial series of laparoscopic single stage orchiopexy for the abdominal or emergent impalpable undescended testis is reported. The first laparoscopic orchiopexy was performed at our institution in October 1991. Between October 1991 and January 1993, 14 patients (16 testes) underwent minimally invasive surgery with 6 months of followup in all cases. No evidence of testicular loss or acute atrophy has occurred, with the entire procedure being accomplished by laparo-endoscopic techniques in all cases. Treatment of 3 of the 16 testicles consisted of stage 2 of staged orchiopexy, and to our knowledge these cases represent the first stage 2 orchiopexy completed by laparoscopic techniques. In those cases stage 1 was performed by laparoscopic clipping of the vessels. The details of the procedure as we now perform it are described.

Use of the appendix in urologic reconstructive operation.

Advances in genitourinary operation have allowed use of an appendicovesicostomy in patients requiring chronic catheterization for bladder emptying. Recent discussions of the appropriateness of incidental appendectomy have ignored the potential usefulness of the appendix as a catheterizable stoma. We present our experience with the Mitrofanoff principle to illustrate the advantages of an appendicovesicostomy and make a plea against incidental appendectomy in certain patient groups.

Laparoscopically assisted continent catheterizable cutaneous appendicovesicostomy.

Described is a case of laparoscopically assisted continent catheterizable cutaneous appendicovesicostomy in a 15-year-old female patient with bilateral ectopic ureteroceles and a totally obliterated bladder neck. The appendix and cecum were mobilized and dissected entirely via laparoendoscopic techniques. The nonrefluxing appendiconeocystostomy required an open cystotomy. The surgical technique and perioperative course are described.

Repair of the complications of hypospadias surgery.

In 1992 a retrospective review was conducted of 190 patients evaluated and treated for complications of hypospadias surgery during 1979 through 1990 at the Devine Center for Genitourinary Reconstructive Surgery of Sentara Norfolk General Hospital and Children's Hospital of the King's Daughters. We could not contact 13 patients and 8 are awaiting a second stage procedure. Of the 177 patients 167 (94.35%) have had a successful outcome, defined as a controllable urinary stream, functional erection and an acceptable cosmetic appearance, and 2 (1.13%) are considered failures. Details of presenting problems, surgical techniques and recent modifications of these procedures are presented.

Laparoendoscopic upper pole partial nephrectomy with ureterectomy.

A 14-year-old white girl presented with a history of recurrent urinary tract infections beginning at age 18 months. Anatomical evaluation revealed bilateral duplication of the collecting systems. On the right side of the upper pole moiety ended at an obstructing orthotopic ureterocele. On the left side the upper pole moiety was associated with an ectopic ureteral orifice. The patient underwent laparoendoscopic right upper pole partial nephrectomy with ureterectomy. To our knowledge this is the first report of a laparoendoscopic upper pole partial nephrectomy. Details of the procedure are discussed.

Phallic construction in prepubertal and adolescent boys.

During the last 10 years we performed microsurgical phallic reconstruction in 7 prepubertal and 4 adolescent boys. Indications for surgery included post-traumatic amputation, circumcision accident, developmental anomalies and micropenis. In addition, we performed phalloplasty on 5 other patients 18 to 24 years old. Total phallic reconstruction consisted of 1-stage microsurgical tissue transfers that included urethral reconstruction, coaptation of erogenous nerves, aesthetic refinement and, in some cases, scrotal reconstruction. All postpubertal patients recovered erogenous sensibility in the reconstructed phallus and the ability to masturbate. Surgical indications, techniques and results are discussed.

Microsurgical forearm "cricket bat-transformer" phalloplasty.

Presently, the donor flap of choice for microsurgical phallic reconstruction is the radial forearm flap. The success of several different design modifications confirms the reliability of the radial and ulnar forearm flaps. Farrow et al. described their experience with the "cricket bat" concept in 1980. To the previous "cricket bat" design, we now wish to add modifications. These modifications utilize longitudinal and transverse rotations of the linear forearm tissues to create a phallus--much like the transformation of a toy robot into a truck. Deepithelialized flaps and a full-thickness skin graft coronoplasty complete glans reconstruction. The "cricket bat-transformer" flap appears to produce the most predictable results in subtotal phallic reconstructions and phallic constructions in the pediatric and transgender patient groups.

Acute bilateral testicular torsion in the adolescent.

A case of acute bilateral testicular torsion in the adolescent is reported. This case reaffirms the need to perform bilateral scrotal exploration in cases of suspected torsion. Although it represents an extreme rarity, delay in exploration may have resulted in bilateral orchiectomy. The case also appears to be another example of intermittent testicular pain as a harbinger of impending torsion in a patient with the bell clapper deformity. Perhaps a more aggressive approach should be taken to explore patients surgically who present with intermittent scrotal pain.

Flow cytometric analysis of DNA content in children with neuroblastoma.

Flow cytometric DNA content analyses were performed in a retrospective study of archival tumor specimens of 15 patients with diagnosed neuroblastoma treated at a single institution. Survival statistics were positively influenced by the presence of aneuploidy. Kaplan-Meier analysis indicated a significant survival advantage for patients with aneuploid tumors (P = .036). Survivors were significantly younger at the time of diagnosis than nonsurvivors, but age at diagnosis did not correlate with DNA content. We conclude that DNA content analysis may be of value in determining the prognosis for children with neuroblastoma.

The use of paraexstrophy flaps for urethral construction in neonatal girls with classical exstrophy.

Primary closure of classical exstrophy was performed with paraexstrophy skin flaps for urethral lengthening in 4 female neonates. In all cases more than 2.0 cm. of urethral length were achieved. Efficacy of bladder closure and subsequent bladder neck revision were enhanced by the use of these flaps. The technique of urethral construction is described and our results are discussed.

Phallic reinnervation via the pudendal nerve.

Total phallic reconstruction presents the genitourinary reconstructive surgeon with one of the most difficult surgical challenges. The development of microsurgical techniques and free tissue transfers have advanced phallic reconstruction by reducing the number of surgical procedures and by allowing more selectivity in choosing the best innervated donor tissue. During the last 5 years 16 patients underwent total phallic reconstruction using free tissue transfers from distant donor sites. The pudendal nerve was coapted routinely to the major sensory nerves of the donor free flap. The most accurate objective baseline parameters of penile sensibility are pressure and vibratory thresholds, and electrically evoked potentials. We examined 30 normal subjects and 7 patients at least 1 year postoperatively for penile (phallic) sensibility. A pressure aesthesiometer, a biothesiometer and electrodiagnostic studies were used for testing. The 7 postoperative patients (in all of whom the pudendal nerve was incorporated into the reconstruction) had an encouraging return of tactile and erogenous sensibility compared to normal subjects. This is a promising advance in phallic reconstruction.

Repair of secondary genital deformities of epispadias/exstrophy.

The aim of secondary genital surgery in epispadias/exstrophy patients is to produce a dangling penis of satisfactory length and shape. The penis should be able to function sexually as well as serve as a conduit for the passage of urine and sperm. The systematic approach to the correction of this defect as outlined in this article should enhance the possibility of a successful result and lessen the need for secondary genital repair.

Transsexual surgery in the genetic female.

The increasing acceptance of the transsexual patient by society as well as a willingness of insurance companies to compensate for associated surgical procedures will result in an increase in patients presenting for sex reassignment. Data from gender identity centers suggest that the multidisciplinary approach leads to the best therapeutic results. Significant advances in phalloplasty techniques are yielding genitalia with more normal function and appearance.

New concepts in phallic reconstruction.

Over the past four years we have performed total phallic reconstructions in 12 patients. Six patients underwent reconstruction following trauma, 3 were female-to-male transsexuals, and 3 had micropenis deformities. These reconstructions were one-stage microsurgical tissue transfers that included urethral reconstruction and coaptation of erogenous nerves. The surgical indications, techniques, and results are discussed.

Microsurgical hypospadias repair.

Successful primary hypospadias repair depends on careful execution of surgical principles, particularly during the urethroplasty portion of the procedure. These principles include careful tissue handling, development of well-vascularized flaps, and avoidance of placing sutures in the uroepithelial surface. Despite meticulous repair, the complication rate requiring secondary surgery is 15 to 30 percent. For the past year, the authors have utilized an operating room microscope, microsurgical instruments, a specially designed microsuture, and a Biooclusive dressing to decrease the postoperative morbidity and subsequent complications requiring secondary surgery (6.5 percent). A comparison of 50 hypospadias patients on whom no microsurgical repair had been used was made with 62 patients on whom microsurgical techniques were employed. The nonmicrosurgical group had 17 complications, 12 of which required reoperation (24 percent). The microsurgical group had 8 complications, 4 of which required reoperation (6.5 percent).