Anatomic findings associated with epispadias in boys: Implications for surgical management and urinary continence.

INTRODUCTION: Pre-operative physical examination of male epispadias allows for classification of epispadias level as glanular (GE), penile (PE) or penopubic (PPE), and for delineation of anatomic anomalies. The incidence of associated extragenital abnormalities, such as vesicoureteral reflux (VUR), bladder neck (BN) abnormality and abnormal pubic diastasis (PD), and their impact on urinary continence has not yet been systematically studied. OBJECTIVE: The goal of this study was to evaluate whether the more proximal level of epispadias correlated with associated extragenital anatomic anomalies seen on initial imaging or endoscopic evaluation, and whether these pre-operative findings contributed to subsequent surgical management and impacted on achieving urinary continence. It was hypothesized that the more severe forms of epispadias may be associated with a higher frequency of associated anomalies. STUDY DESIGN: The study was an IRB-approved, retrospective case study of all male patients treated initially for isolated epispadias at the current institution between 1994 and 2011. Data collection was achieved by chart and radiology review evaluating PD, BN appearance, presence of VUR, surgical treatment, and urinary continence. RESULTS: A total of 26 patients were identified and divided into three groups based on appearance at physical examination: four glanular (GE), eight penile (PE), and 14 penopubic (PPE); 17 patients had an abnormal BN. Reflux was noted in nine of 20 patients who had a voiding cystourethrogram (VCUG), two of which had an episode of pyelonephritis. Of the 22 patients past the age of toilet training, 17 were continent (64% (9/14) penopubic, 63% (5/8) penile, and 75% (3/4) glanular). DISCUSSION: Anatomic classification for male epispadias did not provide sufficient information regarding extragenital findings. This study provided new information regarding PD, BN appearance, presence of reflux, and ultimate urinary continence. Pubic diastasis and BN abnormalities were more frequently seen in more severe forms of epispadias, whereas VUR seemed more prevalent in less severe forms. A template for pre-operative evaluation was outlined. Limitations of the study were its retrospective design and relatively small cohort of patients, which reflected the rarity of the condition. CONCLUSION: Based on the information generated, additional anatomic information was generated regarding boys with epispadias. This information will help guide the evaluation and the management of these patients in the future.

Bilateral renal vein thrombosis in a newborn: a case of prenatal renal vein thrombosis.

Neonatal renal vein thrombosis (RVT) is a well described entity that typically presents after a variety of neonatal stresses. Prenatal RVT is a less common entity found incidentally on prenatal imaging. We report a case of neonatal RVT and a probable prenatal RVT.

Laparoscopy for the evaluation and management of the nonpalpable testicle.

OBJECTIVE: To assess the usefulness of laparoscopy for the diagnosis and treatment of the nonpalpable testicle and to evaluate the results of laparoscopic orchidopexy. METHODS: We retrospectively reviewed the charts of 91 laparoscopies performed over a period of 4 years. We reviewed the following parameters: findings, complications, patency of the processus vaginalis, appearance of the cord structures, and the success of the different procedures performed for the intra-abdominal testicle. RESULTS: Fourteen patients had bilateral and 77 unilateral nonpalpable testicles. The mean age of the patients was 39.5 +/- 50.8 months. There were three laparoscopic complications (3.2%) one of which was a major bowel laceration. Laparoscopy defined the intra-abdominal anatomy accurately in 90 of the 91 cases Of the 26 intra-abdominal testicles above the ring, a one-stage laparoscopic orchidopexy was performed in five and a single-stage standard orchidopexy in seven. All of these testicles remain viable and are in good position. Eight patients underwent a staged Fowler-Stephens orchidopexy with laparoscopic clipping of the spermatic vessels as the first stage. Of these, a second-stage open orchidopexy was performed in five and a laparoscopic orchidopexy in three. Testicular atrophy occurred in two of the patients who underwent the second-stage open orchidopexy. Three one-stage Fowler-Stephens orchidopexies were performed with testicular atrophy occurring in two of these testicles. Laparoscopic orchiectomy was performed on two patients. CONCLUSIONS: Laparoscopy is a valuable tool in the diagnosis and treatment of the nonpalpable testicle. Laparoscopic orchidopexy may decrease the rate of testicular atrophy since most of these can be performed laparoscopically in one stage, thus preserving the vascular supply.

Germ cell tumor of testis in a patient with von Hippel-Lindau disease.

Germ cell testicular tumor is a previously undescribed entity in association with von Hippel-Lindau disease. This case exemplifies the variety of pathologic entities encountered in von Hippel-Lindau disease and stresses the importance of thorough evaluation of the patient, as well as careful follow-up, to ensure early detection of potentially malignant lesions.

Prenatal failure to visualize kidneys: a spectrum of disease.

OBJECTIVES: To better understand the outcomes and management of patients when there is a failure to visualize kidneys on prenatal ultrasound. METHODS: Nine thousand five hundred twelve prenatal ultrasound studies performed on 4900 patients were reviewed retrospectively for the findings of a failure to visualize kidneys. The prenatal ultrasounds, pregnancy outcomes, and postmortem studies were reviewed for each of the 10 patients identified. RESULTS: Nine of 10 patients experienced fetal death in the index pregnancy: 7 had therapeutic abortions, 1 had an intrauterine fetal demise, and 1 gave birth to a stillborn infant. One patient gave birth to a live infant with Bartter's syndrome and grossly normal kidneys, as diagnosed by ultrasound. Developmental renal anomalies were identified in only 4 of 10 cases, and only 2 patients had true bilateral renal agenesis. There was 1 case each of bilateral renal medullary cystic dysplasia and bilateral renal hypoplasia. Three cases had no renal anomalies and included 1 case each of Turner's syndrome, chronic abruption, and a cord accident. In 2 cases, postmortem examinations were not performed because of family wishes. CONCLUSIONS: Prenatal failure to visualize kidneys represents a spectrum of clinical problems not all of which are fatal. Close consultation with an experienced ultrasonographer is essential to provide informed counseling to expectant parents. Pathologic examination should be recommended when there is fetal demise and a suspicion of genitourinary anomalies. Screening of family members of the index patient and genetic counseling may be indicated.

KTP-532 laser ablation of urethral strictures.

In 1988 the KTP-532 laser was used to ablate a series of benign urethral strictures. Rather than using a single incision as in urethrotomy, strictures were treated with 360-degree contact photoradiation. Thirty-one male patients, average age 53.2 years, received thirty-seven treatments; 6 patients underwent a second laser treatment. Stricture etiology was commonly iatrogenic (32%), traumatic (16%), and postgonococcal (10%). Stricture location included mainly bulbar (49%), membranous (20%), and penile (12%) areas. The surgical technique consisted of circumferential ablation, followed by Foley catheter placement (mean, 10 days). Follow-up on 29 of 31 patients ranged from one to sixteen months (mean 9.7). Complete success occurred in 17 patients (59%) who had no further symptoms or instrumentation. Partial success was seen in 6 patients (20.5%) with symptom, but not stricture, recurrence. Six patients (20.5%) failed therapy, requiring additional surgery or regular dilations. No complications were seen. Although longer assessment is required, KTP-532 laser ablation of urethral strictures appears efficacious.

Treatment of urinary complications after total joint replacement in elderly females.

Urinary retention and the possible consequence of infection after total joint replacement is an important subject to urologists and orthopedic surgeons. A prospective, randomized study was performed in 77 elderly female patients with total joint replacement to test whether twenty-four-hour catheterization perioperatively or straight catheterization postoperatively in the recovery room might reduce the control group's incidence of postoperative urinary tract infection (9%), urinary retention (57%), and subsequent urinary catheterizations. In 16 percent of the patients who had straight catheterization in the recovery room a urinary tract infection developed postoperatively, and 65 percent of these patients required at least one more catheterization with 13 percent requiring a subsequent indwelling Foley catheter. The patients who had perioperative (immediately preoperatively and for 24 hours postoperatively) catheter drainage had a zero incidence of retention and only a 4 percent incidence of urinary tract infection. We recommend this regimen for elderly female patients undergoing total joint replacement under spinal anesthesia.

The Mitrofanoff principle. Technique and application in continent urinary diversion.

The Mitrofanoff procedure (appendicovesicostomy and creation of a low-pressure urinary reservoir) is a technically innovative way of providing patients with a form of continent diversion. The principles behind the procedure are use of a narrow, supple conduit, which, when brought out to the skin as a catheterizable stoma, will provide continence by acting as a flap-valve and attachment of the conduit to a low-pressure urine storage reservoir by an antireflux mechanism. Upper-tract contamination is prevented by an antireflux mechanism at the level of the distal ureters. Strict attention to every step of the procedure is mandatory. Emptying of the reservoir is achieved by clean intermittent catheterization. Long-term results are good in a majority of patients with careful selection and education. Life-long follow-up of patients is mandatory.

Continent vesicostomy using a catheterizable posterior bladder tube: modification of the Mitrofanoff principle.

The authors report a case of microcolon-intestinal hypoperistalsis and prune-belly syndrome, with a huge adynamic bladder and a vesicostomy. The patient was treated surgically; a continent stoma was created through which urine is drained by intermittent clean catheterization via a catheterizable conduit constructed from a tubularized strip of posterior bladder wall.

Biliovenous fistula in children after blunt liver trauma: proposal for a simple surgical treatment.

Biliovenous fistula (BVF) with subsequent leakage of bile into the venous system is a rare but serious complication of blunt liver trauma. Nine cases have been reported since 1975. Surgical therapy is indicated; however, there are still controversies as to which operative method should be applied. Based on experience gained in adult surgery, resection of the BVF together with necrotic liver tissue is also recommended in children. We describe both an organ-saving and technically simple method that was applied in two male patients, 2 and 10 years old, respectively, suffering from BVF. After debridement and tamponade of a necrotic cavity of the liver, drainage was carried out. This prevented bilious leak into the venous system. Based on the case history of these two patients, management of BVF fistula will be described. Pathophysiology of bilhemia and the drainage effect will be discussed in light of a review of the literature.

Early diagnosis of fetal bladder outlet obstruction.

Prenatal ultrasonography has facilitated early diagnosis of in utero bladder outlet obstruction. This represents one of the earliest diagnoses of prune-belly syndrome and sheds light on the natural history of the bladder outlet obstruction in the fetus.

Renal cell carcinoma in association with tuberous sclerosis in children.

Tuberous sclerosis, a genetically transmitted multisystem neurocutaneous disorder, is associated with renal lesions in 50% of cases. The expected lesions are angiomyolipomas or renal cysts; renal cell carcinoma has been encountered in adults who have tuberous sclerosis, but is very rare in children. The authors report the case of a 5-year-old girl with tuberous sclerosis for whom atypical computed tomography findings led to the diagnosis of renal carcinoma at an early age. This experience suggests that children with tuberous sclerosis may need earlier screening.

A fetal lamb model of partial urethral obstruction: experimental protocol and results.

The functional effects of bladder outlet obstruction in the developing urinary tract are well recognized in patients born with posterior urethral valves, in whom a spectrum of bladder dysfunction has been described. To better understand the changes occurring in the partially obstructed developing lower urinary tract, a fetal lamb model of partial urethral obstruction was developed. Fetal lambs at 90 days' gestation underwent surgical placement of a silver ring (ex utero) at the level of the proximal bladder neck, with concomitant ligation of the urachus. Control animals underwent urethral ligation only. The lambs were then allowed to go through normal gestation, and ewes were delivered spontaneously. The animals were studied between 2 and 7 days after birth. The postmortem examination showed that the ring was just distal to the bladder neck, around the proximal urethra. This resulted in gradual, partial occlusion of the urethra. Bladder weights, bladder wall thickness, and bladder capacity were significantly increased in the partially obstructed animals as compared with the controls. There was little or no upper tract dilatation in the obstructed group. This animal model, the first to produce gradual outflow obstruction in the fetus, provides a reproducible model of partial urethral obstruction. The model can be used to assay the biochemical and physiological changes found in the developing urinary tract of fetal lambs submitted to intravesical obstruction.

Prenatal diagnosis and the pediatric surgeon: the impact of prenatal consultation on perinatal management.

PURPOSE: Pediatric surgeons are increasingly called on by obstetrical colleagues to counsel parents about the implications of a prenatal ultrasound finding. Our understanding of the natural history of many prenatally diagnosed surgical conditions has grown significantly in recent years. Whether prenatal surgical consultation can influence perinatal course had not been investigated. METHODS: During an 21-month period, 12,865 prenatal ultrasound studies were performed on a total of 4,551 patients, and 221 prenatal surgical consultations were obtained through a newly established fetal treatment program at a tertiary care prenatal diagnostic center. To evaluate the impact of prenatal pediatric surgical consultation on perinatal course, the authors reviewed changes in management including termination of pregnancy, in utero intervention, and altered site, mode, or timing of delivery. RESULTS: Two hundred twenty-one fetuses were referred for consultation; their 234 congenital anomalies included genitourinary (36%), thoracic (16%), intraabdominal (14.5%), abdominal wall (10.6%), neurological (9%), skeletal (6%), and head and neck (2.5%) defects; 2.5% had tumors and 2.5% were twin pregnancies. Pregnancy was terminated in 9.5% of cases, because of patient request, chromosomal abnormality, or dismal prognosis. In 3.6%, the decision to terminate was changed as a result of consultation. Site of delivery was changed as a result of consultation in 37% to facilitate postnatal evaluation and initiate immediate treatment. Mode of delivery was changed in 6.8% to prevent dystocia, hemorrhage into a tumor, as in sacrococcygeal teratoma, or to provide an emergency airway, as in cervical teratoma. The timing of delivery was changed in 4.5% to avoid further damage to fetal organs in cases of obstructive uropathy, gastroschisis, sacrococcygeal teratoma with high-output failure, and hydrocephalus. Five percent (11) underwent treatment in utero for fetal hydrothorax, obstructive uropathy, twin-twin transfusion syndrome, or lymphangioma. The overall perinatal mortality rate was 2.5%. CONCLUSION: Prenatal pediatric surgical consultation may have a significant impact on the perinatal management of the fetus with a surgically correctable congenital anomaly. Providing obstetric colleagues and families with valuable insight into the surgical management of anomalies allows fetal intervention when appropriate, and delivery in an appropriate setting, by the safest mode of delivery, and at the gestational age appropriate to minimize effects of the anomaly.

The desmoplastic round cell tumor: a new solid tumor of childhood.

Three patients with a new, pathologically distinct solid tumor of childhood have been treated recently. The disease is characterized by male predominance, adolescent onset, an extensive abdominal primary tumor, and aggressive metastases to regional lymph nodes, liver, and lung. Two patients presented with vague abdominal pain and the third with testicular pain. All three noted fatigue and malaise of less than two months' duration with minimal associated weight loss. Computed tomography (CT) scans of the abdomen and chest were obtained for initial preoperative staging, and then all three underwent surgical exploration. Widespread disease was found in each case. In no instance was complete tumor extirpation possible because of extensive peritoneal spread and lymphatic and hepatic metastases. Histologically, all three tumors consisted of round blue cells with a dense desmoplastic reaction and focal rhabdoid features. Immunohistochemical markers for epithelial, neural, and muscle elements were positive. Aggressive multidrug chemotherapeutic regimens were used in each case, and all three patients are alive and well but with known residual disease. We conclude that in cases of the desmoplastic round cell tumor of childhood, CT scans underestimate the extent of disease, and exploratory laparotomy is necessary for diagnosis and appropriate staging. Surgery is usually palliative because of extensive spread. Awareness of this newly recognized aggressive solid tumor of childhood is essential to define its natural history and guide the development of effective multidisciplinary therapeutic regimens.

Surgical management of vesicoureteral reflux.

Vesicoureteral reflux (VUR) is a urologic condition in which there is a retrograde flow of urine from the bladder through the ureter back up to the upper urinary tract. The condition may be classified as primary or secondary and is more often identified in Caucasian females. The primary goal in the management of VUR is the prevention of pyelonephritis and subsequent renal scarring.

Hypospadias repair in the 1990s.

Hypospadias is a congenital anomaly in which the urethral meatus is abnormally located anywhere from the glans to the perineum. Refinements in pediatric anesthesia, surgical technique and instrumentation, and a greater understanding of the psychologic and emotional issues related to hypospadias repair have led to this procedure being performed at an earlier age. The goal of reconstruction is to bring the urethral meatus to the tip of the penis, to correct associated penile curvature if present, to create a conical-shaped glans, and to achieve cosmetically acceptable penile shaft skin coverage. The surgical technique employed varies with the penile anatomy and surgeon's preference. Complications may occur both immediately and long after the surgical procedure, and thus follow-up is necessary.

[Treatment of complex hypospadias by the Snodgrass technique]. / Traitement des hypospades complexes par la technique de Snodgrass.

PURPOSE: Successful use of the Snodgrass modification of Tiersch-Duplay urethroplasty for repair of distal hypospadias has been reported. Given the features of the repair: technical simplicity, preservation of the urethral plate, single suture line in the urethroplasty and no need for vascularized pedicle graft, we felt that the technique could be applied to the treatment of proximal hypospadias. METHODS: A retrospective review of the records of 35 patients with either midshaft or penoscrotal hypospadias who underwent a Snodgrass type of hypospadias repair was carried out. Age at surgery was 3 to 54 months (mean age: 8.4 months). No patients with significant chordee were included. All patients had indwelling urethral stents for 5 to 7 days postoperatively. Follow-up ranged from 6 months to 3 years. RESULTS: There were no immediate postoperative complications. Four patients experienced a urethrocutaneous fistula in association with meatal stenosis. After meatal dilatation, 2 of these fistulae closed spontaneously for an overall fistula rate of 5.7%. The overall cosmetic result of the glans and urethral meatus was noted to be excellent. Urinary stream was normal in all cases. CONCLUSION: Our results indicate that the Snodgrass modification of Tiersch-Duplay hypospadias repair provides satisfactory cosmetic and functional results in the treatment of proximal hypospadias with a low surgical complication rate. In young patients, it is our procedure of choice for penile and penoscrotal hypospadias without major degrees of chordee.

Primary nocturnal enuresis: current.

Primary nocturnal enuresis sometimes presents significant psychosocial problems for children and their parents. Causative factors may include maturational delay, genetic influence, difficulties in waking and decreased nighttime secretion of antidiuretic hormone. Anatomic abnormalities are usually not found, and psychologic causes are unlikely. Evaluation of enuresis usually requires no more than a complete history, a focused physical examination, and urine specific gravity and dipstick tests. Nonpharmacologic treatments include motivational therapy, behavioral conditioning and bladder-training exercises. Pharmacologic therapy includes imipramine, anticholinergic medication and desmopressin. These drugs have been used with varying degrees of success.