Psychosocial adaptation of middle childhood boys with hypospadias after genital surgery.

OBJECTIVE: To compare the psychosocial adaptation of boys with hypospadias after genital surgery to a community sample. METHODS: Boys (6 to 10 years) with a history of hypospadias repair (n = 175) were compared with a community sample (n = 333) in a postal questionnaire survey using the Child Behavior Checklist. RESULTS: Few significant differences between cases and controls emerged. Boys with hypospadias were (slightly) lower in social involvement but did not perform more poorly in school. Boys with hypospadias displayed fewer externalizing behavior problems than controls, but a significant difference in nocturnal enuresis was not detected. Level of behavior problems did not differentiate hypospadias severity subgroups, but greater surgical and hospitalization experiences were associated with increased internalizing problems. Poorer cosmetic appearance of the genitals was associated with worse school performance. CONCLUSIONS: Surgically corrected hypospadias should not be considered a risk factor for poor psychosocial adaptation in childhood, but emotional problems increase with the number of hospital-related experiences.

Focal lobular spermatogenesis and pubertal acceleration associated with ipsilateral Leydig cell hyperplasia.

Leydig cell tumors of the testis are the underlying cause in about 10% of the cases of precocious puberty in boys. Leydig cell hyperplasia is a less well-characterized cause, with an undocumented frequency. We describe a boy with precocious puberty associated with ipsilateral testicular enlargement and focal Leydig cell hyperplasia with spermatogenesis limited to the local adjacent testicular tissue.

Epididymitis in children: the circumcision factor?

PURPOSE: Recommendations for circumcision have significantly altered in the last several years. Studies have objectively established an increased risk of urinary tract infection in uncircumcised boys. We evaluated the relationship between epididymitis and circumcision status. MATERIALS AND METHODS: We studied the relationships among the circumcision status of 36 consecutive boys with epididymitis in a review of 128 with acute scrotal inflammation (group 1), circumcision status of 43 in whom the diagnosis of epididymitis at discharge home had been made elsewhere (group 2), New York State hospital discharge figures for circumcision in newborns (group 3) and the regional prevalence of circumcision in 200 consecutive pediatric emergency department patients at the same institution with nonurological diagnoses (group 4). RESULTS: New York State Department figures indicate that 70% of male newborns are discharged home with a hospital code for circumcision. Similarly an evaluation of 200 consecutive male patients without urological diagnoses younger than 18 years in the emergency department revealed that 131 (65%) were circumcised. Comparatively in groups 1 and 2 only 25 and 26% of patients, respectively, were circumcised. The statistical difference in circumcision status among the 4 groups was significant (p >0.0004). CONCLUSIONS: These data demonstrate with highly statistical significance that a relationship exists between epididymitis and the presence of a foreskin. We found that an intact foreskin is an important etiological factor in boys with epididymitis.

Cystic dysplasia of the testis: sonographic diagnosis and differentiation from testicular microlithiasis.

Cystic dysplasia of the testis is an uncommon benign cause of an intratesticular mass in children. It is often associated with renal abnormalities, which reflects the close developmental association between the kidneys and testes. The diagnosis is usually suspected based on sonographic findings. We present a case of cystic dysplasia of the testis occurring in a boy with a multicystic dysplastic kidney, in whom the sonographic findings were initially misinterpreted as testicular microlithiasis.

Intrascrotal hemangioendothelioma in infancy.

Intrascrotal and testicular masses in the pediatric population do not usually present as treatment dilemmas. Herein, we report an unusual case of an enlarging, intrascrotal capillary hemangioendothelioma in a 3-month-old male infant. Conservative management including watchful waiting in the case of purely cutaneous scrotal hemangiomas is the treatment of choice. However, scrotal lesions with a palpable testicular or scrotal mass do not lend themselves to conservative treatment and, as in this case, exploration with intraoperative evaluation and excision is warranted.

Endocrine analysis of childhood monorchism.

PURPOSE: We characterized follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels in boys with surgically documented unilateral absent testes (monorchism) to determine whether measurement of gonadotropin levels could distinguish them from boys with unilateral impalpable cryptorchidism. MATERIALS AND METHODS: Baseline serum gonadotropin levels were prospectively measured in 43 boys 2 months to 14 years old who presented with a unilateral impalpable testis that was confirmed to be absent at surgery. Control serum specimens were obtained from 63 age matched boys undergoing minor surgery with no evidence of hypospadias, or testicular, hormonal or renal diseases. Serum FSH and LH levels were drawn preoperatively and assayed by double antibody radioimmunoassay. A subgroup of 7 boys with monorchism was also evaluated following gonadotropin-releasing hormone (GnRH) stimulation and compared to age matched boys with a unilaterally impalpable testis discovered surgically. RESULTS: In the monorchism group mean plus or minus standard deviation basal FSH was 4.08 +/- 0.28 mIU/ml. and LH was 4.13 +/- 0.33 mIU/ml. In the control group mean basal FSH was 4.36 +/- 1.52 mIU/ml. and LH was 4.66 +/- 0.75 mIU/ml. No statistical difference existed between the 2 groups for mean basal gonadotropin level. While monorchid boys were more likely to have elevated FSH levels (p = 0.016), this was not true for LH (p = 0.21). Since gonadotropin levels less than 5 mIU/ml. are accepted normal values, this threshold was applied to FSH and carried a sensitivity of 23.8%, specificity 93.8%, positive predictive value 71.4% and negative predictive value 65.6%. Lower cutoff values marginally improved sensitivity but reduced specificity. Peak stimulated levels of FSH and LH following GnRH stimulation failed to distinguish between boys with 1 or 2 testes. CONCLUSIONS: Baseline FSH is more likely to be elevated in prepubertal boys with monorchism but it does not appear to be clinically useful when sensitivity and predictive value are poor. Similarly, gonadotropin level following GnRH stimulation is not sufficiently sensitive to advocate the use of hormonal measurements to diagnose prepubertal monorchism.

Prenatally diagnosed neuroblastoma.

BACKGROUND: Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years, and there are now a few reviews based on up to 21 cases. The purpose of this article is to review the clinical and biologic features of prenatally diagnosed neuroblastoma based on a review of 55 cases. METHODS: A review was conducted of 3 cases seen at the study institution and 52 other cases reported thus far in the literature. RESULTS: Prenatal diagnosis was made usually after 32 weeks of gestation. Approximately 93% of the tumors were adrenal in origin, and 44% of these were cystic. Thirty-seven patients (67%) had Stage I disease, 12 (22%) had Stage IV-S disease, and only 3 (5%) had Stage IV disease. The DNA index was favorable (> 1) in 14 of 16 patients studied. None of these 16 patients studied had amplification of the N-myc oncogene. Catecholamines were elevated in only 33% of the patients. The liver was the most common site of dissemination, which was observed in 25% of patients; bone involvement was not observed in any patient. Ultrasonography failed to detect existing hepatic metastasis in three patients. Primary surgical resection was performed in 47 patients (85%). Chemotherapy was given to five patients and radiotherapy to three. Of the 50 patients for whom follow-up information was available, 45 (90%) were alive at a range of 2-120 months from diagnosis. CONCLUSIONS: Prenatally diagnosed neuroblastomas are predominantly adrenal in origin and frequently cystic. The liver is the most common site of dissemination and bone involvement is notably absent. The vast majority of these infants have a favorable stage of disease (I, II, and IV-S) and favorable biologic features, and consequently have an excellent prognosis. Although surgery alone is curative for most patients, a period of observation may avoid surgery in some individuals who may achieve spontaneous regression.

Intraoperative spermatic venography reconsidered.

We review our most recent experience with varicocelectomy and post-ligation venography in 58 adolescents as followup to our previously reported series. A single injection venogram confirmed the completeness of varicocele ligation in 82% of cases while 2 or more injections were required in the remainder. Venography was not performed in 8 cases because of technical difficulties in 5 or surgeon choice in 3. The recurrence rate was 8.6% and was not statistically significant whether or not venography was performed (p = 0.86). Although intraoperative spermatic venography is technically simple and safe, in our series its efficacy in diminishing the recurrence rate after varicocelectomy was unproved.

Gender development in boys born with hypospadias.

Fetal testicular androgens in several mammalian species are responsible for the sexual differentiation of both the genitalia and the brain, the latter effect being related to behavioral sex-dimorphisms. Because prenatal endocrine abnormalities can be inferred from genital defects, studies of individuals born with anomalies potentially elucidate the contribution of androgens to the development of gender-related variation in human behavior. This study concerns the gender-role behavior of middle childhood boys (ages 6-10 years; n = 175) born with hypospadias, an androgen-related genital anomaly. Parents completed standardized gender behavior questionnaires in a postal survey. Hypospadias subjects did not show consistent differences from a community control group (n = 333) in feminine behavior, but significant, small, increases in masculine behavior were found. Severity of the hypospadias was unrelated to gender-role behavior. A number of surgery-related hospitalizations, however, were correlated with increased gender-atypical behavior. It is concluded that the hypoandrogenization associated with hypospadias does not interfere with the development of gender-typical masculine behavior.

Endoscopic management of bladder calculi following augmentation cystoplasty.

OBJECTIVES: We sought to evaluate the effectiveness and utility of an endoscopic approach to calculi that develop in the bladders of children following augmentation cystoplasty. In addition, we aimed to determine the indications for open vesicolithotomy. METHODS: We reviewed our experience between 1981 and 1993 with 26 children who formed bladder calculi following augmentation cystoplasty. Data were retrieved retrospectively with respect to management approach and outcome. RESULTS: Nineteen cases were managed cystoscopically using simple extraction and/or electrohydraulic lithotripsy; 3 cases required open vesicolithotomy and four calculi passed spontaneously. Complete stone extraction was achieved after a single endoscopic treatment in every case approached in this fashion. Every patient resumed preoperative voiding patterns and there were no infections, strictures, or other complications. Calculi reformed in 4 patients and were successfully managed endoscopically. CONCLUSIONS: An endoscopic approach to bladder calculi is a safe and effective method of managing this increasingly prevalent problem in children following augmentation cystoplasty even in the presence of a reconstructed bladder neck. Open vesicolithotomy should be reserved for the very large stone burden.

Optimal renal preservation with timely percutaneous intervention: a changing concept in the management of blunt renal trauma in children in the 1990s.

OBJECTIVE: To report the results of treatment of 132 children with blunt renal trauma and indicate our treatment recommendations. RESULTS: Between 1971 and 1991 132 children presented with blunt renal trauma. Grade I injuries (renal contusion) were seen in 97 children, grade II injuries (renal laceration) in 32 and grade III (shattered kidney and pedicle injury) in three. Twenty-six children with grade II injuries were treated non-surgically. Urinomas developed in three, which were drained percutaneously with complete resolution. Prior to 1985 six patients with grade II injuries underwent total or partial nephrectomy for major renal lacerations. Repair of shattered kidneys resulted in significant morbidity. Conservative management of major blunt injuries with judicious percutaneous intervention resulted in no renal loss. CONCLUSION: We believe that conservative therapy with timely percutaneous drainage of urinomas in children with major blunt renal injury, other than shattered kidney or pedicle injuries, optimizes renal preservation and minimizes secondary complications.

Urolithiasis in children following augmentation cystoplasty.

Until recently urolithiasis in children following augmentation cystoplasty was an infrequently noted problem. We examined our 10-year experience and found urinary calculi to form in 52% of children and young adults undergoing augmentation cystoplasty. Calculi formed at a median interval of 24.5 months after surgery, predominantly in the lower tract. Urinary tract infection was a statistically significant risk factor, while the use of absorbable staples, intestinal mucus and hypocitraturia were also implicated. Calculus composition was primarily a mixture of apatite, struvite and ammonium urate. Bladder calculi were effectively managed endoscopically in the majority of cases without complication. Upper tract calculi presented an endourological challenge.

Feminizing genital reconstruction for male pseudohermaphroditism.

The ambiguous genital structures of Male Pseudo-hermaphrodites may be reconstructed either in a masculinizing or feminizing manner. This decision is made attempting to maximize body image and function. Early assignment of an appropriate sex of rearing is important. Feminizing genitoplasty for Male Pseudo-hermaphroditism may be done at any age, once accurate diagnosis of the underlying condition is reached. Clitoroplasty, labioplasty, and exteriorization vaginoplasty may be done simultaneously in selected cases where an adequate vagina is already present, with excellent functional and cosmetic results anticipated.

Significance of accessory ductal structures in hernia sacs.

The finding of ductal structures resembling a vas deferens during pathological examination of a hernia sac specimen has significant medical and legal implications. A method of distinguishing these accessory structures from an inadvertently transected vas has been lacking and is needed. Between July 1989 and January 1990 we examined 147 hernia sacs from 105 consecutive prepubertal and adolescent boys to determine the incidence and salient histological features distinguishing these ductal structures from a true vas deferens. Luminal diameters of the ductal structures were compared with published normal age-related established vas deferens diameters and with those measured during hernia repair in 10 of our youngest patients. Among the 147 specimens 6 hernia sacs (4.1%) contained ductal structures. We found that the mean ductal diameter (0.263 mm.) was significantly smaller than that of a normal vas deferens (0.69 to 1.5 mm.). Furthermore, the surrounding mantle of tissue of these ductal structures lacked muscle tissue when studied with Masson trichrome stain. We conclude that duct diameter and trichrome staining are simple ways of differentiating these structures from a true vas deferens.

Vesical manifestations of chronic granulomatous disease in children. Its relation to eosinophilic cystitis.

Two children with chronic granulomatous disease involving the bladder are reported. Their clinical course was characterized by lower urinary tract symptoms, decreased vesical capacity, unilateral ureterovesical junction obstruction, and an intense eosinophilic infiltrate on biopsy specimens in each. Chronic granulomatous disease and eosinophilic cystitis appear to have remarkably similar clinical manifestations. A review of urinary tract involvement in chronic granulomatous disease and its comparison with eosinophilic cystitis is presented.