William G. Reiner

An Anatomical Approach to the Surgical Management of the Dorsal Vein and Santorini’s Plexus During Radical Retropubic Surgery

AbstractAn illustrated description of the anatomy of the deep dorsal vein and Santorini’s plexus is presented. The deep dorsal vein of the penis passes through the urogenital diaphragm and trifurcates into a superficial branch and 2 lateral plexuses. There are 3 regional valvular systems in the deep dorsal venous system. An operative technique is presented for the management of this venous system in radical retropubic operations that minimizes blood loss and improves exposure

Comparison of spontaneous and experimentally induced canine prostatic hyperplasia.

Spontaneous prostatic hyperplasia in the beagle appears to progress with age from a glandular to a cystic histological appearance. Prostatic hyperplasia can be induced in young beagles with intact testes by treatment for 4 mo with either dihydrotestosterone or 5 alpha-androstane-3 alpha, 17 beta-diol, alone, or with either of these steroids in combination with 17 beta-estradiol. In contrast, the induction of prostatic hyperplasia in young castrated beagles, in which the gland had been allowed to involute for 1 mo, requires the administration of both 17 beta-estradiol and either 5 alpha-androstane-3 alpha, 17 beta-diol or dihydrotestosterone. Testosterone and 17 beta-estradiol, either singly or in combination, did not produce the hyperplastic condition in intact or castrated beagles. The experimentally induced prostatic hyperplasia is identical in pathology to the glandular hyperplasia that occurs naturally in the aging dog with intact testes. However, cystic hyperplasia was not produced by any of the treatments tested in young animals.

Summary of Consensus Statement on Intersex Disorders and Their Management

Advances in understanding of genetic control of sexual determination and differentiation, improvements in diagnostic testing and surgical genital repair, and the persistent controversies inherent to clinical management were all compelling factors that led to the organization of an international consensus conference. The goals were to acknowledge and discuss the more controversial issues in intersex management, provide management guidelines for intersex patients, and identify and prioritize questions that need additional investigation. This is a summary statement. Advances in molecular genetic causes of abnormal sexual development and heightened awareness of the ethical and patient-advocacy issues mandate reexamination of existing nomenclature for patients with intersex.1 Terminology such as “pseudohermaphroditism” is controversial, potentially pejorative to patients,2 and inherently confusing. Therefore, the term “disorders of sex development” (DSD) is proposed to indicate congenital conditions with atypical development of chromosomal, gonadal, or anatomic sex. Additional rationale for new classification is the need for modern categorization to integrate the modern molecular genetic aspects, to maximize precision when applying definitions and diagnostic labels,3 and to meet the need for psychologically sensitive yet descriptive medical terminology. Nomenclature should be flexible enough to incorporate new information, robust enough to maintain a consistent framework, use descriptive terms, reflect genetic etiology, accommodate phenotypic variation spectrum, and be useful for clinicians, scientists, patients, and families. Hence, we propose a new classification (see “Consensus Statement on Management of Intersex Disorders”4 in this months issue of Pediatrics Electronic Edition ). Three traditionally conceptualized domains of psychosexual development are gender identity (ones self-representation [ie, male or female]), gender role (sexually dimorphic behaviors within the general population, such as toy preferences, aggression, and spatial ability), and sexual orientation (direction[s] of erotic interest). Gender dissatisfaction denotes unhappiness with assigned sex and may result in gender self-reassignment. Psychosexual developmental factors relate to parental psychopathology, parent-child … Address correspondence to Peter A. Lee, MD, PhD, Department of Pediatrics, MC-H085, Penn State College of Medicine, Milton S. Hershey Medical Center, Box 850, 500 University Dr, Hershey, PA 17033-0850. E-mail: plee{at}psu.edu

The Outcome of Patients with Classic Bladder Exstrophy in Adult Life

PURPOSE We determined the outcome of 20 older adult bladder exstrophy patients regarding urinary continence, sexual function, fertility and psychosocial integration. MATERIALS AND METHODS A total of 16 men and 4 women completed an anonymous questionnaire and the charts were reviewed for medical history. RESULTS Of 9 patients who void spontaneously 6 are dry for 4 hours and 3 for 2 hours. Among the remaining 11 patients 5 are dry on clean intermittent catheterization, 3 who had undergone uterosigmoidostomy are dry for more than 4 hours and 3 had an incontinent stoma. Four men and 2 women are married, including 2 men and 1 women who had a total of 7 children. Ten of 16 men (63%) reported that they ejaculate a few cubic centimeters in volume, 3 ejaculate only a few drops and 3 have no ejaculation (2 of whom underwent cystectomy). Semen analyzed in 4 patients (3 with azoospermia and 1 with oligospermia) and average volume of ejaculate was 0.4 cc (range 0.2 to 1). All women reported regular and normal menstrual periods. A total of 15 patients experienced normal erections that were described as satisfactory by 8 and not satisfactory by 6 due to a small penis, with dorsal chordee in 1. Of the 16 men 12 (75%) experienced satisfactory orgasms while 10 had participated in sexual intercourse with complete partner satisfaction in 9. Half of the men and all women describe intimate relationships as serious and longterm. Of the 20 patients 15 (75%) achieved a high level of education. CONCLUSIONS Bladder exstrophy patients generally achieve good results but the fertility of most men is in doubt.

Psychosexual dysfunction in males with genital anomalies: late adolescence, Tanner stages IV to VI.

OBJECTIVE To assess psychosexual function in adolescent males with genital anomalies. METHOD Fourteen consecutive males with bladder exstrophy-epispadias, 14 to 19 years old, Tanner stages IV to VI, were assessed along with their parents, using a developmental questionnaire, Hollingshead socioeconomic status rating, Child Behavior Checklist, Youth Self-Report, semistructured psychiatric interview, detailed sexual history, and 5 written, open-ended questions. RESULTS All subjects showed psychosexual dysfunction in terms of genital satisfaction and genital touching; only 2 had ever undressed in front of anyone; only 2 had ever masturbated and only after age 16; 8 had few friends and only 5 considered any girls as friends; all expressed heterosexuality but only 4 had dated, 1 at age 17 and 2 after age 18; only the two 19-year-olds had experienced sexual intercourse, at the age of 19. All had an anxiety disorder. Half had experienced a major depressive disorder. CONCLUSIONS Psychosexual dysfunction and anxiety were universal and chronic in these males with genital anomalies, leading to social and sexual developmental impairment. Half had a mood disorder. Implications for adulthood as well as for children with other genital anomalies are unclear but deserve further study. Males with genital anomalies should be evaluated for psychosexual developmental impairment.

Case Study: Sex Reassignment in a Teenage Girl

The etiology of gender identity is explored through the case history of a teenager with intersex raised without stigmatization as a girl but who declared himself male at age 14 years. Psychological ramifications of such complex medical anomalies as ambiguous genitalia demand a role for child psychiatry.

CLOACAL EXSTROPHY-IMPROVING THE QUALITY OF LIFE: THE JOHNS HOPKINS EXPERIENCE

PURPOSE Exstrophy of the cloaca is a multisystem anomaly involving the gastrointestinal, nervous, musculoskeletal and genitourinary tracts which should be managed with a multidisciplinary approach. Improvement in management has led to survival for the majority of infants, and the focus has shifted to improvement in quality of life. The experience with management of cloacal exstrophy at a large center is evaluated. MATERIALS AND METHODS Demographic data as well as functional results of management of the multiple anomalies in 37 patients with cloacal exstrophy were evaluated. Surgical reconstruction was aimed at providing the best functional and cosmetic results. RESULTS Average patient age at review was 13.6 years. Most patients (32 of 37) had undergone an initial attempt at bladder closure from birth to greater than 24 months of age. Colostomy was performed when possible and if initial ileostomy was performed, the bowel was augmented later with the hindgut segment. When the hindgut segment was not used for bowel reconstruction, it was preserved for bladder augmentation or genital reconstruction. While urinary continence was achievable in many children, it was usually after augmentation and/or continent diversion. CONCLUSIONS Improvements in perinatal management have increased survival in cloacal exstrophy. Therefore, the focus of reconstruction has shifted to reduction in the numbers of incontinent stomas, assistance with ambulation and improved cosmesis. All of these goals are achievable using a multidisciplinary approach to the management of this complex anomaly.

Nephrogenic adenoma: clinical features and therapeutic considerations.

Nephrogenic adenoma is a benign metaplastic lesion that usually responds to endoscopic treatment. Although occasionally it has been present simultaneously with another malignancy there has been no evidence that a nephrogenic adenoma has ever transformed into a carcinoma. The symptoms of a nephrogenic adenoma can be severe but these lesions can be treated with transurethral surgery. The lesions can occur throughout the bladder and in the urethra. They usually are associated with trauma to the urothelium. Postoperative followup is needed because these lesions tend to have a symptomatic recurrence. An increased awareness of nephrogenic adenoma by urologists and pathologists may lead to its more frequent diagnosis.

Long-term survival after hormonal therapy for stage D prostatic cancer.

Hormonal therapy occasionally produces long-term survival of men with metastatic carcinoma of the prostate. To identify which patients will benefit for long intervals a retrospective analysis was done on 56 men with stage D carcinoma of the prostate treated between 1963 and 1968. Of this group 5 patients lived longer than 10 years. We were unable to identify clinical or pathologic characteristics that would have indicated which patients would have done well.

Total and Partial Urogenital Mobilization: Focus on Urinary Continence

PURPOSE Total and partial urogenital mobilization procedures are the most common contemporary vaginoplasty surgeries for patients with congenital adrenal hyperplasia, urogenital sinus and cloacal anomalies. There is controversy regarding the urinary continence outcomes of these procedures. We reviewed the urinary continence outcomes of children who underwent total or partial urogenital mobilization at our institution and reviewed the literature to determine the continence rates of these procedures. MATERIALS AND METHODS We retrospectively reviewed 25 patients who underwent total or partial urogenital mobilization with a focus on postoperative continence status. Continence was defined as parental report of full toilet training with no accidents during the day and rare accidents (fewer than 2 per month) at night after age 3 years. RESULTS A total of 14 congenital adrenal hyperplasia, 5 urogenital sinus and 6 cloacal anomaly cases were managed by total (18) or partial (7) urogenital mobilization procedures with a mean followup of 4.41 years (range 0.21 to 12.1). In our cohort 21 of 22 patients (95.5%) were continent by age 3 years and there were no urinary complications. A total of 111 patients were identified in the literature with congenital adrenal hyperplasia or urogenital sinus, with 107 in 7 studies being continent (96.4%) by age 3 to 4 years. In 4 studies 32 patients were identified with cloacal anomalies who underwent total or partial urogenital mobilization, of whom 28 (87.5%) were continent by age 3 to 4 years. CONCLUSIONS There was no significant difference between total and partial urogenital mobilization procedures regarding postoperative urinary continence in our cohort and the literature. The urinary continence rate was 96% in the congenital adrenal hyperplasia/urogenital sinus group and 89.5% in the cloacal group.