P.G. Ransley

The clinical application of the malone antegrade colonic enema

Continent, catheterizable enterostomies (Malone stomas) have been formed in 25 patients. A reversed appendicocecostomy was used for the majority, although experience has led to modifications of this procedure. Antegrade enemas are regularly administered via these stomas in the treatment of fecal incontinence and intractable constipation. There have been only two important surgical complications, and the technique has proved to be highly successful. Follow-up of the patients has shown that individual requirements can be met with variations in the administration of the enemas.

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 Yang–Monti ileovesicostomy: a problematic channel?

Objective To compare the differences in the quality of Mitrofanoff channels created using appendix and re‐tubularized small bowel (the Yang–Monti ileovesicostomy).

The relationship between early renal status, and the resolution of vesico-ureteric reflux and bladder function at 16 months.

Objective To examine, in infants presenting with vesico‐ureteric reflux (VUR), the relationship between the presence of initial renal abnormalities with the outcome of VUR and bladder function at 16 months of age.

Combined Mitrofanoff and Antegrade Continence Enema Procedures for Urinary and Fecal Incontinence

PURPOSE Fecal soiling or intractable constipation frequently occurs in association with urinary incontinence in children undergoing major reconstructive urological operations. To treat double incontinence or the combination of wetting and severe constipation, we constructed a Mitrofanoff conduit and a channel for antegrade continence enemas in 18 patients between 1989 and 1995. We review the underlying pathological conditions, various surgical techniques and outcomes of these operations. MATERIALS AND METHODS Underlying abnormalities mainly included spinal lesions, bladder exstrophy, imperforate anus and various cloacal anomalies. Patient age ranged from 2 to 18 years (average 8.4). In 13 patients both procedures were done simultaneously. The appendix was used to construct the antegrade continence enema channel in 8 cases and the Mitrofanoff channel in 5. It was long enough to be divided and used for both procedures in 2 cases but it was missing or unsuitable in 3. Alternative antegrade continence enema conduits were cecal flap in 7 patients and ileum in 1, while the ureter, ileum and detrusor tube were used to establish Mitrofanoff channels in 5, 5 and 1, respectively. Stomas were constructed according to the V-flap or V. Z. Q. technique and situated in close proximity in the right lower abdominal quadrant in 13 cases. RESULTS Convalescence was uneventful except for 1 abscess near an antegrade continence enema stoma. Ten patients needed dilation or minor revisions due to difficulty in catheterizing the antegrade continence enema (5), Mitrofanoff (3) or both conduits (2). Subsequently 3 patients underwent repeat operations for reconstruction of 2 antegrade continence enema channels (cecal flap and ileum) and 1 detrusor tube Mitrofanoff channel. Currently 15 patients are dry on regular clean intermittent catheterization using 10 to 12F catheters. Outcomes of the antegrade continence enema channels are satisfactory in 15 patients who are clean or rarely soil. Failure occurred in 1 patients with severe constipation necessitating colostomy and 2 (1 noncompliant who stopped catheterizing regularly) in whom the channels subsequently closed. CONCLUSIONS Synchronous construction of antegrade continence enema and Mitrofanoff channels is successful in the majority of doubly incontinent patients. Selection of patients with high motivation is important to obtain satisfactory results.

The role of ureter in the creation of Mitrofanoff channels in children

PURPOSE Since 1980 numerous variations of the Mitrofanoff principle have been described. We report on 22 children in whom a ureteral Mitrofanoff channel was created. MATERIALS AND METHODS Between 1986 and 1995 a ureteral Mitrofanoff channel was constructed as a catheterizable conduit in 22 children 2 to 15 years old (average age 6.5) with various abnormalities of the lower urinary tract, mainly exstrophy and neurogenic bladder. Indications included unavailability or unsuitability of the appendix, preference for appendix as a catheterizable colonic stoma for antegrade washouts or concomitant removal of a nonfunctioning kidney, leaving the ureter available for use. Surgical technique was based on the principles of appendicovesicostomy and in 9 cases the ureteral Mitrofanoff channel was reimplanted. RESULTS Followup ranged from 1 to 72 months (average 30.5). Complications included stenosis of the conduit that caused difficult catheterization in 3 patients, necessitating dilation or minor revision in 2 and complete replacement by appendix in 1. Urinary leakage from the Mitrofanoff channel in 5 patients was treated with polydimethylsiloxane injection or oxybutinin. In 1 patient the channel was reimplanted, since the catheter struck the bladder neck during catheterization and caused severe pain. CONCLUSIONS Results of the ureteral Mitrofanoff channel seem somewhat less satisfactory than those of appendicovesicostomy but they remain acceptable and even comparable, strongly supporting its use in certain circumstances.

Upper pole heminephrectomy: is complete ureterectomy necessary?

Objective To determine the re‐operation rate on the distal ureter after upper pole heminephrectomy with incomplete ureterectomy.

The Evolution of Penile Reconstruction in Epispadias Repair: A Report of 180 Cases

From 1978 to 1993, 180 boys with epispadias (85) and the exstrophy/epispadias complex (95) underwent epispadias reconstruction at our institution. The 180 patients were divided into 4 groups: group 1--2-stage epispadias reconstruction with dissection of the corporo (30 patients), group 2--pedicled preputial tube urethroplasty either alone or with a lyophilized human dural patch to the corpora for correction of penile deformity (35), group 3--pedicled preputial tube urethroplasty and corporeal rotation (40), and group 4--a modified Cantwell epispadias repair incorporating complete tubularized urethroplasty, cavernocavernostomy and corporeal rotation. In 46 patients from groups 1 to 3 the primary outcome was not considered satisfactory and they underwent radical penile revision with the modified Cantwell technique. Followup ranges from 1 to 15 years (mean 6 years). We conclude that a good cosmetic result can be achieved in almost all cases using the modified Cantwell technique as a primary procedure in experienced hands. This technique has a low complication rate and can be used as a salvage procedure following previous unsuccessful epispadias reconstruction using other techniques.

Enterocystoplasty in childhood: a second look at the effect on growth

To re‐evaluate the assumption that enterocystoplasty in children has a detrimental effect on linear growth (which is almost exclusively based upon a chance finding in a retrospective study 10 years ago) in a larger cohort and with a longer follow‐up.

Endoscopic Treatment of Urinary Incontinence in Children With Primary Epispadias

Subjects and methods The study included 83 infants and children (51 boys and 32 girls, aged 3 days to 12 years) with no neurological and lower urinary tract pathology but who had undergone or were about to undergo surgery for upper urinary tract or other pathology. They were evaluated using slow-filling cystometry, with simultaneous electromyography recorded using surface electrodes on the perineum. The voiding variables were compared among groups categorized by age, sex and body weight. Results In boys and girls, respectively, the mean (SD) post-void residual urine volume (PVR) was 6.3 (3.9) and 5.4 (4.8) mL. the maximum detrusor pressure duringvoiding was 66.1 (13.1) and 56.6 (14.7) cmH,O and the maximum voiding pressure was 73.9 (16.6) and 62.7 (16.2) cmH,O. There was no significant difference in these variables between the sexes or between infants and children (P >0.05). Detrusor instability (DI) was apparent in nine of 83 (10.8%) infants and children and occurred in the late filling phase. Bladder capacity increased with age and body weight (from 30 mL in neonates to 350 mL in 12-year-old children), and mean (SD) bladder compliance increased with age, from 3.6 (0.5) mUcmH,O in infants to 13.3 (3.0) mL/cmH,O in older children, a t a filling rate of 5-7 mumin. Conclusions In these infants and children with no apparent voiding symptoms, most bladders were stable. DI could occur in the late filling phase of cystometry, voiding was nearly complete, the PVR being usually <10 mL, and bladder capacity increased with age and body weight.