Philip G. Ransley

Preliminary report: the antegrade continence enema

The principles of antegrade colonic washout and the Mitrofanoff non-refluxing catheterisable channel were combined to produce a continent catheterisable colonic stoma. The intention was that antegrade washouts delivered by this route would produce complete colonic emptying and thereby prevent soiling. The procedure has been successfully carried out in five patients with intractable faecal incontinence.

Medical versus surgical treatment in children with severe bilateral vesicoureteric reflux and bilateral nephropathy: a randomised trial

BACKGROUNDnNephropathy associated with vesicoureteric reflux (VUR) and urinary tract infection can result in end-stage renal failure, hypertension, or both. Whether long-term VUR contributes to these outcomes is unknown. We compared, in a randomised trial, medical with surgical management of children with bilateral severe VUR and bilateral nephropathy.nnnMETHODSnWe stratified by age and glomerular filtration rate (GFR) 25 boys and 27 girls aged 1-12 years and randomly assigned them to medical or surgical management. At enrolment and 4 years follow-up we estimated GFR from the plasma clearance of 51Cr-labelled edetic acid (EDTA), and did intravenous urography. We also did a metastable 99mTc-labelled dimercaptosuccinic acid (DMSA) assay and contrast cystography. The change in GFR at 4 years, expressed as a percentage change between enrolment and 4 years, was available for 26 of 27 patients in the medical and 24 of 25 in the surgical group. We assessed GFR in 48 patients 10 years after enrolment.nnnFINDINGSnMean GFR at enrolment was 72.4 mL/min per 1.73 m(2) (SD 24.1) in the medical and 71.7 mL/min per 1.73 m(2) (22.6) in the surgical group. The mean percentage change in GFR at 4 years was 2.4% (SE 4.5) versus 4.7% (5.0) in the medical and surgical groups, respectively. The difference in change in GFR at 4 years between the two groups was not significant (7.1%, 95% CI 6.4% to 20.6%).nnnINTERPRETATIONnOur data do not lend support to the view that the outcome for renal function is improved by surgical correction of VUR in children with bilateral disease.

Preliminary ligation of the gonadal vessels prior to orchidopexy for the intra-abdominal testicle

SummaryA staged technique for orchidopexy for the intra-abdominal testis is described. At a preliminary operation the testicular vessels are ligated in continuity without any mobilization of the testis itself. After an interval to allow enhancement of the blood supply via the vessels accompanying the vas deferens, orchidopexy is performed by the Fowler-Stephens technique.

Self‐assessment of genital anatomy, sexual sensitivity and function in women: implications for genitoplasty

Authors from the USA and UK evaluate female patients’ perception of genital anatomy, and estimate the implications of this for genitoplasty. There are several important observations by the people who completed the questionnaire which should lead surgeons who perform genitoplasty to remember the functional aspects as well as the anatomical appearance after this operation.

The functional sequelae of sacrococcygeal teratoma

Twenty-seven patients with sacrococcygeal teratoma were followed for a mean period of 5 years (range, 2 to 12 years). Eleven (41%) had some form of functional impairment. Fecal and/or urinary incontinence was present in nine children and two had weakness of the lower limbs secondary to sciatic nerve palsy. Tumors with large intrapelvic extensions requiring an abdominoperineal approach for resection were associated with a higher incidence of functional sequelae (67%). It is recommended that patients with sacrococcygeal teratoma (particularly those with a presacral extension) be closely monitored for functional deficits so that early treatment can be instituted.

Long-Term Renal Function in the Posterior Urethral Valves, Unilateral Reflux and Renal Dysplasia Syndrome

PURPOSEnThe syndrome of posterior urethral valves, persistent unilateral reflux and renal dysplasia (VURD) is said to be protective of the contralateral nonrefluxing kidney and the outcome for renal function is reported to be excellent. We tested this hypothesis in our patients by replicating previous studies but with longer followup and glomerular filtration rate data.nnnMATERIALS AND METHODSnWe retrospectively reviewed the records of 183 boys presenting with posterior urethral valves between 1980 and 1989, including 12 who underwent nephrectomy for ipsilateral nonfunction and fulfilled all criteria for the VURD syndrome. Mean age at the most recent followup was 8.5 years. Serial serum creatinine levels and glomerular filtration rates were analyzed and compared to age matched normal values.nnnRESULTSnHistological evaluation revealed dysplasia in all kidneys, confirming the VURD syndrome. Followup plasma creatinine was normal 67% of the patients during year 2 of life, 50% between ages 4 and 5 years, and only 30% between ages 8 and 10 years. Glomerular filtration rate was within the normal range in 25% of boys tested in year 2 of life, and between ages 5 and 8 years.nnnCONCLUSIONSnOur data do not support the protective effect of the VURD syndrome on long-term renal function. All patients with posterior urethral valves require diligent long-term followup.

Congenital mesoblastic nephroma, renin production, and hypertension

Between 1964 and 1987, 12 patients with mesoblastic nephroma were treated. The BP was measured preoperatively in five patients, four of whom were hypertensive. Following nephrectomy, the BP returned to normal. Plasma renin levels were measured in one patient; they were markedly elevated pre-operatively, but returned to normal following tumour excision. Immunoreactive renin staining, using a polyclonal antibody to human renin, was performed in the 12 patients. Staining was positive in ten patients. The most intense staining was noted in the areas of cortex entrapped within the tumour, and in perivascular spaces not associated with entrapped cortex. These findings suggest that hypertension secondary to increased tumour associated renin production is a feature of congenital mesoblastic nephroma.

Timing and nature of reconstructive surgery for disorders of sex development - introduction.

The ideal timing and nature of surgical reconstruction in individuals with Disorders of Sex Development (DSD) is highly controversial. Despite the increasing number of publications on this topic, evidence-based recommendations still cannot be made. However it is generally accepted that optimal care for DSD requires an experienced multidisciplinary team. This means that surgical decisions are now made within the context of a multidisciplinary team and all members of the team - and not just specialist surgeons - may be called upon to discuss choices for surgery with patients and parents. To do this well, every clinician in the team should have an understanding of the range of techniques available for genital surgery, the risks and benefits of procedures and the controversies surrounding timing of surgery. The aim of this paper is to give an overview of the variety of surgical procedures in current use and in what situation a particular technique would be indicated. The short-term risks and benefits are described and where available long-term outcome data is discussed. To date, discussions surrounding genital surgery have been led primarily by surgeons. Some non-surgical clinicians have expressed unease about decision making in genital surgery but have felt ill equipped to comment on an area with which they are unfamiliar. This review gives a detailed explanation of current surgical practice offered in a specialized center for DSD and such information should facilitate a more balanced discussion.

TRANSVERSE RETUBULARIZED ILEUM: EARLY CLINICAL EXPERIENCE WITH A NEW SECOND LINE MITROFANOFF TUBE

PURPOSEnTransverse retubularization of small ileal segments has been described as a new time and labor saving variation of the Mitrofanoff principle in a dog model with good functional results. We report our initial clinical experience with this technique.nnnMATERIALS AND METHODSnFrom May 1996 through January 1997 a new technique of channel formation for intermittent catheterization was applied in 9 children (1 to 16 years old) and 7 adults (18 to 56) with various abnormalities of the lower urinary tract. The new method was used in primary reconstruction of the lower urinary tract and in revision procedures. An ileal segment 2 cm. long was excised. The bowel wall was opened longitudinally about 1 cm. from the mesentery. The resulting rectangle was retubularized over a 14F catheter in transverse direction. The longer portion of the tube was implanted submucosally into the native bladder, the augmented bladder or an intestinal reservoir. The shorter portion was used to form the stoma. In 4 patients we created a double tube.nnnRESULTSnOf the patients 13 (81%) are completely continent day and night with easy catheterization postoperatively. In 2 cases of tunnel failure continuous leakage required reimplantation of the intact ileal tube to achieve continence. Minor leakage with bladder fullness in an 11-year-old boy could be obviated by adjusting the interval of catheterization.nnnCONCLUSIONSnWith the advantage of constant availability, minimal loss of bowel, relative simplicity (no mesentery interfering with implantation, high tube mobility), minimized risk of stone formation (no staples), reliable continence (no leak point) and easy catheterization (longitudinal folds), this straightforward technique is an excellent second choice use of the Mitrofanoff principle.

Surgery in disorders of sex development (DSD) with a gender issue: If (why), when, and how?

Ten years after the consensus meeting on disorders of sex development (DSD), genital surgery continues to raise questions and criticisms concerning its indications, its technical aspects, timing and evaluation. This standpoint details each distinct situation and its possible management in 5 main groups of DSD patients with atypical genitalia: the 46,XX DSD group (congenital adrenal hyperplasia); the heterogeneous 46,XY DSD group (gonadal dysgenesis, disorders of steroidogenesis, target tissues impairments …); gonosomic mosaicisms (45,X/46,XY patients); ovo-testicular DSD; and non-hormonal/non chromosomal DSD. Questions are summarized for each DSD group with the support of literature and the feed-back of several world experts. Given the complexity and heterogeneity of presentation there is no consensus regarding the indications, the timing, the procedure nor the evaluation of outcome of DSD surgery. There are, however, some issues on which most experts would agree: 1) The need for identifying centres of expertise with a multidisciplinary approach; 2) A conservative management of the gonads in complete androgen insensitivity syndrome at least until puberty although some studies expressed concerns about the heightened tumour risk in this group; 3) To avoid vaginal dilatation in children after surgical reconstruction; 4) To keep asymptomatic mullerian remnants during childhood; 5) To remove confirmed streak gonads when Y material is present; 6) It is likely that 46,XY cloacal exstrophy, aphallia and severe micropenis would do best raised as male although this is based on limited outcome data. There is general acknowledgement among experts that timing, the choice of the individual and irreversibility of surgical procedures are sources of concerns. There is, however, little evidence provided regarding the impact of non-treated DSD during childhood for the individual development, the parents, society and the risk of stigmatization. The low level of evidence should lead to design collaborative prospective studies involving all parties and using consensual protocols of evaluation.