Partial urogenital mobilization in cloacal malformation: is it a viable option?

INTRODUCTION: Total Urogenital Mobilization (TUM) has been the standard surgical approach for the urogenital complex in Cloacal Malformations (CM) since its inception in 1997. Partial Urogenital Mobilization (PUM) in CM remains an under-utilized or under-reported option. The main anatomical difference between TUM and PUM is the division of the pubo-urethral ligaments. OBJECTIVE: We explored the feasibility of PUM in a select subset of our patients with CM and report early outcomes. STUDY DESIGN: We retrospectively reviewed prospectively collected data of all our CM patients who had primary reconstruction at our centre from 2012 to 2020. We included in our review the patients who underwent PUM. Mullerian abnormalities, spinal cord involvement, common channel length (CC), urethral length (UL), surgical reconstruction, and outcomes including urinary continence, recurrent UTI, ultrasound and preoperative DMSA/MAG3, cystovaginoscopy post-reconstruction, and post-void residuals were noted. RESULTS: Fifty-three patients had primary reconstruction, and of these, eleven had a common channel less than 3 cm. Of the eleven, only one underwent TUM. In the PUM group, two underwent filum untethering (20%). Mullerian duplication was noted in 5 patients (50%). The median CC length = 1.6 cm (range = 1.5cm-2.7 cm), and median UL = 1.5 cm (range = 1.5cm-2.5 cm). Follow-up ranged from 9 to 134months (median = 63months). Post-reconstruction all had a separate urethral and vaginal opening on examination and cysto-vaginoscopy. The continence outcomes are summarized in Fig.1. DISCUSSION: Although TUM is the most common solution for the urogenital complex in CM, a subset would be suitable for PUM, and this option is under-utilized or under-reported in literature. We presume that many who had TUM probably only needed a PUM, and therefore could report better outcomes from a bladder function aspect. It is important to differentiate the two, and outcomes should be appropriately categorized. Our default approach is a PUM in all CM with less than 3 cm common channel. Only the lateral and posterior aspects of the urogenital complex are mobilized and if the urethra did not reach a satisfactory level for easy intermittent catheterization, then we proceed to a TUM dividing the pubo-urethral ligaments. PUM avoids the potential complications related to dividing the pubo-urethral ligament in TUM. It may also avoid the need for CIC which is encountered in patients who undergo TUM. CONCLUSION: PUM is a viable alternative in cloacal malformations with good outcomes in those with a common channel under 3 cm. This of course requires appropriate patient selection and accurate categorization of interventions to understand the true outcomes.

Radical resection of lumbosacral lipomas in children: the Great Ormond Street Hospital experience.

PURPOSE: In 2009, Pang described a radical resection technique for congenital lumbosacral lipomas, with lower long-term symptomatic re-tethering rates compared with partial resections and conservative management, and low surgical morbidity. We adopted this technique in 2011, and aim to describe our first results. METHODS: In this monocentric retrospective audit, we included dorsal, transitional, chaotic, and caudal-type lumbosacral lipomas. Exclusion criteria were previously operated lipomas, pure filar lipomas, and concomitant major congenital anatomical urogenital/gastrointestinal abnormalities. Neuro-uro-orthopaedic status at presentation and at three months, one year and last postoperative follow-up, intraoperative electrophysiology, and extent of resection were collected. RESULTS: From January 2011 to September 2019, 91 patients were operated (median age 2y2m; 63 transitional; 14 caudal; 8 dorsal; 6 chaotic). Preoperatively, 67% were symptomatic. Preoperative and one-year postoperative rates of impaired ambulation (44% to 43%), hypoesthesia (8% to 5%), urodynamic/uroradiological abnormalities (49% to 37%), and foot/ankle deformities (8% to 5%) were comparable, whilst pain improved (25% to 5%) but catheterisation rates increased (21% to 36%). 23/92 (25%) suffered wound-related complications. 2/91 (2%) developed symptomatic re-tethering requiring second surgery. Mean cord/sac ratio was 0.47. 43% had > 20 mm3 residual fat, which improved with increasing surgical experience. CONCLUSION: Radical lipoma resection, guided by intraoperative neuromonitoring, with reconstruction of the neural placode and expansion duraplasty is technically feasible and results in low rates of late deterioration and re-tethering. Lipoma-type and pre-operative status are important outcome predictors. Operative risks are not insignificant. Future studies need to determine appropriate selection criteria for surgery.

Does conus morphology have implications for outcome in lumbosacral lipoma?

OBJECTIVE: Lumbosacral lipomas (LSL) of the 'dorsal' type have been associated with more favourable outcomes compared with other conus region lipomas. We hypothesised that integrity of the conus on MRI underpins the improved prognosis in this subgroup of LSL patients. METHODS: The definition of 'dorsal lipomas' included lipomas with attachment to the conus, but where the conus could be delineated on MRI (Morota type 1) as reported by Morota et al. (J Neurosurg Pediatr 19:428-439, 2017). Additional inclusion criteria included asymptomatic status at presentation, age >3 years at follow-up, and neurological and urological evaluation at presentation and at last follow-up. Lipoma extent and conus level were recorded. Outcome measures were the need for untethering surgery and neuro-urological status at last follow-up. Urological outcomes were defined by continence and efficacy of bladder emptying. RESULTS: Twenty-six children were included (median age 8.7 years). Conus level was low (at or below L2) in 92%. Nine required untethering surgery: 5 prophylactic, 4 because of clinical deterioration. Twenty-five children were continent at last follow-up, one had stress incontinence, and none required catheterisation. One had persisting ankle weakness after surgery requiring orthotic support. CONCLUSIONS: In LSL of the conus, visualisation of the conus on MRI is associated with good urological and motor outcomes. The integrity of the conus appears to be a more important prognostic factor than anatomical level. An observational approach to this group of LSL patients does not appear to compromise outcomes. These findings support a selective approach to untethering surgery.

Outcomes of urinary tract abnormalities diagnosed by the routine third trimester scan.

OBJECTIVE: The aim of this study is to determine the incidence of congenital abnormalities of the kidneys and urinary tract (CAKUT) detected for the first time in an unselected population undergoing a routine third trimester scan between 30-34 week's gestation. METHODS: This was a retrospective analysis of 8562 routine third trimester ultrasound scans during which the fetal anatomy was evaluated, and, any structural abnormalities detected, recorded onto a fetal database which was subsequently analysed for CAKUT. All postnatal records of antenatally diagnosed CAKUT were obtained and analysed for diagnosis and management. RESULTS: There were 26 cases of urological abnormalities detected for the first time in the third trimester. The most frequent abnormality was unilateral renal pelvis dilatation (73%). Postnatal ultrasound confirmed abnormalities in 19 (73%) newborns, with two (8%) resolving antenatally and four (15%) postnatally. The overall incidence of new CAKUT detected by the third trimester scan was 0.22% (19/8562) with a male to female ratio of 1:1.6. Four patients required surgery, two received cystoscopic injection of Deflux with circumcision, one received cystoscopic valve ablation and one patient received a staged hypospadias repair. CONCLUSION: Routine third trimester scanning is already performed in many countries with proposed benefits primarily directed towards the monitoring of fetal growth and late pregnancy malpresentation. For healthcare systems that still utilize two routine scans, debate is ongoing as to the value of introducing a routine third trimester scan. The ability to detect additional and potentially missed CAKUT is a further benefit, which in isolation is likely of too small an impact to merit implementation. However, the combination of fetal structural assessment, growth velocity monitoring and fetal presentation evaluation presents a strong case for inclusion in an antenatal screening program. The findings of this study highlight the importance of a detailed fetal structural evaluation at each antenatal ultrasound scan.

Isolated bilateral simplex ureteric ectopia: Bladder capacity as an indicator of continence outcome.

INTRODUCTION: Isolated bilateral simplex ectopic ureters (BSEUs) are rare but pose a therapeutic challenge: ureteric reimplantation alone does not accomplish continence in all. Identifying the patients needing additional procedures for continence early could prevent multiple operations. OBJECTIVE: Potential preoperative indicators for postoperative continence are explored in eight BSEU girls without cloacal, anorectal, or spinal anomalies. STUDY DESIGN: With institutional approval, all patients with BSEU between 1985 and 2012 were retrospectively reviewed. Cystoscopy determined the site of ureteric ectopia (6 of 16 at the bladder neck [BN], 5 of 16 below the BN, and 5 of 16 in the distal urethra). Bladders were assessed by a combination of ultrasound, urodynamics, micturating cystourethrogram, cystoscopic, and intraoperative observations. Expected bladder capacity for age (EBCA) was calculated by 30 ml + (30 ml × age in years) or 38 ml + (2.5 ml × age in months) for children greater or less than 2 years, respectively. Continence outcomes were appraised at a minimum of 4 years. The small number of patients precludes credible statistical analysis and therefore raw data are presented. RESULTS: Patients underwent cross-trigonal ureteric reimplantation at 1-5.5 years, in five without BN surgery and in three with a Young-Dees-Leadbetter BN tightening. Of those without BN surgery at reimplantation, four achieved satisfactory continence for their age, but one has had multiple procedures culminating in BN closure, ileocystoplasty, and Mitrofanoff. Among the BN-tightening group, one was in nappies at 4 years, one had residual stress incontinence after two further BN injections, and one proceeded to artificial urinary sphincter after two BN injections. Five patients had significant renal impairment. DISCUSSION: Patients with satisfactory continence after reimplantation alone and those needing further procedures tended to differ in their preoperative observations of bladder capacity and apparent BN competence. This study suggests preoperative observations of an empty bladder on serial ultrasound and/or a wide-open BN with small or even moderate bladder capacity at cystoscopy to indicate the need for BN surgery. In contrast, children with bladder filling to at least 30% of expected bladder capacity for age on preoperative ultrasound or apposition of the BN at cystoscopy may achieve satisfactory continence after ureteric reimplantation alone. Bladder capacity as an indicator of BN competence can also be correlated to continence outcomes in previously published series. Polyuria associated with renal impairment can exacerbate the challenge for continence. CONCLUSION: Preoperative bladder capacity appears to be an indicator of inherent BN function and a thorough assessment of the urinary tract by cystoscopy, ultrasound, micturating cystourethrogram, and functional imaging may guide the surgeon on the need for BN surgery at the time of ureteric reimplantation. Where continence remains elusive, patients should be counselled that a further BN injection is occasionally of value although more significant BN procedures are required for most.

What is the risk of urinary tract infection in children with antenatally presenting dilating vesico-ureteric reflux?

INTRODUCTION: The incidence of recurrent urinary tract infection (UTI) in children with primary vesico-ureteric reflux (VUR) presenting symptomatically is well documented. The risk of UTI in asymptomatic primary VUR diagnosed on investigation of antenatal hydronephrosis (ANH) is less clear. Paradoxically, several previous studies have suggested a lower risk (1-25%). We ascertain the incidence of UTI amongst antenatally-presenting primary VUR and explore risk factors. STUDY DESIGN: All patients <16 years managed for primary VUR between 1997 and 2013 were retrospectively reviewed. Patients were identified by searching 'VUR, vesicoureteric reflux' and 'vesico' in the clinical database. Sex, follow up, antibiotic prophylaxis, age at UTI, grade of VUR, radioisotope imaging findings (CRN-congenital reflux nephropathy, NRD-new renal defects), evidence of bladder dysfunction, surgical intervention and resolution were recorded. UTI diagnosis was based on positive urine culture with symptoms including fever. SPSS statistical package and Pearson's Chi-squared test were used to explore significance. RESULTS: Of 308 patients with primary VUR aged <16 years treated, 242 were diagnosed following presentation with UTI. The remaining 66 (21%) were initially asymptomatic, and VUR was diagnosed on investigation of ANH. All were given prophylaxis from birth. Six months to 16years (median 6years) follow-up was available for 54 (42 males, 12 females). All but two patients had grade III-V VUR (96%), bilaterally in 41 (76%). CRN was evident in 30 (56%; all male) and bladder dysfunction in 12 (22%; 10 males). Twenty-eight patients (52%) developed a UTI. The risk of UTI was 58% in girls, 33% in boys without CRN and 57% in boys with CRN (p = 0.17). Bladder dysfunction was a significant risk factor for UTI (p = 0.03). All 8 (15%; 7 males) with NRD had had a UTI. A single UTI appeared responsible for the majority of NRD (6/8; 75%). UTI occurred in 6/27 (22%) boys after circumcision compared to 17/25 (68%) prior/without circumcision (p < 0.05). CONCLUSIONS: The incidence of UTI in VUR detected after presentation with ANH was 52%. CRN and bladder dysfunction were risk factors for developing a UTI. Circumcision appears to significantly reduce the risk of infection. Antenatal presentation of primary VUR does not carry a reduced risk of UTI. A single UTI, in half before the age of six months, seemed responsible for the majority of NRD. In boys, the highest risk of UTI is in the first few months of infancy, despite antibiotic prophylaxis, and other interventions, particularly circumcision, should therefore be considered as early as possible.

Periurethral and vulval condylomata acuminata: an unusual juvenile venereal disease in a 3-year-old girl.

We present a case of giant condyloma acuminatum (CA) in a 3-year-old girl with rapidly growing vulval and periurethral lesions, which was investigated thoroughly and successfully treated with excision biopsy. Genital warts in the form of giant alarming vestibular, vulval and periurethral lesions of CA are very rare in children. There is need for a multidisciplinary approach with potential sociomedicolegal implications. Surgical excision is safe, effective and provides an opportunity to assess the extent of the lesion and tissue for accurate diagnosis. Genital warts in the form of giant alarming vestibular, vulval and periurethral lesions of CA are very rare in children. Indications for surgical treatment include large, recurrent or refractory lesions, as well as the need for histological identification and acquiring tissue for immunotherapy when necessary. The strategy of an extended follow-up is recommended so as to ensure identification of any risk situations.

Testicular outcome following laparoscopic second stage Fowler-Stephens orchidopexy.

OBJECTIVE: To assess outcome after laparoscopic second-stage Fowler-Stephens orchidopexy (L2(nd)FSO). PATIENTS AND METHODS: Retrospective review of 94 children (aged 0.75-16 years, median 2.75 years), who underwent L2(nd)FSO for 113 intra-abdominal testes between January 2000 and May 2009: 75 unilateral, 19 bilateral (11 synchronous; 8 metachronous). Follow-up (range 3 months-10.9 years, median 2.1 years) was available for 88 children (102 testes: 71 unilateral, 31 bilateral). RESULTS: Testicular atrophy occurred in 9 out of 102 (8.8%), including 8 out of 71 (11.3%) unilateral and 1 out of 31 (3.2%) bilateral intra-abdominal testes (multivariate analysis: p = 0.59). Testicular ascent ensued in 9 out of 102 (8.8%), comprising four (5.6%) unilateral and five (16.1%) bilateral testicles (multivariate analysis: p = 0.11). Of the 18 bilateral testes brought to the scrotum synchronously none atrophied and four (22.2%) ascended, compared to one (7.7%) atrophy and one (7.7%) ascent among the 13 testes brought to the scrotum on separate occasions (Fisher exact test: p = 0.42 and p = 0.37, respectively). Mobilization of the testis through the conjoint tendon tended towards less ascent (multivariate analysis p = 0.08) but similar atrophy (p = 0.56) compared to mobilization through the deep-ring/inguinal canal. Logistical regression analysis identified no other patient or surgical factors influencing outcome. CONCLUSIONS: This is the largest series of L2(nd)FSO to date. A successful outcome is recorded in 85 out of 102 (83.3%) testicles. Atrophy occurred in 8.8% and ascent in 8.8%.

Interventions for impaired bladders in paediatric renal transplant recipients with lower urinary tract dysfunction.

Dysfunctional bladders in paediatric patients were thought to be a contraindication for renal transplantation, but advances in surgical techniques have meant that surgical correction can allow safe transplantation. This study compares the outcomes of renal transplantation for different interventions, and the timing of such interventions, in relation to transplantation. We identified all paediatric renal transplant recipients with LUTD that received intervention for their impaired bladders at two hospitals between 2002 and 2010. Outcome measures included patient and graft survival, perioperative complications, UTI incidence, acute rejection episodes and serum creatinine levels. A total of 288 allografts were transplanted, 77 were in 75 children with LUTD, of which 46 received intervention. Patient survival was 100% in the intervention group and 97% in the nonintervention group (P = 0.815). Death-censored graft survival was 96% and 100% respectively (P = 0.688). In the groups receiving intervention pretransplant or post-transplant, graft survival rates were 95% and 100% respectively (P = 0.476). The follow-up serum creatinine levels were higher in the pretransplant intervention group (P < 0.001). Interventions for dysfunctional bladders can be performed safely in paediatric renal transplant recipients. The mode of intervention and timing of intervention, in relation to transplant, do not influence outcomes if guided by careful assessment and investigation.

Asymptomatic lumbosacral lipomas--a natural history study.

BACKGROUND: Inevitable deterioration due to mechanical tethering is perceived as the natural history for complex congenital spinal lipomas of the conus medullaris region, even if asymptomatic at presentation. The conventional wisdom that prophylactic surgical untethering improves outcome has been challenged recently [1, 2]. This study examines the natural history of asymptomatic un-operated children with lumbosacral lipomas (LSL) and investigates whether predictive factors herald deterioration. METHODOLOGY: Over the past decade, children presenting with complex LSL to a single clinician at Great Ormond Street Hospital (GOSH), London, UK have undergone a thorough assessment focusing on neurological and urological evaluation and MRI of the lumbosacral spine. For children deemed to be asymptomatic, conservative management has been adopted with close periodic surveillance of neurological and urological function, thus avoiding untethering surgery unless symptomatic deterioration occurs. A retrospective review identified this cohort of children asymptomatic of their LSL and their progress closely recorded. DISCUSSION: This study suggests that the natural history of this subgroup of dysraphic patients may be more benign than hitherto considered. Conservative management with adoption of a novel surveillance policy and timely intervention only in the presence of symptomatic deterioration resulted in 71% of this series remaining clinically asymptomatic at mean follow up period of 5.9 years (range, 1.0-19.3 years). At 10 years, the cumulative risk of deterioration determined by the Kaplan-Meier method was 40%. Children aged<2 years, female, with presence of a transitional type of LSL and associated syrinx were independently associated with a higher risk of deterioration.

The predictive value of a repeat micturating cystourethrogram for remnant leaflets after primary endoscopic ablation of posterior urethral valves.

OBJECTIVE: We routinely perform a cystourethroscopy 3 months after initial ablation of posterior urethral valves. The aim of this study was to determine the predictive value of the urethral appearance on preoperative micturating cystourethrogram (MCUG) for further valve resection at check cystoscopy. PATIENTS AND METHODS: We retrospectively reviewed 31 consecutive boys (aged 4-18 months) who underwent check cystoscopy and repeat MCUG between 2006 and 2008. RESULTS: Repeat MCUG suggested remnant valves in 10, but no residual leaflets were identified cystoscopically in 4. In 20 boys, the valves appeared completely ablated on MCUG but valve leaflets received further resection in 10. One study was undiagnostic. Residual valves were resected in 83% (5/6) where valves and urethral dilatation were noted on MCUG. Where MCUG suggested either valves or persistent dilatation alone, further resection occurred in 40% (4/10). Remnant leaflets were also present in half of those (7/14) in whom the repeat MCUG had shown complete ablation and resolved/reduced posterior urethral dilatation. CONCLUSIONS: The positive predictive value of valve leaflets and/or posterior urethral dilatation on repeat MCUG for subsequent resection of valve remnants was 56%; the negative predictive value was 50%. We found repeat MCUG alone imprecise in excluding residual valve tissue and recommend check cystoscopy in all.

Challenges facing renal transplantation in pediatric patients with lower urinary tract dysfunction.

In pediatric patients with end-stage renal disease, renal transplantation is the established therapy of choice. The commonest cause is a congenital abnormality of the kidneys and urinary tract, often associated with lower urinary tract dysfunction (LUTD). Historically, such patients were denied transplantation, but it is now widely accepted that transplant outcomes comparable with the non-LUTD population are achievable. Nonetheless, the optimal management of pediatric end-stage renal disease patients with LUTD is unclear, with no guidelines to distinguish between the need for conservative management or surgical reconstruction of the lower urinary tract. Furthermore, the most appropriate surgical procedure and optimal timing of surgical intervention is far from clear. In this review, we outline common conditions that produce LUTD in children; discuss difficulties encountered in assessing the need for surgical treatment; provide an overview of the surgical procedures available; and consider the evidence for and against surgical intervention before, during, and after renal transplantation.

Renal transplantation or bladder augmentation first? A comparison of complications and outcomes in children.

OBJECTIVE: To identify whether the order of performing transplant and bladder reconstruction operations in children who need both operations affects outcome of either operation. PATIENTS AND METHODS: A retrospective case note review was performed of children identified from our database, who had undergone both renal transplantation and bladder augmentation between 1990 and 2005. RESULTS: In all, 18 renal transplants (eight live-related) were performed in 16 children with 10 transplants done after bladder augmentation and eight transplants done before augmentation. The median age at transplantation was 7.5 years and at augmentation was 7.0 years. The median interval between the operations was 33.5 months and the median follow-up was 58.4 months after transplantation. Outcomes were compared between the two groups of patients: those who received their transplantation before bladder augmentation, and those who were transplanted after bladder augmentation. There was no difference between these groups in: the pre- transplant estimated glomerular filtration rate, inpatient stay after transplantation or after augmentation, and incidence of urinary tract infection in the 3 months after renal transplantation or after bladder augmentation. There was no statistical difference in renal allograft loss with one graft failure in the group who were augmented first, and four graft failures in the group who were transplanted first. However, it is of note that the single graft failure in the patient augmented first was due to renal artery thrombosis on the first day related to a double arterial anastomosis, whilst in the other group, three of the graft failures were in transplants that had initially been drained by ureterostomy. Three patients in the group transplanted first developed significant ureteric pathology, of which one developed graft failure. CONCLUSION: Bladder reconstruction can be performed safely before transplantation; it does not increase complications and might better protect the renal graft and specifically the transplant ureter.

A review of urodynamic evaluation in children and its role in the management of boys with posterior urethral valves.

Posterior urethral valves are the commonest cause of lower urinary tract outflow obstruction in male infants with an estimated incidence of 1:5000 male infants and 1:25,000 live births. Despite treatment with fulguration of the obstructing valves, bladder function is abnormal in up to 70% of older children and adolescents. Bladder dysfunction causes morbidity e.g. urinary incontinence and has been implicated in the late deterioration of renal function in this population. A poor understanding and inappropriate management of bladder dysfunction can result in unnecessary morbidity, which can handicap a child for life. Any method that measures function or dysfunction of the lower urinary tract constitutes a urodynamics investigation. Broadly, the investigations can be classified into simple or noninvasive urodynamics and invasive urodynamics. The objective of urodynamics assessments in children is to reproduce the patient's complaints or symptoms. Video urodynamics can provide additional information that may contribute to a further understanding of the problem under investigation. Urodynamics provides a useful tool to test the efficacy of treatment as well as determine any refinements necessary to improve the outcome of such treatment.