Do adult patients with congenital colorectal conditions know their diagnosis?

PURPOSE: Limited research exists about the knowledge that adult patients have about their congenital colorectal diagnosis. METHODS: This was an IRB approved, prospective study of patients in the Adult Colorectal Research Registry who completed surveys between October 2019 and March 2022. Surveys were administered through REDCap after patients consented to being contacted for research purposes. Patients provided demographic data, which was linked to surgical records, and the diagnoses provided by patients were compared with diagnoses recorded by the original surgeons. RESULTS: One hundred and thirty-one questionnaires were collected, 115 patients had anorectal malformations (ARM) and 16 had Hirschsprung disease (HD). Seven patients who had ARM were unaware that they had an ARM or HD. The type of ARM recorded by the surgeon was unavailable for comparison with the patient's reported diagnosis in four cases. Of the 111 remaining patients with ARM, only 32 of them (29%) knew what their own type of anomaly was. Female patients recalled their diagnosis more often than male patients (42.4% vs 13.5%). All 16 participants with HD correctly identified their diagnosis severity as HD with or without total colonic aganglionosis. CONCLUSION: The results of this study demonstrate patient's limited understanding of their type of ARM and highlight the urgent need to enhance communication and education strategies, such as issuing patients with medical diagnosis identification cards. It is critical for clinicians to better communicate with patients to ensure that they and their relatives truly understand their precise diagnosis. Adequately informed patients can better advocate for themselves, adhere to treatments and precautionary recommendations and navigate the complexities of transitional care. Consequently they can more effectively manage their lifelong complications.

Laparoscopic and robotic pyeloplasty as minimally invasive alternatives to the open approach for the treatment of uretero-pelvic junction obstruction in infants: a multi-institutional comparison of outcomes and learning curves.

BACKGROUND: Since the development of minimally invasive surgery (MIS), laparoscopic and robotic approaches have been widely adopted. However, little has been published detailing the learning curve of MIS, especially in infants. OBJECTIVE: To quantify the learning curve of laparoscopic (LP) and robot-assisted laparoscopic pyeloplasty (RAL-P) for treatment of uretero-pelvic junction obstruction (UPJO) in infants evidenced by number of cases, operative time, success and complications. PATIENTS AND METHODS: Between 2009 and 2017, we retrospectively reviewed pyeloplasty cases for treatment of UPJO in infants at three academic institutions. The primary outcome was success. Secondary outcomes were UPJO recurrence, complications, and operative time as a surrogate of skill acquisition. Continuous variables were analyzed by t test, Welch-test, and one-way ANOVA. Non-continuous variables were analyzed by Chi-squared test or Fisher's exact test. Learning curves (LC) were studied by r-to-z transformation and CUSUM. RESULTS: Thirty-nine OP, 26 LP, and 39 RAL-P had mean operative times (OT) of 106, 121, and 151 min, respectively. LCs showed plateau in OT after 18 and 13 cases for LP and RAL-P, respectively. RAL-P showed a second phase of further improvements after 37 cases. At 16 months follow-up, there were similar rates of success and complications between the three groups. CONCLUSIONS: Despite different duration of learning phases, proficiency was achieved in both LP and RAL-P as evidenced by stabilization of operative time and similar success rates and complications to OP. Before and after achievement of proficiency, LP and RAL-P can be safely learned and implemented for treatment of UPJO in infants.

Prospective study of enhanced recovery after surgery protocol in children undergoing reconstructive operations.

BACKGROUND: Enhanced recovery after surgery (ERAS) protocol is a set of peri-operative strategies to increase speed of recovery. ERAS is well established in adults but has not been well studied in children. OBJECTIVE: The purpose of the current study was to establish the safety and efficacy of an ERAS protocol in pediatric urology patients undergoing reconstructive operations. It was hypothesized that ERAS would reduce length of stay and decrease complications when compared with historical controls. STUDY DESIGN: Institutional Review Board approval was obtained to prospectively enroll patients aged <18 years if they had undergone urologic reconstruction that included a bowel anastomosis. ERAS included: no bowel preparation, administration of pre-operative oral carbohydrate liquid, avoidance of opioids, regional anesthesia, laparoscopy when feasible, no postoperative nasogastric tube, early feeding, and early removal of intravenous fluids (IVF). Recent (2009-2014) historical controls were propensity matched in a 2:1 ratio on age, sex, ventriculoperitoneal shunt status and whether the patient was undergoing bladder augmentation. Outcomes were protocol adherence, length of stay (LOS), emergency department (ED) visits, re-admission within 30 days, re-operations and adverse events occurring within 90 days of surgery. RESULTS: A total of 26 historical and 13 ERAS patients were included. Median ages were 10.4 (IQR 8.0-12.4) and 9.9 years (IQR 9.1-11), respectively (P = 0.94) (see Summary Table). There were no significant between-group differences in prior abdominal surgery (38% vs 62%), rate of augmentation (88% vs 92%) or primary diagnosis of spina bifida (both 62%). ERAS significantly improved use of pre-operative liquid load (P < 0.001), avoidance of opioids (P = 0.046), early discontinuation of IVF (P < 0.001), and early feeding (P < 0.001). Protocol adherence improved from 8/16 (IQR 4-9) historically to 12/16 (IQR 11-12) after implementation of ERAS. LOS decreased from 8 days to 5.7 days (P = 0.520). Complications of any grade per patient decreased from 2.1 to 1.3 (OR 0.71, 95% CI 0.51-0.97). There were fewer complications per patient across all grades with ERAS. No differences were seen in emergency department (ED) visits, re-admissions and re-operations. DISCUSSION: Implementation improved consistency of care delivered. Tenets of ERAS that appeared to drive improvements included maintenance of euvolemia through avoidance of excess fluids, multimodal analgesia, and early feeding. CONCLUSION: ERAS decreased length of stay and 90-day complications after pediatric reconstructive surgery without increased re-admissions, re-operations or ED visits. A multicenter study will be required to confirm the potential benefits of adopting ERAS.

Systematic review of bladder cancer outcomes in patients with spina bifida.

BACKGROUND: In patients with congenital bladder anomalies, bladder augmentation is used as a last resort to reduce intravesical pressure, but concerns about malignant transformation in augmented patients were first raised in the 1980s. The best evidence to date indicates that augmentation does not appear to increase the risk of bladder cancer in spina bifida patients. To date, oncologic outcomes from patients with spina bifida with and without augmentation have only been available in small case reports. OBJECTIVE: To systematically evaluate factors in myelomeningocele patients with bladder cancer, including bladder augmentation, that contribute to overall survival (OS). STUDY DESIGN: A systematic review using PubMed was conducted by cross referencing terms 'myelomeningocele,' 'cystoplasty,' 'bladder cancer' and respective synonyms according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Inclusion criteria were studies with patients with an underlying diagnosis of myelomeningocele and bladder cancer with data on age, stage, and mortality status. Studies were excluded for spinal cord injury, history of tuberculosis or schistosomiasis, or prior ureterosigmoidostomy. RESULTS: Fifty-two patients were identified from 28 studies with a median age at bladder cancer diagnosis of 41 years (range 13-73); 37 (71%) presented with stage III or IV bladder cancer. Overall survival at 1 year and 2 years was 48.5% and 31.5%, respectively. Overall survival was different between those with and without augmentation (P = 0.009) by log-rank analysis. No between-group differences in OS were seen based on age, management with indwelling catheter, diversion with ileal conduit or being on a surveillance program. Only stage remained a significant predictor of OS on multivariate analysis (HR 2.011, 95% CI 1.063-3.804, P = 0.032). Secondary analysis was performed after removing patients with gastric augmentation (n = 8), and no difference in OS was seen between patients with (n = 8) and without augmentation (n = 36, P = 0.112). Of augmented patients, latency to development of bladder cancer was variable (Summary Figure). DISCUSSION: Bladder cancer is a deadly diagnosis in patients with congenital bladder anomalies like spina bifida, and while overall prevalence of the two conditions occurring together is low, bladder cancer will go on to affect 2-4% of spina bifida patients. The present study examined overall survival, and further characterized outcomes in these patients. Presence of a bladder augment did not appear to worsen overall survival. CONCLUSIONS: Patients with myelomeningocele who developed bladder cancer had aggressive disease. Augmentation did not worsen OS, based on cases reported in the literature. Risk of bladder cancer should be discussed with all myelomeningocele patients.

Genitourinary and gastrointestinal co-morbidities in children: The role of neural circuits in regulation of visceral function.

OBJECTIVE: Pediatric lower urinary tract dysfunction (LUTD) is a common problem in childhood. Lower urinary tract symptoms in children include overactive bladder, voiding postponement, stress incontinence, giggle incontinence, and dysfunctional voiding. Gastrointestinal co-morbidities, including constipation or fecal incontinence, are commonly associated with lower urinary tract (LUT) symptoms in children, often reaching 22-34%. This review summarized the potential mechanisms underlying functional lower urinary and gastrointestinal co-morbidities in children. It also covered the current understanding of clinical pathophysiology in the pediatric population, anatomy and embryological development of the pelvic organs, role of developing neural circuits in regulation of functional co-morbidities, and relevant translational animal models. MATERIALS AND METHODS: This was a non-systematic review of the published literature, which summarized the available clinical and translational studies on functional urologic and gastrointestinal co-morbidities in children, as well as neural mechanisms underlying pelvic organ 'cross-talk' and 'cross-sensitization'. RESULTS: Co-morbidity of pediatric lower urinary and gastrointestinal dysfunctions could be explained by multiple factors, including a shared developmental origin, close anatomical proximity, and pelvic organ 'cross-talk'. Daily physiological activity and viscero-visceral reflexes between the lower gastrointestinal and urinary tracts are controlled by both autonomic and central nervous systems, suggesting the dominant modulatory role of the neural pathways. Recent studies have provided evidence that altered sensation in the bladder and dysfunctional voiding can be triggered by pathological changes in neighboring pelvic organs due to a phenomenon known as pelvic organ 'cross-sensitization'. Cross-sensitization between pelvic organs is thought to be mainly coordinated by convergent neurons that receive dual afferent inputs from discrete pelvic organs. Investigation of functional changes in nerve fibers and neurons sets certain limits in conducting appropriate research in humans, making the use of animal models necessary to uncover the underlying mechanisms and for the development of novel therapeutic approaches for long-term symptomatic treatment of LUTD in the pediatric population. CONCLUSION: Pediatric LUTD is often complicated by gastrointestinal co-morbidities; however, the mechanisms linking bladder and bowel dysfunctions are not well understood. Clinical studies have suggested that therapeutic modulation of one system may improve the other system's function. To better manage children with LUTD, the interplay between the two systems, and how co-morbid GI and voiding dysfunctions can be more specifically targeted in pediatric clinics need to be understood.

Study of clitoral hood anatomy in the pediatric population.

INTRODUCTION AND AIMS: Pediatric female genitalia size and morphology have not previously been well described. The aims of this study were to create a standard reference for the anterior vulval structures, and examine the relationship between the clitoral hood and labia minora. METHOD: Female patients between 0 and 16 years of age, and with normal external genitalia had the following measurements taken: length of clitoral hood, length of sides of clitoral hood, clitoral diameter, apex of clitoral hood to base of pubic symphysis, apex of clitoral hood to urethral orifice, distance of clitoral hood to labia majora, and length and depth of labia minora. Qualitative descriptors of the clitoral hood and labia were recorded. Patients were grouped into age ranges for analysis: 0-3 years, 4-8 years, 9-12 years, and 13-16 years. RESULTS: Fifty-eight girls were examined. There was a linear relationship between age and genital structure size. In the majority of patients, the labia minora converged under the clitoral glans, separate to the clitoral hood. Four shapes of clitoral hood were observed: horseshoe, trumpet, coffee bean, and tent. DISCUSSION: This study observed great variation in size and morphology of pediatric female genitalia, which is in keeping with other studies. The study was limited by ability to recruit older patients to the study (girls aged 11-16 years) and, therefore, there were lower numbers in this age category. However, the trends that were observed were present in both younger and older girls. CONCLUSION: External genitalia size and morphology are varied in the pediatric female population. The clitoral hood and labia minora were observed to be distinctly separate structures. This study provides a reference for surgeons undertaking genital reconstruction procedures. The study demonstrated that the clitoral hood and labia minora are anatomically distinct structures.

Inter-rater reliability of postnatal ultrasound interpretation in infants with congenital hydronephrosis.

PURPOSE: The most common measurements of hydronephrosis are the anterior-posterior (AP) diameter and the Society for Fetal Urology (SFU) grading systems. To date, the inter-rater reliability (IRR) of these measures has not been compared in the postnatal period. The objectives of this study were to compare the IRR of the AP diameter and the SFU grading system in infants and to determine whether ultrasound findings other than pelvicalyceal dilation are associated with higher SFU grades. METHODS: Initial postnatal ultrasounds of infants seen from February 1, 2011, to January 31, 2012, with a primary diagnosis of congenital hydronephrosis were included for review. Ultrasound images were de-identified and reviewed by four pediatric urologists. IRR was calculated using the intraclass correlation (ICC) measure. A paired t test was used to compare ICCs. Associations between SFU grade and other ultrasound findings were tested using Chi-square or Fisher's exact tests. RESULTS: A total of 112 kidneys in 56 patients were reviewed. IRR of the SFU grading system was high (right kidney ICC = 0.83, left kidney ICC = 0.85); however, IRR of AP diameter measurement was higher (right kidney ICC = 00.97, left kidney ICC = 0.98; p < 0.001). Renal asymmetry (p < 0.001), echogenicity (p < 0.001), and parenchymal thinning (p < 0.001) were significantly associated with SFU grade 4 hydronephrosis on bivariable and multivariable analysis. CONCLUSIONS: The SFU grading system is associated with excellent IRR, although the AP diameter appears to have higher IRR. Physicians may consider ultrasound findings that are not explicitly included in the SFU system when assigning hydronephrosis grade, which may lead to variability in use of this classification system.

Continuous local anesthetic infusion for children with spina bifida undergoing major reconstruction of the lower urinary tract.

OBJECTIVE: While many options for postoperative analgesia are available to the general patient population, choices are limited for individuals with spinal dysraphism. We hypothesized that the use of continuous local anesthetic infusion following major reconstruction of the lower urinary tract in children with spina bifida would significantly decrease need for opiate use, while maintaining adequate pain control. MATERIALS AND METHODS: Children with spina bifida who underwent major reconstruction of the lower urinary tract at Children's Hospital Colorado were identified from January, 2003 through January, 2013 were identified. In addition to enterocycstoplasty, procedures included Mitrofanoff or Monti creation, bladder neck reconstruction, and Malone antegrade continence enema. Patients who had local anesthetic infusion catheters placed in the incision were compared to patients without catheters. Opioid consumption was calculated by conversion of any opiates into IV morphine (mg/kg) on postoperative days (POD) 0-3. Pain was assessed by mean and maximum FLACC scores on POD 0-2. Use of antiemetic medications and wound related complications were recorded as secondary metrics. Patients with other etiologies for neurogenic bladder and bowel were excluded. Patients whose pain was assessed by other assessment scales were excluded. Chi-squared analysis was used for nominal variables, students t-test was used for analysis of continuous variables. P values <0.05 were considered significant. RESULTS: 36 myelomeningocele patients who underwent primary enterocystoplasty met the inclusion criteria. All surgeries were open procedures. 24 patients in the infusion catheter group were compared to 12 patients who received primary analgesia by PCA or IV narcotics. There were no significant differences in age, sex, weight or spinal defect level between the two groups. Opioid use, as defined by IV morphine equivalents, was significantly less in the wound soaker group on all PODs. The total opioid use after POD #0-3 was 0.55 mg/kg in the wound soaker group vs 1.66 mg/kg in the IV/PCA group (p = 0.03). FLACC scores were uniformly lower in the wound soaker group, but were not significantly different. There was a significant decrease in need for postoperative antiemetic use in the wound soaker group (36.5% vs 83.3%, p = 0.014). Complications and hospital stay were similar between both groups. DISCUSSION: The advantage of local anesthesia is the reduction of systemic opioids and their subsequent adverse side effects. Our results suggest that in children with spina bifida undergoing major reconstruction of the lower urinary tract narcotic consumption is approximately 1/3 when continuous local anesthetic catheters are placed into the incision. The need for antiemetic medication is also significantly less. While this technique has been validated in a variety of other settings, it may be most beneficial in patients with myelomeningocele or other spinal dysraphism where epidural placement is generally contraindicated and narcotic use may have a particularly deleterious effect on preexisting neurogenic bowel function. The primary limitation of our study is that it is a retrospective review of a limited number of patients. Patients were not randomized and subject to other management differences that could have influenced our results in unknown ways. CONCLUSIONS: Continuous local anesthetic catheters are a simple, effective alternative strategy to provide postoperative analgesia while reducing systemic opiate use and associated adverse effects.

How do I do it: Continuous local anesthetic infusion for children with spinal dysraphism undergoing major reconstruction of the lower urinary tract.

Postoperative pain control is a fundamental aspect of contemporary pediatric surgery. While many options for analgesia are available to the general patient population, choices are limited for individuals with spinal dysraphism who undergo major urologic procedures. Continuous infusion of local anesthetics has been shown to improve postoperative pain scores and decreases the need for systemic analgesia. We present our technique for continuous local anesthetic infusion utilizing readily available equipment with limited additional cost.

Prechemotherapy laparoscopic nephrectomy for Wilms' tumor.

Laparoscopic radical nephrectomy is emerging as the treatment of choice for localized renal malignancies in adults. Despite the widespread use of laparoscopic nephrectomy for benign renal disease in infants and children, the laparoscopic approach has not been employed for pediatric Wilms' tumor except following systemic chemotherapy. We report the results of laparoscopic radical nephrectomy for removal of unilateral Wilms' tumor prior to the administration of systemic chemotherapy in two patients.

Vesicostomy vs primary ablation for posterior urethral valves: always a difference in outcome?

OBJECTIVE: A vesicostomy is believed to have a detrimental effect in boys with posterior urethral valves compared to primary valve ablation. We compared the outcomes of boys managed by initial vesicostomy with those undergoing primary fulguration. MATERIALS AND METHODS: The outcomes of 54 boys (23 vesicostomy, 31 primary valve fulguration) over 1 year of age who had not undergone renal transplant were considered. Outcome parameters identified were ultrasound findings, continence status, glomerular filtration rate (GFR) and 1-year creatinine. Dryness was defined as completely dry both day and night with no need to wear pads. Results are presented with 95% confidence intervals. RESULTS: Ultrasound examinations were normal in 9/19 (47.4%) of the vesicostomy group and 11/24 (45.8%) of the fulguration group. Graded ultrasound results were not significantly different (p=0.24). The vesicostomy patients were more often dry (79% vs 64%, p=0.43). The vesicostomy group had on average higher GFR (95.26 vs 85.79) and lower 1-year creatinine (49.58 vs 52.46) values. After accounting for age differences between groups, there was no significant difference in the GFR and 1-year creatinine values (p=0.16 and p=0.87, respectively). CONCLUSIONS: There was a tendency for the major outcomes to be more favourable in the vesicostomy group. Although trends were non-significant, confidence intervals were wide and potential differences of clinical importance could not be discounted.

Myogenin and desmin immunohistochemistry in the assessment of post-chemotherapy genitourinary embryonal rhabdomyosarcoma: prognostic and management implications.

PURPOSE: Posttreatment genitourinary embryonal rhabdomyosarcoma often shows well differentiated rhabdomyoblasts, which are detectable on routine histological staining. Definite areas of residual undifferentiated rhabdomyosarcoma indicate residual/recurrent disease. However, the recent use of immunohistochemical staining with desmin and myogenin in resected specimens and surveillance biopsies following adjuvant therapy may demonstrate scant positive staining cells that appear undifferentiated on light microscopy. To our knowledge the clinical significance of this finding is currently unknown. Therefore, we reviewed our retrospective experience with genitourinary embryonal rhabdomyosarcoma to examine the relationship between immunostain positive undifferentiated cells and subsequent clinical outcome. MATERIALS AND METHODS: A total of 14 children with a median age of 2.75 years (range 8 months to 7 years) with genitourinary embryonal rhabdomyosarcoma were identified in the histopathology database. All had biopsy confirmation of the diagnosis, followed by multi-agent chemotherapy. Two children in whom there was obvious residual active tumor at the resection margins were excluded from further analysis. Histopathological findings in all patients on the resection/posttreatment biopsy were reviewed. All specimens were immunostained with desmin and myogenin to detect residual undifferentiated rhabdomyoblasts. The relation between histopathological findings and outcome was determined. RESULTS: There were 14 cases of genitourinary embryonal rhabdomyosarcoma. In 2 cases (14%) residual embryonal tumor was pathologically confirmed following initial treatment. In 12 cases no obvious residual tumor was present following initial therapy. Rhabdomyosarcoma affected the bladder in 10 cases and the vagina in 2. There were no distant metastases in any child. Ten patients underwent local resection following chemotherapy and 2 underwent followup biopsies only without resection. A total of 11 cases showed well differentiated, posttreatment rhabdomyoblasts that was identifiable on routine hematoxylin and eosin staining with margins apparently free of tumor and 1 showed no morphological evidence of residual rhabdomyosarcoma. However, all cases demonstrated at least scant abnormal desmin and myogenin positive cells in the specimens. Four patients had no further treatment and none had clinical recurrence. All were well 10 years (range 8 to 13) after treatment. Eight patients received further treatment (chemotherapy and/or radiotherapy) based on clinical and pathological findings, followed by further resection in 3. One patient died of disease but 7 were well a median of 7.2 years (range 8 months to 13 years) after treatment. CONCLUSIONS: The significance of undifferentiated myogenin/desmin positive cells in genitourinary embryonal rhabdomyosarcoma in the absence of morphological residual/recurrent embryonal rhabdomyosarcoma remains unclear since such cells can be detected in all cases of posttreatment embryonal rhabdomyosarcoma. In some cases findings are associated with clinical disease recurrence, while others with identical histopathological findings following initial treatment have no clinical sequelae even in the absence of further treatment. In genitourinary embryonal rhabdomyosarcoma close and regular clinical surveillance is essential. Desmin/myogenin immunohistochemistry to detect scattered undifferentiated cells does not appear to provide useful prognostic information.

Laparoscopic transposition of lower pole vessels--the 'vascular hitch': an alternative to dismembered pyeloplasty for pelvi-ureteric junction obstruction in children.

OBJECTIVE: Dismembered pyeloplasty is the traditional technique in the management of ureterovascular pelvi-ureteric junction obstruction (PUJO) in children. Controversy remains regarding the role of lower pole vessels as the sole aetiology for PUJO. Endopyelotomy and concomitant laparoscopic transposition of lower pole vessels for PUJO has been described in adults. We describe our technique of laparoscopic transposition of lower pole vessels in children with PUJO, leaving the PUJ intact. PATIENTS AND METHODS: Thirteen patients (seven boys and six girls) with a mean age of 10.2 years (range 7-16 years) underwent laparoscopic transposition of lower pole vessels. Surgery was indicated on the basis of intermittent pain and ultrasound/MAG3 appearance of obstruction with or without reduced function. The technique involved laparoscopic transperitoneal mobilization of the lower pole vessels from the region of the PUJ thereby freeing the junction and transposing them superiorly onto the anterior wall of the pelvis. The main outcome measures were relief of pain and improvement in ultrasound appearance or drainage parameters on a postoperative MAG3 renogram performed within 4-6 weeks of surgery. RESULTS: Median operating time was 92 min. All patients were discharged within 36 h of surgery. All patients remain pain free at a median of 6 months (range 3-18 months). Twelve patients showed good drainage on the postoperative MAG3 renogram and improvement in ultrasound appearance. One patient had recurrent symptoms requiring insertion of a JJ stent. She has undergone further laparoscopic exploration. The vessels were in their transposed position and there was a kink at the PUJ which was released. She had a vertical pyelotomy and transverse closure over the JJ stent with good results. CONCLUSION: This technique is simple and requires less operating time. No anastomosis or temporary JJ stent is required. Our early results are very encouraging with no serious complications.

Wilms' tumour with persistent intravascular extension: a review of the surgical aspects of management.

AIM: To review the surgical management of Wilms' tumour with persistent intravascular (vena caval +/- atrial) tumour extension. PATIENTS AND METHODS: Data were collected regarding operative details, tumour and 'thrombus' histology, and long-term outcome for patients with Wilms' tumour with cavo-artial extension. RESULT: From 1988 to 2004, 13 patients underwent treatment for Wilms' tumour with persistent intravascular extension. Preoperative chemotherapy was administered in 11/13 patients and postoperative radiotherapy in eight patients. Intravascular involvement was upto IVC (5), and right atrium (8) patients. Techniques employed for excision of intra-vascular component were: local cavotomy (3), extensive infra-diaphragmatic cavotomy without cardiopulmonary bypass (CPB) (1), and excision of cavo-atrial tumour with CPB (+/- hypothermia and cardiac arrest) (9). Mean time on CPB was 90 min. Caval repair was accomplished by primary repair (6) and pericardial graft in (7) patients. There were no intraoperative deaths and few major complications. Tumour thrombus contained malignant cells in 10/13 cases. Mean follow up has been for 55.4 months. To date, seven patients remain disease-free (one lost to follow up), disease recurred in five patients, three of whom have died. There were no symptoms related to the graft. CONCLUSIONS: Surgery for Wilms' tumour with persistent intravascular extension despite chemotherapy is technically challenging. CPB +/- hypothermia and cardiac arrest and extensive caval repair with a graft is safe and reliable in the long term.

Renal outcome following heminephrectomy for duplex kidney.

PURPOSE: We evaluated functional outcome following heminephrectomy in duplex kidneys. MATERIALS AND METHODS: A total of 101 patients were identified who underwent upper or lower pole heminephrectomy between January 1995 and December 2000. We retrospectively reviewed 60 case notes with complete data. A total of 37 cases (62%) were diagnosed prenatally, while 20 (33%) presented as single or recurrent urinary tract infection and 3 (5%) as urinary incontinence. Of the patients 52 (87%) had unilateral duplex and 8 (13%) had bilateral duplex kidneys. In addition, 20 of the 60 patients (33%) had ureterocele. Mean age at surgery was 46 months (range 3 to 10 years). RESULTS: Mean function as assessed by nuclear renography of the duplex kidney before heminephrectomy was 39.9% (range 22% to 61%) and following heminephrectomy was 33.1% (13% to 60%). Mean decrease in function was 6.8%, with a decrease in function of less than 5% in 18 patients, 5% to 10% in 13 and more than 10% in 5. The functional values were increased by 1% to 5% in 9 patients and unchanged in 15 patients after heminephrectomy. CONCLUSIONS: Following heminephrectomy there was a significant decrease in 8% of patients and a small decrease in function in the remaining moiety in 51%.

Cell biology of bladder development and the role of the extracellular matrix.

The bladder is unique. It is the most distensible organ in the body. If the normal pattern of development is disturbed, the bladder can become dysfunctional and poorly compliant. Renal outcome is intimately linked to bladder function and hence bladder development. In this review we describe our current knowledge of the molecular processes that govern bladder development, with particular emphasis on the role of the extracellular matrix. Finally, we suggest a hypothetical model for bladder development.

Tubularized incised-plate urethroplasty for distal and proximal hypospadias.

OBJECTIVE: To describe the tubularized incised-plate (TIP) urethroplasty for distal and proximal hypospadias. PATIENTS AND METHODS: TIP urethroplasty was undertaken in 83 consecutive boys (mean age 14.1 months, sd 7.8) within the past 3 years; 65 (78%) had distal and 18 (22%) proximal penile hypospadias. This was the primary attempt at hypospadias repair in all children. RESULTS: All patients were assessed 7 days after surgery at the time of dressing and catheter removal, and subsequently at a median of 4 months in the clinic, before being discharged. The overall late complication rate was 12% and 10% required a second operation. There was a urethrocutaneous fistula in three (5%) of those with distal and in one of the 18 with proximal penile hypospadias (P = 0.56). Glans dehiscence in distal hypospadias occurred in one (1%) and three, respectively (P = 0.01). Secondary surgery for fistula repair and glans closure was successful in all patients. All children void with a single straight urinary stream in a forward direction, and have a normally situated vertical slit-like glanular meatus. CONCLUSIONS: TIP urethroplasty is a versatile, simple operation with good cosmetic results, which uses the urethral plate to its maximum potential.

Long-term gynecological outcome of patients with persistent cloaca.

PURPOSE: Persistent cloaca is a complex malformation that remains a difficult reconstructive challenge, and data on long-term outcome are scarce. Gynecological abnormalities are common with cloaca but may remain asymptomatic until puberty or adult life. We evaluate long-term gynecological sequelae in these patients with persistent cloaca. MATERIALS AND METHODS: The records and radiographs of postpubertal patients (mean age 16.8 years, range 10 to 32) treated for cloacal malformation at 1 institution from 1970 to 2001 were retrospectively reviewed. Outcome data at puberty were available in 41 patients. Of the patients 24 are currently older than 16 years and outcome data for sexual activity were available in 21 with 3 lost to followup. RESULTS: All 41 patients were evaluated at puberty, and 28 (68%) had uterine function, 13 (32%) were menstruating normally and 15 (36%) presented with hematometra/hematocolpos. All 15 girls with an obstructed uterus required surgery, which included hysterectomy in 2, partial hysterectomy with vaginoplasty in 3 and vaginoplasty in 9. There was 1 complex case of fistula. Etiology of the obstructed uterus was vaginal stenosis after reconstruction in 3 cases, stenosis of persistent urogenital sinus (no previous reconstruction) in 11 and cervical stenosis in 1. Ten patients experienced primary amenorrhoea, which was confirmed in 8 (20%) while 2 (5%) continue to be followed for possible cryptic obstruction. In 10 girls the diagnosis of absent/vestigial uterus was made at early laparotomy but this was erroneous in 6 in whom uterine function developed at puberty. Of the 21 older girls (age at review 17 to 32 years, mean 24) 12 are or have been sexually active and 6 have been examined by a gynecologist and have an adequate vagina but are not sexually active. To date 4 patients have required revision vaginal surgery in adulthood to facilitate intercourse (re-do vaginoplasty in 3, introitoplasty in 1). One woman has postponed vaginal reconstructive procedures and 2 others are currently being followed. There have been no pregnancies in this series to date. CONCLUSIONS: Patients born with persistent cloaca have a high incidence of gynecological problems at the onset of menses and in early adult life. Therefore, it is necessary to reassess these girls at early puberty by ultrasound/magnetic resonance imaging and vaginoscopy. Additional surgery may then be necessary to create a vagina for menstruation and sexual intercourse.

Accuracy of antenatal fetal ultrasound in the diagnosis of duplex kidneys.

OBJECTIVES: To assess the accuracy of ultrasound diagnosis of duplex kidneys in the fetus and to identify prognostic features of antenatal sonography associated with this diagnosis. METHODS: Retrospective identification of cases with an antenatal diagnosis of duplex kidney was made from computerized databases. Results of postnatal radiological investigations were obtained from the unit where delivery took place. RESULTS: A total of 75% of cases seen in a multidisciplinary fetal renal clinic were correctly diagnosed as having a duplex kidney. Prognostic sonographic features are described. Detection of two separate poles or a ureterocele were strongly associated with a correct diagnosis. Accuracy was increased when two or more prognostic features were seen. CONCLUSION: Accurate sonographic diagnosis of duplex kidneys in the fetus is possible in a dedicated multidisciplinary setting. Antenatal counseling and planning of postnatal care for the infant can be made with a high degree of certainty.