Postnatal Calyceal-to-Parenchymal Ratio: A Promising Predictor for Surgical Correction of Ureteropelvic Junction Obstruction in Newborns.

OBJECTIVE: This study aims to explore a new parameter, the calyceal-to-parenchymal ratio (CPR) of postnatal renal ultrasonography (RUS) as a predictor of surgery in newborns with possible ureteropelvic junction obstruction (UPJO). Although UPJO remains the main surgical category of antenatally detected hydronephrosis, there is a lack of a gold-standard test that predicts the need for pyeloplasty. SUBJECTS AND METHODS: We retrospectively reviewed infants with a positive antenatal history of hydronephrosis who were confirmed to have grade 3 or 4 hydronephrosis on postnatal RUS between 2010 and 2020. We compared postnatal CPR between surgical and control groups and tested the correlation between postnatal CPR and diuretic renogram. RESULTS: A total of 79 and 136 kidneys were included in the surgical and control groups, respectively. Kidneys that were managed with pyeloplasty between January 2010 and July 2020 were included in the surgical group, while kidneys from patients with comparable traits who were managed conservatively comprised the control group. At a mean age of 18.9 weeks at presentation and a mean follow-up period of 48.99 months, the median postnatal CPR was significantly greater in the surgical group (3.62 vs. 0.98, p<0.001). A postnatal CPR of 1.68 had a sensitivity and specificity of 96.2% and 84.8%, respectively, in predicting the need for future pyeloplasty (area under the curve (AUC)=0.966). There was a positive and significant correlation between postnatal CPR and the half-life of the renogram (p=0.018) but not significant with the differential function (p=0.090). CONCLUSION: Diuretic renography has little capability for predicting future pyeloplasty. Current RUS grading systems do not offer an objective measure of renal parenchyma. Numerous other RUS parameters are less frequently utilized in clinical practice, and many are challenging to assess and require sophisticated software or equipment. Postnatal CPR is a promising tool for predicting the need for pyeloplasty in newborns with UPJO. Further prospective studies are needed to standardize and assess the reproducibility of this parameter.

Outcome of stented versus unstented mid-shaft to distal hypospadias repair.

Aims: We compared the outcomes of unstented repair (UR) versus stented repair (SR) in patients with mid-shaft to coronal hypospadias (HS) to elucidate if SR has any advantage over the UR. Materials and Methods: We retrospectively studied our mid-shaft to coronal HS repair patients between January 2013 and January 2018. We recorded variables such as degree of HS, age at repair, surgeon, type of repair, suture used, stent usage, and standard early and late complications. Relative risk (RR) was calculated and P < 0.05 was considered significant. Results: We included 120 patients (63 UR, 57 SR). There was no statistically significant difference in any parameters in both the groups. All had either tubularized incised plate or Thiersch-Duplay procedure. Urethroplasty was done with PDS 6/0 in all cases. Trainees performed two-third of the repairs under variable supervision. Early complications included one UR patient having urinary retention needing insertion of urethral catheter, five SR patients having bleeding/swelling, and three UR having dysuria. All were managed conservatively. For late complications, 98 patients were available (UR: 51, SR: 47) with fistula in 17 (17.3%), UR 8 (15.6%) versus SR 9 (19.1%) (P = 0.5, RR = 1.2) meatal stenosis in 3, UR 3 versus SR 0 (P = 0.06, RR = 6.4) and glanular dehiscence 6, UR 4 versus SR 2 (P = 0.25, RR = 1.8). Conclusion: There were no statistically significant differences in the short-term complications between UR and SR for HS. In the long term, RR for meatal stenosis is higher in UR.

Neonatal functional intestinal obstruction and the presence of severely immature ganglion cells on rectal biopsy: 6 year experience.

PURPOSE: We report our experience of managing eight babies who presented with neonatal intestinal obstruction and whose rectal biopsies showed severely immature ganglion cells. METHODS: Neonatal unit records were reviewed to detect patients with suspected Hirschsprung's disease or functional intestinal obstruction. Those with intestinal atresia, anorectal malformation, malrotation, cystic fibrosis and prematurity were excluded. RESULTS: We identified 73 patients born at term. Twenty-seven did not need a rectal biopsy. Twenty-one had biopsy proven Hirschsprung's disease, while 17 had a normal rectal biopsy. Eight patients, all of whom presented with severe abdominal distension, showed immature ganglion cells. Seven had failed to pass meconium after birth. X-rays in all patients showed distended loops of bowel. Two neonates underwent an emergency laparotomy and a stoma. A repeat biopsy at 3 months showed maturation of ganglion cells and the stoma was reversed. Rectal biopsy was repeated in two other patients 2-9 months after the first biopsy and showed mature ganglion cells. At follow-up, one patient still suffers from severe constipation. Seven are asymptomatic now, including the two patients who needed a stoma. CONCLUSION: Immature ganglion cells on rectal biopsy may be an indicator of transient functional immaturity of the intestine.

A Large Congenital Anterior Urethral Diverticulum in a 14-Month-Old Boy.

Congenital anterior urethral diverticulum is a rare condition causing lower urinary tract obstruction in children. It usually arises from the ventral aspect of the anterior urethra, mostly located at the penoscrotal junction. We report a case of a 14-month-old baby boy who presented with a soft ventral swelling over the distal penile urethra, difficulty in passing urine, and a history of recurrent febrile urinary tract infections. A retrograde urethrogram revealed a large distal anterior urethral diverticulum. He underwent diverticulectomy and primary repair with no post-operative complications. The treatment of these depends on the size of the diverticulum and the degree of obstruction. In cases of a large anterior urethral diverticulum, open diverticulectomy and primary repair are recommended.

Flexible ureterorenoscopy to treat upper urinary tract stones in children.

Development of surgical expertise and technology has affected the way renal tract stones are treated. Our hypothesis was that flexible ureteroscopy (FURS) for upper tract stones in children produces good results. Our outcomes were reviewed. A retrospective case note review was performed for children with upper tract calculi who were treated by FURS. There were 56 stone episodes in 36 patients. Median age was 10.6 years. Stones were 3-23 mm (median 8 mm); 64.3% had multiple calculi. Median follow-up was for 17.1 months. After the first FURS there was stone clearance in 42/56 (75%). Although there were no immediate complications, two required re-admission; one with stent symptoms, the other with urinary infection. A second FURS was performed in 11, bringing the cumulative clearance to 89%, although this was often done as "another look" before stent removal. There was no statistically significant difference in stone clearance after first FURS for those with single stones (81.0%) compared to those with multiple stones (72.2%). Clearance rates of more than 70% after first FURS were achieved with stones of up to 17 mm. Unexpected disease was found and treated during FURS in 9 (16.1%) children. FURS is safe in children and good clearance rates are achieved. Multiple stones at different sites may be treated during the same treatment. In addition, FURS allows diagnosis and treatment of unexpected problems.

Outcome of salvage ureteral reimplantation after endoscopic treatment failure for high-grade vesicoureteral reflux compared to primary ureteral reimplantation.

INTRODUCTION: Surgical treatment of vesicoureteral reflux is required after conservative treatment has failed. However, there is a controversy if fibrosis related to previous attempts of dextranomer/hyaluronic acid (Dx/Ha) injection increases the risk of surgical difficulty and postoperative complications. Therefore, the purpose of our study was to compare the outcome of salvage ureteral reimplantation (SUR), after failed endoscopic therapy, to that of primary ureteral reimplantation in patients with high-grade primary vesicoureteral reflux (VUR). MATERIALS AND METHODS: We conducted a retrospective analysis of children, <14 years old, treated for Grade IV or V VUR, between 1998 and 2014. Cases were classified into the SUR or the PUR group. Cases of secondary VUR were excluded. All patients were treated using a cross-trigonal ureteral reimplantation technique by two surgeons. The following demographic and clinical variables were included in the analysis: presentation, reflux severity, scarring on imaging, age at endoscopic injection, total amount of Dx/Ha injected, operative time, postoperative hospital stay, operative complications, incidence of febrile urinary tract infections (UTIs) after surgery, and persistent VUR. Between the groups, differences were evaluated using Fisher's exact test. RESULTS: Twenty-six patients were included, 19 in the SUR and 7 in the primary ureteral reimplantation (PUR) group. In the SUR group, 12 cases had a bilateral VUR and 7 had a unilateral VUR, with 4 bilateral and 3 unilateral VUR cases in the PUR group. In the SUR group, 13 patients had received one Dx/Ha injections, with the other 6 receiving two injections, of 0.5 ml of Dx/Ha (range, 0.5-2.0 ml). A bilateral reimplantation was performed in 14/19 patients in the SUR group and 4/7 in the PUR group. The median age at surgery was 4 years in the SUR group and 3 years in the PUR group (P < 0.02). The median operative time was comparable between the groups (120 and 140 min for the SUR and PUR groups, respectively, P = 0.73), with a comparable length of hospital stay (5 and 6 days, respectively, P = 0.061). Blood loss was generally <10 ml, except in three cases in the SUR group, due to difficult dissection. Over the median follow-up of 1 year, persistent Grade III SUR was identified in only one patient in the SUR group, with no occurrence of febrile UTIs postoperatively. CONCLUSION: SUR for high-grade primary VUR after failed Dx/Ha injection has the same success rate as PUR, with no significant complication rate, although the necessary dissection may be more difficult.

Complications and patient satisfaction with urethral clean intermittent catheterization in spina bifida patients: comparing coated vs uncoated catheters.

INTRODUCTION: Many types of catheters are available in market for clean intermittent catheterization (CIC). Each company claim superiority of their products, but strong evidence is lacking. PURPOSE: To assess the complications due to CIC in spina bifida children and its possible relationship to hydrophilic-coated catheter (HCC) or uncoated catheters (UCCs), with a view to decrease catheter related complications, and improve patients experience and compliance. MATERIALS AND METHODS: The authors retrospectively reviewed the spina bifida patients aged between 0 and 16 years, who had no surgical intervention, and were performing CIC urethrally for at least 6 months. General information was recorded from electronic patient record, followed by telephonic/outpatient interview. Patients were divided into two groups: UCC or HCC. Patients in both groups had also used catheter from the other group at times. This study recorded the type/size of catheter used, its duration, complications, and their possible relation to type of catheter. Carer/patients overall satisfaction was recorded on a scale of 1-10 and their preference about the type of catheter they wish. The data were analyzed using SPSS (P-value < 0.05 as significant). RESULTS: One hundred one patients were included in the study: 53 UCC and 48 HCC. There was no significant difference between gender/associated conditions/age at start of CIC or duration of CIC. The mean time taken to perform the procedure was similar in both groups: UCC 9.7 min vs HCC 8.8 min. Difficulty in insertion was felt in 20, UCC 12 vs HCC 8 (P = 0.15), recurrent UTIs UCC 12 vs HCC 17 (P = 0.09), median patient satisfaction UCC 8/10 (3-10) and HCC 10/10 (7-10) (P = 0.63). Request for change of catheter was made by 10 from UCC group to HCC vs none from HCC (P ≤ 0.05).When given a choice, 28/53 (52.8%) in UCC and none (0%) in HCC group (P ≤ 0.0001) preferred to change to the other type of catheter, mainly for convenience of use of the product. Per year per patient cost was UCC US$ 389 vs HCC US$ 2820. DISCUSSION: Many un-modifiable factors contribute to the outcome of CIC. Despite claims of superiority by manufactures of some catheters over others, strong evidence is lacking especially in children. This study has shown no difference in the complications between UCC and HCC. CONCLUSION: There is no significant difference in complication rates with urethral CIC in patients using either UCC or HCC. A significant majority would prefer to use HCC mainly because of convenience of use of the product though at a seven times higher cost.

Oesophageal replacement in children with indolent stricture of the oesophagus.

OBJECTIVE: To evaluate the indications for and results of oesophageal replacement for acquired oesophageal stricture in children. METHODS: This was a descriptive interventional study. Between 1987 and 2003, patients who had peptic or corrosive stricture were included in the study. Age and gender were analysed and any history of ingestion of caustic agents or untreated reflux was noted. Types of symptoms such as progressive dysphagia to solids and liquids and extent of respiratory infections were evaluated. Nutritional deprivation was also measured. Success or failure of dilatation at the time of endoscopy was taken into account. The criterion for oesophageal replacement was recurrence of stricture within 3 weeks of the previous dilatation. Patients were prepared with general supportive care. Feeding gastrostomy was performed in 12 patients who were severely malnourished. No patients underwent oesophagectomy of the strictured oesophagus. Short- and long-term complications were recorded. RESULTS: Of the 54 patients, 34 were male and 20 were female. Colonic conduit and reverse gastric tube were used in 27 patients each. Three patients had leak and were treated conservatively. Six patients developed anastomotic stricture but benefited remarkably from periodic dilatation and, in the long-term, remained symptom-free. Results were generally very gratifying. CONCLUSION: Most patients who require oesophageal conduit do well and catch up with their growth and development.

Late presentation of Bochdalek hernia with intestinal symptoms.

Bochdalek hernia is a rare condition that usually presents in the neonatal age. Rarely it presents late and is usually misdiagnosed. We came across a Bochdalek hernia, which presented at the age of 15 years and was initially misdiagnosed as pulmonary tuberculosis. The case history, diagnosis and management of this condition along with literature review are presented here.

Presentations of Meckel's diverticulum at Khyber Teaching Hospital Peshawar.

BACKGROUND: This study was conducted with an objective to observe various presentations of Meckel's Diverticulum in our setting and compare it with other national and international studies. METHODS: It was a retrospective analysis of hospital record carried out at the department of paediatric surgery Khyber Teaching Hospital, Peshawar. A total of 63 patients meeting the inclusion criteria were included in the study who had presented to our unit with signs and symptoms of Meckel's Diverticulum and who were diagnosed either by investigations or at surgery. All the patients were explored and resection and anastomosis performed. We did not do H. Pylori culture. Patients with bleeding per rectum were investigated by Meckel's scan. Analysis of the results was done by SPSS version 10. RESULTS: Majority of our patients presented with obstruction (82.5%) in contrast to the Western studies (around 40%) while only 4.7% presented with bleeding per rectum again in contrast to the Western countries (38.56%). CONCLUSION: There appears to be a geographical and/or racial difference in the presentation of Meckel's diverticulum.

British Association of Paediatric Urologists consensus statement on the management of the primary obstructive megaureter.

INTRODUCTION: It is well-known that the majority of congenital megaureters may be managed conservatively, but the indications and surgical options in patients requiring intervention are less well defined. Hence this topic was selected for discussion at the 2012 consensus meeting of the British Association of Paediatric Urologists (BAPU). Our aim was to establish current UK practice and derive a consensus management strategy. METHODS: An evidence-based literature review on a predefined set of questions on the management of the primary congenital megaureter was presented to a panel of 56 Consultant Surgeon members of the British Association of Paediatric Urologists (BAPU), and current opinion and practice established. Each question was discussed, and a show of hands determined whether the panel reached a consensus (two-thirds majority). RESULTS: The BAPU defined a ureteric diameter over 7 mm as abnormal. The recommendation was for newborns with prenatally diagnosed hydroureteronephrosis to receive antibiotic prophylaxis and be investigated with an ultrasound scan and micturating cystourethrogram, followed by a diuretic renogram once VUR and bladder outlet obstruction had been excluded. Initial management of primary megaureters is conservative. Indications for surgical intervention include symptoms such as febrile UTIs or pain, and in the asymptomatic patient, a DRF below 40% associated with massive or progressive hydronephrosis, or a drop in differential function on serial renograms. The BAPU recommended a ureteral reimplantation in patients over 1 year of age but recognized that the procedure may be challenging in infancy. Proposed alternatives were the insertion of a temporary JJ stent or a refluxing reimplantation. CONCLUSION: A peer-reviewed consensus guideline for the management of the primary megaureter has been established. The guideline is based on current evidence and peer practice and the BAPU recognized that new techniques requiring further studies may have a role in future management.

Long-term followup of patients after redo bladder neck reconstruction for bladder exstrophy complex.

PURPOSE: The aim of this study was to determine whether redo bladder neck reconstruction is effective in achieving continence after a failed bladder neck reconstruction procedure. MATERIALS AND METHODS: We retrospectively reviewed the hospital records of patients with bladder exstrophy who had undergone redo bladder neck reconstruction. There were 30 patients in the study, including 20 boys and 10 girls. Mean patient age at redo bladder neck reconstruction was 9.3 years (range 3.2 to 15.5). The patients were divided into 3 groups on the basis of the preoperative pattern of incontinence--incomplete wetters, complete wetters and those on continuous suprapubic drainage. Of the patients 15 already had undergone bladder augmentation, 12 had undergone a Mitrofanoff procedure and 12 had been treated with bulking agents injected in the bladder neck in an attempt to achieve continence. Four patients had undergone more than 1 bladder neck procedure. The patients were investigated with a combination of noninvasive urodynamics, cystoscopy, cystogram and ultrasound. All patients underwent Mitchell's modification of Young-Dees-Leadbetter bladder neck reconstruction. Additional procedures performed included augmentation cystoplasty and Mitrofanoff formation. RESULTS: Mean followup was 6.9 years (range 1.2 to 15.5). Postoperatively 28 patients were using clean intermittent catheterization to empty the bladder (5 per urethra, 23 via Mitrofanoff). Two patients remained on continuous suprapubic catheter drainage. A total of 18 patients (60%) were dry postoperatively (80% of girls and 50% of boys). Among dry patients only 3 were performing clean intermittent catheterization per urethra and 15 via a Mitrofanoff channel. No patient was able to void per urethra without the need for clean intermittent catheterization. The 2 patients on continuous suprapubic catheter drainage continued to remain so. At night only 50% of the patients were dry (5 on free drainage, 4 on clean intermittent catheterization, 6 not on any drainage). Those patients who did not respond satisfactorily to redo bladder neck reconstruction underwent subsequent additional procedures, which included injection of bulking agents (3 patients), insertion of an artificial urinary sphincter (1), Mitrofanoff formation (2) and bladder augmentation plus Mitrofanoff channel (1). Postoperative complications included difficulty with clean intermittent catheterization (8 patients), perivesical leak (1), recurrent epididymo-orchitis (1), upper urinary tract dilatation (2) and incisional hernia (1). Bladder neck closure was being considered in 5 patients. CONCLUSIONS: In our experience redo bladder neck reconstruction cannot achieve continence with volitional voiding per urethra. Although redo bladder neck reconstruction can render a significant number of patients dry, it is only effective if performed in conjunction with augmentation. Failure of the initial bladder neck reconstruction may be a reflection of a bladder that is of inadequate capacity and/or compliance. Therefore, bladder augmentation should be considered in all patients requiring redo bladder neck reconstruction. Bladder neck closure may be a better alternative to redo bladder neck reconstruction.

Injectable polydimethylsiloxane for treating incontinence in children with the exstrophy-epispadias complex: long-term results.

OBJECTIVE: To present our experience with the use of injectable polydimethylsiloxane (Macroplastique, Uroplasty, Minneapolis, MI, USA) for treating incontinence in children with the exstrophy-epispadias complex (EEC), as incontinence continues to be a challenging problem in such children, and although the primary management of EEC has developed over the last few decades, with early closure and reconstruction of the penis, achieving satisfactory continence status remains elusive. PATIENTS AND METHODS: We retrospectively reviewed the hospital records of 52 patients (41 boys and 11 girls, mean age at first injection 6.6 years, range 3.6-16.7) with EEC who had injections with Macroplastique between January 1991 and February 2004; 34 had bladder exstrophy and 18 primary epispadias. For this study we defined success as complete dryness with no use of pads or nappies. Improvement was defined as being occasionally wet but with dry intervals lasting >or= 4 h. RESULTS: The mean (range) follow-up was 4.6 (0.5-9) years. Twenty patients had one injection, 10 had two, 13 had three, six had four, two had six and one had seven injections. In most patients a maximum of three injections predicted the outcome. The injection of Macroplastique was successful in nine patients (17%; with an annual follow-up, two at 1-2 years, three at 2-5 years and four at >5 years), whilst 17 (33%) improved significantly (one at <1 year, two at 1-2 years, eight at 2-5 years and six at >5 years). Those patients comprised five of 18 (27%) with epispadias and four of 34 (12%) with exstrophy. A history of previous surgery and gender had no significant effect on the outcome. Overall half the patients benefited from the procedure. CONCLUSIONS: This series confirms that injection with Macroplastique is minimally invasive, durable in significantly many patients and has a reasonable success rate. A history of previous surgery and gender had no significant effect on the outcome. Patients with epispadias are more likely to benefit from an injection with Macroplastique than those with bladder exstrophy. A maximum of three injections is predictive with reasonable certainty of any benefit from the procedure.

Separation of xiphi-omphalo-ischiopagus tetrapus twins with favorable internal anatomy.

Conjoined twinning is a rare occurrence, and ischiopagus variety is even more rare. So far, only 20 cases have been reported. The incidence is 1 in 500,000. Because of its rarity and variable anatomy, no definite line of treatment can be adapted. Time of operation and mode of treatment have to be tailored according to the local circumstances and organ sharing in each individual pair. The twins in this report were xi phi-omphalo-ischiopagus tetrapus conjoined twins. Although several internal organs were fused and shared, they were complete mirror image sets for each twin. In this case report, details of anatomy and more importantly, preoperative investigations, preparations, and organizational aspects that were designed to suit local conditions are described. Operative details, postoperative care, and short and long-term progress also has been highlighted.