Imran Mushtaq

Antegrade continence enema (ACE) revolutionised the lives of children with chronic constipation and soiling. Parents often ask how long the ACE will be required. We looked at our patients 5 years after ACE formation to answer the question. We reviewed clinical notes of all patients undergoing ACE procedure during January 1990 to December 2010. Only patients with >5 years follow-up were included. Data are given as median (range). 133 patients were included with >5 years of follow-up. Primary pathology was anorectal anomaly (ARA) 64 (48 %); spinal dysraphism (SD) 40 (30 %); functional constipation (FC) 14 (10 %); Hirschsprung's Disease (HD) 10 (8 %) and others 5 (4 %). Median follow-up was 7 years (5-17 years). Overall 74 % still use their ACE; whilst 26 % no longer access their stoma, of whom 47 % recovered normal colonic function. 50 % of HD patient recover colonic function. FC has the highest failure rate at 21 %. Overall 86 % achieved excellent clinical outcome with 74 % of patient still using their ACE at 5 years. HD has the highest recovery rate of 50 %. FC has a more unreliable clinical outcome with 21 % recovered colonic function and 21 % failed. Outcome varied dependent on the background diagnosis.

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. 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. 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). 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.

Page 1. 9 Congenital Anomalies of the Bladder and Urethra Mohan S. Gundeti and Imran Mushtaq CASE 1 ... J Urol. 2000;163(3):782–4. Dewan PA. A study of the relationship between syringoceles and Cobb's collar. Eur Urol. 1996;30(1):119–24. ...

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.

Page 1. CLINICAL QUIZ A patient with polyuria and hydronephrosis: question Graciana Jaureguiberry & William van't Hoff & Imran Mushtaq & Divyesh Desai & Nicholas P. Mann & Robert Kleta & Daniel G. Bichet & Detlef Bockenhauer ...

Page 1. CLINICAL QUIZ A patient with polyuria and hydronephrosis: answer Graciana Jaureguiberry & William van't Hoff & Imran Mushtaq & Divyesh Desai & Nicholas P. Mann & Robert Kleta & Daniel G. Bichet & Detlef Bockenhauer ...

Standard treatment for ureterovascular ureteropelvic junction obstruction has been dismembered pyeloplasty. We previously reported the alternative technique of laparoscopic transposition of lower pole vessels (the vascular hitch) in pediatric patients. This report is an update of this select group of pediatric patients with intermediate followup. Patients underwent diagnostic renal sonography and (99m)technetium-mercaptoacetyltriglycine diuretic renography with additional magnetic resonance angiography in candidate patients. Radiographic criteria included moderate hydronephrosis with no caliceal dilatation and a well preserved cortex, poor renal drainage with preserved split function and lower pole crossing vessels. Intraoperative criteria included a normal ureter and ureteropelvic junction with peristalsis. Postoperatively patients were followed clinically, and with renal sonography and (99m)technetium-mercaptoacetyltriglycine renography at 1 and 2 months, respectively. Success was defined as symptom resolution with radiographic improvement in hydronephrosis and drainage with preserved renal function. Nine boys and 11 girls 7 to 16 years old (mean age 12.5) underwent laparoscopic transposition of crossing vessels, including 3 with da Vinci robot assistance. Mean operative time was 90 minutes (range 47 to 140). Median hospital stay was 24 hours. No ureteral stents or urethral catheters were placed intraoperatively. At a mean followup of 22 months (range 12 to 42) 19 of 20 patients (95%) had been successfully treated. One patient who had recurrent pain underwent successful laparoscopic pyeloplasty. At intermediate followup the laparoscopic vascular hitch procedure has been successful in treating patients with ureterovascular ureteropelvic junction obstruction. In these select patients this technique offers a feasible and durable alternative to standard dismembered pyeloplasty. Ongoing evaluation continues to ensure that the promising results endure.

To review our initial experience with the retroperitoneoscopic approach, which is rapidly becoming the procedure of choice for paediatric adrenalectomy. Swift access to the vascular pedicle makes this approach ideal for adrenal surgery where haemodynamic instability is a common feature. Patients were placed in a fully prone position and the locations of the 12th rib, iliac crest and paravertebral muscles were marked. A 10-mm incision was made lateral to the para-vertebral muscle, halfway between the iliac crest and costal margin. A retroperitoneal working space was created around the kidney using a homemade balloon. A 10-mm Hasson port was then inserted. A 5-mm working port was placed lateral to the tip of the 12th rib and, if necessary, a second was inserted medial to the camera port. Dissection was performed with diathermy and/or Harmonic Scalpeltrade mark and the adrenal vessels were divided between clips. The specimen was retrieved in an endopouch through the 10-mm port. Over 16 months, six adrenalectomies were performed in five patients (three boys and two girls); two right, two left and one bilateral. Median age at surgery was 8 years. Presentation was with hypertension (n=2), Cushing's syndrome (n=2) and abdominal pain (n=1). Median operative time was 154 min. A single-instrument port adrenalectomy was performed in three patients. Histopathological diagnosis included adrenal cyst (n=1), cystic phaeochromocytoma (n=1), adrenal cortical tumour (n=2) and central Cushing's disease (n=1). All were completely resected. All patients remain symptom free with a median follow up of 17 months. These cases represent our initial institutional experience with retroperitoneoscopic adrenalectomy. The senior reporting surgeons have already surmounted the general learning curve for laparoscopy, and this experience has proved vital to expand our repertoire with encouraging early results. This is our technique of choice as it provides a superior view of the adrenal gland and vessels, with good intraoperative haemodynamic stability.

Ibuprofen, a non-steroidal anti-inflammatory drug, is the mainstay in the management of pain and fever in children. In most children, ibuprofen is well tolerated and does not produce significant adverse effects. We report a case of acute papillary necrosis causing bilateral ureteric obstruction induced by ibuprofen.

Laparoscopy is now the established management for infants with inguinal hernia at our institution. Open inguinal herniotomy in children with bladder exstrophy can be a difficult operation and is associated with a high rate of recurrence (15%). Here, we report our initial experience with laparoscopic repair of inguinal hernia in three children with bladder exstrophy. Three boys with repaired bladder exstrophy presented with inguinal hernia during follow up. The clinical presentation was bilateral in one and findings at surgery were bilateral open internal rings in two. Under general anaesthesia, pneumoperitoneum of 10 mmHg was raised with a Hasson port at the umbilicus. Two further instruments were inserted in the right and left flanks without ports. Two 4/0 Prolenetrade mark purse string sutures were placed to securely close the internal ring. The umbilicus was closed with an absorbable suture, and the skin incisions were closed with Indermil Gluetrade mark. All infants made an uneventful recovery, being discharged within 24h. There were no complications. There has been no sign of recurrence during a mean follow up of 16 months. Laparoscopic inguinal herniotomy is a technically straightforward and effective way to treat inguinal hernias in children with bladder exstrophy.

To describe a novel modification of the posterior prone retroperitoneoscopic nephrectomy/nephroureterectomy which allows the entire procedure to be performed through a single instrument port. With the patient in a prone position, a retroperitoneal working space is created with the aid of a homemade balloon. A single instrument port is then placed at the tip of the 11th/12th rib under direct vision. Gerota's fascia is incised, the kidney is reflected anteriorly, and the hilar vessels are identified and divided. The ureter is mobilized as far distally as possible before division. The specimen is removed directly via the camera port or in an endobag. We have employed this technique in 26 children with a high success rate. The median operating time was 58 (range 45-90) min. Blood loss was minimal in all cases and there were no complications. There have been no conversions and the cosmetic outcome has been excellent. Most of the children (24 of 26) were discharged home the day after surgery. The single instrument port laparoscopic (SIMPL) nephrectomy is a feasible and safe technique for the experienced paediatric laparoscopic urologist.

... Journal of Laparoendoscopic & Advanced Surgical Techniques 21:4, 349-351. [Abstract] [Full Text] [PDF] [PDF Plus] [Supplementary material] 2. Ravi Prakash Kanojia, Monika Bawa, Abhilasha T. Handu, Bikas K. Naredi, Prema Menon, KLN Rao. 2010. ...

The increasing role of robotic technology to facilitate surgical procedures has attracted much attention from surgeons and patients alike. In particular, the dramatic increase in the number of laparoscopic radical prostatectomies performed using the da Vinci surgical system has led to interest in using this technology for other procedures. We have evaluated our own experience performing ablative and reconstructive laparoscopic renal surgery using the da Vinci system to determine its potential role. To review our experience of robotic-assisted laparoscopic procedures of the upper urinary tract. Our da Vinci system was installed in June 2004. A prospective database has been maintained concerning all patients and procedures performed from that time. Procedures involving the upper urinary tract were identified and the data was examined. This included patient demographics, operative time, blood loss, hospital stay and patient outcomes. Twenty-six robotic procedures involved the upper urinary tract. Of these, two had to be converted to conventional laparoscopic surgery because of da Vinci mechanical failure. Robotic-assisted procedures included pyeloplasty (n = 15), simple nephrectomy (n = 2), radical nephrectomy (n = 1), nephroureterectomy (n = 2), and live donor nephrectomy (n = 4). The mean operative time was 215 min. The anastomotic time for the pyeloplasties averaged 47 min. The mean blood loss was 75 ml. There were no conversions to open surgery. The complication rate was 8.7%. Postoperative stay averaged 2.9 days. The da Vinci surgical system may be safely used to assist in the performance of laparoscopic renal surgery.

To describe a modification of the posterior prone retroperitoneoscopic nephrectomy, which allows the entire operation to be performed through a single instrument port. With the patient prone, a retroperitoneal working space is created using a custom-made balloon lateral to the sacrospinalis muscle. One instrument port is placed at the tip of the 11th rib under direct vision. The laparoscope and working instrument can both be held by the operating surgeon. Gerota's fascia is incised and the kidney reflected anteriorly. The vessels are identified and divided. The remaining dissection is completed with a harmonic scalpel and the specimen is placed in an endo-catch bag. Care must be taken to avoid even minor bleeding, to keep the operating field clear. The technique was successful in 54 children with a mean (range) age of 4.5 (0.25-14) years; the mean operative duration was 52 (35-96) min. Blood loss was minimal and there were no open conversions. Most children (51) were discharged the day after surgery, and the cosmetic outcome has been excellent in all cases. Compared with the traditional approach, the single-instrument port laparoscopic (SImPL) nephrectomy approach avoids instrument crowding and maximizes the restricted retroperitoneal working space. Avoiding the second port might improve cosmesis and reduce cost. The technique is quickly mastered by both the experienced laparoscopist and trainee, and is feasible and safe.

Meconium ileus (MI) affects 15% of neonates with cystic fibrosis (CF). The authors reviewed the management and outcome of 51 neonates presenting to a single institution between 1976 and 1995 with MI secondary to CF. Clinical presentation included abdominal distension (96%), bilious vomiting (49%), and delayed passage of meconium (36%). A family history of CF was present in 4 cases (8%). Twenty-three neonates presented with MI and evidence of volvulus, atresia, or perforation (complicated MI). Of these, 16 underwent stoma formation, 1 appendicectomy, and 6 resection with primary anastomosis. Twenty-eight neonates presented with uncomplicated MI. Of these, 11 were managed non-operatively by Gastrografin enema (10) or enteral N-acetylcysteine (1). The remainder required stoma formation (15) or bowel resection with primary anastomosis (2). Early postoperative complications occurred in 2 neonates (4%). In this hospital the 1-year survival for this condition has increased from 49% (1953-1970) to 98% (1976-1995) irrespective of the surgical procedure performed or the presence of volvulus, atresia, or perforation. In our experience, bowel resection with primary anastomosis is as safe as stoma formation and is associated with a reduced length of initial hospital stay.

The number of current advances in robotic surgery for the pediatric population is growing every day: the different procedures range from extirpative to reconstructive, including pyeloplasty, reimplantation, catheterizable channels and augmentation. Despite its early success, robotic surgery still poses many challenges in pediatric patients. Robotics also allows the seasoned laparoscopist to become more proficient and refined, providing a greater armamentarium to expand minimally invasive surgery to more complex reconstructive procedures. The procedure most performed with the da Vinci Surgical System in pediatric urology is pyeloplasty for ureteropelvic junction obstruction. There are many other procedures that can be performed with the robot, such as ureteral reimplantation, and nephrectomy, both total and partial. The reconstructive aspect has been taken to the next level where more difficult procedures, such as appendicovesicostomy and bladder augmentation, can be performed in children.

PURPOSE Mixed Gonadal Dysgenesis (MGD) is one of the more frequent intersex disorders. The most common genotype is 45XO/46XY mosaicism and these patients typically present with a streak gonad on one side and a palpable gonad on the other with ...

ABSTRACT The Chicago consensus statement of 2005 was created at the point of cumulative criticisms and debates around the clinical practice of childhood genital surgery. It was drawn up at a time when it had become clearer that medically non-essential paediatric genital operations were associated with poor adult cosmetic outcomes and sexual functioning. However, data were not available for non-intervention. Therefore, parents and clinicians had no reliable information on how a child growing up with atypical genitalia might fare. The most positive recommendation in the consensus statement was the strong recommendation for decisions to be reached by a multidisciplinary team in collaboration with affected families. Importantly, the value of user groups was likewise formally acknowledged. For many services, there has been a sea change in the way surgeons work. Whilst some surgeons may continue with the standard practice of childhood genital surgery, it is becoming clearer that with adequate support, more individuals and families choose to postpone elective interventions. However, these are our observations only. Authoritative evidence must be based on high-quality multi-centre multidisciplinary research to prospectively monitor the long-term multiple outcomes of surgery and no surgery. There is as yet no obvious move towards such an endeavour.

An imperforate hymen associated with urinary retention, bilateral hydronephrosis, and bilateral hydrosalpinx is extremely rare in the neonatal period. We present a case of a 2-day-old neonate with a marked interlabial swelling causing urinary retention. Imaging revealed hydrometrocolpos, hydrosalpinx, and bilateral hydronephrosis. Her symptoms resolved after hymenectomy.

We report a 7-year-old boy who had antenatally diagnosed and postnatally confirmed asymptomatic right congenital hydronephrosis secondary to ureteropelvic junction obstruction with the anteroposterior diameter ranging from 7 to 15 mm on serial ultrasound scans till the age of 6 years. He then presented with recurrent attacks of Dietl's crisis almost every month in the seventh year of his life and eluded diagnosis for over a year at various national hospitals, and ended up with an international referral to us. Investigations of the acute attacks clinched the diagnosis of ureteral polyp causing intermittent crisis and he underwent segmental resection and reconstruction in the form of dismembered Anderson-Hyne pyeloplasty with good recovery. Our case revealed that prenatally detected hydronephrosis may worsen after spontaneous postnatal improvement and a polyp acting as a flip valve may produce intermittent hydronephrosis and symptoms later in life. The child should undergo urgent ...

Robotic-assisted minimally invasive surgery is penetrating pediatric urology. The freedom afforded by the "surgical actuators" has led to the expanding adoption of robotics, and it is unlikely that much of laparoscopy will not trend toward some iteration of robotic influence. The da Vinci surgical system (Intuitive Surgical, Sunnyvale, CA) provides delicate telemanipulation, coalesced with three-dimensional visualization and superior magnification. It has bridged the gap between laparoscopy and open surgery. Nonetheless, a confident understanding of pure laparoscopy is paramount in the event that mechanical malfunction is experienced. Robotic pediatric urologic procedures such as pyeloplasty, ureteral reimplantation, abdominal testis surgery, and partial or total nephrectomy with or without ureteral stump removal are routinely performed at select centers offering robotic expertise. Complex reconstructive surgeries such as appendicovesicostomy, antegrade continent enema creation, and augmentation cystoplasty can be performed but are still in their infancy.