Jeffrey A. Stock

Synchrony in telomere length of the human fetus

Abstract Telomere length, measured by terminal restriction fragments, was examined in tissues from human fetuses of gestational ages estimated as 15–19 weeks. The length of telomeres was similar in most fetal tissues. However, there were significant variations in telomere length among fetuses, with no apparent relationship between gestational age and telomere length. We conclude that synchrony in telomere length exists among tissues of the human fetus. This synchrony is apparently lost during extrauterine life.

CONGENITAL REFLUX NEPHROPATHY AND SEVERE UNILATERAL FETAL REFLUX

PURPOSEnWhen prenatal ultrasound reveals urinary tract dilatation, fetal reflux is suspected. Postnatal voiding cystourethrography confirms the diagnosis. The origin of reflux nephropathy is controversial, and the roles of urinary tract infection and pressure effects of sterile reflux on the developing kidneys are debatable. We evaluate the relationship between sterile reflux and renal scarring.nnnMATERIALS AND METHODSnWe reviewed the records of 100 infants and children seen during a 15-year period in whom fetal reflux had been diagnosed, including 81 with bilateral and 19 with unilateral vesicoureteral reflux. In 12 of the 19 patients voiding cystourethrography revealed unilateral grade IV or V reflux, and they comprise the study group. Split renal function was measured in all 12 patients by radionuclide renal scan shortly after birth and before urinary tract infection developed.nnnRESULTSnIndividual renal function was 0 to 40% in all refluxing renal units. Split renal function was less than 10% in 3 kidneys, 10 to 30% in 5 and 30 to 40% in 4. There were 2 nonfunctioning kidneys. In the remaining 10 kidneys isotope distribution on the nuclear scan indicated decreased renal length and mass. Subsequently 7 patients had breakthrough urinary tract infections while on antibiotic chemoprophylaxis. Nephrectomy, and nephroureterectomy and ureteral reimplantation with or without tapering were performed in 3 and 9 refluxing ureters, respectively. Pathological examination of the 3 nephrectomy specimens revealed severe renal dysplasia consisting of persistent primitive ducts and nests of metaplastic cartilage.nnnCONCLUSIONSnOur study supports the notion that renal impairment associated with severe fetal reflux is present at birth, and it is likely due to congenital dysplasia.

VAGINAL CONSTRUCTION IN CHILDREN

PURPOSEnWe evaluated outcomes in 20 patients 1 to 21 years old who underwent vaginal construction between 1980 and 1996.nnnMATERIALS AND METHODSnA total of 21 vaginal constructions was performed in 20 children using ileum in 13, sigmoid colon in 6, bladder mucosa from a diverticulum in 1 and scrotal skin in 1. The diagnoses included the Mayer-Rokitansky syndrome in 6 cases, micropenis in 5, cloacal exstrophy in 3, penile agenesis in 3, and testicular feminization, classic bladder exstrophy and true hermaphroditism in 1 each.nnnRESULTSnPatients treated with intestinal vaginoplasty had excellent cosmetic results without excessive mucous production or the need for routine dilation. The bladder mucosa vagina achieved good results with periodic dilation. Loss of depth developed in the scrotal skin vagina and it was converted to an ileal vagina. Stenosis at the mucocutaneous junction in 1 patient with a sigmoid and 1 with an ileal vagina was treated with Y-V plasty.nnnCONCLUSIONSnVaginal construction may be performed using isolated bowel segments with excellent results and minimal morbidity. We have found ileum to be the segment of choice in younger patients, while sigmoid colon is preferred for vaginal construction in adolescents. Vaginal construction with isolated bowel segments provides a cosmetic, self-lubricating neovagina with low rates of failure and revision, and without the need for routine dilation.

Müllerian duct remnants: surgical management and fertility issues.

PURPOSEnWe reviewed our experience with mullerian duct remnants, also known as prostatic utricular and mullerian duct cysts, to advance further the understanding of the surgical management of these challenging congenital anomalies. The indications, merits and disadvantages of each surgical approach are presented, and the effects of mullerian duct remnants and their treatment on future fertility are discussed.nnnMATERIALS AND METHODSnWe reviewed the records of 26 patients 1 month to 19 years old with mullerian duct remnants who were seen between January 1984 and October 1998. Clinical presentation included perineoscrotal hypospadias in 10 cases, urinary retention and/or difficult voiding in 7, urinary tract infection in 6, acute scrotum in 2, and recurrent hemospermia and dysuria in 1.nnnRESULTSnOf the 26 patients 13 required surgical intervention for various symptoms and to correct large diverticula. The surgical approach was transvesical transtrigonal in 8 cases, extravesical in 2, perineal in 2 and posterior sagittal in 1. Transurethral fulguration was performed in 2 cases. The initial surgical approach was successful in 11 of the 13 patients. One patient required conversion to a transvesical transtrigonal approach due to inadequate exposure during attempted perineal excision. Two cases treated with transurethral fulguration failed to resolve completely, and in 1 excision was required using the transvesical transtrigonal technique. A total of 13 patients were treated nonoperatively, including 10 in whom the condition was discovered incidentally during screening for perineoscrotal hypospadias. In 5 of the 10 patients urinary tract infection subsequently developed and they were maintained on long-term chemoprophylaxis.nnnCONCLUSIONSnBy tailoring the surgical approach to the type of mullerian duct remnant and the relevant anatomical relationships a high degree of success may be achieved with minimal morbidity.

The rectus myofascial wrap in the management of urethral sphincter incompetence.

Objective u2002To review our experience with a modified rectus/pyramidalis myofascial sling, described more than a century ago for treating refractory urinary incontinence in children with neurogenic sphincteric incompetence.

THE MAINZ II POUCH: EXPERIENCE IN 5 PATIENTS WITH BLADDER EXSTROPHY

PURPOSEnWe report our experience with 5 patients with bladder exstrophy who underwent creation of a Mainz II pouch.nnnMATERIALS AND METHODSnWe retrospectively reviewed the results of the Mainz II pouch as a primary urinary diversion in 2 and a secondary urinary diversion in 3 patients. Each patient underwent multiple surgeries, including osteotomy in 1. All patients were followed postoperatively on a yearly basis.nnnRESULTSnAll patients are continent and the upper urinary tract is stable.nnnCONCLUSIONSnThe Mainz II pouch is appropriate for children born with a small fibrotic bladder, and as a salvage procedure for those who have endured multiple reconstructive procedures and remain incontinent. Furthermore, this procedure deserves serious consideration in children residing in developing countries.

Insurability of Children With Congenital Urological Anomalies

PURPOSEnWe sought to determine life insurance underwriting practices for children diagnosed with multicystic dysplastic kidney or unilateral neonatal hydronephrosis, and evaluate whether management options (observation versus operative intervention) have an influence on such practices.nnnMATERIALS AND METHODSnA questionnaire and history of 1 child with multicystic dysplastic kidney and 1 with unilateral neonatal hydronephrosis were distributed to 348 insurance companies licensed to issue life insurance policies in New Jersey. The medical director of each insurance company was requested to indicate the current underwriting practices for life insurance policies based on these 2 case scenarios, and asked whether observation or operative intervention influenced such decisions.nnnRESULTSnOf the 348 insurance companies licensed to issue life insurance 130 (37.4%) responded, including 5 (3.8%) that did not choose to participate in the study, 56 (43.1%) that did not issue life insurance to children and 69 (53.1%) that completed the questionnaire based on current life insurance underwriting practices. For a child with multicystic dysplastic kidney 10 companies (14.5%) would issue life insurance if treatment involved observation only, while 49 (71%) would do so after nephrectomy. For a child with unilateral neonatal hydronephrosis 19 (27.5%) companies would issue life insurance if treatment involved observation only, while 46 (66.7%) would do so after pyeloplasty.nnnCONCLUSIONSnDespite limited long-term data on and uncertainty about the natural course of multicystic dysplastic kidney and unilateral neonatal hydronephrosis, treatment options offered a child with a congenital urological anomaly may have a significant impact on the ability to obtain life insurance. Children with multicystic dysplastic kidney and unilateral neonatal hydronephrosis can usually obtain life insurance after early operative intervention (nephrectomy and pyeloplasty, respectively), although sometimes at higher cost.

SALVAGE CONTINENT VESICOSTOMY AFTER ENTEROCYSTOPLASTY IN THE ABSENCE OF THE APPENDIX

PURPOSEnWe describe a surgical procedure for a select group of children who had previously undergone augmentation enterocystoplasty, following which intermittent catheterization became more and more difficult or impractical. A new access to the reservoir became necessary, and alternative conduits included the appendix, ureter and tubularized ileum or stomach. Each conduit had its advantages and disadvantages but all required transperitoneal dissection. We report a simple extraperitoneal surgical technique that involves use of the Mitrofanoff and Nissen principles.nnnMATERIALS AND METHODSnA 2x6 cm. flap of the anterior wall, which is usually intestine, of the augmented bladder is raised. The base of the flap is just below the bladder dome. The flap is then tubularized over a 14F catheter and the cystostomy is closed. The bladder is plicated around the base of the tube, similar to the Nissen gastroesophageal fundoplication. The plication extends and covers the proximal 3 cm. of the tube. Intraoperative bladder distention is performed to confirm the competence of the continence mechanism. The distal part of the tube is then anastomosed to the inverted umbilical skin or to a tubularized abdominal wall skin flap.nnnRESULTSnThe aforementioned technique was used in 5 children 3 months to 6 years old. Earlier augmentation ileocystoplasty and bladder neck reconstruction had been performed in 4 children. The appendix was unavailable for a Mitrofanoff vesicostomy in all cases. This procedure was also performed on a 13-year-old boy with severe myogenic detrusor failure, due to posterior urethral valves, and a bladder capacity of 700 ml. There was no significant surgical morbidity and all children are dry between clean intermittent catheterizations 1 to 5 years postoperatively (mean 3.3).nnnCONCLUSIONSnUse of the Mitrofanoff and Nissen principles proved to be simple and reliable, and avoided secondary intraperitoneal exploration and use of bowel in these select cases.

Septic arthritis secondary to vesicoureteral reflux into single ectopic ureter.

Abstractu2002A 3-week-old male infant, born full term without complication, developed septic arthritis of his left shoulder. His joint fluid, blood, and bone marrow were all positive for Escherichia coli. Urinalysis demonstrated pyuria. Urine culture obtained after one dose of ceftriaxone and several doses of nafcillin was negative. Work-up revealed a refluxing, right single ectopic ureter with severe hydroureteronephrosis and a non-functioning ipsilateral kidney. After appropriate management of the musculoskeletal infection, he underwent a right nephroureterectomy. Coliform septic arthritis is exceedingly rare in children, with only a few cases reported. We report the first case of septic arthritis with anomalous genitourinary tract developement as the source of bacterial seeding. This report re-emphasizes the need to screen the urinary tract in all cases of pediatric gram-negative sepsis.

URETERONEOCYSTOSTOMY: TO DRAIN OR NOT TO DRAIN

PURPOSEnIndications for the use of external abdominal drains after ureteral reimplantation are not well defined. We determine the nature of the drainage fluid as well as the current use of drains by pediatric urologists.nnnMATERIALS AND METHODSnWe prospectively evaluated 15 consecutive patients 7 months to 19 years old who underwent unilateral or bilateral intravesical ureteroneocystostomy for primary vesicoureteral reflux. All patients were treated with a urethral Foley catheter and closed suction Jackson-Pratt abdominal drain. Fluid from the Jackson-Pratt drain and Foley catheter was analyzed for urea and creatinine on postoperative day 1, and compared to serum values. The Foley catheter was removed after the urine became clear, and the Jackson-Pratt drain was removed after drainage was 5 ml. or less for 12 hours. In addition, a questionnaire was distributed to 268 pediatric urologists to determine current practice regarding the use of routine postoperative drains.nnnRESULTSnUrea and creatinine from the Jackson-Pratt drains in all 15 patients were consistent with serum values. The Foley catheter and Jackson-Pratt drain were removed an average of 3 and 4 days postoperatively, respectively. There were 186 responses from the 268 questionnaires distributed (69.4%). Of the pediatric urologists surveyed 70.4% performed intravesical ureteral reimplantation exclusively, 5.9% extravesical reimplantation exclusively and 23.7% both techniques. Of the group surveyed 73.1% placed external abdominal Jackson-Pratt or Penrose drains, although 26.5% of those who routinely used external drains believed that they were probably unnecessary. Of the physicians who placed drains 53.7% believed that the drainage fluid had some component of urine.nnnCONCLUSIONSnIn our small prospective study group we demonstrated that external abdominal drainage fluid is consistent with serum despite the popular belief that it may have some component of urine. The gynecological literature has shown repeatedly that there is no increase in morbidity after radical hysterectomy and pelvic lymph node dissection when no external abdominal drains are used. Although to our knowledge there are no previous reports of drain use after ureteral reimplantation, 26.9% of pediatric urologists currently do not place external abdominal drains with no apparent increase in morbidity. Larger prospective cohorts with long-term followup are needed to address adequately the issue of whether drains are needed after uncomplicated ureteral reimplantation.