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Purpose Previous studies have hypothesized that cloacal exstrophy may be caused by errors early in embryological development related to monozygotic twinning. This study reports the prevalence of twins in a large cohort of patients with cloacal exstrophy. Methods Patients with cloacal exstrophy treated 1974–2015 were reviewed for reports of multiple gestation or conjoined twinning. The genetic sex of the patient and their twin, and any mention of anomaly in the twin were recorded. Neither placental exam nor genetic testing results were available to definitively determine zygosity. Results Of 71 patients, 10 had a live born twin (14%), all of whom were of the same genetic sex as the affected patient. One additional patient's twin suffered intrauterine fetal demise, and another patient had a conjoined heteropagus twin. None of the twins were affected by exstrophy–epispadias complex. The rate of twin birth in this cohort was 4.4–7.7 higher than that reported by the Centers for Disease Control in the general population time period ( P Conclusions The highly significant prevalence of same-sex twin pairs within this cohort supports the hypothesis that the embryogenesis of cloacal exstrophy may be related to errors in monozygotic twinning. Level of evidence 2b

Purpose Cloacal exstrophy is associated with multiple comorbidities that affect growth. This report describes long-term growth outcomes in a large cohort of patients with cloacal exstrophy and explores associated comorbidities. Methods Records of 71 patients with cloacal exstrophy who were treated between 1974 and 2015 were reviewed, and 62 patients with growth data from 2 to 20years of age were included. Genetic sex, gender of rearing, and all heights, weights, and comorbidities were noted for each patient. Height-for-age, weight-for-age, and body mass index z-scores (HAZ, WAZ, and BMIZ) were determined using US Centers for Disease Control 2000 growth data, and average patient z-scores were calculated. Results There were 904 height and 1301 weight measurements available for 62 patients. 31 were genetically 46,XY, 21 of whom underwent gonadectomy in infancy and were raised female. 46,XX patients, 46,XY male patients, and 46,XY female patients all had median HAZ and WAZ substantially lower than the general population, with median HAZ less than −2, while maintaining normal BMIZ. Short bowel syndrome and enterocystoplasty with intestine were associated with lower z-scores for all parameters. Conclusions Patients with cloacal exstrophy have significant multifactorial long-term growth failure. These benchmark data can be used to further optimize management. Level of evidence: 2b

The new technique of complete primary repair of bladder exstrophy has offered the promise of improved bladder functional outcomes and yet longitudinal followup in patients with exstrophy who have undergone this form of closure is sparse. We present our median 5-year data on patients who have undergone complete primary repair of bladder exstrophy.The records of patients who underwent complete primary repair of bladder exstrophy were analyzed retrospectively. Patients were followed prospectively using case report forms, radiological and clinical data, and semistructured parental and patient interviews. We reviewed parameters including clinical events, scarring on renal scan, vesicoureteral reflux, surgical procedures, urodynamic studies, urinary and fecal continence status, and episodes of urinary tract infection.From 1994 to 2007 complete primary repair of bladder exstrophy was performed in 32 patients. Of these patients 28 underwent closure within 72 hours of life, including 20 males and 8 females, and 4 underwent it after 72 hours at ages 7 days to 12 months. One patient underwent complete primary repair of bladder exstrophy elsewhere. Median followup was 5 years (range 1 to 13). In 32 patients a total of 193 surgical procedures were done, of which 40% were minor endoscopic cases. Six of 23 males (26%) underwent bladder neck reconstruction at a median age of 4.5 years (range 4 to 10). Two boys and 1 girl received Deflux injection to the bladder neck at ages 3 to 5 years. To date no patient has undergone bladder augmentation. Nine patients (28%) had 1 to 4 episodes of pyelonephritis and 6 had cortical defects on renal scan. A total of 30 patients were voiding or incontinent via the urethra. One patient performed clean intermittent catheterization via the urethra and 1 performed it via appendicovesicostomy. All 4 children who were at least 6 months from bladder neck reconstruction after complete primary repair of bladder exstrophy had urinary continence periods of 2 to 3 hours or greater. Parents did not report any fecal incontinence or soiling in children older than 4 years. Relative to males the females had better urinary continence and a decreased need for bladder neck reconstruction.Complete primary repair of bladder exstrophy has been shown to be safe and efficacious. Prospective followup in this small number of evaluable patients reveals that continence periods of more than 2 hours are possible in patients after bladder neck reconstruction. Long-term followup in patients after complete primary repair of bladder exstrophy continues to be necessary to establish the long-term effects of this procedure.

We assessed bladder growth and dynamics following complete primary repair of bladder exstrophy (CPRE) compared to the staged approach.We reviewed the records of 16 boys and 7 girls who underwent CPRE within 3 days of life from 1996 to 2004 and compared them to the records of 8 boys and 6 girls treated with a staged repair from 1979 to 1996. Screening methods included voiding cystourethrogram, radionuclide cystogram and urodynamic study. We estimated growth curves for bladder capacity following repair in each group, and compared percent predicted bladder capacity (PPBC), compliance and detrusor overactivity between the CPRE and staged repair groups following bladder neck reconstruction.Bladder capacity in the staged repair group was 69.8 ml (95% CI 46.7-104.4) immediately after bladder neck reconstruction and increased by 15.0% per year thereafter (95% CI 6.2-24.5, p = 0.002). In the CPRE group bladder capacity was 29.0 ml (95% CI 21.3-39.5) initially and increased by 28.9% per year thereafter (95% CI 17.4-41.5, p0.001). PPBC started at 45.6% (95% CI 35.7-55.5) and increased 1.2% per year (95% CI -1.1-3.5, p = 0.29) following repair for all genders and surgery groups. Compliance was 124.4% (95% CI 22.6-310.7, p = 0.01) greater in the CPRE group at all times following repair. Detrusor overactivity was present in 0 of 19 patients in the CPRE group and 6 of 13 (46%) in the staged group (exact p = 0.002).Within the CPRE group bladder stability was universal, and sphincter electromyography was normal suggesting no neuromuscular compromise of the pelvic floor. At early followup, our results suggest that PPBC is equivalent irrespective of gender or management. Further objective evaluation is needed in both groups.

Purpose: Complete primary repair of bladder exstrophy (CPRE) represents a paradigm shift from the staged approach for surgical management. We present early clinical outcomes in our patients following CPRE. Materials and Methods: From 1996 to 2004 all newborns with bladder exstrophy were treated with CPRE within 48 hours of birth. We reviewed parameters including transfusions (packed red blood cells), urethral meatal position, complications, findings on renal ultrasound and renal scan, and post-CPRE procedures and infections. Results: A total of 16 boys and 7 girls were treated with CPRE. Followup ranged from 8 to 96 months. Twelve boys (75%) and 2 girls (29%) received packed red blood cells at CPRE (p = 0.066). The tubularized urethral plate could not be brought to the penile tip, resulting in hypospadias in 9 of 11 boys (82%) with the running suture technique and only 1 of 5 boys (20%) with the interrupted technique (p = 0.036). Of the 23 patients 6 had a total of 8 complications after CPRE. Vesicoureteral reflux was present in 17 of 23 patients. After CPRE a total of 93 endoscopic/surgical procedures (median 4, range 0 to 16 per patient) were performed. Five patients had 1 to 4 episodes of pyelonephritis, 16 of 23 had 1 or more episodes of asymptomatic bacteriuria and 5 had cortical defects on renal scan. Conclusions: We recommend that urethral closure during CPRE be performed with interrupted suture technique to prevent hypospadias. An aggressive approach should be taken toward reflux in the setting of urinary infection. Consideration should be given for repair of all aspects of the defect during CPRE, including bilateral ureteral reimplantation.

Purpose: We reviewed our experience with buccal mucosa grafts for reconstructing difficult female urethral problems.Methods and Methods: Since 1994, we have used a buccal mucosa graft to reconstruct the urethra in 7 girls 3 to 13 years old. The underlying pathological condition was a fibrotic urethra after previous operations for cloacal exstrophy, cloacal malformation, iatrogenic urethral stricture, and multiple false passages in a previously reconstructed urethra of vaginal mucosa that made clean intermittent catheterization difficult. A full-thickness buccal mucosa graft was tubularized in situ as the neourethra to the base of the clitoris. In patients with cloacal exstrophy and cloacal malformation the bladder neck and urethra were widely exposed transabdominally by splitting the pubic symphysis. The fibrotic mucosa was excised and the tubularized buccal mucosa graft was wrapped with periurethral tissues. Other patients underwent transvaginal surgery in the prone position and the graft was covered wit...

Purpose: The aim of this study was to evaluate the outcomes of reconstruction of all cloacal exstrophy cases seen by the authors from 1974 to 1999. Methods: A retrospective chart review and personal follow-up was conducted on all 50 patients, who ranged in age from newborn to 35 years. Thirty-eight were secondary cases, 12 were primary (no previous surgery). Twenty-eight (56%) were genetic boys; 6 were raised as boys and 22 as girls. Forty patients underwent extensive reconstructive surgery; 6 await reconstruction, and 4 were seen only in consultation. For urinary continence, 21 had bladder neck narrowing, 7 received a bowel nipple, and 12 had a Mitrofanoff catheterizable conduit on the abdominal wall, using tapered intestine in 8, ureter in 3 and the appendix in 1. Four patients had a urostomy. Bladder augmentation was performed in 35 patients (18 with stomach, 11 small bowel, and 6 with both). Twenty-five patients had pull-through of their colon to the perineum. Thirty-two have had a vagina constructed. Forty-seven of the 50 patients had spinal cord tethering, and most underwent neurosurgical release. Results: Overall survival rate was 98%; 1 patient died preoperatively at another institution. Acceptable bowel continence was achieved with enema washouts in 19 of 25 pullthroughs; 4 failed and were reversed. One case is too recent to judge. Of 40 reconstructed cases, 31 were dry, 3 had slight leakage, and 1 is too early to evaluate. Five had enough leakage to require further surgery. Conclusions: The once hopeless anomaly of cloacal exstrophy is treatable with extensive reconstructive surgery. Continence of urine (mainly by catheterization) and stool (mainly by enema washouts) is achievable in most. The hindgut, including the cecum and the usually rudimentary distal colon, should be saved and used as colon, not for urinary or vaginal reconstruction. In genetic boys, the authors continue to believe that gender assignment should depend on the likelihood for reconstructing an adequate phallus. J Pediatr Surg 36:68-75. Copyright © 2001 by W.B. Saunders Company.

Presentation of Case A 32-day-old girl was admitted to the hospital because of a retroperitoneal mass. The patient had been born to a 31-year-old woman (gravida 4, para 3) after a 39-week gestation. A prenatal ultrasonographic study, performed at four months of gestation, showed an abdominal mass. An ultrasonographic study performed on the first day of life showed no increase in the size of the mass. The alpha-fetoprotein level was 35,000 IU per milliliter. A computed tomographic (CT) scan of the abdomen obtained the next day, after the oral and intravenous administration of contrast material (Figure 1), showed a mass, . . .

Purpose: The exstrophy-epispadias complex represents a spectrum of anomalies with variable implications for upper and lower urinary tract function. Successful treatment of incontinence in this population is challenging and often elusive. To date few studies have focused on urodynamic findings specific to primary epispadias.Materials and Methods: We retrospectively reviewed the records of patients presenting with primary epispadias in the absence of bladder exstrophy. We identified 18 boys and 12 girls, of whom 16 underwent formal urodynamic evaluation before (5), after (6) or before and after (5) surgical narrowing of the bladder neck. Bladder capacity was recorded as percent of expected capacity for age. Bladders were considered hypertonic when end filling pressure was greater than 20 cm. water. End filling pressure was defined as the pressure at which urinary leakage was noted or the patient had discomfort. Uninhibited contractions were considered significant when the amplitude was greater than ...

Purpose: The combined findings of cryptorchidism and hypospadias often indicate the existence of an intersex state. Testicular maldescent and incomplete tubularization of the urethral plate occur in a spectrum with the severity of the 2 processes likely dependent on the degree of pathophysiology in the androgenic hormonal axis. The incidence of intersexuality in children with undescended testes, hypospadias and otherwise nonambiguous male genitalia has been reported to be 27%. Although the likelihood of genotypic or gonadal ambiguity has previously been associated with meatal position in this population, to our knowledge our study is the first to evaluate the incidence of intersexuality relative to whether the undescended testis is palpable or nonpalpable.Materials and Methods: The database at our hospital was searched for all cases of undescended testes (2,105) and hypospadias (1,057) between 1982 and 1996. Radiographic, histological and karyotypic data were compiled for all patients presenting w...

Purpose: The intestinal augmented bladder has an increased propensity to form urinary calculi. Predisposing risk factors include chronic bacteriuria, urinary stasis and mucus production. Gastric reservoirs have negligible mucus production and the ability to acidify the urine. We determined whether they also have a decreased incidence of stones.Materials and Methods: We retrospectively reviewed the records of all patients undergoing augmentation cyptoplasty (215) or creation of a freestanding reservoir (44) between May 1976 and March 1996. Of these cases 83 were augmented with stomach and 179 were constructed from other intestinal segments, (that is ileal, ileocecal, sigmoid and/or a combination of these). Presenting diagnosis, patient age, gender, additional surgical procedures, interval to stone formation and calculous composition were recorded. Patients were excluded from study when there was less than 1 year of followup, as were those with renal or pre-augmentation bladder calculi.Results: Ston...

Caustic injury to the upper aerodigestive system with scarring of the pharynx, hypopharynx, and esophagus is a challenging reconstructive problem. The authors report on seven patients who required total esophageal replacement from the pharynx to the stomach. Injury occurred from alkali in six and acid in one. Age at injury ranged from 14 months to 14 years (mean, 4.5 years.) in five boys and two girls. Time from injury to esophageal replacement was 6 months to 10 years (mean, 3.5 years). Two required pharyngeal reconstruction before and one after esophageal replacement. Six patients had an isoperistaltic right or transverse colon interposition. One who had gastric necrosis had an ileo-right colonic substernal interposition with creation of a jejunal reservoir. Results of barium swallows showed intact anastomoses in all patients. There were no leaks. Most had some degree of mild to moderate aspiration, and one who had left vocal cord paralysis had initially massive aspiration. Three patients currently eat regular diets; four eat but still require supplemental tube feeds. The authors conclude that children who have hypopharyngeal scarring and obliterated esophageal inlet can undergo a successful colonic esophageal replacement with high proximal pharyngocolic anastomosis.

Seventy-five years ago, a 7-year-old girl underwent the first successful ligation of a patent ductus arteriosus. This procedure transformed her health and was a milestone in the development of cardiac surgery. The operation was performed by Dr. Robert E. Gross, then the surgical chief resident at Boston Children's Hospital, who went on to have a distinguished career in pediatric surgery. The patient is now a great-grandmother and the oldest known survivor of cardiac surgery.

Purpose: We attempted to develop a rational and consistent scheme for surgical reconstruction in patients with genitourinary rhabdomyosarcoma.Materials and Methods: We reviewed the records of 35 patients with resectable genitourinary rhabdomyosarcoma treated from 1970 to 1993.Results: Primary sites included bladder in 11 cases, prostate in 13, vagina/uterus in 9 and pelvic tumors of uncertain origin in 2. A total of 33 patients underwent surgery, including partial and radical cystectomy in 17 (bowel conduit diversion in 10, continent urinary diversion in 6 and ureterosigmoidostomy in 1). Overall 30 of the 33 surgical patients are free of disease 4 months to 24 years after diagnosis.Conclusions: A nonrefluxing colon conduit is appropriate at cystectomy. Continent diversion fashioned from the original conduit may be planned as the patient achieves a durable disease-free status.

Fibroepithelial polyps are benign mucosal projections that can be found throughout the urinary system. We review our experience with fibroepithelial polyps of the ureteropelvic junction in children to define more clearly this entity and its outcome following treatment.We reviewed the records of all children with fibroepithelial polyps causing ureteropelvic junction obstruction treated at our institution between December 1967 and February 2002.Nine patients 6 weeks to 9 years old had 11 ureteropelvic junction obstructions secondary to fibroepithelial polyps, representing a 0.5% incidence of all ureteropelvic junction obstructions seen during that period. The majority of the patients were male (89%) and had obstruction on the left side (78%). Only 22% of the patients had a diagnosis of obstructing polyps suggested preoperatively. All patients underwent dismembered pyeloplasty but 1 required subsequent nephrectomy due to progressive loss of renal function. All lesions were characterized microscopically by polypoid projections of fibrous tissue covered by epithelium and demonstrating varying degrees of inflammation. No recurrences were seen during a mean followup of 44 months.Fibroepithelial polyps are rare lesions that cause ureteropelvic junction obstruction in children, primarily in males and on the left side. Filling defects were diagnosed preoperatively in 22% of the patients in this series. Excision and dismembered pyeloplasty were curative, and recurrences were not observed. The etiology of this disease remains obscure.

Obstetrical sonography has helped diagnose and define the features of some congenital malformations and tumors. We present five fetal neuroblastomas detected by routine prenatal sonography. All were adrenal tumors diagnosed between 26 and 39 weeks gestation. All 5 tumors were completely resected postnatally and the patients have remained disease free from 2 months to 10 years after resection without adjuvant therapy. A literature review collated 16 other cases of fetal neuroblastoma detected by sonography between 29 and 38 weeks gestation. These cases included 1 cervical, 1 thoracic, and 14 adrenal tumors. Thirteen neonates had Evans stage I or II tumors, and three had more advanced disease. Eleven mothers did not have hypertension or preeclampsia during the pregnancy, and the neonates all had stage I or II disease. Four mothers had hypertension or preeclampsia. Three of these neonates had stage IV or IVS disease with liver metastases, and all three had fetal hydrops. Review of the congenital neuroblastoma literature documented 71 cases diagnosed soon after birth, and several of these cases had unusual features that could have been detected by prenatal ultrasound. Four of the tumors were so large that dystocia resulted and fetal dismemberment was required for delivery. Eight of the tumors metastasized to the placenta, and 1 metastasized to the umbilical cord with subsequent fetal death. We conclude that fetal neuroblastoma can be diagnosed by prenatal sonography. Accurate staging is difficult by sonography, but in mothers with no preeclampsia symptoms the chance of widely disseminated disease is small.(ABSTRACT TRUNCATED AT 250 WORDS)

Solid mediastinal masses in infancy and childhood occur most frequently in the posterior mediastinum. From 1972 to 1989, 63 patients presented with a posterior mediastinal mass. The median age at diagnosis was 6 years (range, 1 day to 26 years). Thirty patients were female. Forty-five percent of the patients presented with respiratory symptoms or chest pain; 13% had neurologic symptoms, one half of which were related to spinal cord compression; and 5% had a palpable mass. In 32% of patients the mass was an incidental finding. The tumors were of neurogenic origin in 89% of patients, of which neuroblastoma was the most common. Of all patients with posterior mediastinal masses, 60% had malignant tumors. Median follow-up for 62 of 63 evaluable patients was 45 months (range, 1 to 289 months). One patient was lost to follow-up. Of the 62 patients followed, 84% are alive and free of disease. All but 4 of the 32 patients with neuroblastoma are alive and free of disease with a median follow-up of 73 months (range, 7 to 289 months). Patients with neuroblastoma who were diagnosed in the first year of life had a significantly better survival pattern than those presenting after the first year. There were seven deaths in the series: four from neuroblastoma, two from primitive neuroectodermal tumor, and one from malignant schwannoma. Preoperative diagnostic evaluation of a posterior mediastinal mass should include posteroanterior and lateral chest roentgenograms, and either CT or MRI of the chest and abdomen to assess the extent of the mass.(ABSTRACT TRUNCATED AT 250 WORDS)

A major goal of bladder exstrophy management is urinary continence, often using bladder neck reconstruction. We report our experience with bladder neck reconstruction after complete primary repair of exstrophy.Patient history, ultrasound, voiding cystourethrogram, examination using anesthesia and urodynamics were performed during a prospective evaluation. Continence was assessed using the International Children's Continence Society classification and the dry interval. Bladder capacity was measured by examination using anesthesia, voiding cystourethrogram and/or urodynamics. Urodynamics were also done to assess bladder compliance and detrusor muscle function.From 1994 to 2010 we treated 31 male and 15 female patients with bladder exstrophy after complete primary repair of exstrophy. Of patients 5 years old or older bladder neck reconstruction was performed after complete primary repair in 9 of 21 males (43%) and in 3 of 11 females (27%) at a mean age of 6.3 and 8.1 years, respectively. By the International Children's Continence Society classification 6 of 12 patients (50%) were continent less than 1.5 years after bladder neck reconstruction and 2 of 9 (23%) were evaluable 1.5 years or greater after reconstruction. Median bladder capacity was 100 ml before, 50 ml less than 1.5 years after and 123 ml 1.5 years or greater after bladder neck reconstruction. Three males and 2 females emptied via an appendicovesicostomy. Two boys underwent augmentation.In our experience most patients with bladder exstrophy require bladder neck reconstruction after complete primary repair of exstrophy. The need for reconstruction is more common in males. Our rates of bladder neck reconstruction after complete primary repair of exstrophy and of continence after bladder neck reconstruction are similar to those in other reports.

Since 1985, 7 children 2 to 17 years old with urinary incontinence from cloacal exstrophy underwent bladder augmentation and creation of a small bowel nipple at the bladder outlet. There were 3 genetic female and 4 genetic male patients being reared in the female role. In 3 early cases augmentation was done with small bowel, while gastric augmentation was used in 3 later cases and 1 had both types. Six patients became completely dry and they empty by intermittent selfcatheterization. In the youngest patient the operation failed because the nipple became infarcted and disappeared. Reoperation will be performed after at least 1 year has passed. In cloacal exstrophy if there is insufficient local tissue to create a bladder neck and urethra, a reversed, catheterizable bowel nipple may be an option to consider.

A personal experience with 105 cloacal malformations is described, showing the wide range of anatomy that can occur with the urogenital sinus, the vagina(s), and the rectum, as well as in the external features of the perineum. Follow-up is presented on 98 patients, excluding 4 who died preoperatively, 2 who are preoperative, and 1 lost to follow-up but never repaired. Fifty-seven patients had vesicoureteral reflux. Data are given on urinary control, bowel control, and sexual function for those who are now adults. Two have had babies. Operation often requires multiple positions, including the posterior sagittal approach, laparotomy, and lithotomy position. Isolated rectal pull-through should never be performed in these patients, because the urogenital aspects of the malformations are the most difficult to repair and are the most life-threatening to the patient. Six recent cases are presented to illustrate the breadth of the cloacal spectrum. In most of these patients a satisfactory functional result can be achieved for urinary and bowel control as well as sexual function.

21-hydroxylase deficiency (P450 CYP21) accounts for 90% of cases of congenital adrenal hyperplasia (CAH), which is associated with abnormally low cortisol and high production of androgen precursors and is the most common cause of ambiguous genitalia. Increased androgen causes in utero virilization of the fetus, consisting of clitoral enlargement, an urogenital sinus, and labioscrotal enlargement and fusion. This is the first case in an experience covering more than 30 years, of a 46,XX patient raised as a boy. The authors report a case of a Pakistani patient born of a consanguineous union, who came to medical attention at age 3 because of severe genital ambiguity; genetic analyses showed that the child was a compound heterozygote for CAH. The surgical management of this patient consisted of (1) staged hypospadias repairs preceded by testosterone therapy, (2) creation of a bladder graft neourethra, (3) removal of mullerian structures, (4) correction of bifid prepenile scrotum, and (5) insertion of testicular prostheses. The commitment to raise a 46,XX child as a boy is a very rare event. With a series of staged complex surgical procedures and careful steroid replacement, normal secondary sexual characteristics can be achieved in these children.

Background: Patients with cloacal malformations at birth usually require multiple surgical procedures to correct their anatomic defects. In addition, many also have associated Mullerian anomalies. Those who conceive after repairs invariably are considered “high-risk” pregnancies and are considered poor candidates for maintaining multiple gestations. Further, because of the nature of their defects and their repairs, following such patients with multiple gestation presents unique challenges. Case: A 29-year-old multipara conceived triplets and delivered at 30 weeks with a good maternal and neonatal outcome. Conclusions: Patients with repaired cloacal abnormalities present unique challenges and risks compared to the general population with regard to the risks of multiple pregnancies.

Congenital anomalies of the thoracic duct are rare, poorly characterized, and difficult to manage. The spectrum of pathophysiologic perturbations, presenting symptoms, ra- diographic findings, and interventions performed in 4 pa- tients are shown. Accurate anatomic delineation of the mal- formation was only possible by direct injection contrast lymphangiography. Therapies tailored to address the ana- tomic aberrations included intralesional sclerotherapy, sur- gical excision and ligation, lymphovenous anastomosis, and omentalinterposition to interrupt dysfunctionalcol l ONGENITAL ANOMALIES of the thoracic duct are extremely rare, poorly characterized, and dif- ficult to manage. The functional, rheologic, and hemo- dynamic properties of the central lymphatics have not been well elucidated. Some clinical manifestations of central lymphatic dysfunction such as chylous pleural effusions and chylous ascites often are considered diffi- cult or impossible to eradicate. Imprecise nonanatomic therapies such as pleurodesis, prosthetic pleuro-perito- neal or peritoneovenous shunts, or even radiation therapy often are used. The lymphatics of the lower extremities and lower torso join with those of the bowel mesentery in the cisterna chyli, which lies in the lumbar prevertebral plane behind the aorta and inferior vena cava. Confluent with the cisterna chyli, the thoracic duct ascends in the tho- racic prevertebral plane, behind the esophagus and aorta. The lymphatic drainage of the mediastinum, upper torso, arms, head, and neck join the central lymphatic channels via thoracic tributaries. The thoracic duct proper empties into the central venous system near the junction of the left internal jugular vein and subclavian vein. The acces- sory thoracic duct empties in a similar fashion on the

We treated 33 patients 6 months to 19 years old with penile chordee without hypospadias from 1966 to 1990. In all cases the penile shaft was degloved, the urethra was widely mobilized and chordee was resected. The shaft skin then was closed, usually using Byars' flaps to shift some preputial skin ventrally. This method sufficed to straighten the penis in 10 patients. In 3 patients urethral mobilization plus placement of a dermal graft to the shaft accomplished straightening of the penis. A total of 20 patients also required lengthening of the penile urethra. Lengthening was done with a graft taken from the prepuce in 14 patients, bladder in 2 and arm in 4. Four of those patients also required a dermal graft to the shaft. There were 8 complications in 7 patients, including anastomotic stenosis, ballooning of a graft, balanitis xerotica obliterans in a graft, urethral fistula and persistent chordee. Reoperation corrected each complication. All end results were satisfactory.

Urodynamic study was performed in patients with exstrophy to determine the effect of bladder neck reconstruction and the ability to achieve normal urodynamics following surgery.A total of 30 exstrophy cases at different stages of reconstruction were retrospectively reviewed in terms of bladder capacity, compliance, stability and presence of detrusor contractions following urodynamic study.Bladder capacity increased from a third predicted volume for age to half after reconstruction. Approximately 80% of patients had compliant and stable bladders before bladder neck reconstruction. Following bladder neck reconstruction approximately half of the patients maintained normal compliance with a smaller number maintaining normal stability. A quarter of patients maintained normal filling dynamics following bladder neck reconstruction, and 19% maintained normal filling and voiding dynamics after reconstruction.The majority of closed exstrophy bladders have normal filling dynamics before bladder neck reconstruction. Compliance and stability are impaired following bladder neck reconstruction. Approximately 25% of patients with exstrophy may maintain normal detrusor function following reconstruction. However, less invasive alternatives to the Young-Dees-Leadbetter bladder neck reconstruction should be sought.

Physicians who see infants and children provide the first line of defense in diagnosing malformations and conditions that may require surgical care. In this article, most of the problems in the rectal and perineal area with which primary care pediatricians should be acquainted are discussed. Prompt diagnosis and referral to an appropriate surgeon usually ensures a good outcome.

Methods: From 1975 to 1996 a urethra was constructed using tubularized anterior vaginal wall covered with a buttock flap in 40 female patients in whom urinary incontinence was associated with a short or absent urethra. The underlying pathology included bilateral single ectopic ureters, cloacal malformation, urogenital sinus malformation, previous failed surgery of the urethra, severe trauma, myelodysplasia, female hypospadias, ectopic ureterocele with destruction of the urethra, congenital epispadias, bladder exstropny, and previous hysterectomy and vaginectomy for clear cell carcinoma caused by intrauterine exposure to diethylstillbestrol. Other reconstructive procedures in these patients included bladder neck narrowing from above in 33 patients, ureteral reimplantation in 35, and bladder augmentation in 21. In the prone position, a tube of vaginal wall was used to create a urethra when it was absent or lengthen the urethra if it was too short. The neourethra was extended up to the base of the clitoris, using a multilayered closure of soft tissue over it distally with introital muscle and adjacent mucosa. The proximal neourethra was covered with a buttock flap. Results: All patients were originally wet. Thirty-four are now dry. Four patients have minimal stress incontinence. Two patients have urinary diversions. The majority (n=25) void normally. Fourteen patients self-catheterize to empty their bladders. One patient wears an external appliance on the abdominal wall for collection of urine. Complications included: creating stress incontinence in one patient who had been previously dry by extracting a stone from the bladder, necrosis of a buttock flap from applying a tight perineal dressing, fistula requiring surgical closure in three patients, and slippage of the buttock flap in one patient. Conclusion: This operation is a useful method to correct diverse congenital and acquired pathology that causes incontinence in girls.

To our knowledge nephrogenic adenoma, an unusual benign metaplastic lesion of the urothelium, has not been reported previously following urethral reconstruction using bladder mucosa. Histopathological features, etiological factors and clinical features are discussed.We reviewed the records of 117 patients who underwent hypospadias repair with free grafts of bladder mucosa from 1982 to 1995.Five children 6 to 11 years old and a 39-year-old man had nephrogenic adenoma of the urethra. Hematuria or dysuria was the usual reason for presentation. The lesions were located at the site of anastomosis between the graft and proximal urethra in 3 cases and the distal graft in 2, and they involved the whole graft in 1. Successful treatment consisted of endoscopic resection of the polyps in 4 patients, while open surgical correction was performed in 2, requiring complete excision of the previous graft in 1 and segmental resection in 1.The gross appearance of the lesion may be confused with that of transitional cell carcinoma or clear cell adenocarcinoma of the urethra, necessitating biopsy to rule out malignancy. Endoscopic management is possible for small tumors, although severe nephrogenic adenoma involving most of the reconstructed urethra may require complete resection of the graft and creation of a new urethra.

Continent urinary diversion has become increasingly important for treating childhood urinary tract pathology that cannot be managed by direct reconstructive techniques. We review our 9-year experience with continent diversion.Since 1986 continent diversions were created in 74 patients 3 to 38 years old (mean age 13.7). The underlying pathological condition was the exstrophy/epispadias complex in 34 patients, neurological disorders in 23, malignancy in 13 and other congenital anomalies in 4. Followup averaged 5.2 years after the last procedure. Nonbladder reservoirs in 39 patients (53%) were fashioned from ileocolic (17), colic (7), gastrocolic (6), sigmoid (3), gastrosigmoid (2), ileosigmoid (2), ileal (1) and gastroileac (1) segments. When possible, the native bladder was incorporated into the reconstructive strategy. A total of 26 patients underwent bladder augmentation with intestine or stomach, including ileal (11), gastric (8), sigmoid (3), gastroileac (2) and ileocolic (2) segments. Nine other patients did not require bladder augmentation. Continence mechanisms were a flap valve (Mitrofanoff principle) in 50 patients, nipple valve in 15 and ileal plication (Indiana pouch) in 9. When the Mitrofanoff principle was used with a native bladder reservoir in 30 cases, outlet resistance was altered by bladder neck division (15), fascial sling placement (6) or Young-Dees-Leadbetter bladder neck reconstruction (2). In the remaining 7 patients the bladder neck remained intact.Excellent continence was obtained. The Mitrofanoff principle initially provided continence in 41 patients (82%). Six of the 9 incontinent patients were dry after a single revision. A total of 13 patients (87%) with nipple valves and 7 (78%) with Indiana pouches were dry, and the remaining 5 were cured after a single revision. Ultimately continence was achieved in 71 of the 74 patients (96%) after a maximum of 2 operations. Of the 48 complications in 29 patients the most common were difficulty in catheterizing (11), stones (11), infection (8) and upper tract deterioration (4).Many options exist for reconstructing complex anomalies. Choices must be individualized based on patient anatomy. The dry state may be achieved in most cases without resorting to a bag on the abdomen.

Three unique cases of urethral duplication, often referred to as congenital posterior urethral perineal fistulae, are presented. Presenting signs and symptoms, evaluation, and treatment are discussed, and the literature is reviewed. Although labeled as a fistula in the literature, this entity most likely represents a distinct type of urethral duplication. A functional dorsal urethra separates it from the other forms of urethral duplication.

In the most severe form of congenital adrenal hyperplasia in females, the vagina is located above the level of the external urethral sphincter. Sixteen of these patients had reconstruction with a pull-through technique described previously. Nine are now adults; six have coitus and three do not. Five are young and sexually immature. Two have been lost to follow-up.

Exstrophy of the cloaca, a rare anomaly, is among the most complex malformations of infancy. Features include omphalocele, imperforate anus, and exstrophy of two hemibladders, between which lies the everted cecum. A small colon ends blindly in the pelvis, and the terminal ileum often prolapses out of the exposed cecum. Formerly, most of these infants died. From 1974 to 1992, 20 of these patients were treated, the majority in the past decade. Six were managed primarily; 14 were referred after prior surgery elsewhere. Current treatment of a newborn with cloacal exstrophy includes closure of the omphalocele, separation of the gastro-intestinal tract from the hemibladders, and closure of the two hemibladders as a single viscus. The colon can be pulled through in some cases, either immediately or later (6 cases). The bladder must be augmented in all cases to provide adequate volume and compliance; stomach is ideal for this (10 cases). A bladder continence mechanism must be constructed by narrowing the outlet (9 cases), or inserting a reversed small bowel nipple (7 cases). Intermittent catheterization is needed for the patient to empty the reconstructed bladder. Although two thirds of these patients are genetic males, they should be raised as females because they lack adequate tissue to construct a phallus. Magnetic resonance imaging of the spine has shown tethering of the cord in all patients; most were released neurosurgically. Lessons provided by these cases demonstrate that a satisfactory surgical and social outcome can be achieved today in most children with cloacal exstrophy.

Gastrocystoplasty was performed in 29 female and 14 male patients 1 to 23 years old (average age 11 years). Urodynamic studies were completed in 20 patients preoperatively and within 6 months postoperatively. When postoperative studies were compared with the preoperative evaluation, it was noted that overall capacity increased an average of 220% (range 20 to 750%). Compliance also improved dramatically postoperatively. Bladder pressures increased from a mean volume of 34% of capacity preoperatively to 73% of the post-augment capacity. The average maximum filling pressure decreased by 32%, from 57 to 39 cm. water. Uninhibited contractions resolved completely in 6 of 10 patients who had them preoperatively. No one had uninhibited contractions postoperatively. Cyclical contractions of the stomach segment not exceeding 50 cm. water developed in 6 patients. The gastric patch seems to be an adequate source for improving the dynamics of a poorly functioning detrusor muscle in most patients. When a diamond-shaped patch of stomach is harvested and added to the bladder a spherical configuration is created, enhancing the storage properties. This result is in sharp contrast to other bowel segments, which must be detubularized to produce a suitable reservoir. The long-term effects of gastric augmentation remain to be determined.

The case of a boy with hyperimmunoglobulin E syndrome or Job's syndrome is presented to demonstrate the occurrence of pneumatoceles in this syndrome as well as their unusual natural history and failure to spontaneously resolve. Surgical resection was required for two complications in this patient, persistent bronchopleural fistula and a pulmonary abscess that destroyed one lung and required pneumonectomy. Pathologic examination of the specimens demonstrated the wall of the cysts consisted of granulation tissue with chronic active inflammation surrounded by infarcted pulmonary parenchyma with coagulative necrosis. The mechanism responsible for increased immunoglobulin E production in this syndrome is unknown, as is the manner in which elevated immunoglobulin E levels impair normal immune function.

In 12 of 264 children treated with enterocystoplasty 15 spontaneous perforations occurred. Of the 12 children 9 had myelodysplasia. All segments of the gastrointestinal tract were used for the augmentation and most were detubularized. Surgery to increase bladder outlet resistance was done in 8 cases. At the time of each perforation 9 children had sterile cultures, however, 3 died of overwhelming sepsis. Presenting signs included abdominal pain in 8 cases, septic shock in 4 cases and shoulder pain in 4 older myelodysplastic children with diaphragmatic irritation from escaping urine. Cystography demonstrated a leak in 10 of 11 cases. Urodynamic studies revealed good compliance with low maximum filling pressure in 8 of 10 children. Hyperreflexia was noted in only 5 cases and outlet resistance greater than 85 cm. water was demonstrated in 5. Histological analysis showed changes in the bowel wall consistent with ischemia but suture granulomas were present in areas adjacent to the perforation site or thinned areas in biopsy or autopsy specimens. In addition to the theory that overdistention may cause enterocystoplasty perforation, current detubularization techniques may produce areas of relative ischemia, which become accentuated when the augmented bladder is distended beyond a reasonable volume.

The management of paratesticular rhabdomyosarcoma has evolved more slowly than that for testicular germ cell tumors, mainly due to its lower incidence and the lack of standardized treatment protocols. With gradual refinements in staging and therapy the prognosis has improved. Between 1960 and 1988, 18 patients 2 to 18 years old were treated at the Children's Hospital. Management consisted of inguinal orchiectomy, staging evaluation and retroperitoneal lymph node dissection, followed by chemotherapy and radiotherapy according to the stage of the disease. In 11 patients (61%) the disease was confined to the scrotum, while 7 presented with spread to the retroperitoneal lymph nodes, including 3 children with more distant metastases. Histological study revealed predominantly embryonal characteristics except for 1 alveolar cell type. Chemotherapy consisted of actinomycin D in 2 patients, vincristine, actinomycin D and cyclophosphamide in 2, pulse vincristine, actinomycin D and cyclophosphamide in 10, and doxorubicin plus vincristine, actinomycin D and cyclophosphamide in 4. A total of 17 patients remain free of disease (median followup 4 years) with 1 death. The actuarial survival without relapse and over-all survival rate are 89 and 94%, respectively. We conclude that paratesticular rhabdomyosarcoma represents a favorable subgroup of rhabdomyosarcoma.

From 1977 to 1989 bladder augmentation was performed in 56 male and 73 female patients from 1 to 35 years old (average age 12.7 years). In 59 cases augmentation was performed as part of an undiversion operation. Indications for augmentation included neurogenic bladder, severely scarred bladder from prior operations, noncompliant bladder after previous treatment of severe urethral valves, small bladder in former exstrophy patients, epispadias with a small bladder, cloacal exstrophy, and small, noncompliant bladder after therapy for cancer, trauma, cloaca and miscellaneous conditions. We used 145 bowel segments, since 16 patients had 2 bowel segments. Segments included cecum in 65 cases, sigmoid in 46, small bowel in 24, stomach in 4 and left colon in 1. The most common complication was stones. Detubularized bowel for augmentation of small noncompliant bladders allows functional reconstruction in a wide range of urological disorders that were formerly treated by diversion. Reconstruction is possible in many previously diverted patients if bladder augmentation is used.

We evaluated urodynamically 41 patients with posterior urethral valves because of signs or symptoms of incontinence (35), frequency (3), hydronephrosis (2) and infection (1). Findings included normal urodynamic evaluations in 3 patients, 2 had high voiding pressures secondary to outlet resistance and 1 had incontinence on the basis of external urethral sphincter damage. In the remainder 3 patterns of bladder dysfunction were identified. Myogenic failure with overflow incontinence occurred in 14 patients. In this group clean intermittent catheterization or Valsalva's voiding was used for emptying. Hyperreflexic bladders were seen in 10 patients. Pharmacological suppression of instability was effective in 5 of 7 patients treated; 1 required bladder augmentation. Eleven children had a small capacity bladder and poor compliance. Post-void residuals were low and these bladders were generally but not always stable. Pharmacological bladder relaxation was successful in 3 patients, 3 underwent augmentation, 1 did well with alpha-agonists and followup is unavailable on the other 4. These 3 patterns of bladder dysfunction represent an overlapping constellation of residual urodynamic abnormalities due to previous bladder outlet obstruction. Individual patients may show facets of several types of dysfunction associated with 1 predominant pattern.

In the last 13 years splitting the pubic symphysis has been used in 46 patients 1 to 32 years old for wide access to the bladder neck and entire urethra in female, or the bladder neck and urethra down into the bulb in male patients. Each patient had a complex problem to reconstruct and most had undergone a prior operation. Underlying pathological conditions included exstrophy in 16 patients, epispadias in 11, cloacal exstrophy in 6, posterior urethral valves in 3, severe trauma in 2, bilateral single ectopic ureters in 2, radical surgery for sarcoma in 2, myelodysplasia in 2, cloacal anomaly in 1 and iatrogenic injury of the urethra in 1. The pubis, or interpubic ligament in exstrophy cases, was divided in the midline. The pubic rami were spread with a laminectomy spreader to create a gap sufficient to operate on that segment of the urethra, which is usually not accessible with an intact pubis. No patient had a problem of nonunion or pubic osteitis after using this approach. This series includes 2 patients undergoing simultaneous iliac osteotomies for a widened symphysis pubis. In no patient was a segment of pubis resected, as has been used in transpubic repair of certain traumatic urethral strictures.