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PubMed1998

Vaginal construction in children.

PURPOSE: We evaluated outcomes in 20 patients 1 to 21 years old who underwent vaginal construction between 1980 and 1996. MATERIALS AND METHODS: A 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. RESULTS: Patients 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. CONCLUSIONS: Vaginal 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.

Adolescent↗
PubMed1997

Further experience with the periurethral expander: a new type of artificial sphincter.

OBJECTIVE: To determine the long-term effects of the periurethral expander, a new device for the treatment of urinary incontinence in children. PATIENTS AND METHODS: Twenty-five patients (mean age 11.2 years, range 3-22) had a periurethral expander implanted around the bladder neck within the last 41 months. Twelve of these patients had neurogenic bladders as a result of myelomeningocele, 12 had undergone unsuccessful exstrophy reconstructions and one had megalourethra. Bladder augmentations were carried out in 23 of the patients. The mean follow-up was 23 months. RESULTS: Ten patients had the device removed due to erosion and/or infection; patients with exstrophy were more susceptible to complications. Patients with neurogenic bladders use clean intermittent catheterization and the remainder void spontaneously. The mean pressure in the cuff was 58.7 cmH2O. CONCLUSION: The periurethral expander seems to be a valid option in the treatment of selected cases of urinary incontinence in children and young adults. There were more complications in patients with exstrophy/epispadias and such patients must be considered unsuitable for this treatment.

Adolescent↗
PubMed2000

Complete duplication of the bladder, urethra, vagina, and uterus in girls.

UNLABELLED: OBJECTIVES. To present our experience with 3 girls with complete duplication of the bladder, urethra, vagina, and uterus, a very rare anomaly, and review published reports. Associated anomalies in these cases represent a wide spectrum. METHODS: We report 3 girls with complete duplication of the bladder, urethra, vagina, and uterus. The associated anomalies were different in each child. The first patient had a symphysis diastasis creating an abdominal hernia with two bladders dislocated into this space. The second patient had anal atresia with colon duplication. The third had cloacal exstrophy with one opened bladder and a second closed, with persistent urogenital sinus. Each patient was carefully evaluated, but the anatomy in 2 patients was not completely defined until surgical exploration. RESULTS: Each girl underwent surgical repair. In the first patient, iliac osteotomy, approximation of the symphysis pubis, and excision of the hernia was done. In the second, excision of the common wall between the two descending colons and posterior sagittal anorectoplasty were performed. In the third patient, staged reconstruction of the cloacal exstrophy with anastomosis of the bladders and vaginas was done. CONCLUSIONS: Complete duplication of the urogenital system in girls is associated with different anomalies. Which surgical procedures will be required depends on the multiple anatomic variables.

Abnormalities, Multiple↗
PubMed1998

Modifications of and extended indications for the Pippi Salle procedure.

Total urinary incontinence is a difficult problem faced by the urologist. Several techniques to increase ureteral resistance have been described. The majority of them rely on intermittent catheterization for bladder emptying, especially in neurogenic incontinence. We have developed a new procedure in which a bladder flap is used to create a neourethra. This urethral extension acts as a flap valve to provide continence. Bladder emptying is accomplished by clean intermittent catheterization. Urethral lengthening with an anterior bladder-wall flap was performed in 18 patients aged a mean of 8.9 years who had neurogenic incontinence (14) or exstrophy (4). Patients with previous bladder interventions received a lateralized anterior flap. Bladder augmentation was performed in 14 of the 18 patients [detubularized ileum (11), detubularized colon (3)]. The average follow-up period is currently 29.3 months. Continence was achieved in 13 of the 18 patients (72%). Complications included urethrovesical fistulae, which developed in two patients. Two patients could not perform catheterization due to pain but had no obstruction to passage of catheter (exstrophy). Ureteral lengthening with an anterior bladder-wall flap is a useful alternative for the surgical treatment of urinary incontinence. This technique achieves a good continence rate and presents few problems with catheterization.

Bladder Exstrophy↗
PubMed1988

[Urinary diversion with Kock pouch: clinical results in 75 cases focusing on late complications].

Internal urinary reservoir type urinary diversions have been getting popularity since Kock reported a continent ileal reservoir in 1982. From November 1984 through October 1987, we performed Kock pouch operation in 75 patients (male 64, female 11; from 24 to 82 years old, mean age 56 years old). The underlined diseases were mostly bladder cancer patients; bladder cancer 70, rectal cancer 2, prostatic sarcoma 1, vesical exstrophy 1 and neurogenic bladder 1 case. The end results for 71 evaluable cases, followed up more than 3 months, were excellent in 49 (69%), good 14 (20%), fair 6 (8.5%) and poor 2 (2.8%), with success rate 89%. The most common complication was the nipple malfunction; prolapse, including intermittent prolapse, in 7 patients, slippage or sliding in 1, and eversion in one patient. Stone formation occurred in 6 patients, mild acidosis in 2 and a entero-pouch fistula in a patient who was re-diverted from a ileal conduit. There were 2 deaths postoperatively. Late complications were prominently decreased due to several important modifications of the operative technique, such as usage of Cavitron Ultrasonic Surgical Aspirator (CUSA) for defatting the mesentery, and fixing the nipple to the pouch. In conclusion, Kock pouch can be a safe and sound operative modality for patients who need urinary bladder replacement.

Adult↗
PubMed2006

Long-term effects of dextranomer endoscopic injections for treatment of urinary incontinence: an update of a prospective study of 31 patients.

PURPOSE: A prospective study was initiated 7 years previously to assess the efficacy of endoscopic dextranomer based implants for pediatric structural incontinence. Preliminary results revealed that at 3 years 50% of the patients were either dry or significantly improved. We report long-term results in the same cohort of patients. MATERIALS AND METHODS: A total of 33 children and adolescents 5 to 18 years old with severe incontinence due to sphincteric incompetence (exstrophy-epispadias complex in 13, neuropathic bladder in 16, bilateral ectopic ureter in 4) were enrolled. Of the patients 13 underwent 2 and 4 underwent 3 treatment sessions to achieve a definitive result. Mean injected volume was 3.9 ml (range 1.6 to 12) per session. At each evaluation patients were considered cured (dryness interval 4 hours), significantly improved (minimal incontinence requiring no more than 1 pad daily and no further treatment required) or treatment failures (no significant improvement). Videourodynamics were used to study the evolution of the bladder capacity, activity and compliance. A total of 31 patients were followed 3 to 7 years after the last injection. RESULTS: At 3 years after treatment 15 of 30 patients (50%) were dry or improved. One patient who had leakage after 3 years of dryness due to bladder deterioration subsequently underwent ileocystoplasty. At 4 years 12 of 25 patients (48%) were dry or improved. At 5 years 9 of 21 patients (43%) were dry, as were 4 of 11 (36%) at 6 years and 2 of 5 (40%) at 7 years of followup. The success rate according to pathological evaluation was comparable in neuropathic bladders (7 of 14, or 50%), exstrophy (3 of 6, or 50%) and epispadias (3 of 7, or 43%). Of 12 patients who underwent bladder neck plasty before the injection of bulking agent 7 (58%) were either dry or improved. The success rate was higher in males (13 of 23, or 57%) than in females (3 of 8, or 38%). Also, at puberty 2 males who were improved became dry. Bladder capacity increased in 12 of 18 initially small bladders and remained normal and stable in 9, while 4 initially dry patients had development of recurrent leakage secondary to bladder deterioration and underwent augmentation. Otherwise, there were no long-term side effects observed related to the injection of the bulking agent. CONCLUSIONS: Endoscopic treatment of severe organic urinary incontinence with dextranomer is durable for up to 7 years of followup in 40% of the patients.

Adolescent↗
PubMed2003

Risk factors for spontaneous bladder perforation after augmentation cystoplasty.

OBJECTIVES: To present our experience with the use of stomach, ileum, and colon for augmentation cystoplasty to examine the incidence of, and risk factors for, spontaneous perforation. Spontaneous bladder perforation is a potentially life-threatening complication of augmentation cystoplasty with a reported incidence of up to 13%. METHODS: A retrospective review of medical records from 1988 to 2001 identified 107 children (57 males and 50 females) who underwent augmentation cystoplasty at our institution. The etiology for bladder dysfunction included myelomeningocele, VATER (vertebral defects, imperforate anus, tracheoesophageal fistula, radial and renal dysplasia) syndrome, bladder and cloacal exstrophy, posterior urethral valves, and pelvic malignancy. Thirteen patients also had end-stage renal disease. RESULTS: The median follow-up was 7.4 years. Gastrocystoplasty was performed in 50 children (47%), ileocystoplasty in 37 (35%), colocystoplasty in 18 (17%), and gastric-ileal composite neobladder in 2 (2%). Augmentation cystoplasty procedures were performed using a standard technique that included a two-layer anastomosis. Additional procedures at the time of reconstruction included Mitrofanoff neourethra in 66 patients (62%) and bladder neck repair in 44 (41%). Postoperatively, most patients started a strict incremental catheterization regimen. The overall incidence of bladder perforation was 5%, with one traumatic (1%) and four spontaneous (4%) perforations. All patients recovered uneventfully after exploratory laparotomy. CONCLUSIONS: We believe that the relatively low incidence of spontaneous bladder perforation encountered in this series may be explained by the large number of patients with gastrocystoplasty, as well as our strict adherence to a postoperative incremental catheterization program.

Adolescent↗
PubMed1996

Combined use of enterocystoplasty and a new type of artificial sphincter in the treatment of urinary incontinence.

PURPOSE: We report the results of the combination of enterocystoplasty and a periurethral expander, a simplified type of artificial sphincter, in the treatment of urinary incontinence. MATERIALS AND METHODS: The new 1-piece device has an adjustable cuff connected to a port positioned at the subcutaneous space in the abdomen. The cuff is adjusted to the bladder neck and the pins are fixed according to the diameter of the urethra. The port is punctured percutaneously and saline is injected until continence is achieved. Eight boys and 3 girls underwent nonsecretory sigmoid cystoplasty and placement of the device at a single operation. Nine patients had neurogenic bladder and in 2 exstrophy reconstruction had failed. Followup ranged from 4 to 26 months. RESULTS: All patients were continent with improved bladder compliance 6 to 8 weeks after the operation, when the device was activated. In 1 case the device was extracted after 2 months due to frequent episodes of hematuria and edema at the port site. Two patients had erosion of the skin at the port site. Urodynamics were repeated at the time of activation. Maximal urethral closing pressure increased from 16.27 to 157.44%. Two patients needed a second injection to achieve continence. The patients with exstrophy void spontaneously and those with neurogenic bladder are on clean intermittent catheterization. CONCLUSIONS: Although more followup is needed, the combination of these procedures seems to offer a new option for the treatment of urinary incontinence in children.

Adolescent↗
PubMed1988

OEIS complex (cloacal exstrophy): prenatal diagnosis in the second trimester.

OEIS complex (exstrophy of the cloaca) is an association of fetal malformations that includes omphalocele, exstrophy of the bladder, imperforate anus, and spinal defects. Most cases should be prenatally detectable by maternal serum alpha-fetoprotein screening, but an accurate diagnosis is essential for appropriate counselling of the family.

Adult↗
PubMed1986

William E. Ladd, M.D.: great pioneer of North American pediatric surgery.

William E. Ladd (1880-1967) was the great pioneer pediatric surgeon of North America. His family had been New England merchants. He was educated at Harvard and worked within the Harvard Medical School during his entire surgical career. He began to limit his surgical practice to the care of infants and children at the Boston Children's Hospital soon after World War I. One basis for his great contributions lay in the establishment of an accurate medical records system with a thorough follow-up of the patients. Another basic factor was the development of uniform policies and methods for each type of surgical disease. These policies and methods then underwent evolution as potential improvements appeared. Using this general approach, Ladd influenced the improvement in care of almost all of the common types of pediatric disease treatable by surgery. Among the earliest were intussusception and pyloric stenosis. Later, among many other things, he developed useful systems for the treatment of hernias, rectal bleeding, biliary atresia, failure of rotation of the G.I. tract, rectal anomalies, and exstrophy of the bladder. He made unusual contributions to the care of Wilms' tumor and atresia of the esophagus. He was a great teacher. His pupils have become the teachers of many other pediatric surgeons throughout the world.

General Surgery↗
PubMed1982

Four cases of hyperphenylalaninaemia: studies during pregnancy and of the offspring produced.

Four cases of variant hyperphenylalaninaemia during pregnancy are presented. Babies born to mothers with blood phenylalanine concentrations of 4-8 mg/dl were normal and did not exhibit significant microcephaly, although one did have a major congenital anomaly (exstrophy of the bladder). The mother with blood phenylalanine concentrations of 6-12 mg/dl during pregnancy delivered three of four infants with mild microcephaly but normal intellectual function. The data suggest that phenylalanine concentrations of 4-8 mg/dl are reasonable and desirable during pregnancy in hyperphenylalaninaemic women. Furthermore, the data do not support or refute the justification hypothesis of Bessman et al.

Adult↗
PubMed2002

Terminal myelocystocele.

Terminal myelocystocele is an unusual form of occult spinal dysraphism. It consists of a cystic dilatation of a low-lying terminal cord herniated posteriorly through a skin covered lumbosacral spina bifida. This condition is often associated with OEIS complex i.e. opmphalocele, exstrophy of the bladder, imperforate anus and spinal abnormality. We studied 4 cases of terminal myelocystocele. They revealed no preoperative neurological deficit. None of these had associated OEIS complex. One of the cyst was unique due to presence of copious amount of pus in its cavity. All four cases underwent successful repair and surgery remained uneventful in all of them. No child showed neurological deterioration during the follow-up.

Female↗
PubMed1997

Perinatal features of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS complex) associated with large meningomyeloceles and severe limb defects.

Omphalocele-Exstrophy-Imperforate anus-Spinal defects (OEIS complex), a combination of omphalocele, exstrophy of the bladder, an imperforate anus and spinal defects, arises from a single localized defect in the early development of the mesoderm that will later contribute to infraumbilical mesenchyme, cloacal septum, and caudal vertebrae. In this report, we document the perinatal features of two cases of OEIS complex associated with meningomyeloceles and severe lower limb defects, and discuss the prenatal diagnosis, inheritance, and differential diagnosis of this association of malformations. Although long-term survival can be achieved by successful corrective surgery, the associated structural defects such as large meningomyelocele and severe limb aplasia or hypoplasia, as seen in our patient, can influence the patient's quality of life. We would like to emphasize that an accurate prenatal diagnosis of OEIS complex and associated malformations is important for the detailed counseling of the family as well as appropriate perinatal management by the obstetricians, pediatric surgeons, urologists, neurosurgeons, and neonatologists.

Abnormalities, Multiple↗
PubMed1978

Urography in children: when should it be done? 2. Conditions other than infection.

Conditions that are often associated with an increased incidence of renal anomalies include imperforate anus, congenital vertebral abnormalities, and Fanconi anemia; excretory urography should be done if such a condition is present. Urography is also useful to provide baseline data in conditions associated with later development of urinary problems, such as myelodysplasia, prune-belly syndrome, and exstrophy of the bladder. In addition, urography serves as a periodic check for complications of treatment (hydronephrosis, obstruction) in patients with urinary diversion. Certain signs, eg, dribbling, hematuria after mild trauma, unexplained pneumothorax or pneumomediastinum in a neonate, and neonatal abdominal mass, call for immediate urography. In many conditions that were formerly thought to be associated with major urinary abnormalities, urography is not called for. Such is the case in hypospadias, deformities of the external ear alone, and undescended testes. Dehydration is the only absolute contraindication to urography.

Child↗
PubMed1996

Outcome analysis of Mitrofanoff principle applications using appendix and ureter to umbilical and lower quadrant stomal sites.

PURPOSE: We compared the indications for and results of application of the Mitrofanoff principle using appendix and ureter to umbilical and lower quadrant stomal sites. MATERIAL AND METHODS: We created continent catheterizable conduits in 60 patients 3 days to 20 years old (mean age 8.8 years). The primary indication was refractory urinary incontinence associated with bladder or cloacal exstrophy, or anomalies in 31 cases. We constructed 38 umbilical (all appendix) and 22 lower quadrant (10 appendix and 12 ureter) stomas. Mean followup was 3 years. RESULTS: We preferred the umbilicus as a stomal site for its cosmetic value. The most common indication for a lower quadrant stomal site was preservation of the retroperitoneal course of the ureter. The stomal stenosis rate was 13 and 4% in patients with umbilical and lower quadrant stomas, respectively. We preferred the appendix as a conduit due to availability. Indications for the ureter as a catheterizable conduit were absence of an adequate appendix or presence of a healthy ureter after nephrectomy. The appendiceal and ureteral conduits were catheterizable in 94 and 84% of patients, respectively, and continence was achieved in 97%. CONCLUSIONS: In a complex group of patients with incontinence application of the Mitrofanoff principle yielded good results for either combination of stomal site or catheterizable conduit. Appendicovesicostomy to the umbilicus remains our preferred procedure.

Adolescent↗
PubMed1980

Female epispadias: report of a case and review of the literature.

Female epispadias is a congenital anomaly representing a mild form of the spectrum of exstrophy of the bladder. Unrecognized an untreated, the handicap associated with the physical malformation can create overwhelming clinical and psychological problems for the affected female. Radiographic findings of spinal dysrhaphism with diastasis of the public bones are often associated with, and can afford the first clue to, the physical findings of absent clitoris, ununited labia and patulous and foreshortned urethra.

Child, Preschool↗
PubMed1994

Intraoperative anaphylaxis due to exposure to latex (natural rubber) in children.

Intraoperative anaphylaxis secondary to exposure to latex is a serious and potentially life-threatening phenomenon that has been recognized more frequently in recent years. Between 1989 and 1992, twenty-one patients had a Type-I (anaphylactic) reaction to latex intraoperatively at The Children's Hospital of Philadelphia. Twelve (57 per cent) of these patients had spina bifida. Six patients (29 per cent) had cerebral palsy, and five of them had a ventriculoperitoneal shunt. Of the remaining three patients, one (5 per cent) had exstrophy of the bladder, one had VATER syndrome, and one had Duchenne muscular dystrophy. All of the patients had had at least two previous operative procedures. Overall, sixteen patients (76 per cent) had a ventriculoperitoneal shunt. The manifestations of the allergic reaction included a rash in fifteen patients (71 per cent), hypotension in fifteen, tachycardia in eleven (52 per cent), bronchospasm in ten (48 per cent), bradycardia in two (10 per ent), and cardiac arrest in two. The symptoms occurred within minutes after the induction of the anesthesia in all but one patient, in whom hypotension and cardiovascular arrest developed approximately one hour after the beginning of the operation. Two patients had a full cardiopulmonary arrest while under the anesthesia. All twenty-one patients responded to management; there were no deaths. Of the six patients who had cerebral palsy, five had been managed with a ventriculoperitoneal shunt because of hydrocephalus following a previous intraventricular hemorrhage that was related to prematurity.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1989

[Initial measures in newborn infants with surgically correctable abnormalities].

In newborns with surgically correctable malformations the quality of primary care has considerable influence on the results of the eventual surgical repair. This is particularly true for extensive diaphragmatic and abdominal wall defects as well as for esophageal and intestinal atresia, exstrophy of the bladder and urogenital malformations. The neonatal management comprises measures generally valid for all diseased newborns and measures specific for the particular malformation. The general care concerns heat regulation, fluid balance and other vital functions. Specific regimens are necessary as primary care for diaphragmatic hernia, esophageal atresia, abdominal wall defects and neonatal bowel obstruction. Furthermore this primary management must be extended to include the care of the pregnant mother. Already in utero infants with diaphragmatic and esophageal malformations should be referred to the gynecological-neonatal center.

Abnormalities, Multiple↗