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Implications of antenatal ultrasound screening in the incidence of major genitourinary malformations.

Over the past 3 decades, infant mortality has decreased nearly 50%. Although neonatal intensive care deserves much of the credit, the recent increase in prenatal ultrasonography use, from 33% of pregnancies in 1980 to 78% in 1987, has improved early detection. The authors wished to evaluate the impact on major genitourinary malformations. Data obtained from the Malformations Surveillance Program at Brigham and Women's Hospital between 1974 and 1994, tracked 163,431 pregnancies and termination rates of fetuses with spina bifida, bladder exstrophy, prune belly syndrome, and posterior urethral valves. Hospital data showed 65% of fetuses with spina bifida, 46% with posterior urethral valves, 31% with prune belly syndrome, and 25% with exstrophy, were terminated electively. Clearly, surveillance programs and improved accuracy of antenatal ultrasound has allowed early diagnosis of major genitourinary malformations. Many factors influence decision making in these affected fetuses, including the financial and emotional impact of these major anomalies over a lifetime. Future societal decisions, and the reduction in these anomalies may influence our training programs, manpower needs, medical facility requirements, and the character of our practices. These findings may have significant implications in the field of pediatric urology.

Female↗

Combined Mitrofanoff and antegrade continence enema procedures for urinary and fecal incontinence.

PURPOSE: Fecal soiling or intractable constipation frequently occurs in association with urinary incontinence in children undergoing major reconstructive urological operations. To treat double incontinence or the combination of wetting and severe constipation, we constructed a Mitrofanoff conduit and a channel for antegrade continence enemas in 18 patients between 1989 and 1995. We review the underlying pathological conditions, various surgical techniques and outcomes of these operations. MATERIALS AND METHODS: Underlying abnormalities mainly included spinal lesions, bladder exstrophy, imperforate anus and various cloacal anomalies. Patient age ranged from 2 to 18 years (average 8.4). In 13 patients both procedures were done simultaneously. The appendix was used to construct the antegrade continence enema channel in 8 cases and the Mitrofanoff channel in 5. It was long enough to be divided and used for both procedures in 2 cases but it was missing or unsuitable in 3. Alternative antegrade continence enema conduits were cecal flap in 7 patients and ileum in 1, while the ureter, ileum and detrusor tube were used to establish Mitrofanoff channels in 5, 5 and 1, respectively. Stomas were constructed according to the V-flap or V. Z. Q. technique and situated in close proximity in the right lower abdominal quadrant in 13 cases. RESULTS: Convalescence was uneventful except for 1 abscess near an antegrade continence enema stoma. Ten patients needed dilation or minor revisions due to difficulty in catheterizing the antegrade continence enema (5), Mitrofanoff (3) or both conduits (2). Subsequently 3 patients underwent repeat operations for reconstruction of 2 antegrade continence enema channels (cecal flap and ileum) and 1 detrusor tube Mitrofanoff channel. Currently 15 patients are dry on regular clean intermittent catheterization using 10 to 12F catheters. Outcomes of the antegrade continence enema channels are satisfactory in 15 patients who are clean or rarely soil. Failure occurred in 1 patients with severe constipation necessitating colostomy and 2 (1 noncompliant who stopped catheterizing regularly) in whom the channels subsequently closed. CONCLUSIONS: Synchronous construction of antegrade continence enema and Mitrofanoff channels is successful in the majority of doubly incontinent patients. Selection of patients with high motivation is important to obtain satisfactory results.

Adolescent↗

Latex allergy in children with urological malformation and chronic renal failure.

PURPOSE: Children with spina bifida, bladder exstrophy and anorectal anomalies are at risk for latex allergy. Severe intraoperative anaphylaxis in a boy treated with kidney transplantation prompted this study to evaluate the prevalence of latex allergy in a cohort of children with chronic renal failure (CRF). MATERIALS AND METHODS: Between 1996 and 2002, 57 boys and 28 girls were investigated at a median age of 10.5 years (range 1.3 to 22.9). Urological malformations were the underlying cause of CRF in 33 patients (39%). Of the patients 39 were on conservative treatment, 20 were on dialysis and 26 had a functioning renal graft. Latex reaction was assessed by a careful history, specific serum latex IgE and skin prick test. RESULTS: A total of 19 patients (22%) showed latex reaction, of whom 8 had allergy (clinical symptoms included severe intraoperative anaphylaxis in 1) and 11 had sensitivity (positive IgE or prick test without symptoms). Of these 19 patients 11 had urological malformations. The number of surgical procedures, young age at operation and atopy were significant risk factors. When operations were analyzed separately, ie urological vs nonurological surgery, only urological surgery was significantly associated with latex reaction. A significant correlation was also found between the overall number of operations and latex radioallergosorbent class. CONCLUSIONS: All children with CRF who undergo early and multiple urological surgery are at high risk for latex reaction. Primary latex prevention, ie the routine use of latex-free gloves, tubes and catheters, should be implemented in all children with complex urological malformations.

Adolescent↗

Comparative study of the Yang-Monti channel and appendix for continent diversion in the Mitrofanoff and Malone principles.

PURPOSE: Continent conduits for Mitrofanoff and Malone procedures are widely used for incontinence management. Because the appendix is not always suitable or available, Monti proposed a transversely retubularized short segment of ileum. We present our experience and compare outcomes according to the type of conduit. MATERIALS AND METHODS: In a retrospective study from 1988 to 2003 case notes were reviewed specifying conduit characteristics, underlying disease, overall surgical management of incontinence and subsequent complications. Comparison was based on followup, details of complications and treatments. RESULTS: In 32 men and 14 women with a total of 65 conduits (Mitrofanoff in 41 and Malone procedure in 24) the condition was spina bifida in 29, bladder exstrophy in 5 and sacral agenesia in 3. Other cases included various origins. Mean age at operation was 14.2 years (range 1 to 31). Mean followup was 5.3 years (range 1 to 14.7). Appendix was used in 23 cases and a Yang-Monti channel was performed in 18 (ileum in 7 and sigmoid in 11) for urinary diversion. The Malone procedure was performed using appendix in 11 cases and a Yang-Monti tube in 13. At followup 61 conduits (93.8%) were in use and 4 (6.2%) were abandoned. Complications were cutaneous stenosis, kink blockage and stomal leakage. No significant differences were observed between the types and purposes of the conduit. CONCLUSIONS: With constant availability and various possibilities of construction the Yang-Monti channel is recommended for continent diversion creation when appendix does not look suitable or is already in use or absent.

Adolescent↗

Epidemiological assessment of misoprostol teratogenicity.

OBJECTIVE: To verify if any of the 15 congenital defects already reported in association with misoprostol can be found within an epidemiological registry of congenital defects. DESIGN: Case-control study including case-sick and case-health controls. METHODS: Comparison of misoprostol exposure for each specific defect, using the exposure for the rest of defects as a reference group. POPULATION: Four thousand six hundred seventy-three consecutive newborn infants with malformations of unknown aetiology, in the Latin American Collaborative Study of Congenital Malformation. RESULTS: There was no difference in exposure rate between the malformed (34/4,673) and nonmalformed (23/4,980) newborns. Four of the five more frequently cited defects in the literature were found to be in excess: constriction ring, terminal transverse-limb defects, hydrocephalus, and arthrogryposis. Equinovarus feet had a normal frequency in our study. Thirteen different defects not described in the literature were seen in our misoprostol exposed cases, but only holoprosencephaly and bladder exstrophy significantly exceeded the expected number. CONCLUSIONS: The confirmation from an epidemiological registry of an association for four of the five more commonly observed congenital defects among misoprostol exposed children described in the literature seems indicative of a real teratogenic effect. The defects are of vascular disruption type. However, additional attempts to achieve abortion could not be excluded as a concurrent contribution.

Abnormalities, Drug-Induced↗

'Sting' procedure in the treatment of secondary reflux in children.

The authors present their experience (24 children) with the endoscopic subureteral injection of Teflon paste in the correction of secondary vesicoureteral reflux: reimplantation failure; ureterocele; obstructive bladder; exstrophy; postchemocystitis, and Hutch's diverticulum. Of these 24 cases (31 refluxing ureters), 18 were treated with success. Each cause justifies a comment on the particularities of its endoscopic treatment. The 'sting' can be particularly attractive to cure secondary reflux, avoiding the difficulties and complications of open surgical reimplantation.

Child↗

Congenital malformations in the North-Eastern Democratic Republic of Congo during Civil War.

OBJECTIVE: To report on changes in the incidence of congenital malformations in a context of an ongoing civil conflict. DESIGN: Retrospective study. SETTING: Centre Médical Evangélique (CME) of Nyankunde, a 250 bed referral hospital, in the North Eastern Democratic Republic of Congo (DRC) during the period 1993 to 2001. SUBJECTS: A total of 8824 babies were delivered alive at our maternity; and 36 of them (0.41%) were born with a clinically diagnosed congenital malformations. RESULTS: The breakdown of the observed malformations was as follow: clubbed foot nine, congenital hydrocephalus eight, spina bifida six, cleft lip four, encephalocele two, syndactyly two, imperforated anus two, Anencephaly one, lymphangioma one, bladder exstrophy one. There was a significant increase in the annual incidence of congenital malformations (p<0.001). CONCLUSION: Ongoing civil conflicts or wars ultimately have a negative impact on the incidence of congenital malformations. This is an indirect and multifactorial consequence. We conclude that ongoing civil conflict is a contributing factor to the increasing incidence of congenital malformations seen in the North-Eastern DRC.

Congenital Abnormalities↗

[Latex allergy in pediatric age: an interdisciplinary perioperative management and case reports].

BACKGROUND: Latex intraoperative allergy is more and more frequent, especially in at groups risk (patients with spina bifida or congenital genitourinary abnormalities, pluri-operated patients, atopic subjects) and in pediatric age. The main problem of this allergy consists in the necessity of a strict collaboration of many specialists, in order to identify and safeguard the patient. METHODS: Our experience has pointed out an interdisciplinary perioperative management able to: 1. identify patients affected by latex allergy; 2. submit them to a latex-safe perioperative proceeding; 3. check their conditions with periodical tests. Selecting patients through a history and a list of questions, identifying profiles of typical risk patients, organizing the operating room with latex-free materials and equipment were the most important issues. Since November 1997 to December 1999 eighteen latex-safe perioperative proceedings have been carried out on 8 subjects (2 with esophageal atresia, 4 with bladder exstrophy and 2 with cloacal exstrophy); 2 of them were emergency cases. RESULTS: No allergic reactions and no proceeding-linked complications have been registered. Operators have always been satisfied by materials and equipment. Anesthesiological and surgical times resulted equal to those without latex-safe management. CONCLUSIONS: This perioperative management of potential or verified latex allergic patients turned out to be valid, safe and easy in practical application.

Adolescent↗

[Cutaneous trans-jejunal ureterostomy: an original technique used in 29 patients].

The authors report an original technique of transintestinal cutaneous diversion using the jejunum A 10 to 15 cm segment of jejunum is raised from the second loop and is attached to the skin transperitoneally. The stoma lies below the ureterojejunal anastomosis and the two ureters, sutured according to Wallace's technique, are implanted end-to-end into the proximal and extraperitonealised end at the height of the sacral promontory. 29 patients have been treated by this technique for various indications: 21 bladder tumors, 5 neurogenic bladders, 1 bladder exstrophy, 1 cervical cancer, 1 prostatic cancer. 15 patients (52%) had previously received pelvic irradiation. The mean follow-up was 47 months (2-192 months) and the mortality was zero. 4 patients (13.8% developed early complications: 2 cases of urinary tract obstruction and 2 intestinal obstructions requiring 3 operations. 6 patients developed 7 minor late complications (24.1%): 2 cases of pyelonephritis, 4 cases of renal stones, only one of which required an operation, and 1 case of prolapsed stoma. None of the patients developed any alteration in renal function or dilatation of the upper urinary tract after the operation. The addition of sodium bicarbonate was found to be useless, as none of the patients developed any metabolic disorders. Transjejunal cutaneous ureterostomy achieves excellent drainage by means of a short graft with a stoma situated below the level of the ureteric implantation, as reflected by the absence of any long-term deterioration in renal function despite full lumen ureterojejunal implantation without an antireflux device. This technique is simple to perform and is indicated in all situations, particularly after pelvic irradiation. It is associated with low morbidity, no mortality and ensures an excellent long-term result.

Adolescent↗

[Application of multihole U-shaped drainage stent in complex posterior urethra surgery].

OBJECTIVE: To study a method for using a new drainage stent following complex posterior urethral operation. METHODS: Fifty-five patients,15 of whom had complex posterior urethrorectal fistula, 35 had complex posterior stricture or atresia, and 5 had bladder exstrophy, received surgical treatment, after which multihole U-shaped drainage stent was applied. RESULTS: All the patients were normal in micturition and no complications occurred during the follow-up period lasting for 1 to 10 years. CONCLUSION: Multihole U-shaped drainage stent performs the functions of both stenting and drainage, and is applicable in complex posterior urethral surgery.

Adolescent↗

Appendix as continent urinary reservoir outlet.

The submucosally embedded in situ appendix offers an ideal continence mechanism in patients with ileocaecal urinary reservoir. Ninety-two patients who underwent this procedure at three urology centres (48 patients in Mainz, 20 in Wuppertal-Barmen and 24 in Marburg) are reviewed. The appendicocutaneous stoma was placed in the lower right abdominal quadrant in 12 patients (most of whom previously had bladder exstrophy) and into an umbilical or, in some exstrophy cases, a neoumbilical tunnel in 80 patients. Urodynamic investigations of the submucosally embedded appendix showed maximum closure pressure greater than 80 cm H2O.

Appendix↗

[Complications of Bricker ileal conduit urinary diversion: analysis of a series of 246 patients].

OBJECTIVE: The objective of this retrospective study was to evaluate the mortality and early and late morbidities of Bricker ileal conduit urinary diversion. PATIENTS AND METHODS: Between January 1990 and December 2002, 246 Bricker ileal conduit urinary diversion was performed in our centre in 164 males (67%) and 82 females (33%) with a mean age of 64 years (range: 9 to 90 years). Bricker diversion was performed in 73.6% of cases for underlying tumour (prostate, bladder, cervical, colon cancer), and in 26.4% of cases for benign disease (neurogenic bladder radiation bladder bladder exstrophy, incrusting cystitis). Cystectomy was associated with Bricker diversion in 62.2% of cases. The mean follow-up was 24 months (range: 1 to 151 months). The following parameters were studied: mortality and early and late medical or surgical, urological and gastrointestinal complications. RESULTS: The postoperative mortality was 6.9% (17 deaths, 16 in patients in whom Bricker diversion was performed for cancer). The early morbidity was marked by gastrointestinal complications (ileus, fistula, evisceration) in 46 cases (1.7%), 25 of which required re-operation. A medical complication was observed in 41 patients (16.7% of the series), responsible for 60% of the postoperative mortality (10 of the 17 deaths). A urinary fistula was observed in 7 patients (2.8%). The late morbidity consisted of parietal complications (incisional hernia, peristomal hernia, stricture of the stoma) in 18.3% of cases. Urological complications consisted of acute pyelonephritis (11%), ureteroileal stricture (4.9%) and urinary stones (4.9%). CONCLUSION: Bricker ileal conduit urinary diversion is associated with considerable mortality, especially in cancer patients. Early complications are essentially gastrointestinal, while late complications tend to be parietal or urological.

Adolescent↗

Management of the fetus with an abdominal wall defect: experience of 31 cases.

The diagnosis of ventral abdominal wall defect can now be made prior to birth. With this diagnosis, the family can make decisions and a planned optimal management can lead to a successful outcome. There were 31 cases of ventral wall defect identified at Chang Gung Memorial Hospital (CGMH) from January 1979 through March 1988. Twenty of them were classified as gastroschisis; among them, 17 (85%) were born in outside clinics and none of them had associated anomalies. In contrast, among 11 cases of omphalocele, there was a lower frequency of transferred cases (27% vs 85%), and 4 cases had additional defects, including two multiple anomalies and two bladder exstrophies. There were no significant differences between gastroschisis and omphalocele in the mortality rate (30% vs 36%), in the incidence of intrauterine growth retardation (IUGR) (30% vs 27%) and in the Cesarean section rate (15% vs 18%). All 4 cases of prematurity (less than 36 weeks of gestational age) expired after delivery and 2 of these had body weights of less than 1500 g. Three out of 5 cases delivered by Cesarean section expired; the mortality (60%) was higher than that of vaginal delivery (28%). All 3 cases were gastroschisis, 2 of them were transferred from outside clinics and all expired due to sepsis. The diagnosis of ventral wall defect should be made prenatally, with obstetric ultrasonography, maternal serum alpha-fetoprotein screening and fetal karyotyping. Therefore, fetal transport in utero to a referral center and optimal perinatal care for those fetuses with potentially correctable lesions can be well planned.

Abdominal Muscles↗

Continent, nonrefluxing urinary reservoir constructed from ileum: report of two cases.

Most procedures for diverting urine sacrifice continence, but Kock's modification of the ileal conduit promises the ideal of appliance-free urine storage with voluntary control of emptying. The authors report on two men who underwent this procedure. One was a paraplegic who had total urinary incontinence after a sphincterotomy. The other had a carcinoma of the rectosigmoid obstructing the right ureter. He had congenital bladder exstrophy and had undergone ureteric implantation into the sigmoid colon at 3 years of age. In both patients, the reservoir was created from an isolated 60-cm segment of the middle portion of small bowel. Two nipple valves were created - one to prevent reflux and one to provide continence of urine. Follow-up was 27 and 17 months respectively. One patient had his outlet valve revised because of sliding and incontinence. He is now completely continent of urine and he intubates his reservoir three to four times daily. Postoperatively, the second patient had a leak at the ureteroileal anastomosis, but this healed spontaneously. The continent urinary diversion is a major intestinal operation and should not be performed in conjunction with exenteration. It should be confined to a few centres where greater experience can be accumulated.

Adult↗

Chloroprocaine for epidural anesthesia in infants and children.

The authors discuss their experience with chloroprocaine for epidural anesthesia in five pediatric patients. While bupivacaine remains the most commonly used local anesthetic in children, recent reports of toxicity document the risks of this agent. The major advantage of chloroprocaine is its rapid metabolism, which thereby minimizes the risks of toxicity, especially in patients with preexisting problems such as young age or underlying hepatic dysfunction, which may limit the metabolism of local anesthetics of the amide class. In three cases, the epidural infusion was combined with the general anesthetic. The cases included hepatic resection, repair of bladder exstrophy, and correction of duodenal atresia. In two other cases, epidural anesthesia was used instead of general anesthesia in a former preterm infant who was undergoing inguinal herniorrhaphy and for lower extremity orthopedic procedures in a child with myotonic dystrophy. In all cases, chloroprocaine was chosen because of preexisting or associated conditions that might increase the risk of bupivacaine toxicity, such as hepatic resection, repeated dosing in a neonate, or the need for higher concentrations of local anesthetic to achieve adequate surgical conditions. Adequate intraoperative conditions were achieved in all five patients. No complications related to chloroprocaine epidural anesthesia were noted. This initial experience suggests that chloroprocaine offers an acceptable alternative to bupivacaine for epidural anesthesia in the pediatric population.

Anesthesia, Epidural↗

[Colonic adenocarcinoma after Coffey's operation. A case].

The authors describe a case of colonic adenocarcinoma in an 41-year-old woman who had undergone at age 3 an uretero-colic anastomosis because of bladder exstrophy. The tumor looked like an ovarian tumor and histology confirmed it was a Krûckenberg tumor of colonic origin. The authors review literature and insist on the necessity of a life long endoscopic follow-up of uretero-colic anastomosis.

Adenocarcinoma↗

[OEIS complex. Description of a case associated with cardiopathy].

We describe a newborn affected by OEIS complex (Omphalocele--Exstrophy of bladder--Imperforate anus--Spinal defect) associated with atrial septal defect. Terminal ileostomy, closure of the abdominal wall defect and suture of the cecal and vesical plates were performed. The patient died at 5 months and four days.

Abnormalities, Multiple↗

[Latex hypersensitivity in children].

Latex (natural rubber), which is obtained from the Hevea brasiliensis tree, is known to cause contact urticaria and aggravate pre-existing atopic dermatitis. We present a 4.5 year-old boy with bladder exstrophy and epispadias who developed generalized urticaria and bronchospasm during general anesthesia prior to correction of the epispadias. He had undergone previous urologic operations uneventfully. Allergic evaluation revealed atopy with specific IgE antibodies against latex. Immediate skin tests performed with the medications given during the general anesthesia were negative, supporting the possibility that his anaphylactic reaction was caused by exposure to latex during intubation and ventilation. Similar cases have recently been described, especially in children with congenital urogenital abnormalities who are frequently exposed to latex (catheters, surgical gloves). Exposure to latex in these children should be minimized. In addition, they should be prepared with orally administered corticosteroids and antihistamine medication prior to operation.

Anaphylaxis↗