The roles of extracellular matrix proteins, apoptosis and c-kit positive cells in the pathogenesis of ureteropelvic junction obstruction.

AIM: To investigate histopathological changes in ureteropelvic junction obstruction (UPJO) from an etiological perspective. PATIENTS AND METHODS: Medical records of patients with UPJO were reviewed and pathological specimens collected. Nephrectomy materials from forensic autopsies were taken as controls. Specimens were assessed with light microscopy. Fibronectin, type 4 collagen, laminin, Bax and Bcl-2 expression for apoptosis, together with interstitial cells of Cajal determination with c-kit were determined immunohistochemically. Staining scores were evaluated semiquantitatively. Results were evaluated using Mann-Whitney U-test. RESULTS: Control group comprised 14 children (median age, 3.5 years; 6 months-17 years). Study group comprised 22 children with UPJO (median age, 9 months; 1 month-10 years). Light microscopy revealed non-specific inflammation, epithelial proliferation and atrophy with fibrosis in the smooth muscle of the UPJ in all patients. Fibronectin, type 4 collagen and laminin were found to be significantly increased in UPJO at the intrafascicular space of smooth muscle and the matrix of stroma. Bcl-2 expression was increased in UPJO. c-Kit was unable to stain interstitial cells of Cajal, but staining for mast cells was significant. CONCLUSIONS: High expression of fibronectin, laminin and type 4 collagen may indicate a relation to the pathogenesis of UPJO. Defective kidney morphogenesis, during branching and tubulogenesis of ureteric bud, may be responsible for this congenital pathology.

Feminizing surgical management of intersex patients.

The study's objective was to evaluate the results of surgical modalities for children with ambiguous genitalia. The records of 55 patients who were reared as females between 1985 and 2001 were reviewed regarding diagnosis, age at surgery, operative procedures, and outcome. The mean age at surgery was 3.5 years, and the follow-up period averaged 4.1 years with a range of 2 months-17 years. The types of reconstructive surgical techniques were clitorovaginoplasty in 29, staging clitoral surgery and vaginoplasty in seven, clitoroplasty in five, total urogenital mobilization (TUM) in three, vaginal bowel substitution in two, clitoridectomy in one, and gonadectomy in six, and two are waiting for vaginal substitution surgery after gonadectomy. The main complications were vaginal stenosis in four patients. All of the TUM patients had good appearances of their urethral orifice and vagina, all of them were continent, and none of them had urinary tract infections. With our limited experience with the TUM procedure, we feel that it is possible to obtain a better cosmetic and functional result with an easier technique. Among the 10 patients of postpubertal age, none of them had had sexual experience. Eight of the postpubertal patients asked questions about their reproductive status. Patients with an intersex disorder should be informed about their problems, especially about their reproductivity.

Modified Barcat technique in distal hypospadias repair.

AIM: A modified Barcat operation, when carried out properly, provides an anatomically and cosmetically successful result in distal hypospadias repair in each glans configuration. We retrospectively evaluated our experience with this technique in the last five years. PATIENTS AND METHODS: 78 children with primary distal type hypospadias were operated between 1998 - 2003, using a modified Barcat technique. RESULTS: The mean age of the patients was 3 years (6 months - 14 years). The mean follow-up period was 2 years (1 month - 60 months). Nesbit plication was performed in four cases with chordee. Fistula developed in five (6.5 %) cases and required reoperation. Stenosis which did not require surgical intervention occurred in 5 children and resolved with self-dilatations. CONCLUSION: The Barcat technique is an appropriate operation for distal type hypospadias cases but should be modified by creating second tissue layers over the neourethra and a wide meatus.

Changes in the threshold voltage and alterations of smooth-muscle physiology after bladder outlet obstruction.

The purpose of this study was to determine the resultant changes in the bladder function after partial bladder outlet obstruction (BOO), with particular emphasis on the threshold voltage and on the role of NO. A total of 12 adult rabbits were used in the study. New Zealand-type rabbits were subjected to no intervention and 3 weeks duration of a partial outlet obstruction. They underwent an in vivo cystometric evaluation before and after the BOO, histological studies, bladder-strip stimulation studies using acetylcholine, electrical field stimulation, and relaxation studies using NO donor nitroprusside. Statistical significance was determined by two-way ANOVA for multiple variations and Student's t-test. Histology sections demonstrated smooth-muscle hypertrophy, hyperemia of the vessels in the wall and widely set mononuclear cell infiltration in the rabbits with partial BOO. Cystometry showed markedly decreased bladder capacities, and decrease of compliance from 4.3 +/- 1.8 to 1.6 +/- 0.3 in the obstructed group. Tissue bath studies demonstrated no meaningful change with cholinergic stimulation, increased contractility in response to electrical field stimulation, and increased threshold voltage values from 57.5 to 93.3 compared to controls. Nitroprusside did not induce relaxation of the neostigmine and acetylcholine-precontracted bladder in all animals. Hypertrophy was observed due to adaptation of the detrusor against the obstruction. During this adaptation period, bladder capacity decreases and the elasticity disappears, thus causing higher pressures with lesser volumes. In other words, compliance decreases. The neurogenic damage can be shown with increasing values of threshold voltage, and higher voltages are needed to start a contraction. Nitroprusside has no inhibitory effect on smooth-muscle tone in the rabbit bladder.

Urodynamic findings of posterior urethral valve patients.

The most frequently observed cause of obstructed bladder in children is the posterior urethral valve (PUV). In this report, we analysed the urodynamic findings of 26 patients whose valves were fulgurated 12.6 months previously (range: 2 days - 8 years,after the fulguration). The mean age of the patients at the time of the procedure was 4.5 years (range: 2 months -13 years). Bladder capacity was decreased in 15, increased in 6, and normal in 5 patients; hypo-compliance was observed in 13, hyper-compliance was observed in 4, and normo-compliance was observed in 9 children. Generally, hypo-compliance and decreased bladder capacity was more frequent in patients younger than 4 years of age. There were 10 patients with instable detrusor contractions(IDC) and high residual urine was present in 8 patients. Eight patients developed chronic renal failure and 6 of these patients had high residual urine. All the patients who required bladder augmentation during follow-up were the ones treated after 2.5 years of age; 4 of these 5 patients had hypo-compliance and low bladder capacity on urodynamic studies. In conclusion, all patients with PUV had pathological urodynamic findings that could change with age, and early relief of the infravesical obstruction could have an improving effect on bladder function. Urodynamic investigations may help us to design the proper treatment according to the bladder function.

Catheterisable continent urinary diversion (Mitrofanoff principle)--clinical experience and psychological aspects.

48 Mitrofanoff principles (MTR) were performed on 46 patients (male : female ratio, 30 : 16) with a mean age of 9.1 years (range 2.5 to 24 years). The primary diagnoses were neurogenic bladder in 11, infravesical obstruction in 7 and bladder exstrophy-epispadias complex in 28 patients. The most common type of conduit was appendix (38 cases); other conduits were constructed from the ileum (seven) and ileocaecum (one). In two cases with bladder substitution the uterine tube and tubularised bladder stump were used as perineal MTR. 33 of the 46 children underwent augmentation cystoplasty in conjunction with the MTR procedure. The Malone procedure for antegrade colonic enema (ACE) was performed at the same stage with MTR in eight cases. To achieve continence, bladder neck reconstruction was performed in 32 patients and the bladder neck was closed in four patients during the same operation and MTR procedure. The mean follow-up period was 28.7 months (range one month to 57 months). To assess the psychological aspects of the MTR procedure, the Rosenberg Self-Esteem Scales were completed by 12 children older than eight years of age. Nine patients had problems with the MTR (19.5 %). Three appendiceal MTR had strictures at the skin level, 2 of which needed minor surgical revisions. A mucocele formation at the skin level of an appendix was removed successfully. We did not observe any complaints among the other appendiceal conduits. All the tapered ileum conduits were difficult to catheterise, and 1 of them had a leakage from the stoma. None of the three transversely tubularised ileum MTRs had problems with catheterisation or leakage. A stricture of the conduit from the uterine tube was observed. 36 of the 42 patients are now continent, giving a ratio of 86 %. The results of the Rosenberg Self-Esteem Scales revealed that there was an increase in the percentage of patients with high self-esteem, and a decrease in depressive feelings after the MTR procedure. We conclude that the MTR procedure provides excellent continence, offers good prospects of a socially acceptable life with increased self-esteem, and the appendix seems to be the ideal organ for continent urinary diversion, with the transverse tubularised ileal tube as a second choice.

Experience with laparoscopy in nonpalpable testis.

AIM: The aim of this study was to investigate the feasibility and benefits of diagnosis and interventional laparoscopy in those paediatric patients with nonpalpable testes (NPT). PATIENTS AND METHODS: Between 1992 and 1999, 75 patients with NPT (mean age 3 years, range 6 months to 14 years) were treated. 86 testes were evaluated. RESULTS: According to the laparoscopic findings 4 groups of testes were identified: Vanishing testis (n = 32), low abdominal testis (< 2 cm to the internal ring) (n = 26), high abdominal testis (> 2 cm to the internal ring) (n = 24) and intersex patients (n = 4). Of the first group, 19 testes (one bilateral) had blind-ending spermatic cord and vessels and if an atrophic testicular tissue was identified, it was removed laparoscopically. For those with spermatic cord and vessels beyond the internal ring (13 testes), atrophic testes were removed through a high scrotal incision. 19 testes of the second group had a laparoscopy-assisted orchidopexy. In the same group a laparoscopic orchidopexy was performed on 7 testes. 24 testes in the 3rd group had a Fowler-Stephens (FS) stage 1 and 18 testes had a laparotomy performed for FS stage 2 procedure (laparotomy and orchidopexy) after 6 months. At laparotomy there was no evidence of testicular atrophy in all but one testis, which was removed and the FS stage 2 procedure was completed in 17 testes. The follow-up period was between 6 months and 4 years, and two more testicular atrophies were noted after FS stage 2. The results were satisfactory in 15 out of 18 testes (83%). In the intersex group, the patient with testicular feminization underwent laparoscopic orchiectomy. The other patient with bilateral nonpalpable testis was identified as having an uterus and two intraabdominally located gonads on laparoscopy and gonadal biopsies were obtained for diagnosis. Histology demonstrated bilateral ovotestes, confirming the diagnosis of a true hermaphrodite. CONCLUSION: We are of the opinion that laparoscopy decreases the number of laparotomies in NPT, allows a single-stage procedure in low abdominal testis, and facilitates clip ligation of the testicular artery in high abdominal testis. Laparoscopy also provides diagnostic and therapeutic options for vanishing testis and intersex patients.

A critical observation about the pathogenesis of abdominoscrotal hydrocele.

Abdominoscrotal hydrocele is a very rare anomaly. Although various theories about this condition have been proposed, controversy still continues on the etiology of this lesion. The authors present two consecutive cases of abdominoscrotal hydrocele in infancy. On their first examination, these patients only had inguinoscrotal hydroceles. After a 2-month period of observation, these inguinoscrotal hydroceles developed into abdominoscrotal hydroceles. These findings also were confirmed by ultrasonography. The authors question the currently proposed theories of abdominoscrotal hydrocele development.

Effects of male sex hormones on urodynamics in childhood: intersex patients are a natural model.

The effects of sex hormones on bladder function have been evaluated in adult females, especially in regard to postmenopausal incontinence and bladder irritability syndromes. These have not been investigated in children in regard to urodynamic findings. An intersex patient whose bladder is under the influence of androgens is a natural model to investigate the effects of male sex hormones on bladder function in females. To evaluate the urodynamic findings and clinical symptoms in a group of intersex patients and to determine how androgens influence bladder function in female children, clinical and urodynamic records of 12 intersex patients with adrenogenital syndrome were investigated retrospectively. The mean age was 9 +/- 5.7 years (1.5-18) and the mean follow-up period was 5.1 +/- 4.4 years (1-12). Congenital adrenal hyperplasia (CAH) was present in all cases. Only 3 patients had urinary symptoms and incontinence, but these findings did not correlate with their urodynamic findings. None of the patients required medications for their urinary symptoms. Nine are still being treated medically by the pediatric endocrine team with hydrocortisone for CAH. The upper urinary tract was found to be normal with no hydronephrosis. The mean bladder capacity (269 +/- 122 ml) was lower (86.7%) than the estimated capacity for age. The mean compliance was 20 +/- 13.7 ml/cmH2O. No unstable detrusor contractions were encountered. The most remarkable finding was this reduced bladder capacity of androgenized female patients for age, which shows the antagonistic effect of androgens on bladder urodynamics in females.

Modification of the Koyanagi technique for the single stage repair of proximal hypospadias.

PURPOSE: We describe a modification of the Koyanagi technique for hypospadias. Use of opposing parameatal-based skin flaps that extend distally to incorporate the inner layer of the prepuce was modified to preserve blood supply to the flaps in an attempt to reduce complications and improve results. MATERIALS AND METHODS: During the last 7 years 20 boys underwent treatment of proximal hypospadias using the modified hypospadias repair. RESULTS: Cosmetic and functional, long-term (mean 34 months) results were excellent. Complications consisted of 4 urethrocutaneous fistulas (20%). There were no instances of meatal stenosis, diverticulum or urethral stricture. CONCLUSIONS: The modified technique permits 1-stage repair of proximal hypospadias with a low complication rate.

Duplicated exstrophy in one of identical twins.

The characteristic of duplicated exstrophy is the presence of a patch of exstrophic bladder mucosa in the infraumbilical region with a low-set umbilicus, in addition to the normal bladder. Musculoskeletal defects, diastasis pubis, and rectus abdominis can also be found in these patients. We report the first case of a female twin baby with appearance of a low-set umbilicus and diastasis pubis associated with a pubic sinus. The external genitalia and urinary continence were normal. Duplicated exstrophic mucosal remnant was excised, and the histopathologic study of the specimen confirmed urothelium.

Multidisciplinary approach to Wilms' tumor: 18 years of experience.

BACKGROUND: The aim was to evaluate the characteristics of Wilms' tumor and the results of combined modality treatment obtained in our center in Turkey. METHODS: From January 1978 to December 1996, 106 patients with Wilms' tumor were diagnosed. Of these 106 patients, 61 were male and 45 were female (M/F = 1,35); the median age at diagnosis was 39 months. The distribution of the 106 patients according to clinical stage was stage I 10%, stage II 42%, stage III 35%, stage IV 9% and stage V 4%. Histologically, 102 of the cases could be evaluated: favorable histology was diagnosed in 88.2% and unfavorable histology in 11.8% of the patients. Ninety-eight patients were treated according to NWTS and eight patients according to SIOP protocols. RESULTS: The EFS and overall survival rates at 2 years were 74.2 and 79.5% respectively, and at 5 years 72.4 and 76.6% respectively. CONCLUSION: As a developing country we evaluated our survival rates and report an improvement in treatment in recent years.

Esophageal replacement using the colon: a 15-year review.

Colonic interposition is a treatment option in childhood when esophageal replacement (ER) is necessary. We reviewed 18 children who underwent ER by colon between 1984 and 1999. There were 5 with esophageal atresia and 13 with corrosive esophagitis; 15 had long-term follow-up (mean 38 months). Three procedures were performed by the Waterston technique and 12 by the retrosternal technique. ER was completed in a single stage in all but 1 patient. Pyloroplasty or antireflux surgery were not done routinely during colonic interposition. As early complications, we observed 11 cervical leaks and 2 pulmonary problems. As late complications, there were 4 redundancies, 3 gastrocolic refluxes, 2 cervical anastomotic stenoses, and 1 each intestinal obstruction due to adhesions, cologastric stricture, cosmetic deformity of the thorax, and bulging of the neck. Six patients with complications required secondary surgery. There were 4 deaths, 2 of them unrelated to the surgery. Cervical leakage, which was the most commonly observed problem, healed well. We believe the colon is still one of the best substitutes for the esophagus and that there is no need to perform a routine pyloroplasty or antireflux procedure as an adjunct to the primary surgery.

Bladder augmentation: urodynamic findings and clinical outcome in different augmentation techniques.

OBJECTIVE: To compare the urodynamic findings and clinical outcome in different bladder augmentation techniques. PATIENTS AND METHODS: From 1987 to 1996, 32 bladder augmentations were performed in 30 (M:F/18:12) patients. Mean age was 8.1 years (range: 1-15 years) and the mean follow-up period was 3.2 years (range: 6 months to 8 years). The following techniques were used for bladder augmentation: sigmoid colon in eleven cases, stomach in nine cases, ileum in six cases, ileocaecum in two cases and rectus abdominis muscle flap (RAMF) in four cases. Seven patients also underwent Mitrofanoff procedure to create a continent stoma. RESULTS: According to the urodynamic evaluation the mean capacity was 237 +/- 120 ml in the colonic group, 115 +/- 86 ml in the gastric group, 240 +/- 45 ml in the ileal group, 250 +/- 0 ml in the ileocaecal group and 30 +/- 10 ml in the RAMF group. The mean compliance was 20.6 +/- 14 ml/cm H2O in the colonic group, 10.7 +/- 10.4 ml/cm H2O in the gastric group, 21.6 +/- 9 ml/cm H2O in the ileal group, 25.5 +/- 5.5 ml/cm H2O in the ileocaecal group, 5.8 +/- 1.5 ml/cm H2O in the RAMF group. The upper urinary tract was normal without hydronephrosis in all but except two patients. These two patients are still treated medically for chronic renal failure by the pediatric nephrology team. The patients are using their Mitrofanoff or native urethra with clean intermittent catheterization (CIC) or self-CIC technique for continence. CONCLUSION: Comparing these five different techniques, ileal, ileocaecal and colonic groups were in advantage to achieve high volume reservoirs, followed by the gastric group. Regarding complications; in colonic cases, mucus problems, stone formation and surgical complications related to gastrointestinal tract (intestinal obstruction, perforation) were the most common ones. Perineal dermatitis was the main complication in gastric cases. In the ileal group, complication rate was low. Absence of mucus production and the low postoperative complication rates were the two major advantages of RAMF technique. However this technique is not recommended as an augmentation procedure due to its low capacity and compliance rates. Recently, ileal augmentation has became the most popular method in our institution.

[Time of disappearance of artificial pneumoperitoneum in children. Diagnostic value]. / La durée de disparition du pneumopéritoine artificiel des enfants. Intérêt diagnostique.

Postoperative pneumascos has always been interesting and provocative for surgeons because its "correct" disappearance time still remains ill-defined. Fifty-six children, for whom Golstein's intraoperative diagnostic pneumascos was carried out to demonstrate a contralateral hernia during the correction of a unilateral inguinal hernia, were followed up for disappearance of their pneumascos with abdominal radiographs taken at the 4th postoperative hour and on the 1st, 2nd, 4th and 8th days. The children were divided up into three groups: 9 children were younger than 6 months, 20 were 6 to 24 months old, and 27 were older than age 2. The intraperitoneal free air present in all subjects 4 hours after surgery has completely disappeared from the radiographs of the children younger than 6 months on the fourth day, and on the eighth day for the children aged 6 to 24 months, while a trace of subphrenic air still persisted in the 7% (2 out of 27) of children who were older than age 2. This work is aimed at establishing a correlation between the child's age and the time of disappearance of the pneumascos.

[Perioperative diagnostic pneumoperitoneum for unilateral inguinal hernias in children]. / Pneumopéritoine diagnostique per-opératoire pour les hernies inguinales unilatérales des enfants.

Fifty-six pediatric patients (2.5 months-13 years old) presenting with unilateral inguinal hernia underwent intraoperative diagnostic pneumoperitoneum (Goldstein test) to evaluate the controlateral groin. Fourteen patients (25%) had positive results and underwent controlateral hernia repair. The positivity is found more frequent in females (37.5%) and in the left sided hernias (50%). The percentage of positive tests has found to be inversely proportional to the age of the patient which is 33% for children under 6 months of age and 18% over 24 months. It is also observed that none of the children with negative test admitted with the complaint of hernia within two years following the operation. Intraoperative diagnostic pneumoperitoneum is a rapid, safe and accurate means to evaluate the contralateral groin in children presenting with a unilateral inguinal hernia. The test eliminates the high negative exploration rate and possible injury to cord structures with routine bilateral exploration.

Congenital lobar emphysema--a report of 5 cases.

Five infants with congenital lobar emphysema whose main symptoms included dyspnea, cyanosis and recurrent respiratory infections, are presented herein. The most reliable diagnostic tool was plain chest X-ray films with antero-posterior and lateral views, while radio-isotopic investigation of the lung perfusion state took second place in the diagnosis of this disease. The affected lobes were the left upper lobe in four patients and the right middle lobe in one. Lobectomy was performed with good results in 4 patients, however, 1 infant was lost following an emergency thoracotomy.

Extensive intestinal aganglionosis.

Extensive aganglionosis very close to the ligament of Treitz and total intestinal aganglionosis are rare forms of Hirschsprung's disease. In these cases, nutrition, fluid, and electrolyte balance are a problem. Although the myectomy-myotomy technique of Ziegler appears hopeful, no effective surgical method has been put into the practice for the treatment of these rare forms of Hirschsprung's disease. We report four cases with extensive intestinal aganglionosis, one of which was total intestinal aganglionosis with involvement of the stomach.

[Early and late results of heterotopic spleen autotransplantation in pediatric splenic trauma]. / Früh- und Spätresultate heterotoper Milzautotransplantation beim kindlichen Milztrauma.

In this study, early and late results of heterotopic splenic autotransplantation in 18 children with type IV splenic injuries (Upadhyaya-Simpson classification) are presented. Splenic scintigrams, quantitative analysis of Howell-Jolly inclusion bodies, immunoglobulin (IgG, IgM, IgA) and complement C3 levels and T and B lymphocyte counts were analysed in the postoperative evaluation of the autotransplantation group. The total follow-up period was 7 years. According to our results, splenic implants increased complement C3 levels and improved filtration function of the splenectomized children. This autotransplantation group has two important characteristics: a) it is one of the largest series of the literature (pediatric age group), with b) longest follow-up period.