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.

Antegrade continence enema--an analysis of 20 children with faecal incontinence.

AIM OF THE STUDY: We present our experience with the Malone antegrade continence enema technique in children suffering from severe anal incontinence. METHODS: The Malone antegrade continence enema technique was performed using the appendix, caecum or ileum in 20 children with faecal incontinence. The stoma was kept catheterised for three weeks after the operation in all patients and irrigation with saline was initiated after that period. Tap water was used for irrigations after discharge from hospital. The functional success was evaluated according to the classification defined by Curry et al. RESULTS: The mean age and the mean follow-up period was 11 years and 5 years, respectively. Strictures developed in five of the 20 cases (25 %) in this series. Four of these patients underwent revisional surgery, but in one case with appendiceal tube the stricture and catheterisation difficulty resolved spontaneously. Full continence was achieved in 13 of the children (65 %). CONCLUSIONS: The Malone procedure is an effective procedure in children with faecal incontinence. It should be performed after confirming that the patient is completely clean using Peña's programme and aften ensuring that both the child and the parents will properly follow the recommendations.

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.

Hypospadias reoperations.

AIM: To retrospectively evaluate the experience of a single surgeon (YS) with hypospadias reoperations. PATIENTS AND METHOD: 105 hypospadias patients were reoperated by the same surgeon between 1994 - 2003. The patients were classified into three groups according to the surgical technique employed. Urethral plate tubularisation was performed in Group I. Repair with genital skin or mucosa was carried out in Group II and repair with extragenital tissues was performed in Group III. RESULTS: Mean age at operation was 6.5 years (range: 1.5 - 23 yrs). Mean number of operations performed was 2 (1 - 7). Group I consisted of 33, Group II of 64, Group III of 8 cases. Reasons for reoperation were complete or partial neourethral loss in 71 cases; big and multiple fistulas in 29; neourethral or meatal stenosis in 11 children. One had diverticula. Two had partial corpus cavernosal loss. Residual or secondary fibrotic ventral flexion was detected in 31/105. Complication rates were 15%, 25% and 0% in Group I, II and III, respectively. Overall complication rate was 20% (21/105). CONCLUSION: Severe complications can develop not only in proximal but also after distal hypospadias repairs. Local supportive tissues can be provided more easily in proximal reoperations. Repair with buccal mucosa had no complications in our series as it was only used in well selected cases. If urethral plate is present and wide enough, its tubularisation should lead to better results in reoperations. If it is absent or narrow, meatal based or onlay island flaps should be employed. Though the onlay island flap is known to be well vascularised theoretically, our results with meatal based flaps were better. We preferred using the tunica vaginalis when urethral plate was absent and penile skin was not appropriate. We suggest using the onlay technique for large penile defects of the shaft and not in the glanular area. But its use as local supportive tissue in reoperations can be very helpful.

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.

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.

Is intestinal ischemia a risk of laparoscopy? An experimental study in rabbits.

BACKGROUND AND PURPOSE: In the literature, there are few reports documenting intra-abdominal organ necrosis following laparoscopic procedures. This experimental study was planned to investigate whether intestinal ischemia develops during laparoscopic procedures and if laparoscopy could cause intestinal necrosis. MATERIAL AND METHODS: Two experimental groups, each consisting of 10 adult New-Zealand rabbits, were used in this study. The first group comprised the study group which had pneumoperitoneum, the second group comprised the normal animals serving as the controls. A cervical tracheostomy was performed to achieve successful general anesthesia in both groups. In the study group, intraperitoneal CO2 insufflation was carried out and intraabdominal pressure (IAP) was adjusted so as not to exceed the arterial blood pressure. After 20 min high IAP period, the intraabdominal gas was aspirated. Five minutes later, samples of both small intestine and colon tissue were taken. In the control group, tissue samples were taken 25 min after anesthesia was achieved. Xanthine oxidase (XO) and malondialdehyde (MDA) levels were measured as indicators of intestinal ischemia and lipid peroxidation in the intestinal tissues. Statistical analysis was done to compare the XO and MDA levels of the small intestines and colons of both groups. RESULTS: The mean colonic XO levels were 1.323+/-1.17 and 0.217+/-0.27 (U/mg protein) in study and control groups, respectively. This difference was statistically significant (t = 2.60, p<0.05). The other comparisons with regard to XO and MDA levels were statistically not significant. CONCLUSION: Our results demonstrate that intraperitoneal CO2 insufflation in which intraabdominal pressure was adjusted to be lower than arterial blood pressure may affect oxygenization of the colon.

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.

Association of biliary atresia and urogenital sinus.

A case of an association of extrahepatic biliary atresia (EHBA) and urogenital sinus (UGS) anomaly that was diagnosed as an urachal remnant antenatally is reported. Diagnostic laparoscopy of the abdominal cavity in the postnatal period was the most helpful step for making the diagnosis. Biliary atresia and urogenital sinus, which was the cause of bladder outlet obstruction, were treated successfully. To the authors' knowledge, this is the first case of this type of association introduced to the literature. J Pediatr Surg 36:635-637.

Diagnostic laparoscopy in prolonged jaundice.

BACKGROUND: The early diagnosis of surgical jaundice in a neonate is an important step for the surgical success in extrahepatic biliary atresia. Diagnostic laparoscopy, as in many areas in surgery, is included in the conventional diagnostic methods of extrahepatic biliary atresia. METHODS: Since 1992, 24 infants with prolonged jaundice, in whom extrahepatic biliary atresia and neonatal hepatitis could not be differentiated with conventional diagnostic interventions, have been evaluated laparoscopically. RESULTS: A coarse, irregular, greenish-brown liver with some degree of fine angiomatous development and an atretic gallbladder are the findings of laparoscopic evaluation in an infant with extrahepatic biliary atresia. However, in neonatal hepatitis, the liver is smooth, sharp-edged, and chocolate brown in color, and simultaneously performed cholangiography should show the passage of the contrast material both into the proximal biliary tracts and the intestinal system. In this series, 10 of 24 cases were proved to be neonatal hepatitis diagnosed by laparoscopy, so unnecessary laparotomy was avoided in 42% of the cases. CONCLUSION: When the diagnostic laparoscopy, in which the liver and the gallbladder are directly visualized, is combined with the cholangiographic examination, the most accurate and earlier diagnosis in an infant with prolonged jaundice can be achieved, and the important period of time for the surgical success in extrahepatic biliary atresia will be minimally wasted.

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.

A case of wandering spleen causing urinary symptoms after correction of a posterolateral diaphragmatic hernia.

A 12-year-old girl who had had an operation for a posterolateral diaphragmatic hernia was admitted to the hospital because of enuresis. Physical examination and radiologic studies revealed the spleen behind the urinary bladder, left-sided hydronephrosis, and a high volume of residual urine. The hydronephrosis and residual urine resolved immediately following splenectomy.

Anorectal manometry during the neonatal period: its specificity in the diagnosis of Hirschsprung's disease.

In this study the reliability of anorectal manometry (ARMM) in Hirschsprung's disease (HD) in the neonatal period is evaluated. ARMM was performed in 59 patients (age ranging between 2 to 90 days) in whom HD was suspected. Rectal biopsy was performed in 23 newborn whose rectoanal inhibitory reflex (RAIR) was not identified in the ARMM study and in 13 RAIR-positive newborn due to strong clinical signs supporting HD or persistence of symptoms during their follow-up. Other RAIR-positive patients were followed up to 4 months to 3.5 years and no problems were encountered. Among the 36 newborn with rectal biopsies, if the results were compared to ARMM studies, there were one false-positive and two false-negative results. The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of ARMM in the diagnosis of HD are 91.6%, 95.6%, 84.6% and 91.6% respectively. We conclude that ARMM can be used as a screening test in patients in whom HD is suspected during the neonatal period, but for definitive diagnosis it must be combined with other diagnostic tests.

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.

Neurovesical dysfunction in patients with anorectal malformations.

UNLABELLED: The aim of the study is to investigate the incidence of neurovesical dysfunction (NVD) in patients with operated anorectal malformations (ARM) and its relationship with sacral abnormalities. Between September 1992 and April 1994 cystomictiometric and electromyography investigations were performed for 33 patients operated on for anal atresia. The age of these 14 male and 19 female patients ranged between 1-17 years (mean age: 7.7). The level of ARM was supralevator in 10 patients and infralevator in 23 patients. The x-ray of 14 patients showed lumbosacral abnormalities. In urodynamic investigation, NVD was observed in 15 patients (45.4%). 4 of the patients had signs developed secondary to organic subvesical obstruction. The urodynamic results of 14 patients were in the normal range. NVD rate was found to be 70% in patients with supralevator anal atresia, 34.7% in patients with infralevator anal atresia, 64.2% in patients with sacral abnormalities and 31.5% in patients with no sacral abnormality. CONCLUSION: NVD incidence increased in patients with supralevator ARM and in patients with sacral abnormalities. However, presence of infralevator abnormality or absence of sacral abnormality does not eliminate NVD. We think that, whenever NVD is suspected in a patient with ARM, urodynamic investigations should be performed as a routine investigation of the urinary tract.

Wandering spleen in childhood: a report of three cases.

Wandering spleen is a rare cause of abdominal pain in children, and an accurate diagnosis is seldom made preoperatively. A splenectomy is the treatment of choice in cases of splenic torsion and infarction, while in patients with chronic symptoms splenopexy may also be attempted. We herein report three patients with wandering spleen, of whom two presented with acute torsion of the splenic pedicle and one demonstrated an asymptomatic mobile abdominal mass. In the first case splenopexy was attempted, but during follow-up the spleen was found to have undergone atrophy. The presentation, diagnostic procedures, and treatment modalities in pediatric wandering spleen are reviewed.

Comparison of urodynamic investigations before and after posterior sagittal anorectoplasty for anorectal malformations.

METHODS: The authors evaluated 21 patients (10 boys, 11 girls) who had anorectal malformations (ARM). Ten of them had infralevator (low-group I) and 11 of them had supralevator (high-group II) type ARM. All of the patients underwent urodynamic investigation before and after posterior sagittal anorectoplasty (PSARP) operation. RESULTS: Sacral and spinal anomalies were found in 54% (6 of 11) patients who had supralevator type ARM. None of the patients with infralevator type ARM had additional sacral or spinal anomalies. Before PSARP operation urodynamic investigations of all infralevator type ARM patients were within normal limits, whereas 82% (9 of 11) of patients with supralevator type ARM showed neurovesical dysfunction (NVD). There was no significant difference between the preoperative and postoperative urodynamic findings. The possibility of additional sacral or spinal anomalies and NVD in supralevator type ARM was high. After PSARP operation no additional lower urinary tract dysfunction was detected in the urodynamic evaluation of ARM patients.

The effects of intranasal midazolam on urodynamic studies in children.

OBJECTIVE: To determine the influence of midazolam on the function of the lower urinary tract when used to sedate the patient. PATIENTS AND METHODS: Urodynamic studies were performed on 20 patients (seven boys and 13 girls, mean age 4.04 years, range 1 day to 10 years) before and after the intranasal administration of 0.5 mg/kg of midazolam. The patients' heart rate, respiratory rate and oxygen saturation were monitored and the level of sedation recorded by an anaesthesiologist and a surgeon during the study. RESULTS: Heart rate and respiratory rate did not change and the percentage oxygen saturation remained stable throughout the study period. The childrens' behaviour began to change 3-5 min after the intranasal administration of midazolam. The sedation lasted 2 h and the full co-operation of the patient was assured. The difference between the initial and post-midazolam evaluation of maximal cystometric capacity, contractility, compliance, intravesical pressure, maximum flow rate, intravesical pressure at maximum flow and the amount of residual urine were statistically insignificant (P > 0.05). Electromyographic characteristics showed no significant change with the use of midazolam (P > 0.05). CONCLUSION: Despite the suggestion that benzodiazepines, especially diazepam, are useful for the relaxation of pelvic floor striated musculature in voiding dysfunctions, there was no effect on urodynamic variables when midazolam was used in a single dose intranasally. The anxiolytic and sedative effects allowed children to undergo urodynamic studies in comfort.