Botulinum injection is useless on fibrotic neuropathic bladders.

OBJECTIVE: Studies on the use of intradetrusor botulinum toxin A injection for children with neuropathic bladders are insufficient and the results are controversial. The aim of the present study was to evaluate the effect of intradetrusor botulinum toxin A injection for children with neuropathic bladders that are resistant to anticholinergic treatment, and to reveal any criteria indicating treatment success. PATIENTS/METHODS: Hospital records were reviewed of 16 children with neuropathic bladders due to myelomeningocele, and who had botulinum toxin A injections between 2007 and 2010. Botulinum toxin A (10 units/kg) was injected endoscopically into various sites of the detrusor, except the trigone. The success was defined as complete dryness between clean intermittent catheterizations. Urodynamic studies before and after the application were evaluated and parameters, including bladder capacity (measured/expected) and compliance, were also analyzed. Reviewing the results, patients were then classified into two groups: as having fibrotic bladders (noncompliant, acontractile bladders with high pressures) or overactive bladders. Urodynamic findings and therapy success were then compared between the groups. RESULTS: A total of 19 injections, including repeat injections in three patients, were performed. Results of the 16 initial injections were evaluated. Nine patients had detrusor overactivity, and five out of nine (56%) applications in this group resulted in complete dryness between clean intermittent catheterizations. In bladders with typical detrusor overactivity, there was a significant increase in both the capacity (from 0.53 to 0.74) and compliance (from 4.7 to 8.6 ml/cm H2O). Looking at the seven patients that displayed fibrotic bladders with very low compliance and no contraction at all, none of them presented with notable clinical improvement from injections. Comparing the urodynamic findings, there was no significant difference in compliance (3.1 ml/cm H2O before and 3.5 ml/cm H2O after) and bladder capacity (0.58 before and 0.52 after the treatment) in the fibrotic bladders. DISCUSSION: Despite its worldwide usage and FDA approval, studies on the effectiveness of botulinumtoxin A on neuropathic bladders in children are controversial. There are now numerous studies attesting to the good results of BoNTA in neuropathic detrusor overactivity; however, only scarce reports comment on the specific features of the disease process among patients and reasons for failure in some. In our study, reviewing the urodynamic findings carefully, it was observed that the patients who did not respond to injections were the ones with no contractions, despite high pressures and low compliance. Therefore, describing the indications of BoNTA as neuropathic detrusor overactivity and urinary incontinence despite anticholinergic medications may lead to mistreatment of patients in the decompensated phase of a hyper-reflexive detrusor. Pretreatment urodynamic evaluation might be a good indicator, without biopsies, of estimating the degree of fibrosis and the patients who will benefit from the injection. CONCLUSION: Botulinum toxin A injection in the neuropathic bladder of myelomeningocele patients was found to be ineffective if the detrusor was fibrotic, of low compliance and had lost contractility. Urodynamic findings should be carefully analyzed in order to select appropriate patients that may benefit from Botulinum injection.

Success of Malone's antegrade continence enema (MACE) from the patients' perspective.

PURPOSE: Fecal incontinence (FI) is a devastating problem for children. The failure of optimal medical treatment may require further interventions such as appendicocutaneostomy. We report on a patients' perspective of the success of a Malone procedure for FI. PATIENTS AND METHODS: The records of 32 patients who had undergone ACE procedure in the past 9 years were reviewed. Patients and families were contacted, and telephone inquiries were conducted to assess the overall success of the operation. The questionnaire covered the concerns of patients/families about the stoma, functional results and changes in the patients' quality of life (QOL). RESULTS: The indications for ACE stomas were meningomyelocele in 17 patients, anorectal malformation in 8, Hirschsprung/NID in 3, spinal tumor in 3 and traumatic spinal injury in one. 7 laparoscopic and 25 conventional operations were performed. The vermiform appendix was used in 27 of the patients and a cecal flap was used in 5. The most frequent complication was stenosis of the stoma orifice observed in 14 patients. 8 patients responded to dilatations while 6 patients required minor surgical revision. There were 2 perforations during catheterization, and they required surgical repair of the conduit. 5 patients had mild leakage from the stoma; all 5 were open ACE procedures, and required revision. 25 patients could be reached by phone. 5 of these children stopped using the stoma due to previous perforation in 2 patients and stricture in 1. The remaining 2 stopped using their stomas because "they did not like the idea of it". 16 patients are completely clean. 3 patients have occasional soiling. Only one patient was not satisfied with using the ACE stoma and stated that it did not sufficiently improve her condition. 8 patients complained about the duration of the enema (longer than 1 h); however, 5 of them refuse to use the stoma every day. All patients but one perceived a significant improvement in their QOL. Mean QOL scores before and after the procedure were 5.8 (2-9) and 11.5 (5-14), respectively. CONCLUSIONS: ACE stomas provide a satisfactory improvement in patients' quality of life. Stoma-related complications are not uncommon. The most common problems are strictures, followed by stoma leakage.

Factors affecting renal scar development in children with spina bifida.

BACKGROUND/AIMS: Prevention of renal scarring is the main therapeutic goal in children with spina bifida. We aimed to determine factors affecting renal scar development in these patients. MATERIALS AND METHODS: Records of 312 children admitted between 1994 and 2005 with spina bifida were reviewed. Age on admission, gender, presence of previous febrile urinary tract infections (UTIs), vesicoureteral reflux (VUR), and initial urodynamic findings were noted. Patients were grouped regarding presence/absence of renal scars on DMSA scans. Chi2 and Student's t tests were used for statistical evaluation. RESULTS: Seventy-two patients had renal scars on admission. Mean age was 4.62 +/- 4.59 years for patients without renal scars and 6.35 +/- 4.9 years for patients with scars. Male/female ratio was 1:1 in the scarless group and 1:2 in the group with scars. Previous febrile UTI was present in 11 of 240 scarless patients in contrast to 7 out of 72 patients in the scar group (p > 0.05). VUR was present in only 16.3% of cases without scars, whereas 36.1% of patients in the scar group had VUR. Detrusor overactivity and detrusor sphincter dyssynergia were observed in 67.1% of scarred patients, whereas this figure was 42.4% in the scarless group. The comparison of age on admission, gender, detrusor overactivity, and detrusor sphincter dyssynergia revealed significant differences between patients with and without renal scars. CONCLUSIONS: Late referral, female gender, overactive detrusor, and detrusor sphincter dyssynergia have detrimental effects on renal parenchymal function in spina bifida patients. Patient selection for aggressive treatment using these features may prevent renal parenchymal deterioration.

The influence of urine osmolality and other easily detected parameters on the response to desmopressin in the management of monosymptomatic nocturnal enuresis in children.

AIM: The aim of this paper was to examine the early morning spot urine osmolality and some other parameters easily detected from home chart recordings and history as predictive of the therapeutic response to desmopressin in children with monosymptomatic nocturnal enuresis. METHODS: Sixty seven monosymptomatic nocturnal enuretic children were included in the study. Age, sex, family history, the number of family members and siblings, existence of urgency symptoms, the history of urinary tract infection, sleep patterns, the number of wet nights per month and bedwetting in the same night were recorded. Additionally, spot morning urine osmolality was examined. All children were given desmopressin for at least 2 months. At the end of the treatment period, patients considered as responders and non-responders were compared in all these parameters. RESULTS: Although there was considerable overlap between groups, lower spot urine osmolality was the only data we found statistically significant as predictive of response to desmopressin. Moreover, male predominance, fewer wet nights per month and bedwetting per night were also associated with a better response. CONCLUSIONS: We believe that it is important to characterize such different subgroups that could be used as predictors of a good response to desmopressin.

Bilateral testicular teratoma in infancy: report of a rare case treated by testis-sparing surgery.

Testicular tumors are rarely seen in childhood, with germ-cell tumors as the most common type. Teratoma is second only to yolk sac tumor in frequency. Bilateral testicular teratoma is extremely rare. Orchiectomy has been the standard treatment for most tumors of the testes in the past. However, in children, testis-sparing surgery has become the treatment of choice for benign lesions including teratoma. A 10-month-old infant with synchronous bilateral testicular teratomas, one of which was disclosed by ultrasonography, is presented here. While the larger teratoma required unilateral orchiectomy, the smaller tumor in the contralateral testicle was enucleated by testis-sparing surgery. The follow-up at three years was uneventful with normal development of the testis and the boy. Testicular teratoma may reside in both testicles without clinical symptoms. Ultrasound of both testes provides coherent preoperative diagnosis, allowing the surgeon to consider testicular-sparing procedures, thus preventing anorchia in these rare occurrences.

Clinical results with anterior diagonal iliac osteotomy in bladder exstrophy.

PURPOSE: We report our clinical experience with anterior diagonal iliac osteotomy in 10 patients who underwent surgery for bladder exstrophy. Technique and long-term results are discussed. MATERIALS AND METHODS: A total of 10 boys 1 month to 9 years old with bladder exstrophy underwent this procedure during a 2-year period. RESULTS: None of the patients had bladder closure dehiscence or prolapse after the operation. There were neither infectious complications nor injury to the vessels or nerves in any case. Blood loss was minimal for anterior diagonal iliac osteotomy. The only significant complication in our series was the polypropylene erosion of the urethra, necessitating endoscopic removal in 1 patient 1 month postoperatively. All patients had wide diastasis of the pubis preoperatively (average pubic distance 53.3 cm., average pubic ratio 0.9). At surgery suturing the symphysis after bilateral osteotomy resulted in a satisfactory symphyseal approximation and tension-free closure of the abdominal wall was easily achieved in all cases. Radiological studies at a mean followup of 34.6 months (range 14.8 to 49.5) revealed significant recurrent diastasis of the pubic bones in all but 1 patient in whom bone grafts were applied between the iliac fragments. Mean interpubic distance was 42 cm. and mean pubic ratio was 0.6 at long-term followup. CONCLUSIONS: Diagonal osteotomy may correct the principal bony deformity in exstrophy and enables initial symphyseal approximation. Pubic diastasis may recur, probably due to opening forces generated by soft tissue elements of the pelvis.

Management of instrumental perforations of the esophagus occurring during treatment of corrosive strictures.

BACKGROUND: The initial symptoms of esophageal perforations (EP) may be subtle, but the progression is very rapid, and the outcome may be disastrous unless the diagnosis is made early and proper treatment is started immediately. METHODS: Between 1976 and 1996, 1,249 patients with caustic esophageal burns were treated at Ege University. The study group is composed of 52 patients with instrumental ER Perforations occured during dilatation attempts of esophageal strictures. Twelve patients were referred from other institutions after the occurrence of EP. RESULTS: In two patients, emergency surgical repair of the perforation was possible. Seventeen patients with unilateral and two patients with bilateral empyema were treated by pleural drainages. Anterior retrosternal mediastinal drainage was needed in one patient, and 11 patients required posterior mediastinal drainages. Three patients were treated by both anterior and posterior mediastinal drainage. Tracheoesophageal fistulas (TEF) developed in eight patients immediately after a dilatation attempt. Seven of these patients required esophageal replacement with colon to bypass the fistulas, and one patient in this group healed spontaneously. EP healed in 42.5 +/- 49.4 days. Twelve (23%) patients died of mediastinitis and sepsis. CONCLUSION: When EP is diagnosed and treated with these methods, the mortality rate should approach zero.

Is resolution of vesicoureteric reflux by conservative management predictable in patients with myelodysplasia?

OBJECTIVE: To evaluate the probable factors that might predict the outcome of conservative management of vesicoureteric reflux (VUR) in myelodysplastic patients. PATIENTS AND METHODS: A retrospective review of 24 children with VUR secondary to neurogenic bladder (15 girls and 9 boys, age range 1-18 years) out of 75 myelodysplasia patients between 1994 and 1996 was made. Patients were grouped according to their response to conservative management: Group I: patients with their VUR resolved or downgraded (n = 15), and group II: patients with their VUR unchanged or increased (n = 9). The following parameters were compared between the two groups: age, sex, VUR grade and laterality, urodynamic parameters (bladder capacity, compliance, leak point pressure), type of bladder neuropathy, accompanying neuropathology (walking problem, anal incontinence). RESULTS: Most of the parameters studied failed to predict the outcome of conservative management of VUR in patients with neurogenic bladder dysfunction. Higher grades of VUR if bilateral seem to benefit more from conservative management than lower grades do. Conservative management appears to be more effective in hyperreflexic bladders than areflexic bladders in terms of VUR resolution. CONCLUSION: Although prediction of patients resistant to conservative management of VUR could save myelodysplastic children from prolonged risk of renal damage, current methods of evaluation are of very little help in this aspect.

Two siblings with fetal hydantoin syndrome.

An association between anticonvulsant drugs taken during pregnancy and congenital abnormalities was first identified by Meadow et al. in 1968. Manson and Frederic clarified teratogenic effects of hydantoin in their epidemiological studies in 1973. Varied malformations due to hydantoin intake during pregnancy include digit and nail hypoplasia, growth retardation, typical facial appearance, rib anomalies, abnormal palmar creases, hirsutism, and low hairlines. Ambiguous genitalia is rarely associated with this syndrome. We present two siblings, aged three years and three months, with fetal hydantoin syndrome (FHS). Both were born to an epileptic mother who was given diphenylhydantoin (DPH) and phenobarbital throughout her pregnancies. The patients showed many characteristics of FHS, and ambiguous genitalia. Clinical and laboratory examinations revealed that both have normal female internal genital organs and female karyotypes.

Blood transfusion requirements in children with blunt spleen and liver injuries.

The records of 174 children sustaining blunt spleen and liver injuries in a 16-year period were analyzed retrospectively to determine blood transfusion requirements in surgically versus conservatively managed patients. The whole study group consisted of 97 spleen, 70 liver, and 7 combined spleen and liver injuries respectively. Seventy-eight patients were managed conservatively while 96 children had undergone various operative procedures. The hematocrit (Hct) values, transfused blood volumes (ml/kg), and length of hospital stay were compared between the non-operative and operative treatment groups. Although the initial Hct values were found to be similar in non-operative versus operative groups (26.7% +/- 2.7% vs. 24.8% +/- 3.5%), transfused blood volumes were significantly higher in the surgically treated group respectively (20.9 ml/kg vs. 39.5 ml/kg) (p < 0.05). Similarly, mean length of hospital stay was longer in the surgical groups. Associated injuries were seen in 105 (60.3%) patients distributed randomly among two study groups. Twelve patients in the series died of other system/organ involvement. There were no deaths in isolated spleen and/or liver injuries. Increased blood transfusion requirement in surgical groups may be due to excessive bleeding prior to the operation which virtually provides the indication for laparotomy. Manipulation and suturing enhances additional bleeding to elevate the total volume to be delivered. Operative choice should be spared for very selected cases, and conservative management is safe; procedures such as splenorrhaphy are not superior to observation therapy as a means of controlling intraabdominal bleeding.

The place of Mitrofanoff neourethra in the repair of exstrophy-epispadias complex.

The authors present their experience with seven patients having an exstrophy-epispadias complex who had undergone Young-Dees-Leadbetter bladder neck reconstruction with an adjuvant Mitrofanoff neourethra. All the patients, but one, were male with a mean age of 7.4 +/- 3.9 years. Six of them had had previous primary closure of their exstrophied bladders accompanied by anterior diagonal iliac osteostomies, and one having pure epispadias had an incompetent bladder neck and a very low bladder capacity. Four of these patients had breakdown of their reconstructed bladder necks due to clean intermittent catheterization (CIC) via urethra. Ileocystoplasties were performed in all of the patients for their unacceptably low bladder capacities (mean 20.9 +/- 12.9 ml) with an adjuvant Mitrofanoff neourethra. The patients were put on CIC every 3-4 hours via Mitrofanoff channel. Six were totally continent, and one had to use a pad during daytime. There were no complications related to the Mitrofanoff stoma except two patients complained about temporary difficulty on catheterization. The authors conclude that incorporating a Mitrofanoff stoma to bladder neck reconstruction procedures in exstrophy-epispadias complex, prevents injury to the bladder neck during CIC, and thus lowers the risk and incidence of failure.

Characteristics of multisystem organ failure in neonates.

The records of 45 neonatal deaths in a four year period were reviewed retrospectively. Sixteen patients (35.5%) developed multisystem organ failure (MSOF). The criteria for pulmonary, hepatic, renal, hematologic, cardiac and microvascular failures were established. The onset of the first organ involvement was calculated in days prior to death. The earliest organ involved was kidney (14.2 +/- 15.1) followed by microvascular (9.4 +/- 7.6), hematologic (84. +/- 10.1), liver (6.8 +/- 6.7), lung (6.3 +/- 6.6) and cardiac (6.0 +/- 8.7) failure. Blood culture analyses revealed 5 patients with culture-positive sepsis. Yeast was the leading septic agent (n = 3) followed by Klebsiella pneumoniae (n = 2), Pseudomonas sp. (n = 1) and E. coli (n = 1). The first organ involvement was noted at 17.6 +/- 23.2 days. We concluded that the sequence of neonatal MSOF is different from that of adults, yet the inciting events are not clear-cut. Lung, which is the first organ involved in adults, seems to be a lately involved organ in neonates.

Effects of H2 receptor blocking agents on bacterial translocation in burn injury.

We experimentally studied the effects of H2 receptor blockers (ranitidine) on bacterial translocation (BT) in 42 male albino rats. Sham group (Group I, n = 12 rats) were exposed to 21 degrees C water while Burn group (Group II, n = 15 rats) and Ranitidine group (Group III, n = 15 rats) were exposed to 95 degrees C hot water for 10 seconds to produce a full thickness burn in 30% of total body surface area. 300 mg/kg ranitidine was administered to Group III starting immediately after the burn injury. Rats were sacrificed on the fifth postburn day. Sham group gained weight while groups II and III had significant weight loss. Gastric pH increased with the administration of ranitidine. Both gram negative and total number of bacteria were found to be reduced in cecal stool cultures in ranitidine group. Significant increase in BT was observed in Group III, and translocating bacteria were found to be different in burn and ranitidine groups with a final conclusion that administration of ranitidine changes intestinal ecological equilibrium and promotes BT.

The effect of suturing technique and material on complication rate following hypospadias repair.

Ninety-seven children with distal hypospadias were treated surgically using perimeatal-based flap urethroplasty (Mathieu procedure) in a two and a half years period. A review of the medical records revealed two distinct groups of patients according to the suturing type and suture material. In the first group of 36 patients (group I), neourethra was constructed using 6/0 polyglactine (Vicryl) in a single layer, full-thickness, uninterrupted fashion. Skin flaps were approximated using interrupted simple 5/0 polyglactine (Vicryl) sutures. In the second group of 61 patients (group II), 7/0 polydioxanone (PDS) was used in the urethral anastomosis performed in a subcuticular, uninterrupted fashion. The skin flaps were closed using interrupted simple 5/0 rapidly absorbable polyglactine (Rapid Vicryl) sutures. Patients were followed-up from 6 to 12 months. Urethral or meatal stenosis was not observed in any patient. There was no infectious complication. Urethrocutaneous fistula rate was significantly higher in group I (16.6%) compared to group II (4.9%) (p < 0.01). Complication rate following hypospadias repair can be reduced by the use of a subcutaneous suture technique utilizing polydioxanone suture material in urethroplasties.

Safer and much cheaper circumcision using fibrin glue in severe haemophilia.

Circumcision is a traditional ceremony for Muslims and Jewish people. In order to reduce the high cost of factor concentrates, we administered locally manufactured heat-treated fibrin glue during operation. Circumcision can be performed safely and is much cheaper using fibrin glue plus a small amount of factor concentrates.

Evaluation of the lower urinary tract function in caudal duplication (dipygus) anomaly.

Caudal duplication is a rare anomaly with less than 30 reported cases. For those patients who also have double bladders, there are not enough data regarding the function of the lower urinary tract. A boy with caudal duplication anomaly was evaluated fluoroscopically and urodynamically. The results of the evaluation showed that the bladders were filling and emptying synchronously with normal and almost identical detrusor pressures. The child did not require either or both bladders to be resected. Because the level of the duplication in dipygus cases varies, thorough evaluation of the lower urinary tract, including urodynamics, should be considered for every case.

Gastrointestinal perforations in children: a continuing challenge to nonoperative treatment of blunt abdominal trauma.

The present trend towards conservative management of hemodynamically stable pediatric trauma patients may be increasing the risk of delay in the diagnosis of traumatic hollow viscus perforations (HVP). The purpose of this study is to determine whether there is a delay in the diagnosis of HVP because of expectant management. A survey of factors leading to diagnostic delay was also made and the value of current diagnostic tools were reevaluated. In 1,283 trauma admissions between 1980-1994, 34 patients were operated for HVP caused by blunt abdominal trauma. Sites of perforation were; stomach (four), duodenum (five), jejunum (12), ileum (nine), and jejunum/ileum (four). Signs of peritoneal irritation were positive in 32 of 34 patients. There was free air in only six of 24 abdominal roentgenograms. Free peritoneal fluid without solid organ injury was detected in only four out of 13 patients with ultrasound. Peritoneal lavage was diagnostic in eight of nine patients. Time from admission to operating room averaged 24 +/- 4.1 (mean +/- standard deviation) hours. Eleven patients died after the operation mostly because of accompanying head injury. Only two of the deaths were the result of sepsis originating from the perforated bowel. There is an apparent delay in the diagnosis of traumatic HVP in this series. Signs of peritoneal irritation are the most consistent findings of HVP after blunt abdominal trauma in children. Persistence of abdominal signs indicates peritoneal lavage, which has a high diagnostic sensitivity for HVP compared to other diagnostic modalities.