[Pediatric urologic guidelines]. / Kinderurologische Leitlinien.

Because of the enormous progress of science and technology and increasing possibilities in diagnostics and therapeutics guidelines are also necessary in pediatric urology. Guidelines must have the non-committal character of recommendations in order not to reduce the freedom of medical action in diagnosis and therapy to the detriment of patient interests. The special premises and conditions of guidelines must be defined. Due to the large variety of diseases and disturbances in pediatric urology, it is important to limit the number of guidelines.

Urethral valves during the first year of life--a retrospective, multicenter study.

The diagnoses, treatment, and clinical courses of 178 children with urethral valves were analyzed in a retrospective, multicenter study. In group I the diagnosis had been made during the first 4 weeks of life, in group II between the 2nd and 12th months of life, after which treatment was begun. Statistical analysis of the patient data and results revealed clearly poorer results for patients in group I, who had the most severe congenital abnormalities: 10% of the infants in group I died within the 1st year of life as compared to 1% in group II. Among the survivors, 25% of children in group I had renal insufficiency as opposed to 8.2% in group II. The following parameters proved to be statistically significant risk factors with regard to prognosis: 1. Birth weight 2. Age at diagnosis 3. Renal function, particularly when initial creatinine levels exceed 200 mumol/l and fail to decrease below 100 mumol/l after successful therapy 4. Bilateral grade IV or V vesicoureteral reflux 5. Late incontinence (after conclusion of the 5th year of life). Statistical significance could be demonstrated for parameters 1-4, but due to the small number of patients not for parameter 5. The study showed that numerous neoimplantations associated with high complication rates were carried out in both groups, however, no statistically significant effect on long-term prognosis could be established. Our results did not confirm those of the Toronto group, who proposed that broadening the indications for supravesical urinary diversion would provide demonstrable parenchymal protection. No statistically significant relationship between therapeutic procedure and long-term clinical course could be shown.(ABSTRACT TRUNCATED AT 250 WORDS)

Urinary diversion in children.

At the present time, urinary diversion in children remains an last resort type of treatment that must be carried out in selected cases when there is no alternative available. All forms of urinary diversion have the common goal of gaining control of a life-threatening situation; all, however, are fraught with serious disadvantages for the affected children. The newer forms (undiversion, pouch, ileocystoplasty, augmentation) undoubtedly improve the patient's quality of life, but the risks of long-term metabolic and renal complications and malignant degeneration persist. Harzmann, who for years has concerned himself with urinary diversion and, in particular, with malignant changes, has posed two difficult questions: 1. Is urinary diversion using bowel segments defensible for benign disorders, e.g., in children? 2. Which follow-up examinations should be performed after urinary diversion using bowel segments, and at what time intervals? Our goal should be to keep the number of urinary diversions as small as possible by means of appropriate treatment, prophylaxis, and in particular the means and type of primary reconstructive therapy. Children have a life-time ahead of them, and the complications of urinary diversion increase decade by decade. This fact has been demonstrated in children who have undergone ureterosigmoidostomy or ileal conduit. Grounds for the assumption that fewer serious long-term complications are to be expected after colon conduit, pouch, neobladder, or augmentation procedures have not been established.

[Prenatal diagnosis and postpartal therapy of a rare sequela of gastroschisis: short bowel syndrome]. / Pränatale Diagnostik und postpartale Therapie einer seltenen Folge der Gastroschisis: das Kurzdarmsyndrom.

Report about a rare complication of gastroschisis by early volvalus. Following this occasionally the prenatal diagnosis there was a very ultrasonogram of a mostly solid tumour before the abdominal wall. The postpartal operative therapy with resection and later doubling according to Bianchi B described.

[Diagnosis, therapy and follow-up of infants with urethral valves, treatment concept in the 1st year of life]. / Diagnostik, Therapie und Verläufe von Säuglingen mit Harnröhrenklappen, Behandlungskonzept im 1. Lebensjahr.

Within the framework of a joint follow-up we report on the treatment of 57 infants with urethral valves (35 babies from Munich and 22 from Siegen) in the first year of life, studied between 1974 and 1986. In 16 newborn the diagnosis and initiation of treatment was effected during the first four weeks of life (Group I) and in 41 babies during the 2nd to 12th month (Group II). In three cases, diagnosis of suspected urethral valve was made prenatally. Primary management and the concept of further treatment are described. Primary therapy is determined by the general condition after birth, presence of urosepsis and the extent of any existing renal insufficiency. 2 children died, both from Risk Group I. 5 children are in a state of compensated renal insufficiency. In 2 newborn we were compelled to perform supravesical urinary drain and in 3 children a suprapubic drain. Secondary nephrectomy became necessary in 4 children, 3 of whom belonged to Risk Group I. In 23 of 57 children valve resection followed by transurethral drainage was the only therapy. In 22 of 57 children corrective surgery was performed subsequently (neo-implantation, with and without modellage, stenosis of the ureter exit). The article reports on the course in each case.

[Bladder exstrophy and quality of life]. / Blasenexstrophie und Lebensqualität.

The primary objectives of operative management of exstrophy of the urinary bladder are to obtain secure closure of pelvic girdle and abdominal wall, unimpeded voiding, urinary continence, absence of reflux, preservation of renal function, functional and cosmetically acceptable external genitalia, and the absence of malignancy. These objectives cannot be fully achieved by only one of the established procedures such as functional bladder closure, internal or external urinary diversion and epispadias repair. Early individual and creative surgical management including changing concepts when needed and careful life-long followup are thought to provide the basis for a satisfying social adjustment and quality of life.

Simple help for spina bifida children with anal incontinence.

The search for an effective means of helping children with anal incontinence has been facilitated by tissue-compatible material which has already been in use for a long time in proctology. Polyvinyl alcohol foam is characterized by its elasticity, malleability under the influence of liquids and its good tissue compatibility. We have had anal plugs manufactured in the shape of hourglasses. These are available in various sizes and can be adapted individually. We consider their application to be indicated in about one third of myelomeningocele children who either have partial or complete fecal incontinence. We have also tested the plugs in healthy children and experience is available in the meantime in eight children after operation on a high anal atresia and 15 children with spina bifida. The period of application is in childhood. Our experience so far is encouraging. The handling is described in detail.

[Cecil-Duplay/Cecil-Leveuf urethral reconstruction in severe hypospadias]. / Harnröhrenaufbau bei schwergradigen Hypospadien nach der Methode von Cecil-Duplay/Cecil-Leveuf.

A total of 199 boys with severe hypospadias were subjected to surgery during 1981 to 1984 at the Department of Surgery of the Red Cross Paediatric Hospital in Siegen and from 1971 to 1984 at the Department of Surgery of the Municipal Paediatric Hospital in Cologne. Plastic reconstruction of the urethra was performed according to a multiple-stage technique after Cecil-Duplay/Cecil-Leveuf. It is evident that good results can be obtained in such severe forms of hypospadias by means of differentiated multistep surgery. A fistula rate of 14% is an essential improvement over the results previously obtained by the urethral reconstruction method after Denis-Browne.

[Adrenal cortex carcinoma in childhood and adolescence]. / Das Nebennierenrinden-Karzinom im Kindes- und Jugendalter.

The presented paper includes the medical records of two girls with adrenal cortical carcinomas on the one hand and the detailed analysis of a collected series of 150 equal cases in pediatric literature on the other hand. In our patients there were a primarily metastatic, non-functioning tumour and a locally invasive, hormone-secreting neoplasm respectively. The latter one produced a Cushing's syndrome with signs of virilization. In this case surgical removal was followed by postoperative irradiation and adjunctive cytostatic therapy with cyclophosphamide and adriamycin for one year. But tumour recurrence occurred within 6 months. A treatment with the antitumour agents aminogluthetimide and o,p' DDD was transiently effective. However drug-induced side effects necessitated the stop of this regimen after 5 months. The analysis of 150 case reports revealed a peak between 1 and 3 years of age, a striking prevalence of the female sex in all age groups, and a metastasizing rate of about 30% at the time of diagnosis. Regional structures, liver and lung were the main locations of metastatic lesions. The occurrence of second primary neoplasms and the association with a group of other conditions particularly congenital disorders have proved to be a remarkable feature of the disease. In primary adrenal cortical tumours the determination of the neoplasm's biological behaviour by morphologic criteria alone can often be very problematical. This fact is especially stressed.

Anomalies of the external urethral orifice in girls.

In analyzing 1,000 of 1,475 micturating cystourethrograms in girls, the absolute number and the percentages of the different variations of the urethra were related to the frequency of urethrovaginal reflux and to anomalies of the meatus. This allowed the following deductions: There are no radiological findings in these girls that indicate the presence of subvesical obstruction. Anomalies of the meatus cannot be related to variations of the urethra as seen in the micturating cystourethrogram. They can, however, be the cause of enuresis, chronic urinary infection, urethrovaginal reflux, and disturbances in micturition. The marked susceptibility for chronic urinary infection seen in girls between the ages of 3 to 13 years is as yet not fully explained. The fact that the urethra opens near the anus and the vagina, inadequate hygiene, a too short urethra, and possibly missing hormonal protection against ascending infection, as well as the special urological anomalies described above, may all be responsible. Therefore, in girls there are a number of other disturbances micturition apart from of organic subvesical obstruction. These other disturbances may be termed functional, or the urethra syndrome.

Normal urinary flow for girls aged 3-14 years.

103 healthy girls aged 3-14 in whom urological illness or chronic urinary tract infection could be ruled out were examined uroflowmetrically with a "Mictiograph" operating on the rotation-dynamic principle. The 103 girls were subdivided into 2 groups: 3-7 years (N:53) and 8-14 years (N:50). For both age groups we could establish uroflowmetric results (volume, micturition time, average flow, maximum flow, time required for attaining maximum flow) and subject them to statistical scrutiny. For all 5 uroflow parameters we figured the mean, variance, and standard deviation for both age groups. In a variance analysis we were able to document significant group differences for volume, average flow and maximum flow. Basing on these results it was possible to represent the correlation of average and maximum flow with volume for both age groups using lines of regression. Moreover, in the group aged 8-14 there existed a linear relation of volume, average flow and maximum flow to age. Naturally, some girls will continue to require a combined urodynamic examination. But by means of the statistically validated uroflow norms presented here, better differentiation from pathological results can be attained, and uroflow measurements in girls can now be regarded as reliable indicators.

Renal growth and operative correction of severe vesicoureteral reflux in children.

We report about 32 operated children with 40 complicated refluxes. 10 of the operated children suffered from double kidneys with double ureters. We examined these children before and 3 years after the operation. The data were registrated according to the recommendations of Jacobsson, Claesson, Olsson and Ringertz. We operated on 22 children because of 30 complicated refluxes, 20 out of the 30 measured kidneys showed normal data before the operation, 8 of these 20 kidneys developed normally after the operation, 12 times this was not the case. However, all of these unsatisfactory or worsened data were within the standard deviation. In this group pathological values were not found. Among 10 out of 30 renal units which were out of the standard deviation, when measured before the operation, 3 kidneys improved and showed data within standard deviation after the operation. 7 kidneys continued to have pathological data, all out of the standard deviation. 2 of these 7 afflicted kidneys showed even worse values after the operation in comparison to the preoperative pathological values.

The upper urinary tract in neurogenic bladders without diversion---a ten-year follow-up.

Within the first 5 years of life, 50% of children with neurogenic bladders developed subvesical obstruction. Children with neurogenic bladders who did not developed subvesical obstruction (according to group I) up to the age of 5 years are not likely to develop obstruction unit they reach the age of 10 years. Children who do develop such an obstruction before the age of 5 years are possible candidates for urinary diversion.

Long-term results of obstructive neurogenic bladder in children with myelomeningocele.

A total of 91 children with myelomeningocele and neurogenic bladder were followed up for periods of between five and 10 years. The results of investigations of the upper and lower urinary tracts at the ages of three months, five years and 10 years showed that subvesical obstruction was rare in the infant period but that it increased year by year as the children grew older. By the age of five years 50 per cent of the children in this sample had severe obstructive changes. The study shows that children with neurogenic bladder who reach five or six years of age without developing severe symptoms of progressive subvesical obstruction will stand a good chance of being able to be treated conservatively.