[Diagnosis and therapy of pyonephrosis in infancy and childhood]. / Diagnostik und Therapie der Pyonephrose im Säuglings- und Kindesalter.

A diagnostic schedule in pyonephrosis was followed in thirteen infants and children. Obstruction of the urinary tract could be demonstrated in 12 patients, non-obstructive pyonephrosis was present in one case. Congenital obstructive malformation of the urinary tract was found in 7 patients, obstruction was acquired in 5. Sonographically, pyonephrosis could be diagnosed in 8 patients (64.3%). Excretory urography (10/13) was helpful in no actual case. Sonographical-guided percutaneous nephrostomies were performed in 5 patients and operative nephrostomies in 4. Emergency-nephrectomies were done in 4 patients. A tap of the renal pelvis is the best way for early diagnosis whenever pyonephrosis is clinically suspected.

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.

[Teratomas in the cervical region]. / Teratome der Halsregion.

Teratomas in the cervical region have rarely been observed in infants. There are only a small number of reports in the literature of teratomas in this region. The complication rate after sugical removal of such a tumour is relatively high. A case report is presented of a newborn with a cervical teratoma which was removed without serious sequelae and with acceptable cosmetic and functional results.

Urological operations for solitary kidneys in children.

Depending on the underlying disease, children with solitary kidneys who have to undergo urological operations have the same good or poor prognosis as children with two kidneys (Whiting et al. 1983; Redman and Birsada 1976; Stackl et al. 1983). However, the problem of long-term prognosis persists. It is similar to that for children who have undergone surgery for reflux: a certain number of them will develop renal insufficiency in 20-30 years. Nephrologists estimate that 20% of adults who suffer from renal insufficiency and become dialysis dependent formerly had reflux nephropathy. The influence of reimplantation, performed with good results during the past few years, cannot be assessed so far. Large prospective studies are needed.

[Accomplishments of tumor surgery in pediatric nephroblastoma]. / Leistungen der Tumorchirurgie beim kindlichen Nephroblastom.

The cure rate of Wilm's tumor in childhood could be increased to more than 80% by systemic adjuvant chemotherapy in combination with operation and radiation. The importance of histological grading is discussed. Especially preoperative chemotherapy has made progress possible in reducing risks of operative techniques and has improved the prognosis for survival.

[Cystomanometric investigations in children with enuresis and chronic urinary tract infection (author's transl)]. / Cystometriche Untersuchungen der Enuresis und des chronischen Harnwegsinfektes im Kindesalter.

Ninety one patients with urinary tract infection, infection with enuresis or enuresis alone, but without any malformation of the lower urinary tract have been examined clinically as well as by cystomanometric und uroflow-metric studies. It could be shown that the most important parameters to evaluate blader function were the bladder compliance and the detrusor contraction during the filling of the bladder. The bladder compliance was estimated from the volume pressure relationship under resting conditions, at the first urgency to voide and at the moment when the maximal bladder capacity was reached, Concerning the detrusor contraction we distinguished partial isovolumetric detrusor contractions with an amplitude of 1--8 mm Hg and uninhibited detrusor contractions with an amplitude of more than 10 mm Hg. According to these two parameters it was possible to differentiate cystomanometrically seven different types of irritable and non irritable bladder, and to introduce a new theory of the pathogenesis of enuresis. According to this theory we suppose that enuresis in childhood is mostly caused by neurovegetative psychogenic disorders similar to anorectal sphincter achalasia in patients with overflow encopresis.

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.

[The use of split and regional renal clearance for indication for surgery in pediatric urology (author's transl)]. / Stellenwert der seitengetrennten und regionalen Nieren-clearance für die Operationsindikation in der Kinderurologie.

The differential and ipsilateral clearance of Hippuran allows for a quantitative assessment of the function of both kidneys, a single kidney and a part of one kidney. We have performed this investigation 179 times in 156 children in relation to dysplastic kidneys, obstructive lesions in the upper urinary tract, duplex kidneys and in follow-up situations. In many of these patients this investigation produced an indication for an organ-preserving operative procedure. The radiological findings are compared with the clinical picture.

[Urinary incontinence in epispadias and bladder exstrophy with special reference to free autologous muscle transplantation]. / Urininkontinenz bei Epispadie und Blasenexstrophie unter besonderer Berücksichtigung der freien autologen Muskeltransplantation.

This is a report on 16 patients with epispadias of grades II to IV and on 35 patients with exstrophy of the bladder, grades III and IV. The following surgical treatment was carried out: in children with epispadias, simple closure of the urethra in one case, neck of the bladder plasty according to Young-Dees in two cases, the method after Thiersch-Duplay in three cases, whereas four patients were treated after Williams, five after Johnston and one patient after Ombrédanne in reverse. Seven additional operations were done mainly for prepuce correction and closure of fistula after urethroplasty. Three of the 16 patients were continent already before the operation. 4 children were partially continent. Two of these showed improved continence, thus achieving prolonged periods of dryness. 9 children remained incontinent. Hence, free muscle transplantation to the bladder was performed in four patients. A total of 45 operations were performed on children with exstrophy of the bladder. Of these, 12 concerned primary closure of bladder; in 13 cases, an ileum bladder was constructed, whereas cystosigmoideostomy was done in 5 times, uretersigmoideostomy 5 times, cutaneous ureterostomy once, rectum bladder once, and a colon conduit once. The postoperative continence performance of the 12 patients with primary bladder closure was unsatisfactory. Three children only attained periods of dryness up to two hours; 5 remained completely incontinent, whereas in 4 it was subsequently necessary to construct a urinary passage into the intestine by means of an ileum bladder in 2 cases, a colon conduit in one case, and ureterosigmoideostomy in one case. Free muscle transplantation to the bladder was performed in two patients. Free muscle transplants were also performed on 2 children, in one case after traumatic tearoff of the urethra and in one case after iatrogenic lesion of the musculus sphincter vesicae externus in adrenogenital syndrome (AGS) according to Prader III and IV. This means that a total of eight free muscle transplants were performed. All children were primarily incontinent. Postoperatively, complete continence could be achieved in four cases, once an improvement with dry periods up to 2 hours. The two patients with bladder exstrophy remain incontinent, in one patient with epispadias the postoperative interval has been too short so far to arrive at a final judgement. It follows from the examinations that free muscle transplantation is a good method for treating urinary incontinence, the suspension effect playing an important part. However, in patients with bladder exstrophy the indication should be considered with caution.

[Surgical separation of symmetrical conjoined ischiopagus twins]. / Zur Trennung eines symmetrischen Ischiopagenpaares.

This is a case report on symmetrical ischiopagus tetrapus. We made exact preoperative examinations of the topographic anatomical relationships before performing the separation at the age of ten months. Both twins survived the operation without complication. A second procedure to correct the misformed pelvices of both children was successfully performed. The twins have been released from the hospital. One child, who can walk, lives with his parents. The second child has a perinatal brain defect, and as such is cared for in a nursing home.

[Juvenile rhabdomyosarcoma. Diagnosis and new therapeutic possibilities]. / Rhabdomyosarkom im Kindesalter Diagnostik und neue Therapiemöglichkeiten.

Seventeen children with rhabdomyosarcoma stage I to III diagnosed since 1973 are presented. The coordinated management utilizing surgical excision, irradiation (5000 rad) and systemic adjuvant multiagent chemotherapy is described. The favorable sites were head and neck sites and genitourinary region. The mean survival is at present 19 months. 14 children are alive and well. 13 children had no tumor recidivation, 1 child had 8 months after beginning of the therapy lung metastases which after irradiation with 2000 rad disappeared. 3 children (stage III) died 9, 14 and 20 months after diagnosis during therapy by metastases. Acute and late effects on normal tissues from radiation and chemotherapy were noted in 12 cases. A review of the literature and therapeutic alternatives in the future are indicated.

[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.

[Radiation therapy of the renal tumor in children (author's transl)]. / Strahlentherapie des Nierentumors beim Kind.

Close cooperation of pediatric surgeons and pediatric oncologists with radiotherapists, using coordinated treatment schedules conformable to the stage of the disease, and combining tumor nephrectomy, postoperative irradiation and the treatment with AMD and Vincristine, has increased the 3-year survival rate from 27% (13 of 49) up to 67% (14 of 21) for children with Wilm's tumor.

Pyonephrosis in childhood--is ultrasound sufficient for diagnosis?

The validity of ultrasound in the diagnosis of pyonephrosis in infants and children was retrospectively investigated in 14 patients. The disease was unilateral in 13 patients and bilateral in one. The diagnosis was proven by percutaneous nephrostomy in 7 and by operation in 7 patients. Ultrasound was true positive in 9 patients (10 kidneys) and false negative in 5. Large staghorn calculi were present in 2 of the 5 false negative cases. A group of 20 patients with simple hydronephrosis, investigated by percutaneous punctures, served as a control group. There were two false positive cases in this group. The sensitivity of sonography for the diagnosis of pyonephrosis was only 66.7%, which is considerably lower than in previous reports. We therefore recommend early sonographically guided percutaneous puncture of the renal pelvis whenever pyonephrosis is suspected.

[Improved prognosis in Wilms' tumor due to adjuvant combination drug therapy]. / Verbesserte Prognose beim Wilms-Tumor durch adjuvante Kombinationschemotherapie.

78 children with Wilms' tumor stage I--IV diagnosed since 1960 are presented. There were two groups of patients: one group consisting of 35 patients which received adjuvant chemotherapy for 1 year, the other group consisting of 43 patients which received no chemotherapy. Surgical excision and irradiation was identical in both groups. The prognosis was greatly improved by adjuvant chemotherapy: 4-year survival rates increased from 25 to 69%. Late side effects from radiation and chemotherapy were noted: 15/78 patients suffered from scoliosis and all patients treated by chemotherapy had a decreased lymphotoxin activity over years. The following factors appear to be related to prognosis: the extent of disease in patients determined by better techniques, histopathology of the tumor, and the nature of treatment.