R. Beetz

Renal abscess in childhood : diagnostic and therapeutic progress

During the past decade new techniques such as computed tomography (CT) and ultrasonography have been reported to have changed the diagnostic investigation and treatment of renal abscess in adults. To evaluate whether similar changes have taken place in the pediatric age group, a retrospective study of all patients seen between 1979 and 1989 was performed. Seven patients, 0.8 to 14 (mean, 9) years old, with renal abscesses in eight kidneys were identified. Ultrasound and computed tomography proved to be the most valuable diagnostic tools, revealing the diagnosis by showing a hypoechoic or hypodense mass. All patients had an initial trial of intensive antibiotic treatment, which led to resolution of the abscesses in two of the eight kidneys. In all other cases the abscesses were additionally drained, which was done surgically in two and by ultrasonography- or CT-guided percutaneous drainage in four patients. Abscess cultures grew Staphylococcus aureus (three), Escherichia coli (one) and Salmonella Group B (one) and were sterile in one case. Drainage was unsuccessful in only one patient, who subsequently underwent nephrectomy for uncontrolled infection of a diffusely damaged kidney. We conclude that the diagnosis of renal abscesses is greatly facilitated by ultrasonography and CT and that most patients can be cured without operation by antibiotics and, if necessary, by additional percutaneous drainage.

Social integration, sexual behaviour and fertility in patients with bladder exstrophy- a long-term follow up

After primary bladder closure or urinary diversion, other factors apart from the reconstruction itself gain importance for individuals with the exstrophy-epispadias complex: social integration and, after reaching puberty, sexuality and fertility. Between 1968 and July 1994 115 patients with bladder exstrophy or incontinent epispadias underwent surgery at our institution. A total of 104 patients could be followed, 2 of whom died in the meantime. Of the remaining 102 patients 48 attend school, 4 are in college, 40 have completed or are currently undergoing vocational training, 3 are unemployed, 1 lives in a therapeutic centre and 6 are younger than 6 years of age. A total of 95% of the patients with continent urinary diversion are continent day and night, whereas only three of five patients with a sling plasty (incontinent epispadias) or with primary bladder closure followed by a Young-Dees procedure are continent. None of the patients showed deterioration of renal function. In 25 females the external genitalia were reconstructed. Fixation of the uterus was done in 13 to correct or prevent uterine prolapse. Of the 17 women older than 18 years of age with genital reconstruction, 16 are satisfied with the cosmetic result. All adults engage in sexual intercourse. Five women have delivered seven children by Caesarean section. Of the 35 male adults 32 underwent reconstruction of the external genitalia and 34 males achieve erection. One developed necrosis of the penis early in life following primary bladder closure performed at an outside hospital. Penile deviation was present in 11 of the 32 patients with genital reconstruction, which is distressing in only 2. Thirty patients are satisfied with the cosmetic result. After genital reconstruction 9 males developed epididymitis, necessitating two orchiectomies and three vasectomies. No patient with reconstruction of the external genitalia can ejaculate normally or has fathered children, whereas ejaculation was normal in the three men who did not undergo genital reconstruction and in two patients prior to post-pubertal reconstruction. Furthermore, two of these three men have fathered four children.

Rectosigmoid Pouch (Mainz Pouch II) in Children

PURPOSEnContinent anal urinary diversion is a therapeutic option in bladder exstrophy. We report our long-term results with the rectosigmoid pouch (Mainz pouch II), a modification of the classic ureterosigmoidostomy.nnnMATERIALS AND METHODSnA total of 38 children with a mean age of 5 years (range 0.5 to 17) underwent a Mainz pouch II procedure between 1991 and 2004. Most patients (33) had bladder exstrophy or incontinent epispadias. In 14 children (37%) urinary diversion was performed after failed primary reconstruction. In 6 children conversion was performed from an incontinent type of urinary diversion. Renal function, continence and metabolic changes were analyzed. A total of 35 children were followed for a mean of 112 months (range 5 to 147).nnnRESULTSnAll children were continent during the daytime but 3 (8.6%) suffered from nighttime incontinence requiring pads. With respect to the upper urinary tract, 6 children (15.8%) had development of pyelonephritis, mostly with stenosis of the ureterointestinal anastomosis. Reimplantation of the ureter was required in 10 of 69 RU (14.5%), of which 7 (10.1%) were due to ureterointestinal stenosis and 3 (4.3%) were due to reflux. Serum creatinine was within normal limits in all children. During followup acid-base balance was monitored, and early alkali supplementation was initiated in 24 of 35 children (69%) when the base excess was less than -2.5 mmol/l. One child had development of clinical acidosis requiring hospitalization. After followup of more than 10 years annual rectosigmoidoscopy was performed in 16 children/young adults without pathological findings.nnnCONCLUSIONSnThe Mainz pouch II procedure for children with genitourinary anomalies promises excellent continence rates. However, periodic followup studies are important to check the upper urinary tract and prevent metabolic acidosis. Due to the risk of malignancy at the ureterointestinal anastomosis, endoscopy should be performed annually beginning at postoperative year 10. The Mainz pouch II procedure is safe in the long term. Without stoma, appliance or catheterization this type of continent urinary diversion is specifically suitable for children.

Urinary diversion in children and adolescents with neurogenic bladder: the Mainz experience

After failure of conservative treatment of neurogenic bladders (deterioration of the upper urinary tract/incontinence) continent cutaneous diversion has to be considered in those patients with irreparable urethral sphincter defects or those who are unable to perform trans-urethral self-catheterization. In this second part of the study we investigated the long-term safety of using the Mainz pouchxa0I with regard to protecting the upper urinary tracts and to provide urine continence. Between 1985 and 2002, operations to form an ileocaecal pouch with umbilical stoma (Mainz pouchxa0I) were performed on 70 children and adolescents of median age 15.3 years (range 5.7–20 years). During the follow-up period five patients died 2.4–14 years postoperatively of causes not related to urinary diversion. A follow-up period of 8.7 years (0.9–18) was achieved in 65 patients with 118 renal units (RUs). As compared to preoperatively, the upper urinary tracts had remained stable or improved in 113/118 RUs (95.8%) at the latest follow-up. Complete continence was achieved in 97% of patients with a continent cutaneous diversion. Surgical revisions were required for: incontinence of the outlet mechanism in 9%, stoma prolapse in 2%, stoma stenosis in 23%, pouch calculi in 15%, symptomatic reflux in 1%, ureter stenosis in 16% of the RUs with submucosal tunnel and in 3% of the RUs with an extramural tunnel. We conclude that, in patients with irreparable sphincter defect and those who are unable to perform urethral self-catheterization, continent cutaneous urinary diversion with the Mainz pouchxa0I provides a high continence rate with preservation of the upper urinary tracts in the long run. In patients with dilated ureters, the extramural tunnel technique results in a lower complication rate.

Long-Term Followup Of 158 Young Adults Surgically Treated For Vesicoureteral Reflux In Childhood: The Ongoing Risk Of Urinary Tract Infections

PURPOSEnWe recorded urinary tract infections in the long term after surgical reflux correction.nnnMATERIALS AND METHODSnA total of 158 of 189 patients (160 females and 29 males) who were followed in 1985, an average of 10.8 years after reflux surgery were contacted again in 1995. At that time median patient age was 26 years (range 15.7 to 38.8) and the average period of observation was 20.3 years (range 13.4 to 26).nnnRESULTSnIn 82% of the patients febrile and in 18% afebrile symptomatic urinary tract infections had developed preoperatively. In the first 10-year period after operation 46% of patients continued to have symptomatic urinary tract infections compared with 52% in the second 10-year interval. In the 2 periods the incidence of febrile urinary tract infection was about 17%. In the whole postoperative observation period symptomatic urinary tract infections developed in 66% of all patients, including 74% of female patients. Symptomatic urinary tract infections were observed during 8 of 46 pregnancies (17%).nnnCONCLUSIONSnAfter successful surgical reflux correction susceptibility to urinary tract infection continues for a number of years in many girls and women. However, postoperatively urinary tract infections are primarily afebrile.

Vitamin metabolism in patients with a Mainz pouch I : Long-term followup

PURPOSEnWe assessed whether creation of the Mainz pouch I, which requires 24 to 36 cm. of terminal ileum and 12 cm. of ascending colon including the ileocecal valve, leads to metabolic disturbances.nnnMATERIALS AND METHODSnIn 137 patients the levels of vitamins A, B1, B2, B6, B12, D and E, folic and bile acids, ammonia, and intracorpuscular vitamin B12 and folic acid were evaluated and a red blood count was performed. Patients were divided into children and adults, and into 3 groups according to number of years after surgery (2 or less, more than 2 to 4 and more than 4).nnnRESULTSnIn all patients the levels of vitamins A, B1, B2, B6, D and E, folic and bile acids, and ammonia as well as red blood count were within normal ranges. In the 51 children there was no significant decrease in vitamin B12 postoperatively. In the 86 adults mean serum vitamin B12 plus or minus standard deviation decreased significantly from 402 +/- 182 ng./l. during the first 2 years postoperatively to 292 +/- 204 ng./l. after year 4 (normal 240 to 1,100). There was no significant decrease in intracorpuscular vitamin B12 during this period.nnnCONCLUSIONSnIn addition to regular examinations, the determination of vitamin B12 levels 4 years after Mainz pouch diversion is mandatory. It remains unclear whether substitution is necessary. However, substitution is easy to achieve and even less expensive than the regular determination of vitamin B12.

Renal tubular dysgenesis (RTD) - an important cause of the oligohydramnion-sequence: Report of 3 cases and review of the literature

Renal tubular dysgenesis (RTD) is a disorder characterized by neonatal renal failure and regular gross renal architecture, although the histological features of immature and shortened proximal tubules lead to neonatal death. The pathogenesis of this condition includes a congenital familial condition, a twin-twin transfusion syndrome, and an angiotensin-converting enzyme inhibitor intake by the mother. The clinical picture shows an association with oligohydramnia, pulmonary hypoplasia, and skull ossification defects. In the present paper, we report the occurrence of RTD in three infants of a consanguinous couple and compared our data with those of the literature. Our data confirm that late second trimester demonstration of oligohydramnion, with structurally normal kidneys and with or without skull ossification defects, allows the diagnosis of renal tubular dysgenesis, which, however, has to be confirmed by histological and immunohistological examinations of the kidney.

Medullary sponge kidneys and unilateral Wilms tumour in a child with Beckwith-Wiedemann syndrome.

The occurrence of a Wilms tumour in a 4-year-old girl with bilateral medullary sponge kidney, Beckwith-Wiedemann syndrome and congenital hemihypertrophy demonstrates the close relationship between these disorders. Another six cases from the literature with congenital hemihypertrophy and with medullary sponge kidney are discussed, two of them also developed intra-abdominal neoplasm.

Technetium-99m mercaptoacetyltriglycine clearance : reference values for infants and children

Six hundred and thirty-nine clearance studies performed in children aged 7 days to 19 years utilizing technetium-99m mercaptoacetyltriglycine (MAG 3) were retrospectively analysed. Standardized conditions for the investigation included: parenteral hydration (60 ml/hxm2 body surface) in addition to normal oral fluid intake, weight-related dose of99mTc-MAG 3 (1 MBq/kg body weight, minimum 15 MBq) and calculation of clearance according to Bubeck et al. Of the 513 children, 169 included in this analysis could be classified as “normal” with regard to their renal function. Normal kidney function was judged by the following criteria: normal GFR for age, normal tubular function (absence of proteinuria and glucosuria), normal renal parenchyma (on ultrasonography, MAG 3 scan and intravenous pyelography), absence of significant obstruction and gross reflux (>grade 1), no single kidney and no difference in split renal function >20%. Results showed increasing MAG 3 clearance values for infants during the first months of life, reaching the normal range for older children and adults between 7 and 12 months.

Diagnostik und Therapie von Harnwegsinfektionen

ZusammenfassungHarnwegsinfektionen (HWI) im Säuglings- und Kleinkindesalter verlaufen oft mit unspezifischen Symptomen. Für die exakte mikrobiologische Diagnose ist die Art der Uringewinnung entscheidend. Sie sollte im Säuglingsalter durch eine suprapubische Blasenpunktion oder über einen transurethralen Einmalkatheter erfolgen. Die kalkulierte Therapie bei v.xa0a. Pyelonephritis besteht bei jungen Säuglingen aus der Kombination eines Cephalosporins der Gruppexa03a oder eines Aminoglycosids mit Ampicillin. Ab dem Kleinkindesalter kann eine perorale Behandlung mit einem Cephalosporin der Gruppexa03 bei unkomplizierter Pyelonephritis durchgeführt werden. Zum Ausschluss von Nieren- oder Harnwegsfehlbildungen wird bei erster Pyelonephritis eine sonographische Untersuchung empfohlen. Die Strategien zur Refluxdiagnostik und zur Erkennung von Parenchymdefekten werden kontrovers diskutiert. Die aktuellen AAP-Guidelines für fieberhafte HWI im Säuglings- und Kleinkindalter werden voraussichtlich die noch ausstehenden evidenzbasierten Leitlinien hierzulande beeinflussen.AbstractIn infants and young children, urinary tract infections (UTI) often present with unspecific symptoms. Appropriate techniques of urine sampling play an important role for accurate microbiological diagnosis. In infants urine sampling by bladder puncture or transurethral catheter is recommended. In young infants with suspected pyelonephritis, calculated antibiotic treatment should be initiated parenterally with a combination of a third generation cephalosporin or an aminoglycoside with ampicillin. After the age of 3-6 months group 3 oral cephalosporins can be used in uncomplicated pyelonephritis. With the first febrile UTI early sonography is recommended to provide information about renal parenchymal involvement and to exclude malformations of the kidneys and urinary tract. Strategies for the recognition of vesicoureteral reflux and renal damage are under discussion. Recently published guidelines by the American Academy of Pediatrics for the diagnosis and management of UTI in febrile children and infants aged 2-24 months will most likely influence the still pending German guidelines.In infants and young children, urinary tract infections (UTI) often present with unspecific symptoms. Appropriate techniques of urine sampling play an important role for accurate microbiological diagnosis. In infants urine sampling by bladder puncture or transurethral catheter is recommended. In young infants with suspected pyelonephritis, calculated antibiotic treatment should be initiated parenterally with a combination of a third generation cephalosporin or an aminoglycoside with ampicillin. After the age of 3-6 months group 3 oral cephalosporins can be used in uncomplicated pyelonephritis. With the first febrile UTI early sonography is recommended to provide information about renal parenchymal involvement and to exclude malformations of the kidneys and urinary tract. Strategies for the recognition of vesicoureteral reflux and renal damage are under discussion. Recently published guidelines by the American Academy of Pediatrics for the diagnosis and management of UTI in febrile children and infants aged 2-24 months will most likely influence the still pending German guidelines.