Severe hypospadias repair with meatal based paracoronal skin flap: the modified Koyanagi repair.

PURPOSE: Various techniques have been reported to repair severe hypospadias. The Koyanagi repair uses a meatal based foreskin flap. We modified this procedure and reviewed the outcome of the repair of severe hypospadias. MATERIALS AND METHODS: The modified Koyanagi repair was performed in 151 children 7 months to 15 years old (mean age 3 years) with severe hypospadias. The records of these patients were reviewed. In all cases the meatus was at or proximal to the penoscrotal junction. Followup was 6 months to 16 years (mean 6 years). Flap design is the same as in the Koyanagi repair, although our modified technique requires removal of the subcutaneous tissue of the distal portion of the flap, which acts as a full-thickness free skin graft. RESULTS: A fistula developed in 19 patients (12.6%), meatal stenosis in 3 (2%) required meatoplasty and infection in 2 (1.3%) resulted in a regressed meatal position. Good cosmetic results were achieved in all except the latter 2 cases. CONCLUSIONS: Our modified Koyanagi repair affords an excellent cosmetic appearance. The complication rate is relatively low and compares favorably with that of other techniques.

[Long-term follow-up of children with surgically treated primary vesicouretral reflux].

We reviewed the symptoms of hypertension and proteinuria and evaluated the renal function in patients aged 15 and over with surgically treated primary vesicoureteral reflux. Twenty-two patients were enrolled in this study. There were no patients with hypertension except one patient with proteinuria. The extraction factor by Heyman's method in these patients tended to increase with age at operation and decrease with the grade of reflux at operation. There findings suggest that long-term follow-up is particularly essential to patients who had severe reflux and underwent anti-reflux surgery in the early stage of life.

[Clinical review of 39 cases with ectopic ureteroceles].

BACKGROUND: There are many controversies surrounding the management of ectopic ureteroceles (EUC). The aim of this study is to review our cases with EUCs and to show our policy of choice of treatments of EUCs. METHODS: The medical records of 39 patients with EUCs treated at Kobe. Children's Hospital from 1978 to 1998 were reviewed retrospectively. Patients' age, affected site, presentation, treatment, and postoperative course were recorded. RESULTS: The age at presentation ranged from 0 month to 13 years (mean; 6 years). The left EUCs were found in 15 patients, the right in 17 patients, and the bilateral in 7 patients. The EUCs with duplicated system of the kidney were involved in 35 cases (42 kidneys) and single system in 4 cases (4 kidneys). The most common mode of presentation was urinary tract infection (n = 24) followed by abdominal distention (n = 6) and fetal ultrasonography (n = 6). One patient presented with incontinence and in two patients EUCs were discovered incidentaly. Thirty-five cases (42 kidneys) were followed up over six months. In these cases diversion including nephrostomy and ureterostomy was performed in 5 kidneys, heminephrectomy and/or excision of the EUC and ureteral reimplantation in 8 kidneys, nephrectomy in 3 kidneys, pyeloureterostomy in 2 kidneys, excision of the EUC and ureteral reimplantation in 10 kidneys, and transurethral incision (TUI) of the EUC in 14 kidneys. After these treatments the second surgery was totally required in 15 kidneys(36%) including 7 kidneys in which TUI was performed. Furthermore, in two kidneys the third operation was performed. CONCLUSIONS: Reoperation was required in about one-third of patients with ectopic ureteroceles. It is easy to perform TUI, however the rate to reoperation is high.

[Gastrocytoplasty in pediatric patients--initial clinical experience with 5 cases].

Gastrocytoplasty was undertaken for 5 patients with bladder dysfunction and incontinence caused by neurogenic bladder in 3 cases, bladder exstrophy in 1 case and trauma in 1 case respectively. The age of the patients ranged from 5 years through 14 years at the operation. Decreased renal function was recognized in 3 cases with neurogenic bladder before reconstruction. The other 2 cases had normal renal function. Gastric segment was isolated with right gastroepiploic artery as a pedicle. Bladder was opened vertically and augmented with gastric segment. In 4 cases urethrogastrostomy with submucosal tunnel was performed in both sides. In neurogenic bladder cases, urethra was left without any surgical intervention, while other 2 cases underwent continent diversion using Mitrofanoff principle with urethra being closed. Postoperative follow up period was 18 to 22 months. All cases had increased bladder volume and the dilatation of upper urinary tract disappeared or decreased in size in those who had upper urinary tract dilatation before operation. Urinary incontinence completely disappeared in continent diversion cases. In neurogenic bladder cases urinary leakage through urethra was negligible with 4 hour interval clean intermittent catheterization. Laboratory examination showed no metabolic derangement in blood gas analysis and electrolytes even in those who had decreased renal function. Blood urea nitrogen (BUN) and serum creatinine showed a little improvement or the same level as before. We didn't encounter any troubles in CIC such as the obstruction caused by mucus produced by gastric segment. From our experience with those 5 pediatric cases underwent gastrocystoplasty, we thought gastric segment had some advantage as a tissue for augmentation cystoplasy compared with intestinal segment.

Spontaneous gonadotropin and testosterone concentration profiles in prepubertal and pubertal boys: temporal relationship between luteinizing hormone and testosterone.

To investigate the detailed pattern of change in circulating gonadotropin and testosterone concentrations around the onset of puberty and to determine the temporal relationship between the gonadotropin and testosterone secretion, plasma gonadotropin and testosterone were measured at 20-min intervals for 24 h in 21 normal short boys. The obtained plasma hormone concentrations were analyzed by Cluster pulse detection algorithm, cosinor analysis, and cross-correlation analysis. The 21 subjects were divided into the prepubertal (n = 16) and early pubertal (n = 5) groups. All subjects showed nocturnal LH and FSH pulses and had significant circadian LH and FSH rhythms. Except for six boys of prepubertal group, all subjects showed nocturnal testosterone pulses and had significant circadian testosterone rhythms. The acrophase (clocktime of maximal value) of circadian testosterone rhythm was 0308-0428 h. Cross-correlation analysis demonstrated significant positive cross-correlations between LH and testosterone that were maximum at a testosterone lag of 60-120 min. Further, to eliminate intrinsic autocorrelations within the LH and testosterone time series, we filtered the data before subjecting them to the cross-correlation analysis. As a result, significant positive cross-correlations were found at a testosterone lag of 40 min in 10 peripubertal boys. We conclude that testosterone concentration profiles are pulsatile and show marked circadian rhythm well before the onset of puberty. LH and testosterone time series are significantly coupled when testosterone lags LH by about 40 min. This time lag might correspond to the time for synthesizing and secreting testosterone in Leydig cells after binding of LH to the Leydig cell receptors.

Comprehensive planning of operative strategy for separation of ischiopagus tripus twins with particular reference to quality of life.

A 27-year-old mother was diagnosed by prenatal ultrasonography as having triplets at gestational age 32 weeks. Following cesarean section at 37 weeks, a pair of female babies were noted for the first time to be joined by a common pelvis with three lower limbs. They had separate upper gastrointestinal tracts, which joined in the distal ileum, leading to a common colon, rectum, and a single anus. Each twin had a functioning kidney, with a single ureter leading to a common bladder. A common urethra originating from the bladder neck ran into the urogenital sinus of one baby. Prior to the surgical separation, placement of four tissue expanders and 20 pneumoperitoneums were performed, in order to stretch the parietes for easier approximation of the wound edges. At 13 months of age, separation was performed, requiring 17 hours. The skin and musculature from the conjoined third leg was used as a fillet for abdominal wall closure in each patient. One infant was given the distal half of the colon and an entire anus with a temporary jejunostomy, and the right half of the bladder with the urethra. The other infant was given the proximal half of the colon with a permanent colostomy, and the left half of the bladder with permanent cystostomy using appendiceal pedicle graft (Mitrofanoff's procedure). This is the 10th case of surgical separation in ischiopagus tripus twins reported in the literature, and the seventh successful separation with both patients alive.

Comparison between spontaneous gonadotropin concentration profiles and gonadotropin response to low-dose gonadotropin-releasing hormone in prepubertal and early pubertal boys and patients with hypogonadotropic hypogonadism: assessment by using ultrasensitive, time-resolved immunofluorometric assay.

To assess whether nocturnal gonadotropin concentration profiles in children could be predicted by measurement of peak gonadotropin levels after gonadotropin-releasing hormone (GnRH) administration, we measured spontaneous gonadotropin levels every 20 min and the gonadotropin responses to low-dose GnRH using an ultrasensitive, time-resolved immunofluorometric assay in 61 boys with short stature and/or delayed puberty. Spontaneous nocturnal LH pulses were observed in 58 out of 61 patients. After GnRH administration in a dose of 25 ng/kg, all of the 61 patients had significant LH and FSH responses, and GnRH-stimulated peak LH and FSH levels were highly correlated with maximal spontaneous nocturnal LH and FSH levels, respectively (r = 0.83 for LH and r = 0.91 for FSH; p less than 0.00001). Analysis of individual subjects revealed that GnRH-stimulated peak LH levels were almost identical to maximal nocturnal LH levels in the subjects whose GnRH-stimulated peak LH levels were between 5 and 10 IU/L, whereas GnRH-stimulated peak LH levels tended to be higher than maximal nocturnal levels in the subjects whose GnRH-stimulated peak LH levels were 5 IU/L or lower. To determine if there were any parameters in the gonadotropin response to GnRH that might be useful in distinguishing early pubertal boys from prepubertal boys, we evaluated the gonadotropin response to GnRH in 44 prepubertal and 10 early pubertal normal short boys. Although maximal nocturnal LH levels did not overlap between prepubertal and pubertal groups, GnRH-stimulated LH peak levels overlapped considerably between the two groups. Even the GnRH-stimulated peak LH to peak FSH ratio overlapped between the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)

[Cystometric examination for neurogenic bladder of neonates and infants].

We examined 38 neonates and infants with spina bifida using cystometry and cystography. Among 18 cases with myelomeningocele, only 1 case was evaluated as normal, 15 cases were abnormal on cystometry and 2 cases were unable to be evaluated. In the 15 abnormal cases, 8 had low-compliance and high pressure pattern which suggested future deterioration of upper urinary tract. Clean, intermittent catheterization was started in 3 cases. In 20 cases with spina bifida occulta associated with lipoma, 14 were evaluated as normal but 5 were abnormal and 1 case was unable to be evaluated. One of the 4 cases with low-compliance and high pressure was treated with clean, intermittent catheterization. Cystometric examination is a simple and useful modality in neonates and infants with neurogenic bladder not only to evaluate the function but also to predict the future deterioration of the upper tract, and to select the proper treatment.

Reconstructive surgery for cloacal anomalies.

This paper describes a variety of surgical techniques which have been employed in three patients with cloacal anomalies, at the Kobe Children's Hospital during a period from 1977 to 1983. The patients presented with malformations in which the urethra, vagina and rectum converge at the high level of cloaca. Staged approach was mandatory in these patients. A loop colostomy with the distal loop of colon separated from the fecal stream and a decompressing vaginostomy are the initial procedures for this form of malformations. Vaginoplasty is required in some patients for pullthrough to the perineum. In those in whom the vagina and colon are separately pulled through, a posterior sagittal approach is convenient.

Clinical evaluation of pivmecillinam in intractable urinary-tract infections with complications. A comparative study with amoxicillin by a randomized double-blind technique.

A comparative study was made by the double-blind technique in order to make clear the usefulness of pivmecillinam in the treatment of intractable complicated urinary-tract infections using amoxicillin as a reference drug. Pivmecillinam was given in dosage of 400 mg (potency) per day which was one-fifth the dose of amoxicillin 2,000 mg (potency) per day. In global judgement, pivmecillinam was found superior to amoxicillin. It showed a "significant" superiority over amoxicillin for the treatment of, among others, the urinary-tract infections after prostatectomy, which are intractable diseases. When evaluated by symptoms, pivmecillinam improved bacteriuria "significantly" better than amoxicillin. When seen by causative organisms, the pivmecillinam treatment was "significantly" superior to the amoxicillin treatment against E. coli infections. Pivmecillinam was active against amoxicillin-resistant E. coli. Incidence of adverse reactions was less frequent with pivmecillinam than with amoxicillin. These results indicate that pivmecillinam is a drug of high usefulness for the treatment of intractable complicated urinary-tract infections when evaluated using amoxicillin as a reference drug.

[Comparative results of cephacetrile with cefazolin in complicated urinary tract infections by means of double-blind trials (author's transl)].

In order to elucidate the usefulness of cephacetrile (CEC), comparative trials with cefazolin (CEZ) were carried out in the patients with complicated infections of the urinary tract, and the following results were obtained: 1. There were no statistically significant differences or tendencies observed between the groups given CEC and CEZ in either of the global effects, bacteriological effects, rate of superinfection, relapse, relapse with bacterial alternation, drug usefulness, rate of improvements in symptoms and findings in all the cases. 2. According to a result of stratified analyses, there were statistically significant differences observed, showing more inferior results of CEC than CEZ in either stratum of "below 50" in the age, "acute" in the disease phase, "infections of the upper urinary tract" in the disease pattern, and "E. coli including mixed infections with gram-positive organisms" in the pathogenic strains. However, it should be taken into consideration that certain background factors influence greatly in the effects and there were some problems on deviated backgrounds in these strata. On the other hand, CEC displayed better bacteriological effects than those of CEZ in higher stratum of "chronic" in the disease phase and "Klebsiella, Proteus, Citrobacter, Enterobacter including mixed infections" in the pathogenic strains, and statistically significant differences were not observed. 3. In consequence of bacteriological studies, CEC showed stronger resistance than CEZ against beta-lactamase produced in all the strains of gram-negative bacilli. Particularly there were significant differences or tendencies observed in those of Proteus, Cirtobacter, and Enterobacter, and at the same time there were great differences in the activity of beta-lactamase between CEC and CEZ as the substrate. 4. In correlation of the MIC and effects, CEC showed weaker actions than CEZ against susceptible organisms, but slightly stronger actions against moderately or highly resistant strains. A study of the relationship between the MIC of pathogenic strains, beta-lactamase, and bacteriological effects, showed possibilities that even the high activity of beta-lactamase results in remarkable effects in susceptible strains and the drugs with stronger resistance against beta-lactamase given better results in moderately or highly resistant organisms. 5. The incidence rate of adverse reactions was 2 out of 51 cases in the CEC group (3.9%) and 3 out of 50 cases in the CEZ group (6.0%), showing no significant difference, and those symptoms were similar. In conclusion, when the usefulness of CEC in complicated infections of the urinary tract is compared with that of CEZ, the former is said to be equal to the latter without significant difference as a whole...