H. Norman Noe

The long-term results of prospective sibling reflux screening.

A prospective study was begun more than 10 years ago to identify the incidence of vesicoureteral reflux in the siblings of patients with reflux. A total of 354 siblings of 275 index patients was screened with a voiding cystourethrogram for the presence of reflux. Of the siblings tested 119 (34%) were found to have reflux, including 75% who were asymptomatic. Reflux was present in a significant percentage of younger siblings. No correlation with index patient reflux grade, sex or established renal damage could be related to the likelihood of sibling reflux. A slightly higher rate of reflux was found in the female siblings of female index patients, which is a variation from the initial study. The incidence of renal damage was significantly reduced in the siblings with reflux compared to the index patients, which was also true in the youngest patients, who are believed to be the most susceptible to reflux-mediated renal damage. Sibling reflux screening can be justified due to a high percentage of siblings found to have reflux without symptoms and a significant decrease in renal damage compared to the index patients. Aggressive screening in young children (less than 5 years old) is still advisable, although this recommendation has been modified for older children. Additional information will be needed before the genetic transmission of reflux can be clarified.

The Transmission of Vesicoureteral Reflux from Parent to Child

Vesicoureteral reflux is now recognized to be hereditary and familial. The incidence of reflux in siblings has proved to be significant but less is known about the incidence of reflux in the offspring of known reflux patients. In an ongoing prospective series of reflux screening we identified 23 patients of childbearing age with a known history of reflux and screened their 36 offspring with an awake voiding cystourethrogram. Of these 36 offspring 24 (66%) exhibited vesicoureteral reflux. The literature was also reviewed to determine the incidence of parent/child reflux from reported cases. This review revealed a 65% rate of reflux in the offspring of known patients. Our preliminary results coupled with those in the literature signify a need to screen the offspring of known reflux patients and suggest a rethinking of the genetic transmission for this trait. While vesicoureteral reflux could still be a multifactorial genetic trait with a major gene, consideration must also be given to an autosomal dominant inheritance pattern.

Familial Vesicoureteral Reflux: A Prospective Study

A prospective study was established to identify the incidence of vesicoureteral reflux in the siblings of patients with reflux. Of 78 patients with reflux 104 siblings were screened with an awake voiding cystogram and 34 (32 per cent) were found to have reflux. Of these 34 siblings 25 (73 per cent) had no abnormal voiding symptoms or history of urinary tract infection. The highest incidence of reflux was found in the siblings of those patients with radiographic evidence of renal scarring. Early detection and followup in this high risk group may be a vital tool in determining more about the natural history of vesicoureteral reflux and its relationship to renal damage.

Hematuria preceding renal calculus formation in children with hypercalciuria

Five children were evaluated for painless hematuria; currently recommended diagnostic studies were unsuccessful in determining a diagnosis. A renal calculus subsequently was passed or detected radiographically in each child from 14 to 20 months after the initial evaluation. Hypercalciuria was then demonstrated by quantitation of 24-hour urinary calcium excretion or by measurement of urinary calcium/creatinine ratio. Calculi and hematuria have not recurred following thiazide therapy. We infer that idiopathic hypercalciuria was a probable cause of the unexplained painless hematuria.

Clinical experience with pediatric urolithiasis

Our clinical experience with 47 pediatric patients with stones is reviewed. Surgical therapy was standard with successful stone manipulation in 12 of 13 patients. In 91 per cent of our patients factors causing or predisposing to stone disease were discovered. A thorough metabolic evaluation, including an oral calcium loading test in 20 children, proved to be helpful. A new patient subgroup relating unexplained hematuria to eventual stone formation is described. Our protocol for metabolic evaluation and recommendations for treatment based on the results of such an evaluation are given. We have found the metabolic evaluation of the child with stones meaningful and particularly helpful in planning subsequent therapy for these patients.

The role of dysfunctional voiding in failure or complication of ureteral reimplantation for primary reflux.

Detrusor and/or urethral sphincter dysfunction is associated with increased failure or complication of ureteral reimplantation. A group of 400 children who underwent ureteral reimplantation was reviewed and the failure rate was determined. Those patients in the primary reflux category who failed were reviewed in detail for voiding dysfunction. Excluding technical error or ureteral dysfunction, the greatest risk for failure or complication of ureteral reimplantation seems to be related to detrusor and/or urethral sphincter dysfunction. Voluntary detrusor-sphincter discoordination seems particularly prevalent in this high risk group. The findings are discussed in relation to the over-all management of these patients.

Management of Neonatal Testicular Torsion

PURPOSE We evaluated the safety and efficacy of emergency exploration of neonatal torsion. MATERIALS AND METHODS We retrospectively reviewed the charts of 27 neonates (30 affected testicles) in a 13-year period. RESULTS Of the 10 testicles that were explored and fixed within 6 hours of discovery 2 (20%) were normal at physical examination at 1 year of followup. Of the remaining 20 testicles 19 were removed. One patient was lost to followup after orchiopexy. No operative or perioperative complications were associated with emergency exploration. CONCLUSIONS Emergency exploration of neonatal torsion is safe and prudent, and may result in higher testicular salvage rates. Given reports of contralateral torsion, we also recommend contralateral orchiopexy.

SAFETY OF KETOROLAC IN THE PEDIATRIC POPULATION AFTER URETERONEOCYSTOSTOMY

PURPOSE Ketorolac has been used to provide effective postoperative analgesia in children and decreases hospitalization for pediatric patients undergoing ureteroneocystostomy. However, it can cause severe side effects, including increased bleeding and renal insufficiency, which can be devastating in a child. Little has been reported on the safety of ketorolac by evaluating creatinine, hematocrit and complications. MATERIALS AND METHODS An institutional retrospective review was performed during an 18-month period in which 118 patients underwent ureteroneocystostomy. One group containing 50 patients received caudal anesthetic preoperatively and narcotic analgesics postoperatively, while another 68 received caudal anesthetic preoperatively and ketorolac postoperatively. Patient ages, type of procedure, preoperative and postoperative creatinine and hematocrit, and complications were noted in each cohort. RESULTS Average patient age of the control analgesic and ketorolac groups was 5.3 years (range 1 to 17) and 5.5 (1 to 12), respectively. There was no statistical difference between postoperative creatinine (0.68 and 0.65 mg./dl.) and hematocrit (33% and 34%) between the groups. One patient in each group had increased creatinine postoperatively. Minor complications, for example ileus and bladder spasms, were equivalent in both groups. No patient receiving ketorolac had any allergic or hypersensitivity reaction to the medication, and no major complications were reported. CONCLUSIONS Ketorolac given after ureteroneocystostomy did not cause a significant decrease in hematocrit, increase in creatinine or overall complications. Because of the safety of ketorolac in our series, and ability to decrease hospital stay and narcotic requirements in children as reported previously, it is used as standard postoperative protocol after ureteroneocystostomy at our institution.

Unilateral presentation of adult-type polycystic kidney disease in children.

Adult polycystic kidney disease is characterized by autosomal dominant inheritance, diffuse bilateral cystic kidney disease and onset during the young adult years. Relatively few cases of adult-type polycystic kidney disease in children have been reported. We describe a 4-year-old girl who appeared to have multiple, unilateral simple renal cysts by excretory urography and ultrasonography. There was no known familial renal cystic disease. When renal histology was consistent with adult-type polycystic kidney disease, screening nephrosonograms revealed that the mother also had the disease. We review 10 published cases of unilateral presentation of adult-type polycystic kidney disease in children. This report emphasizes the necessity of screening studies in parents of children with diffuse unilateral or bilateral cystic renal disease. Awareness of the unexpected unilateral presentation of adult-type polycystic kidney disease in children may allow a nonoperative diagnosis to be made.

Neonatal Hypertension from a Unilateral Multicystic, Dysplastic Kidney

Multicystic renal dysplasia is an extremely uncommon cause of hypertension in children and the few reported cases have not been of newborns. We report on a neonate in whom severe hypertension associated with elevated peripheral plasma renin resulted from a unilateral multicystic dysplastic kidney. Hypertension and plasma renin activity normalized after unilateral nephrectomy. No evidence of perfusion or excretory renal function in the dysplastic kidney was present on a radionuclide renal scan. This child demonstrates a relationship between hypertension and unilateral multicystic renal dysplasia.