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Biomedical subjects

M L Ritchey

Publications and source records attributed to M L Ritchey.

At least 19 recordsLinked to original sources

Nephroblastoma.

Effective diagnosis, staging, and multimodality therapies dramatically decreased the morbidity and mortality of children with Wilms Tumor. Increasing awareness of the disease, biology, genetics, and epidemiology improved assessment of clinical syndromes and led to more risk-based treatment. The role of tumor imaging is being defined, and primary exploration with nephrectomy remains of value to confirm histological diagnosis and disease stage. Primary chemotherapy with delayed resection is evolving into the preferred approach for large inoperable tumors, bilateral disease, and those tumors with extensive intravascular involvement. Current therapeutic trials will be nonrandomized and emphasize biological tumor features for future patient stratification. A selected group of young patients with small localized favorable histology tumors will be treated with surgery alone.

Child

Renal failure in Wilms' tumor patients: a report from the National Wilms' Tumor Study Group.

This report defines the incidence and determines the etiology of renal failure (RF) in patients undergoing treatment for Wilms' tumor (WT). The database of the National Wilms' Tumor Study (NWTS) was searched to identify all children reported to have developed chronic renal failure. There were 55 patients found to have RF. Of these, 39 patients had bilateral tumors, 15 with unilateral disease and one with a WT in a solitary kidney. The median interval from diagnosis to the onset of renal failure was 21 months. The incidence of RF in bilateral WT was 16.4% for NWTS-1 & -2, 9.9% for NWTS-3, and 3.8% for NWTS-4. The incidence of RF in unilateral WT remained stable. The most common etiologies of RF were: bilateral nephrectomy for persistent or recurrent tumor (24 pts), Drash syndrome (12 pts), progressive tumor in the remaining kidney (5 pts), radiation nephritis (6 pts), and other causes (5 pts). The etiology of renal failure was not reported in three children. Children with unilateral WT and a normal contralateral kidney have a very low incidence of RF, and this review does not support a recommendation for parenchymal sparing procedures in these patients. Children with bilateral WT are at risk for the development of RF, and parenchymal sparing procedures are warranted.

Child

Renal salvage procedures in patients with synchronous bilateral Wilms' tumors: a report from the National Wilms' Tumor Study Group.

Synchronous bilateral Wilms' tumor accounts for 4% to 6% of all Wilms' tumors. Renal salvage procedures (partial nephrectomy and enucleation) have been recommended to conserve renal parenchyma. The objective of this study was to review the results of renal salvage operations performed in children who had bilateral neoplasms. The authors reviewed the records of 98 children enrolled in the Fourth National Wilms' Tumor Study who had synchronous bilateral tumors and underwent renal salvage procedures. One hundred thirty-four kidneys were managed with renal salvage procedures. Complete excision of gross disease was accomplished in 118 (88%) of the 134 kidneys. Local tumor recurrence in the remnant kidney or tumor bed occurred in 11 cases (8.2%). Overall, 72% of the kidneys were preserved, and the 4-year survival rate was 81.7%. The surgical morbidity after a salvage procedure was comparable to that of a complete nephrectomy in patients with unilateral Wilms' tumor. Although the incidence of positive surgical margins is worrisome, it did not invariably lead to local recurrence in the remnant kidney or the tumor bed.

Biopsy

Cost-effective endoscopic examination of the contralateral inguinal ring.

OBJECTIVES: Laparoscopic examination of the contralateral inguinal ring has recently been advocated to exclude contralateral hernia in young children. We report a modification using nondisposable cystoscopic equipment, which is quick and reliable. METHODS: Either an 8 or 10 F soft straight catheter or a cystoscope sheath is passed through the open hernia sac. Air is insufflated into the abdomen with a syringe. Once the pneumoperitoneum is completed, a 4-mm cystoscope lens (110 degrees) is used to inspect the contralateral ring. RESULTS: Twenty-four children between the ages of 6 weeks and 4 years (median, 6 months) underwent exploration for presumed unilateral inguinal hernia and laparoscopic examination of the contralateral inguinal ring. Thirteen patients (54%) had an open processus vaginalis and underwent contralateral inguinal herniorrhaphy. No false-positive or false-negative results were found, and there were no complications associated with the procedure. CONCLUSIONS: This method of laparoscopic examination of the contralateral inguinal ring using nondisposable cystoscopic equipment is rapid, safe, reliable, and cost effective in evaluating a contralateral patent processus vaginalis. Laparoscopic examination spares the need for formal surgical exploration in patients with a closed processus vaginalis.

Child, Preschool

Sibling reflux: a dual center retrospective study.

PURPOSE: Siblings of index patients with vesicoureteral reflux are known to have an increased incidence of reflux. Previous reports may be biased due to significantly more female siblings undergoing screening. In addition, it has been suggested that screening is unnecessary in older children. MATERIALS AND METHODS: We retrospectively reviewed the records of probands with vesicoureteral reflux. A total of 422 families comprising 622 siblings was identified at our centers from 1985 to 1994. Of the 255 boys and 277 girls newborn to 16 years old (mean age 6.2 years) 85% were evaluated. Almost 43% of the probands (225) were older than 7 years. RESULTS: Vesicoureteral reflux was noted in 144 siblings (52 boys and 92 girls, 27%). The majority had low grade reflux but in 111 (77%) maximum reflux grade was II or III. Reflux nephropathy was demonstrated on a nuclear renal scan in 18 of the 132 tested siblings (13.6%). In all of these children reflux was grades II to IV and 27% were older than 10 years. CONCLUSIONS: When vesicoureteral reflux is discovered, all siblings should be considered for screening. The evaluation of older siblings remains controversial, since they comprise only a small fraction of all siblings with reflux but may have a significant portion of reflux nephropathy. There was a 27% overall incidence with a 33% rate in girls. The incidence of reflux in siblings decreased after age 7 years but reflux nephropathy was identified even in the older children.

Adolescent

Decreased bladder compliance in patients with myelomeningocele treated with radiological observation.

PURPOSE: Recently others advocated frequent radiological surveillance to detect upper urinary tract deterioration in children with neurogenic bladder secondary to spina bifida. We reviewed the consequences of such expectant management on bladder compliance and urinary continence. MATERIALS AND METHODS: We retrospectively reviewed the records of 214 children presenting to our spina bifida clinic in a 13-year period. Follow-up is available for 95 girls and 86 boys. Imaging studies of the kidneys were repeated at 6 to 12-month intervals. Urodynamics were performed when upper urinary tracts deteriorated or in incontinent school age children. RESULTS: On radiographic study there was evidence of upper urinary tract deterioration in 79 children, including hydronephrosis in 34, hydronephrosis and vesicoureteral reflux in 19, and reflux only in 26. Follow up studies performed after clean intermittent catheterization and pharmacological therapy were instituted revealed resolution or improvement of upper tract deterioration in 52 patients (69%), while bladder compliance improved in only 42%. Surgical intervention was required in 34 children, despite improvement of upper tract changes in many of these patients on follow up radiographic studies. CONCLUSIONS: Although radiological surveillance of patients with myelomeningocele allows recognition of upper tract changes, the effects of elevated outlet resistance on bladder compliance are not as readily reversible as the initial radiographic findings. The incidence of enterocystoplasty exceeds that reported for patients treated prospectively based on urodynamic findings, which should be considered in the treatment of these children.

Female

Accuracy of current imaging modalities in the diagnosis of synchronous bilateral Wilms' tumor. A report from the National Wilms Tumor Study Group.

BACKGROUND: Five percent of patients with Wilms' tumor will present with bilateral disease. Recent reports suggest that preoperative imaging studies can exclude contralateral disease reliably, obviating the need for formal surgical exploration of the contralateral kidney. This study was undertaken to determine the accuracy of preoperative imaging in diagnosing bilateral Wilms' tumor. METHODS: The charts of 122 patients with synchronous bilateral Wilms' tumor enrolled in National Wilms Tumor Study-4 were reviewed. With the exception of one child, all had an abdominal computed tomography (CT), ultrasound, or magnetic resonance imaging performed. RESULTS: There were nine patients (7%) in whom the diagnosis of bilaterality was missed by the preoperative imaging studies. All but one of the missed lesions were small, five less than 1 cm and three 1-3 cm. The accuracy of each imaging modality was correlated with tumor size. Computed tomography was more sensitive in detecting bilaterality than ultrasound. However, there was not a single study that was able to detect more than 50% of lesions less than 1 cm in greatest dimension. CONCLUSIONS: This review indicates that even with current advances in imaging technology, synchronous bilateral Wilms' tumor will go unrecognized in 7% of patients if formal exploration of the contralateral kidney is omitted. Although this represents a small percentage of all patients presenting with Wilms' tumor, preoperative diagnosis of bilaterality is essential if parenchymal-sparing procedures are to be performed. Until a more reliable indicator of bilateral disease is found, exploration of the contralateral kidney continues to be recommended.

Child

Neurourologic consequences of accidental intrathecal vincristine: a case report.

Inadvertent intrathecal administration of vincristine results in severe neurotoxicity. Motor and sensory bladder dysfunction have been reported but the long-term urologic consequences are unknown due to the associated high mortality. We report a case of accidental intrathecal vincristine administration with patient survival and follow-up of 24 months. The child developed a lower motor neuron neuropathic bladder requiring intermittent self-catheterization of the bladder. Urinary tract management of these children should be based on the urodynamic findings.

Child

Neonatal Wilms tumor.

Neonatal renal tumors are very rare. We have identified 15 cases of Wilm's tumors diagnosed in the first 30 days of life. The most common presentation was an abdominal mass on routine newborn examination, and three tumors were noted on an antenatal ultrasound. All of the infants underwent primary excision of the tumor. Five infants were not given postoperative chemotherapy after surgical excision. One of these children had a relapse and later died of progressive disease. The remaining children are alive at a median follow-up of 31 months. Eight of the tumors had evidence of nephrogenic rests. More frequent follow-up physical and imaging examinations are indicated in children not given adjuvant chemotherapy or whose tumors show nephrogenic rests.

Combined Modality Therapy

Modified dartos pouch orchiopexy.

Experimental evidence suggests that transparenchymal suture fixation may risk testicular damage. Even very small sutures can result in inflammation and tubular damage. An alternative to transparenchymal suture fixation of the testis is presented.

Child

Outcome analysis of bilateral Cohen cross-trigonal ureteroneocystostomy.

OBJECTIVES: The perioperative and long-term outcomes of children with vesicoureteral reflux (VUR) treated by cross-trigonal ureteroneocystostomy were ascertained. METHODS: One hundred ten consecutive children with VUR who underwent bilateral cross-trigonal ureteroneocystostomy were studied retrospectively. Nineteen children with neurovesical dysfunction or megaureters requiring tapered reimplants were excluded. Outcome parameters of the remaining 91 children consisted of operative time, length of hospitalization, days of Foley catheter drainage perioperative complications, correction of reflux, subsequent morbidity, and parental satisfaction. RESULTS: Of 182 renal units, 11 (6%) had grade 0 VUR, 18 (10%) had grade I, 43 (24%) had grade II, 59 (32%) had grade III, 36 (20%) had grade IV, and 15 (8%) had grade V reflux. The mean operative and hospitalization times were 180 minutes and 5.6 days, respectively. No postoperative complications occurred. Three children were lost to follow-up, and the remaining 88 children had an extended mean follow-up in excess of 3 years. Voiding cystourethrogram documented a 98.3% (173 of 176 renal units) success rate. Sixteen children (18%) experienced nonfebrile clinically symptomatic cystitis episodes and 3 children (3%) experienced one febrile episode each. Telephone parental survey of overall surgical experience revealed a 94% very satisfied, 2% satisfied, and 3% dissatisified rate. CONCLUSIONS: Cross-trigonal ureteroneocystostomy is a safe and effective technique that is virtually complication free and has high parental satisfaction. The results of this study provide a baseline for comparison of non-operative treatment of reflux as well as laparoscopic and endoscopic techniques.

Adolescent

Perinephric urinoma secondary to neurogenic bladder in myelodysplasia.

We report on a girl with infected perinephric urinoma and myelodysplasia who presented with a febrile urinary tract infection. A complex perinephric fluid collection with internal echoes was found on renal ultrasound, necessitating placement of a percutaneous drainage tube. Initial urodynamic testing after treatment of the infection revealed a noncompliant bladder with an elevated detrusor leak point pressure. Neurovesical dysfunction with elevated leak point pressure is believed to be the cause of urinoma. Clinical presentation, treatment and pathophysiology of this entity are discussed.

Cysts

Percutaneous endopyelotomy in infants and young children after failed open pyeloplasty.

PURPOSE: We assessed the efficacy and safety of percutaneous endopyelotomy in infants and young children with secondary ureteropelvic junction obstruction after previous open pyeloplasty. MATERIALS AND METHODS: Three boys and 2 girls with persistent ureteropelvic junction obstruction after open pyeloplasty underwent percutaneous antegrade cold knife endopyelotomy via an 18F nephrostomy tract. RESULTS: Percutaneous endopyelotomy was successfully performed in all 5 children with minimal complications. At a mean followup of 2.5 years endopyelotomy was successful in 4 of the 5 children based on the absence of symptoms, normal pressure-perfusion studies and normal or improved diuretic renal scintigraphy studies. One child in whom endopyelotomy failed underwent successful ureterocalicostomy. CONCLUSIONS: Percutaneous antegrade endopyelotomy is a safe and efficacious method of treating secondary ureteropelvic junction obstruction in children. This method offers a minimally invasive alternative to conventional repeat open pyeloplasty.

Child, Preschool