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Biomedical subjects

D A Bloom

Publications and source records attributed to D A Bloom.

At least 19 recordsLinked to original sources

Musculoskeletal aspects of prune-belly syndrome. Description and pathogenesis.

OBJECTIVE: To determine the types and prevalence of musculoskeletal involvement in children with prune-belly syndrome, and to analyze the pathogenesis of the syndrome in relationship to the musculoskeletal deformities. DESIGN: A retrospective review of charts and roentgenograms along with a comprehensive review of 188 cases from the literature. SETTING: Tertiary care children's hospital. PARTICIPANTS: Twelve boys treated between 1975 and 1990. MEASUREMENTS/MAIN RESULTS: The prevalence of musculoskeletal involvement in patients was 45%. The involvement can be congenital (eg, clubfeet, limb deficiencies, teratologic hip dysplasia, and vertebral malformations) or developmental (eg, renal osteodystrophy, scoliosis, and pectus excavatum and/or pectus carinatum). The embryologic characteristics of congenital musculoskeletal problems correlate better with the embryologic theory of the prune-belly syndrome (an aberration of mesenchymal development around 6 weeks of gestation) than with the distal urinary tract obstructive theory. CONCLUSION: Since children with prune-belly syndrome are now living into adulthood, these musculoskeletal aspects will become important regarding potential morbidity.

Adolescent

Single system ectopic ureters and ureteroceles associated with dysplastic kidney.

Eight children forming an uncommon subgroup of renal obstructive dysplasia are presented. Each child had a nonfunctioning dysplastic kidney with a single collecting system with ectopic ureteral insertion and/or ureterocele. Five of the children had classic multicystic dysplastic kidneys, one had the hydronephrotic type of multicystic dysplastic kidney and two had hypoplastic kidneys. Other significant medical problems in 5 of the 8 children (63%) included VACTERL association, congenital heart disease and other genitourinary malformations. Unlike some children with unilateral multicystic dysplastic kidney, this subgroup of children has an increased risk of infection. They must be correctly identified on imaging so that tailored clinical management decisions can be made and associated anomalies detected.

Abnormalities, Multiple

The treatment of urinary incontinence in children using glutaraldehyde cross-linked collagen.

Periurethral injection of polytetrafluoroethylene (PolyTef) paste has been used to correct urinary incontinence for 2 decades. The controversy over distant migration of the paste has led to an interest in alternative materials particularly for the pediatric patient. Glutaraldehyde cross-linked collagen, derived from bovine dermal collagen, is effective in treating urinary incontinence in adults. Eight children with structural urinary incontinence underwent glutaraldehyde cross-linked collagen injection. Fluorourodynamic studies preceded and followed the injections. An average of 10.9 cc glutaraldehyde cross-linked collagen was injected per treatment (range 2.5 to 20). The number of injections per child ranged from 1 to 6 (mean 2.1) and followup averaged 13.75 months (range 6 to 33). Overall, 88% of the patients were cured or improved: 5 (63%) became continent, 2 were improved and 1 had no effect.

Adolescent

Retroperitoneal neurogenous choristoma.

A 6-year-old girl with urinary complaints underwent ultrasonography, which revealed a retroperitoneal cystic mass adjacent to the left kidney. Computerized tomography did not demonstrate whether the mass was an adrenal cyst or a duplicated upper pole segment. Exploration revealed a neurogenous choristoma, which is a collection of histologically normal tissue that is ectopically situated. We believe that this is the first report of this rare entity occurring in the retroperitoneum.

Child

Low-dose DDAVP in nocturnal enuresis.

A five-year experience with the vasopressin analogue desmopressin acetate (DDAVP) for nocturnal enuresis is described in 59 children. The initial starting dose of 5 micrograms at bedtime is lower than that reported in other series. Eighty-one percent of patients required 10 micrograms or less to achieve improvement or resolution of bedwetting.

Adolescent

Needle-tip localization during CT-guided abdominal biopsy: comparison of conventional and spiral CT.

OBJECTIVE: This study was performed to determine whether the time required for needle-tip localization during biopsy of the abdomen would be reduced if continuous-volume data acquisition, also known as spiral CT, were used for guidance instead of conventional CT. SUBJECTS AND METHODS: Forty patients had biopsies of an abdominal mass; half underwent needle-tip localizations with conventional CT and half with spiral CT. The times required to localize the needle for 104 needle passes were calculated; scanning and reconstruction times were included, and the radiologist's technique and procedural difficulties were deliberately excluded. The mean needle localization times with conventional and spiral CT were compared for the upper abdominal and pelvic regions by using the two-tailed unpaired Student's t-test. RESULTS: The mean time (+/- SE) for spiral CT was 35 +/- 2 sec compared with 105 +/- 18 sec for conventional CT (p < .001). When analyzed by region, times with spiral CT were shorter in both the upper abdomen (means, 37 sec for spiral CT vs 150 sec for conventional CT, p < .001) and pelvis (means, 25 sec for spiral CT vs 74 sec for conventional CT, p = .038); the magnitude of the improvement was greater in the upper abdomen. CONCLUSION: The time required to find the needle tip during guided biopsy of an abdominal mass is reduced with spiral CT compared with conventional CT. This improvement is partly a result of the ability to eliminate respiratory misregistration with spiral CT, which is not possible with conventional multisectional CT; hence the greater advantage in upper abdominal biopsy.

Abdominal Neoplasms

Sonography of the bladder after ureteral reimplantation.

Pre- and postoperative sonograms were reviewed in 59 children who underwent ureteral reimplantation to assess the frequency, location, extent, and evolution over time of focal thickening of the posterior bladder wall and bladder base after reimplantation. The interval between reimplantation and follow-up sonography ranged from 2 weeks to 12.36 years (mean, 1.29 years). Thirty-two patients (54.2%) had focal thickening of the posterior bladder wall and trigone after reimplantation. The earlier after reimplantation the children were first evaluated with sonography, the much more frequently was thickening observed. Thirteen had fusiform thickening along the submucosal tunnel and twenty-four had a hyperechoic nodule at the trigone. The thickening resolved in one third of the children and is presumed to have represented postoperative edema in these cases. However, on the sonographic appearance alone it was not possible to differentiate transient changes resulting from postoperative edema from those representing developing fibrosis or granuloma formation.

Adolescent

Renal obstructive dysplasia: ultrasound diagnosis and therapeutic implications.

57 cases of renal obstructive dysplasia (defined as the abnormal development of nephronic and ductal structures due to in utero obstruction of the urinary tract) were evaluated in terms of sonographic findings, renal and other associated anomalies, and current status of the child. More than one-third of the cases had bilateral disease and although not uniformly fatal bilateral involvement was associated with significant morbidity and mortality. In 12 of the 33 cases with unilateral dysplasia there was an association with contralateral renal problems including uretero-pelvic junction obstruction, vesicoureteral reflux and aplasia. Almost one-half of the cases had congenital anomalies, these included VACTERL association, congenital heart disease, cranial abnormalities and gastrointestinal malformations. Fifteen stillborns and 12 of the patients with bilateral involvement and four with unilateral involvement have died. Four patients are on dialysis (two with bilateral involvement and two with unilateral renal obstructive dysplasia). Only one-quarter are otherwise normal. More serious problems are reported in this mixed age population of patients with obstructive renal dysplasia than has been identified in previous studies. Management decisions of the fetus and child must be based on this new age-expanded population.

Abnormalities, Multiple

Two-step orchiopexy with pelviscopic clip ligation of the spermatic vessels.

Ten boys with nonpalpable testes located in the peritoneal cavity underwent celiopelviscopy and clip ligation of the spermatic vessels with the intent of augmenting vasal collateral blood flow. After intervals in excess of 6 months, 7 patients underwent vas-based orchiopexy following division of the occluded spermatic vessels. Six boys had good results as judged by size of the relocated testes. In 1 boy the testis atrophied. He had undergone previous inguinal-only exploration before referral to us for celiopelviscopy and at stage 2 orchiopexy a silk suture was found on the vas deferens.

Child, Preschool

Correction of vesicoureteral reflux by intravesical injection of collagen in a canine model.

A reflux model was created in 12 dogs to test the application of subureteric injection of collagen as a biodegradable substitute for ureteroneocytostomy. The injection technique was initially successful in 64% of dogs. Failure was related to deficiency in intramural tunnel and was unrelated to bladder function. Cystograms in 4 successfully corrected survivors showed resumption of reflux, although postmortem histology showed persistence of the collagen implant at the injection sites.

Administration, Intravesical

Urethral dilation improves bladder compliance in children with myelomeningocele and high leak point pressures.

Among 350 children with myelomeningocele 18 with elevated leak point pressures were managed by urethral dilation and followed for 1 to 5 years. Longitudinal measurements of bladder compliances revealed durable improvement in the pressure-volume relationships after dilation. These data suggest that noncompliant bladders are acquired because of high outlet resistance, and early intervention may improve long-term bladder storage characteristics.

Child

Urethral dilation in the management of urological complications of myelodysplasia.

Urethral dilation was performed in 11 of 65 myelodysplastic children with high intravesical pressures. These 11 children had persistent bladder pressure elevations uncontrolled by intermittent catheterization and anticholinergic agents. After dilation intravesical pressures decreased, and upper tract function and measured bladder compliance improved. No discernible effect on continence function occurred. The procedure appears to be a safe alternative to temporary vesicostomy in myelodysplastic children with high intravesical pressures that cannot be controlled by intermittent catheterization and anticholinergic agents.

Child

Bladder compliance in meningomyelocele children.

The 2 components of detrusor compliance were analyzed in 32 children with myelomeningocele and compared to compliance in 15 age-matched controls with nonneuropathic enuresis. In myelomeningocele initial compliance was variable, whereas terminal compliance was relatively constant. Low terminal compliance corresponded with vesicoureteral reflux, deterioration of upper tract morphology and diminished renal function.

Child

Percutaneous nephrostogram in the newborn with bilateral renal cystic disease.

Percutaneous antegrade pyelography should be considered in the few, select, critically ill newborns with bilateral renal cystic disease when the diagnosis is critical to management and difficult with the usual imaging procedures. Two extremely ill newborns with severe oliguria and cystic abnormalities in both kidneys by ultrasound underwent sonographic guidance for percutaneous antegrade nephrostograms in the first week of life. With injection of contrast medium, definitive diagnoses were made of a multicystic dysplastic kidney on one side and an obstructed hydronephrotic kidney on the other, thereby directing decompression of the obstructed kidney to preserve native renal function. This procedure can provide a definitive diagnosis in these rare but difficult cases.

Humans