Sydney Heyman

Changing concepts in management of primary obstructive megaureter

The management of neonatal urinary tract dilatations represents one of the most challenging dilemmas in pediatric urology today. We have been confronted with 44 renal units in 35 neonates diagnosed as having primary obstructive megaureter during the last 6 years. Of these units 23 in 17 infants were diagnosed antenatally and 20 (87 per cent) have been managed without surgical intervention. Notably, 16 renal units were graded as moderate to severe megaureters by an excretory urogram. The decision to manage conservatively was based on the initial extraction of the 99mdiethylenetriaminepentaacetic acid renal scan (the extraction factor). This estimate of absolute renal function has been used to differentiate dilatations with obstructive implications for the renal parenchyma from those without. Significantly, expectant treatment has resulted in improvement of dilatation on sequential excretory urograms in 15 megaureters and none has shown a deterioration of function by renal scan. Similar diagnostic criterion also has resulted in conservative management for 12 of 21 additional neonatal megaureters seen during this period with symptoms or they were discovered serendipitously. Only 2 of these 12 megaureters required surgical correction. The neonatal primary megaureter appears in many cases to represent a different entity than those that commonly presented before the advent of antenatal and perinatal diagnosis.

Managing Apparent Ureteropelvic Junction Obstruction in the Newborn

A total of 97 newborns with apparent ureteropelvic junction obstruction was evaluated from mid 1984 to 1989. Evaluation and management are described. Of these patients 39 with an affected kidney showing good initial differential function (greater than 35%) by diethylenetriaminepentaacetic acid scan and 2 showing diminished function (less than 35%) were followed nonoperatively. Six patients (15%) eventually required pyeloplasty for diminishing function (4), urinary tract infections (1) or symptoms of colic (1). The 4 patients with diminishing function improved after pyeloplasty to at least the initial level. A total of 12 patients with good initial function (greater than 35%) of the affected kidney underwent early pyeloplasty (within 6 weeks of diagnosis). They were compared to the similar group of patients managed nonoperatively and followed by sequential renal scans. Eventual changes in percentage differential function in the nonoperative and early surgery groups were +2.8% and +4.1%, respectively. Changes in extraction factor were +0.8% (nonoperative group) and +0.9% (surgery group). No statistically significant difference was found. In the kidney with apparent ureteropelvic junction obstruction and good function, an initial nonoperative approach with sequential renal scan followup and pyeloplasty as needed appears to be reasonable and has resulted in no permanent loss of function.

Targeted radiotherapy with submyeloablative doses of 131I-MIBG is effective for disease palliation in highly refractory neuroblastoma.

Purpose Treatment of refractory neuroblastoma remains a significant clinical problem. Targeted radiotherapy with 131I-MIBG has demonstrated antitumor activity in heavily pretreated neuroblastoma patients with recurrent disease. Response rates may be correlated with total radionuclide dose per kilogram body weight delivered, but higher dose levels are associated with protracted grade 4 hematologic toxicity. The optimal method for using single-agent 131I-MIBG for patients with relapsed high-risk neuroblastoma has not been defined. This study was designed to retrospectively determine the clinical response to 131I-MIBG therapy at submyeloablative doses in patients with refractory neuroblastoma and to describe the toxicities. Patients and Methods A retrospective chart review of 20 patients with neuroblastoma treated with 131I-MIBG at the Childrens Hospital of Philadelphia from 1988 to 2000 was performed. Demographic data, 131I-MIBG dose delivered, toxicities, and clinical responses were reviewed. Results A median dose of 9.5 mCi/kg of 131I-MIBG was delivered in 32 courses to 20 patients. Three patients were treated in first complete response, and the remaining 17 patients for residual and/or progressive disease. The objective response rate to the first therapy was 31%, and the remaining patients achieved disease stabilization. In addition, 9 of 11 patients with pain at study entry had significant improvement. Disease response was not correlated with 131I-MIBG dose delivered. No unanticipated toxicities were observed. Conclusions Submyeloablative-dose 131I-MIBG is an effective and relatively nontoxic method for neuroblastoma disease palliation. Most patients show subjective improvement in pain and/or performance status. Increased availability and experience with 131I-MIBG therapy would benefit a large number of children with end-stage neuroblastoma and no realistic hope for cure.

131I MIBG therapy in neuroblastoma: Mechanisms, rationale, and current status

131I MIBG has been used as palliative treatment of neuroblastoma patients with recurrent or persistent disease who failed other modalities of treatment. Since the results were promising, the concept arose of using it in conjunction with other modalities, either as an up-front treatment or as combination therapy. This article reviews the principle of 131I MIBG treatment, in conjunction with other modalities currently used for the treatment of neuroblastoma, in an attempt to improve the final outcome.

The extraction factor: an estimate of single kidney function in children during routine radionuclide renography with 99mtechnetium diethylenetriaminepentaacetic acid.

An estimate of absolute renal function in children is described using the initial extraction of 99mtechnetium diethylenetriaminepentaacetic acid. The extraction factor correlates well with the glomerular filtration rate as determined by the clearance from the blood of 99mtechnetium diethylenetriaminepentaacetic acid with a correlation coefficient of 0.92. The normal mean extraction factor in the newborn is 1.5 per cent by each kidney, which increases to 2.5 per cent in the first year of life. The method is simple to perform and it is reproducible provided care is taken. We recommend that this procedure be included as part of the routine renal scan in children.

A case of microgastria in association with splenic-gonadal fusion

Microgastria is a rare congenital anomaly usually associated with asplenia. In this 21/2-year-old presenting with left hydrocele and inguinal hernia multiple accessory spleens were found in the inguinal-scrotal region compatible with splenic-gonadal fusion.99mTc-sulfur colloid scanning is helpful in microgastria searching for the presence of splenic tissue and in splenic-gonadal fusion for the location of accessory heterotopic spleens.

The Radionuclide salivagram for detecting the pulmonary aspiration of saliva in an infant

A radionuclide study is described utilizing a small volume of technetium 99m sulfur colloid to demonstrate the pulmonary aspiration of saliva. This is a possible cause of recurrent pulmonary infections even in patients off oral feeding. The salivagram is a useful technique to document these cases.

Skeletal scintigraphy of pseudo-osteomyelitis in Gaucher's disease. Two case reports and a review of the literature.

Two patients with known type 1 Gauchers disease had signs and symptoms of osteomyelitis. Decreased perfusion and impaired uptake were seen on bone scan. Patient 1 had a culture-negative aspirate of the site. Patient 2 had no surgical procedures. Both improved on bed rest and nonsteroidal anti-inflammatory agents. It is difficult to distinguish osteomyelitis clinically from pseudo-osteomyelitis. The authors suggest that photopenia on bone scan performed within 1 to 3 days of presentation is suggestive of pseudo-osteomyelitis in Gauchers disease.

The lymphatic spread of osteosarcoma shown by Tc-99m-MDP scintigraphy.

Osteosarcoma usually spreads via the blood stream, resulting in pulmonary and skeletal metastases. The value of bone imaging in the management of these patients is widely accepted. Less commonly, the disease spreads via the lymphatics. Such a case is reported to show that bone imaging will detect progressive involvement of the lymph nodes.

Pediatric gastrointestinal motility studies

Radionuclide studies for evaluating gastrointestinal transit in adults have been adapted for use in infants and children for assessing esophageal transit, gastroesophageal reflux, and gastric emptying. However, the measurement of small- and large-bowel transit times in these patients has been limited.