Marc S. Keller

Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein

Congenital absence of the portal vein with systemic visceral venous return in a 8-year-old girl with oculoauriculovertebral dysplasia (Goldenhar Syndrome) had been previously reported following its discovery during preoperative evaluation of a liver mass which was diagnosed as focal nodular hyperplasia after open biopsy. Subsequently, an enlarging diffuse hepatic neoplasm developed with associated elevated alpha feto-protein levels. Repeat biopsy and imaging showed the tumor to be a hepatoblastoma involving both lobes of the liver. The patient was treated by hepatic resection and orthotopic liver transplantation and is doing well at 18 months follow-up.

Early presentation of metastatic medullary carcinoma in multiple endocrine neoplasia, type IIA : Implications for therapy

A girl 5 years 11 months of age, belonging to an extensive kindred with multiple endocrine neoplasia, type IIA (MEN IIA), was found to have multifocal medullary thyroid carcinoma with metastasis in one paraglandular lymph node after positive findings on a calcium-pentagastrin stimulation test. Her sister, 3 years 8 months of age, also had an elevated calcitonin level, and thyroidectomy revealed C-cell hyperplasia and a focus of medullary thyroid carcinoma. These two cases underscore the need for prophylactic thyroidectomies in MEN IIA patients as young as 5 years of age and strict yearly provocative screening beginning at age 1 year.

Umbilical venous catheterization and the risk of portal vein thrombosis.

Portal vein thrombosis has been associated with umbilical venous catheterization. We studied the incidence of portal vein thrombosis associated with umbilical venous catheterization with the catheter tip not in the portal venous system. Appropriate placement of an umbilical venous catheter in sick neonates is associated with a low risk of portal vein thrombosis (actual incidence, 1.3%).

Giant Intracranial Aneurysm in a Child with Tuberous Sclerosis: CT Demonstration

The authors describe the first case of giant intracranial aneurysm in a child with tuberous sclerosis. The subject of vascular disease in this condition is reviewed.

Imaging of surgical diseases of the newborn chest : Intrapleural mass lesions

Prenatal detection of intrapleural mass lesions is commonplace. Diagnostic treatment plans often are formulated before birth. The radiologists involvement in dealing with congenital lobar emphysema, congenital cystic adenomatoid malformation, extralobar pulmonary sequestration, and congenital diaphragmatic hernia has changed. The need for immediate postnatal diagnosis has been de-emphasized, but the demand for precision and efficiency in preoperative cross-sectional imaging, monitoring progress and complications of treatment, and assistance with nutritional support has increased.

The Ultrasonographic Differentiation of Obstructive Versus Nonobstructive Hydronephrosis in Children: A Multivariate Scoring System

PURPOSE We identified sonographic prognosticators to aid in distinguishing obstructive from nonobstructive hydronephrosis in children. MATERIALS AND METHODS Twelve sonographic variables were initially analyzed to determine significant associations between the variables and the presence of urinary tract obstruction as defined by diuretic radionuclide renography. The significant findings were subsequently subjected to logistic regression models to identify potential predictors for obstructive hydronephrosis. RESULTS The 7 variables associated with a significantly higher risk of urinary tract obstruction were increased echogenicity, parenchymal rims 5 mm. or less, contralateral hypertrophy, resistive index ratio 1.10 or greater, resistive index difference with diuresis of 70% or greater, ureter diameter 10 mm. or greater and aperistaltic ureter. These variables were used for the development of a multivariate scoring system. CONCLUSIONS The obstructive scoring system shows promise as a screening method for the sonographic differentiation of obstructive from nonobstructive hydronephrosis in children.

CT differentiation of benign and malignant lung nodules in children

The superiority of computed tomography (CT) for detection of lung nodules has been documented and attempts have been made to distinguish benign from malignant lesions in adults. We attempted to characterize lung nodules in 12 children with solid malignant tumors (aged 8 months to 17 years) in an effort to differentiate benign from metastatic disease. All scans were performed at 10-mm contiguous intervals on a GE 9800 CT scanner. The scans were retrospectively viewed by two pediatric radiologists independently and without knowledge of the pathological findings. All biopsies were done via open thoracotomy. The CT findings were correlated with pathology results. Twelve children had 13 nodules biopsied. Six of these showed malignancy, two showed inflammatory changes, and two had a reactive subpleural lymph node. In three children, no abnormality was found and a biopsy was not obtained. One child had a metastatic nodule in one lung, and a simultaneous inflammatory nodule in the other. The radiologists agreed with each other on the CT interpretation in 11 of 13 surgically explored areas. They correctly predicted malignancy in four cases and correctly excluded it in two cases. However, they were simultaneously incorrect in five instances. Our conclusion is that, contrary to reports in adults, a tiny nodule may be either benign or malignant. Malignancy cannot be separated from benign disease by CT established criteria.

Sonographic evaluation of ureterectasis in children : the significance of peristalsis

The presence or absence of ureteral peristalsis was noted during real-time sonography of 61 dilated ureters in children. The findings were correlated with diagnoses established using standard radiographic and radionuclide imaging techniques. Of the 47 dilated ureters that exhibited peristalsis 44 were classified as not obstructed when assessed with standard imaging and functional studies. The most frequent etiology for ureteral dilatation associated with peristalsis was high grade vesicoureteral reflux (31 ureters). Three peristaltic ureters were shown to be mildly to moderately obstructed. Absence of peristalsis was noted in 14 ureters: 13 were severely obstructed, while in 1 the involved kidney had no function. In the pediatric age group the demonstration of peristalsis in a dilated ureter is frequently associated with vesicoureteral reflux and is seldom associated with obstruction. Obstruction, if present, usually is mild. Aperistaltic ureterectasis implies severe obstruction or poor renal function.

IPSILATERAL PYELOURETEROSTOMY FOR MANAGING LOWER POLE REFLUX WITH ASSOCIATED URETEROPELVIC JUNCTION OBSTRUCTION IN DUPLEX SYSTEMS

PURPOSE In duplex renal systems with a normal upper pole collecting system, and a lower pole collecting system with reflux and ureteropelvic junction obstruction or a kink at the ureteropelvic junction (that is reflux with trapping), treatment must address reflux and obstruction. We reviewed the results of ipsilateral pyeloureterostomy as a single corrective procedure. MATERIALS AND METHODS We retrospectively reviewed our experience with 10 children who had complete ureteral duplication with lower pole reflux (grades III to V) and associated lower pole ureteropelvic junction obstruction or a ureteral kink with trapping. All patients were treated with ipsilateral end-to-side pyeloureterostomy. Followup ranged from 1 to 14 years. RESULTS All patients had a good postoperative result, there have been no immediate or long-term complications and no subsequent surgical procedure was needed. All patients had continued lower pole renal growth with good function, 9 had resolution or a marked decrease in pelvicaliceal dilatation and none had evidence of obstruction. CONCLUSIONS Ipsilateral end-to-side pyeloureterostomy for treating reflux into the lower moiety of a duplicated collecting system with associated ureteropelvic junction obstruction or a kink appears to be a single, safe corrective procedure that simultaneously manages vesicoureteral reflux and relieves the proximal obstructive process.

Hemophilia presenting in an infant as a radial artery pseudoaneurysm following arterial puncture

Abstract The case presented is of an infant who developed a radial artery pseudoaneurysm following arterial puncture and was subsequently diagnosed with hemophilia. A discussion of radial artery pseudoaneurysms follows.