The renal axes in spina bifida: issues of confusion and fusion.

The occurrence of horseshoe kidneys in myelodysplasia has been suggested to be overestimated because of the concurrent prevalence of kyphotic spine in the spina bifida population. Pseudohorseshoe kidneys result from the actual medial migration and apposition of the lower renal poles in the deep fossa created by the gibbus deformity. The presence of a lumbosacral kyphosis, however, does not imply there is not a true horseshoe kidney. In our myelodysplasia population of 189 patients, occurrence of true horseshoe kidneys (13 patients) and pseudohorseshoe kidneys (14 patients) was increased. The finding of lumbosacral kyphosis was twice as common in the pseudohorseshoe population as in the true horseshoe population. True horseshoe kidneys were three times more commonly seen in association with congenital vertebral anomalies cephalad to the dysraphic spine. Diagnosis in this study was predominantly based on renal cortical scintigraphy with the posterior pin-hole magnification technique.

Nephrocalcinosis in X-linked hypophosphatemia: effect of treatment versus disease.

While the literature has suggested that vitamin D and phosphate therapy benefits children, and possibly adults, with X-linked hypophosphatemia (XLH), there have been increasing reports of nephrocalcinosis by renal ultrasound examination in treated patients. Although it has been assumed that nephrocalcinosis is the result of treatment, there has been no systematic comparison of treated and untreated XLH patients with long-term follow-up. Therefore, we studied 10 adults and 4 children by renal ultrasound examination with no history of medical therapy and 10 adults and 8 children with a history of medical therapy. None of the untreated patients had nephrocalcinosis; 5 treated adults and 5 treated children had nephrocalcinosis. In 3 of 4 treated children, serial renal ultrasounds did not show progression of the nephrocalcinosis. One patient progressed from baseline to year 1 only. Comparisons of means between treated patients without and with nephrocalcinosis showed statistically significant differences for urine calcium/creatinine ratios and urine phosphorus/creatinine ratios, differences not seen between untreated patients and treated patients without nephrocalcinosis. Phosphate dose, but not vitamin D dose, was statistically significantly different between the two treated groups. Our studies point to a convincing role for the association between nephrocalcinosis, as diagnosed by renal ultrasound examination, and medical therapy.

Ultrasonographic features of gastrointestinal duplications.

The variable sonographic appearance of duplication cysts is presented. Eighteen sonograms from 14 patients, aged 1 day to 8 years, were reviewed over an 8 year period. Water and other aqueous contrast agents were used in six patients as part of the sonographic evaluation. All lesions were confirmed by surgery. All but two patients were symptomatic. Twenty-four cysts were detected, ranging in size from 1.7 to 15.5 cm. The duplication cysts revealed a spectrum of sonographic findings (cystic to solid appearing masses). The mass characteristics, including the "muscular rim sign," and internal debris or hemorrhage, were demonstrated. Multiple unsuspected cysts (3 of 14 or 20%) and complications such as perforation were readily seen with ultrasonography. Serial sonograms demonstrated the changing morphology of two cysts. Other unsuspected intra-abdominal and pelvic pathologic conditions, including pyloric stenosis and ovarian cysts, were identified. Identification of the muscular rim sign is the most reliable indication of a duplication cyst. Multiple masses as well as possible accompanying anomalies in the abdomen and pelvis are readily evaluated with sonography. Lesions are easily followed with serial studies if there is no surgical intervention.

Cervical trachea: dynamics in response to herniation of the normal thymus.

Nineteen infants aged 2 months to 2.5 years, first seen predominantly with stridor, were noted to have intermittent soft-tissue mass effect in the upper airway during routine evaluation with fluoroscopic or radiographic methods. The cervicothoracic trachea was always buckled posteriorly and, in almost all instances, to the right during forced exhalation (crying). Real-time ultrasound examination with the neck extended was used in these patients to define the cause of the tracheal dynamics. The intermittent cephalic movement of the homogeneous echotextured thymus from the anterior mediastinum into the neck was determined to be the probable cause of the mass effect in these infants. Magnetic resonance imaging in three infants confirmed this finding. The intermittent, physiologic suprasternal movement of the thymus in these infants did not by itself cause any luminal compromise of the trachea and did not result in any respiratory difficulty in these infants.

Correlative imaging of pelvic soft tissue sarcomas in children.

The authors retrospectively reviewed 15 cases of pelvic soft tissue sarcoma treated at the Children's Hospital of Philadelphia during a recent 6-year period. These patients were treated before the availability of magnetic resonance imaging. The authors compared ultrasonography, computed tomography, and excretory urography/voiding cystourethrography for establishing the diagnosis and following the progression/regression of the disease. Results were correlated with clinical, surgical, and pathologic findings. Although computed tomography and ultrasonography were both capable of characterizing the size and texture of the lesion, computed tomography was superior in defining disease arising from the pelvic sidewall. Both techniques effectively identified residual mass at the tumor site, but neither could differentiate active tumor from inactive tumor, posttherapy inflammation, or fibrosis. Excretory urography/voiding cystourethrography provided no information that could not be gleaned from either ultrasonography or computed tomography.

Outpatient assessment of infants with bronchiolitis.

Two hundred thirteen infants younger than 13 months with bronchiolitis were prospectively followed up to identify the historical, physical, and laboratory clues at initial emergency department evaluation that would help to predict disease severity. Based on their total course of illness, the patients were classified as having mild (139 patients) or severe (74 patients) disease, and the initial emergency department evaluation findings of these two groups were compared. Six independent clinical and laboratory findings were identified that were strongly associated with more severe illness: (1) "ill" or "toxic" general appearance; (2) oxygen saturation less than 95%, as determined by pulse oximetry; (3) gestational age, younger than 34 weeks; (4) respiratory rate, 70/min or greater; (5) atelectasis on a chest roentgenogram; and (6) age, younger than 3 months. The infant's oxygen saturation as determined by pulse oximetry was the single best objective predictor of more severe disease.

Diagnosis and follow-up of children with Wilms' tumor: correlative study of ultrasound and computed tomography. Report of eighteen cases.

A retrospective study of 18 children with Wilms' tumor was performed to compare the efficacy of ultrasound (US) and computed tomography (CT) for the diagnosis, staging, and follow-up evaluation of these patients. US and CT were found to be complementary to one another in evaluation of Wilms' tumor. In one patient, CT was more accurate than US in determining the organ of origin of a very large tumor. US is superior to CT for the evaluation of thrombosis in IVC. It can define whether or not the thrombus is attached to the wall of the IVC. This finding has a major impact on the management of the patient.

Ultrasound guidance. For interventional and intraoperative techniques in infants and children.

Since the advent of improved realtime ultrasound (US) imaging and the availability of the needle biopsy attachment, US-guided percutaneous biopsy and the drainage procedure can be performed safely in the pediatric patient. The authors review their experience over 3 years using US-guided interventional techniques in 96 children aged 5 days to 21 years. A wide variety of procedures were performed and included renal biopsy, nephrostomy tube placement, renal mass biopsy, puncture of bladder sinus tract and pancreatic pseudocyst, ventriculoperitoneal shunt placement and intraoperative localization of brain or renal tumor and renal calculi. No complication occurred. US can be safely and accurately used as guide for interventional procedures in children as long as the area of interest can be clearly demonstrated and the depth and necessary angle of needle insertion can be predetermined as feasible.

Mayer-Rokitansky-Kuster-Hauser syndrome: US aid to diagnosis.

The Mayer-Rokitansky-Kuster-Hauser syndrome is composed of vaginal atresia with other variable Müllerian duct abnormalities such as bicornuate or septated uterus. The fallopian tubes, ovaries, and broad and round ligaments are normal. Unilateral renal and skeletal anomalies are associated in 50% and 12% of cases, respectively. Patients have a normal female karyotype and normal secondary sexual development. Previously, one had to rely on radiographic contrast studies and surgical exploration for accurate definition of the reproductive tract anatomy. The authors performed ultrasound (US) examinations on 12 patients, aged 5 days to 18 years, with the Mayer-Rokitansky syndrome. US allowed correct identification of the genitourinary anomalies found in these girls, including eight cases of unilateral renal agenesis; one absent, one rudimentary, and ten duplicated or obstructed uteri; eight duplicated or obstructed vaginas; and associated complications such as endometriosis. Eight of the patients had lower abdominal pain, often cyclical in nature. The findings demonstrate that high-resolution, real-time US in conjunction with water vaginography permits the anatomy of these complex anomalies to be defined.

Ultrasound evaluation of neck masses in children.

Neck masses in children are a frequent occurrence and in some instances create a diagnostic dilemma for the clinician. The authors' two-year experience using ultrasonography in the evaluation of 34 children with neck masses is reviewed. Twenty-two patients had lesions arising outside the thyroid gland; 12 lesions arose from the thyroid gland. Ultrasonography proved to be an accurate imaging modality for localizing the mass and demonstrating its relationship to the thyroid gland, trachea, and major neck vessels. The borders and extent of the lesions were well outlined, as well as their internal consistency. Based on this review, the authors recommend that ultrasonography be the first screening procedure in pediatric patients who present with perplexing neck masses.

Fibrous dysplasia of the facial bones and mandible.

Five cases of fibrous dysplasia isolated to the facial bones and mandible are presented. A review of the clinical and roentgen features of facial fibrous dysplasia are described.