Biomedical subjects
Publications and source records attributed to J L Strife.
Barium esophagrams were obtained in two patients with respiratory problems whose underlying congenital heart disease was pseudotruncus arteriosus type I. The esophagrams revealed vascular indentations on the esophagus, one posteriorly, and the other anteriorly. Both types of indentations were secondary to aorto-pulmonary communicating arteries from the descending aorta.
The presenting chest radiographs of 27 new-born patients with D-transposition of the great vessels (D-TGV) were evaluated for the degree of pulmonary flow as well as other findings classically described in D-TGV (narrow superior mediastinum, radiographically absent thymus, inapparent main pulmonary artery, non-visualization of the malpositioned aortic arch, asymmetric pulmonary blood flow, and cardiomegaly). Of the 27 patients, 22 (82%) demonstrated normal or decreased flow. The majority of the D-TGV patients also failed to demonstrate any of the other classically described radiographic findings. A normal chest radiograph is the most common presenting scenario in the neonate with D-TGV.
Eighty-one cases of multicystic dysplastic kidney (MCDK) in children were diagnosed over the past 11 years at the authors' institution: 25 children had their kidneys surgically removed, eight with bilateral total involvement died, and 48 underwent serial follow-up ultrasonography (US) of their kidneys. Follow-up included 193 serial ultrasound (US) studies (mean, four per patient) for a total of 1,468 months (mean, 30.5 months). In the 48 patients followed up, 32 (67%) kidneys showed a decrease in size, nine (19%) showed no change, and five (10%) increased in size, and in two (4%), a change in size could not be determined. In seven of the 48 (15%) children, the MCDKs decreased in size, and, at follow-up US, no renal tissue could be found. In those patients in whom MCDKs decreased in size. Serial US characteristics changed from predominantly an enlarged cystic structure to a small dysplastic or absent kidney. Two of the five kidneys that increased in size were surgically removed, and MCDK was pathologically confirmed. A nonsurgical approach to the treatment of patients with MCDK is supported by this study.
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Velopharyngeal insufficiency was assessed using multiview videofluoroscopy on eight patients with hypernasality, 10 patients with hypernasality and audible nasal emission, and 10 patients with nasal turbulence (rustle). Patients demonstrating hypernasality, with or without audible nasal emission, were found to have a significantly larger velopharyngeal gap than those with nasal rustle. This finding suggests that the degree of the velopharyngeal insufficiency can be predicted to some extent based on perceptual assessment. If the presence of a nasal rustle suggests a small velopharyngeal gap, despite the severe distortion of speech, then speech therapy should be considered prior to surgery in those cases.
The limited availability of suitable size-matched donor whole organs has been a major cause of morbidity and mortality in infants and children requiring liver transplantation. Techniques for transplanting segments of the liver have successfully addressed this problem. The surgical anatomy of the left hepatic lobe transplant is unique in three primary respects: the cut edge, the presence of an enteric Roux loop for biliary drainage, and the alteration in the position and number of hepatic vessels. The spectrum of normal imaging findings in 17 left-lobe allografts is illustrated.
Paired voiding cystourethrogram (VCU) and nuclear cystogram (NC) studies in 68 children with vesicoureteral reflux (VUR) were analyzed to determine the variability of VUR on sequential studies. Sequential paired examinations included 82 studies where a VCU was followed by a repeat VCU study and 128 studies where a VCU was followed by a NC study. Grade I VUR on the initial VCU was difficult to duplicate on repeat cystography, regardless of the follow-up modality. When the initial VCU showed no VUR, 20% of follow-up studies demonstrated VUR. Follow-up VCU and NC studies showed similar results in detecting increases and decreases in the grade of VUR. Low-grade VUR may be intermittent, and clinical therapeutic decisions should take this variability into account.
Fifty-five children (34 boys, 21 girls; age range, 1 day to 18 years) with increased echogenicity of the renal medullary pyramids at ultrasound evaluation were identified. The clinical diagnoses associated with hyperechoic medullary pyramids could be separated based on the presence or absence of hypercalciuria. Patients with drug-induced hypercalciuria included 10 infants treated with furosemide, two treated with long-term steroid therapy, and one treated with excessive amounts of vitamin D. Other clinical conditions associated with hypercalciuria included renal tubular acidosis (n = 10), Bartter syndrome (n = 5), hyperparathyroidism (n = 3), Williams syndrome (n = 2) and medullary sponge kidney (n = 2). Ten children with transient renal insufficiency and three with sickle cell disease had normal urine calcium concentration. Isolated disease entities accounted for the remainder of cases. A specific diagnosis can usually be made in a patient with hyperechoic renal medullary pyramids by using a systematic clinical approach that includes evaluation of patient age, serum and urine calcium concentration, and renal function.
The natural progression of nephropathic cystinosis to end stage renal disease can be delayed, sometimes by many years, by the reducing agent, cysteamine, which lowers intracellular cystine content to near normal. We report on two patients with nephropathic cystinosis who were treated with cysteamine and developed structural genitourinary abnormalities which may have contributed to an increase in the rate of decline of renal function. One patient, aged 11 years, was found to have massive megacystis and hydroureteronephrosis but no anatomic bladder outlet obstruction. His abnormality was presumed to be related to chronic high urine volumes leading to megacystis and physiologic ureteral obstruction. Vesicostomy stabilized renal function. The second patient, aged 11 1/2 years, was found to have bilateral renal cystic disease which presumably was acquired and may have been related to long-standing hypokalemia. Minor renal abnormalities were found by ultrasound in five additional cystinotic children. We concluded that older children with nephropathic cystinosis may be prone to acquire structural abnormalities of their kidneys or urinary tract.
The use of an air enema for diagnosis and treatment of intussusception has recently gained popularity. The current end point for reduction is the reflux of air into the terminal ileum. The authors report three cases in which air freely refluxed into the terminal ileum without complete reduction of the intussusceptum. Thus, reflux of air alone cannot be relied on as the sole criterion for reduction. Close examination of the cecum for a persistent filling defect is imperative to exclude unsuccessful reduction.
Diagnostic imaging plays a major role in the diagnosis and treatment of chronic liver disease in children. Decisions regarding the need for sclerotherapy, palliative shunts, and anatomic suitability for hepatic transplantation are based on the upper abdominal anatomy. Thirty-six children with various forms of chronic liver disease were referred for diagnostic imaging, including MR and sonography. Each study was evaluated independently without knowledge of the other examination. The first six patients were evaluated retrospectively and the last 30 patients prospectively. The size of the portal vein and inferior vena cava varied in this population, with excellent agreement between sonography and MR. A 2-mm portal vein was shown only on MR imaging in two patients, but MR missed a 3-mm portal vein. Portosystemic collateral vessels also were evaluated and noted to be detected more readily with MR imaging (64%) than with sonography (22%). In particular, paraumbilical veins were detected more often with MR (28% vs 6% by sonography). Associated or coexisting anomalies were relatively common in children with chronic liver disease (14%). These included polysplenia (two cases), preduodenal portal vein (one), unilateral dysplastic kidneys (two), ureteropelvic junction obstruction (one), and splenic (one) and renal (one) cysts. MR imaging was more sensitive than sonography for detection of abnormal anatomy in this group of patients and should be considered the pretransplantation imaging technique of choice in children with end-stage liver disease.
In infants, the site of the aortic arch is frequently inferred from the position of the intrathoracic trachea. We retrospectively reviewed the plain frontal chest radiographs of 72 patients with cyanotic congenital heart disease and right aortic arch documented by cardiac catheterization. In infants, the position of the trachea was variable: 47% had the trachea on the right, 33% had the trachea midline and in 7% the trachea was on the left. As the child grows, the aortic arch progressively indents the trachea, and the descending aorta is better visualized. Since the position of the trachea in infants with right aortic arch is variable, one must be cautious in inferring the site of the aortic arch by the position of the trachea.
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This retrospective study documents the findings on nuclear cystography and renal sonography of 455 girls who had urologic imaging for a proved urinary tract infection (UTI). Nuclear cystograms were normal in 313 (69%) of 455 patients. Vesicoureteral reflux was seen in 142 patients (31%): six with grade I, 90 with grade II, 43 with grade III, and three with grade IV. Twelve percent of patients with vesicoureteral reflux had renal parenchymal scars. Increasing grades of reflux were associated with an increase in the severity and number of parenchymal scars. Normal renal sonograms were seen in 83% of patients. Abnormalities noted on sonograms included parenchymal scarring in 31 (7%) of the 455 patients, anomalies in 19 patients (4%), mild to moderate dilatation of the renal pelvis and or ureters in 45 patients (10%), and bladder wall thickening in 45 patients (10%).
Articulation, resonance, and velopharyngeal function were evaluated before and after Le Fort I maxillary advancement in 16 patients (seven with cleft lip and palate, one with cleft lip only, and eight without clefts). On the postoperative evaluation, seven of 11 patients with preoperative articulation errors showed an improvement in articulation after surgery. Two patients without clefts showed slight changes in nasal resonance, and two patients (one with cleft lip and palate and one with cleft lip only) developed mild nasal emission. Nine patients showed diminished velopharyngeal contact during speech on videofluoroscopic studies. Compensatory changes in velopharyngeal function were also observed, which included velar stretching and lengthening and increased lateral pharyngeal wall movement.
For neonates with ischemia of an extremity or extensive thrombosis of the aorta after umbilical artery catheterization, prompt recognition and management decisions are necessary. The cases of eight infants with symptomatic thrombosis who were treated with fibrinolytic agents were retrospectively reviewed to study means of diagnosis and response to therapy. Peripheral thrombosis was seen in two otherwise healthy infants; fibrinolytic therapy produced complete lysis in one and partial lysis in the other. The six infants with central thrombosis presented with low Apgar scores and multiple clinical problems; umbilical catheters were already in place. To assess the clot, real-time sonography was performed in all six patients, and umbilical arteriograms were obtained in five. Fibrinolytic therapy produced complete lysis of clot in five of the six infants. The one death occurred in a premature infant in whom a large intracranial hemorrhage developed 6 hours after institution of therapy.