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Biomedical subjects

Peter J Strouse

Publications and source records attributed to Peter J Strouse.

At least 19 recordsLinked to original sources

CT of tracheal agenesis.

BACKGROUND: Tracheal agenesis is a rare and usually lethal anomaly. In the past, opaque contrast medium was injected via the esophagus to demonstrate the anatomy. OBJECTIVE: To demonstrate the utility of helical and multidetector CT in delineating the aberrant anatomy in newborns with tracheal agenesis. MATERIALS AND METHODS: Four newborns with tracheal agenesis were identified from three institutions. Imaging studies and medical records were reviewed. Each child was imaged with chest radiography. One child was imaged on a single-detector helical CT scanner and the other three on multidetector scanners. RESULTS: Helical and multidetector CT with 2D and 3D reconstructions clearly delineated the aberrant tracheobronchial and esophageal anatomy in each infant. Minimum intensity projection reformatted CT images were particularly helpful. One infant each had type I and type II tracheal agenesis. Two infants had type III tracheal agenesis. All four infants died. CONCLUSION: CT is a useful tool for delineating the aberrant anatomy of newborns with tracheal agenesis and thus helps in making rational clinical decisions.

Artifacts↗

Late migration of a Sideris buttoned device for occlusion of atrial septal defect.

We describe a 17-year-old patient with an atrial septal defect who underwent device closure with a second generation Sideris buttoned device at 4 years of age. She presented 13 years after the procedure with the acute onset of chest discomfort, at which time a chest radiograph showed migration of the wire of the right atrial counter-occluder to the lateral aspect of the right atrial wall, with an associated right atrial perforation. The patient underwent uneventful surgical device retrieval and atrial septal defect closure. Late migration of a portion of the Sideris buttoned device can occur, suggesting the need for continued follow up, and a high index of suspicion for device failure should a patient become symptomatic.

Adolescent↗

FDG PET for the study of primary hepatic malignancies in children.

PURPOSE: This manuscript describes the use of FDG PET in a series of 7 children (11 scans) with primary hepatic malignancies (5 patients with hepatoblastoma, 2 patients with hepatic embryonal rhabdomyosarcoma), together with other imaging (CT and MRI), serum tumor markers, and tumor pathology. MATERIALS AND METHODS: Seven children with pathologically proven hepatic malignancies underwent 11 FDG PET scans for staging (1 patient) or restaging (6 patients). Tumor uptake of FDG was assessed qualitatively and compared with biochemical and radiological findings. RESULTS: Abnormal uptake was demonstrated in 6 of 7 patients (10 of 11 scans). Three patients subsequently underwent partial hepatic resection, and one underwent brain biopsy, confirming in each that the abnormal uptake of FDG indicated viable tumor. In one patient, intense uptake was due to necrotizing granulomas. In one patient, images were suboptimal due to noncompliance with fasting. CONCLUSION: Primary hepatic tumors of childhood usually demonstrate increased glycolytic activity, which allows them to be imaged using PET and the tracer 18F-FDG. The technique is probably most useful for assessing response to therapy, in following AFP (alfa fetoprotein) negative cases and for detecting metastatic disease although a large series of patients will need to be studied to confirm our initial findings. Non neoplastic inflammation may also accumulate FDG and could be confused with malignancy. As these tumors are rare, prospective multicenter studies are necessary to determine the true clinical utility of FDG PET imaging in the management of children with primary hepatic malignancies.

Child, Preschool↗

High resolution computerized tomography of the chest and pulmonary function testing in evaluating the effect of tobramycin solution for inhalation in cystic fibrosis patients.

To evaluate the sensitivity of high-resolution computerized tomography (HRCT) of the chest compared to spirometry measures in evaluating the effects of tobramycin solution for inhalation (TSI) in cystic fibrosis (CF) patients.Thirty-two subjects >/=6 years old with mild to moderate CF lung disease were enrolled in a randomized, double-blind, placebo-controlled pilot study. Duration was 28 days; 31 subjects completed the study.HRCT scores decreased 4.06 +/- 3.20 (mean +/- SD) for TSI and decreased 0.17 +/- 1.78 for placebo subjects (P = 0.13). Mean forced expiratory flow during middle half of forced vital capacity (FEF(25%-75%)) predicted increased 6.08 +/- 4.86 for TSI and decreased 0.60 +/- 2.34 for placebo (P = 0.23). Percentage forced expiratory volume in 1 s (FEV(1)) predicted increased slightly for both TSI and placebo (1.29 +/- 3.33 for TSI and 1.17 +/- 1.4 for placebo) (P = 0.97). Two of eight HRCT component scores (atelectasis and inhomogeneity) were observed to be highly discordant with observed HRCT global total score and other HRCT component scores. A modified total score was calculated by dropping them from the global total score. The modified HRCT total scores decreased 6.68 +/- 3.09 for TSI subjects and increased 0.02 +/- 2.0 for the placebo subjects (P = 0.07). Sample sizes were calculated to show statistical significance by differences in modified total HRCT scores, global total HRCT scores, FEF(25%-75%) predicted or FEV(1) % predicted. A total of 60, 100, 200, and over 800 patients would be necessary respectively.HRCT can be a useful measure of change in CF pulmonary disease, requiring a smaller sample size than that required to show treatment effect by pulmonary function testing (PFT) alone.

Administration, Inhalation↗

Sonographic evaluation of the child with lower abdominal or pelvic pain.

At many centers, CT has become the primary imaging modality for children who have abdominal pain. CT, however, delivers a substantial radiation dose, which is of particular concern in the pediatric patient. In contrast, sonography does not expose the patient to ionizing radiation. Properly performed, sonography is capable of providing useful diagnostic information in the child who has lower abdominal or pelvic pain. In many children and with many disorders, sonography proves to be the only imaging modality that may be required. In this article, the usefulness of sonography in evaluating disorders producing lower abdominal or pelvic pain in a child is reviewed.

Abdominal Pain↗

Antiangiogenic therapy for a large splenic hemangioma.

Hemangiomas involving the spleen are rare and seldom symptomatic. Treatment options for large lesions usually consist of splenectomy, embolization, or both. Antiangiogenic treatment has not been reported previously as an effective alternative for this type of lesion. We report our experience of successfully using glucocorticoids in an infant with a large hemangioma of the spleen.

Angiogenesis Inhibitors↗

Posterior mediastinal hemangioma.

We report posterior mediastinal hemangiomas in a 4-month-old and a 6-month-old girl. The masses were identified on radiographs of the chest followed by contrast-enhanced CT. Histological evaluation of the surgical specimens established the final diagnosis. Although mediastinal hemangiomas have been described, they remain a rare entity. A diagnosis can be suggested by relatively high attenuating masses on contrast-enhanced CT. Posterior mediastinal hemangiomas sometimes mimic neuroblastomas, which is the most common posterior mediastinal in this age group.

Contrast Media↗

Bilateral giant abdominoscrotal hydroceles complicated by appendicitis.

Abdominoscrotal hydrocele is a rare entity, with fewer than 100 cases reported in children. Bilateral abdominoscrotal hydroceles are even less common, with 14 cases reported in children. Various complications of abdominoscrotal hydrocele have been reported in the literature. We present a 4-month-old boy with bilateral giant abdominoscrotal hydroceles who developed appendicitis apparently because of obstruction from the right hydrocele. We discuss the various imaging modalities used to establish the diagnosis and plan the operative approach.

Abdomen↗

Prenatal diagnosis in pregnancies at risk for Joubert syndrome by ultrasound and MRI.

OBJECTIVES: To describe the prenatal imaging findings in fetuses at risk for Joubert syndrome (JS), review the literature and propose a protocol for prenatal diagnosis of JS using ultrasound and MRI. METHODS: We reviewed prenatal ultrasound and fetal MRI studies in two pregnancies at 25% recurrence risk for JS and correlated these findings with gross neuropathology in one affected fetus. RESULTS: While abnormalities such as occipital encephalocele or enlarged cisterna magna have been identified before mid-trimester, the definitive diagnosis of JS, based on core cerebellar findings, has only been possible after 17 weeks' gestation. CONCLUSIONS: With longitudinal monitoring, it is possible to diagnose JS in at-risk pregnancies before 24 weeks' gestation. On the basis of our data and review of the literature, we propose a protocol for monitoring pregnancies at risk for JS, utilizing serial ultrasounds combined with fetal MRI at 20-22 weeks' gestation to maximize the accuracy of prenatal diagnosis.

Ataxia↗

Failure of cholecystokinin-octapeptide to prevent TPN-associated gallstone disease.

PURPOSE: Gallstone formation is a common problem in neonates on prolonged courses of total parenteral nutrition (TPN). The authors hypothesized that the use of cholecystokinin-octapeptide (CCK), given at the time of TPN administration, would prevent gallstone formation in a high-risk group of patients with TPN. METHODS: A prospective, randomized, blinded, controlled trial of neonates who were on a prolonged course of TPN for prematurity (25 infants), necrotizing enterocolitis (NEC, 8 infants), or abdominal surgery (5 infants) were selected randomly to receive CCK vs placebo. Patients remained on the study until taking more than 50% of energy enterally. Children were recalled between 2 and 4 years after completing TPN for ultrasonographic examination of their hepatobiliary tree. RESULTS: Neonates (38 studied) required a mean (+/-SD) of 33 +/- 16 days of TPN. Cholelithiasis was detected in 4 (10%) infants. Cholecystokinin-octapeptide was not effective in preventing the formation of gallstones (3 stones in infants receiving CCK, P = .51). Diagnosis (P = .56), birth weight (P = .54), gestational age (P = .18), and duration of TPN (P = .53) did not correlate with gallstone formation. To address the management of these stones, all 4 were placed on a prolonged course of ursodeoxycholic acid (mean duration, 11.6 +/- 5.4 months). Two additional infants (not in the original study) with TPN-associated gallstone disease were also given a trial of ursodeoxycholic acid. Serial ultrasounds were performed every 6 months. No patient achieved any degree of stone dissolution. One patient underwent cholecystectomy for symptomatology. CONCLUSIONS: Total parenteral nutrition-associated gallstones were detected in 10% of children, and most are nonsymptomatic. Cholecystokinin-octapeptide prophylaxis was not effective in preventing TPN-associated gallstones. In addition, the use of ursodeoxycholic acid did not dissolve gallstones, once identified. Future methods will be needed to address the prevention and treatment of these stones.

Cholagogues and Choleretics↗

Use of cholecystokinin-octapeptide for the prevention of parenteral nutrition-associated cholestasis.

OBJECTIVE: To determine whether cholecystokinin-octapeptide (CCK-OP) would prevent or ameliorate parenteral nutrition-associated cholestasis (PNAC) among high-risk neonates treated with total parenteral nutrition. STUDY DESIGN: This was a multicenter, double-blind, randomized, controlled trial conducted between 1996 and 2001. PATIENTS: Neonates at risk for the development of PNAC included very low birth weight neonates and those with major surgical conditions involving the gastrointestinal tract. SETTING: Tertiary care hospitals. INTERVENTION: Patients were randomized to receive CCK-OP (0.04 mug/kg per dose, twice daily) or placebo. Eligible infants were all <30 days of age. Patients were enrolled within 2 weeks after birth or within 7 days after surgery. OUTCOME MEASURES: The primary outcome measure was conjugated bilirubin (CB) levels, which were measured weekly. Secondary outcome measures included incidence of sepsis, times to achieve 50% and 100% of energy intake through the enteral route, number of ICU and hospital days, mortality rate, and incidences of biliary sludge and cholelithiasis. RESULTS: A total of 243 neonates were enrolled in the study. CCK-OP administration did not significantly affect CB levels (1.76 +/- 3.14 and 1.93 +/- 3.31 mg/dL for CCK-OP and placebo groups, respectively; mean +/- SD). Secondary outcome measures also were not significantly affected by the study drug. CONCLUSIONS: Use of CCK-OP failed to reduce significantly the incidence of PNAC or levels of CB. CCK-OP had no effect on other secondary measures and should not be recommended for the prevention of PNAC.

Bilirubin↗

Disorders of intestinal rotation and fixation ("malrotation").

Malrotation with volvulus is one of the true surgical emergencies of childhood. Prompt radiological diagnosis is often paramount to achieving a good outcome. An understanding of the normal and anomalous development of the midgut provides a basis for understanding the pathophysiology and the clinical presentation of malrotation and malrotation complicated by volvulus. In this essay, the radiologic findings of malrotation and volvulus are reviewed and illustrated with particular attention to the child with equivocal imaging findings.

Duodenum↗

Congenital short-gut syndrome.

A case of congenital short gut is reported in a 4-month-old boy presenting with failure to thrive. Upper gastrointestinal examination (UGI) with small bowel follow-through (SBFT) demonstrated dilation of the duodenum and jejunum, with rapid transit to rectum. On barium enema (BE), rapid transit of barium was noted to the dilated proximal small bowel seen on UGI/SBFT. Neither study delineated the ileocecal region, and the overall length of bowel appeared short. The diagnosis of congenital short gut was confirmed at surgery. Congenital short-gut syndrome is a rare entity. The diagnosis can be suggested by imaging, but is usually confirmed operatively.

Barium Sulfate↗

Pulmonary involvement in pediatric lymphoma.

BACKGROUND: The prevalence of pulmonary lymphoma in the pediatric age group is not documented in the literature. OBJECTIVE: This study was designed to assess the prevalence of pulmonary parenchymal lymphoma in children with Hodgkin disease (HD), non-Hodgkin lymphoma (NHL) and post-transplant lymphoproliferative disorder (PTLD). MATERIALS AND METHODS: A 10-year retrospective analysis of 161 lymphoma patients (62 girls and 99 boys), mean age of 12.4 years, was performed. The definition of pulmonary lymphoma excluded those with isolated pleural disease and/or mediastinal adenopathy. RESULTS: Eighty-two patients had HD, 65 had NHL, and 14 had PTLD. Overall prevalence of pulmonary parenchymal involvement was 13% (21/161), including 12% of patients with HD, 10% of patients with NHL, and 29% of patients with PTLD. CT findings included: pulmonary nodules (90%) or mass (38%); interstitial (9%) or alveolar (9%) disease; cavitation (9%); and pleural based mass (9%). CONCLUSIONS: Pulmonary parenchymal disease in our pediatric lymphoma population was more prevalent than expected (13%). This is significant for patient management. New pulmonary lesions in patients with known lymphoma should be regarded with suspicion. In the setting of immune suppression, pulmonary lesions treated as infection may actually represent lymphoma. Expeditious biopsy of lesions failing to respond promptly to antibiotic therapy should be considered.

Adolescent↗

Transient small-bowel intussusception in children on CT.

OBJECTIVE: To determine the frequency and significance of small-bowel intussusception identified in children on CT. MATERIALS AND METHODS: All abdomen CT reports between July 1995 and April 2002 were reviewed to identify patients with small-bowel intussusception. Intussusceptions were identified as an intraluminal mass with a characteristic layered appearance and/or continuity with adjacent mesenteric fat. Ileocolic intussusceptions and intussusceptions related to feeding tubes were excluded. Imaging studies and medical records were reviewed. RESULTS: Twenty-five pediatric patients (16 boys, 9 girls; mean age 11.2 years) were identified with small-bowel intussusception on CT. No patient had a persistent intussusception requiring surgery. Fourteen had limited immediate repeat CT images as part of the same examination, ten of which demonstrated resolution of the CT abnormality. Follow-up CT [ n=13 (6 within 24 h)], ultrasound ( n=3), small-bowel follow-through ( n=4) and surgery ( n=3) showed no intussusception. In four patients with persistent symptoms, underlying pathology was identified requiring treatment (giardiasis, 2; small-bowel inflammation/strictures, 1; abscess and partial small-bowel obstruction after perforated appendicitis, 1). In 21 other patients, direct correlation of symptoms to CT abnormality was absent or questionable, no treatment was required, and there was no clinical or imaging evidence of persistence or recurrence. CONCLUSION: Most small-bowel intussusceptions identified in children by CT are transient and of no clinical significance.

Adolescent↗

Characterization of a stapes ankylosis family with a NOG mutation.

OBJECTIVE: To characterize the otologic phenotype in a family with autosomal dominant stapes ankylosis, hyperopia, and skeletal abnormalities caused by a mutation in the noggin gene (NOG). STUDY DESIGN: Case series. SETTING: Academic tertiary care center. PATIENTS: Eight affected and 3 unaffected family members. MAIN OUTCOME MEASURES: History, physical and radiologic examination, and surgical outcomes. RESULTS: Although affected members were initially presumed to have typical nonsyndromic otosclerosis, the clinical data were most consistent with an autosomal dominant congenital stapes ankylosis syndrome. Eight of eight affected family members had bilateral low-frequency conductive hearing loss. Six of eight underwent fenestration procedures and/or stapedectomies. All members with initial postoperative closure of the air-bone gap returned to their baseline conductive loss within 2 years. Two affected family members had documented maximal conductive hearing loss by age 4, and two members without previous otologic surgery have not experienced sensorineural hearing loss. High-resolution temporal bone computed tomography showed stapes ankylosis and indistinction of the incudomalleal junction bilaterally and bony regrowth over the stapedotomy for those with stapedectomies. Detailed physical and radiologic examination identified multiple other skeletal abnormalities. CONCLUSIONS: Although this phenotype may present as classic otosclerosis to the otolaryngologist, detailed investigation revealed a congenital stapes ankylosis syndrome. Because is essential in regulating normal bone development and maturation, mutations in this gene may be associated with excessive bony overgrowth and refixation of the stapes footplate after initial successful surgery. Patients with hereditary conductive hearing loss should be assessed to rule out subtle features of a skeletal syndrome.

Ankylosis↗

Pediatric renal pelvic fullness: an ultrasonographic dilemma.

PURPOSE: We conducted a prospective study to define normal renal pelvic size in children. MATERIALS AND METHODS: Institutional Review Board approved consent was obtained to perform renal ultrasound during excretory urography (IVP) scheduled for medical management. Mean patient age (17 females, 11 males) was 5.2 years. Renal ultrasound was conducted concurrent with 10-minute IVP. RESULTS: Fifty kidneys were imaged with 51 collecting systems. IVP defined 44 collecting systems as normal. Mean anteroposterior pelvic diameter on sonography for these 44 systems was 3.3 mm. One normal collecting system on IVP had a diameter greater than 10 mm on ultrasound (14 mm). No sonographic caliceal dilatation was seen in any kidney appearing normal on IVP. The 7 dilated systems on IVP had a mean ultrasound diameter of 17.1 mm. Two dilated collecting systems smaller than 10 mm in diameter on sonography had caliceal distention on ultrasound. CONCLUSIONS: Normal renal pelvis threshold diameter was 10 mm in asymptomatic children. We recommend further evaluation in children with caliceal dilatation and/or dilatation of the anteroposterior renal pelvis greater than 10 mm. Using these criteria, no system appearing abnormal on IVP would have been missed.

Adolescent↗