Fetal lung interstitial tumor: Prenatal presentation of a rare fetal malignancy.

Fetal lung interstitial tumor (FLIT) is a rare fetal malignancy that is typically diagnosed in the postnatal period, or, if recognized prenatally can mimic a benign lesion such as congenital pulmonary airway malformation. We present the earliest case of a FLIT tumor described by ultrasound and MRI at 26 weeks of gestation. Our case highlights features suggestive of FLIT including presentation later in gestation in combination with findings on fetal MRI such as a solid appearance with radiating curved bands of high signal within and along the periphery of the lesion (not as intensely high signal as the typical CPAM), possibly detailing a radiographic signature for these tumors. The role of betamethasone for these tumors is not known.

Acute pyelonephritis: comparison of diagnosis with 99mTc-DMSA, SPECT, spiral CT, MR imaging, and power Doppler US in an experimental pig model.

PURPOSE: To compare the sensitivity and specificity of technetium-99m dimercaptosuccinic acid (DMSA) single photon emission computed tomography (SPECT), spiral computed tomography (CT), magnetic resonance (MR) imaging, and power Doppler ultrasonography (US) for the detection and localization of acute pyelonephritis by using histopathologic findings as the standard of reference. MATERIALS AND METHODS: Bilateral vesicoureteric reflux was surgically created in 35 piglets (70 kidneys). One week later, a liquid bacterial culture of Escherichia coli was injected into the bladder. Three days after induction of urinary infection, imaging studies were performed, and the kidneys were removed for histopathologic examination. SPECT images were obtained 2-3 hours after injection of 99mTc-DMSA. Transverse and coronal MR images were obtained with gadolinium-enhanced fast inversion recovery. Transverse CT images were obtained before and after injection of contrast agent. Power Doppler US was performed in longitudinal, transverse, and coronal planes. Each kidney was divided into three zones for correlation of findings. RESULTS: Histopathologic examination revealed pyelonephritis in 102 zones in 38 kidneys. Sensitivity and specificity for detecting pyelonephritis in the kidneys were 92.1% and 93.8% for SPECT, 89.5% and 87.5% for MR imaging, 86.8% and 87.5% for CT, and 74.3% and 56.7% for US. Sensitivity and specificity for detecting pyelonephritis in the zones were 94.1% and 95.4% for SPECT, 91.2% and 92.6% for MR imaging, 88.2% and 93.5% for CT, and 56.6% and 81.4% for US. The pairwise comparison of these modalities showed no statistically significant difference among them except for US. CONCLUSION: 99mTc-DMSA SPECT, spiral CT, and MR imaging appear to be equally sensitive and reliable for the detection of acute pyelonephritis; power Doppler US is significantly less accurate.

Prevalence of Müllerian duct anomalies detected at ultrasound.

The true prevalence of müllerian duct abnormalities is not well established. We used standard ultrasound examinations to establish the prevalence of müllerian duct abnormalities in girls and women who were evaluated for reasons unrelated to the presence of uterine anomalies. Prospective ultrasound examinations for nonobstetric indications in 2,065 consecutive girls and women (aged 8-93) showed that 8 had anomalies, including bicornuate uterus, septate uterus, and double uterus. In this first attempt to determine the prevalence of uterine anomalies in the general population, using noninvasive methodology, the rate of anomalies was 3.87 per 1,000 women (exact 95% confidence interval: 1.67-7.62), or approximately 1 in 250 women. This prevalence estimate may be greater than the true rate if selection biases are strong, or less than the true prevalence if ultrasound detection rates are low. As a first attempt to establish the prevalence by pelvic ultrasound in a consecutive series, these data may serve as a baseline estimate.

Inflammatory bowel disease in children and young adults: correlation of sonographic and clinical parameters during treatment.

OBJECTIVE: The objective of this study was to evaluate the sonographic findings of inflammatory bowel disease activity in children undergoing treatment. SUBJECTS AND METHODS: Eighty-eight sonograms were obtained of 23 bowel segments in 17 children and young adults (age range, 10-21 years; mean, 16 years) with new or recurrent inflammatory bowel disease. Sixteen segments were involved with Crohn's disease and seven with ulcerative colitis. Serial sonography (range, two to eight examinations; mean, four per segment) was performed while patients underwent treatment. Bowel wall thickness measurements and color and power Doppler sonography grading were recorded and compared with clinical data. RESULTS: All 17 patients had at least one abnormal bowel segment on initial sonography. The correlation was significant (p < 0.01). Agreement was 91% on direction of change over time between bowel wall thickness and Doppler grades, with 100% correlation between color and power Doppler sonography grades. In patients with Crohn's disease, the correlation was significant (p < 0.05) between bowel wall thickness and Doppler grades with two of seven and four of seven clinical parameters, respectively. In patients with ulcerative colitis, the correlation was significant (p < 0.05) between bowel wall thickness and Doppler sonography grades with four of seven and three of seven clinical parameters, respectively. The erythrocyte sedimentation rate correlated with all sonographic measurements in both patient groups. Combining bowel wall thickness and Doppler sonography, the percentage of agreement was significant in the direction of change, with five of seven clinical parameters in both patient groups. CONCLUSION: Gray-scale and color or power Doppler sonography can show changes in disease activity in children and young adults undergoing treatment for inflammatory bowel disease.

Sonographic detection of uterine and ovarian abnormalities in female survivors of Wilms' tumor treated with radiotherapy.

OBJECTIVE: The purpose of this study was to use sonography to evaluate the size of the ovaries and uterus in survivors of Wilms' tumor who underwent radiotherapy. SUBJECTS AND METHODS: Eighteen survivors of Wilms' tumor had their ovaries and uterus measured on sonography. Their ages at diagnosis and treatment ranged from 14 months to 6 years. Four girls were prepubertal (age, 5-9 years), 11 were postpubertal (age, 11-30 years), and three had primary ovarian failure (age, 15-23 years) at the time of imaging. Findings were compared with those of a control group of 25 prepubertal and 25 postpubertal girls and women. Gonadotropin levels were measured. RESULTS: Three patients who underwent whole abdomen radiotherapy had elevated levels of gonadotropin and primary ovarian failure. Neither ovary was seen in two of the three patients and both ovaries were abnormally small (< or = 1 cm3) in the third patient. The uterus was abnormally small (length, < or = 4 cm) in all three of these patients even though two were being treated with hormone replacement therapy. Ten postpubertal patients who underwent hemiabdomen radiotherapy had normal gonadotropin levels and a normal-sized uterus on sonography; the ovary on the side that received radiotherapy was not seen in three of the 10 patients or was abnormally small (< or = 1.4 cm3) in two of the 10 patients compared with all normal ovaries in the postpubertal control group (p < .0001). One postpubertal patient with bilateral renal bed radiotherapy had normal ovaries and a normal-sized uterus. Significantly more patients in the postpubertal and ovarian failure radiotherapy group (5 [36%] of 14 patients) had one or both ovaries not seen than the control group (none [0%] of 25 patients; p = .0014). The uterus was significantly smaller than normal in three (23%) of the 13 patients in the postpubertal hemiabdomen and ovarian failure radiotherapy group versus none of the 25 patients in the postpubertal control group (p = .0339). CONCLUSION: Postpubertal female survivors of Wilm's tumor who underwent radiotherapy as children may have one or two small or absent ovaries and a small uterus that can be detected by sonography. The response of the uterus to hormone replacement therapy can also be assessed on sonography.

Imaging in pediatric urology.

A multitude of disorders of the genitourinary tract can occur in children. Although some entities may be diagnosed clinically, radiologic imaging is often necessary for diagnosis and management. The radiologic work-up has been discussed using a problem-oriented approach in five clinical situations: urinary tract infection, hydronephrosis or hydroureter, trauma, swollen scrotum, and hematuria. This discussion provides some general guidelines, although the evaluation of each child may need to be individualized depending on their specific clinical symptomatology.

Colonic diverticulitis causing partial bowel obstruction in a child with cystic fibrosis.

Children and adolescents with cystic fibrosis (CF) may manifest bowel pathology with resulting bowel obstruction. Recognized causes of bowel obstruction in CF patients include meconium ileus, intussusception, distal intestinal obstruction syndrome and postoperative adhesions. Additionally, the development of colonic strictures in children with CF has recently been described. We report an unusual cause of partial obstruction of the ascending colon in a child with CF due to pathologically proven diverticulitis.

Uterine anomalies in Wilms' tumor survivors.

BACKGROUND: The association between Wilms' tumor (WT) and genitourinary (GU) anomalies has long been appreciated; however, associated GU anomalies have been described almost exclusively in males. METHODS: To investigate whether females with WT also have an increased prevalence of GU anomalies, the authors evaluated the uterine anatomy of 24 WT survivors using magnetic resonance imaging and ultrasonography. RESULTS: Two of 24 female survivors (8%) had anomalies. One had a septate uterus, and a limited molecular analysis of her constitutional DNA revealed no mutations or deletions of the tumor suppressor gene WT1. Another survivor with the WAGR syndrome (WT, aniridia, GU anomalies, and retardation), with the characteristic 11p13 deletion including WT1, had a uterine anomaly (hypoplastic vs. unicornuate). CONCLUSIONS: Because uterine malformations are rare in the general population, this finding suggests an association between WT and uterine malformations and also may partially explain the fertility deficit previously demonstrated in adult female WT survivors. Pelvic ultrasonography in adult female WT survivors can alert survivors and clinicians to possible fertility problems that may lead to problem pregnancies and adverse pregnancy outcomes.

Perinatal and postnatal chest sonography.

Sonography is the primary method used to image the fetal chest. Many significant congenital anomalies such as pleural effusion, congenital diaphragmatic hernia, cystic adenomatoid malformation, pulmonary sequestration, and congenital heart disease can be detected during early prenatal sonography. Fetal sonography also permits accurate assessment of the severity of these processes, allowing for parental counseling and optimal planning of postnatal care. After birth, sonography is the primary method for evaluating cardiac anatomy and diagnosing congenital heart disease. Sonography also serves as a useful adjunct to plain film radiology and other modalities in evaluation of the mediastinum, diaphragm, pleura, and chest wall.

Appendicitis: usefulness of US in diagnosis in a pediatric population.

One hundred eighty pediatric patients with suspected appendicitis were prospectively examined with graded compression ultrasonography (US) to assess the sensitivity, specificity, and accuracy of graded compression US in the diagnosis of appendicitis in children and to compare those results with results of clinical assessment in the diagnosis of this disorder. Patients were assigned to one of three groups prior to US based on the clinical level of confidence that appendicitis was present and on the planned management decision. Of 141 patients in the low- and intermediate-clinical risk categories, 20 (14%) had appendicitis: US had a sensitivity of 100%, specificity of 97%, and accuracy of 97% in these two groups. Of 39 patients in the high-clinical risk category, 32 (82%) had appendicitis: US had a sensitivity of 81%, specificity of 86%, and accuracy of 82%. Of 52 patients with surgically proved appendicitis, the initial management decision was to discharge to home or admit for observation and further testing in 18 (35%). Results at US were positive for appendicitis in all 18 patients in the latter two categories.

Pelvic inflammatory disease in the adolescent: comparison of transabdominal and transvaginal sonographic evaluation.

Transabdominal (TA) and transvaginal (TV) sonograms (n = 116) were obtained in 84 patients aged 12-21 years (mean, 16.2 years) with the clinical diagnosis of acute pelvic inflammatory disease (PID). The studies were compared for image quality and unique diagnostic information. TV sonography demonstrated superior resolution of 25 dilated fallopian tubes. Heterogeneous pelvic masses, described as tubo-ovarian abscesses on TA sonograms, could be separated on TV sonograms into various stages of PID including pyosalpinx, hydrosalpinx, tubo-ovarian complex, and tubo-ovarian abscess. Thirty-one TA and TV studies were normal despite patients fulfilling strict clinical criteria for PID. The level of severity of PID, as determined at TA sonography, was altered in 28 cases, with medical therapy changed in 23 cases because of additional TV sonographic findings. TV sonography provided superior anatomic detail in the evaluation of patients with PID, demonstrating abnormalities that were not seen at TA sonography in 71% of patients.

Radioaerosol assessment of lung improvement in cystic fibrosis patients treated for acute pulmonary exacerbations.

We compared bronchopulmonary distribution homogeneity of a radioaerosol before and after hospitalization in 20 patients with cystic fibrosis (CF) with pulmonary exacerbations in order to assess lung improvement. Deposition homogeneity was quantified in terms of skew (an index of distribution symmetry), derived from frequency distribution histograms generated from gamma camera images of the lungs following radioaerosol inhalation. Lower skew values indicate enhanced distribution homogeneity. Right lung skew (RLS) was significantly reduced following therapy (1.00 +/- 0.49 to 0.84 +/- 0.47), whereas skew in the left lung was unchanged (0.95 +/- 0.38 to 0.87 +/- 0.40). The reduction in RLS was significant in patients with Shwachman-Kulczycki (SK) clinical scores less than 50 (1.27 +/- 0.53 to 0.90 +/- 0.42), but not in patients with scores greater than 50 (0.81 +/- 0.38 to 0.80 +/- 0.52). These results indicate that treatment affected the right lung more than the left lung, particularly in patients with SK scores less than 50, and suggests that radioaerosol lung imaging may be valuable in identifying sites of impairment to be targeted during treatment. Statistically, skew was less sensitive an indicator of acute change than several other clinical indices that improved following hospital treatment.

Obstructed uterovaginal anomalies: demonstration with sonography. Part I. Neonates and infants.

The sonograms of five neonates with an obstructed urogenital sinus and four with an obstructed cloaca were retrospectively reviewed to determine the sonographic features and the role of pre- and postnatal sonography in the diagnosis and management of hydrocolpos. On pre- and/or postnatal sonograms, the obstructed genital tract was visualized in seven neonates as a large cystic pelvic-abdominal mass, which was the markedly distended urine-filled vagina. One neonate had multiple cystic masses, which resulted from an obstructed duplex genital tract. In one of the neonates with an obstructed cloaca, the anomaly was not diagnosed. The bladder was compressed by the distended vagina and was not visualized in five patients. A vaginal fluid-debris level in six patients was a key finding that distinguished the vagina from the bladder. Obstruction of the urinary tract was an associated feature. An obstructed uterovaginal anomaly with renal dysplasia and oligohydramnios on prenatal sonograms indicates a poor prognosis. Sonography contributes to the diagnosis of an obstructed genital tract and helps define the internal genital anatomy.

Obstructed uterovaginal anomalies: demonstration with sonography. Part II. Teenagers.

The sonograms of 13 patients with obstructed uterovaginal anomalies were reviewed to determine the role of sonography in diagnosis and management. In a retrospective analysis, the authors were able to define the cause of the obstruction and the upper level and length of the obstruction by combining the sonographic findings with those from the physical examination (ie, bulging hymen, blind vaginal pouch, no vaginal depth, normal vagina) or by combining transabdominal sonography with simultaneous digital insertion into the vaginal orifice. When the sonogram demonstrated hematometrocolpos or hematocolpos, the lesion was due to an imperforate hymen (n = 1) or high, middle, or low transverse vaginal septum (n = 7). When the sonogram demonstrated a hematometra, the lesion was due to Mayer-Rokitansky-Küster-Hauser syndrome with functioning uterine anlage(n) (n = 2), cervical dysgenesis (n = 1), and an obstructed uterine horn (n = 2). The presence and patency or absence of the cervix was established. Since the differentiation between a transverse vaginal septum, Mayer-Rokitansky-Küster-Hauser syndrome with active anlagen, and cervical dysgenesis with absent vagina cannot be made clinically, sonography provides important presurgical information.