Ultrasound of female genital anomalies

Congenital anomalies of the female genital tract result from müllerian duct anomalies and/or abnormalities of the urogenital sinus or cloaca. Due to the close developmental relationship between the genital and the urinary tracts, association of anomalies in both systems are common. This article reviews the appearance of developmental anomalies of the female urinary and genital tracts and points out common associated malformations to allow an early and complete sonographic assessment of affected infant.     

Ultrasound of female genital anomalies

Congenital anomalies of the female genital tract result from müllerian duct anomalies and/or abnormalities of the urogenital sinus or cloaca. Due to the close developmental relationship between the genital and the urinary tracts, association of anomalies in both systems are common. This article reviews the appearance of developmental anomalies of the female urinary and genital tracts and points out common associated malformations to allow an early and complete sonographic assessment of affected infant.     

Applicability of Magnetic Resonance Imaging in the Assessment of Fetal Urinary Tract Malformations

ObjectiveTo assess the applicability of magnetic resonance imaging (MRI) to complement ultrasound in the diagnosis of fetal urinary tract anomalies.MethodsThis was a retrospective cohort study that included 41 women between 19 weeks and 37 weeks and 6 days of gestation carrying fetuses with malformations of the urinary tract which were initially diagnosed by ultrasound and then referred for MRI. In all cases, the diagnosis was confirmed after birth either through imaging or autopsy. A surface coil was positioned over the abdomen and T2-weighted sequences were obtained in the axial, coronal, and sagittal planes; T1 in at least one plane; and three-dimensional (3-D) TRUFI in fetuses with dilatation of the urinary tract.ResultsMean gestational age at the time of MRI examination was 28.21 weeks. The rapid T2 sequences allowed all the anomalies of the fetal urinary tract to be assessed, whereas 3-D TRUFI sequencing proved very useful in evaluating anomalies involving dilatation of the urinary tract. The signs of pulmonary hypoplasia characterized by hypointense signal in the T2-weighted sequences were identified in 13 of the 41 fetuses.ConclusionMRI confirmed and added information to the ultrasound regarding fetal urinary tract anomalies, as well as information related to the other associated malformations, their progress in the prenatal period, and possible postnatal prognosis.     

Anomalies of the distal ureter, bladder, and urethra in children: embryologic, radiologic, and pathologic features.

Congenital anomalies of the lower urinary tract are a significant cause of morbidity in infancy. Radiologic investigation is an important source of clinical information in lower urinary tract disorders but should not inconvenience the patient, expose the patient to unnecessary radiation, or delay surgical correction. In pediatric patients with suspected underlying urologic structural anomalies, screening ultrasonography is commonly the initial diagnostic study. If dilatation of the urinary tract is confirmed, voiding cystourethrography is performed to determine the presence of vesicoureteral reflux (VUR) and other causes of upper tract dilatation. If VUR is confirmed, follow-up with nuclear cystography or echo-enhanced cystosonography may be performed. If VUR is excluded, nuclear diuresis renography is the primary test for differentiating between obstructed and nonobstructed megaureter. Intravenous urography can be used to specifically identify an area of obstruction and to determine the presence of duplex collecting systems and a ureterocele. Computed tomography and magnetic resonance (MR) imaging are unsuitable for general screening but provide superb anatomic detail and added diagnostic specificity. MR imaging is mandatory in the evaluation of associated spinal anomalies. MR urography can demonstrate ectopic extravesical ureteric insertions, thereby providing a global view of the malformation. Familiarity with anomalies of the lower urinary tract is essential for correct diagnosis and appropriate management.     

Urodynamics in enuretic children.

Although the incidence of an organic cause is low and most of the detected genitourinary anomalies are not responsible for enuresis, a simple, noninvasive screening test is preferable in the analysis of children with enuresis. Extension of standard radionuclide renography as a method of screening the upper as well as lower urinary tract function in one session is noninvasive and at least as good as conventional direct urine flow rate recording. This method was carried out in 124 children. In 30%, some abnormality probably related to the enuresis was identified. Upper urinary tract anomalies were identified in 5% of the children, anomalies of the upper and the lower urinary tract in 9.6%, and functional voiding disturbances in 16%.     

Evaluation of dysfunction and malformations of the urinary tract in patients with meningomyelocele, by renal dynamic scintigraphy and direct radionuclide cystography. An Indian perspective

Meningomyelocele (MMC) is the most common form of neural crest closure defect and may be associated with dysfunction and malformations of multiple organs including urinary tract. It affects as many as 2-4 in 1000 live births, however there are no epidemiological studies available from India. Anatomical and neurogenic abnormalities of the urinary tract are known to occur in these patients. Scanty literature is available regarding the utility of radionuclide procedures in the evaluation of urinary tract in patients of MMC. The aim of this study was to evaluate urinary tract dysfunction and malformations in patients operated of MMC by diuretic renal dynamic scintigraphy (RDS) and direct radionuclide cystography (DRCG). In this retrospective analysis, 140 operated patients for MMC, mean age: 4.46, range: 1-18 years; 83 male and 57 female, referred to our department for evaluation of the upper renal tract, were studied. Neurogenic bladder (NB) was associated in 69 patients. The diagnosis of NB was based on clinical features like incontinence, expressibility of urine bladder and on urodynamic studies. RDS was performed after the intravenous administration of 10.36 MBq/kg of technetium-99m diethylene triamine penta-acetate or 99mTc-L, L-ethylenedicysteine. All patients with hydronephrosis (HDN) or hydroureteronephrosis (HDUN) on RDS underwent DRCG using 11.1-18.5 MBq of 99mTc-sulphur colloid. Our results showed that: out of the 140 patients, 78 (56%) including 33 with NB, had normal renal scintigraphy. Six patients (4%) had congenital renal anomalies. Twenty patients (14%) revealed non-obstructive HDN and had no vesico-ureteric reflux (VUR). Thirty-six patients (26%) with bilateral HDUN also had NB of which 19 (53%) had VUR. Fourteen (74%) of these patients with VUR had impaired renal function. In discussion, patients with MMC may present with disordered innervation of the detrussor muscle and external sphincter, which adversely affects the bladder function and potentially threatens the upper urinary tract. Patients of MMC with or without NB can be evaluated with RDS for congenital anomalies and initial assessment of renal function. Patients with NB and particularly those with HDUN can be evaluated with DRCG for the presence of VUR to prevent renal scarring and subsequent impairment of renal function. Long-term surveillance of the urinary tract is important in the management of these patients and this can be accomplished with RDS and DRCG. In conclusion, in our patients with MMC, the tests of RDS, and DRCG could differentiate those with normal or our abnormal renal anatomy and function while the major group of patients had bilateral HDUN and NB, and/or VUR or impaired renal function.     

Malformations of the fetal urinary tract of surgical significance. Diagnosis on the basis of pre- and post-natal imagings

Out of the 29 cases of fetal urinary tract anomalies detected by sonography, the authors report on the 17 cases with potentially correctable malformations. A critical analysis of the diagnostic contribution offered by the radiologic and sonographic studies is presented. The role of ultrasound examination in the choice of a suitable investigation strategy is emphasized, underlining the importance of an accurate approach to avoid diagnostic errors and unuseful procedures. The indications for fetal and neonatal antegrade pyelography are discussed.     

Harntransportstörungen bei Neugeborenen

Malformations of the urinary tract are one of the most common types of anomalies in the neonatal period. Approximately 15-20% of all prenatally detected malformations require acute postnatal imaging. Sonography is the primary imaging modality in the evaluation of nephro-urologic problems in infancy. Hydronephrosis represents the most common pathology, followed by multicystic-dysplastic kidney. DISCUSSION: The result of the sonographic examination directs further imaging. An adequate diagnostic algorithm is decisive for accurate diagnosis, for the appropriate therapeutic regimen and for the prognosis of the patients.     

Complementary role of MRI after sonography in assessing bilateral urinary tract anomalies in the fetus

OBJECTIVE: The objectives of our study were to evaluate the contribution of adding MRI findings to inconclusive sonographic data when assessing fetal urinary tract anomalies and to determine how this addition may affect the management of pregnancy. SUBJECTS AND METHODS. We prospectively used MRI to study 16 third-trimester fetuses in whom sonography suggested bilateral urinary tract anomalies but failed to provide a definite diagnosis. These anomalies included enlarged hyperechoic kidneys (n = 6), bilateral pelvicaliceal dilatation (n = 6), renal cystic lesions (n = 2), and renal agenesis associated with severe oligohydramnios (n = 2). RESULTS: The addition of MRI to sonography modified the diagnosis in five fetuses. In a fetus with suspected bilateral ureteropelvic obstruction, the diagnosis of bilateral ureterohydronephrosis associated with reflux or ureterovesical junction obstruction was made. In a fetus with an enlarged bladder at 32 weeks' gestational age, a possible diagnosis of megacystic microcolon was excluded on the basis of the normal appearance of the colon. In two fetuses with enlarged hyperechoic kidneys, MRI showed localized medullary hyperintense lesions suggesting autosomal recessive polycystic kidney disease in one fetus and medullary cystic dysplasia in another fetus with Jeune's syndrome. In a patient with suspected unilateral renal agenesis, MRI showed bilateral agenesis. In four fetuses, the addition of MRI to sonography led to a diagnosis that modified the decision to continue or terminate the pregnancy. CONCLUSION: MRI can accurately show many urinary tract anomalies in third-trimester fetuses. It may be a useful complementary tool in the assessment of bilateral urinary tract anomalies of fetuses, particularly in cases with inconclusive sonographic findings.     

Urinary tract infection in children. A reappraisal of its radiologic investigation

In a prospective study, 100 children with either an acute or a previous history of urinary tract infection were investigated by intravenous urography, micturition cystourethrography, and ultrasonography. The results from the three diagnostic modalities were compared: The urinary tracts in 59 patients were normal, and revealed some abnormality in 41. Ultrasonography proved to be superior to intravenous urography in outlining renal contours and in detecting subtle cortical changes secondary to urinary tract infection (such as slight increases in cortical thickness and edema or cortical scarring). The mucosa of the renal pelvis and bladder was more easily assessed by ultrasound than by intravenous urography. Both modalities were "equally" accurate in detecting important congenital malformations of the urinary tract. Ultrasound failed to detect 24 of 28 ureters demonstrating reflux on voiding cystourethrography. We propose that carefully performed abdominal ultrasonography can replace intravenous urography in the initial investigation of urinary tract infection in children. It should be done in association with a radiographic or radionuclide voiding cystogram. Intravenous urography would then become a complementary examination for abnormal or problematic patients.     

The spectrum of imaging in Currarino triad

The aim of this study was to evaluate the spectrum of findings and the efficacy of different imaging modalities in order to formulate recommendations for diagnostic imaging of Currarino triad (ASP syndrome), including screening of relatives. The imaging films of five female patients (age range 6 weeks to 12 months) were analysed retrospectively. The studied material consisted of US and MRI of the lower spine (5 patients each), lumbosacral plain radiography (4 patients), contrast enema (4 patients), urinary US (2 patients), genitography (1 patient) and myelo-CT (1 patient). Depiction of pathological findings with different imaging modalities was reviewed and validated with special respect to their demonstrability by US. Ultrasonography detected the sacral bony defect as well as the presacral pathology (meningocele and/or tumour) and thereby gave the basic diagnosis in all of the cases. It also depicted tethered cord and urinary tract abnormalities correctly. Magnetic resonance imaging gave a more distinct visualization of pre- and intraspinal pathology with additional demonstration of intraspinal lipoma in two cases. Regarding anorectal and genital malformations, radiographic contrast agent studies had been used in all patients. Two blind-ending retrorectal fistulas, depicted by enema, were missed by MRI. Patients with congenital or early infancy obstipation, anorectal malformations and complex urinary tract malformations should have spinal and pelvic sonography first. A plain film of the sacrum is recommended in equivocal cases. The need for MRI and contrast agent studies depends on the individual pathology, whereas presently MRI has made further radiographic imaging increasingly dispensable. A screening program with lumbosacral US or plain radiography for families with Currarino triad should be obligatory. Received: 22 May 1998; Revision received: 12 November 1998; Accepted: 6 January 1999     

Radionuclide imaging of rare congenital renal fusion anomalies

Demonstration of a congenital renal anomaly plays an important role in the treatment of patients with renal infection. These patients are prone to infections because of coexisting urinary tract anomalies such as duplicated ureter, ureter opening anomalies, and urinary stasis. Assessment of renal parenchymal damage resulting from acute or chronic renal infection is the primary indication for radionuclide imaging with Tc-99m DMSA. In addition, this technique allows congenital anomalies to be identified. The authors review congenital renal fusion anomalies identified in children through Tc-99m DMSA imaging. They conclude that Tc-99m DMSA imaging can reveal important diagnostic information about various congenital anomalies, including fusion anomalies.     

Double-blind ureteral duplication: report of two cases

Blind ending of ureteral duplication is one of the most rare anomalies of the upper urinary tract. We report two cases of ureteral duplication with a blind ending both superiorly and inferiorly, and with no definite communication with the urinary tract.     

The sonographic evaluation of fetal anomalies in oligohydramnios between 16 and 30 weeks gestation

The sonograms of all patients with oligohydramnios between 16 and 30 weeks gestation seen over a 4-year period were reviewed to determine (1) whether sonographically detectable fetal anomalies were present, and (2) when these anomalies were present, how this information was used in maternal fetal management. Cases of ruptured membranes and fetal demise were excluded from the study. Sixteen patients with severe oligohydramnios were identified. On postmortem examination, nine had urinary tract anomalies, one had evidence of a chronic intrauterine infection, and four had no anomalies. There were only two neonatal survivors: one had no anomalies while the other had posterior urethral valves. These findings confirm that second trimester oligohydramnios has a poor prognosis and is often associated with anomalies of the urinary tract. Sonography aids in the clinical management of such patients.     

Moderne Bildgebung beim Harnweginfekt im Kindesalter

Imaging in childhood urinary tract infection (UTI) is still a matter of debate. There are established guidelines, however new knowledge and the changed medical environment have enhanced this ongoing discussion. These new insights have impacted therapy and consequently the imaging algorithm. Modern imaging methods -- particularly MRI and modern ultrasound (US) -- are less invasive with a lower radiation burden. Additionally, it has been shown that VUR is a poor predictor for renal scarring out, which affects long-term results. Furthermore, the majority of UT malformations is depicted by prenatal US. The most crucial aspect of improving long-term outcome appears to be the early and reliable depiction of UTI and effective treatment to prevent renal scarring. This review tries to present this new knowledge and to discuss the potential of modern imaging. Recent changes in imaging algorithms are highlighted and an outcome-oriented algorithm that addresses these recent developments is proposed, without lightly abandoning established standards. It consists of an orienting US and -- for depiction of renal involvement -- amplitude coded color Doppler sonography or renal static scintigraphy (considered the gold standard, particularly for evaluating scars); in future MRI may play a role. Based on this concept, only patients with renal damage as well as patients with complex urinary tract malformations or intractable recurrent UTI may have to undergo VCUG.     

The fetal genitourinary tract

Early recognition of fetal urinary tract anomalies may dramatically influence obstetric or neonatal management. Major fetal urinary abnormalities, including renal agenesis, obstructive lesions, and cystic disease, may be clinically silent but readily identified by sonography. Careful evaluation of the fetal genitourinary tract should therefore be an important component of the routine screening obstetric sonogram. Appreciation of normal sonographic appearances of the fetal genitourinary tract may facilitate early recognition of the abnormal fetus. Accurate prenatal sonographic characterization of a renal abnormality and evaluation of renal function are essential for fetal prognosis and management.     

Ureteral dilatation in children with febrile urinary tract infection or bacteriuria

Little information is available on the relationship between urinary infection in children and infants, with or without vesicoureteral reflux, and dilatation of the urinary tract. The purpose of this study was to determine the effects of infection and reflux on the diameter of the ureter at excretory urography in children with acute, febrile urinary tract infections and in infants with bacteriuria found at screening. Standardized measurements of ureteral diameter were obtained for 79 children (2 months to 6 years old) with urinary tract infections and for 45 infants with bacteriuria. Patients with urinary tract obstruction or malformations were excluded. Seventy-one children with febrile urinary tract infection had ureteral visualization that allowed measurements. Ureteral diameter in this group was significantly wider than in a reference group, and 42 children (59%) had ureteral diameters that were more than 2 standard deviations above the normal mean. Ureteral diameter at excretory urography increased with increasing grades of reflux, but dilatation occurred also in the absence of reflux. Twenty-two of the 45 infants in the group with bacteriuria had sufficient ureteral visualization for measurements. The ureters in this group were wider than in the reference group, and eight infants had ureteral diameters that were more than 2 standard deviations above the normal mean. We conclude that ureteral dilatation is a common effect of acute urinary tract infection and bacteriuria in children.     

Imaging the pediatric prostate

The prostate gland is not often the target of imaging in children but may be imaged during investigation of symptoms related to the lower genitourinary tract such as hematuria, urinary retention, dysuria, and incontinence or during an evaluation for suspected congenital anomalies. Ultrasound and voiding cystourethrography are useful for initial evaluation of congenital and neoplastic disorders of the prostate. MR imaging and CT are useful in delineating more detailed anatomy before surgical planning and in determining the organ of origin in a patient who has a large pelvic mass.     

Bronchopulmonary and neurenteric forms of foregut anomalies. Imaging for diagnosis and management

Bronchopulmonary foregut malformations encompass a great variety of anomalies that may arise from abnormal differentiation of the respiratory and alimentary tracts, abnormal separation of the two systems, or abnormal development of blood supply, perhaps singly or in combination, during early embryogenesis. The pulmonary and neurenteric forms share the common features of a pulmonary parenchymal opacification and/or intrathoracic mass, with the addition of vertebral anomalies in the instance of the neurenteric malformations. Plain radiographs serve as the starting point for diagnostic evaluation and sometimes are all that is needed; more often, though, the plain film findings suggest which road to follow in further imaging. No single imaging approach can be advocated for all patients. The lesion may be found initially, for example, on prenatal sonography. Ultrasonography also can establish the cystic nature of a mass and may help define vascular supply, although it cannot yet be said to have supplanted angiography's role in evaluating suspected sequestrations. For most intramediastinal or intrapulmonary masses without associated vertebral anomalies, CT will satisfactorily establish the cystic nature and should afford a reasonably confident preoperative diagnosis of bronchogenic cysts or type 1 or 2 cystic adenomatoid malformations. Type 3 cystic adenomatoid malformation, because of its relatively "solid" imaging characteristics, may not be diagnosed confidently by preoperative imaging. The finding of vertebral anomalies associated with a mediastinal mass especially warrants MR imaging to define intraspinal involvement. However, the association of vertebral or rib anomalies with apparent unilateral pulmonary agenesis or hypoplasia arouses suspicion of a bronchopulmonary foregut malformation that communicates with the gastrointestinal tract, and this uncommon situation calls for a barium examination. Radionuclide scintigraphy has an ancillary role in assessing foregut anomalies, but many findings from scintigraphy are relatively nonspecific and anatomic definition is poor. Scintigraphy may yield supportive information in cases of sequestration that have inconclusive findings as determined by plain radiographs and CT.     

Ascites during the first week of life (author's transl)]

Retrospective analysis of 22 cases with neonatal ascites showed the most common cause to be anomalies of the urogenital system (urethral valve, hydronephrosis, rupture of bladder, rupture of ovarian cyst). Next came malformations of the gastro-intestinal tract and of the liver and congenital infections (toxoplasmosis, cytomegaly). In 4 cases the cause was not found. More than one half of the cases permitted a definitive radiologic diagnosis, mainly in malformations requiring surgery. We suggest a standardized procedure of investigation of all cases with neonatal ascites. If this procedure does not result in a positive diagnosis and if the ascites fluid does not contain blood or bile, we fell that laparotomy is not indicated.