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PubMed2000

Periurethral muscle complex reassembly for exstrophy-epispadias repair.

PURPOSE: Continence is a difficult goal in exstrophy-epispadias complex repair. It is presumed that all anatomical components involved in the exstrophy-epispadias abnormality are present but laterally and anteriorly displaced. The penile disassembly technique for epispadias restores the normal anatomical relationship of the male genital components. Its extension to complete primary bladder exstrophy closure enables deeper positioning of the bladder neck within the pelvic diaphragm. We identified the perineal striated muscular complex and present its appropriate periurethral reassembly as a main step in exstrophy-epispadias complex repair. MATERIALS AND METHODS: Bladder exstrophy and epispadias repairs were performed in 10 male and 3 female consecutive patients with the exstrophy-epispadias complex, including 1-stage reconstruction in 2 male newborns and 2 females with exstrophy, and as further surgery in a female with cloacal exstrophy and previous failed 1-stage repair, 4 males with incontinent epispadias (secondary repair in 1) and 4 males with epispadias in whom exstrophy closure had been previously done. In the males after bladder plate closure and corporeal body splitting a sagittal incision was made in the intersymphyseal tissue and extended posteriorly to the perineal body midline. The bipolar electrical stimulator was used to identify pelvic muscle components in the sagittal plane and reapproximate them along the tubularized posterior urethra to form the periurethral muscle complex. In the 3 females the urethral plate and vagina were similarly mobilized posterior through the sagittal incision of the perineal body. No patient underwent bladder neck plasty. RESULTS: At 9 months to 4 years of followup cosmesis was good in 12 patients, while 1 required secondary glanular urethroplasty. There was mild pyelectasis in 3 cases but no severe hydronephrosis and no renal function deterioration. Pyelonephritis developed in 6 patients (46%). Cystography at 1 year showed that bladder capacity was 35 to 80 and 65 to 120 cc in exstrophy and epispadias cases, respectively. There was cyclic voiding with 30 to 90-minute dry intervals in 7 patients (54%), of whom 5 had exstrophy and 2 had epispadias. Daytime voiding control with a 2 to 3-hour voiding interval was achieved in 1 female with exstrophy and 2 patients with epispadias (23%). Incontinence was present in 2 patients with previous exstrophy closure and 1 with cloacal exstrophy (23%). CONCLUSIONS: Early restoration of a physiological vesicourethral balance of coordinated activity is feasible for the progressive achievement of continence in patients with the exstrophy-epispadias complex. Sagittal splitting of the perineal tissue with identification of the muscle components as well as midline reassembly of the periurethral striated muscular complex helps to reconfigure the pelvic anatomy in a more normal fashion and allows better restoration of coordinated vesicourethral activity.

Bladder Exstrophy↗

PubMed2000

[Prenatal diagnois of uropathies. Ten case reports].

UNLABELLED: Obstructive uropathies are a frequent cause of child renal failure. The prenatal diagnosis of such abnomalities should lead to appropriate management. OBJECTIVE: To evaluate the interest of ultrasonographic prenatal diagnosis of urinary tract malformations. PATIENTS AND METHODS: Retrospective study of 10 cases of urinary tract malformations diagnosed in utero. The mean age of the patients was 34.9 years and the mean gestational age at the prenatal diagnosis was 28.2 weeks of amenorrhea. The abnormalities diagnosed were: 2 cases of ureteropelvic junction obstruction, 3 cases of posterior urethral valves and 5 cases of mild hydronephrosis. The fetal karyotype, realised in 8 cases, was normal. In one case the urinary abnormalities was part of a multiple malformation syndrome and led to the termination of the pregnancy. 7 cases had a post-natal follow-up: 5 had a surgical management and two new borns had transient pyelectasies. The prediction of postnatal renal function is made by ultrasonographic appearance of the kidney and the amniotic fluid. Although the analysis of fetal urine for the assessment of renal function leads to conflicting results, it seems that sodium and beta-2 microglobulin urinary rates provide useful informations.

Adult↗

PubMed2000

[Diagnostic approach to newborns with prenatal diagnosis of hydronephrosis].

Prenatal ultrasound examination has radically changed physician's attitude toward hydronephrosis. Fetal hydronephrosis must be searched out and looked at with a wider vision than it was done in the past. A mechanical obstruction must be suspected indeed, but often we have to face a functional dilatation, caused by immaturity, which can clear up in uterus or which will simply require a careful follow-up during the post natal period. Vesicoureteral reflux may count for 10 to 33% of urinary tract dilatations. There are no pathological limit values clearly stated in literature: a pyelectasis can only be the expression of a normal anatomical variability, without features of disease. In creating a diagnostic protocol for the newborn with prenatal diagnosis of hydronephrosis, all these possibilities must be accounted for the risk of focusing on mechanical obstruction is to overestimate and over-treat children that will never obtain any benefit from a surgical procedure.

Algorithms↗

PubMed2001

Prenatal sonographic markers of trisomy 21.

OBJECTIVE: To describe the sonographic characteristics of fetuses with trisomy 21. DESIGN: A prospective descriptive analysis. SETTING: Department of Obstetrics and Gynecology, Faculty of Medicine, Maharaj Nakorn Chiang Mai Hospital, Chiang Mai University. SUBJECTS: Pregnancies at risk of trisomy 21 between 14-27 weeks' gestation. RESULTS: Thirty-six fetuses with subsequently proven trisomy 21 were prenatally evaluated by ultrasound in the second trimester. The main indications for detailed ultrasound examinations were advanced maternal age and abnormal findings on routine ultrasound. All of them had chromosome analysis by amniocentesis or cordocentesis. Nineteen (52.78%) had one or more abnormal findings. The common sonographic findings included thickened nuchal fold (33.33%), short femur (19.44%), and mild pyelectasis (22.22%). The other uncommon abnormalities included major anomalies (cardiac malformations, ventriculomegaly, duodenal atresia, esophageal atresia), hyperechoic bowel, echogenic intracardiac foci, abnormalities of extremities. In this study, rare minor markers but more specific markers including sandal gap, clinodactyly and mid-phalanx hypoplasia of the fifth finger were demonstrated. CONCLUSION: About half of the fetuses with trisomy 21 had abnormal sonographic findings in the second trimester. The most common marker was thickened nuchal fold. Although prenatal ultrasound can not permit a definite diagnosis of trisomy 21, about half of them have sonographic markers, warranting cytogenetic testing.

Abnormalities, Multiple↗

PubMed2002

Correlation of ultrasound findings and biochemical markers in the second trimester of pregnancy in fetuses with trisomy 21.

OBJECTIVE: The aim of the present study was to assess possible correlations between ultrasound findings and maternal serum biochemical ('triple test') markers among fetuses with trisomy 21 in the second trimester of pregnancy. METHODS: The study was a retrospective cohort study of 72 pregnancies affected by trisomy 21 who had a second trimester ultrasound and biochemical screen performed at a single center between 1990 and 1999. The biochemical screen consisted of alpha-fetoprotein (AFP), total beta human chorionic gonadotrophin (hCG) and estriol (uE(3)). Marker levels were expressed in multiples of the median (MoM). The ultrasound findings assessed were major structural anomalies, short humerus length, short femur length, increased nuchal fold thickness (NF), hyperechoic bowel, echogenic intracardiac focus (EIF), ventriculomegaly, choroid plexus cysts and renal pyelectasis. RESULTS: Second trimester maternal serum biochemical markers and ultrasound findings appeared to be largely independent of each other. However, some significant correlations were observed. Estriol was significantly lower when a fetal cystic hygroma was detected on ultrasound compared to those with no cystic hygroma (0.40 vs. 0.70 MoM, p<0.05). The median hCG level was significantly lower in those pregnancies with a normal second trimester fetal ultrasound compared to those with positive ultrasound findings (2.07 vs. 2.87 MoM, p<0.05). Median hCG levels were also significantly higher in those cases with NF> or =5 mm as compared to those with NF<5 mm (2.99 vs. 2.49 MoM, p<0.05). This difference persisted after exclusion of the five cases with cystic hygromas (2.99 vs. 2.49 MoM, p<0.05). A significant positive correlation was observed between log(10) hCG and log(10) NF MoM (Spearman's rho=0.252, p<0.05). NF was significantly greater among fetuses with an identifiable cardiac defect compared with those without a detectable cardiac defect (median of 7.0 mm vs. 3.8 mm, p<0.01). This difference persisted when expressed as multiples of the median (2.8 vs. 1.3 MoM, p<0.01). CONCLUSION: Second trimester ultrasound and biochemical markers are largely independent in fetuses with trisomy 21, however significant correlations between the two were observed in the present series. These may be important in screening protocols that combine second trimester ultrasound and biochemical markers.

Chorionic Gonadotropin↗

PubMed2002

The role of ultrasound in screening for fetal Down syndrome.

Antenatal Down syndrome screening begins with maternal age-specific risks. It also may incorporate the maternal serum screen and antenatal ultrasound. These complementary tests improve the efficacy of maternal age in Down syndrome screening. Ultrasound enhances antenatal Down syndrome screening by 1) precise dating of a pregnancy for the interpretation of serum screen results; 2) detecting major structural anomalies that occur in approximately 20% of all second-trimester Down syndrome fetuses; 3) detecting minor markers, such as nuchal fold thickness, pyelectasis, echogenic bowel, short long bones, sandal gap, and hypoplasia of the mid-phalanx of the fifth digit, which are more commonly seen in second-trimester fetuses with Down syndrome; and 4) acting in combination with serum screening in the first trimester using fetal nuchal translucency as the marker. Ultrasound enhances the efficacy of either age or age and the serum screen for the antenatal detection of Down syndrome.

Adult↗

PubMed2002

[Urolithiasis induced by combined ACTH and zonisamide treatment in a patient with startle induced epilepsy].

A 5-year-old boy had periodic spasms and startle-induced drop attacks. Zonisamide (ZNS) was partially effective for the former seizures, and propranolol for the latter. An add-on therapy with ACTH resulted in a transient disappearance of seizures and an improvement of EEG. However, the patient developed urolithiasis with resultant hematuria and pyelectasis during ACTH therapy. ZNS can induce urolithiasis by increasing urinary pH and calcium (Ca) excretion, and ACTH may facilitate this rare adverse effect of ZNS by further increasing the urinary Ca. Hydrochlorothiazide could resolve the urolithiasis by decreasing the urinary Ca excretion.

Adrenocorticotropic Hormone↗

PubMed2003

[Management of isolated fetal dilatations of the kidney pelvis].

The most common sonographic abnormality found in the fetal urinary tract is collecting system dilatation, accounting for 4.5% of examinations. Numerous renal pelvis measurements and various gestational age cut-off points have been recommended in the assessment of obstructive fetal uropathy. As a result, renal pelvic measurements are not well standardized. The first measurements of the renal pelvis diameter in the 80's suggested that a threshold value of 10 mm was significant in order to distinguish between physiologic and pathologic dilatations. The routine use of prenatal ultrasound and the improving resolution of ultrasound equipment allow the detection of smaller dilatations. The third-trimester threshold value for the antero-posterior renal pelvis diameter of 7 mm is the best ultrasound criterion to predict postnatal renal abnormalities with a positive predictive value of 69%. A renal pelvis dilatation during the second trimester of pregnancy can be used as a warning sign for urinary tract malformation, and should not be ignored even if there is no further progression in the third trimester, because this finding may reveal a significant urologic abnormality in 12% of cases. Isolated pyelectasis can signal the presence of vesico-ureteral reflux. Normal appearing urinary tract on 2 successive neonatal ultrasound rarely coexist with significant abnormal findings on voiding cysto-urethrography. Therefore, in such cases, invasive investigations are not justified.

Decision Trees↗

PubMed2003

[Pentalogy of Cantrell: first trimester prenatal diagnosis and association with multicistic dysplastic kidney].

Pentalogy of Cantrell is a rare congenital anomaly consisting of the following features: 1) midline supraumbilical abdominal wall defects; 2) deficiency of the anterior diaphragm; 3) defects in the diaphragmatic pericardium; 4) defects of the lower sternum; 5) congenital intracardiac defects. We report 3 cases of pentalogy of Cantrell diagnosed respectively at 13, 18 and 24 weeks of gestation. In case 1 Cantrell's pentalogy was diagnosed during the 1(st) trimester. Case 2 revealed the coexistence of cystic hygroma. Case 3 showed an association with dysplastic left kidney and mild pyelectasis of the right kidney. Our results confirm the possibility of an early detection of Cantrell's pentalogy and reveal the possibility of associations with other pathological findings.

Abdominal Wall↗

PubMed2005

[Comparison between pre- and postnatal echographic screening of malformative uropathies].

AIM: The aim of this study has been to compare the validity of postnatal echographic screening in respect of prenatal echography in early diagnosis of malformative uropathies (MU). METHODS: In 6578 infants, who have been submitted to fetal echography, and to a postnatal screening of MU in our Neonatal Service of Echography (University of Messina), we have compared the diagnostic agreement of prenatal with postnatal echography. RESULTS: Our comparison demonstrates that, in respect of postnatal screening, only 35.71% of pyelectasies and 73.17% of hydronephrosis have been diagnosed by fetal echography, and, in particular, only 18.75% of no-dilated MU. CONCLUSIONS: These data confirm that, in our country, the postnatal screening of MU has still significance and suggest that, before excluding this screening, it is necessary to verify everywhere the validity of fetal echography.

Female↗

PubMed2006

Ultrasonographic soft markers of aneuploidy in second trimester: are we lost?

Chromosomal abnormalities occur in 0.1% to 0.2% of live births, and the most common clinically significant aneuploidy among live-born infants is Down syndrome (trisomy 21). Other sonographically detectable aneuploidies include trisomy 13, 18, monosomy X, and triploidy. Second-trimester ultrasound scan detects 2 types of sonographic markers suggestive of aneuploidy. Markers for major fetal structural abnormalities comprise the first type; the second type of markers are known as "soft markers" of aneuploidy. These latter markers are nonspecific, often transient, and can be readily detected during the second-trimester ultrasound. The most commonly studied soft markers of aneuploidy include a thickened nuchal fold, rhizomelic limb shortening, mild fetal pyelectasis, echogenic bowel, and echogenic intracardiac focus and choroid plexus cyst. There is a great deal of interest in the ultrasound detection of aneuploidy, as evidenced by the large number of publications in the literature on this topic. Unfortunately, studies evaluating the significance of the soft markers of aneuploidy vary widely and show contradictory results. In this article, we review the most common ultrasonographic soft markers used to screen aneuploidy and discuss ultrasonographic technique and measurement criteria for the detection of soft markers. We also review the clinical relevance of soft markers to aneuploidy risk assessment and evidence-based strategies for the management of affected pregnancies with each of these markers in light of current literature.

Aneuploidy↗

PubMed1990

Ureteropelvic junction obstruction in utero and ex utero.

Seventy cases of ureteropelvic junction obstruction, bilateral or unilateral, were followed prospectively throughout gestation and postnatally for an average of 2.3 years. Cases of ureteropelvic junction obstruction with a renal pelvis dilated less than 1 cm uniformly did well; those with a pyelectasis more than 2 cm, both bilateral and unilateral, had a favorable outcome in approximately three-quarters. Surprisingly, pelvis dilatation between 1-2 cm had a better outcome if bilateral than if unilateral.

Female↗

PubMedDate not supplied

[Urologic consequences of severe genital prolapse].

From three observations, the authors analyse the ureteric dilatation and genital prolapse association from an anatomo-clinic point of view. The bladder-base displacement situated in the vaginal prolapse and the resulting very sharp angle formed by the terminal ureter portion are enough to explain the pathogenesis of the observed urinary lesions. The repercussions of advanced prolapses upon upper urinary tracts are rarely mentioned in literature. The three reported clinic cases are absolutely demonstrative: for every one, the major uretero pyelectasis is associated with a progressive renal insufficiency. Authors insist upon urologic symptoms quick regression after a prolapse cure. This correction is made either by putting a pessary, either by a surgical cure unless the risk should be too important for the patient.

Aged↗

PubMed1977

[Incidence of pyelectases and their surgical correction].

Among 1611 urological patients 90 (5.4%) displayed pyelectasis. In almost one-third of the cases dilatation was caused by the presence of a calculus In 33 cases plastic surgery mainly of the Anderson-type was applied. 78% of the operations was successful. The incidence of nephrectomy was higher in the group in which complaints were of more than two years standing (10 out of 33) than in the group where treatment was started less than two years after the appearance of complaints (7 out of 41). In 26 cases dilatation was caused by occlusion by a calculus; in one half of these cases, nephrectomy was the only operation which could be performed.

Dilatation, Pathologic↗

PubMed1977

[Urologic involvement caused by pancreatic cyst].

Pancreas-cysts seldom occur in surgical practice and rarely cause urological complications. The history of a patient is presented in whom dislocation of the kidney and compression of the ureter together with pyelectasis have been observed. Operation revealed that the urological symptoms were elicited by a cyst on the pancreas. Attention is drawn to this rare disease.

Diagnosis, Differential↗

PubMed1994

Prenatal sonographic findings in trisomy 13, 18, 21 and 22. A review of 46 cases.

A study of 46 patients with trisomic fetuses was performed to determine if there are one or more second-trimester ultrasonic findings predictive of aneuploidy. Videotapes of ultrasonography performed prior to amniocentesis on the 46 fetuses with autosomal trisomy and from a control group of 50 chromosomally normal fetuses were reviewed without knowledge of the karyotype. Fetuses with autosomal trisomies had short long bones, especially femurs, as well as high biparietal diameter/femur length ratios. In addition, a nuchal thickness of > 5 mm, abnormal heart anatomy, slight pyelectasis, increased bowel echogenicity and/or abnormal flexion of the hands were all predictive of autosomal trisomies.

Adult↗

PubMed1996

[Prognosis in gynecologic diseases].

Besides early diagnosis, the prognosis of gynecological disease is dependent on the state of the urinary tract. Mainly tumours and site anomalies of the female genital system as well as endometriosis and chronic-inflammatory masses of the appendages lead to malfunction of urinary bladder, ureter and kidneys. Ureteric changes are seen in 3 to 55% of genital tumours and in 20 to 40% of cases of endometriosis. Ureteric and kidney dysfunction can occur even more often in longstanding site anomalies. Typical renal complications of gynecological diseases are pyelonephritis, pyelectasy and hydronephrosis. Their frequency lies between 8 and 40% depending on kind and duration of the underlying disease. The figures shown, indicate that renal and ureteric changes sometimes are more serious than the genital disease. Improvement of prognosis can only be achieved by early recognition of the underlying gynecological disease together with the timely proof of renal and ureteric dysfunction.

Cause of Death↗

PubMed1996

[Ultrasonic screening of neonates].

Till june of 1990 all newborns borned in the authors' hospital were examined with ultrasound before discharge. 7582 newborns were screened during 5 years. 120 urinary abnormalities were found (1.58%). Eleven of 26 hydronephrosis were operated and one of the two multicystic kidneys. 6 unilateral agenesis of kidney, 12 unilateral hypoplasies and 30 duplex kidneys were found. 2 of the laters had hydronephrosis, too. The form of position of 37 kidneys were abnormal. 463 newborns had pyelectasy (6.1%). All were recovered spontaneously but one of them had a gross vesico-ureteric reflux. 31 cerebral malformations were found, one of them was a progressive hydrocephalus. Two of the three babies with cerebral arterio-venous malformation died because of circulatory insufficiency. There were 38 abdominal malformations: 3 hepatic and 7 splenic cysts, 3 bile stones and one neuroblastoma were the most important. 86 delivery injuries were found: 60 adrenal apoplexies, 2 hepatic ruptures and 24 intracranial hemorrhages. All healed. The ultrasound screening in the 19-20th weeks of pregnancy showed only 24% of serious renal and cerebral malformations. The authors think important to screen newborns with ultrasound.

Congenital Abnormalities↗