Background: Consanguineous marriage is one of the most important etiology of morbidity and anomaly in neonates and children, for which annually thousands of neonates and children sacrifice. This study was performed in Shabihkhani hospital in Kashan to determine the prevalence of CONGENITAL ANOMALIES among consanguineous marriages. Material and methods: All neonates born in Shabihkhani hospital in 2000-01 were included and initial data including sex, gestational age, type of delivery, prenatal care, drug history, special disease in mother, type of anomaly, history of still birth or anomaly in previous pregnancy were recorded. Type of anomaly in the first 24 hours was distinguished for further classification. Paraclinic tests and consultations were also used. Results: Of 3529 neonates, 768 (21/8%) were born of a consanguineous marriage and the remaining 2761 (78.2%) from non-consanguineous marriage. Totally, 109(3.08%) deliveries were led to neonates anomaly, of these 7.03% were in the consanguineous marriage group (72.3% in first-cousin marriage and 27.7% in second-cousin marriage) and 2% in the other group. Conclusion: The prevalence of consanguineous marriage in our study was relatively high and the rate of neonate anomaly of consanguineous marriage was more than what expected. Obviously, in case of consanguineous marriage, the rate of anomaly clearly increased.

Background: Reports on associated intraspinal and other organ ANOMALIES in CONGENITAL scoliosis are variable. We are reporting such findings in one of the orthopaedic referral centers in Tehran.Methods: A series of 381 patients with CONGENITAL scoliosis were evaluated for intraspinal abnormalities with physical examination, radiographs, MRI and/or myelograms. They were also assessed for cardiac urogenital or ANOMALIES in other areas by physical exam, sonography and echocardiography.Results: Cord anomaly was present in 83 (21%) cases, 26 of whom had neurologic abnormality. The third cord syndrome was the most common neurologic problem in 38 patients (45.8%), followed by diastematomyelia in 37 cases. ANOMALIES in other parts of body detectable by physical examination were observed in 155 patients, and detected by help of paraclinical tests in 34 patients. 84 cases had trunk ANOMALIES, 4 genitourinary and 10 heart anomaly, 37 head and neck, 7 maxillofacial, 3 upper and 23 lower extremity, 8 cases of gastrointestinal ANOMALIES were also detected.Conclusion: The ANOMALIES of nervous system associated with CONGENITAL scoliosis are often without clinical manifestations. Cardiac and urogenital ANOMALIES are not very common in CONGENITAL scoliosis.

Mullerian duct ANOMALIES are an uncommon but often treatable cause of infertility.Patients with müllerian duct ANOMALIES are known to have a higher incidence of infertility, repeated first-trimester spontaneous abortions, fetal intrauterine growth retardation, fetal malposition, preterm labor, and retained placenta. The role of imaging is to help detect, diagnose, and distinguish surgically correctable forms of müllerian duct ANOMALIES from inoperable forms (see the images below).In some correctable lesions, the surgical approach is altered based on imaging findings.MRI of the uterus (in benign conditions such as CONGENITAL ANOMALIES or fibroid evaluation) is performed following administration of 1.0 mg IM of glucagon to decrease motion artifacts associated with bowel peristalsis.For the diagnosis of most ANOMALIES, 5 main sequences are sufficient:• Coronal single-shot fast spin-echo (FSE) images of the\ ureters and pelvis provide good localized and survey views of ureters.• Axial T1 spin-echo (without fat-saturation pulse) images.• Sagittal T2 FSE (without fat-saturation pulse) images.Fat signal is useful to delineate pelvic structures.• Oblique long-axis T2 FSE (without fat-saturation pulse) images. This plane is ideal for visualization of the uterine cavity and uterine fundal contour.• Oblique short-axis T2 FSE (without fat-saturation pulse) images are perpendicular to the long axis and sagittal planes (usually oblique axial), providing short-axis (target) views of the uterine cavity useful for visualizing a transverse septum if present.

Introduction: CONGENITAL ANOMALIES (CAs) are a worldwide problem and an important cause of childhood death, chronic illness, and disability. They result from defective embryogenesis or intrinsic abnormalities in the development process and often lead to long-term disability, which may have significant impacts on individuals, families, health-care systems, and societies. The most common, severe CONGENITAL ANOMALIES include heart defects, neural tube defects and the Down syndrome. Although CONGENITAL ANOMALIES may be the result of one or more genetic, infectious, nutritional or environmental factors, it is often difficult to identify the exact causes. Some CONGENITAL ANOMALIES can be prevented,timely vaccination of the mother, adequate intake of folic acid or iodine through fortification of foods or supplementation during pregnancy, and adequate antenatal care are just 3 examples of primary preventive methods. Physicians involved in the provision of health care for children and adolescents, need a basic understanding of how to evaluate and when to refer children and adolescents with CONGENITAL ANOMALIES to the respective subspecialty. Therefore, the aim of this article is to review the incidence, and different patterns of birth defect or CONGENITAL ANOMALIES in several Eastern Mediterranean developing countries. Materials and Methods: In this study, the terms Birth Defects, CONGENITAL ANOMALIES and CONGENITAL Malformations are synonymous and have been used interchangeably throughout this article. Conclusion: This study provides useful information about the extent and range of CONGENITAL malformations diagnosed immediately after birth in infants and highlighted the prevalence and types of CONGENITAL malformations. Maternal vaccination, adequate intake of folic acid or iodine through fortification of main foods or supplements during pregnancy, and adequate prenatal care are just three examples of contraceptive methods.

Background & Objective: CONGENITAL spinal deformities usually present in infancy and make parents  worry about their child future. Because of CONGENITAL nature of these deformities, cord ANOMALIES and other organ abnormalities must be evaluated. This study was done to determine CONGENITAL spinal deformity and associated ANOMALIES.Materials & Methods: This cross sectional study was done on 97 patients who referred to Shafa hospital in Tehran- Iran with CONGENITAL back deformities from October 2005 to January 2007. All patients were assessed with physical examination, spinal radiography, MRI, urinary system sonography and echocardiography.Results: Mean age of patients at presentation was 81 month. 81 patients had CONGENITAL scoliosis and 16 patients had CONGENITAL kyphosis. Mean cobb angle was 52 degrees for CONGENITAL scoliosis and 70 degrees for CONGENITAL kyphosis. 22 patients had neurologic or skin signs that 81% of them had cord abnormalities (p<0.05). Cord ANOMALIES were seen in 36 scoliosis (46.5%) and 2 kyphosis (15%) (p<0.05). Most common cord ANOMALIES were syingomyelia,diastematomyelia and tethered cord. CONGENITAL heart ANOMALIES were seen in 3 patients (4%) and valvular heart disease in 10 patients (13.5%) and kidney ANOMALIES in 7 patients (12%). Only one patient with CONGENITAL heart disease and one patient with kidney anomaly had kyphosis.Conclusion: Regarding to prevalence of CONGENITAL spinial deformity (CSD) in scoliosis and kyphosis patients, all of patients with CSD should screued for determiation of conganital ANOMALIES by MRI, Eco cardiography and kidny sonography.

Background/Purpose: Genitourinary ANOMALIES in patients with imperforate anus are a frequent souree of significant morbidity. Variability of reports on the incidence of associated ANOMALIES with imperforate anus mandates further investigation on this issue.Material and Methods: The case records and imaging studies of 63 patients who underwent reparative surgery for imperforate anus over a 9-year period were retrospectively reviewed.Results: Genitourinary ANOMALIES were seen in 29 patients (16.03%). A voiding cystourethrogram was performed in 36 patients, 11 of whom were shown to have vesicoureteric reflux and 3 of whom required surgical correction. Vesicoureteral reflux was the most prominent urologic anomaly observed. Uretropelvic junction (UPJ) stenosis, hydronephrosis, hypospadiasis, renal agenesis and undescended tests were also common in these patients.Conclusions: Patients with anorectal malformations should be evaluated for urinary tract and spinal problems.

Introduction: In the second part of this article, CONGENITAL urethral ANOMALIES other than posterior urethral valve were reviewed. Materials and Methods: The ANOMALIES considered in the current review were anterior urethral valve, lacuna magna, syringocele, Cobbs collar, duplication of urethra, megalourethra, and prostatic urethral polyps. The literature was extensively reviewed concerning the presentations, diagnosis, different types of treatment modalities, morbidity, mortality, and new concepts for the above disorders.Result: Anterior urethral valves or diverticula are the most prevalent CONGENITAL ANOMALIES of anterior urethra. The lacuna magna is the largest depression in the dorsal aspect of the fossa navicularis. It is demonstrable on a well-performed voiding cystourethrography of the distal urethra. The dilated Cowpers gland duct is the other missed diagnosed anomaly of the urethra in boys. The CONGENITAL narrowing of the bulbar urethra with a variable clinical presentation and obstruction grade and different types of anterior urethral obstruction are the most common presentation of these ANOMALIES. However, other symptoms or signs including, hematuria, bloody spotting on underwear, discomfort or sever pain in the vicinity of the glance, interrupted voiding, infection, bulging of anterior urethra, enuresis, and postvoiding dribbling are the only nonspecific manifestations of these disorders.Conclusion: All of these disorders are demonstrable on a well-performed voiding cystourethrography of the distal urethra. The urologist must be aware about these uncommon CONGENITAL ANOMALIES and the anterior urethra should be carefully evaluated for such ANOMALIES. Diagnosis of these entities is elusive unless the physician is looking for them. Nonspecific symptoms mentioned here besides radiographic findings can be a valuable clue for diagnosis.

Cervical abnormalities may be CONGENITAL or acquired. CONGENITAL ANOMALIES of the cervix are rarely isolated, and more commonly accompany other uterine ANOMALIES. Various imaging tools have been used in the assessment of Müllerian duct ANOMALIES (MDAs). Currently, magnetic resonance imaging (MRI) is the modality of choice for definitive diagnosis and classification of these MDAs. Hysterosalpingography is a basic tool for evaluation of infertility and allows us to detect a spectrum of anatomical malformations of the utero-cervix in the setting of MDAs. It provides good outlines of the uterine cavity and fallopian tubes, as well as the cervical canal and isthmus. However, hysterosalpingograms (HSG) cannot be performed in patients with isolated CONGENITAL maldevelopment (agenesis/disgenesis) of the cervix. This part of pictorial review illustrates the various radiographic appearances of CONGENITAL malformations of the utero-cervix with a brief overview of the embryologic features. Accurate diagnosis of such cases is considered essential for optimal treatment and categorization of each anomaly.

Radiological findings of ten patients with CONGENITAL obstructive ANOMALIES of anterior urethra (eight cases with saccular diverticula, one case with globular dilatation of entire urethra, and one case with globular dilatation of distal urethra) showed that the VCUG and retrograde urethrography were diagnostic in all of patients.

Objectives: The aim of this study was to compare the prevalence of minor physical ANOMALIES in schizophrenic and physically ill patients. Method: This was a case-control study.50 patients with the diagnosis of schizophrenia were com- pared with 50 physically ill patients on the basis of the prevalence of CONGENITAL ANOMALIES. Selected in a three month period, the first group was comprised of patients in two psychiatric hospitals in Tehran. The physically ill patients were selected randomly from a general outpatient center during the same time in Tehran. Collection of data was completed by clinical examination, psychiatric interview, and a questionnaire. Findings: The rate of minor CONGENITAL ANOMALIES in schizophrenic patients was higher than the control group. Total scores were 258 and 143 respectively for the schizophrenic and the control groups; the average number of ANOMALIES was 5.5 for each schizophrenic patient and 2.6 for each participant in the control group. The most ANOMALIES sited were the mouth area (25%) in schizophrenic patients and the feet (25%) in control group. The complete cohesion of auricle to the face was the most frequently identified anomaly in both groups. Except for lax and soft auricle, on the whole, ANOMALIES were more prevalent in schizophrenic patients than non psychotic ones; the commonness of ANOMALIES was more in males than females in both groups. Results: The prevalence of minor physical ANOMALIES in schizophrenic patients was more than physically ill patients.