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.

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- Anomalous coronary artery disease is more common among patients with Tetralogy of Fallot (ToF).Methods- In order to determirie the coronary ANOMALIES in patients with ToF, 135 patients (80 males) with ToF, 9 months to 40 years of age vere studied over 7 years (1995-2002) at Modarres Hospitalin Tehran, Iran.Results-Eight out of the 135 patients (4 males) with Tof were ASSOCIATED with a surgically- relevant coronary artery anomaly (5.9%). The anomalous coronary arteries consisted of single ostium of the coronary artery (n =5), the left anterior descending coronary artery originating from the right coronary artery (n= 2) or single righf coronary artery (n = 2) and the right coronary artery arising from the left coronary artery (n = 1). Surgical technique employed in three ToF patients was transverse incision on the right ventricle, and commissurotomy via the pulmonary artery was the technique used for three other patients. In another patient, a composite graft between the right ventricle outflow tract and main pulmonary artery was inserted. In the remaining four patients with single ostium coronaries, routine repairs were done.Conclusions-Anomalous coronary artery disease is more common among patients with ToF. Bearing these ANOMALIES in mind during primary repair could decrease the risk of surgery in patients with ToF

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.

Objective: The associations between imperforate anus and spinal and vertebral abnormalities and neurologic deficits are well recognized; these neurologic deficits have been considered static rather than progressive. However, recent experience indicates that some patients may develop progressive neurologic problems due to spinal cord lesions that are amenable to neurosurgical correction.Materials & Methods: The medical records of 105 patients with imperforate anus, operated on by us, were retrospectively reviewed from 1996 to 2005. Patient's sex, anorectal type lesion and vertebral or spinal ANOMALIES were determined by ultrasound, excretory urography, voiding cystouretherography (VCUG) and lumbosacral x-ray.Results: A hundred and five cases, consisting 48 (45.7%) boys and 57 (54.3%) girls, with anorectal malformations were studied; 70 patients were in high and intermediate type level, and totally 25 patients (35.7%) with spinal and vertebral ANOMALIES were found in this group.Conclusion: All patients with anorectal malformations (ARM) should be investigated for spinal and vertebral ANOMALIES to improve treatment outcomes in ARM.

Background: Congenital ANOMALIES are a physical impairment that occurring to baby at birth. About 10% of ANOMALIES are caused by teratogenic effect, including chemicals, viruses, physical agents, and medications. Objectives: This study was conducted to determine the risk factors of congenital ANOMALIES in newborns. Methods: 332 infants with congenital ANOMALIES (case group) and 332 healthy infants (control group) were compared in this casecontrol study, which was conducted in Akbar AbadiHospital from April 2016 to April 2017, oninfantswhoare diagnosed with congenital ANOMALIES, based on a first pediatric examination. The data obtained from these infants were analyzed, based on questionnaires and clinical records. Results: The adjusted odds ratio (OR) estimate of congenital ANOMALIES were 1. 045 for increasing maternal age, 2. 47 for consanguineous parents, 4. 42 for positive maternal disease (hypertension, Diabetes, hyperthyroidism and hypothyroidism) compared to negative maternal disease, 1. 92 for cesarean section compared to natural vaginal delivery, 3. 02 for positive history of abortion compared to negative history of abortion, 1. 136 for father’ s age, 2. 47 for smoking mothers compared to non-smoking mothers, 3. 27 for mothers with the history of having child with abnormality compared to mothers who did not have child with abnormality, and 0. 91for gestational age. Conclusions: maternal disease, the history of having child with abnormality, and the history of abortion were the most effective factors in ANOMALIES. In the next step, the consanguineous parents, smoking, the type of delivery, and father’ s age were important risk factors. Finally, maternal age and gestational age had significant effect on anomaly.

Introduction & Objective: Congenital scoliosis is among the most challenging deformities for treatment and even more is the presence of underlying cord ANOMALIES. The result will be catastrophic if we can not predict underlying cord ANOMALIES prior to surgery. The goal of this study is to find the clues to this puzzle.Materials & Methods: For 381 registered patients with congenital scoliosis, physical exam, plane xray, and intra-canal imaging (MRI or myelography) were done to assess the possible underlying ANOMALIES.Then correlation of cord anomaly with other ANOMALIES in other part of body was considered in SPSS software and Paired t-test.Results: Cord ANOMALIES was seen in 83 patients (21.8%) and neurologic deficit found in physical exam in 26 patients (6.8%). Abnormal neurologic findings correlated with cord anomaly (P-value = 0.0001). Skin mark (hair patch, dimple, nevi, etc) had positive correlation with cord anomaly (P-value = 0.001). Type III congenital scoliosis had positive correlation with cord anomaly (P-value = 0.01).Conclusions: It is suggested to be careful with neurologic deficit in physical exam, skin mark and type III congenital scoliosis when preparing the patients for surgical treatment of scoliosis. We should remember that normal physical examination does not rule out cord anomaly.

A 22-year-old man was admitted with chief complaint of dyspnea on exertion. The patient had a history of operation 10 years ago for coarctation of the aorta. Transthoracic echocardiography showed bicuspid aortic valve, aortic regurgitation, left ventricular (LV) enlargement with ejection fraction of 45%, and a deeply trabeculated LV with deep intertrabecular recesses communicating with left ventricular cavity as demonstrated by color Doppler flow, the characteristic of the left ventricular noncompaction. Medical therapy and aortic valve replacement was performed. In the follow up, he was free of symptoms 12 months later.