Reconstructing Forked Corpus Spongiosum in Hypospadias Repair
HypospadiasHypospadias is one of the most common genital malformations in children. The high incidence of hypospadias, which occurs in 1 in 200 to 300 live births, means that it affects a large number of patients. Surgery is the only way to repair hypospadias. Over 400 techniques have been described for hypospadias repair. However, the surgical success rate of hypospadias is still not ideal. Although the surgical success rate of distal hypospadias has reached more than 85%, the complications of proximal hypospadias are still as high as 30-68%. How to improve surgical skills and reduce the postoperative complications is quite a challenge for pediatric urologists. In the preliminary clinical work, the investigators have tried to apply the technique of reconstructing forked corpus spongiosum (FCS) in hypospadias repair with urethral plate preservation. It has been confirmed that this technique was effective in reducing postoperative complications of this type of hypospadias repair. In order to promote the technology of reconstructing FCS, the investigators need to perform this technology in various types of hypospadias and evaluate its true effectiveness. Therefore, the investigators need to design a prospective, randomized, parallel-controlled, single-blind, and superior clinical trial to analyze the efficacy of reconstructing FCS in hypospadias repair. In this study, the investigators will perform one-stage surgical repair on children with primary hypospadias by the same surgeon in Urology Department, Children's Hospital of Fudan University, Shanghai, China. Participants will be random grouped: Routine standardized surgery with reconstructing FCS group and Routine standardized surgery group. All participants will be closely followed up and regularly evaluated after surgery, including postoperative complications, HOSE objective score of cosmetic outcome and voiding function. By collecting all data and conducting statistical analysis, the investigators will evaluate the followings: (1) the correlation between the penile curvature and the development of FCS; (2) the effect of reconstructing FCS on the complications of primary hypospadias repair; (3) the effect of reconstructing FCS on the cosmetic outcome; (4) the influence of reconstructing FCS on postoperative voiding function. Based on this clinical randomized controlled study, the investigators intend to prove the feasibility and effectiveness of the new technology of reconstructing FCS in various types of hypospadias repair. The study will provide a reliable basis for the promotion of this technology for hypospadias repair in order to improve the quality of life for children with hypospadias.
Long-term Assesment of Patients Operated for Hypospadias in Their Childhood : Urinary, Aesthetical,...
HypospadiasThe purpose of this study is to assess the long term urinary, sexual, cosmetic and psycho-social outcomes of adult patients operated for hypospadias during childhood
Biochemical and Phenotypical Aspects of Smith-Lemli-Opitz Syndrome and Related Disorders of Cholesterol...
Smith Lemli Opitz SyndromeCHILD Syndrome2 moreBackground: Smith-Lemli-Opitz Syndrome (SLOS) is a genetic disorder. It can cause birth defects and developmental delays. There is no cure for SLOS or other inherited diseases related to cholesterol production or storage. The data gained in this study may help researchers find ways to measure how well future treatments work. Objective: To learn more about SLOS and related disorders and how these diseases affect participants and relatives. Eligibility: People of any age who have or are suspected to have SLOS or another inherited disease related to cholesterol production or storage. Relatives are also needed. Design: Participants will be screened with a medical record review. Participants will have visits every 6 to 12 months. They will have a physical exam. They will fill out a survey about their medical and behavioral history. They may have an eye exam. They may have a neurodevelopmental assessment. They may have a hearing test. Their outer and middle ears may be examined. Their ability to speak, understand speech, eat, and swallow may be assessed. They may get X-rays while they chew and swallow. Their functional ability and needs for adaptive devices or braces may be assessed. They may have a lumbar puncture. Photographs may be taken of their face and body. Participants who cannot visit the NIH and relatives will have a virtual visit once a year. They will talk about their medical history and symptoms. They give blood, urine, and skin samples at a lab near their home. They will fill out a survey about their medical and behavioral history. Participation will last for several years.
Does Graft Improves the Outcome TIP Repair for Primary Distal Hypospadias?
Penile HypospadiasHypospadias is a common congenital anomalies in male children affecting 1 in 200-300 male births, the penis urethral opening is found ventrally, penile curvature and a lack of foreskin (1). Multiple operations are described , Tubularised incised-plate is the preferred option for distal hypospadias and with trial to extend the operation indication to proximal and redo hypospadias (2). Its simple operative technique made TIP repair gain worldwide acceptance in addition to the low complication rate & good cosmetic outcome (3).It has several complication as stenosis meatus, fistula formation , uretheral stricture and failed repair (4). Objective scoring systems were introduced to allow better judgment and identification of the postoperative results, depending on pre-operative and intra-operative criteria (5). Modifications of the T.I.P operation was done to reduce complication and allow better results by using a graft , Snodgraft vs Snodgrass operation are nearly equal regarding the outcomes (6). To our knowledge there is no definite recommendation.
Hypospadias and Exome: Identification of New Genes for Familial Hypospadias
HypospadiasHypospadias is the second most common malformation of the male genitalia and consists in a congenital hypoplasia of its ventral face. Hypospadias results from abnormal development of the penis that leaves the urethral meatus proximal to its normal glanular position. Meatus position may be located anywhere along the penile shaft, but more severe forms of hypospadias may have a urethral meatus located at the scrotum or perineum. Glandular and penile anterior represents approximately 70% of all the diagnosed cases. The frequency of family reached depend on the severity of hypospadias. The number of children which have hypospadias mutation discovered by classical technique is low. Families are often the cause of the discovery of new genes involved in sexual differentiation because they allow comparison of genomes of related persons, and detect more easily mutations.
Clinical Trial to Assess the Safety and Efficacy of Investigational Product in Patients Due to Hypospadias...
HypospadiasThe purpose of this trial is to assess the safety and efficacy of Holour in patients suffering from hypospadias treatment failure. Holour is intended for urethral replacement and is made from ex vivo expanded autologous oral mucosal cells including stem cells.
Glans Size in Hypospadias Compared to Normal
HypospadiasSurgery Non HypospadiasThe investigator will collect data on penile and glans size by age, weight and ethnicity in both patients undergoing routine urological surgery and hypospadias surgery.
Caudal vs. Pudendal Block in Peds GU
HypospadiasPenile Torsion2 moreThis study will compare two techniques to minimize pain during and after penile surgery in children undergoing certain urologic surgeries. These two approaches include the caudal nerve block and the pudendal nerve block.
Long Term Follow-up for Hypospadias
HypospadiasHypospadias is a frequent malformation. The definition of hypospadias is a ventral tissues hypoplasia, with an abnormal position of the urethral meatus, a chordee of the penis and an open foreskin. It can be associate with other genital malformation; in that case it can be classified in the wider field of the disorder of sex development (DSD). There is a lot of surgical technics for those malformations. In the pediatric surgical department, in Lyon, three technics are the most used because of their reliability and their good outcomes in short and mid term. This is essential for investigator to have a long term evaluation, with a physical exam and paraclinical exams, after the puberty, to assess the outcomes after the changes especially at the puberty.
Genetics of Differences of Sex Development and Hypospadias
Disorders of Sex DevelopmentHypospadiasThis study seeks to identify genetic causes of conditions that affect the gonads and genitals, and to study the impact on families of receiving genetic results.