Spătaru Radu-Iulian1,2, Avino Adelaida3,4, Iozsa Dan-Alexandru1,2, Ivanov Monica2, Serban Dragos5,6, Tomescu Luminiţa Florentina7, Cirstoveanu Cătălin8,9. 1. Discipline of Pediatric Surgery, Faculty of Medicine, "Carol Davila" University of Medicine and Pharmacy, 020021 Bucharest, Romania. 2. Department of Pediatric Surgery, Emergency Clinic Hospital for Children "Maria S. Curie", 41451 Bucharest, Romania. 3. Department of Plastic and Reconstructive Surgery, Clinical Emergency Hospital "Prof. Dr. Agrippa Ionescu", 011356 Bucharest, Romania. 4. Discipline of Plastic and Reconstructive Surgery, Faculty of Medicine, Doctoral School, "Carol Davila" University of Medicine and Pharmacy, 020021 Bucharest, Romania. 5. Discipline of General Surgery, Faculty of Medicine, "Carol Davila" University of Medicine and Pharmacy, 020021 Bucharest, Romania. 6. Department of General Surgery, Emergency University Hospital, 050098 Bucharest, Romania. 7. Department of Interventional Radiology, Clinical Emergency Hospital "Prof. Dr. Agrippa Ionescu", 011356 Bucharest, Romania. 8. Discipline of Pediatrics, Faculty of Medicine, "Carol Davila" University of Medicine and Pharmacy, 020021 Bucharest, Romania. 9. Neonatal Intensive Care Unit, Emergency Clinic Hospital for Children "Maria S. Curie", 41451 Bucharest, Romania.
Abstract
Background and Objectives: Caudal duplication syndrome is a rare association of anatomical anomalies describing duplication of the hindgut, spine, and uro-genital structures, leading to varied clinical presentations. The current literature focuses on case reports which describe the embryological etiology and anatomical spectrum of the condition giving little attention to the surgical preparation, the need for a well-structured follow-up program, or the transition into adult healthcare of these complex patients. No reviews have been published regarding this complex pathology. Materials and Methods: A review of caudal duplication syndrome cases was done to assess the range of the clinical malformations, timing, and types of surgical interventions. Inconsistencies in multidisciplinary care, follow-up, and risk events were described. Results: Hindgut duplication always involved the anorectal region. Anorectal malformations were evenly distributed as unilateral and bilateral. Colon duplication extended from the anal region to the transverse colon or ascending colon in most of the cases and less to terminal. In females, genital duplication was present in all cases. The follow-up period varied between 3 months and 12 years. In all adult females, the motive of presentation was related to pregnancy (complications after successful delivery, fertility evaluation) or late complications (fecalith obstruction of the end-to-side colon anastomosis, repeated UTIs with renal scarring). Conclusions: Complex malformations affecting multiple caudal organs may have a strong impact in many aspects of the long-term quality of life; therefore, patients with caudal duplication syndrome need increased awareness and joined multidisciplinary treatment.
Background and Objectives: Caudal duplication syndrome is a rare association of anatomical anomalies describing duplication of the hindgut, spine, and uro-genital structures, leading to varied clinical presentations. The current literature focuses on case reports which describe the embryological etiology and anatomical spectrum of the condition giving little attention to the surgical preparation, the need for a well-structured follow-up program, or the transition into adult healthcare of these complex patients. No reviews have been published regarding this complex pathology. Materials and Methods: A review of caudal duplication syndrome cases was done to assess the range of the clinical malformations, timing, and types of surgical interventions. Inconsistencies in multidisciplinary care, follow-up, and risk events were described. Results: Hindgut duplication always involved the anorectal region. Anorectal malformations were evenly distributed as unilateral and bilateral. Colon duplication extended from the anal region to the transverse colon or ascending colon in most of the cases and less to terminal. In females, genital duplication was present in all cases. The follow-up period varied between 3 months and 12 years. In all adult females, the motive of presentation was related to pregnancy (complications after successful delivery, fertility evaluation) or late complications (fecalith obstruction of the end-to-side colon anastomosis, repeated UTIs with renal scarring). Conclusions: Complex malformations affecting multiple caudal organs may have a strong impact in many aspects of the long-term quality of life; therefore, patients with caudal duplication syndrome need increased awareness and joined multidisciplinary treatment.
Authors: Y Y Chan; K Jayaprakasan; A Tan; J G Thornton; A Coomarasamy; N J Raine-Fenning Journal: Ultrasound Obstet Gynecol Date: 2011-10 Impact factor: 7.299