Federica Lena1,2, Chiara Pellegrino3, Antonio Maria Zaccara1, Maria Luisa Capitanucci1, Giacomo Esposito4, Barbara Daniela Iacobelli5, Daniela Longo6, Tamara Caldaro7, Diletta Bruno8, Francesca Bevilacqua9, Francesca Santato9, Giulia Lucignani6, Carlo Efisio Marras4, Enrico Castelli8, Pietro Bagolan5,10, Giovanni Mosiello1. 1. Division of Neuro-Urology, Bambino Gesu' Children's Hospital, IRCCS, Piazza di Sant'Onofrio 4, 00165, Rome, Italy. 2. University of Genoa, DINOGMI, Largo Paolo Daneo 3, 16132, Genoa, Italy. 3. Division of Neuro-Urology, Bambino Gesu' Children's Hospital, IRCCS, Piazza di Sant'Onofrio 4, 00165, Rome, Italy. chiara.pellegrino@opbg.net. 4. Department of Neuroscience, Neurosurgery Unit, Bambino Gesu' Children's Hospital, IRCCS, Piazza di Sant'Onofrio 4, 00165, Rome, Italy. 5. Medical and Surgical Department of the Fetus-Newborn-Infant, Newborn Surgery Unit, Bambino Gesu' Children's Hospital IRCCS, Piazza di Sant'Onofrio 4, 00165, Rome, Italy. 6. Imaging Department, Neuroradiology Unit, Bambino Gesu' Children's Hospital, IRCCS, Piazza di Sant'Onofrio 4, 00165, Rome, Italy. 7. Division of Digestive Surgery and Endoscopy, Bambino Gesu' Children's Hospital, IRCCS, Piazza di Sant'Onofrio 4, 00165, Rome, Italy. 8. Department of Neurorehabilitation, Bambino Gesu' Children's Hospital, IRCCS, Piazza di Sant'Onofrio 4, 00165, Rome, Italy. 9. Department of Neuroscience, Unit of Clinical Psychology, Bambino Gesu' Children's Hospital, IRCCS, Piazza di Sant'Onofrio 4, 00165, Rome, Italy. 10. Department of Systems Medicine, University of Rome "Tor Vergata", Piazza di Sant'Onofrio 4, 00165, Rome, Italy.
Abstract
PURPOSE: Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported. AIM: To share our experience and to discuss the management of ARM-UD-OSD association. METHODS: We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UD was detected during screening for associated malformation. RESULTS: All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms' worsening, 2 children had detethering surgery. At a mean follow-up of 9.5 years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy). CONCLUSIONS: UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.
PURPOSE: Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported. AIM: To share our experience and to discuss the management of ARM-UD-OSD association. METHODS: We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UD was detected during screening for associated malformation. RESULTS: All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms' worsening, 2 children had detethering surgery. At a mean follow-up of 9.5 years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy). CONCLUSIONS: UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.
Authors: Giorgia Totonelli; Raffaella Messina; Francesco Morini; Giovanni Mosiello; Paolo Palma; Marianna Scuglia; Barbara D Iacobelli; Pietro Bagolan Journal: Pediatr Surg Int Date: 2017-06-10 Impact factor: 1.827
Authors: George Haleblian; David Kraklau; Duncan Wilcox; Patrick Duffy; Philip Ransley; Imran Mushtaq Journal: BJU Int Date: 2006-03 Impact factor: 5.588