New advances in the pathophysiologic and radiologic basis of the exstrophy spectrum.
The exstrophy-epispadias complex is a rare spectrum of anomalies affecting the genitourinary system, anterior abdominal wall, and pelvis. Recent advances in the repair of classic bladder exstrophy (CBE) and cloacal exstrophy (CE) have resulted in significant changes in outcomes of surgical management (including higher continence rate, fewer surgical complications, and better cosmesis) and health-related quality of life in these patients. These noteworthy changes resulted from advances in the pathophysiological and genetic backgrounds of this disease and better radiologic assessment of the three-dimensional anatomy of the bony pelvis and its musculature. A PubMed search was performed with the keyword exstrophy. The resulting literature pertaining to genetics, stem cells, imaging, tissue engineering, epidemiology, and endocrinology was reviewed. The following represents an overview of the advances in basic science understanding and imaging of the exstrophy-epispadias spectrum and discusses their possible and future effects on the management of CBE and CE.
Robert D Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institute, the Johns Hopkins University School of Medicine, 1800 Orleans St. Suite 7304, Baltimore, MD 21287, USA. Electronic address: alitourchi@pedurol.com.
Ali
A
Tourchi
Robert D Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institute, the Johns Hopkins University School of Medicine, 1800 Orleans St. Suite 7304, Baltimore, MD 21287, USA.
Brian M
BM
Inouye
Robert D Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institute, the Johns Hopkins University School of Medicine, 1800 Orleans St. Suite 7304, Baltimore, MD 21287, USA.
Heather N
HN
Di Carlo
Robert D Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institute, the Johns Hopkins University School of Medicine, 1800 Orleans St. Suite 7304, Baltimore, MD 21287, USA.
Ezekiel
E
Young
Robert D Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institute, the Johns Hopkins University School of Medicine, 1800 Orleans St. Suite 7304, Baltimore, MD 21287, USA.
Joan
J
Ko
Robert D Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institute, the Johns Hopkins University School of Medicine, 1800 Orleans St. Suite 7304, Baltimore, MD 21287, USA. Electronic address: jgearha2@jhmi.edu.
John P
JP
Gearhart
D001746:Bladder Exstrophy / Q000000981:diagnostic imaging* / Q000503:physiopathology* / Q000601:surgery*; D005260:Female; D006801:Humans; D021621:Imaging, Three-Dimensional / Q000379:methods; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D011247:Pregnancy; D011296:Prenatal Diagnosis / Q000379:methods*; D058996:Quality Improvement; D011788:Quality of Life; D011859:Radiography; D019651:Plastic Surgery Procedures / Q000379:methods; D016896:Treatment Outcome; D013520:Urologic Surgical Procedures / Q000379:methods
Bladder exstrophy; Prenatal diagnosis; Radiology