Anatomic Findings Associated with Epispadias in Boys: Implications for Surgical Management and Urinary Continence
Marc Cendron, M.D., Patricia S. Cho, M.D., Ilina Rosoklija, M.P.H., Melanie Pennison, M.P.H., David A. Diamond, M.D., Joseph G. Borer, M.D..
Boston Children's Hospital, Boston, MA, USA.
Introduction: Preoperative physical examination of boys with epispadias allows for classification of the epispadias level as glanular (GE), penile (PE) or penopubic (PPE) and for precise delineation of the anatomic anomalies. While penile anomalies in epispadias have been well described, associated genitourinary abnormalities have not been systematically studied. Thus, the frequency of vesico-ureteral reflux (VUR), bladder neck (BN) abnormality and abnormal pubic diastasis (PD) in this population are not well characterized. The aim of this study is to evaluate the incidence of these findings relative to epispadias level and determine the impact on urinary continence.
Methods: A case series of male epispadias patients treated surgically between 1994 and 2011 was compiled. Patients with bladder exstrophy were excluded. Twenty-six patients were identified and grouped by anatomic classification: GE (n=4), PE (n=8), and PPE (n=14). Preoperative impression of BN appearance (normal/coapted vs. abnormal/patulous) was obtained from voiding cystourethrogram (VCUG) or cystourethroscopy. Normal vs. widened (intersymphyseal distance >2cm) PD was determined from plain abdominal radiograph of the abdomen or from VCUG scout film. Presence and grade of VUR was diagnosed on VCUG. Data regarding surgical techniques including BN procedure (BNP), iliac osteotomies (IO), and ureteroneocystostomies (UNC) was recorded. The incidence of pyelonephritis in these patients was recorded, as was urinary continence status as defined by ICCS criteria.
Results: Median age at repair was 10.9 months (range 4.21-138.5 months). Abnormal BN was noted in 11 out 24 (46%; 3 PE and 8 PPE) with 4 patients (all with PPE) undergoing BN surgery. By VCUG, VUR was detected in 9 patients (35%; 2 GE, 3 PE, and 4 PPE) and was grade 3 or less. Of these patients, 5 underwent UNC, with 4 (all with PPE) being perform in conjunction with BNP. Three patients (8%) experienced one episode of pyelonephritis (2 with VUR, 1 with no VUR). Iliac osteotomies were not performed in any of the patients. The majority of patients were found to be continent in follow-up, with rates of continence of 64% PPE, 63% PE, and 75% GE. Suprisingly, continence did not correlate closely with abnormal BN appearance or PD.
Conclusion: This study demonstrates that associated anatomic abnormalities are present in a majority of male patients with epispadias. While VUR may not increase the disease burden significantly (as demonstrated by a low rate of pyelonephritis), the presence of an abnormal BN and pubic diastasis, especially in the more severe forms, may predispose to incontinence and call for a more aggressive surgical approach. This study highlights the need for a thorough preoperative evaluation and exemplifies the spectrum of anatomic and functional abnormalities seen with these patients.
Table 1. Summary of Epispadias Patients
|Epispadias Type||n||Abnormal BN||Abnormal PD||VUR||Continence|
|Glanular||4||0||1 (25%)||2 (50%)||3 (75%)|
|Penile||8||3 (38%)||3 (38%)||3 (38%)||5 (63%)|
|Penopubic||14||8 (57%)||12 (86%)||4 (29%)||9 (64%)|
|Total||26||11 (42%)||16 (62%)||9 (35%)||17 (65%)|
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