Journal of Perinatology (2007) 27, 193195 r 2007 Nature Publishing Group All rights reserved.

0743-8346/07 $30
www.nature.com/jp

IMAGING CASE BOOK

Superior vesical ssure: variant classical bladder exstrophy

TE Herman1, MJ Siegel1 and PF Austin2
Department of Radiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis Childrens Hospital, St Louis, MO, USA and 2Department of Surgery, Division of Urology, Washington University School of Medicine, St Louis Childrens Hospital, St Louis, MO, USA
Journal of Perinatology (2007) 27, 193195. doi:10.1038/sj.jp.7211656
1

urethral catheter (Figure 3). The patient was then taken to the operating room. Denouement and discussion This neonate has an uncommon variant of classic bladder exstrophy called superior vesical ssure. The cystogram demonstrated a small bilobed urinary bladder with a normal urethra. Bilateral grade one vesicoureteral reux was also observed. There was discrete connection or ssure from the bladder to the mucosa on the infra-umbilical anterior abdominal wall (arrows). At the time of surgical repair (Figure 4), this anterior mucosa was closed over the bladder, the bladder returned to the pelvis and the anterior abdominal wall closed. Classical bladder exstrophy is an uncommon anomaly in which the bladder hemitrigones are on the anterior abdominal wall below the umbilicus and the pubic bones are separated with diastasis of

Case presentation A 3177 g female infant was born at 38 weeks gestation to a 28-year-old mother with an uncomplicated pregnancy except for the development of maternal cholecystectomy just before delivery. At birth the child was noted to have an obvious urogenital abnormality. Red, moist, 2 cm diameter mucosa was present on the anterior abdominal wall below the umbilicus through which urine was seen to ow (Figure 1). This mucosa was immediately covered with clean plastic wrap to prevent damage to the exposed bladder mucosa. The patient had a single clitoris and a urethral meatus, which allowed passage of a 5 French feeding tube. The symphysis was palpably widened, which was conrmed by plain roentgenogram (Figure 2), demonstrating a 3 cm separation of the pubic bones. The patient then had a cystogram performed by instilling contrast through a

Figure 1 Photograph of the pelvis of the infant demonstrating the exposed mucosa below the umbilicus.
Correspondence: Dr T Herman, Department of Radiology, Mallinckrodt Institute of Radiology, St Louis Childrens Hospital, 510 South Kingshighway Blvd, St Louis, MO 63110, USA. E-mail: hermant@mir.wustl.edu Received 26 October 2006; accepted 9 November 2006

Figure 2 Plain radiograph of the pelvis demonstrating a markedly widened symphysis pubis (arrows).

Superior vesical ssure TE Herman et al

194

Figure 3 (a) Frontal cystogram radiograph demonstrates small bilobbed bladder. (b) Lateral cystogram lm demonstrating ssure (arrows) with ow of contrast onto the anterior abdominal wall. (c) Bilateral grade I vesicoureteral reux is also appreciated (arrows).

Journal of Perinatology

Superior vesical ssure TE Herman et al

195

presence of a normal urethra in superior vesical ssure, the long-term prognosis for continence is very high, unlike patients with classic bladder exstrophy. Superior vesical ssure has been described in both male and female subjects, in association with trisomy 18, omphalocele4 and with duplication of the urethra.5 The bladder mucosa on the anterior abdominal wall may increase the patients risk of adenocarcinoma of the bladder, as is seen in classic exstrophy. Most of the cases of adenocarcinoma of the bladder in exstrophy patients occur between the third and sixth decades of life.6 This patient tolerated closure well and was discharged home on the third postoperative day in excellent condition.
Figure 4 Operative photograph demonstrating closure of the ssure and release of the bladder into the pelvis.

References
1 White P, Lebowitz RL. Exstrophy of the bladder. Radiolog Clin North Am 1977; 15: 93107. 2 McAlister DM, Webb HR, Wheeler PD, Shinault KA, Teague DC, Fish JR et al. Pubic symphyseal width in pediatric patients. J Pediatr Orthop 2005; 205: 725728. 3 Marshall VF, Muecke EC. Variations in exstrophy of the bladder. J Urology 1962; 88: 766796. 4 Siebert JR, Rutledge JC, Kapur RP. Association of cloacal anomalies, caudal duplication and twinning. Pediatr Dev Pathol 2005; 8: 339354. 5 Sharma AK, Sarin YK, Wakhlu A. Superior vesical ssure associated with urethral duplication in a male neonate. J Pediatric Surg 1994; 29: 13991400. 6 deRiese W, Warmbold H. Adenocarcinoma in exstrophy of the bladder. A Case Report and Review of the Literature. Int Urol and Nephrol 1986; 18: 159162.

the rectus muscles.1 The width or separation of the symphysis pubis in a neonate should be less than 9 mm.2 Several variants of classical bladder exstrophy have been described.3 These include pseudoexstrophy in which the symphysis pubis is widened, but there is a normal anterior abdominal wall, bladder and urinary tract. Duplicate exstrophy is a variant in which the pelvic bones are also abnormal, the bladder is normal but there is a small piece of bladder mucosa on the anterior abdominal wall. Superior vesical ssure, as in the case presented here, is a variant in which pelvic deformity occurs, but only the upper bladder is open near the umbilicus. In a large series of classic exstrophy only one superior vesical ssure was found among the 72 patients.1 Because of the

Journal of Perinatology