Pediatr Radiol (1992) 22:50%511

Pediatric Radiology 9 Springer-Verlag 1992

Congenital bladder rupture and urine ascites secondary to a sacrococcygeal teratoma A. C. Zaninovic 1, S. J. Westra 1, T. R. HaW, M. P. Sherman 2, L. Wong 3, M. I. Boechat Departments of 1 Radiological Sciences and 2Pediatrics, UCLA Medical Center, Los Angeles, USA 3Department of Radiological Sciences, Olive View Medical Center, Sylmar, USA Received: 27 May 1992; accepted: 11 June 1992

Abstract. A case of congenital b l a d d e r rupture with urine ascites and bilateral h y d r o n e p h r o s i s s e c o n d a r y to a sacrococcygeal t e r a t o m a obstructing the b l a d d e r outlet is presented. A s no evidence of p u l m o n a r y hypoplasia and renal dysplasia was present, with rapid r e c o v e r y of renal function after decompressive surgery, the infravesical obstruction p r o b a b l y o c c u r r e d late in the fetal period. T h e b l a d d e r rupture resulting in internal d e c o m p r e s s i o n m a y have constituted an additional protective m e c h a n i s m for the preservation of renal function. Congenital b l a d d e r rupture can be associated with bladder outlet obstruction such as posterior urethral valves, urethral atresia, presacral mass and n e u r o g e n i c dysfunction of the b l a d d e r [1, 2]. T h e b l a d d e r rupture in these cases is m o s t c o m m o n l y intraperitoneal, leading to congenital urine ascites [3]. T h e present case r e p o r t describes the postnatal radiographic, sonographic and magnetic reso n a n c e imaging ( M R I ) findings in a n e w b o r n with sacrococcygeal t e r a t o m a causing b l a d d e r outlet obstruction, leading to b l a d d e r rupture, urine ascites and herniation of bowel loops into the b l a d d e r lumen, to our k n o w l e d g e not previously reported. Case report After a pregnancy with no prenatal care, a girl weighting 4015 g was born by vaginal delivery. At the time of birth, oligohydramnios was noted on physical examination. The Apgar score was 7 and 8 at 1 and 5 min, respectively. On physical examination, abdominal distention, a midline pelvic mass, anal and vaginal prolapse and poor tone of lower extremities were noted. There were no signs of respiratory distress. Laboratory tests showed evidence of renal failure with a serum creatinine level of 3.1 mg/dl. The patient passed meconium normally but had no urinary output. After catheterization of the bladder, 350 ml of blood-stained urine were obtained. An abdominal ultrasound examination demonstrated an inhomogeneous echogenic mass anterior to the sacrum, bilateral hyCorrespondence to: S. J. Westra, M. D., Department of Radiological Sciences, UCLA School of Medicine, Center for the Health Sciences, 10833 Le Conte Avenue, Los Angeles, CA 90024-1721, USA

dronephrosis with a normal appearance of the renal parenchyma, and a large amount of ascites. The bladder was displaced cranially out of the pelvis with marked thickening of the wall. A defect in the posterior bladder wall was seen in the superior scans suggesting free communication between the bladder lumen and the peritoneal cavity (Fig. 1). A plain radiograph demonstrated abdominal distention, a soft tissue mass with punctate calcifications in the pelvic and perineal region, diastasis of the symphysis pubis and the urinary catheter tip in a unusual high position in the abdomen, suggesting that it was located outside the bladder (Fig. 2 a). A bell-shaped thorax with clear lungs was noted on a chest radiograph (Fig. 2b). A cystogram confirmed the radiological diagnosis of a ruptured bladder, with extravasation of contrast material into the peritoneal cavity (Fig. 2b). An MRI scan was performed in order to evaluate the extension of the tumor. On T1- and T2-weighted spin echo images, the mass was markedly inhomogeneous with areas of signal intensity consistent with fluid-filled and fatty components. The mass was occupying the presacral space, and was noted to displace the rectum and uterus anteriorly and the bladder superiorly. The lumbosacral spine and the spinal cord appeared normal. A large defect was present in the bladder dome, allowing bowel loops to herniate into the lumen (Fig. 3). Complete resection of the 6 by 8 cm pelvic tumor, including the coccyx, was successfully performed, through a perineal incision. The histopathological diagnosis of the resected specimen was of a benign teratoma. A laparotomy was also required in order to repair the bladder rupture and a vesicostomy was performed. Five days after surgery, urinary output and serum creatinine levels had normalized. On sonographic follow up, the bilateral hydronephrosis improved rapidly.

Discussion B l a d d e r outlet obstruction usually causes bilateral hydronephrosis and h y p e r t r o p h y of the b l a d d e r wall. U r i n e ascites m a y be s e c o n d a r y to rupture of the urinary tract at the level of the calyceal fornices or transudation of urine f r o m an intact urinary tract [2, 4, 5]. U r i n e ascites secondary to b l a d d e r rupture is u n c o m m o n [1, 5]. In patients with early prenatal obstructive uropathy, such as caused by posterior urethral valves, the developm e n t of the u p p e r urinary tract m a y be compromised, resulting in cystic renal dysplasia, which carries a p o o r prognosis as far as renal function is c o n c e r n e d [4]. In these

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Fig.1 a,b. Abdominal ultrasonography, transverse views, aBladder (B1) with thickened wall and echogenic debris in lumen. Bilateral hydronephrotic kidneys (RK, right kidney; LK, left kidney; A, aorta) b (angulated cranially) Defect (arrow) in postero-superior bladder wall; urine ascites (UA);bowel (Bo) Fig.2. a Plain abdominal radiograph after catheterization of the urethra: abdominal distention; diastasis of pubic symphysis secondary to a large perineal soft tissue mass with calcifications. Note the unusual high position of catheter tip at the level of the second lumbar vertebral body (arrow), probably outside the vesical lumen, b Cystogram confirms rupture of bladder dome with loops of bowel within

entities, the early onset of renal dysfunction and associated oligohydramnios also cause pulmonary hypoplasia, which m a y lead to severe respiratory distress at birth. As no significant respiratory distress was present at birth in this patient, the bell shaped appearance of the thorax was probably secondary to the abdominal distention and not to pulmonary hypoplasia. Although oligohydramnios was present at birth, the amount of amniotic fluid has not been reduced long enough for extrathoracic compression of the lungs to occur during fetal development, and urinary output probably has been sufficient during the critical period for pulmonary development. In this patient with a normally developed urinary tract and a rapid and complete recovery of renal function after urinary diversion, the bladder outlet obstruction probably developed gradually due to the slowly growing tumor, leading to bladder wall hypertrophy and finally rupture as

it (arrows) and extravasated contrast throughout peritoneal cavity F:balloon of Foley catheter. Note the bell-shaped appearance of the chest Fig.3 a,b. Magnetic Resonance scan, Tl-weighted Spin Echo images: a Mid-sagittal image (TR = 550, TE = 15), large inhomogeneous presacral tumor, displacing the rectosigmoid anteriorly (arrow). Bladder (B1) is displaced superiorly out of the pelvis, with a wall defect allowing bowel loops (arrowhead) to herniate into its lumen. b Axial image (TR = 500, TE = 15) through hydronephrotic kidneys and bladder, with herniated bowel loops (compare with 1 a)

a late event. This may have constituted an additional protective mechanism for the preservation of renal function. The herniation of the bowel loops into the ruptured bladder after drainage of the ascites through catheterization of the bladder, as illustrated in this case report, has not been described previously in the literature. The diagnosis of a sacrococcygeal t e r a t o m a leading to bladder outlet obstruction can be suggested with ultrasonography pre- or postnatally by the presence of a presacral mass, bilateral hydronephrosis and ascites. Postnatally, a distended a b d o m e n in association with an unusual large volume of urine obtained through a urinary catheter is suggestive of bladder outlet obstruction or congenital rupture of the bladder, which can be differentiated with a cystogram. CT or M R I m a y be required to characterize the underlying presacral mass, in order to differentiate diagnostic possibilities such as teratoma, anterior me-

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ningocele, neuroblastoma and sacral chordoma. The presumptive histopathological diagnosis based on the radiological findings determine which surgical subspecialist is to perform the resection: general pediatric surgeon, urologist or neurosurgeon. The preoperative assessment of the extent of the tumor and its relationship to surrounding structures such as bladder and rectum indicates the most suitable surgical approach: perineal, abdominal or combined. In conclusion, this case demonstrates an unusual presentation of bladder rupture with herniation of bowel into the vesical lumen. As complete bladder outlet obstruction by the tumor probably occurred late in utero, renal and pulmonary development were not compromized.

References 1. Mann CM, Leape LL, Holder T M (1974) Neonatal urinary ascites: a report of 2 cases of unusual etiology and a review of the literature. J uro1111:124-128 2. Moncada R, Wang JJ, Love L, Bush I (1968) Neonatal ascites associated with urinary outlet obstruction (urine ascites). Radiology 90:1165-1170 3. Zerin JM, Leobwitz R L (1989) Spontaneous extraperitoneal rupture of the urinary bladder in children. Radiology 170:487-488 4. King L R (1985) Obstructive uropathy - posterior urethra. In: Kelalis PR King LR, Belman A B (eds) Clinical pediatric urology, 2nd edn. Saunders, Philadelphia, pp 527-558 5. Weller MH, Miller K (1973) Unusual aspects of urine ascites. Radiology 109:665-669

Literature in pediatric radiology American Journal of Diseases of Children

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