Complex cloacal malformations: UUse of rotational fluoroscopy and 3-D reconstruction in diagnosis and surgical planning
Division of Interventional Radiology, Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH 45229, USA. Pediatric Radiology
(Impact Factor: 1.57).
11/2011; 42(3):355-63. DOI: 10.1007/s00247-011-2282-1
A cloacal malformation is a congenital malformation in which the urinary tract, gynecological system and distal rectum fail to separate and form a common channel with a single perineal opening. Precise anatomical information is required to plan surgery and predict prognosis for children with this abnormality. Conventional fluoroscopic studies provide limited information, primarily due to the overlap of structures and inability to make accurate measurements. Rotational fluoroscopy and 3-D reconstruction help clarify overlapping structures and allow for precise measurement of the common channel, thereby helping to predict the complexity of the surgical case as well as the long-term prognosis regarding bowel, bladder and sexual function.
Available from: Andrea Bischoff
- "The length of the common channel is cystoscopically determined after birth and it correlates with the complexity of surgical reconstruction that will be required as well as the functional outcome . The use of a rotational fluoroscopy with 3D reconstruction  prior to the main surgery provides a more accurate anatomical assessment. "
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ABSTRACT: With the advance of prenatal imaging, more often pediatric surgeons are called for prenatal counseling in suspected cases of cloaca or cloacal exstrophy. This presents new challenges for pediatric surgeons since no specific guidelines have been established so far. The purpose of this review is to analyze our experience in prenatally diagnosed cloaca or cloacal exstrophy and to provide some guidelines for prenatal counseling of these complex congenital anomalies.
A retrospective review of the medical charts of patients with prenatally diagnosed cloaca and cloacal exstrophy who received postnatal care in our institution between July 2005 and March 2012 was performed. Representative images of prenatal studies were selected from 13 cases to illustrate different scenarios and the recommendations given. In addition, a review of the literature was performed to support our advice to parents.
Eleven patients were female and two patients were male. The postnatal diagnoses were cloacal exstrophy (6), cloaca (5), posterior cloaca variant (1), and covered cloacal exstrophy (1). The selected abnormal prenatal imaging findings in these 13 patients included hydronephrosis (12), neural tube defect (8), omphalocele (7), lack of meconium at expected rectal location (7), vertebral anomaly (7), non-visualize bladder (5), distended bladder (5), hydrocolpos (4), dilated or echogenic bowel (3), umbilical cord cyst (3), separated pubic bones (2), and the "elephant trunk" sign (2). The prenatal diagnosis was correct in 10 cases, partially correct in two cases, and it was missed in one case. All parents received prenatal counseling depending on the specific diagnosis.
The continuous technologic innovations in prenatal imaging make it possible to prenatally diagnose more complex anomalies including cloaca and cloacal exstrophy with increased levels of confidence and enhance the benefit of prenatal counseling. Together, these allow the parents to be better prepared for the condition and the care team to provide the best possible initial management in order to improve the outcomes of these challenging patients.
Available from: Daniel H Teitelbaum
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ABSTRACT: Cloacal anomalies represent one of the greatest surgical challenges in pediatric surgery. A recent advancement in the imaging of these anomalies is 3D rotational fluoroscopic cloacagram. However, a disadvantage is that obstructed lumens and extraluminal soft tissue structures are poorly delineated. We describe the development of a novel imaging technique whereby 3D rotational fluoroscopy is combined with high-resolution 3D pelvic MR imaging.
3D rotational fluoroscopic cloacagram was initially performed. Catheters were directed into the urinary bladder, colon, and vagina. Low-osmolality iodinated contrast material mixed with a small amount of gadolinium contrast material was used to distend the catheterized cavities. After securing the catheters in place, patients underwent high-resolution 3D MRI of the pelvis. 3D MRI reconstructions were created.
3D rotational fluoroscopic cloacagram provided excellent luminal definition and accurate measurements of channel/fistula lengths, using intraoperative findings as the reference standard. 3D pelvic MRI measurements were easily and accurately made (using intraoperative and 3D rotational fluoroscopic cloacagram as reference standards), and soft tissue structures outside the lumens of the bladder, vagina, and rectum were well-defined. 3D pelvic MRI was able to identify a small vesicovaginal fistula that was not seen at 3D rotational fluoroscopy.
This novel form of preoperative imaging results in excellent delineation channel/fistula lengths, luminal anatomy, and extra-luminal soft tissue structures to aid in operative planning.
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ABSTRACT: Children born with a persistent cloaca undergo complex pelvic reconstruction early in their life. Long-term risks of bladder dysfunction and chronic kidney disease (CKD) are well described. In this study, we report upper urinary tract outcomes and the risk of CKD stage progression.
A retrospective cohort study was performed of all patients undergoing posterior sagittal anorecto-vagino-urethroplasty (PSARVUP) at a single institution from 2006-13. Inclusion criteria included complete urological care at our institution. CKD stage was calculated from cystatin C or nuclear medicine GFR.
44 patients met inclusion criteria. 12 patients had a vesicostomy or ureterostomy. 19 had hydronephrosis, 19 had VUR, and 15 had a tethered spinal cord. Median length of the common channel was 3.5 cm. Median age at PSARVUP was 7.3 months. Median follow-up was 5.3 years. 30 patients had a neurogenic bladder. 27 require CIC, and 3 patients have a vesicostomy. 38 patients had CKD Stage I or II. 5 patients had CKD stage III, and 1 had CKD Stage IV. In the follow-up period, no patients with initial CKD stage I to III have had stage progression. The one patient with CKD Stage IV had a renal allograft placed at 34 months of age prior to the need for dialysis.
In this series, early outcomes in patients with chronic kidney disease stage I to III show that renal function can be maintained despite a high rate of lower urinary tract dysfunction. Aggressive bladder management may help prevent progressive renal injury in this population.
Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.
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