Long-term urological outcome of cloaca patients with multidisciplinary management.

PURPOSE: Urological management of Cloacal Malformation (CM) focuses on preserving renal function and continence. Study aim was to analyze urinary and intestinal outcomes in CM patients, considering the length of common channel (CC) and presence of occult spinal dysraphism (OSD). METHODS: Retrospective review of CM treated at our institution by a multidisciplinary team from 1999 to 2020. Patients with follow-up < 2.5 years were excluded. Length of CC, renal function, urinary and bowel outcomes, presence of associated anomalies (especially OSD) were evaluated. RESULTS: Twenty patients were included, median age at follow-up: 8 years (4-15). A long CC > 3 cm was described in 11 (55%). Chronic kidney disease was found in 3 patients. Urinary continence was achieved in 8/20 patients, dryness (with intermittent catheterization) in 9/20. Fecal continence was obtained in 3/20, cleanliness in 14 (under bowel regimen). OSD was present in 10 patients (higher prevalence in long-CC, 73%). Among OSD, 1 patient reached fecal continence, 7 were clean; 2 achieved urinary continence, while 6 were dry. CONCLUSIONS: Length of CC and OSD may affect urinary and fecal continence. An early counseling can improve outcome at long-term follow-up. Multidisciplinary management with patient centralization in high grade institutions is recommended to achieve better results.

Surgical validation of functional magnetic resonance urography in the study of ureteropelvic junction obstruction in a pediatric cohort.

INTRODUCTION: Ureteropelvic junction obstruction (UPJO) is one of the most common urological diseases in children. The etiology can be intrinsic, extrinsic (crossing vessel [CV] or adhesions), or mixed. To date, ultrasonography and scintigraphy are considered gold-standard imaging techniques for the study of UPJO. Functional magnetic resonance urography (fMRU) combines anatomical and functional information and has been recently evaluated for the detection of CVs in UPJO. OBJECTIVE: The objective of the study was to evaluate the concordance between fMRU and surgery in determining the etiology of UPJO and the presence of obstructing/non-obstructing CVs. STUDY DESIGN: Patients with unilateral hydronephrosis who underwent surgery after an fMRU were included in the sample. Surgical data regarding the etiology of UPJO were compared with radiological results. The etiology was divided into intrinsic, extrinsic due to CV, extrinsic due to adhesions, and mixed or cicatricial (postoperative). The concordance was calculated by means of the Cohen's kappa coefficient. RESULTS: The observed agreement between fMRU and surgical findings regarding the etiology and the presence of CV were 83.2% and 89.4%, respectively (with substantial Cohen's kappa coefficient). The sensitivity and specificity of fMRU were 0.84 and 0.93, respectively; the positive predictive value (PPV) and negative predictive value (NPV) were 0.889 and 0.897, respectively. The observed agreement regarding the type of vessel was 88.3% with a Cohen's kappa coefficient of 0.787 (substantial). DISCUSSION: In children with hydronephrosis, it is very important for the surgeon to quantify the extent of dilation, define the etiology of the obstruction, and the presence or absence of CVs. fMRU is a 'one-stop-shop' technique which provides both anatomical and functional information showing a high concordance with surgical findings, avoiding radiation exposure. CONCLUSIONS: fMRU should be considered a valid imaging technique in the study of pediatric UPJO, as it provides the surgeon with important information regarding the etiology of the obstruction for the preoperative planning.