Duodenal atresia associated with midgut deletion in cloacal exstrophy: a new association?
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to P Ransley.
Explore the source record for details and available documents.
PURPOSE: Demographic data, clinical presentation, associated abnormalities, and radiologic findings were evaluated to outline diagnostic criteria that may lead to the diagnosis of xanthogranulomatous pyelonephritis in children. METHODS: Eleven boys and 8 girls with a mean age of 3.4 +/- 1.7 years were classified into obstructive xanthogranulomatous pyelonephritis (n = 13), which was associated with nephrolithiasis and nonobstructive (n = 6), which mimicked Wilms' tumor. RESULTS: Twelve children with obstructive diffuse involvement of the renal parenchyma, 1 with left-sided obstructive focal involvement in a horseshoe kidney (group 1), and 6 with nonobstructive diffuse xanthogranulomatous pyelonephritis (group 2) showed a male to female ratio of 1.2:1 and 2:1, respectively. Mean age was 4.1 +/- 1.2 years in group 1 versus 1.8 +/- 1.5 years in group 2; P =.001. The common features were renal mass, hematuria, and anemia (100%; P =.07) and leucocytosis (77% v 83%; P =.097). Main differences between the 2 groups were acute inflammatory syndrome (0 v 33%; P =.01), recurrent urinary infection (54% v 17%; P =.05), isolation of Proteus mirabilis as a pathogen (69% v 0; P =.001), and renal stones (100% v 0; P =.001). Preoperative diagnosis was accurate in all 13 (100%) children with obstructive xanthogranulomatous pyelonephritis. Radiologic features that were not consistent with Wilms' tumor in group 2 were absence of sharp definition and encapsulation of the mass, ill-defined margins with inflammatory infiltration of the perinephric fat and focal inflammatory tissue destruction. Nephrectomy was technically difficult because of extensive adhesions to the retroperitoneum, psoas muscle, and surrounding structures in both groups. CONCLUSIONS: Xanthogranulomatous pyelonephritis must be considered in the differential diagnosis of a child presenting with a renal mass, anemia, and elevated inflammatory markers. Treatment by nephrectomy is curative. J Pediatr Surg 36:598-601.
The early ultrasound detection of fetal urinary dilatations raises dilemmas about their physiological and pathological meanings. Dilatation does not always mean pathological obstruction of the urinary tract. The chronology of the embryology of the Wolffian system is partially unknown and some hypotheses can be developed concerning the urine drainage in the fetus. The physiology of the fetal kidney is also unclear because of the inadequacy of the functional assessments and their dependence on the mother's parameters. The study of the pressures inside the different segments of the fetal urinary tract could be an efficient tool in the understanding of its progressive permeabilization. The difference between primary kidney dysgenesis and kidney dysplasia secondary to urine hyperpressure appears to be of paramount importance in the fetal treatment of these dilatations and in the long-term prognosis. Thus one can better understand how difficult the decision of shunting the fetal urine toward the amniotic cavity. Can be no one can prove its benefit for the fetal development and some lethal complications of these in utero procedures have been described. However, this new approach of the fetal urinary tract obstruction leads us to a change in the therapeutic attitudes in newborns, which stresses a better understanding of this development.
References on the anatomical course of the neurovascular bundles of the penis in epispadias are rare. We studied the anatomy of the neurovascular bundles in 5 patients undergoing primary epispadias repair and 13 adolescents undergoing correction of erectile deformities. In all primary cases the neurovascular bundles were truly lateral as they ran along the distal and middle portions of the corporeal bodies, and became anterolateral only proximally. The bundles were identified in only 5 of 13 secondary cases and were in the same position as in the primary cases. Knowledge of this anatomy is important to protect these structures from surgical injury in cases of epispadias and exstrophy.
Explore the source record for details and available documents.
Explore the source record for details and available documents.