[Clinico-radiological study and therapeutic problems of vesico-ureteral relfux in childhood].
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Publications and source records attributed to L Musi.
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Fifty-four cases of anomalies of position in 52 children were reviewed. Of the 52 children 24 (46%) had anomalies involving organs outside the urinary tract and contralateral renal units. Twenty-five ectopic kidneys showed major urological complications (46%). Four were treated conservatively and 21 with surgery. The postoperative results have been excellent in all the cases. Renal anomalies of position often are associated with congenital renal and extrarenal malformations. Nearly 50% of ectopic kidneys are involved by pathological process that often require surgery. Surgical treatment of urological complications of renal ectopia is discussed.
One to two per cent of children and up to 11% of adolescent have arterial hypertension. In most cases children and adolescent are not recognized to be hypertensive because physicians do not routinely measure blood pressure. Often the diagnosis is recognized only when the pediatric patients develop a complication: seizure, stroke, heart failure or paraplegia. Renovascular hypertension in children and adolescents is more common than all of the other causes combined, except for coarctation of the aorta. The diagnosis is not so easy and includes the usual history, physical examination (signs and symptoms of coarctation of the isthmic or abdominal aorta or of an abdominal mass or of one of the adrenal causes of hypertension), laboratory studies, abdominal ultrasound study and chest x-ray. Sometime a CAT can be usefull. The next steps are the early and rapid-sequence IVP, renal angiography and peripheral and renal renin activity. The management of renovascular hypertension in children and adolescent includes a conservative approach (percutaneous transluminal renal angioplasty or renal embolization), rarely used in pediatric age, and the surgical treatment. This latter includes all the surgical procedures of renal revascularization and, in unilateral renal parenchymal diseases, the nephrectomy or a partial nephrectomy. The postoperative results are very good in a high percentage of cases. In bilateral cases, the revascularization surgical procedures improve or normalize also the impaired renal function.
In a overall series of 1621 hypospadias operated on, the Authors describe the technical aspects and report the precise indications and results in 141 cases of anterior and middle penile hypospadias using the one-stage Mathieu-Righini repair. This technique has resulted in remarkably few complications and very nice aesthetic results. There was a remarkably low (3.5%) rate of fistula and no urethral stenosis without necessity of postoperative urethral dilatations. The penis looks "normal" in appearance as well as in function.
The authors report a case of a supernumerary hepatic lobe at the umbilicus of a newborn. They review the different symptoms, varietes and frequencies of supernumerary hepatic lobes. The importance of this embryological anomaly is underlined in order to be differentiated from urachal and omphalo-mesenteric duct malformations.
We report two cases of perirenal urinary extravasation one of which associated with urinary ascites. An underlying congenital obstructive uropathic condition was discovered in both patients. A localised parenchymal perforation was found in both cases. Prompt treatment with correction of metabolic acidosis and decompression of the tense abdomen and the urinary tract is recommended. The patients survive in good health.
The prune belly syndrome consists of congenital absence or deficiency of the anterior abdominal muscle, bilateral cryptorchidism and anomalies of urogenital tract. Neurological problems are common in these patients as respiratory, orthopedic, gastrointestinal and chromosomal anomalies. We have treated 17 cases of prune belly syndrome. Mortality is 23.5%: two patients died in the neonatal period of respiratory failure; one patient, aged two months, died of septicemia in postoperative period, another, 14 years old, died of respiratory failure with normal renal function. Two patients are female without urological anomalies, one male has an incomplete form with normal urinary tract. Two patients with complete syndrome are treated conservatively. Ten patients with vesicourethral reflux or ureteral dilatation for distal obstruction are treated surgically. We performed 20 ureteral reimplantations with 16 tailoring of the ureters. In three patients ureteral reimplantation failed and in these patients we performed successfully a transureteroureterostomy. All patients have adequate complete bladder emptying without surgical procedure. Renal function is normal in seven, slightly reduced in one and reduced but stable in the others. Orchidopexy was performed successfully in eleven patients (10 Fowler-Stephens procedures). Orchiectomy with testicular prosthesis was performed in a patient 12 years old. Five patients have moderate respiratory insufficiency treated with chest physiotherapy. Urinary tract abnormalities are the most common cause of morbidity and mortality in patients with prune belly syndrome. Treatment regimen must be individualized. Respiratory problems are also frequent and is mandatory a precocious chest physiotherapy.
The goal of the treatment involving the children with exstrophy of the bladder must include urinary continence, protection of renal function, good reconstruction of the penis and a good quality of life. The staged approach (Jeff's protocol) includes bladder closure with penile lengthening, when necessary, in the first week of age, epispadias repair and finally bladder neck reconstruction with bilateral ureteral reimplantation. The authors present their experience on staged functional reconstruction of classical bladder exstrophy (40 patients). They had no dehiscences of the abdominal wall and bladder at the primary bladder closure, at any age, with or without bilateral iliac osteotomy. The final successful rate in the staged functional bladder reconstruction was about 70% and it is continuously improving. In the authors' opinion the successful initial bladder closure is perhaps the most important factor for obtaining a larger bladder more quickly and for achieving a high urinary continence; it must be performed in tre first days of life.
The goal of the therapy involving the children with exstrophy of bladder must include urinary continence, protection of renal function and a good quality of life. Primary closure of the bladder would seem to be the ideal solution, but when it is impossible or fails, ureterosigmoidostomy can provide satisfactory solution. We have treated 15 selected exstrophied patients with ureterosigmoidostomy. The average length of follow up was 8 years and 9 months (range is from 19 years to 18 months). All patients have normal renal function; in 13 patients upper urinary tract is normal and two have a moderate ureteral dilatation. In 13 patients the fecal and urinary continence is complete, in two it's acceptable. We report radiological and manometric studies of the rectum and sigma after ureterosigmoidostomy. The long term results are excellent; it is mandatory to have correct indications, meticulous care in the operative technique producing a long submucosal tunnel with direct anastomosis of the ureter to the bowel, post operative management and follow up care.
Ectopic ureter is quite a rare malformation, and it can be more or less serious depending on its anatomy (monolateral or bilateral ectopy, duplicated ureter, single ureter) and the associated malformations. It's more common in female patients. Ectopic ureteral orifice can be either intravesical (bladder neck) or, more often, extravesical. Ectopia in women is more frequently found in the urethra and the vaginal vestibule; much less frequently in vagina and in uterus. In men it is mostly found in the posterior urethra; in the male genital tract is very rare. In most cases ectopic ureter is associated with pyelo-ureteral complete duplication: the upper kidney usually works badly or doesn't work at all, due to renal dysplasia or pyelonephritis. More rarely ureteral ectopia affects a single urinary system and can be monolateral or, in the most serious forms, bilateral. The main clinical signs are urinary incontinence accompanied by regular micturition (more exactly pseudoincontinence) in the female patients, and urinary infection. In the male there is no urinary incontinence. Authors report their experience about 54 ectopic ureters in 51 children. Treatment is always by surgery: the choice has to be made between conservative or radical attitude. 38 total exeresis of the ectopic excretory pathway (upper heminephroureterectomy or nephroureterectomy), 9 ureteral reimplantations, 1 uretero-pyeloanastomosis have been done. Outcome is always satisfactory in monolateral forms, more uncertain in bilateral forms with single ectopic ureter, due to incontinence and reduced bladder capacity problems and the possible associated renal failure.
The management of the urethral strictures in children has involved dilatation, internal urethrotomy and urethroplasty. Each method of management has varying degrees of success and is associated with special problems. The authors describe their experience in the management of urethral strictures in 32 children by inflatable balloon catheter. The procedure involves atraumatic catheterization by an urethral catheter under radioscopic vision, followed by introduction of a balloon catheter on a flexible guide which is then filled by contrast medium. The stenosis is visible on the balloon and the rapidity with which it "yields" can readily be appreciated. Control of dilatation is effected by voiding urethrography. This method entails much less discomfort and more advantages than conventional urethral dilatation. No complication was observed. All patients underwent a second balloon dilatation, without radioscopy and 25 also a third prior to obtain a good and permanent result.
The actual hypothesis on the etiology and pathogenesis of neonatal hepatitis, intrahepatic and extrahepatic biliary atresia and choledocal cyst is that these disorders can be different results or permissible outcomes of a single basic process: infantile obstructive cholangiopathy. This hypothesis can explain the failure of many infants with operable extrahepatic biliary atresia to do well following surgically successful anastomosis. Very possibly no surgical mode of therapy will cure a significant fraction of infants with biliary atresia (correctable and non-correctable types) because the basic disease process actually produces portal fibrosis and destroys intrahepatic bile ducts as well. However, since the obliterative process can resolve, even if not usually completely, surgical procedures of conventional or of hepatic-portoenterostomy type should be considered for all infants who are found to have biliary atresia. Probably biliary atresia is more in need of preventive or prophylactic measures than of new surgical procedures.
The Authors analyze the diagnostic contributions of echography and computer-assisted tomography in the diagnosis of duodenal hematoma in pediatric age.
Rebleeding, the long time high occurrence of encephalopathy and the impairment of hepatic function in the successful cases have led to increasing dissatisfaction in the last years with portasystemic shunt procedures. In the past 12 years we have operated on 14 children for bleeding esophageal varices using the Sugiura procedure (esophageal transection with paraesophagogastric devascularization); in two cases the entire procedure was performed through the thoracic approach. We had no mortality. Complications include bleeding in the early postoperative period in two children and partial leakage from the esophageal suture in two others. Patient follow-up has been between 16 months and 11 1/2 years with an average of 6 1/2 years. The long term controls have been gratifying in 12 patients with disappearance of the varices and no evidence of rebleeding, esophageal strictures, gastroesophageal reflux, encephalopathy or impairment of the hepatic function; in two other patients the operations was unsuccessful. In our opinion the Sugiura procedure should be the elected operation in the surgical treatment of esophageal varices bleeding in pediatric age, after an attempt with sclerotherapy.
Ureterocele is an infrequent urinary malformation in pediatric age, but it is frequently clinically severe. Ureterocele in a duplex pyelo-ureteral system often presents very difficult treatment problems not uniformly dealt with. Authors report their experience with 79 ureteroceles observed in 72 children. 27 children were less than one year old. In the authors experience simple ureterocele with a single collecting system doesn't give any surgical problem. On the contrary ectopic ureterocele, almost always associated with a pyeloureteral duplication, frequently gives some problems of surgical treatment. Treatment is always individualized but in our experience the best results have been achieved with unroofing of the ureterocele and upper heminephroureterectomy in one stage, eventually associated with lower ureteral reimplantation. This treatment can be used successfully in the majority of ectopic ureteroceles in pediatric age.
Primary vesicorenal reflux (P.V.R.R.) is the most important cause in the pathogenesis of chronic pyelonephritis, better known in medical literature as "reflux nephropathy". The Authors stress out the criteria adopted in their Center for surgical treatment of P.V.R.R. and report the results in 1158 refluxing ureters (858 children) operated on. In 5 ureters an ureteronephrectomy and in 1153 remaining ureters and ureteral reimplantation was carried out. About 57% of ureters operated on was affected by severe reflux (IV - V grade). The Authors obtained 98.6% of surgical successes. In 16 ureters operated on, stricture (9 ureters) and reflux (7 ureters) were postoperatively present. The percentage of successes in infants less than 2 years old at operation was 98%.
In this paper the authors have analyzed the management and the outcome of 81 cases of posterior urethral valves which occurred during the period January 1972 - April 1985. 53 children presented very severe urethral valves (grade 4 according to Hendren), 28 mild valve type. All the children of the first group but two had a dilatation of the U.U.T. Vesico-renal reflux, usually severe, was present in 51 ureters; in another 50 ureters a dilatation without reflux was present. 47% of the children of the first group had a renal function within the limits of normality at the moment of presentation and the remaining 53% a reduced renal function. In 9 patients (8 under 50 days of age) a cutaneous vesicostomy according to Blocksom followed, at the age of 10-18 months, by transurethral valve destruction was done. In 6 infants, in the early part of the series, the valve was removed with a hook via the perineal approach. In 38 patients we performed a transurethral valve destruction with the n. 3 Bugbee electrode. 36 out of 53 children (68%) had exclusively a removal of the valvular obstruction. After the removal of the obstruction, 32 out of 37 non refluxing dilated ureters (86.5%) showed a clear improvement. In 7 out of 29 refluxing ureters a nephrectomy was carried out. In the remaining 22 ureters the reflux vanished in 17 and improved in the other 5. 17 children had other types of operations after valvular removal. 23 ureters in 13 patients were reimplanted, with 3 failures (13%). In the 53 children with very severe valve (grade 4) supravesical diversions were not carried out. In the follow-up of 51 children (from 6 month to about 14 years) the renal function was within the limits of normality in 74% (before the operation it was 47%). The best results were obtained in children diagnosed and treated in the first months of life. Cutaneous vesicostomy showed itself to be a very useful method of treatment in very young babies with severe complications. We observed a slight terminal urethral stricture, easily dilatable, in only 1 child. All the children over the age of 12-13 years were continent. In 28 children with mild valves, as well as transurethral valve destruction, an ureteric reimplantation was carried out with success in 5 children (8 ureters) and a vesical diverticulectomy in another 2.