Biomedical subjects
Publications and source records attributed to G Belloli.
The malignant rhabdoid tumor of the kidney determines clinical and prognostic evaluations due to the poor therapeutic answer and to the uncertain prognosis. The Authors describe a case of nephroblastoma with anaplasia of the blastemal elements associated to rhabdomyosarcoma areas.
The authors describe a rare case of primary intestinal lymphangiectasis resolved with surgical treatment. Usually the natural course of the disease is relatively mild and medical nutritional treatment can be sufficient. In this case the lymphatic intestinal anomaly was generalized to the entire small intestine but a distal ileal segment was particularly involved. The surgical resection of this intestinal tract resolved the symptomatology.
Paraesophageal inflammatory pseudotumor is the cause of anaemia and intermittent fever. The surgical treatment determines a rapid and complete clinical resolution.
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.
This is a case report of a newborn with bilateral multicystic kidney; The Authors report the etiology and the histologic features of the renal multicystic dysplasia considering the role of the sonographic examination in its precocious diagnosis. Peritoneal dialysis and renal transplantation in the first year of age are dealt about as the possible current therapeutic measures in the bilateral cases.
The authors present the study and results in 100 subjects operated of hypospadia. They interviewed patients within 15 and 22 years old, in randomized selection and in permanent percent relations as far as: different types of pathology, the age of surgery effected in Vicenza Hospital - Pediatric Surgery Department (1971-1981). Through a structured questionnaire they tried to analyse psychological lived in operated subjects as far as: surgery, convalescence, psychologic problems before and after surgery, way of life when they became adults. Results explain that exist some psychologic problems in operated patients (8.5 years old) before surgery and during convalescence, while there is no problem after surgery. Moreover when these subjects became adults their kind of life (school, profession, sexuality) is without psychopathological problems. This is because of positive attitudes pre-acquired, brief convalescence and above all the high and positive quality of surgery effected.
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.
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.
Retrotendineus ureter is an exceptional anomaly presenting, in our experience, without clinical signs of urinary infection and obstruction. The radiological diagnosis was retrocaval ureter. The Authors report a case of a 9 years old boy who was successfully treated by ureteral section, excision of the obstructed ureteral segment and uretero-ureteral anastomosis. It seems that the case is the first reported in the medical literature of this anomaly.
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.
Hydronephrosis in infancy and childhood is a frequent urinary malformation and is almost always congenital. In over 80% of the cases it is due to ureteropelvic junction obstruction; in about 17%, anomalous renal vessels at the ureteropelvic junction are present. The authors analyze their experience in the surgical treatment of hydronephrosis. 360 hydronephrosis out of 335 patients were operated on from November 1971 to November 1988. 108 patients were less than 1 year old (32.2%). Primary nephrectomy was carried out only in 11 of the 360 hydronephrosis operated on (about 3%). An anterior approach and a simple dismembering pyeloplasty were performed in the majority of the cases. From the radiological point of view there was an impressive amelioration or normalization in about 92% of the cases operated on. From the functional point of view the radionuclide scan showed a normal or slightly reduced renal function in almost 90% of the cases operated on. The preoperative and postoperative radionuclide study showed a moderate increase of the renal function in the majority of the cases controlled. The authors stress the importance of prenatal ultrasound diagnosis to improve long term results of an early surgical treatment.
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.
Transureteroureterostomy is a method of internal urinary diversion available to maintain the integrity of the urinary system, when the lower ureter is compromised. The usual requirements are a donor kidney with a minimal renal function on one side and a normal contralateral recipient kidney and ureter. From January 1972 to October 1988 in our Division transureteroureterostomy has been done in 52 children. In some cases the procedure was used simultaneously with reimplantation or cutaneous ureterostomy. The majority of patients had been referred with previous failed ureteral reimplantation or with unilateral reflux or ureteral obstruction in neurogenic bladder with small capacity and compliance. Results (14 years to 6 months follow-up) are very good with excellent results for both kidneys in 98% of the cases. Our experience and that of other authors confirm that transureteroureterostomy is a technique simple with low morbidity and excellent results.
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.
Chronic constipation in childhood, with or without megarectum, with or without encopresis is a symptom of many diseases. In the majority of this patients a detailed history and an accurate physical examination differentiate functional and aganglionic constipations by other causes of constipations. Anorectal manometry is an excellent diagnostic technique for diagnosis of ultrashort segment Hirschsprung's disease. The rectoanal inhibitory reflex is present in severe chronic functional constipations and absent in aganglionic constipations. Anorectal manometry is a simple and perfectly harmless technique. The possibility of error is very small in patients aged over two years. Surgical treatment is recommended in ultrashort segment Hirschsprung's disease. Medical treatment is recommended in chronic functional constipation and after surgical treatment. It is very important that the period of treatment is long enough to avoid relapses.
The tethered cord syndrome is a form of spinal dysraphism in which a short, thickened filum terminale prevents the ascent of the conus medullaris and intraspinal lipoma causes compression upon the caudal part of the spinal cord. In pediatric age the first symptoms are urological: urinary incontinence and infection. Diagnosis is performed with urodynamic studies followed by nuclear magnetic resonance. Tethered cord syndrome is no obvious cause of a neurogenic bladder. When this pathological conditions is recognized properly and an operation is performed early the likelihood of improvement is rather high. The authors report a case of tethered cord syndrome in a girl seven years old.
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.