Biomedical subjects
Publications and source records attributed to J F Schillinger.
Priapism may be classified as the classical veno-occlusive form or the more recently described high flow, arterial type. We report a case of posttraumatic arterial priapism in a 7-year-old boy. The successful management with percutaneous transcatheter embolization of the internal pudendal artery is described.
An infant who presented with a unilateral renal cystic process underwent nephrectomy due to hypertension. There was no evidence of any other disease process. Family history and investigation were negative for renal cystic disease or other genetic disease process. The pathology most resembled autosomal dominant polycystic kidney disease or a specific entity described as unilateral renal cystic disease.
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Nephrogenic adenoma, an unusual proliferative lesion, often occurs in association with chronic inflammation of the bladder and is rarely found in children. Two cases are reported, one occurring in a young child born with bladder exstrophy, and one in a young female, with both interstitial cystitis and chronic use of immunosuppressive therapy. We believe these are the first case reports of nephrogenic adenoma occurring in this specific clinical setting.
An unusual form of crossed renal ectopia is presented. Both kidneys are at the same level on the same side with fusion throughout their entire lengths.
Over a ten-year period, of more than 1,000 children whose primary vesicoureteral reflux was treated surgically, there were 54 children with 80 single ureters which were massively dilated. Primary reconstruction with ureteral tailoring was done in 25 ureters with a success rate of 92 per cent. Forty-five ureters were temporarily diverted because of uncontrolled infection and/or azotemia. In almost half of these instances, temporary diversion resulted in improvement in ureteral caliber to such an extent that nontailored reimplantation could be performed with a success rate of 87 per cent. Six kidneys were removed and four have been maintained in the diverted state with severe renal dysplasia. Eighty-eight per cent of the refluxing megaureters were reconstructed with a success rate of 89 per cent.
Permanent urinary diversion has been the standard recommended treatment of massive dilatation of the upper tracts in the child with neurogenic vesical dysfunction. Reimplantation of relatively normal caliber ureters into neurogenic bladders has been shown to be effective. However, attempts to save urinary diversion in 39 neurogenic megaureters have been unrewarding, with the salvage rate of 15 per cent, and 64 per cent have been diverted permanently. Upper tract decompression by continuous or intermittent catheterization, nephrostomy or end cutaneous ureterostomy was used in this series. When ureteral caliber responded to decompression a reasonable success rate from non-tailored reimplantation may be anticipated. However, in those instances in which ureteral caliber failed to respond or when tailoring or ureteral caliber was done in conjunction with reconstruction the success rate was much lower.
Carbon dioxide urethral pressure profiles were analyzed under the same conditions for 113 spina bifida and 87 non-spina bifida patients categorized by sex and age. Either duplicate or triplicate measurements were obtained from each patient for parameters of continence length, closure pressure and functional length. The degree of reproducibility of each parameter varied depending on age, sex and diagnosis. Except for 1 age-sex category closure pressure exhibited the smallest degree of relative variation for spina bifida patients. Results for non-spina bifida patients were mixed in that the measurement of no one parameter resulted in a uniformly smallest degree of relative variation. Reproducibility based on within patient variability generally was least for the continence length.
Of 105 boys with posterior urethral valves managed during a 10-year period most were managed by primary valve ablation. However, 39 of these boys required concomitant or additional procedures to 71 massively dilated ureters because of azotemia, infection and/or progressive upper tract deterioration. In 17 boys 25 ureters could be reconstructed primarily by varying degrees of ureteral tailoring. When there was severe infection, azotemia and/or doubt as to the function of the affected renal unit, staged reconstruction was initiated by cutaneous ureterostomy. One-fourth of these ureterostomy diverted children died of azotemia despite free urinary drainage. Those who went on to have staged reconstruction, despite multiple surgical procedures, retained intact urinary systems with acceptable function.
Two cases of bladder diverticula causing posterior urethral obstruction, which was corrected surgically, are presented. Five similar cases have been found in the literature.
A case of staphylococcal endocarditis, complicated by a leaking mycotic aneurysm of the right artery in a boy is presented. An emergency nephrectomy was lifesaving. The clinical course and pathology are discussed and the literature is reviewed.
From 1965 through 1975 we treated 30 children with 44 megaureters that were acquired after the reimplantation of normal caliber ureters. One-third of these children had had at least 2 prior surgical procedures on the bladder and 30 per cent were azotemic. There was no difference in the salvage rate of acquired megaureters with reflux versus obstruction. Temporary proximal diversion often was useful in the short-term management. Only 70 per cent of the patients completed reconstruction and only 70 per cent of those reconstructed were successful by all parameters. Ureteral tailoring was generally a necessary adjunct to successful repeat reimplantation. There was a significantly lesser salvage rate in those ureters that had had more than 1 prior surgical procedure. The best results were obtained when all surgical maneuvers were done transvesically because there was a significant incidence of acquired atonic vesical dysfunction after repeated dissections in the perivesical area.
From 1964 to 1975, of 83 children with primary megaureter 33 had unilateral and 8 bilateral massively dilated ureters that required surgical treatment, and of these 73% had febrile infection, 5% were septic and 10% were azotaemic. Surgical treatment consisted of ureteric reimplantation with excision of the atonic distal segment, preceded by diversion when indicated. Tailored reimplantation was successful in 98% of ureters and non-tailored reimplantation was uniformly unsuccessful. All failures were successfully corrected by tailored reimplantation. Thus the requirements for successful surgical management of massive ureteric dilatation in children secondary to an atonic ureteric segment are excision of the atonic segment, transvesical or extravesical tailoring of the ureter and reimplantation.
A form-fitting glans condom has been developed for use in small uncircumcised males with neurogenic bladders to avoid the problems inherent with diapers. The method to make the glans condom and early successful use in 19 of 33 patients are noted.
Fifteen boys with prune belly syndrome cared for over a ten-year period required surgical treatment because of uncontrolled infection and/or progressive azotemia. Five died of renal dysplasia. Primary or staged reconstruction resulted in significant improvement when ureteral reimplantation was combined with tailoring of ureteral caliber.
Cystometry in 21 cases of closed continent bladder exstrophy revealed that 14 patients had a normal reflexic bladder. A simultaneous anal plug electromyogram showed an appropriate response in all patients when correlated with a cystometrogram.
The relationship of the onset of continence, staged surgical procedures and continence length was investigated in 21 patients with closed continent exstrophy. Continence occurred within a year of operation in 13 patients and 10 of these 13 patients had a Young-Dees urethroplasty and Marshall-Marchetti-Krantz suspension. In 11 of these 13 patients the continence length was greater than 1 cm. In the remaining 8 patients continence was delayed beyong a year. Only 1 of these 8 patients had undergone both bladder neck procedures and in 5 patients the continence length was greater than 1 cm.
The urethral pressure profiles for 36 patients were analyzed specifically for continence zone by 3 techniques: 1) triangle, 2) planimeter and 3) weight. Graphically, the best agreement was between the weight and planimetry methods but, statistically, there was a significant difference among techniques except for the subgroup that had the smallest continence area.