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Biomedical subjects

H M Snyder

Publications and source records attributed to H M Snyder.

At least 127 records · Page 7Linked to original sources

Urological complications of sickle cell disease in a pediatric population.

We surveyed 321 patients 1 to 18 years old who were followed at the sickle cell clinic at the Children's Hospital of Philadelphia between 1970 and 1984 for urological complications of the disease. Mean followup was 5 years and all patients exhibited a typical spectrum of hemoglobin types. The urological problems encountered were those cited in the literature, namely hematuria, urinary tract infection and priapism. Surprisingly few of our patients experienced significant renal bleeding. Although the number of patients with infection evaluated radiographically was small, the frequency of renal parenchymal scarring was disturbingly high despite the reported rarity of reflux in black subjects. Our survey and a review of the literature indicate that most sicklemic children with urinary infection are not subjected to urological evaluation. We question the wisdom of that policy. Finally, we found that priapism responds most often to nonsurgical therapy and that it rarely results in impotence in young sickle cell patients.

Anemia, Sickle Cell↗

The dorsal lumbotomy incision in pediatric urological surgery.

Between January 1, 1983 and September 30, 1985, 42 upper urinary tract operations were performed using the dorsal lumbotomy incision, representing 28 per cent of all upper tract procedures performed. Operations included pyeloplasty, partial and total nephroureterectomy, pyelolithotomy, ureterolithotomy and renal exploration with cyst marsupialization. The major contraindications to lumbotomy were malignancy and malrotated or malpositioned kidneys. Patient age ranged from 1 month to 20 years. A modification of the Gil-Vernet vertical lumbotomy incision was used in all cases. This approach involves successive incisions through the layers of the lumbodorsal fascia to gain access to the kidney and ureter, and emphasizes a muscle-retracting technique. Incision of the costovertebral ligament allows 12th rib elevation and improves the exposure obtained. There were no postoperative complications related to the lumbotomy incision itself. Decreased surgical morbidity is suggested by our results. By 3 days postoperatively 64 per cent of our patients required no analgesic medication, 93 per cent were tolerating a regular diet and 83 per cent were evaluated as having a good appetite. Mean hospital length of stay for all lumbotomy patients was 5.6 days. We conclude that the dorsal lumbotomy incision can be used to perform a wide variety of upper tract operations in children, resulting in minimal morbidity and short hospital stays.

Adolescent↗

Yolk sac carcinoma of the testis in children.

We reviewed the records of 11 patients with yolk sac carcinoma of the testis seen at the Children's Hospital of Philadelphia from 1971 through 1983. Each child was less than 2 years old at diagnosis, and each had stage I disease (localized to the testicle). Initial management consisted of radical inguinal orchiectomy in all 11 patients. Four patients then underwent retroperitoneal node dissection and none had pathological evidence of retroperitoneal tumor spread. After primary surgical management 5 patients received no further initial treatment. Of these 5 patients 3 are alive with no evidence of disease at 1.5, 2.7 and 4.5 years after diagnosis. The tumor recurred in 2 patients, 1 of whom was salvaged with pulmonary radiation therapy and chemotherapy. The other 6 patients received chemotherapy postoperatively and only 1 has suffered relapse. Over-all, of 3 patients in whom pulmonary metastases developed 1 died of tumor and 1 died of treatment-related pneumonopathy. Our experience and that of others have led us to conclude that patients with localized disease and normal postoperative serum alpha-fetoprotein levels do not benefit from retroperitoneal node dissection, postoperative abdominal irradiation or chemotherapy. Patients with retroperitoneal nodal involvement, widely metastatic disease or recurrent disease can be treated successfully with chemotherapy and in some cases with radiation therapy.

Antineoplastic Combined Chemotherapy Protocols↗

Outpatient pediatric urologic procedures.

Outpatient pediatric urologic surgery is not a new concept. However, it has recently become extremely popular. The general experience nationwide and particularly the experience at Children's Hospital of Philadelphia and the University of Virginia Hospital indicate that outpatient surgery can be very safe and satisfactory for patients. The outpatient approach requires close cooperation among anesthesiologists, surgeons, and outpatient personnel. Excellent communication with the patient and family is essential. This includes extensive educational printed material and 24-hour availability by telephone. Once the infrastructure is in place, outpatient pediatric urologic surgery results in efficient use of facilities and a more pleasant surgical experience for the patient. The psychological, social, and economic benefits of outpatient surgery make it imperative that a large percentage of pediatric procedures be done on an ambulatory basis. We do not, however, intend to suggest that all minor procedures can be done on an outpatient basis, as certain special situations will mandate hospitalization.

Ambulatory Surgical Procedures↗

Ultrasound evaluation of scrotum in pediatrics.

Scrotal abnormalities are difficult to assess using clinical criteria alone. Ultrasound provides an accurate means of demonstrating the scrotal contents so that appropriate therapy may be instituted. In a retrospective study, 119 ultrasound examinations of 96 patients (aged 4 days to 23 years) have been compared with the clinical diagnosis, surgical/pathologic findings, and other imaging modalities. The gamut of disease identified included congenital anomalies, neoplasm, trauma, torsion, varicocele, hydrocele, epididymo-orchitis, epididymal cyst/spermatocele, and post-radiation fibrosis. The ultrasound findings correlated well in 93/96 patients. In inconclusive cases, sequential imaging helps differentiate traumatic and inflammatory lesions from neoplastic processes.

Adolescent↗

Continent urinary diversion: variations on the Mitrofanoff principle.

In 1980 Mitrofanoff described a continent urinary diversion, consisting of the bladder with a surgically closed bladder neck emptied by intermittent catheterization of the appendix, which had its distal end implanted in a submucosal nonrefluxing fashion in the bladder and its proximal end brought out as an inconspicuous stoma. Alternatively, Mitrofanoff described a high transureteroureterostomy in which the distal end of the ureter was brought out as a catheterizable stoma. The theme of this approach is the creation of an adequate capacity storage reservoir that is emptied intermittently by the catheterization of a small caliber conduit implanted in a nonrefluxing manner in the reservoir. In addition to the Mitrofanoff procedure, we have modified the principle in a variety of clinical situations and report 10 cases to demonstrate the value of this new concept in continent urinary diversion.

Adolescent↗

Primary site as a prognostic variable for children with pelvic soft tissue sarcomas.

From 1974 to 1983 we treated 16 children between 1 and 16 years old for soft tissue sarcoma arising in the pelvis, bladder or prostate. An incisional biopsy was obtained in every patient. Each child then was treated with a combination chemotherapy program, incorporating vincristine, actinomycin D and cyclophosphamide with or without doxorubicin, cis-platinum and etoposide. Of the 16 patients 13 (81 per cent) also received radiation therapy. In 8 children with urinary obstruction or hematuria sarcomas arose in the bladder or bladder-prostate region, including 7 who had localized tumors and 1 who had lung metastases at diagnosis. The median tumor diameter in these patients was 5 cm. Of these 8 patients 3 eventually required total cystectomy and prostatectomy to eradicate persistent local tumor, and 6 are alive and remain free of recurrent sarcoma for 1 to 9 years after initiation of therapy. The 8 other children had a pelvic mass at diagnosis, which arose adjacent to but outside of the bladder or prostate, and 2 had lung metastases at diagnosis. The median tumor diameter in these patients was 15 cm. Only 3 of these 8 children are alive and remain free of sarcoma for 1 to 8 years after initiation of therapy. In only 1 of these children was complete tumor excision ever possible despite the use of local radiation therapy and aggressive chemotherapy. Sarcomas arising in the bladder-prostate region are found when relatively small, perhaps because they soon produce overt signs, and they appear to have a better prognosis than those arising in the retroperitoneum-pelvis outside the bladder. Better treatment strategies are needed for the latter group of tumors that often are locally uncontrollable.

Adolescent↗

Outpatient pediatric urological surgery: techniques for a successful and cost-effective practice.

Of 674 operations performed in the pediatric urology division between January and October 1984, 62 per cent were done on an outpatient and 12 per cent on a 1-night stay basis. The outpatient procedures included major proximal hypospadias repairs and intra-abdominal procedures, as well as inguinal, penile, scrotal and endoscopic procedures. No child required subsequent hospitalization. This successful approach was contingent upon proper patient selection, patient and family education, modern anesthetic and monitoring techniques, and careful followup. For elective procedures, such as hypospadias repairs, patients are operated on when they are 6 to 18 months old. Modern anesthetic techniques include isoflurane to maintain a light level of general anesthesia and adjunctive regional blocking procedures. The availability of a physician support service on a 24-hour basis is fundamental to the success of this approach. Benefits include patient, parental and physician satisfaction, diminished potential for nosocomial disease transmission and avoidance of the emotional stress of parent-child separation. Increased use of outpatient surgery is a means to produce a significant decrease in the cost of medical care.

Ambulatory Surgical Procedures↗

Mayer-Rokitansky-Kuster-Hauser syndrome: US aid to diagnosis.

The Mayer-Rokitansky-Kuster-Hauser syndrome is composed of vaginal atresia with other variable Müllerian duct abnormalities such as bicornuate or septated uterus. The fallopian tubes, ovaries, and broad and round ligaments are normal. Unilateral renal and skeletal anomalies are associated in 50% and 12% of cases, respectively. Patients have a normal female karyotype and normal secondary sexual development. Previously, one had to rely on radiographic contrast studies and surgical exploration for accurate definition of the reproductive tract anatomy. The authors performed ultrasound (US) examinations on 12 patients, aged 5 days to 18 years, with the Mayer-Rokitansky syndrome. US allowed correct identification of the genitourinary anomalies found in these girls, including eight cases of unilateral renal agenesis; one absent, one rudimentary, and ten duplicated or obstructed uteri; eight duplicated or obstructed vaginas; and associated complications such as endometriosis. Eight of the patients had lower abdominal pain, often cyclical in nature. The findings demonstrate that high-resolution, real-time US in conjunction with water vaginography permits the anatomy of these complex anomalies to be defined.

Adolescent↗

Use of the Mitrofanoff principle in urinary reconstruction.

The catheterization of a urinary reservoir by way of a narrow conduit such as appendix or ureter has proved a very successful additional technique in urinary reconstruction. The Mitrofanoff principle can be added to our armamentarium in a variety of clinical settings. Closure of the vesical neck, as indicated in previous reports, is not necessarily required.

Adolescent↗

Testicular and sexual function in adults with prune belly syndrome.

Testicular and sexual function was investigated in 9 patients between 16 and 28 years old with prune belly syndrome. At personal interview all patients claimed to experience normal erections and orgasm but 7 had retrograde ejaculation. Examination showed a normally developed male habitus. Orchiopexies had been performed in late childhood or adolescence. Two patients were anorchic and the surviving testicles were small in the remaining 7. Serum testosterone was low in 1 patient and normal in 6 but at the expense of high luteinizing hormone levels in 4. All patients had a high serum follicle-stimulating hormone level, reflecting the azoospermia found in 5 patients tested. Three biopsies from atypical cases showed poor histological findings but 2 revealed early spermatogenesis. The testes in patients with prune belly syndrome are similar to undescended testes in otherwise normal men. Late orchiopexy produces sexually active but infertile men.

Adolescent↗

Single unilateral vaginal ectopic ureter: is it really a rarity?

Although previously regarded as a rarity, 13 of the 20 published cases of single unilateral vaginal ectopic ureter have been reported within the last year. We have seen 4 cases of a single unilateral vaginal ectopic ureter within a 4-month interval. The relationship of vaginal ureteral ectopia to müllerian and mesonephric duct anomalies is discussed. If the ureteral bud originates laterally from the mesonephric duct then the ureter may retain its connection with the mesonephric duct while failing to connect to the bladder. This condition combined with persistence of the distal mesonephric (Gartner's) duct, which ruptures vaginally, accounts for vaginal ureteral ectopia and incontinence. The diagnostic studies for vaginal ureteral ectopia include excretory urography, radionuclide scanning, ultrasonography and administration of oral phenazopyridine in conjunction with careful vaginoscopic examination. The combination of lifelong wetting and solitary kidney on excretory urography should alert the urologist to this syndrome. The single unilateral vaginal ectopic ureter may be more common than has been suggested previously.

Child↗

Ureteroceles in children: followup of management with upper tract approach.

Ureteroceles can be associated with obstruction and/or reflux to any of the involved renal units. Their management is controversial and the possible options are numerous. From a population of 58 children with ureteroceles we analyzed those patients who underwent upper pole heminephrectomy alone as to the secondary operative rate. Followup examination of those patients whose management was initiated with upper pole partial nephrectomy and partial ureterectomy revealed a higher than expected incidence of persistent reflux.

Child↗