Biomedical subjects
R D Jeffs
Publications and source records attributed to R D Jeffs.
Exstrophy and cloacal exstrophy.
Classic bladder exstrophy and cloacal exstrophy are grotesque anomalies in the eyes of young parents. An immediate evaluation by an experienced team is required to assess the variations in the condition and to devise a plan of treatment that can reassure the parents as to eventual prognosis and rehabilitation. This plan of treatment must include provisions for renal preservation, urinary control, cosmetic appearance, and sexual function. The newborn is usually normal in respect to nutrition and its cardiopulmonary state, and will tolerate surgery well. Passive parental immunity provides better resistance to intercurrent illness at this time than will be present in the later months of infancy. When applicable, the initial stages of surgery should be undertaken at birth when the pelvic ring can be approximated without osteotomy and the bladder mucosa has not deteriorated from inflammatory changes. Parental attitudes toward the child as well as successful reconstruction may both be best served by immediate surgery to begin reconstruction and reduce the visible defect.
Safe placement of nephrostomy tube.
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Multicystic dysplastic kidney.
We reviewed 29 cases of congenital multicystic dysplastic kidneys. Isolated renal pelvic atresia has an excellent prognosis but lower ureteral atresias are associated with a high incidence of contralateral renal disease and have a worse prognosis.
Surgical treatment of the massively dilated ureter in children. Part II. Management by primary reconstruction.
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Open ureterocolonic anastomoses.
A simple technique for open ureterocolonic anastomosis is presented. This technique provides adequate exposure for mucosal-to-mucosal approximation and allows the construction of an antireflux ureterocolonic anastomosis.
Ureteral structure and ultrastructure. Part III. The congenitally dilated ureter (megaureter).
A selected group of congenitally dilated ureters was examined under light and electron microscopy, and the findings were compared to the clinicoradiologic features of each ureter. These dilated ureters exhibit qualitative and/or quantitative muscular deficiencies. The obstructed megaureters secondary to posterior urethral valves and the megaureters with reflux show non-specific pathologic changes in response to increased work load or decompensation but infection within the ureter is detrimental to the muscle cells. The ureters in babies with prune belly syndrome suffer from reduced muscle cell population and a distinct myopathy involving the myofilaments within the muscle cells. The dysplastic ureter is morphologically and end stage ureter wherein the muscle cells are severely compromised. Whether this is an underdevelopment or exhaustion from repeated infections is a matter for speculation.
Ureteral structure and ultrastructure. Part IV. The dilated ureter, clinicopathological correlation.
The potential of electron microscopy in the study of ureteral dilatation is seldom exploited but ureteral biopsies from 29 congenitally dilated ureters were examined under light and electron microscopy. A correlation between the pathological findings and the result of the surgical correction of these ureters is shown. The prospects of ureteral functional recovery can be predicted when the muscle cell integrity is considered.
Surgical treatment of the massively dilated ureter in children. Part I. management by cutaneous ureterostomy.
One of the major therapeutic challenges facing the pediatric urologist is the massively dilated ureter. When conservative measures, such as relief of obstruction or treatment of infection, have failed or are likely to fail, surgical treatment must be directed at the massive dilatation itself. These circumstances were encountered in 366 massively dilated ureters in 244 children during a 10-year period. Loop and/or terminal cutaneous ureterostomy was used to manage 125 of these ureters in 74 children. Indications for temporary non-intubated proximal urinary diversion included uncontrolled infection, sepsis, azotemia, significant ureteral redundancy and tortuosity, and questionable over-all renal function. Advantages of loop ureterostomy included more proximal drainage and less stomal problems but terminal ureterostomy required a significantly lesser number of surgical procedures in reconstructing the urinary tract. Temporary splinting of the ureter in dry ureteral reimplantations has diminished markedly the incidence of ureterovesical complications. No instances of permanent vesical contracture were noted after long periods of defunctionalization. After initial reconstruction of 47 cutaneous ureterostomies good results by all parameters were obtained in 68 per cent. Additional surgical procedures in selected initial failures have resulted in a final success rate of 85 per cent.
The early assessment for individualized treatment in the prune belly syndrome.
Patients with prune belly syndrome present a spectrum of abnormality, both in the abdominal wall and the urinary tract. Ureteral pathology has characteristic features and the ureter may be more severely involved at the bladder end than in its upper portion. Early neonatal investigation is required to determine which patient can be treated in a conservative manner and which require neonatal reconstruction or temporary vesical or upper tract drainage.
Ureteral structure and ultrastructure.
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Functional closure of bladder exstrophy.
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Wilms' tumour: adjuvant treatment with actinomycin D and vincristine.
In an unselected series of 49 children with Wilms' tumour treated in 1969-74 the 5-year relapse-free survival and survival rates were 78% and 81%, respectively, whereas in the series of children treated in 1963-68 the corresponding rates were 49% and 70%. The significant improvement in the relapse-free survival rate was a result of adjuvant treatment with actinomycin D and vincristine (AMD + VCR), which, in some patients, eradicated occult metastatic disease. In the treatment of lung metastases the combination of whole-lung irradiation and maintained chemotherapy with AMD + VCR proved excessively toxic: in 5 of 11 patients acute diffuse pneumonitis developed, and it was fatal in 3. Adjuvant AMD + VCR therapy is advocated in all patients with Wilms' tumour except children less than 12 months old with a tumour of moderate size, limited to the kidney and completely resectable.
Ureteral structure and ultrastructure. Part I. The normal human ureter.
Twenty-eight normal human ureters were examined under the light and electron microscope. Three uretero-trigonal units were microdissected. The ureter is a non-layered muscular tube wherein the anatomical unit is muscle bundles that contain heterogeneously oriented muscle cells. Functional continuity of muscle cells is provided through the nexus, which are clearly defined structures. A group of morphologically distinct muscle cells are identified and these may represent the ureteral pacemaker cells. The functional status of muscle cells causes certain morphologic changes and, thus, the cells fixed in contraction differ from those fixed in relaxation and in vitro testing of muscle cell contractility is feasible. The ultrastructure of the normal urothelium and the adrenergic and cholinergic components of ureteral nerves are presented briefly.
Ureteral structure and ultrastructure. Part II. Congenital ureteropelvic junction obstruction and primary obstructive megaureter.
The structure and ultrastructure were studied of 48 specimens from cases of congenital ureteroplevic junction obstructions and primary obstructive megaureters. Under light microscopy a spectrum of findings occurs, extending from the nearly normal to the clearly abnormal obstructive segments. However, under electron microscopy there were consistent abnormalities: 1) excessive collagen fibers between and around the muscle cells and 2) a group of compromised muscle cells proximal to the collagenous segment. These findings are responsible for functional discontinuity via the nexus and indistensibility of the pathologic areas. The high resolution of the electron microscope allows clearer definition of these obstructions and the impact of these findings on surgical remodeling is discussed.
Surgical correction of vesicoureteral reflux in children with neurogenic bladder.
The reported success rate for correction of vesicoureteral reflux in the neurogenic bladder has been low. Reflux was corrected in 33 of 37 ureters in 23 selected children with neurogenic bladder-treated by reimplantation. Selection was based on good bladder capacity, little or no trabeculation and the expectation that the bladder would be useful in future management of the patient. Preoperative preparation and surgical technique are emphasized.
Wilms' tumour: treatment of 113 patients from 1960 to 1971.
The crude 5-year survival rate among children with Wilms' tumour increased from 54% for those diagnosed from 1960 to 1965 to 81% for the period 1966 to 1971. This resulted from an increased ability to cure metastatic disease and, to a lesser extent, to an increased ability to prevent relapse. It is proposed that, after resection and postoperative irradiation, maintained combination chemotherapy with actinomycin D and vincristine should be used electively to prevent relapse, but that this use should also be selective in order that overall morbidity be minimized. Of urgent priority, therefore, is improved delineation of present-day prognostic factors in children with Wilms' tumour.
Primary obstructive megaureter in children.
Sixty-five cases of primary obstructive megaureter were retrospectively evaluated. The clinicoradiologic features of the abnormality are characteristic; however, a complete urologic workup is necessary to exclude the more common causes of ureteral dilatation. Treatment must be planned according to status of renal and ureteral functions. Surgical excision of the obstructive segment, reduction of ureteral caliber when indicated, and reimplantation achieved the best results in this series. A brief period of nephrostomy drainage prior to surgical remodeling of the lower ureter can be useful in selected cases. Conservative treatment of children with relatively few symptoms and slight ureteral dilatation is worthwhile.