Biomedical subjects
Publications and source records attributed to B E Hardy.
We constructed a continent ileal urinary reservoir (Kock pouch) in a 9-year-old boy. The length of bowel required to achieve a satisfactory outcome was scaled down for this young, small child.
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Seventeen children with renovascular hypertension caused by intrinsic renal artery lesions received treatment during the past 10 years. At presentation nine were asymptomatic, four had headaches, and one had epistaxis; three infants had anorexia and failure to thrive. Routine intravenous pyelogram and radionuclide renal scan findings were abnormal in 29% and 31% of patients, respectively. Arteriography showed a branch artery stenosis in seven patients and a main artery lesion in 10. A renal vein renin ratio of greater than or equal to 1.5 between the affected and the contralateral kidney was obtained in 10 of 17 patients. Of 16 patients available for follow-up, 15 are normotensive after a mean follow-up of 3.7 years. Cure was achieved by partial nephrectomy and ligation of a stenosed vessel in two and nephrectomy in five (three having undergone an unsuccessful angioplasty procedure). Autotransplantation or angioplasty was curative in a further six. Transluminal balloon angioplasty was attempted in seven patients but was successful in only two with main renal artery stenoses. With preservation of renal parenchyma as the main goal, medical and surgical therapy can be individualized for each patient.
The records of 170 patients with unilateral and 18 patients with bilateral Wilms tumor and 6 patients with congenital mesoblastic nephroma were reviewed for abnormalities of the external genitalia. There were 4 patients with cryptorchism, 1 with hypospadias, 1 with mixed gonadal dysgenesis, and 3 with male pseudohermaphroditism. Of the group, these 9 patients had earlier symptoms; 6 of them were under two years old. Five patients (27.7%) with bilateral Wilms tumors had external genital anomalies in contrast to only 4 (2.3%) of those with unilateral tumors. One of the 9 children with genital anomalies had unilateral congenital mesoblastic nephroma; the others had Wilms tumor. In all cases the histologic subtypes were unusual. These observations substantiate the previously reported association between external genital abnormalities and Wilms tumor and also represent a somewhat higher than expected incidence. The children with this association are remarkable for the high frequency of a particular histologic type of tumor and for the high incidence of bilaterality. The high incidence of Wilms tumors in the male pseudohermaphrodite population presented suggests that such patients should be monitored for development of renal tumors.
We found persistent müllerian duct structures in 2 phenotypically normal brothers who had undergone an operation for inguinal hernia. The older boy had transverse testicular ectopia and his younger brother had unilateral cryptorchidism. Bilateral orchiopexy was done with excision of the fallopian tubes, uterus and upper vagina. Histological examination showed normal testes in both patients. Since there is a risk of testicular malignancy in such patients, long-term followup is mandatory.
Three boys were treated for arteritis of the aorta and great vessels and bilateral renal artery stenosis. One presented at age 6 months with failure to thrive, excessive sweating, and vomiting: hypertension and cardiac failure were subsequently diagnosed. The two older boys (7 and 14 years) presented with symptomless hypertension. The clinical and angiographic findings in the three patients suggest that the illness may have been Takayasu's arteritis, which should be included in the differential diagnosis of hypertension in infancy and childhood. Renal autotransplantation was performed in all three patients with good results. Early renal autotransplantation may reduce the morbidity associated with this disease.
Experience with male pseudohermaphrodites at The Hospital for Sick Children, Toronto, from 1976 to 1981 was reviewed to identify factors that had influenced gender assignment. Seventeen children with microphallus, ambiguous genitalia, and 46 XY karoytype were studied. Five had been raised as females and 12 as males. Of those raised as females, 3 were from families with prior experience of androgen insensitivity. The other 2 had gross perineal abnormalities in addition to genital ambiguity. Of those raised as males 6 were not considered for gender reassignment, 4 because they were too old at presentation, and the other 2 because of parental refusal. In this series the parents were not prepared to accept gender reassignment on the basis of a small phallus alone. Androgen stimulation so far has been a reliable indicator of phallic growth potential, but long-term follow-up is necessary to determine its true value.
Thirty-five children with anomalies of the urachus which have required surgical management have been encountered in this institution over a 20-year period. Of these 35, 19 were classified as patent urachus, 12 as urachal cyst, and 4 as urachal sinus. All cases were treated by excision or drainage. There was one death. Gastrointestinal and other genitourinary anomalies were commonly seen in these patients. Intravenous pyelography was helpful in identifying unsuspected associated genitourinary anomalies while cystography was not. Investigation of the gastrointestinal tract does not appear to be warranted in the absence of symptoms.
To determine the important factors involved in the etiology of renal scarring we studied 37 children with renal scars seen at our hospital since 1965. This is the second largest series reported to date. Children who had neurogenic bladders or any structural abnormalities of the urinary tract other than vesicoureteral reflex were excluded. The study group included 36 girls and 1 boy. The average age at first detection of renal scars was 5.7 years. Acute pyelonephritic episodes, which were treated early and aggressively, infrequently led to renal scarring. However, the initial prolonged or poorly treated episode of acute pyelonephritis was followed invariably by the development of renal scarring. The severity of renal scarring was related to the grade of vesicoureteral reflux (p less than 0.05), although some scars did develop in the absence of reflux. Neither the shape and position of the ureteral orifice nor the ureteral tunnel length correlated with the severity of renal scarring. Treatment with prophylactic antibiotics may have lessened the severity of renal scarring (0.1 less than p less than 0.2) but treatment with reimplantation surgery did not appear to alter the course of renal scarring. This study suggests that the key to the prevention of renal scarring is the early and aggressive treatment of acute pyelonephritis.
Thirty-nine cadaveric renal allografts were performed in 28 children under 6 years of age. Common primary renal diseases were glomerulonephritis, dysplasia/hypoplasia, and reflux/obstructive nephropathy. After a mean follow-up of 40 months of patients with surviving grafts, 19 patients had functioning grafts, 3 had returned to dialysis, and 6 had died. These children required an extraordinary amount of care postoperatively because of anorexia, diarrhea, and ileus. Their psychomotor and physical development was retarded prior to transplant; this reversed dramatically after transplant, but catch-up growth occurred in only 4 patients. Many patients were noticeably more active and distractible for 1 to 2 years post-transplant. Major causes of graft failure were primary nonfunction of 5 donor kidneys (4 from donors under 1 year old) and renal vessel thrombosis in 5 recipients (3 with native kidneys in place who received kidneys from donors over 10 years old). Other causes were recurrence of hemolytic uremic syndrome and Wilms tumor, rejection, and sepsis. Kidneys from donors under 1 year old proved unsatisfactory, and large donor kidneys in small children tended to thrombose, especially when native kidneys with high urine output were left in situ.
The treatment of patients with posterior urethral valves has improved dramatically in recent times. Our personal experience with 207 patients with posterior urethral valves between 1957 and 1978 reveals a striking reduction in both mortality and morbidity during the latter decade. We have attempted to identify clearly the incidence, etiologic factors, preventive measures, and surgical treatment of complications arising in these patients. The overall improvement in management is certainly attributable to a growing experience and the constant advancements in the medical and surgical armamentarium. Our next goal at this time is to maximize long-term renal function and growth potential in these patients.
One hundred and nine children with urinary tract calculi were reviewed and in some cases reinvestigated. Eighteen children had lower urinary tract calculi, which in all cases were associated with an underlying urodynamic abnormality. Sixty percent of 91 children with upper urinary tract calculi could be classified into 4 similarly sized etiological groups: an underlying urodynamic abnormality; urinary tract infection without a urodynamic abnormality; metabolic disorders; idiopathic hypercalciuria. An underlying abnormality was not found in 32% of cases. A painless presentation occurred in 39% of those with upper tract calculi. A family history of urinary calculi occurred in approximately one-half of children with either an idiopathic calculus or a calculus associated with cystinuria or idiopathic hypercalciuria. We conclude that urinary tract calculi, though rare in children, require extensive investigation to rule out urodynamic, infective and metabolic abnormalities. If such abnormalities are not found, the recurrence rate in the remainder is small and conservative treatment can usually be recommended.
In this study we compare the cosmetic and functional results of the common reconstructive techniques used in the management of patients with clitoral hypertrophy. We find impressive benefits in a surgical procedure that includes excision of the clitoral shaft with preservation of the glans and ventral mucosa combined with simple introitoplasty. We recommend reserving definitive vaginoplasty as needed until the age of puberty. Our experience is this clinical area is being offered with attention to the long-term followup of patients.
Clean intermittent catheterization was instituted in 34 myelodysplastic children in an attempt to improve upper urinary tract changes, reflux, recurrent urinary tract infections or inefficient bladder evacuation. Compliance was excellent and no serious complications were encountered. Upper tract changes and reflux responded favorably to the procedure and the incidence of urinary tract infections decreased. The use of clean intermittent catheterization even in neonates is feasible and safe, and produces excellent preservation of urinary tract integrity.
A significant number of neonates with myelomeningocele are born with potentially damaging abnormalities of the urinary tract. Therefore, it is logical to investigate these patients aggressively and initiate therapy as early as possible to preserve renal function and maintain lower tract integrity for future continence management. Results of excretory urograms and voiding cystourethrograms were reviewed in 68 consecutive neonates with myelomeningocele. They indicate clearly that only a third of the patients with normal excretory urograms had a normal voiding cystourethrogram, which implies that both studies be performed for complete evaluation of the urinary tract. In other words, of these 68 patients 55 (80.9 per cent) had a normal excretory urogram but only 17 (30.9 per cent) had a normal voiding cystourethrogram. Of the 13 patients (19.1 per cent) with an abnormal excretory urogram 12 (92.3 per cent) had an abnormal voiding cystourethrogram. Reflux occurred in 16.2 per cent of patients; 6 of 7 patients with grade II reflux and 1 of 3 patients with grade III reflux had a normal excretory urogram.
As an alternative to the practice of obtaining repeated laboratory cultures for patients at high risk of renal impairment from recurrent UTI, a program of bone monitoring using a mail-in culture dipspoon was started. A study involving 454 children with neurogenic bladder or other urologic abnormalities showed (1) that the incidence of UTI infection in patients for whom long term antimicrobial therapy had been prescribed was not significantly lower than that in patients who were not on antimicrobials and (2) that at least 50% of dipspoons inoculated due to presentation of UTI symptoms showed no or insignificant growth. These findings suggest that need for further assessment of the efficacy of long term prophylactic antimicrobials in preventing recurrent UTI and the advisability of obtaining a urine culture result before initiating treatment when symptoms are not severe.
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