Biomedical subjects
Publications and source records attributed to A W Middleton.
A 13-year-old girl with Turner's syndrome and bleeding from intestinal venous ectasia is reported. The various types of vascular anomaly of the bowel associated with Turner's syndrome are discussed. Awareness of these anomalies may help prevent unnecessary laparotomy in children with this syndrome.
A review of the literature reveals 9 cases of spontaneous renal rupture in pregnancy, to which we add another case. Most cases occurred in hydronephrotic kidneys. Four cases consisted of renal rupture without associated infection, or tumor, 3 were associated with abscesses, and 3 ruptured through renal hamartomas. Previously, all ruptured kidneys were removed. In our case the kidney was salvaged satisfactorily by prompt placement of a nephrostomy tube and drainage of the perirenal tissue. Attempt at renal salvage is urged for renal supture in pregnancy.
A series is reviewed of 153 pyeloplasties in 130 pediatric patients. Dismembering pyeloplasty was performed in most cases, usually with temporary nephrostomy drainage. A satisfactory anastomosis resulted in each case. The important technical aspects of pyeloplasty are emphasized.
In a retrospective review of all excretory urograms and voiding cystourethrograms performed in a 1-year period in 391 children evaluated with a history of urinary infection 56 (14 per cent) had vesicoureteral reflux as well as a normal excretory urogram. Of the 89 children in the study with vesicoureteral reflux 56 (63 per cent) had a normal excretory urogram. The grade of reflux was II or greater in 49 per cent of the cases, which also had a normal excretory urogram. We conclude that an excretory urogram without a voiding cystourethrogram is an inadequate study for a child with a history of urinary infection.
The Whitaker test, a urodynamic study, combined with antegrade pyelography has been used recently to evaluate persistent upper urinary tract dilatation after operative correction of obstruction. This test will differentiate patients with residual or recurrent obstruction from those with dilatation secondary to permanent changes in the musculature. It is useful in evaluating patients with questionable ureteropelvic or ureterovesical junction obstruction, or primary defects in the ureteral musculature, such as prune-belly syndrome. It also will establish when urinary diversion safely can be discontinued in postoperative patients. Percutaneous puncture of the renal pelvis is performed or an indwelling nephrostomy tube is used. The upper urinary tract is then perfused at a constant rate of 5--10 ml/min with saline or diluted contrast media, and a serial pressure recording is made in the renal pelvis and bladder. The high flow rate used will be tolerated easily in a nonobstructed system without a progressive rise in renal pelvic pressure. In obstructed systems abnormally high pressure above 12 cm water or a constant rise in pressure will be recorded. Videotaping of ureteral peristalsis and spot films of the upper urinary tract complete the evaluation.
We find that, when metastases are present, a nephrectomy for renal cell carcinoma is not justified in the vast majority of cases. The only noncontroversial indication is for palliative nephrectomy which is performed to relieve intractable symptoms in the properly chosen patient. However, it is unusual to see a patient with tumor-related symptoms that cannot be conservatively managed. A relative indication for nephrectomy is found in the patient with osseous metastases only, as some studies have demonstrated a prolongation of survival by adjunctive nephrectomy in this setting. Another relative indication is in the patient with limited metastases that are amenable to surgical or radiation ablation coupled with nephrectomy; patients in whom the metastasis appears years after the nephrectomy have the best chance for a successful outcome. When therapy that is effective against metastatic tumor is eventually found, adjunctive nephrectomy as a debulking procedure may become indicated; until then, surgery is not justified. On the basis of data presently available, adjunctive nephrectomy in the hope of inducing spontaneous regression of metastases is never indicated.
Tumors in 23 patients who presented with a low grade, non-invasive transitional cell carcinoma of the bladder were studied for blood group antigens A, B or O on the cell surface. Of 14 patients without cell surface antigens initially 13 suffered an invasive tumor subsequently and 1 had diffuse carcinoma in situ. Of 9 patients with cell surface antigens initially 8 did not have an invasive recurrence during a 5 or more-year followup and 1 did. The presence or absence of blood group cell surface antigen on a low grade, non-invasive transitional cell carcinoma of the bladder would seem to have value in predicting future recurrence with muscle invasion.
Over a one-year period 216 children had a radiographic survey of their urinary tracts performed for the evaluation of enuresis with many of these having a precedent history of urinary infection as well. Significant urinary tract abnormalities were found in 27 per cent of the children. Clinical correlation was obtained in 135 of these children with 19.3 per cent requiring surgery. The groups of children with a history of diurnal enuresis or urinary infection were quite different from the group with nocturnal enuresis alone. These differences are discussed.
Three cases are reported in which ileum has been used to replace irreparably damaged ureters despite preexisting moderate to severe pyelocaliectasis. Two cases have had satisfactory results while one had increasing pyelocaliectasis and azotemia requiring subsequent cutaneous diversion. The rationale for using ileum to replace ureter is presented with a discussion of objectives to be achieved surgically to assure the success of the procedure.
Radical retropubic prostatectomy with bilateral pelvic lymphadenectomy for prostatic carcinoma was done with pubectomy in 8 patients and without pubectomy in 8 other patients. A comparison of the postoperative courses reveals an excess morbidity in patients who had pubectomy, with severe protracted pelvic girdle pain in 75 per cent, lower extremity thrombophlebitis in 37 per cent (one of which resulted in a non-fatal pulmonary embolus) and varying degrees of urinary incontinence persisting for 6 months or more in 75 per cent. In contrast, in patients who did not have a pubectomy mild transient pelvic girdle pain was noted in only 25 per cent of the cases, late lower extremity thrombophlebitis in 12 per cent and no late urinary incontinence. Pubectomy should not be used routinely with radical retropubic prostatectomy.
The 45 ileal conduits performed on children at the Massachusetts General Hospital from 1955 to 1963 are reviewed and compared to the 45 ileal conduits performed from 1964 to 1970. Late complications involving the conduits occurred in 60 per cent of the early group and in 51 per cent of the late group. Of the renal units judged normal pyelographically preoperatively in the early group 77 per cent went on to at least some deterioration, while 62 per cent of the late group judged normal later deteriorated. Combining all renal units, 34 per cent remained unchanged, 26 per cent improved and 41 per cent showed some degree of deterioration after ileal conduit urinary diversion. The late complication and renal deterioration rates seem to increase progressively with time. There was no apparent urinary obstruction in 13 per cent of the renal units that deteriorated. Theoretical and experimental considerations indicate the reflux of infected urine as the etiology of the renal deterioration. Because of the late complications and the unacceptably high rate of renal deterioration we no longer perform ileal conduits in children. Instead every effort is made to reconstruct the urinary tract or if urinary diversion is necessary, a colon conduit with non-refluxing ureterocolonic anastomoses is performed.
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Congenital agastria has never before been reported. Congenital microgastria is rare but is well documented. We have cared for a Melanesian child in whom there was no radiological, anatomical, or physiological evidence of either a rudimentary stomach or a pylorus. The esophagus joined the first part of the duodenum directly. At this esophago-duodenal junction there was microscopic evidence of fundic-type gastric mucosa. The intestine was normally rotated and there was a functioning spleen. The child also had a severe micrognathia and a cleft soft palate and required a tracheostomy to relieve upper airway obstruction. The combination of micrognathia and agastria presented a difficult nutritional delivery problem. A jejunal pouch was constructed and placed between the esophagus and duodenum to provide some storage capacity. The child grew and developed well for almost 3 years. He suffered three episodes of severe acute enteritis, probably related to lack of the protective acid-pepsin barrier against ingested microorganisms. Unfortunately, the third episode of acute enteritis caused his death 3 months after returning home to his own country.