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Biomedical subjects

C B Manley

Publications and source records attributed to C B Manley.

14 recordsLinked to original sources

Prune belly syndrome in females: a triad of abdominal musculature deficiency and anomalies of the urinary and genital systems.

We describe seven female patients with deficient abdominal wall musculature and urinary tract and genital anomalies that represent the female equivalent of the prune belly syndrome. Urethral atresia, uterine duplication, and anorectal anomalies occurred frequently. The perinatal mortality rate was high; of the four surviving patients, renal failure developed in two and renal transplantation was required. The analysis of these cases suggests that urethral obstruction is an important factor contributing to the development of the prune belly syndrome in females.

Abdominal Muscles

Ischiopagus tetrapus twins: urological aspects of separation and 10-year followup.

Conjoined twins occur once in 50,000 births. Only 6% of conjoined twins are of the ischiopagus type in which the twins are joined symmetrically at the pelvis and fusion begins at the level of the common umbilicus. The longitudinal axis extends in a straight line in opposite directions and the genitourinary and gastrointestinal tracts are shared. Tetrapus is a subtype in which all 4 lower extremities are present and oriented at right angles to the axis of the common trunk. Two sets of female ischiopagus tetrapus twins were born in 1977 and successfully separated at the St. Louis Children's Hospital in the following year. We describe the genitourinary and associated anomalies, surgical separation and long-term urological followup of these 2 sets of ischiopagus tetrapus twins.

Abnormalities, Multiple

Endopyelotomy for secondary ureteropelvic junction obstruction in children.

Percutaneous endopyelotomy has been shown to be successful in treating ureteropelvic junction obstruction in adults. Little data have been published regarding this procedure in children. We describe 4 patients 6.5 weeks to 5.5 years old who underwent percutaneous endopyelotomy to treat ureteropelvic junction obstruction following failed open dismembered pyeloplasty. Preoperative obstruction was demonstrated by a nephrostogram, diuretic renogram and/or ultrasonography. Percutaneous endopyelotomy was successful in relieving the obstruction in all 4 patients, although 2 required secondary endoscopic procedures. One patient had persistent obstruction 40 days after endopyelotomy at the ureteropelvic junction and, subsequently, required percutaneous resection of a persistent flap of obstructing tissue. In another patient a ureterovesical stricture was noted at the time of stent removal, which was treated by endoscopic incision. All patients have been followed from 1.5 to 3 years postoperatively. Followup diuretic renograms, ultrasound and/or excretory urography demonstrated a patent ureteropelvic junction in all patients and all have remained asymptomatic. Endopyelotomy appears to be safe and effective in treating secondary ureteropelvic junction obstruction in children.

Child, Preschool

Caudal epidural anesthesia reduces blood loss during hypospadias repair.

We studied 24 boys who were randomized to receive caudal epidural anesthesia with 0.33 ml.kg.-1 0.25% bupivacaine either before (group A) or after (group B) Mathieu repair of distal hypospadias. The 2 groups did not differ in regard to age or weight, and all boys received a standardized anesthetic with halothane and nitrous oxide in oxygen. Intraoperative blood loss was measured with sponge weights and microcalibrated suction canisters. Halothane requirements were reduced in group A (0.5 versus 1.1 plus or minus 0.1%, p less than 0.001). Measured blood loss was reduced in group A (16 plus or minus 10 ml. versus 31 plus or minus 17 ml., p less than 0.01). Operating time was reduced in group A (92 plus or minus 13 minutes versus 103 plus or minus 14 minutes, p less than 0.05). There was no apparent difference in postoperative pain relief between the 2 groups. We conclude that caudal epidural anesthesia can reduce blood loss and improve surgical conditions during hypospadias repair.

Anesthesia, Caudal

Bilobed testicle.

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Adolescent

Acute ureteral obstruction secondary to bullous cystitis of the trigone: report of 2 cases.

Two boys presented with acute bullous cystitis limited to the trigone and periureteral zone and producing marked but transient acute ureteral obstruction. One patient was anuric and likely would have died without intervention. Cystoscopy in both cases showed localized bullous edema of the bladder mucosa; biopsy showed acute inflammation, mucosal edema, and lymphangiectasis. Radiological findings included distal ureteral obstruction and numerous nodular filling defects in the bladder, caused by the edema. The etiology of the cystitis could not be determined in either case.

Acute Disease

Prognosis for resolution of moderate, primary reflux in girls.

Detailed cystoscopic grading of ureteral orifices has proved unnecessary in our experience for determining the prognosis of moderate, primary reflux in girls. Medially placed orifices strongly predict spontaneous resolution with preventive medication, while radiographic pyelonephritic scarring and/or grade III reflux strongly predict failure to resolve. While laterally placed ureteral orifices and low volume reflux suggest a need for operation, they are prognostically less useful, particularly when those with grade III reflux are excluded. Cystitis cystica and unrelated anomalies seem to be associated with the ultimate need for operation but are of uncertain significance.

Child