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Biomedical subjects

W Hardy Hendren

Publications and source records attributed to W Hardy Hendren.

6 recordsLinked to original sources
PubMed2005

Early outcome following complete primary repair of bladder exstrophy in the newborn.

PURPOSE: Complete primary repair of bladder exstrophy (CPRE) represents a paradigm shift from the staged approach for surgical management. We present early clinical outcomes in our patients following CPRE. MATERIALS AND METHODS: From 1996 to 2004 all newborns with bladder exstrophy were treated with CPRE within 48 hours of birth. We reviewed parameters including transfusions (packed red blood cells), urethral meatal position, complications, findings on renal ultrasound and renal scan, and post-CPRE procedures and infections. RESULTS: A total of 16 boys and 7 girls were treated with CPRE. Followup ranged from 8 to 96 months. Twelve boys (75%) and 2 girls (29%) received packed red blood cells at CPRE (p = 0.066). The tubularized urethral plate could not be brought to the penile tip, resulting in hypospadias in 9 of 11 boys (82%) with the running suture technique and only 1 of 5 boys (20%) with the interrupted technique (p = 0.036). Of the 23 patients 6 had a total of 8 complications after CPRE. Vesicoureteral reflux was present in 17 of 23 patients. After CPRE a total of 93 endoscopic/surgical procedures (median 4, range 0 to 16 per patient) were performed. Five patients had 1 to 4 episodes of pyelonephritis, 16 of 23 had 1 or more episodes of asymptomatic bacteriuria and 5 had cortical defects on renal scan. CONCLUSIONS: We recommend that urethral closure during CPRE be performed with interrupted suture technique to prevent hypospadias. An aggressive approach should be taken toward reflux in the setting of urinary infection. Consideration should be given for repair of all aspects of the defect during CPRE, including bilateral ureteral reimplantation.

Bladder Exstrophy↗
PubMed2005

Bladder growth and development after complete primary repair of bladder exstrophy in the newborn with comparison to staged approach.

PURPOSE: We assessed bladder growth and dynamics following complete primary repair of bladder exstrophy (CPRE) compared to the staged approach. MATERIALS AND METHODS: We reviewed the records of 16 boys and 7 girls who underwent CPRE within 3 days of life from 1996 to 2004 and compared them to the records of 8 boys and 6 girls treated with a staged repair from 1979 to 1996. Screening methods included voiding cystourethrogram, radionuclide cystogram and urodynamic study. We estimated growth curves for bladder capacity following repair in each group, and compared percent predicted bladder capacity (PPBC), compliance and detrusor overactivity between the CPRE and staged repair groups following bladder neck reconstruction. RESULTS: Bladder capacity in the staged repair group was 69.8 ml (95% CI 46.7-104.4) immediately after bladder neck reconstruction and increased by 15.0% per year thereafter (95% CI 6.2-24.5, p = 0.002). In the CPRE group bladder capacity was 29.0 ml (95% CI 21.3-39.5) initially and increased by 28.9% per year thereafter (95% CI 17.4-41.5, p <0.001). PPBC started at 45.6% (95% CI 35.7-55.5) and increased 1.2% per year (95% CI -1.1-3.5, p = 0.29) following repair for all genders and surgery groups. Compliance was 124.4% (95% CI 22.6-310.7, p = 0.01) greater in the CPRE group at all times following repair. Detrusor overactivity was present in 0 of 19 patients in the CPRE group and 6 of 13 (46%) in the staged group (exact p = 0.002). CONCLUSIONS: Within the CPRE group bladder stability was universal, and sphincter electromyography was normal suggesting no neuromuscular compromise of the pelvic floor. At early followup, our results suggest that PPBC is equivalent irrespective of gender or management. Further objective evaluation is needed in both groups.

Bladder Exstrophy↗
PubMed2003

Congenital adrenal hyperplasia: surgical considerations required to repair a 46,XX patient raised as a boy.

21-hydroxylase deficiency (P450 CYP21) accounts for 90% of cases of congenital adrenal hyperplasia (CAH), which is associated with abnormally low cortisol and high production of androgen precursors and is the most common cause of ambiguous genitalia. Increased androgen causes in utero virilization of the fetus, consisting of clitoral enlargement, an urogenital sinus, and labioscrotal enlargement and fusion. This is the first case in an experience covering more than 30 years, of a 46,XX patient raised as a boy. The authors report a case of a Pakistani patient born of a consanguineous union, who came to medical attention at age 3 because of severe genital ambiguity; genetic analyses showed that the child was a compound heterozygote for CAH. The surgical management of this patient consisted of (1) staged hypospadias repairs preceded by testosterone therapy, (2) creation of a bladder graft neourethra, (3) removal of müllerian structures, (4) correction of bifid prepenile scrotum, and (5) insertion of testicular prostheses. The commitment to raise a 46,XX child as a boy is a very rare event. With a series of staged complex surgical procedures and careful steroid replacement, normal secondary sexual characteristics can be achieved in these children.

Adrenal Hyperplasia, Congenital↗
PubMed2003

Triplets after cloacal malformation repair.

BACKGROUND: Patients with cloacal malformations at birth usually require multiple surgical procedures to correct their anatomic defects. In addition, many also have associated Müllerian anomalies. Those who conceive after repairs invariably are considered "high-risk" pregnancies and are considered poor candidates for maintaining multiple gestations. Further, because of the nature of their defects and their repairs, following such patients with multiple gestation presents unique challenges. CASE: A 29-year-old multipara conceived triplets and delivered at 30 weeks with a good maternal and neonatal outcome. CONCLUSIONS: Patients with repaired cloacal abnormalities present unique challenges and risks compared to the general population with regard to the risks of multiple pregnancies.

Adult↗
PubMed2003

Fibroepithelial polyps causing ureteropelvic junction obstruction in children.

PURPOSE: Fibroepithelial polyps are benign mucosal projections that can be found throughout the urinary system. We review our experience with fibroepithelial polyps of the ureteropelvic junction in children to define more clearly this entity and its outcome following treatment. METHODS: We reviewed the records of all children with fibroepithelial polyps causing ureteropelvic junction obstruction treated at our institution between December 1967 and February 2002. RESULTS: Nine patients 6 weeks to 9 years old had 11 ureteropelvic junction obstructions secondary to fibroepithelial polyps, representing a 0.5% incidence of all ureteropelvic junction obstructions seen during that period. The majority of the patients were male (89%) and had obstruction on the left side (78%). Only 22% of the patients had a diagnosis of obstructing polyps suggested preoperatively. All patients underwent dismembered pyeloplasty but 1 required subsequent nephrectomy due to progressive loss of renal function. All lesions were characterized microscopically by polypoid projections of fibrous tissue covered by epithelium and demonstrating varying degrees of inflammation. No recurrences were seen during a mean followup of 44 months. CONCLUSIONS: Fibroepithelial polyps are rare lesions that cause ureteropelvic junction obstruction in children, primarily in males and on the left side. Filling defects were diagnosed preoperatively in 22% of the patients in this series. Excision and dismembered pyeloplasty were curative, and recurrences were not observed. The etiology of this disease remains obscure.

Child↗