Biomedical subjects

W H Hendren

Publications and source records attributed to W H Hendren.

At least 19 recordsLinked to original sources

PubMed1992

Pneumatocele complicating hyperimmunoglobulin E syndrome (Job's Syndrome).

The case of a boy with hyperimmunoglobulin E syndrome or Job's syndrome is presented to demonstrate the occurrence of pneumatoceles in this syndrome as well as their unusual natural history and failure to spontaneously resolve. Surgical resection was required for two complications in this patient, persistent bronchopleural fistula and a pulmonary abscess that destroyed one lung and required pneumonectomy. Pathologic examination of the specimens demonstrated the wall of the cysts consisted of granulation tissue with chronic active inflammation surrounded by infarcted pulmonary parenchyma with coagulative necrosis. The mechanism responsible for increased immunoglobulin E production in this syndrome is unknown, as is the manner in which elevated immunoglobulin E levels impair normal immune function.

Aspergillosis↗

PubMed1992

Cloacal malformations: experience with 105 cases.

A personal experience with 105 cloacal malformations is described, showing the wide range of anatomy that can occur with the urogenital sinus, the vagina(s), and the rectum, as well as in the external features of the perineum. Follow-up is presented on 98 patients, excluding 4 who died preoperatively, 2 who are preoperative, and 1 lost to follow-up but never repaired. Fifty-seven patients had vesicoureteral reflux. Data are given on urinary control, bowel control, and sexual function for those who are now adults. Two have had babies. Operation often requires multiple positions, including the posterior sagittal approach, laparotomy, and lithotomy position. Isolated rectal pull-through should never be performed in these patients, because the urogenital aspects of the malformations are the most difficult to repair and are the most life-threatening to the patient. Six recent cases are presented to illustrate the breadth of the cloacal spectrum. In most of these patients a satisfactory functional result can be achieved for urinary and bowel control as well as sexual function.

Abnormalities, Multiple↗

PubMed1992

Ileal nipple for continence in cloacal exstrophy.

Since 1985, 7 children 2 to 17 years old with urinary incontinence from cloacal exstrophy underwent bladder augmentation and creation of a small bowel nipple at the bladder outlet. There were 3 genetic female and 4 genetic male patients being reared in the female role. In 3 early cases augmentation was done with small bowel, while gastric augmentation was used in 3 later cases and 1 had both types. Six patients became completely dry and they empty by intermittent self-catheterization. In the youngest patient the operation failed because the nipple became infarcted and disappeared. Reoperation will be performed after at least 1 year has passed. In cloacal exstrophy if there is insufficient local tissue to create a bladder neck and urethra, a reversed, catheterizable bowel nipple may be an option to consider.

Abnormalities, Multiple↗

PubMed1992

Perforation of the augmented bladder.

In 12 of 264 children treated with enterocystoplasty 15 spontaneous perforations occurred. Of the 12 children 9 had myelodysplasia. All segments of the gastrointestinal tract were used for the augmentation and most were detubularized. Surgery to increase bladder outlet resistance was done in 8 cases. At the time of each perforation 9 children had sterile cultures, however, 3 died of overwhelming sepsis. Presenting signs included abdominal pain in 8 cases, septic shock in 4 cases and shoulder pain in 4 older myelodysplastic children with diaphragmatic irritation from escaping urine. Cystography demonstrated a leak in 10 of 11 cases. Urodynamic studies revealed good compliance with low maximum filling pressure in 8 of 10 children. Hyperreflexia was noted in only 5 cases and outlet resistance greater than 85 cm. water was demonstrated in 5. Histological analysis showed changes in the bowel wall consistent with ischemia but suture granulomas were present in areas adjacent to the perforation site or thinned areas in biopsy or autopsy specimens. In addition to the theory that overdistention may cause enterocystoplasty perforation, current detubularization techniques may produce areas of relative ischemia, which become accentuated when the augmented bladder is distended beyond a reasonable volume.

Adolescent↗

PubMed1992

Chordee without hypospadias: experience with 33 cases.

We treated 33 patients 6 months to 19 years old with penile chordee without hypospadias from 1966 to 1990. In all cases the penile shaft was degloved, the urethra was widely mobilized and chordee was resected. The shaft skin then was closed, usually using Byars' flaps to shift some preputial skin ventrally. This method sufficed to straighten the penis in 10 patients. In 3 patients urethral mobilization plus placement of a dermal graft to the shaft accomplished straightening of the penis. A total of 20 patients also required lengthening of the penile urethra. Lengthening was done with a graft taken from the prepuce in 14 patients, bladder in 2 and arm in 4. Four of those patients also required a dermal graft to the shaft. There were 8 complications in 7 patients, including anastomotic stenosis, ballooning of a graft, balanitis xerotica obliterans in a graft, urethral fistula and persistent chordee. Reoperation corrected each complication. All end results were satisfactory.

Adolescent↗

PubMed1990

Rectal duplications.

Recent experience with two cases of rectal duplication, which had been misdiagnosed as hemorrhoids, or fistula-in-ano with resultant delay in diagnosis, prompted us to review our prior experience with 11 of these unusual cases. Age at presentation ranged from newborn to 18 years (mean, 17 months). The most common presenting sign was a perianal or anal fistula, observed in five children. Two children presenting with fistulae had concomitant infection in the duplication. Other presenting signs included obstruction or prolapse caused by the rectal mass in three patients, rectal bleeding in three, and urinary retention in one. Some children presented with more than one finding. No associated spinal or vertebral anomalies were observed. Total excision was performed using a transanal approach in eight patients, postanal (transcoccygeal) in two, and posterior sagittal in one. Postoperative continence was normal in all patients. These cases illustrate that rectal duplications can be confused with other types of anorectal pathology including hemorrhoids, fistula-in-ano, and perirectal abscess. Total excision performed using a posterior sagittal, transanal, or transcoccygeal approach is curative.

Child↗

PubMed1990

The urodynamic consequences of posterior urethral valves.

We evaluated urodynamically 41 patients with posterior urethral valves because of signs or symptoms of incontinence (35), frequency (3), hydronephrosis (2) and infection (1). Findings included normal urodynamic evaluations in 3 patients, 2 had high voiding pressures secondary to outlet resistance and 1 had incontinence on the basis of external urethral sphincter damage. In the remainder 3 patterns of bladder dysfunction were identified. Myogenic failure with overflow incontinence occurred in 14 patients. In this group clean intermittent catheterization or Valsalva's voiding was used for emptying. Hyperreflexic bladders were seen in 10 patients. Pharmacological suppression of instability was effective in 5 of 7 patients treated; 1 required bladder augmentation. Eleven children had a small capacity bladder and poor compliance. Post-void residuals were low and these bladders were generally but not always stable. Pharmacological bladder relaxation was successful in 3 patients, 3 underwent augmentation, 1 did well with alpha-agonists and followup is unavailable on the other 4. These 3 patterns of bladder dysfunction represent an overlapping constellation of residual urodynamic abnormalities due to previous bladder outlet obstruction. Individual patients may show facets of several types of dysfunction associated with 1 predominant pattern.

Child↗

PubMed1990

Bladder augmentation: experience with 129 children and young adults.

From 1977 to 1989 bladder augmentation was performed in 56 male and 73 female patients from 1 to 35 years old (average age 12.7 years). In 59 cases augmentation was performed as part of an undiversion operation. Indications for augmentation included neurogenic bladder, severely scarred bladder from prior operations, noncompliant bladder after previous treatment of severe urethral valves, small bladder in former exstrophy patients, epispadias with a small bladder, cloacal exstrophy, and small, noncompliant bladder after therapy for cancer, trauma, cloaca and miscellaneous conditions. We used 145 bowel segments, since 16 patients had 2 bowel segments. Segments included cecum in 65 cases, sigmoid in 46, small bowel in 24, stomach in 4 and left colon in 1. The most common complication was stones. Detubularized bowel for augmentation of small noncompliant bladders allows functional reconstruction in a wide range of urological disorders that were formerly treated by diversion. Reconstruction is possible in many previously diverted patients if bladder augmentation is used.

Adolescent↗

PubMed1990

The importance of visualizing the ureter before performing a pyeloplasty.

Records were reviewed of 100 patients who underwent an operation for ureteropelvic junction obstruction from 1978 to 1989: 14 cases were bilateral and 17 were antenatally diagnosed. The ureter was opacified preoperatively in all but 1 patient: in 65 by retrograde pyelography, in 18 by antegrade pyelography, either through a nephrostomy tube with which the patient was referred or during a Whitaker test, in 9 by vesicoureteral reflux seen on voiding cystourethrography performed before an operation for ureteropelvic junction obstruction and in 7 by an excretory urogram. Of the 65 patients who underwent retrograde pyelography 29 had a discrete area of narrowing. However, 36 patients had something more, including a longer segment of narrowing (13), tortuosity of the upper ureter (8), more than 1 area of narrowing (11), high insertion of the ureter on the renal pelvis (3) and compression of the ureter by the lower pole of the kidney (1). In 10 patients referred for reoperation after failed pyeloplasty there was narrowing of the ureter below the level of the prior pyeloplasty. Although indications for retrograde pyelography are fewer today with the various modern imaging modalities in current use, we believe a retrograde examination should be performed before pyeloplasty if the ureter has not been well shown by other means. The retrograde catheter should be small and soft, so as to create no edema or other injury to the lower ureter. The study is done with the patient under the same anesthesia as the pyeloplasty and not at a previous time. All 114 pyeloplasties in these 100 patients were successful.

Adolescent↗

PubMed1990

Paratesticular rhabdomyosarcoma: results of therapy in 18 cases.

The management of paratesticular rhabdomyosarcoma has evolved more slowly than that for testicular germ cell tumors, mainly due to its lower incidence and the lack of standardized treatment protocols. With gradual refinements in staging and therapy the prognosis has improved. Between 1960 and 1988, 18 patients 2 to 18 years old were treated at the Children's Hospital. Management consisted of inguinal orchiectomy, staging evaluation and retroperitoneal lymph node dissection, followed by chemotherapy and radiotherapy according to the stage of the disease. In 11 patients (61%) the disease was confined to the scrotum, while 7 presented with spread to the retroperitoneal lymph nodes, including 3 children with more distant metastases. Histological study revealed predominantly embryonal characteristics except for 1 alveolar cell type. Chemotherapy consisted of actinomycin D in 2 patients, vincristine, actinomycin D and cyclophosphamide in 2, pulse vincristine, actinomycin D and cyclophosphamide in 10, and doxorubicin plus vincristine, actinomycin D and cyclophosphamide in 4. A total of 17 patients remain free of disease (median followup 4 years) with 1 death. The actuarial survival without relapse and over-all survival rate are 89 and 94%, respectively. We conclude that paratesticular rhabdomyosarcoma represents a favorable subgroup of rhabdomyosarcoma.

Actuarial Analysis↗

PubMed1990

Urinary tract re-functionalization after long-term diversion. A 20-year experience with 177 patients.

From 1969 to 1990, previously diverted urinary tracts were 'undiverted' in 177 patients whose ages ranged from 1 to 31 years. Fifty-six of the patients (32%) had been diverted for 10 years or longer. There were 67 female and 110 male patients. Forty-four patients had only one kidney and in two of those patients it was a previous renal transplant. One patient was anephric at the time of reconstruction, having had two unsuccessful transplants. Most of the diversions had been considered permanent. Types of diversions that were reversed include ileal loop, colon conduit, loop ureterostomy or pyelostomy, end ureterostomy, cystostomy or vesicostomy, long-term nephrostomy, and ureterosigmoidostomy.

Adolescent↗

PubMed1990

The cloacal malformation: radiologic findings and imaging recommendations.

The imaging studies and records of 65 patients with the cloacal malformation seen from 1969 to 1989 were reviewed. The malformations were described according to cloacal configuration (urethral, vaginal), type of urinary-cloacal communication (urethral, vesical), and level of rectal communication (vaginal, cloacal, vesical, other). Lower urinary tract abnormalities were frequent (reflux, ureteral ectopia, bladder diverticula, bladder duplication, urachal remnants, urethral duplication), as were genital abnormalities (uterine duplication, vaginal duplication, uterine atresia, vaginal atresia), abnormalities of the bony pelvis (partial sacral agenesis, pubic diastasis), and renal abnormalities (agenesis, obstruction, horseshoe kidney). Contrast material studies of the cloaca and the distal limb of the colostomy with fluoroscopy in various projections were essential for diagnosis. Voiding cystourethrography was important for detecting vesicoureteric reflux. Sonography was of limited value for evaluation of the malformation but was valuable for imaging the kidneys. MR imaging revealed that spinal cord abnormalities cannot be predicted based on the appearance of the lumbosacral spine and are more common than previously thought.

Abnormalities, Multiple↗

PubMed1990

Splitting the pubis for exposure in difficult reconstructions for incontinence.

We have presented our technique for and experience with splitting the symphysis to provide wide exposure in reconstructive surgery for incontinence. Our experience has demonstrated that the technique is widely applicable to many reconstructive situations in patients of all ages. We believe that spreading the pubic symphysis provides access to the urethra and bladder neck not otherwise possible and regard it as an important factor in the success of many difficult operations to correct incontinence. Although seemingly a radical maneuver, it did not cause complications in any of 51 cases.

Child↗

PubMed1989

Genitourinary rhabdomyosarcoma in children.

Although rhabdomyosarcoma of the genitourinary tract occurs infrequently in children, it presents a challenging problem to the surgeon, radiotherapist, and oncologist. Thirty-six children with genitourinary rhabdomyosarcoma were treated at our institutions from 1957 to 1985. The primary site of tumor involved the bladder, prostate, or both in 15 patients, the paratesticular structures in 12, and female reproductive organs in nine. Although treatment modalities and philosophies of care varied over the past 27 years, the majority of patients were treated with a combination of surgery, chemotherapy, and radiation therapy. The event-free survival for all patients is 74%. Patients with paratesticular tumors had the best prognosis (88% event-free survival). Regional nodal radiation therapy is not necessary in this favorable group if the retroperitoneal lymph node dissection is negative. Of 15 children with bladder/prostate tumors nine had initial conservative surgery. Of these nine six are long-term survivors, but only three patients have intact bladders. The girls with tumors of the reproductive organs had an event-free survival of 63%; all patients in this group had hysterectomies. Chemotherapy and radiotherapy have not substituted for radical surgical procedures in most children with bladder or prostate rhabdomyosarcomas. Innovative therapeutic approaches are necessary for this group of patients.

Adolescent↗

PubMed1989

Surgical treatment of pancreas divisum causing pancreatitis in children.

Although controversial, pancreas divisum has been implicated as a cause of acute pancreatitis when there is stenosis of the accessory papilla that drains the duct of Santorini. Over the past 5 years, four children with pancreas divisum and recurrent pancreatitis were successfully treated surgically. The diagnosis was made by endoscopic retrograde cholangiopancreatography (ERCP) in each case. Surgical treatment included sphincteroplasty to the accessory papilla to improve drainage of the duct of Santorini, opening the ampulla of Vater to expose the ostium of the duct of Wirsung to enlarge it, and cholecystectomy.

Acute Disease↗

PubMed1989

Esophageal adenocarcinoma 20 years after esophageal atresia repair.

We report a case of esophageal adenocarcinoma 20 years after esophageal atresia repair. From one case report it is premature to recommend cancer surveillance for all esophageal atresia patients. However, the first survivors are now reaching an age when esophageal cancer related to chronic esophagitis may become more prevalent.

Adenocarcinoma↗

PubMed1989

Splitting the pubis for exposure in difficult reconstructions for incontinence.

In the last 13 years splitting the pubic symphysis has been used in 46 patients 1 to 32 years old for wide access to the bladder neck and entire urethra in female, or the bladder neck and urethra down into the bulb in male patients. Each patient had a complex problem to reconstruct and most had undergone a prior operation. Underlying pathological conditions included exstrophy in 16 patients, epispadias in 11, cloacal exstrophy in 6, posterior urethral valves in 3, severe trauma in 2, bilateral single ectopic ureters in 2, radical surgery for sarcoma in 2, myelodysplasia in 2, cloacal anomaly in 1 and iatrogenic injury of the urethra in 1. The pubis, or interpubic ligament in exstrophy cases, was divided in the midline. The pubic rami were spread with a laminectomy spreader to create a gap sufficient to operate on that segment of the urethra, which is usually not accessible with an intact pubis. No patient had a problem of nonunion or pubic osteitis after using this approach. This series includes 2 patients undergoing simultaneous iliac osteotomies for a widened symphysis pubis. In no patient was a segment of pubis resected, as has been used in transpubic repair of certain traumatic urethral strictures.

Adolescent↗

PubMed1989

Congenital obstructed megaureters in early infancy: diagnosis and treatment.

Fetal screening during maternal ultrasonography has changed the mode and age of presentation of congenital uropathies, particularly congenital obstructed megaureter. We studied 47 infants less than 8 months old with primary obstructed megaureter. Surgical correction in 42 patients with moderate to severe obstruction was performed at a mean age of 1.8 months in those detected prenatally and 3.8 months in those presenting after birth. All infants showed functional and structural urographic improvement with a mean followup of 2.3 years. Reflux was seen postoperatively in 8 patients, which subsided spontaneously in 3, continues to be followed in 2 and resulted in repeat reimplantation in 3. Five infants had mild obstruction, which was managed nonoperatively and 2 showed progressive obstruction, which required repair at ages 20 and 28 months. Repair of obstructed megaureters in early infancy improves renal drainage and offers the potential for preventing renal damage before the development of symptoms or infection. With proper attention to detail, excellent results may be achieved.

Dilatation, Pathologic↗