W. Hardy Hendren

Adrenogenital syndrome: the anatomy of the anomaly and its repair. Some new concepts.

Abstract Female hermaphrodites with congenital adrenalcortical hyperplasia have normal internal genitalia, i.e., tubes, ovaries, uterus, and upper vagina, but the degree of virilization of the lower genitalia can vary greatly. Similarly, we believe that the degree of masculinization of the lower urinary tract can vary greatly in these female patients. In many the vagina lies just beneath the perineum and can be exteriorized by a simple “cutback” procedure. This, together with clitorectomy which is usually desirable, is all that is required. In other infants, however, the vagina enters the urethra quite high and proximal to the external urethral sphincter. This anatomic variant may be much more common than previously realized. A transperineal operation is presented by which the high placed vagina can be brought down to the perineum without injury to the external sphincter. It has been employed in 7 cases with satisfactory results.

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.

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 Valsalvas 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.

Ileal Conduits in Children at the Massachusetts General Hospital From 1955 to 1970

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.

A graded organ culture assay for the detection of mullerian inhibiting substance

Abstract A sensitive, reliable, semiquantitaive organ culture assay has been developed to detect the presence of Mullerian Inhibiting Substance, both in whole testicular fragments, and in cell-free extracts. This in vitro assay allows regression to occur in a manner which mimics the in vivo regression of the male fetal Mullerian duct. Timed exposure studies demonstrate that the Mullerian duct must be exposed to the testicular fragments for at least 12 hr before regression is initiated. Seventy-two hours are required for the morphological changes of regression to be fully expressed. Mullerian Inhibiting Substance is a compound of considerable importance in sexual differentiation. This assay will allow a more systematic approach to the study of this unique fetal regressor.

Continent Urinary Diversion: The Children's Hospital Experience

PURPOSE Continent urinary diversion has become increasingly important for treating childhood urinary tract pathology that cannot be managed by direct reconstructive techniques. We review our 9-year experience with continent diversion. MATERIALS AND METHODS Since 1986 continent diversions were created in 74 patients 3 to 38 years old (mean age 13.7). The underlying pathological condition was the exstrophy/epispadias complex in 34 patients, neurological disorders in 23, malignancy in 13 and other congenital anomalies in 4. Followup averaged 5.2 years after the last procedure. Nonbladder reservoirs in 39 patients (53%) were fashioned from ileocolic (17), colic (7), gastrocolic (6), sigmoid (3), gastrosigmoid (2), ileosigmoid (2), ileal (1) and gastroileac (1) segments. When possible, the native bladder was incorporated into the reconstructive strategy. A total of 26 patients underwent bladder augmentation with intestine or stomach, including ileal (11), gastric (8), sigmoid (3), gastroileac (2) and ileocolic (2) segments. Nine other patients did not require bladder augmentation. Continence mechanisms were a flap valve (Mitrofanoff principle) in 50 patients, nipple valve in 15 and ileal plication (Indiana pouch) in 9. When the Mitrofanoff principle was used with a native bladder reservoir in 30 cases, outlet resistance was altered by bladder neck division (15), fascial sling placement (6) or Young-Dees-Leadbetter bladder neck reconstruction (2). In the remaining 7 patients the bladder neck remained intact. RESULTS Excellent continence was obtained. The Mitrofanoff principle initially provided continence in 41 patients (82%). Six of the 9 incontinent patients were dry after a single revision. A total of 13 patients (87%) with nipple valves and 7 (78%) with Indiana pouches were dry, and the remaining 5 were cured after a single revision. Ultimately continence was achieved in 71 of the 74 patients (96%) after a maximum of 2 operations. Of the 48 complications in 29 patients the most common were difficulty in catheterizing (11), stones (11), infection (8) and upper tract deterioration (4). CONCLUSIONS Many options exist for reconstructing complex anomalies. Choices must be individualized based on patient anatomy. The dry state may be achieved in most cases without resorting to a bag on the abdomen.

Effect of dry and humidified gases on the respiratory epithelium in rabbits

A laboratory study was conducted in rabbits to compare histologic changes in the lungs and bronchi after prolonged dry anesthesia and humidified anesthesia. Dry anesthesia caused severe damage to the respiratory epithelium, which was not observed with humidified controls.

RESERVOIR CALCULI: A COMPARISON OF RESERVOIRS CONSTRUCTED FROM STOMACH AND OTHER ENTERIC SEGMENTS

PURPOSE The intestinal augmented bladder has an increased propensity to form urinary calculi. Predisposing risk factors include chronic bacteriuria, urinary stasis and mucus production. Gastric reservoirs have negligible mucus production and the ability to acidify the urine. We determined whether they also have a decreased incidence of stones. MATERIALS AND METHODS We retrospectively reviewed the records of all patients undergoing augmentation cyptoplasty (215) or creation of a freestanding reservoir (44) between May 1976 and March 1996. Of these cases 83 were augmented with stomach and 179 were constructed from other intestinal segments, (that is ileal, ileocecal, sigmoid and/or a combination of these). Presenting diagnosis, patient age, gender, additional surgical procedures, interval to stone formation and calculous composition were recorded. Patients were excluded from study when there was less than 1 year of followup, as were those with renal or pre-augmentation bladder calculi. RESULTS Stones formed in 32 of 207 patients (15%) after an average interval of 3.6 years (range 0.5 to 8.6). The majority of stones were composed of struvite (magnesium ammonium phosphate). Reservoirs containing gastric segments were significantly less likely to form calculi than those augmented with other intestinal segments (2 of 70 versus 30 of 137, p <0.0001 Fishers exact test). Seven additional patients with pre-augmentation bladder stones who subsequently underwent gastrocystoplasty remain stone-free at an average followup of 3.0 years. CONCLUSIONS Calculous formation is rare in reservoirs that incorporate a gastric segment. In patients with gastric augmentation stones appear only to develop when the patient is on histamine blockade or has a propensity to form stones that only favor an acidic environment, such as uric acid.

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.

Mullerian Inhibiting Substance in human testes after birth

Tiny testicular biopsies from humans were assayed for Mullerian inhibiting Substances using a graded organ culture technique. Mullerian Inhibiting Substance activity was high during the first year of life, declined during the second year of life, and then disappeared. Mullerian Inhibiting Substance activity is lower in boys with undescended testis than in children with normal or intersex testes at comparable ages under two years. It is possible that Mullerian Inhibiting Substance affects testicular descent as well as causing Mullerian duct regression.