Biomedical subjects
Publications and source records attributed to Jonathan H Ross.
The 2 cases of megalourethra presented in this report illustrate the variability in the severity of congenital malformations associated with this condition. Surgical repair of a scaphoid-type megalourethra is discussed. Also, a digital video of megalourethra in a degloved penis is presented that uniquely illustrates the functional anatomy of this condition.
We report a case of a single system solitary ectopic ureter to the rectum with bladder agenesis in a viable newborn male. Only 2 cases of ectopic ureter to the rectum and 18 cases of bladder agenesis have been reported in viable newborns. To our knowledge, this case constitutes the first reported combination of these defects in a living neonate. Associated anomalies also included renal dysplasia, anal stenosis, and urethral-rectal fistula. Nephrectomy is often necessary to prevent infection or hypertension.
An 18-month-old girl was referred for evaluation of vaginal bleeding and a renal mass. Ultrasonography and computed tomography revealed multiple simple cysts within the left kidney and a normal right kidney. At vaginoscopy, a 1-cm frond-like papillary lesion overlying the cervix was identified. The pathologic diagnosis was mullerian papilloma, a rare benign tumor of the vagina and uterine cervix. None of the approximately 40 previously reported cases have been associated with renal anomalies, despite the historical classification of these lesions as mesonephric papillomas. We report the first patient with both a renal anomaly and mullerian papilloma of the uterine cervix.
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PURPOSE: We evaluate the feasibility of applying minimally invasive techniques for fetal vesicostomy. We also evaluate whether transuterine fetal vesicostomy can be performed laparoscopically. MATERIALS AND METHODS: A total of 25 pregnant ewes were time dated at approximately 90 days of gestation. With the animals under general anesthesia a low open abdominal incision was made and the uterus was brought out through the incision. With a 14 gauge needle the amniotic sac was filled with 1 to 2 l warm glycine. Three to 4, 5/12 blunt tip balloon trocars were placed in the uterus. Using laparoscopic techniques, a low transverse incision was made in the fetal abdomen, the bladder was opened at the dome and 2 running sutures were placed approximating the fetal abdominal wall to the edge of the fetal bladder. The trocar sites in the uterus were closed, and the maternal abdominal incision was closed. RESULTS: Of the 25 pregnant ewes the technique was developed in the initial 15. In the subsequent 10 animals the complete procedure was accomplished successfully. Following these 10 procedures 5 abortions occurred on postoperative day 2, and there was 1 intrauterine fetal demise. Three fetuses were alive and delivered by cesarean section on postoperative days 10, 30 and 31. In the first fetus in which we used an interrupted suture for the vesicostomy a large hernia was noted at the vesicostomy site. The other 2 fetuses had a patent, well healed vesicostomy and were alive at cesarean section delivery on postoperative days 10 and 31. The last fetus was allowed to deliver at term by standard vaginal delivery. The fetus was alive and well, and the vesicostomy had strictured down to a pinhole in size, which was not unexpected as it was not an obstructed model. CONCLUSIONS: Although technically challenging, transuterine laparoscopic fetal vesicostomy is technically feasible in the ewe model. Continued evaluation of this technique should include intensive fetal monitoring and the use of tocolytics to decrease the incidence of spontaneous abortion.
Because prepubertal testis tumors are rare, their management has historically been based on experience with the more common adult testis tumors. However, recent studies highlighting the natural history of these tumors and their response to therapy have resulted in a modern management algorithm that optimizes testicular preservation and minimizes the morbidity of adjuvant therapies. Many prepubertal testis tumors are benign and can be managed with testis-sparing tumor excision. Localized malignant tumors (yolk sac tumors) may be managed with excision alone. Recurrent tumors and metastatic disease can almost always be treated successfully with platinum-based chemotherapy.
PURPOSE: Little data are available regarding sports participation and appropriate long-term followup of children with a solitary kidney. We determine the current practice patterns and recommendations among pediatric urologists regarding sports participation and followup of these patients. MATERIALS AND METHODS: A survey was mailed to the 231 active members of the American Academy of Pediatrics, Section on Urology. The survey included questions regarding counseling of patients with a solitary kidney and physician estimates of long-term risk to overall renal function. RESULTS: Of the 231 surveys 182 were returned for an overall response rate of 79%. Of the respondents 68% recommend that patients with a solitary kidney avoid contact sports. Recommendations in regard to participation in contact sports were further stratified as strongly against participation (27%), against participation with rare exceptions (30%), no recommendation either way (14%), allow participation (25%) and no restrictions be made (4%). Of the respondents 88% agreed that the estimated risk of renal loss from a child participating regularly in contact sports is less than 1% and 60% recommended special medical followup. CONCLUSIONS: Despite the consensus that the risk of renal injury in contact sports is low, a significant number of pediatric urologists advise avoidance. There appears to be a lack of consensus regarding long-term medical surveillance of these patients. Studies designed to obtain accurate clinical data regarding these issues are warranted to establish evidence based guidelines for the long-term treatment of children with a solitary kidney.
PURPOSE: The Prepubertal Testis Tumor Registry was established by the Urologic Section of the American Academy of Pediatrics in 1980 to record data on a large number of prepubertal testis tumors regarding presentation, treatment and outcome to define appropriate management better. We reviewed the registry data in the context of other modern studies to elucidate the appropriate management of these rare tumors. MATERIALS AND METHODS: Relevant data in the prepubertal testis tumor registry were tabulated and analyzed. RESULTS: There were 395 prepubertal patients who had a primary testis tumor. Generally benign tumors accounted for 38% of cases. A significant proportion of tumors were benign regardless of patient age. alpha-Fetoprotein levels for patients with benign and malignant tumors overlapped in children younger than 6 months. Of the patients with yolk sac tumor 80% presented with stage 1 disease and overall survival was excellent. There were no metastases or deaths among the patients with teratoma. Of all patients with stromal tumors metastases developed in only 1. CONCLUSIONS: We recommend initial excisional biopsy for all amenable prepubertal testis tumors, except those with an alpha-fetoprotein level that is clearly increased for patient age. Patients with benign tumors may be released from oncological followup. Patients with stage I yolk sac tumor should be monitored closely, and those with recurrent or metastatic yolk sac tumor should be treated with chemotherapy. Retroperitoneal lymph node dissection is reserved for patients with recurrent retroperitoneal masses following chemotherapy. Aggressive treatment of metastatic Sertoli cell or undifferentiated stromal tumors is warranted.