Disorders of Sex Development (DSD) remain a fascinating challenge for the paediatricians, endocrinologists, biologists, psychiatrists, geneticists, radiologists, surgeons and for the whole society. This article aims at highlighting the current controversies and questions met with genital reconstruction in children born with abnormal genitalia. The main current techniques of masculinization and feminization are reviewed with their progress and their problems. The tools of decision used to assign a gender in some newborns with complex DSD are discussed showing that at the dawn of the third millenium, one still does not know why a boy is a boy, and a girl is a girl.
Laparoscopic Radical Cystectomy (LRC) has been proposed since 1999 as a less invasive alternative to Open Radical Cystectomy (ORC). Pioneers of the technique claim that LRC led to faster recovery, shorter hospital stay and more rapid return to daily activities respect to ORC while offering the same functional and oncological results. About 900 cases are published in peer reviewed papers. The greatest series is formed by a cohort of 85 patients. The preferred urinary diversion is the ileal conduit (46%) although in recent series the ileal neobladder is increa-singly adopted. Urinary diversion is usually performed extracorporeally through an abdomen incision of about 5–10 used also for the extraction of the specimen. The mean or median follow up of LRC series does not exceed 31 months and the longest follow up reported up to now is 58 months in the series examined. Overall survival rate varies from 72% to 95%. While feasibility of LRC has been demonstrated, cancer control has far from been assured, mainly in consequence of limited follow-up of the series and an unexpected low disease free survival rate. Moreover the advocated advantages related to LRC seem to be related to patients’ selection rather than to less invasiveness. Actually when characteristics of the patient and of the disease are similar, outcomes of LRC and ORC, in terms of hospitalization and recovery, are comparable. LRC is currently an experimental procedure which can not be considered at the present time a concrete alternative to ORC.
OBJECTIVES: To present the unpublished original book on Urological Therapeutics of Dr. Victoriano Molina, a great urologist from Valencia, and to outline his biographical sketch.METHODS: We read his treaty on therapeutics and his publications with detail. To elaborate his biography we obtained information from his descendants and the people who worked with him.RESULTS: When he was locked in the besieged Madrid during the Civil war he dedicated his time to write a therapeutics manual for personal use, which when finished became an excellent treaty in which all the urological pathology was gathered with various clinical scenarios, establishing the medical treatment with precise indications, enriched with numerous magistral formulas and preparations from the time.Dr Molina trained with Dr de la Peña and worked in Hospital de la Princesa with Drs. Cifuentes, father and son, and he was included by Dr. Jimenez Diaz in the staff of the Medical Research Institute, center in which he completed his professional career.CONCLUSION: The treaty of Urological Therapeutics, written in 1938, represents an excellent unpublished urological text, without comparison in the bibliography due to its contents, a manual which could have bécame an adequate consultation manual in any urological office.The author, remembered by his goodness, his knowledge and his dedication to Urology, is discovered thanks to this work as one of the relevant personalities of the Spanish Urology.
OBJECTIVES: To evaluate the incidence of hydrocele following laparoscopic Palomo varicocele ligation in pediatric and adolescents in our institution.METHODS: Between 1997 and 2009, 180 boys diagnosed as having varicocele who underwent Palo-mo laparoscopic ligation were evaluated retrospectiveArchly. Outcome variables recorded for analysis were age at presentation, symptoms, varicocele grade (Dubin-Amelar classification), testicular atrophy, length of hospital stay, perioperative complications, recurrence and hydrocele formation after surgery. Mean follow up was 5.8 years (6 months- 9 years).RESULTS: Age at diagnosis ranged between 9 and 19 years. Mean age at operation was 14.1 ± 1.8 years. There were 177 left-side varicoceles (98%) and four ca-ses were bilateral. Testicular atrophy was noted in 45. Mean operative time was 38 min. The last 63 surge-ries were performed on a two-trocar basis with Ligasure vascular sealing device and operative time decreased significantly to 22 min. Median hospital stay was 31h. Twenty-three patients developed hydrocele (13%); 11 of these underwent Winkelman-Lord’s hydrocelectomy at least 1 year after Palomo (9% of total). Of the remaining 12, two resolved spontaneously and 10 were stable at mean 4-year follow up.CONCLUSIONS: Laparoscopic Palomo procedure is a safe successful method to correct varicoceles in pedia-tric and adolescent males. Reactive hydrocele formation following laparoscopic varicocelectomy is a worrisome problem particularly in cases with longer follow-up.
OBJECTIVES: Dorsal urethroplasty as described by Barbagli has gained wide acceptance in the treatment of urethral stricture, given the simplification afforded by obtaining a free graft versus a flap.We present a series of 50 patients treated in our de-partment of urethral stricture by dorsal onlay free graft urethroplasty (Barbagli’s technique), in combination or not to other techniques in more complex strictures.METHODS: The average age of patients was 48.8 years (23-77), the mean follow up 42.9 months (12-96) and the most frequently observed etiology has been the inflammatory urethral stricture (50%).RESULTS: Overall success rate was 82% (41 cases) and failure 18% (9 cases). In the longer follow-up group, there was a slight drop in success rate of 80% (24 ca-ses) without any significant differences between groups (p= 0.98).Analyzing the variables length of free graft (p= 0.50, p> 36= 0.53), age (p= 0.12, p>36= 0.59), etiology of stricture (p= 0.77, p>36 = 0.77) and type of graft used (p=0.24, p>36= 0.38) did not show any influence on the final outcome of surgery, both in the total sample and the subgroup with follow-up > 36 months.The location of the stricture in bulbar urethra has shown better functional outcome than those operated on for stric-tures affecting also other urethral locations (p= 0.001) maintaining that result in the group of longer follow up (p>36= 0,001).The lack of treatment prior to urethral surgery has influen-ced the success of it, since 90.6% of patients without prior treatment before urethroplasty have seen a good func-tional outcome, compared to treatment prior to surgery that obtained a 66.7% (p= 0.03). This significance is also demonstrated in the group of follow-up > 36 months (p>36= 0.01).CONCLUSION: The dorsal onlay free graft urethroplas-ty is a versatile and reproducible technique with accep-table results which allows combination with other tech-niques when the stenosis extends to the penile urethra. In exceptional cases of panurethral strictures secondary to lichen sclerosus when there is no significant spongiofi-brosis and an acceptable urethral plate, can be applied to the whole urethra.
OBJECTIVE: To present a case of Kimura disease associated with kidney epithelial neoplasia.Kimura disease is a rare inflammatory disease, which usually presents with enlarged lymph nodes and masses in the head and neck regions. The differential diagnosis should include lymphomas, reaction lymphadenopathy to drugs or parasites. Amid the masses and enlarged lymph nodes a nodule suggestive of neoplasm can be found. There is a case, in the literature that describes Kimura Disease associated with kidney neoplasm.METHOD: We present the case of a young female patient who showed intra and retroperitoneal nodulations, including one in the lower pole of left kidney. After the period of treatment with corticoids, computed tomography of abdomen confirmed the permanence of the lesion with features of malignancy. A partial left nephrectomy was performed, and postoperative evolution was favorable.RESULT: The patient is without signs of Kimura Disease and no signs of recurrence of the tumor after a period of 18 months from surgery.CONCLUSION: Kimura disease is associated with some neoplasias that should be investigated by the medical team. Partial nephrectomy seems a good therapeutic option for well-defined neoplastic nodules located on renal poles.
OBJECTIVE: To describe a case of urachal adenocarcinoma with late brain metastases in a sixtyone year old man who presented abdominal discomfort and hematuria during six months.METHODS: The clinical suspicion was bladder tumor and diagnostic studies were performed (urinary cytology, cystoscopy, abdominal ultrasound and abdominopelvic CT scan). Surgical treatment was performed. RESULTS: Negative urinary cytology. Cystoscopy showed a lesion with infiltration of the bladder dome. Ultrasound and CT scan showed a five centimeter rounded lesion, with intermediate density, internal echoes and calcifications on the anterior supravesical middle line, that infiltrated the bladder. The extension study had not findings. Partial cystectomy and lymphadenectomy were performed. The histopathologic diagnosis was mucin-secreting urachal adenocarcinoma. After five years without disease the patient suffered lung and brain metastases.CONCLUSIONS: Urachal adenocarcinoma is a tumor which must be distinguished of primary bladder adenocarcinoma. The mucing-secreting adenocarcinoma can be associated with calcifications that can be demostrated on imaging studies. Late metastases without signs of local recurrence (after five years without disease) are an infrequent clinical-pathologic finding.
OBJECTIVE: To report a new case of bladder schistosomiasis. METHODS: A 21-year-old patient came to our consultation with gross hematuria several weeks before, existing reddish lesions in the cystoscopy that suggested the existence of an atypical bladder tumor. RESULTS: After intense blood and urine test, ultrasonography and intravenous urography, transurethral resection of bladder was performed and the diagnosis on histological examination was bladder schistosomiasis. CONCLUSIONS: Given the high prevalence of schistosomiasis in the sub-Saharan countries and later clinical epidemiologic implications, the appearance of macro or microscopic hematuria in black race immigrants, or even any urinary symptoms, it is necessary a complete evaluation, at least with a parasitological analysis (being the bilharziasis the most probably reason). The patients who have suffered severe urinary schistosomiasis must complete long-term follow-up to prevent bladder carcinoma.
OBJECTIVE: To review the presentation, physiopathology, diagnosis and therapeutic alternatives of stuttering priapism with the contribution of a new clinical case.METHODS: A 25 year old man, studied in another center for recurrent episodes of priaprism for about 18 months. These episodes occur daily, significantly interfering with patient´s quality of life. RESULTS: Initially he was treated with Bicalutamide 50mg/24h with no improvement. Blood test, penile Doppler ultrasound and selective arteriography of pudendal arteries showed no abnormalities. Tadalafil 5mg/24h was given for two months without response. Subsequently were treated with Diazepam 10 mg/24h and Terbutaline 5 mg/24h allowing control of the disease, remaining asymptomatic at present. CONCLUSIONS: Stuttering priapism is a rare form of presentation of this disease, caused by an alteration in the regulatory mechanisms of erection mediated by 5PDE and cGMP. Several drugs have been proposed in treatment with variable effectiveness, though there is no series long enough to recommend either as first choice. The use of inhibitors 5PDE so long, has been used successfully by some groups. Knowledge of these alternatives is important for the treatment of this complex and unusual pathology.