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[Skin gangrene of the external genitalia. Report of 44 cases].

STUDY AIM: The aim of this study is to report 44 cases of male external genitalia cutaneous gangrene which have been observed at the Principal Hospital of Dakar (Senegal) during a 4-year period. PATIENTS AND METHODS: The patients all belonged to a black and poor population (mean age: 60 years). Diabetes was present in 11% of the patients. In ten patients, no aetiology was found. The other 34 cases were secondary mainly to urogenital pathology (50%). In 50% of the cases, the lesions were localised on the external genitalia, in the other 50%, the lesions had spread to the hypogastrium and/or the perineum. Medical treatment included intensive care and triple antibiotic therapy, penicillin, gentamycin and metronidazole. A hyperbaric oxygen therapy was associated in 25% of the cases. The surgical treatment in the acute period included incising, debridement, paring, draining, urinary derivation (n = 36), and colostomy (n = 5). Thirteen patients had the benefit of sequential and prospective bacteriological tests. RESULTS: Spontaneous healing was obtained in 48% of the patients within 2 to 3 months. Secondary reconstructive surgery consisted mainly in cutaneous grafts. Global mortality rate was 34%, mortality rate was 30% in the secondary gangrenes, 40% in the primitive gangrenes. Mean hospitalisation duration was 6 weeks. Main sequelae were cheiloïd scars. CONCLUSIONS: The authors try to clarify the nosological imprecisions of this pathology by distinguishing between the secondary types and the primitive types corresponding to Fournier's gangrene, which still inspires many questions concerning its etio-pathogenesis. The surgical treatment must eradicate all necrosis by suited iterative procedures, associated with local care. Hyperbaric oxygen therapy was not efficient in this series. This pathology, although rare, needs to be better known, because only an early and efficient surgical and medical treatment will be able to decrease the exceptional gravity of the prognosis.

Adult↗

Central precocious puberty in girls: internal genitalia before, during, and after treatment with long-acting gonadotropin-releasing hormone analogues.

Pelvic ultrasonography was systematically performed on 33 girls with idiopathic central precocious puberty to investigate the impact of treatment with gonadotropin-releasing hormone analogues on female internal genitalia. All girls were treated with a long-acting gonadotropin-releasing hormone analogue (Decapeptyl Depot; Ferring Co., Copenhagen, Denmark) 75 micrograms/kg every 4 weeks. Before, during, and after treatment, pelvic ultrasonography was performed and ovarian and uterine volumes were calculated. The size of follicles > 5 mm were accurately measured. The results were related to a normative study of healthy Danish schoolgirls. Our data demonstrated that ovaries and uterus are enlarged in a significant number of girls (50%) with the diagnosis of central precocious puberty at the time of diagnosis. Median ovarian volume at time of diagnosis was 1.1 standard deviation scores (range -0.6 to 3.2 SD), median uterine volume was 1.8 standard deviation scores (range 0.0 to 3.5 SD). Within 3 months of treatment, both ovarian and uterine volumes decreased significantly (p < 0.01) to normal values appropriate for age. Median ovarian volume after 3 months of treatment was 0.0 SD (range -2.4 to 1.5 SD); median uterine volume was 0.7 SD (range -0.6 to 4.1 SD). Ovarian and uterine volume remained within normal range (< 2 standard deviation scores) after discontinuation of treatment. Follicles and macrocysts regressed during treatment. None of the girls' ovaries had a polycystic appearance during or after treatment with the gonadotropin-releasing hormone analogue. Our results confirmed pelvic ultrasonography as a reliable tool for investigation of internal genitalia in girls with precocious puberty and as a valid method for evaluation of the efficacy of treatment with gonadotropin-releasing hormone analogues. We suggest that repeated investigations be performed when evaluating treatment because the morphologic changes, including follicular maturation or regression, reflect ovarian stimulation or suppression. We found no evidence that girls with precocious puberty treated with long-acting gonadotropin-releasing hormone analogues have enlarged polycystic ovaries develop.

Adnexa Uteri↗

Granulomatosis of external genitalia: a new disease or part of a spectrum?

Several efforts have been made to classify the various diseases that can cause ulcerative necrosis of the skin and mucous membranes. All of these lesions have granulomatous inflammation as a common denominator. Wegener's granulomatosis belongs to this area of interest. Most of these diseases affect the head, eyes, nose, respiratory system and kidneys. We present a case of ulcerative necrosis with pathological findings of granulomatous inflammation involving the external genitalia, thighs, perineum and eyes. To our knowledge this is the first reported case of involvement of the external genitalia by such a disease.

Granulomatosis with Polyangiitis↗

Acquired immunodeficiency syndrome-related Kaposi's sarcoma of the male genitalia: management with radiation therapy.

Kaposi's sarcoma of the genitalia is an infrequent complication of the acquired immunodeficiency syndrome but it often is difficult to manage. A total of 19 men (mean age 39 years) with penile and/or scrotal lesions underwent radiotherapy at dosages of 600 to 3,000 cGy. in fractions of 150 to 800 cGy. Of the patients 6 achieved a complete and 10 a partial response, while 2 had no response and 1 died too soon after treatment to be evaluated. Recurrences were noted in 8 patients, 7 of whom received additional radiation (1 patient had 2 recurrences and both responded). Of the retreatments 3 effected complete and 4 partial responses. Over-all, of 25 evaluable treatment courses 9 complete (36%) and 14 partial (56%) responses yielded a total response rate of 92%. There was 1 complication, a skin ulceration. Radiation therapy is well tolerated and will result in effective palliation in the majority of patients with Kaposi's sarcoma of the genitalia.

Acquired Immunodeficiency Syndrome↗

Eosinophilic sclerosing lipogranuloma of the male genitalia not caused by exogenous lipids.

A type of sclerosing lipogranuloma of the male genitalia that is different from cases caused by injection or application of foreign bodies is reported in 4 patients. The characteristics of the disease are a symmetrical Y-shape of the granuloma, spontaneous regression, severe infiltration of eosinophils in the granuloma, eosinophilia of the peripheral blood and absence of any exogenous lipids by chemical analyses of the granuloma tissue. Ultrastructural study showed intracytoplasmic fat vacuoles of varied size in histiocytes and multinuclear giant cells. This is a new type of sclerosing lipogranuloma of the male genitalia and the pathogenesis of the disease is still unknown.

Adult↗

Outcome analysis in patients with primary necrotizing fasciitis of the male genitalia.

OBJECTIVES: To characterize patients with primary necrotizing fasciitis of the male genitalia (Fournier's gangrene) and to identify risk factors and prognostic variables of survival. METHODS: Fifty consecutive patients with primary necrotizing fasciitis of the male genitalia treated at our institution during a 15-year period between 1984 and 1998 were retrospectively analyzed. Of these patients, 44 (88.0%) were found to be eligible for analysis of the outcome parameters. Univariate survival analysis was performed using the Kaplan-Meier algorithm followed by multivariate analysis of statistically significant variables. Six patients (12.0%) who were severely immunocompromised were studied separately. RESULTS: Medical comorbidities were prevalent, with diabetes being the most common condition (50%). The overall mortality rate was 20% (10 of 50). Three statistically significant predictors of outcome were identified among the variables analyzed. These were the extent of the infection (P = 0.0262), the depth of the necrotizing infection (P = 0.0107), and treatment with hyperbaric oxygen (P = 0.0115). Multivariate regression analysis of these variables identified the extent of the infection (P = 0.0234) as the only statistically significant, independent predictor of outcome in the presence of other covariables. CONCLUSIONS: The involved body surface area appears to be the most important prognostic variable, with a significant impact on outcome. Given the high mortality of the disease entity and a trend toward the improved survival of patients receiving hyperbaric oxygen, this treatment form appears indicated in more severe cases. Immunocompromised patients, who frequently have an atypical and fulminant clinical course, appear to constitute a separate group with a dismal prognosis.

Adult↗

[Sarcomas of the female genitalia: therapy and prognosis (author's transl)].

216 sarcomas of the female genitalia were treated at the University hospitals for women of Freiburg and Tübingen between the years 1957 to 1977. 76% originated from the uterine corpus; 28% (46/164) of the sarcomas of the corpus uteri were detected as incidental findings. The 5 year survival rates is 54% in stage I, 25% in stage II, and 40% in all stages (52/131). Postoperative irradiation of stage I cases of sarcomas of the corpus uteri diminished local recurrence but not metastases. Extirpation of the ovaries has no influence on local recurrence. Sarcomas of the vulva and cervix have a comparatively good prognosis, whereas the prognosis of the sarcoma of the ovary is very bad. The bad prognosis of sarcomas of the female genitalia points out the necessity of developing cooperative clinical trials on the effectiveness of adjuvant chemotherapy.

Female↗

Undermasculinized genitalia in a boy with an abnormally expanded CAG repeat length in the androgen receptor gene.

We report an 11-year-old boy with undermasculinized genitalia and an abnormally expanded CAG repeat length at exon 1 of the androgen receptor (AR) gene. He had microphallus and scrotal hypospadias with chordee, and underwent urethroplasty at 4 years of age. At 11 years of age, a gonadotropin releasing hormone (GnRH) test yielded a relatively high leutinizing hormone (LH) response (0.7-->20.4 IU/l) and a relatively low follicle-stimulating hormone (FSH) response (1.7-->4.8 IU/l), and an human chorionic gonadotropin (hCG) test showed sufficient responses of testosterone (0.7-->23.0 nmol/l) and dihydrotestosterone (0.38-->2.95 nmol/l). The CAG repeat length was 44 for the boy and ranged from 12 to 32 for 100 control males. The DNA sequences of the AR gene were normal for the exons 1-8 and for the splice donor, splice acceptor and branch sites. The markedly expanded CAG repeat length appears to be relevant to the undermasculinized genitalia of this boy, because such an expandsion, which has previously been reported only in spinal and bulbar muscular atrophy, is known to reduce AR function.

Age of Onset↗

Pubertal maturation of the internal genitalia: an ultrasound evaluation of 166 healthy girls.

Pelvic ultrasound is an important tool in the management of children with disturbances of pubertal development; interpretation requires an understanding of the normal relationship between maturation of internal genitalia and the appearance of secondary sex characteristics. We performed pelvic ultrasound examinations in 166 healthy females aged 6.4-25.4 years, and related uterine and ovarian volumes and size of follicles to age and pubertal stage. We demonstrated growth of the uterus and ovaries before the appearance of breast development and pubic hair growth. In prepubertal girls, uterine and ovarian growth was related to height (p = 0.008 and p = 0.010, respectively). From breast stages 1-5, median uterine and ovarian volumes increased from 1.6 ml to 43 ml, and from 1.2 ml to 7.3 ml, respectively (p < 0.0001). Uterine growth continued several years after menarche. Postmenarcheal uterine growth was related to the number of years after menarche (p < 0.001), but not to height, weight or age. Follicles were seen in 86% of prepubertal girls and in 99% of pubertal girls. Follicles up to 8 mm in diameter were observed in prepubertal girls. Maturation of the internal genitalia begins before the onset of clinical puberty, and extends well into the second decade.

Adolescent↗

Wilms' tumor, nephron disorder and ambiguous genitalia.

Two male cases with Wilms' tumor in association with ambiguous genitalia and nephrotic syndrome are described. Seventeen similar cases reported in the literature are also analyzed. In these patients, nephrotic syndrome was noticed at younger ages, and progressed more rapidly than usual. Renal damage was so serious that at least 11 out of 9 patients died of renal failure, though not of Wilms' tumor itself. It should be stressed that the clinical course and associated anomalies were quite similar among these patients. We suggest that Wilms' tumor, ambiguous genitalia and this type of nephrotic syndrome may have some etiologic connection.

Abnormalities, Multiple↗

Ambiguous genitalia: comparative role of pelvic ultrasonography and genitography.

Ambiguous genitalia represents a true medical and social emergency which needs a multi-disciplinary team approach for elucidation. The paediatric radiologist plays an important role in defining the genital anatomy which remains one of the most important factors in sex determination. Aiming to compare the predictive value of pelvic ultrasonography and genitography in sex determination in patients with ambiguous genitalia, we retrospectively reviewed the medical records of 69 patients, 53 females and 16 males, where both procedures were employed. In female pseudohermaphroditism, the presence of a uterus with or without vagina was predicted in 46 (86.8%) patients by ultrasound compared with 44 (83%) patients in whom a genitogram revealed a vagina +/- uterus. In six (11.3%) patients, a genitogram revealed a male-type urethra. The combination of ultrasound and genitogram, however, was more sensitive and predicted the presence of a uterus with or without vagina in 52 (98.1%) patients. In male pseudo-hermaphroditism, there was no false positive by ultrasound, and a genitogram revealed a male-type urethra in 12 (75%) patients. In conclusion, although real time pelvic ultrasonography is less invasive than genitography, its yield in elucidating genital anatomy is comparable. The combination of both procedures is more informative and has a better yield.

Child↗

Juvenile granulosa cell tumor--another neoplasm associated with abnormal chromosomes and ambiguous genitalia. A report of three cases.

Three infants, 3 months of age or younger with abnormal karyotypes and ambiguous genitalia, had gonadal juvenile granulosa cell tumors. Two of the patients had mixed gonadal dysgenesis and the third had an intersexual disorder of undetermined type. Two tumors arose in undescended testes, and the third in an undescended gonad of uncertain nature. The occurrence of this uncommon neoplasm in these infants indicates that it is another type of neoplasm that may develop in the gonad of a patient with an abnormal karyotype and ambiguous genitalia.

Chromosome Aberrations↗

Glomus tumor of the female external genitalia: a report of two cases.

Glomus tumors occurring in the external genitalia of two women aged 53 and 46 years, respectively, are reported. One of the tumors was clitoral and the other arose in the periurethral area of the vulva. The diagnosis in one case was supported by immunocytochemical analysis. Electron microscopy was performed on both tumors. Only two glomus tumors involving the female external genitalia have been reported in the literature. The tumors we describe appeared to be morphologically similar to the glomus tumors that typically arise in the distal extremities.

Female↗

Lymphedema of the external genitalia.

PURPOSE: This article presents a simple classification of lymphedema of the external genitalia, which is useful for selecting the appropriate therapy, and evaluates our experience with the various therapeutic options used to treat this disorder. MATERIALS AND METHODS: The literature was reviewed and the records of patients treated for the disorder were analyzed. RESULTS: A convenient classification of the disorder divides cases into congenital and acquired. Therapy is primarily dependent on whether the disease is self-limited and whether there has been any pathological change in the skin, lymphatics and subcutaneous tissue. For self-limited diseases in which no permanent pathological sequelae occur conservative therapy is appropriate. For most chronic conditions a surgical procedure is required. Excisional techniques are most effective for severe forms of the disease. In select cases subcutaneous tissue excision with preservation of the overlying skin is appropriate. However, for most patients excision of the skin and subcutaneous tissue with split-thickness grafting is most effective. CONCLUSIONS: When patients with lymphedema of the external genitalia require surgery and are properly selected for the appropriate procedure, the functional and cosmetic results are excellent and patient rehabilitation is likely.

Adult↗

Morphological features of external genitalia in hypospadiac rat model: 3-dimensional analysis.

PURPOSE: We examined 3-dimensionally the process of external genitalia formation in the experimental hypospadiac rat model. MATERIALS AND METHODS: We administered 7.5 mg flutamide daily, a blocker of androgen receptor, into the abdomen of naturally pregnant female Sprague-Dawley rats from gestational days 14 to 20 to produce a hypospadiac rat model. The control group consisted of male offspring not exposed to flutamide. The fetal phallus was obtained at gestational days 17.5, 19.5 and 21.5. We observed them by scanning electron microscopy. RESULTS: In the 17.5-day-old embryo the projection on the ventral side of the phallus was observed from the base of the phallus to the coronary sulcus in the control rat. This finding demonstrated that the urethra develops from the base of the phallus to the coronary sulcus. On the other hand, this projection was not observed in the hypospadiac rat and the urethra was not seen on the ventral side of the phallus. In the 19.5-day-old embryo the ventral preputial closure appeared in the proximal phallus of the control rat but not of the hypospadiac rat. In the control rat the scrotum was discerned in the perineum and divided by a median fold (the raphe). The raphe reached the base of the phallus, where it was concurrent with the preputial fold that covers the urethra. On the other hand, the hypospadiac rat did not have a raphe and the hollow at the base of the phallus appeared to give rise to the future orifice of the urethra. In the 21.5-day-old embryo the control rat prepuce completely surrounded the distal phallus, whereas that of the hypospadiac rat was dorsally hooded with a ventral cleft and the external appearance resembled the morphology of human penoscrotal hypospadias. CONCLUSIONS: This hypospadiac rat model is considered valuable for further studying penile growth and differentiation, and the molecular mechanisms in external genitalia formation in hypospadias.

Animals↗

Fournier's gangrene: necrotising fasciitis of the male genitalia.

The aetiology, clinical syndrome and outcome of nine patients with necrotising subcutaneous infections of their external genitalia have been analysed. These patients constituted just under 1% of all of our urological admissions during a nine-month period. Eight of the nine patients suffered from the secondary variety of this disease with urethral strictures and extravasation being the most common precipitating conditions. Necrotising fasciitis of the genitalia still carries a high risk of mortality and morbidity, frequently necessitating long periods of hospitalisation and repeated surgical procedures. Special attention was given to the bacteriology of the disease and recommendations concerning antibiotic therapy are made.

Adult↗

An abattoir survey of bull genitalia.

This paper describes a survey of 504 bull genitalia collected after slaughter at 3 Western Australian abattoirs over 18 months. Details of the gross pathology, histopathology, and microbiology of the genitalia, and physical dimensions of the testicles are presented. The major breeds represented were Hereford (30%), Shorthorn (16%), Friesian (14.3%), and Aberdeen Angus (13.9%). The mean age of 263 bulls (52.6%) was 4.4 years. The mean length and breadth of 469 paired testicles was 10.8 cm and 6.9 cm respectively. Pathological conditions of the testicles were present in 61/469 (13%) bulls and in the epididymides of 37/469 (7.9%) bulls. Campylobacter fetus was isolated from 20 (4%) bulls, Tritrichomonas foetus was isolated from 6 (1.2%) bulls, and infectious bovine rhinotracheitis virus was isolated from 14/158 (8.9%) bulls. Histories were supplied by stock owners for 307 (60.9%) bulls and these did not relate well to the findings of the survey. The findings of this survey were compared, in particular, to similar studies carried out in Australia.

Abattoirs↗

Squamous cell carcinoma of the equine external genitalia: a review and assessment of penile amputation and urethrostomy as a surgical treatment.

A retrospective analysis was performed on 48 cases of suspected neoplasia involving the equine external genitalia. Forty nine squamous cell carcinomata (SCC) and 23 squamous papillomata (SP) were identified. Two cases of epithelial hyperplasia and one case of habronemiasis were also recognised histopathologically. The glans penis was the most common site of tumour development accounting for 53 per cent of cases of SCC and 61 per cent of SP. The urethral diverticulum, urethral process or urethra was involved in 27.6 per cent (8/29) of cases of SCC. Twelve per cent (5/41) of cases of SCC were confirmed to have metastatic involvement of the local lymph nodes. Papillomata showing pre-malignant changes were identified in five cases. Seventy five per cent (36/48) of the animals presented were ponies. All were geldings, mean age 16.5 years. Penile amputation and urethrostomy, preputial ablation and proximal urethrostomy or local excision were used in the surgical management of these animals; three cases were inoperable and destroyed at presentation. Oedema, haemorrhage and stenosis of the urethrostomy site were encountered as post operative complications. Sixty six per cent (6/9) of animals with SCC of the glans penis (without urethral involvement), which had a known history over the follow up period, treated by penile amputation and urethrostomy survived for longer than 18 months. Similarly, of those with urethral involvement only 28.6 per cent (2/7) survived for a similar follow-up period. SCC not uncommonly affects the external genitalia of aged pony geldings and frequently the glans penis. If the lesion is identified before local metastasis occurs, penile amputation and urethrostomy is indicated and the prognosis is favourable.

Amputation, Surgical↗