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PubMed1991

Multifocal malignant melanoma arising in vesicovaginal melanosis.

An 80-year-old white woman developed vulvar melanosis and malignant melanoma of the labia majora and clitoris and underwent a simple vulvectomy. She subsequently developed melanosis of the urinary bladder and presented 3 years later with multifocal malignant melanoma involving the vagina, urethra, and urinary bladder in a background of extensive melanosis with variable degrees of atypia. She underwent radical surgery but died 18 months later with liver metastases and liver failure. Malignant melanoma is uncommon in the vulva and vagina and is rare in the urinary bladder. This case illustrates the previously described association between melanosis and malignant melanoma. The unusual features are the widespread distribution of the melanosis (vagina and bladder) and the subsequent development of multifocal malignant melanoma.

Aged↗

PubMed1990

A case of congenital adrenal hyperplasia with concomitant abnormalities of steroid 21- and 11 beta-hydroxylase activities.

Abnormalities in the steroid 21-hydroxylase and 11 beta-hydroxylase activities were suspected in a 25-year-old female with congenital adrenal hyperplasia (CAH). The patient showed signs of masculinization such as hirsutism, amenorrhea, and enlarged clitoris, but the blood pressure was normal. Adrenocorticotropic was increased to 200 pg/ml. Plasma levels of deoxycorticosterone and 11-deoxycortisol as well as progesterone and 17-hydroxyprogesterone were elevated. Plasma cortisol level was normal at 5.8 micrograms/dl. CT scan revealed enlargement of the bilateral adrenal glands. This case suggests that enzyme abnormalities in CAH are more diverse than have been generally considered.

Adrenal Hyperplasia, Congenital↗

PubMed1990

[Duplication of the urethra in a young girl. Apropos of a case].

Duplication of the female urethra is quite uncommon. A case of complete urethral duplication is reported in a 2.5 year old girl seen for pyuria. Physical examination showed and epispadiac meatus with dribbling urine just above a bifid clitoris associated with an orthotopic meatus. The two urethras were patent and situated on the same sagittal plane as seen on the cystography. The treatment consisted of excising the whole epispadic urethra using a combined perineal and suprapubic approach enlarged by symphyseal section for a better repair of the bladder outlet. Postoperatively, the patient was content and free of symptom. The main features of urethral duplication in girl are reviewed.

Child, Preschool↗

PubMed1990

[Transsexualism].

Transsexuals correspond in terms of their chromosomes, hormones and anatomy to their phenotypic sexual characteristics, but feel, with respect to their sexual identity that they unmistakably belong to the opposite sex, and wish for this reason to be reassigned to their psychological sex by means of endocrinological and operative treatment. They have a complete and permanent transposition of their sexual identity. The cause of transsexualism is unknown. There are male-to-female and female-to-male transsexuals. The diagnosis is made by a psychiatric evaluation. Hormonal treatment before operation and lifelong substitution after castration are described. The surgical technique in male-to-female consists in castration and construction of a neovagina by inversion of the penisskin, evtl. mammaplasty -in female-to-male: mammatransformation, vaginal colpohysterectomy and adnectomy and in a second step perineoplasty and construction of a phallic urethra up to the glans of the enlarged clitoris.

Breast↗

PubMedDate not supplied

45,XO/46,XY in a newborn with the stigmata of Turner syndrome: report of one case.

A newborn with ambiguous external genitalia and the stigmata of Turner syndrome presented with the following features: short stature, hypertelorism, bilateral epicanthal folds, ptosis, low-set ears with prominent auricles, high-arched palate, low posterior hairline, webbed neck, broad and short chest, widely-spaced and hypoplastic nipples and clitoris-like phallus with hypospasdias. He also had patent ductus arteriosus, the secundum type of atrial septal defect and mitral stenosis. Chromosomes of peripheral blood showed mosaicism of cells with 45,XO/46,XY. An exploratory laparotomy was performed at five months of age. The right side ovotestis-like gonad was removed. The left side gonad in the scrotum was normal. No pathological gonadoblastoma was found.

Abnormalities, Multiple↗

PubMed1990

Value of four models for selecting patients for local excision of invasive squamous cell carcinoma of the vulva.

For 75 women with squamous cell carcinoma of the vulva who underwent radical vulvectomy and inguinofemoral lymphadenectomy, the authors assessed the efficacy of four models for selecting patients who could have been treated adequately with local excision of the tumor. Each of the three models proposed by Andreasson and Nyboe covered 25% of the patients, none of whom had groin metastases or died of cancer. Local recurrence in the vulva occurred in 10%. A model suggested by the International Society for the Study of Vulvar Disease covered almost 10% of the patients. One of seven patients had groin metastases, none died of cancer, and one of seven developed local recurrence in the vulva. The criteria of the clinically best suited model are tumor not situated on the clitoris and less than 4 cm in diameter, with only slight hyperchromasia. This model ought to be tested in a randomized study.

Adult↗

PubMedDate not supplied

[Use of CO2 laser in the treatment of condylomata acuminata of the vulva in women].

CO2 laser was used in the treatment of 35 women with vulvar condylomata acuminata. The mean age of the patients was 31.6 years. Isolated lesions were removed as well as extensive conglomerates involving the whole vulva and clitoris and anal region. Small condylomata were evaporated. Large ones were cut off at the base and additionally evaporated. After one laser application cure was obtained in 87.5% of cases, while after repeated procedure 93.8% of the patients were cured. The tolerance of laser procedures was good in all cases. Healing was rapid, no scars and hyperpigmentation were noted. The possibility of application of laser in pregnant women is another advantage of this method.

Adolescent↗

PubMed1989

[A case of infantile virilizing adrenocortical tumor].

The patient was a 16-month-old girl, born by mature natural delivery and weighing 3,320 g. Hirsutism was noted on birth. Development of pubic hair and hypertrophy of the labia minora were noted after 8 months. At the time of admission, the height was 80 cm and body weight 14.5 kg. Systemic obesity, facial acne, systemic hirsutism, low pitched voice and hypertrophied clitoris were observed. Androstenedione, dehydroepiandrosterone-sulfate and cortisol showed high levels in the blood, and the urinary 17-KS was obviously high, along with an increase in urinary 17-OHCS. The subject did not respond to either the dexamethasone inhibition test or ACTH load test. The abdominal CT revealed a tumor in the front upper position of the left kidney, and adrenal scintigraphy disclosed an obvious accumulation image in the adrenal gland on the left side. Based on the diagnosis of a left adrenal tumor, left adrenalectomy was performed. The tumor measured 5.0 x 4.5 x 3.7 cm, and weighed 57 g. Histopathologically it was diagnosed as adrenocortical adenoma. The infantile virilizing adrenocortical tumor is reported together with some discussion of the literature.

Adenoma↗

PubMed1989

Feminizing genitoplasty: one-stage genital reconstruction in congenital adrenogenital syndrome.

Complete one-stage genital reconstruction was made in five children with congenital adrenogenital syndrome. Removal of the cavernous body of the clitoris was made by preservation of the glans with its vascular and neural supply. The lesser lips of the pudendum were fashioned from the skin of the penis and the prepuce. During the same operation, the vaginal orifice was created by the inversion of an U-shaped flap taken from the perineum into the introitus vaginae. There were no surgical complications. The patients recovered with good cosmetic results. It is necessary to regularly dilate the new vaginal orifice.

Adrenal Hyperplasia, Congenital↗

PubMed1989

[Clinical symptoms and prognosis in malignant melanoma of the pubic region].

Seven cases of malignant melanoma in genital portion were investigated. As the result, genital malignant melanoma was 2% of total number; the male was 2, the female 5, the female was many. In the part of incidence 3 cases were cervical region, others were small labium, clitoris, penis, scrotum respectively. ALM were 5 cases in pathological type the prognosis was no good, generally many cases were found later. The part of metastasis was skin, lung, liver, bone in many cases. In this report, the part of incidence, pathological type and survival ratio were investigated in another 345 cases of malignant melanoma which were experienced until December in 1987.

Aged↗

PubMed1986

[Value of evoked sacral potentials in studying bladder sphincter disorders in peripheral neuropathies and central nervous system diseases. A study of 110 cases].

The sacral spinal cord is one of the main sites of integration of bladder and anal sphincter and sexual function. Clinical tests (bulbocavernous reflex, perineal reflexes) are often inadequate and urodynamic investigations (cystomanometry, urecholine test, Susset's test) are sometimes unhelpful. The integrity of this structure is essential for normal sphincter function and disease at this level (or of its afferent or efferent limbs) produces a peripheral type of disorder. Sacral evoked potentials are an electrophysiological means of testing the bulbocavernous reflex by external stimulation of the dorsal nerve of the penis or clitoris and recording by a needle bipolar electrode in the striated muscle of the sphincter or the bulbocavernous muscle. An objective study of the sacral reflex are (S2, S3, S4) can thus be performed. One hundred and ten patients have undergone this investigation. Normal latency established in a series of 26 healthy volunteers was less than 42.1 ms. Prolongation of this interval is a sign of disease of the reflex are and correlated with urinary or sexual problems in 46 cases of clinical or electrical peripheral neuropathy. The reflex time was normal in all patients with CNS disease (32 cases) and an impotence of psychological origin (6 cases).

Adult↗

PubMed1987

[A new approach to urinary continence disorders in women: urodynamic ultrasonic examination by the vaginal route].

The vesicourethral junction, the urethra cannot be seen when using the abdominal approach because of the interposition of the pubic symphysis. The ultrasonic vaginal approach makes it possible. 53 patients were ultrasonically observed during an urodynamic exploration. The simultaneous utilization of both methods has permit to observe the mechanisms of normal or pathological voiding. When initiating a voluntary micturition, an area (called "prepubic muscle") located in front of the pubic symphysis between the clitoris and the urethral meatus, exert a traction on the periurethral sphincteric area. This sphincteric area, which is well shown by ultrasound, contracts longitudinally (causing shortening of the urethra and opening of the bladder neck) and causes a drop in urethral closure pressure. The increase in the distance between the inferior part of the pubic symphysis and the anterior vaginal wall comes about because of slackening of the elevator ani muscles. This slackening occurs at different times before the bladder contracts. The urethra opens; the complete course of this organ is well defined. Things return to their previous state when voiding finishes. In the case of stress incontinence, the lack of transmission of pressure urodynamically found when the woman is coughing can be seen as a sliding mechanism within the space of Retzius and at the urethro-vesical junction behind the symphysis pubis. The degree of sliding depends on the strength of the cough. In all cases of pure stress incontinence without there being low urethral closure pressure, a maximum stress caused by coughing will produce more than 5 MM sliding before the urethra opens. If the urinary incontinence is due to low urethral closure pressure, the urethra opens without sliding of the urethro-vesical junction whenever the abdominal pressure increases. Urethral instability resembles voluntary voiding but without any voluntary command. "Prepubic" contractions, longitudinal contractions in the sphincteric area and slackening of the levator ani muscles, alone or in association, explain why urethral closure pressure drops. Sometimes this drop is followed by an increase in bladder pressure.

Female↗

PubMed1987

An anatomical investigation on the muscles of the pelvic outlet in crab-eating monkey (Macaca irus) and Japanese monkey (Macaca fuscata) with special reference to their nerve supply.

For a fuller understanding of the nerve supply to the muscles of the pelvic outlet in man, 4 pelvic-halves of crab-eating monkeys and 6 pelvic-halves of Japanese monkeys were minutely dissected. 1) The composition of the pudendal plexus tends to be limited to 2 segments, S1 and S2. 2) If we consider the origin of the nerves, the pudendal plexus is divided into 2, the first or medial and the second or lateral parts. From the first part arise the pelvic splanchnic nerves, the nerve to the iliopubocaudalis and the nerve to the ischiocaudalis. From the second part the dorsal nerve of the penis or clitoris, the perineal nerve and the inferior rectal nerve originate. 3) A laminate arrangement exists among the 3 nerves from the first part at their origin; the pelvic splanchnic nerves are situated ventrally, and the nerve to the ischiocaudalis dorsally, with the nerve to the iliopubocaudalis in between. No obvious laminate arrangement exists among the 3 nerves from the second part. 4) The part of the iliopubocaudalis, contiguous with the rectal wall but without attaching to it, has no branches of supply from the perineal nerve and the inferior rectal nerve. 5) A detailed discussion of our findings in relation to man, is given below.

Animals↗

PubMed1986

Cutis marmorata telangiectatica congenita with multiple congenital anomalies.

Cutis marmorata telangiectatica congenita (CMTC) is a rare cutaneous vascular anomaly. Congenital persistent livedo reticularis, telangiectases, and superficial ulceration are the hallmarks of this syndrome. There is an association of CMTC with other congenital abnormalities in at least 50% of the patients. A newborn girl is described with a diagnosis of CMTC combined with multiple congenital abnormalities including imperforate anus, rectovaginal and urethrovaginal fistulas, and absent clitoris. These abnormalities have not been previously reported with CMTC. Congenital abnormalities that have been recognized with this syndrome are reviewed.

Abnormalities, Multiple↗

PubMed1986

[Familial esophageal leiomyomatosis associated with Alport's syndrome in a 9-year-old boy].

The authors describe a family in which the mother and one son are affected by oesophageal leiomyomatosis and nephritis with haematuria. The mother also presents hypertrophy of vulva and clitoris, and her son has perceptive deafness and congenital cataract. In the medical literature only 15 cases of oesophageal leiomyomatosis in children and adolescents could be found. The association with Alport's syndrome was first described by Torres and Guarner in 1983.

Adult↗

PubMed1985

[45,X/46,X,del(Yq) sex chromosome mosaicism--analysis of the phenotypic expression].

Three female patients with Turner-syndrome (sexual infantilism, short stature and somatic Turner-stigmata) have been analysed cytogenetically by means of different banding techniques. A deletion of the distal heterochromatic band Yq12 of the Y chromosome was observed in a mosaic with a 45,X-cell line, i.e. the karyotype is 45,X/46,X,del(Y)(q12). In order to get information about the phenotypic expression of the 45,X/46,X,del(Yq) mosaicism all previously published cases have been reviewed. Comparing the phenotypes of all 45,X/46,X,del(Yq) mosaic cases three different phenotype categories of sexual development can be distinguished: female individuals with sexual infantilism and Turner-stigmata, individuals with ambiguous genitals, ranging from clitoris hypertrophy of female genitals to hypospadia of males, male individuals, who are infertile (azoospermic). A comparison of the appearance of external genitals with the status of gonads of all patients revealed an unequivocal relationship between the gonad status and the resulting phenotype category. Furthermore, the role of Y-chromosomal loci determining testicular differentiation (biological function of H-Y antigen) for male development has been emphasized. The effect of the 45,X-cell line on the expression of short stature and somatic Turner-stigmata is independent of sexual development. Considering the great phenotypic variability of the 45,X/46,X,del(Yq) mosaicism it seems impossible to deduce a definitive phenotype. This problem is acute in prenatal diagnosis especially.

Adolescent↗

PubMed1985

Innervation of the anterior perineal muscles and its morphological consideration.

3 male and 3 female pelvic-halves were dissected carefully under a stereomicroscope in order to obtain more detailed data on the nerve supply to the anterior perineal muscles. At its origin from the pudendal plexus, the perineal nerve has a close relationship with the dorsal nerve of the penis or clitoris, while it tends to be separate from the inferior rectal nerve. The ischiocavernosus and the transversus perinei profundus are in general supplied by a common branch which arises from the perineal nerve at the posterior part of the ischiorectal fossa and takes a more lateral course, independent of the cutaneous branches. The bulbospongiosus and the transversus perinei superficialis receive several twigs from the medial and intermediate cutaneous branches of the perineal nerve. From the view point of nerve supply, the anterior perineal muscles may be classified into 2 groups: the lateral group comprising the ischiocavernosus and the transversus perinei profundus, the medial group comprising the bulbospongiosus and the transversus perinei superficialis.

Female↗

PubMed1979

[Pelvic visceral arteries of rabbits (Oryctolagus cuniculus)].

40 pelvic preparations of rabbits (oryctolagus cuniculus) were bilaterally studied by dissection under the stereomicroscope and angiography. The arterial pattern of the pelvis, i.e. origin and branching of the umbilical, urogenital and internal pudendal arteries (visceral branches), is described. The main characteristics observed are as follows: (1) The umbilical artery is permeable in adults and gives origin to the cranial vesical artery and a caudal branch that irrigates the pelvic urogenital organs and, eventually, the rectum, with six patterns of branching in both sexes. (2) Usually, the urogenital artery is the continuation of the visceral branch of the internal iliac artery. In 1 animal, unilaterally, it arises from the median sacral artery. In 12 animals (6 bilaterally and 6 unilaterally) the urogenital artery is absent. When present, it forms two branches, a cranial and a caudal one, that irrigate of the urogenital organs in both sexes. (3) The internal pudendal artery is the direct continuation of the internal iliac artery and gives to rise to some visceral branches that irrigate the penis, bulbourethral gland and rectum (with six patterns of branching) in males, and the vagina, clitoris and rectum (with three patterns of branching) in females.

Animals↗