Spontaneous Cervicovaginal Fistula in Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Case Report.

BACKGROUND: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare diagnosis; patients classically present with dysmenorrhea and increasing pelvic pain. Unusual manifestations of OHVIRA syndrome might occur as a result of patient anatomy and the rupture or relief of the obstructed hemivagina. CASE: We present a 15-year-old patient with OHVIRA syndrome who developed a spontaneous cervicovaginal fistula and subsequently underwent hemihysterectomy.

Congenital urethrovaginal fistula with transverse vaginal septum.

Congenital urethrovaginal fistula is an extremely rare genitourinary anomaly. Literature search identified only five reported cases, all of which were associated with urogenital abnormalities. Transverse vaginal septum is another rare condition, resulting from abnormalities in the vertical fusion between the vaginal components of the Mullerian ducts and the urogenital sinus; and associated fistulous connection of the vagina with the urethra is even rarer. Herein we describe the case of a 35-year-old woman who presented with dyspareunia, and a 1-year history of infertility, who was found to have a urethrovaginal fistula with low transverse vaginal septum. The patient was successfully treated with excision of the septum and closure of the urethrovaginal fistula.

A study of the functional aberration of the pouch in anorectal malformation associated with congenital pouch colon.

OBJECTIVE: There are no in vitro studies of congenital pouch colon (CPC) associated with anorectal malformation (ARM) to understand its mechanism of contractility. There is no consensus regarding the utilization of the CPC in repair of anorectal malformation. In view of the above, it was decided to carry out detailed in vitro physiological study of the excised CPC. METHOD: The excised specimens of CPC were taken and 15- to 20-mm long both longitudinal and transverse strips of muscle were obtained both from the proximal and distal part. Contraction was recorded using software chart-5 for windows. Drugs used were acetylcholine, histamine, adrenaline, atropine and pheniramine maleate. The amplitude of contractions was converted to tension gram and then the tension so developed was expressed as tension per unit mass (per gram of wet tissue). RESULTS: There were total of 21 specimens of high ARM with CPC. Five specimens were of the complete pouch, which did not show a response to any drug and the remaining 16 had an incomplete pouch. The mean longitudinal and circular muscle contractions of these 16 samples were statistically higher for the proximal segment than the distal segment both for acetylcholine and histamine. The effect of atropine was not significant but that of adrenaline and pheniramine maleate were significant on the distal segment muscle. CONCLUSION: Congenital pouch colon being deficient or having poorly developed receptors in their wall can not function properly as a reservoir for faeces and, hence, it should be excised to allow adequate function.

Congenital urethrovaginal fistula with imperforate hymen: a first case report.

BACKGROUND: Congenital urethrovaginal fistulas are rare, and all five reported cases have been associated with urogenital abnormalities. We describe a case of congenital urethrovaginal fistula with an imperforate hymen and no other urogenital abnormalities. CASE: A 25-year-old female sought medical advice regarding cyclical hematuria, dyspareunia, and infertility of four years' duration. Investigations disclosed a urethrovaginal fistula with an imperforate hymen. The patient was successfully treated with excision of the hymen and closure of the urethrovaginal fistula. CONCLUSION: In this instance, the diagnosis of congenital urethrovaginal fistula was delayed until adulthood because the presence of urinary incontinence, the usual presentation of a urethrovaginal fistula, was concealed by the imperforate hymen. We could find no previously reported case of urethrovaginal fistula with imperforate hymen.

Management of congenital pouch colon based on the Saxena-Mathur classification.

BACKGROUND: Management guidelines with regard to congenital pouch colon (CPC) are not clearly defined with regard to the type of pouch present. The aim of this study was to outline the management strategy and surgical approach to CPC using the Saxena-Mathur classification based on anatomical morphology of the pouch. METHODS: During a 12-year period (1995-2007), 426 patients were surgically managed for anorectal malformations at the RNT Medical College, Udaipur. Congenital pouch colon was documented in 80 patients and categorized into 5 types according the anatomical morphology. The management strategy depended upon the location of the pouch and its condition at the time of the surgery. RESULTS: In type 1 and type 2 CPC, a 1-stage (pouch excision and pull-through) or 3-stage procedure (ileostomy, pouch-coloplasty with pull-through, and ileostomy closure) was performed depending on the condition of the pouch (ischemic or healthy). In type 3 and type 4 CPC, a 3-stage procedure (pouch excision with colostomy, pull-through, and colostomy closure) was performed in all patients. In type 5 CPC, a 3-stage procedure (distal pouch excision with proximal pouch-coloplasty with ileostomy, pull-through, and colostomy closure) was successful. CONCLUSION: Management of CPC patients according the Saxena-Mathur classification provides a well-defined algorithm in the surgical approach according to the anatomical morphology of the pouch.

Fetal urogenital sinus with consecutive hydrometrocolpos because of labial fusion: prenatal diagnostic difficulties and postpartal therapeutic management.

OBJECTIVE: To elucidate the differential diagnoses of tumorous dilations in the fetal pelvic region detected by prenatal ultrasound and the postnatal management demonstrated on a fetus with 29 weeks of gestation with a retrovesical located bottle-like cystic structure measuring 54 x 31 x 27 mm within the pelvis. Postnatal findings were a labial fusion and a consecutive hydrometrocolpos due to a urethrovaginal fistula. METHOD: Case report of a fetus affected by an intricate cloacal anomaly. RESULTS: The long-term prognosis for this nonsyndromic form of hydrometrocolpos without any other structural defects or organic failures after operative sanitation is excellent. Final reconstruction is planned at about 12 months of age. CONCLUSION: Prenatal diagnosis of tumorous dilations in the fetal pelvic region often involves difficulties because of numerous differential diagnoses and possible presentation in late pregnancy. Magnetic resonance imaging could be a useful complementary tool for assessing these anomalies when ultrasonography is inconclusive. In some cases, the final diagnosis cannot be confirmed until after delivery.

Management of congenital and acquired H-type anorectal fistulae in girls by anterior sagittal anorectovaginoplasty.

METHODS: Thirteen girls with congenital or acquired H-type anorectal fistulae underwent surgery between 1991 and 1996. In all cases, besides a normally placed anal canal, there was a fistulous communication between the anorectum and the genital tract. On the basis of the level of fistulous communication, these cases were divided into three groups: high, intermediate, and low (perineal canal). All patients underwent anterior sagittal anorectovaginoplasty. Surgical technique included division of all intervening tissue in midline between the perineal skin and the fistula. The whole fistulous tract was excised, and the remaining surrounding tissue was repaired in different layers. Of 13 patients, 12 were operated on without a protective colostomy. RESULTS: There was no recurrence in any case, and all patients had good cosmetic results with a normal sphincter control. Although various techniques have been suggested for the surgical correction of H-type anorectal fistulae, most of them are applicable only to the low-lying fistula (perineal canal). CONCLUSIONS: To date, there is no satisfactory method available for correction of high fistula. The methods suggested for high fistula (abdominoperineal pull-through and endorectal pull-through) appear to be too extensive for this condition. Our technique of anterior sagittal anorectovaginoplasty can be used not only for low fistula but can also be used for intermediate and high types of fistulae. This technique is simple, safe, takes less time, and achieves good anatomic and functional reconstruction of the perineum.

Persistent urogenital sinus: prenatal diagnosis and pregnancy complications.

Prenatal diagnosis of fetal hydrocolpos caused by persistent urogenital sinus is described. The urethrovaginal communication allowed urine to empty from the bladder into the vagina, which subsequently dilated, causing bilateral hydronephrosis, distention of the fetal abdomen, decreased urinary output, oligohydramnios, and abdominal dystocia at delivery.

Genital abnormalities in females with Bardet-Biedl syndrome.

The major manifestations of the Bardet-Biedl syndrome are digital anomalies, tapetoretinal degeneration, obesity, renal abnormalities, and hypogenitalism (described mainly in males). We report on 2 girls with Bardet-Biedl syndrome who also had vaginal atresia. A similar association in females with Bardet-Biedl syndrome was suggested in published reports of 11 affected individuals who had structural genital abnormalities, (some of which were missed in childhood), including persistent urogenital sinus, ectopic urethra, hypoplasia of the uterus, ovaries and fallopian tubes, uterus duplex, and septate vagina. The association of atresia of the vagina and other malformations of female genital structures in individuals with Bardet-Biedl syndrome has often been missed in childhood and should be looked for more systematically.

Congenital anal fistula with normal anus.

Three patients with a congenital anovestibular/perineal fistula were treated at the Montreal Children's Hospital. Two females (one of East Indian and the other of Japanese origin) had anovestibular fistulae that became symptomatic in the first few months of life. The third patient, a boy of Korean descent, presented at 9 months of age with a chronically draining perineal fistula. During surgery, a small fistula tract was easily dissected out and excised. Microscopic examination showed a well-preserved rectal mucosa throughout the tract. Most male patients described to date had anourethral fistulae, often accompanied by other major anomalies such as esophageal atresia or renal malformations. We believe our patient is the first male to be described with a congenital perineal fistula; this suggests that some fistula-in-ano in male infants may be due to a congenital sinus that secondarily becomes infected and drains to the skin.

Chromosomal evaluation of a ewe lamb with atresia ani vaginalis.

Chromosomes of a ewe lamb born with atresia ani vaginalis were examined after a 72-hour culture of peripheral whole blood. The 2n number of chromosomes was 54,XX, with no apparent deviation from normality. Pedigree analysis of the ewe lamb indicated that her sire and dam were only slightly related (Rsd = 0.59%); therefore, the amount of inbreeding of the lamb was small (Fx = 0.30%).

Failure of fusion between an upper and a lower segment of the vagina.

A unique malformation of the vagina with a tiny fistula between an upper and a lower segment of the vagina is presented with a brief summary of the theories concerning the development of the vagina.