Bifid Clitoris Research Articles

Management of cloacal exstrophy: Experience from tertiary hospital, Tanzania. Case series

Introduction and importanceCloacal exstrophy (CE) is defined as a complex anomaly that affects the urogenital and intestinal tracts. It is the most serious form of anomaly that is described within the so-called exstrophy-epispadias complex. These malformations usually present a challenge in the management of particular conditions, as most of these forms require multiple surgeries, resulting in the use of multidisciplinary approaches, including reconstructive urologists, pediatric surgeons, orthopedic surgeons, endocrinologists, pediatricians, psychologists and nutritionists. Additionally, these patients present with ambiguous genitalia, which is another aspect that needs to be taken into consideration during the management of this condition. Case presentationThe first patient, a baby who was 8 days of life and referred from a peripheral hospital, presented with classic features of cloaca exstrophy. He underwent first-stage cloacal exstrophy repair. The intraoperative findings included a bi-halved bladder and phallus, and the ureters were not appreciated, but there was continuous urine leakage from the bi-halved bladder and no uterus or ovaries. Poorly formed cecum, cecal-cutaneous fistula with an everted part of the terminal ileum protruding outside (mucosa-out), no transverse, no descending colon, collapsing small bowel, left undescended testis in the inguinal region, and right abdominal undescended testis. He first underwent surgery, which involved ileostomy, omphalocele closure and proper bladder exstrophy construction.The second patient, a 6-day-old female, had a similar presentation and physical findings as the first patient did, except that she had elephantoid trunk deformity with a cecal fistula, bifid clitoris, two cervical orifices, and two uteri completely separated with ovaries. Mobilization of the hindgut, closure of the cecal fistula, and proper bladder exstrophy after repair of the posterior wall were performed.The third patient was a 10-day-old female, similar to the second patient, but this patient presented with a left leg deformity with wide diastasis. In this case, the urinary bladder was not bivalved, and the cecal fistula had perforated just below the posterior wall of the urinary bladder. A mild omphalocele, bifid clitoris and vagina, one cervical orifice, and two uteri completely separated, with ovaries observed. Mobilization of the hindgut, closure of the cecal fistula, and proper bladder exstrophy after repair of the posterior wall were performed. The postoperative period was uneventful. Clinical discussionSurgical management of cloacal exstrophy is typically undertaken in the newborn period (48–72 h) as a combined effort between pediatric surgery and urology. In the setting of associated spinal dysraphism, neurosurgical consultation and closure should be undertaken as soon as the infant becomes medically stable. Early operation minimizes bacterial colonization of exposed viscera and may decrease the need for pelvic osteotomy. The goals of treatment include securing the abdominal wall and bladder closure, preserving renal function, preventing short bowel syndrome, creating functional and cosmetically acceptable genitalia, and attaining acceptable urinary and fecal continence. ConclusionCloacal exstrophy remains a rare and complex congenital anomaly characterized by an array of anatomical defects affecting multiple organ systems. With respect to the approach of this congenital malformation, it is therefore important that these individuals and their families remain under the care of a multidisciplinary team of providers who can offer medical care, counseling and life-long follow-up.

Complete Female Epispadias with Urinary Incontinence: A Single-Stage Perineal Urethroplasty and Cystoscopic-Guided Bladder Neck Plication

Abstract Isolated female epispadias without bladder exstrophy is an extremely rare congenital anomaly. The presenting features of female epispadias are urinary incontinence and abnormal anatomical features. A 4-month-old girl presented with primary urinary incontinence since birth. On physical examination, she had a bifid clitoris and labia minora along with an anteriorly placed introitus and wide-open bladder neck. The vagina and hymen were normal. With the diagnosis of isolated complete female epispadias, a single-stage reconstruction of the urethra, labia minora and clitoris was performed along with cystoscopic-guided bladder neck plication. This index case highlights the surgical technique and the necessity to operate on these patients at a younger age in infancy with a single-stage perineal repair to achieve better functional and cosmetic outcomes.

Delayed presentation of isolated female epispadias with urinary incontinence: a case report

BackgroundFemale epispadias is a rare congenital anomaly, with a quoted incidence of 1 in 480,000.These patients present with urinary incontinence and varying degrees of external genitalia abnormality. This report presents the delayed presentation and management of a 14-year-old female with urinary incontinence due to isolated epispadias.Case presentationThis is the case report of a 14-year-old female with urinary incontinence noticed since infancy. Examination of the genitalia revealed a bifid clitoris, depressed mons pubis, underdeveloped labia, patulous urethral opening, and normal vagina. She had surgical repair of the anomaly and has subsequently achieved continence. She has been continent in the last 4 ½ years of follow-up and is now married with a 1-year-old child.ConclusionFemale epispadias is a rare but often missed cause of incontinence. A high index of suspicion and careful genital examination will help in identifying this anomaly. Surgical correction achieves satisfactory outcome as regards continence and cleanliness. Long-term follow-up is essential.

Congenital urinary incontinence in a 4-year old girl due to isolated epispadias: A case report and literature review

Epispadias is a congenital anomaly in which the urethral meatus opens dorsally on top of the penis, medial to the glands, or with absent or bifid clitoris in females. It often occurs as part of a complex anomaly called the bladder extrophy epispadias complex (BEEC) along with exposed bladder and bifid pelvic brim. The diagnosis of bladder extrophy epispadias complex is often clinical with identifications of the various component of the anomalies. Although this condition is rare it present with wide spectrum of deformities which ranged from mild to more complex anomalies, its management especially the complex variety has been a major challenge to pediatric surgeons worldwide. With a male to female ratio of 2-3:1, the occurrence of isolated epispadias in female patient is even rarer. Our patient is a 4-year old girl who presented with urinary incontinence since birth. She has never achieved urinary continence up to the age of 4-years, there is no history of any prior urethral procedures, no history of any trauma to the perineum, no passage of stone or blood in the urine. Child is the second of her mother’s three children and her younger ones has achieved urinary continence. Pregnancy, delivery and puerperal history were essentially uneventful. On examination, we found a young girl, calm, but apprehensive otherwise stable, abdomen was full, moves with respiration, no area of tenderness, no palpable organomegaly, no intra-abdominal masses felt, there was distorted female genitalia with bifid clitoris and patulous urethral meatus. There was a normal vaginal orifice with intact hymen with poorly developed mons pubis and labia. A clinical diagnosis of isolated epispadias with urinary incontinence to rule out ectopic ureter in a 4-year old girl was made. She was worked up and prepared for surgical repair, and had a single stage procedure done. Post-operative cosmetic appearance of the genitalia was acceptable. Urethral catheter was removed on the postoperative day 10 and patient had achieved some continent of urine. She generally did well and has been on outpatient follow up visits at the pediatrics surgical outpatient clinic.

Single-stage repair for female epispadias with urinary incontinence: A case report and literature review.

BackgroundEpispadias is a rare condition. Epispadias in females is two times less common than in males. Female epispadias range from 1 in 160,000 to 480,000 live births. Epispadias can be diagnosed through careful physical examination of the genital. Surgery is the management of epispadias. Surgical management of epispadias is quite tricky and requires expertise. The literature that discusses female epispadias is challenging to be found. In this paper, we would like to report surgical management of isolated female epispadias in Cipto Mangunkusumo Hospital, Jakarta.Case presentationA 7-year-old girl was admitted with a chief complaint of urinary incontinence since birth, during daytime and nighttime. Urinary incontinence was not induced by activities nor worsened by eating/drinking. Physical examination showed that the patient's external genitalia has underdeveloped labia minora, patulous urethra, bifid clitoris, and multiple hypopigmentation lesions. Laboratory results were in the normal range. The voiding cystourethrography (VCU) result revealed urine leaks during the filling phase. The bladder wall was normal, and no vesicoureteral reflux (VUR) appeared. The urethrocystoscopy shows a more vertical OUE, a wide-open bladder neck, and a urethral length of 1.5 cm. The patient underwent single-stage surgical procedures that consist of epispadias repair and bladder neck reconstruction through a subsymphyseal perineal approach. No complications occurred intra-operative and post-operative. At the 1-week and 6-months follow-up, the patient achieved urinary continence, and the surgical wound healed normally.DiscussionEpispadias is a rare condition that could occur in various degrees, from mild to severe degrees. To a severe degree, there is a split at the entire urethral and involves the bladder neck causing constant incontinence for the patient. Epispadias cases are quite challenging to diagnose. The physician needs to separate the labia majora and perform the physical examination carefully. The objective goals of surgical management of epispadias are to achieve urinary continence, restoration of anatomy, function, and cosmetic appearance of urethra and genitalia. Single-stage reconstruction is the current surgical method used to repair isolated female epispadias.ConclusionFemales epispadias is a rare congenital anomaly that is quite often to miss diagnosed. The single-stage technique is the recommended surgical technique for isolated female epispadias. This patient has achieved urinary continence, and no complications occurred during and post-surgery.

The Richard Grady Monsplasty: A vertical Z-plasty technique

Classic bladder exstrophy in the female results in an exstrophic bladder and urethra, an anterior introitus with a bifid clitoris and short labia minora. During closure, the lower abdominal wall is closed and the bifid clitori are brought into close apposition, but are often not completely closed to prevent injury to the clitoral bodies, thus leaving a persistent gap between the clitoral bodies that grows over time. We demonstrate a vertical z plasty closure to provide a 2 layer closure of the mons that decreases tension and improves cosmetic appearance by recreating a clitoral hood that provides a more normal appearance of the external genitalia for girls with bladder exstrophy.

V09-09 THE RICHARD GRADY MONSPLASTY

INTRODUCTION AND OBJECTIVE: Classic bladder exstrophy in the female results in an exstrophic bladder and urethra, an anterior introitus with a bifid clitoris, and short labia minora. During closure, the lower abdominal wall is closed and the bifid clitori are brought into close apposition, but are often not completely closed to prevent injury to the clitoral bodies. The lower abdominal wall is often under tension, leading to gradual spreading of the distance between the clitoral bodies. We demonstrate a vertical z plasty closure to provide a 2-layer closure of the mons that decreases tension and improves cosmetic appearance of the female external genitalia by creating a clitoral hood. METHODS: This technique was developed as part of an international collaboration focused on the repair of bladder exstrophy and epispadias. After closure of the lower abdominal fascia, 2 lateral incisions are made just superior to the clitoral bodies. The inferior edges of these incisions are re-approximated in the midline in a subcuticular fashion with absorbable monofilament suture, thus forming the deep, posterior ledge of the skin closure. Next, the superior cut edges are re-approximated in a similar fashion, creating the superior/outer ledge of the mons. This closure is then extended and joined with the abdominal skin closure. RESULTS: This technique has been used as an adjunct in newborn primary closures and in older patients who present for cosmetic revision. The extension of the mons to create a clitoral hood eliminates the appearance of a protruding bifid clitoris. There have been no wound infections, skin breakdowns, or early clitoral separations. CONCLUSIONS: The vertical Z-plasty closure of the mons creates a more normal appearance of the external genitalia of girls with bladder exstrophy and epispadias by developing a clitoral hood, while also providing a robust, multi-layer closure that decreases tension and the risk of wound dehiscence. Source of Funding: None

Exstrophy of Cloaca Sequence (Oeis Complex) With Multiple Cardiac Malformations

Omphalocele, Exstrophy, Imperforate anus, Spinal defects (OEIS) complex is the most severe birth defect within the exstrophyepispadias complex. There is exstrophy of the cloaca, failure of fusion of the genital tubercles and pubic rami, omphalocele and incomplete development of the lumbosacral vertebrae with hydromyelia. The diagnosis of OEIS complex mainly relies on sonographic findings. Our case presented with microcephaly, omphalocele, syndactyly, hydromyelia, imperforate anus, single cloacal opening, bifid clitoris, prominent unfused pubic rami and left renal agenesis. In addition, multiple severe cardiac malformations were found on echocardiography. Prognosis is poor when the OEIS complex is compounded by life-threatening malformations. We report a rare case of a preterm neonate presenting with features of OEIS complex with multiple cardiac malformations.

Fertility and the Reproductive Tract in a Woman with Caudal Duplication Syndrome

Abstract Background: Caudal duplication syndrome is a rare entity characterized by complete duplication of the genitourinary system and hindgut structures. There have been 13 case reports of women with genitourinary duplication; only 3 had complete caudal duplication and only 1 had a successful term pregnancy. There are no case reports to date of patients having more than 1 successful pregnancy with this syndrome. Case: A 28-year-old multiparous woman with known caudal duplication syndrome presented with recurrent urinary tract infections and hydronephrosis. This patient was interested in having a unification of her two vaginas. An examination under anesthesia revealed two gluteal clefts, three labia minora, two vaginas widely separated by >4 cm, a bifid clitoris, two normal cervices, two urethras, a right perforate anus, and a left imperforate anus. Exploratory laparotomy revealed uterine didelphys, 2 bladders, 2 ureters, and 2 kidneys. Results: Excision of the left bladder and ureteral reimplantation re...

V7-04 MONS PLASTY: A TECHNIQUE FOR OPTIMAL AESTHETIC OUTCOME

You have accessJournal of UrologyPediatrics1 Apr 2017V7-04 MONS PLASTY: A TECHNIQUE FOR OPTIMAL AESTHETIC OUTCOME Kelly Nast, Diana Cardona-Grau, George Chiang, and Antoine Khoury Kelly NastKelly Nast More articles by this author , Diana Cardona-GrauDiana Cardona-Grau More articles by this author , George ChiangGeorge Chiang More articles by this author , and Antoine KhouryAntoine Khoury More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2017.02.1922AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES It has been suggested that women who have undergone repair for exstrophy-epispadias complex (EEC) have unsatisfactory cosmetic results possibly leading to decreased self-esteem. Here we aim to show a mons plasty technique to be incorporated at the initial EEC closure that creates anatomically correct external female genitalia as well as improves the aesthetic outcome. METHODS Briefly, the surgical technique incorporates approximation of the bifid clitoris in the midline, aligning the labia minora along the lateral aspect of the introitus, creation of a clitoral hood, superomedial rotation of the labia majora, and finally mobilization of peripubic adipose to create a mons. RESULTS In our experience the surgical technique was successful in creating anatomically normal appearing external genitalia with subjective parental satisfaction. The patient has not had any complications. A post-operative VCUG showed grade 3 left vesicoureteral reflux. She is not yet potty trained but does have apparent normal voiding with dry periods throughout the day. CONCLUSIONS The technique described restores normal anatomic appearance as well as improves the cosmetic outcome. This technique should be considered in one-stage female EEC closure. © 2017FiguresReferencesRelatedDetails Volume 197Issue 4SApril 2017Page: e824 Advertisement Copyright & Permissions© 2017MetricsAuthor Information Kelly Nast More articles by this author Diana Cardona-Grau More articles by this author George Chiang More articles by this author Antoine Khoury More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

A rare case of isolated female epispadias

Isolated female epispadias without bladder exstrophy is an extremely rare entity. It usually presents with primary urinary incontinence and abnormal external genitalia. We describe this rare congenital anomaly in a 7-year-old girl who presented with urinary incontinence and abnormal external genitalia. She had a bifid clitoris and labia minora were widely separated. The vagina was normal. Voiding Cysto-urethrography (VCUG) showed small capacity bladder with no reflux. Surgical reconstruction of the urethra, labia minora and clitoris was performed.

A case of isolated female epispadias and our approach to treatment

Cases of isolated female epispadias (IFE) without exstrophy of the bladder are quite rare. The clinical symptoms of IFE are abnormal external genitalia with primary urinary incontinence.Our patient was a 7-year-old girl with total urinary incontinence. Physical examination revealed a patulous urethra, bifid labia minora, and a bifid clitoris. The vagina and hymen appeared to be normal. Uroflowmetry demonstrated an overflow-type pathological voiding pattern with low voiding pressure. A voiding cystourethrogram showed no indications of reflux.To treat our IFE patient, we performed a single stage genital approach reconstruction of the urethra, clitoris, and labia minora. Follow up with the patient ensured that she was continent and that her external genitals took on an acceptable appearance.

V3-06 FEMALE EPISPADIA: A RARE ABNORMALITY TREATED BY PERINEAL AND INFRAPUBIC APPROACH

You have accessJournal of UrologyPediatrics1 Apr 2014V3-06 FEMALE EPISPADIA: A RARE ABNORMALITY TREATED BY PERINEAL AND INFRAPUBIC APPROACH Antonio Macedo, Rodrigo Trivelato, Marcela Leal, Sergio Ottoni, Gilmar Garrone, Riberto Liguori, Bruno Leslie, and Valdemar Ortiz Antonio MacedoAntonio Macedo More articles by this author , Rodrigo TrivelatoRodrigo Trivelato More articles by this author , Marcela LealMarcela Leal More articles by this author , Sergio OttoniSergio Ottoni More articles by this author , Gilmar GarroneGilmar Garrone More articles by this author , Riberto LiguoriRiberto Liguori More articles by this author , Bruno LeslieBruno Leslie More articles by this author , and Valdemar OrtizValdemar Ortiz More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2014.02.1307AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Female epispadia is a rare congenital anomaly occurring in 1 of 500,000 live births. The male/female ratio is 3 to 4:1. Most of the patients present with urinary incontinence and a genital deformity characterized by a bifid clitoris, depressed mons pubis, and ill-defined labia minora, is covered by smooth glabrous skin, and terminates anteriorly with the corresponding half of the bifid clitoris. The vagina and internal genital organs are usually normal. We aimed to demonstrate the perineal infrapubic approach to treat this rare abnormality. METHODS Patient presented at an age of . We defined and created a flap of the mucosal urethral plate located from the epispadic meatus up to anterior supra-pubic area between both bifid clitoris. The flap was moved inferiorly and gave access to pubic bone. A careful infra-pubic dissection of the flap was performed to allow further bladder neck and proximal urethra tightening. We tubularized the mucosal flap excising redundant mucosal tissue adapting the urethra for a 6F catheter. Subsequent overlapping and folding of adjacent muscular and subcutaneous tissue in two layers was done. The labia minora plasty and monsplasty were performed and bifid clitoris were approximated in the midline. Vulvoplasty was completed and distal urethral meatus was anchored cranially inferiorly to clitoral hood. RESULTS Patient had an uneventful postoperative course, the Foley tube was removed after 7 days. Present follow-up is 5 months, parents reported urinary continence of a 3 hours period. CONCLUSIONS The one-stage perineal urethroplasty increases the urethral and bladder neck resistance, mainly by elongating urethral segment and folding adjacent muscular and subcutaneous tissue. It is a simple and effective method for the development of both continence and cosmesis in female epispadias, avoiding initial abdominal surgery that could be reserved for the failure cases. © 2014FiguresReferencesRelatedDetails Volume 191Issue 4SApril 2014Page: e422-e423 Advertisement Copyright & Permissions© 2014MetricsAuthor Information Antonio Macedo More articles by this author Rodrigo Trivelato More articles by this author Marcela Leal More articles by this author Sergio Ottoni More articles by this author Gilmar Garrone More articles by this author Riberto Liguori More articles by this author Bruno Leslie More articles by this author Valdemar Ortiz More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

Female Epispadiasis: A Case report

Isolated female epispadias without exstrophy is rare to see. It is often overlooked cause of incontinence in a female child. A case of isolated female epispadias without exstrophy in an 11-year-old girl who presented with primary urinary incontinence since birth is reported. She had never had a genitalia examination done by any treating clinician. On examination, she had a bifid clitoris and ill developed labia minora. Voiding cystourethrogram showed small sized bladder with no reflux. Bladder neck reconstruction was done transvesically, with tubularisation of urethra. The case report reflects the importance of physical examination in primary incontinence which can make an early diagnosis and treatment and prevent stigmata of incontinence. DOI: http://dx.doi.org/10.3126/jnps.v31i3.4415 J Nep Paedtr Soc 2011;31(3): 244-246

Female Covered Urethral Duplication With Urogenital Sinus

We report a covered urethral duplication in a girl presenting prenatally with an enlarged fluid-filled vulvar cyst, genital duplication, and urogenital sinus revealed by fetal magnetic resonance imaging (MRI) and serial ultrasounds. Physical examination revealed an enlarged vulvar mass covering the clitoris, a single orifice, and normally sited anus. Congenital adrenal hyperplasia was ruled out at birth. MRI in addition showed an accessory duct between the sinus and the urine-filled vulvar pouch with a bifid clitoris. A total urogenital sinus mobilization with resection of the accessory urethra and vulvoplasty was performed with uneventful follow-up.

Diagnosis and management of epispadias

Epispadias is a rare diagnosis and most commonly described as a part of the bladder exstrophy complex. Epispadias is characterized by failure of the urethral plate to tubularize on the dorsum with the defect ranging from a glandular to a penopubic location. In addition, male patients demonstrate a dorsal chordee whereas female patients exhibit a bifid clitoris. The goal of surgical correction is the placement of the meatus in its anatomical position and the creation of functional genitalia with good cosmetic outcomes. Two different major reconstruction principals, the modified Cantwell-Ransley and the Mitchell Bagli repair, are used in specialized centers worldwide. Both procedures comprise the major principles of epispadias surgery but differ in timing and the extent of urethral mobilization. In addition, both techniques have advantages and specific risks involved, and reports from supporting institutions demonstrate favorable results. Regardless of the approach, the reconstruction should be performed by experienced surgeons.

Single‐stage subsymphyseal cystoscopic‐guided bladder neck plication and urethrogenitoplasty in female epispadias: presentation of long‐term follow‐up

• To investigate the feasibility of an endoscopic technique as a guide to evaluate bladder outlet resistance during bladder neck plication (BNP) for treatment of female epispadias. • The postoperative outcomes with long-term follow-up of 10 girls with epispadias were reviewed (four had a previous history of bulking agent injection into the BN). • After taking a skin flap from the area between the crura of the bifid clitoris, subsymphyseal dissection was continued to the BN. • Under cystoscopic guidance, BNP was performed using absorbable sutures (two cases) or non-absorbable sutures (eight) aiming to attain a smooth closure of the BN during gradual withdrawal of the cystoscope. • Finally, urethrogenitoplasty was accomplished by urethral reconstruction with tubularization of the inter-clitorial flap followed by monsplasty and approximation of the two hemi-clitorises. • The mean (range) follow-up was 9.1 (5-15) years. • After primary surgery seven of the 10 girls were continent and all could void normally. • Of the three failed cases (including two that underwent BNP using absorbable suture), two became dry after injection of bulking agent into the BN and the remaining girl underwent BN reconstruction; she was socially continent at the final follow-up. • Ultimately, all 10 girls were socially continent, including six who were completely dry. • Urodynamic studies showed an increase in mean bladder capacity (P < 0.001) and a significant time-dependent improvement of the leak-point pressure and maximum urinary flow rate. • The present series suggests that single-stage subsymphyseal urethrogenitoplasty together with cystoscopic-guided BNP for management of female epispadias is reliable and effective with definite advantages. • The high success rate and safety of the procedure are important factors for the introduction of this method as a valid option for treatment of this rare congenital anomaly.

Female epispadias

Isolated female epispadias without bladder exstrophy is an extremely rare congenital anomaly. The symptoms of female epispadias are primary urinary incontinence and abnormal anatomical features. A 7-year-old girl presented with partial incontinence of urine. On physical examination, bifid clitoris and labia minora were seen. The vagina and hymen were normal. Voiding cystourethrogram showed no reflux. With the diagnosis of isolated female epispadias, single stage reconstruction of the urethra, labia minora and clitoris was performed.

Unusual Case of an Ectopic Clitoris

Purpose An ectopic clitoris is an extremely rare condition which is usually associated with severe defects of the urinary tract. Variances of this anomaly as a bifid clitoris are usually seen in epispadias-extrophy in girls with cloacal anomalies. There are only a few reports of an ectopic clitoris in the current literature. Material and Methods We present the case of an 11 year-old girl who underwent operative correction of anal atresia as a newborn. She was referred to our department for further evaluation because of an unusual appearance of the external genitalia. Clinical examination revealed malposition of the labia minora and absence of a clitoris. On the inner part of the left thigh a structure was seen which could be identified as an ectopic clitoris. X-Ray examination showed a 2 cm diastasis of pubic symphysis. During surgery the ectopic clitoris was completely mobilized and a neurovascular bundle with a single cavernous body was identified. The glans clitoridis and the neurovascular bundle were dissected from the cavernous body and the latter was transsected at the level of the left pubic ramus. Using a subcutaneous tunnel the clitoris was adapted tension free and brought to its correct anatomical position. The impression of the mons pubis was corrected with two overlapping subcutaneous flaps. Results Six months postoperatively a very good cosmetic and functional result was achieved. Conclusions We present the successful correction of an extremely rare genital anomaly in a female patient. In evaluating the relevant literature, we did not find a comparable case describing the surgical management.

Aesthetic aspects of reconstructive clitoroplasty in females with bladder exstrophy–epispadias complex

Genital anomalies in females born with classic bladder exstrophy-epispadias complex (EEC) include a bifid clitoris and anterior displacement of vagina. The aesthetic aspects of the genitals acquire greater significance with age and may affect self-esteem. We review our experience with clitoral reconstruction in an EEC population with an emphasis on aesthetic outcomes. The study group had 26 female patients; three patients with isolated epispadias and 23 with bladder exstrophy. The patients were separated into four separate groups based on reconstructive technique. Group 1 (n=3) had tubularisation of skin between clitoral bodies for urethral reconstruction; group 2 (n=12) underwent staged exstrophy reconstruction; group 3 (n=8) had initial surgery elsewhere and were referred for secondary reconstructive surgery with clitoroplasty accompanied by puboplasty to reconstruct the fourchette; and group 4 (n=3) had total urogenital sinus mobilisation. The aesthetic outcome was assessed subjectively by the surgeon, parent and age-appropriate patient during follow-up. A total of 33 clitoroplasties were performed. Primary clitoroplasty was performed in 18 patients, while a secondary clitoroplasty was done in 15. The follow-up ranged from 3 months to 31 years. Three patients had partial clitoral atrophy and in one patient the gap between clitoral bodies was widely separated and incapable of being joined. Twenty-three of the26 patients (88%) had a satisfactory or excellent aesthetic outcome following clitoroplasty. Excellent aesthetic outcomes can be achieved by clitoroplasty in EEC patients by using a variety of surgical techniques. We recommend a 'second look' reassessment near puberty to identify poor cosmesis and offer secondary clitoroplasty to improve aesthetic outcome.