Hydrocolpos causing bowel obstruction in a preterm newborn: a case report.

BACKGROUND: Imperforate hymen is the most common congenital defect of the female urogenital tract. The spectrum of clinical manifestations is broad, ranging from mild cases undiagnosed until adolescence to severe cases of giant intraabdominal masses. The most common complication of hydrocolpos is bladder compression, resulting in obstructive uropathy and hydronephrosis. CASE PRESENTATION: We present here the case of a preterm neonate who was admitted to the surgical neonatal intensive care unit for bowel obstruction. The baby did not appear septic or unwell, a small amount of meconium passed frequently, and no bilious gastric residuals occurred. Based on these findings, acute abdominal obstruction was doubtful, and the surgeon chose a conservative (watch and wait) approach. Subsequently, we performed abdominal ultrasound and magnetic resonance imaging based on unclear information about a suspicious abdominal mass raised by the gynecologist shortly before the emergency C-section. The final diagnosis was congenital hydrocolpos due to imperforate hymen. The pediatric gynecologist indicated an incision of the imperforate hymen under general anesthesia. The incision resolved abdominal distention as well as the bowel obstruction. CONCLUSION: The presentation of hydrocolpos was not typical (no bulging in the vaginal introitus) in our case, and clinical symptoms implied acute bowel obstruction shortly after birth. The surgeon chose a conservative (watch and wait) approach as the baby did not appear unwell on the second day of life. Fortunately, diagnostic laparotomy was not required as the next step in bowel obstruction management. All clinical symptoms resolved after a minor surgical procedure.

Hydrometrocolpos: a Contemporary Review of the Last 5 Years.

PURPOSE OF REVIEW: The goal of this review is to provide a comprehensive overview of hydrometrocolpos, covering disease etiology, pathophysiology, clinical presentation, and diagnostic and management techniques, and known outcomes. RECENT FINDINGS: This narrative review presents the literature on hydrometrocolpos in the pediatric population from the past 5 years. We highlight the 69 reported cases of hydrometrocolpos and classify them based on type of obstruction or associated anomaly, discuss new diagnostic algorithms based on imaging, and present novel and underutilized surgical techniques for definitive management. Hydrometrocolpos, a condition characterized by retained fluid causing a distended vagina and uterus in the setting of a distal vaginal outflow obstruction, has a wide range of presentation severity based on the type of obstruction. Whether hydrometrocolpos is due to an isolated condition like imperforate hymen, a complex abnormality like cloacal malformation, or a part of a large congenital syndrome, the mainstay of treatment is decompression of the dilated vagina and surgical correction of the outflow obstruction. Imaging-based diagnostic algorithms and new treatment techniques reported in the literature, as well as longitudinal and patient-reported outcome research, can improve the lives of children affected by this condition.

Fetal ascites in cloacal malformations-a red flag.

INTRODUCTION: Cloacal malformation is a rare anomaly that remains a diagnostic challenge prenatally, despite the current advances in ultrasonography and MRI. This condition can in some, present with isolated ascites or with other findings, such as a pelvic cyst or upper urinary tract dilatation. In a minority, the ascites may be progressive, questioning the role of antenatal intervention. METHODS: We report on ten patients that have been identified from our Cloaca database between 2010 and 2022. RESULTS: The presence of ascites was associated with extensive bowel adhesions and matting, leading to a challenging initial laparotomy and peri-operative course. CONCLUSIONS: Antenatal finding of ascites in newborns with cloacal malformations should raise a red flag. The surgeon and anaesthetist should be prepared for the operative difficulties secondary to bowel adhesions and the higher risk of haemodynamic instability at the initial surgery. An experienced team at initial laparotomy in such patients is vital. LEVEL OF EVIDENCE: II.

A case of sacrococcygeal teratoma associated with antenatally acquired urethrovaginal fistula and hydrocolpos.

BACKGROUND: Sacrococcygeal teratomas (SCTs) are known to cause urological complications, but urethrovaginal (UV) fistula as a complication of SCT is rare. We herein report a case of SCT with UV fistula and hydrocolpos. CASE PRESENTATION: A 1-day-old female neonate presented to our department with prominent swelling in the sacrococcygeal region. She was born at 37 gestational weeks via spontaneous vaginal delivery from a 39-year-old woman. The weight of the baby was 2965 g, and her Apgar scores were 4/10 (at 1 and 5 min). An MRI examination confirmed an 11 × 11 cm Altman classification typeII SCT associated with hydrocolpos, a dilated urinary bladder, and bilateral hydronephrosis. When she was 5 days, the SCT was excised totally and a coccygectomy was performed. After the operation, as her urinary output appeared unstable, a cystoscopic examination was performed on the third postoperative day. This revealed that the UV fistula was located approximately 1 cm from the urethral opening. In addition, the proximal urethra was unobstructed and connected to the bladder. The cystoscope allowed for the passage of a urinary catheter through the urethra. After 1 month of catheter placement, she was discharged from the hospital at 57 days of age. Follow-up was uneventful, with neither urinary infection nor retention. CONCLUSIONS: SCTs are associated with not only trouble with rectal function and lower extremity movement but also urinary complications. The pathogenesis of this UV fistula is thought to be the rapid growth of the SCT that developed in the fetal period, resulting in obstruction of the urethra by the tumor and the pubic bone, which in turn caused urinary retention and the formation of a fistula as an escape route for the pressure. Because SCTs can cause a variety of complications depending on the course of the disease, careful examination and follow-up are necessary.

Riñón pélvico fusionado con uréter único, hidrometrocolpos y ano imperforado: conjunción de malformaciones infrecuentes en una adolescente / Pancake kidney with a single ureter, hydrometrocolpos, and imperforate anus: a set of uncommon malformations in a female adolescent

Una niña de 11 años de edad con antecedentes de ano imperforado, infección urinaria y episodios de constipación intermitentes se presentó a la consulta con cólicos abdominales y náuseas de una semana de evolución. Estudios radiológicos revelaron hidrometrocolpos y fusión renal pélvica con uréter único hidronefrótico. El examen vaginal evidenció un tabique transverso no permeable. Se evacuó temporalmente la colección con resolución de los síntomas. La paciente fue programada para cirugía vaginal reconstructiva definitiva. Se destaca en este caso no solo la asociación de malformaciones infrecuentes, sino una sintomatología muy común en la práctica pediátrica a causa de una patología rara vez considerada en el diagnóstico diferencial, y la importancia de una evaluación precoz y completa de este tipo de malformaciones para un tratamiento oportuno.

An 11-year-old girl with a history of imperforate anus, urinary tract infection, and intermittent episodes of constipation presented with abdominal pain and nausea for 1 week. The x-rays revealed hydrometrocolpos and fused pelvic kidney with a single hydronephrotic ureter. The vaginal examination revealed a non-permeable transverse vaginal septum. The collection was temporarily drained and symptoms resolved. The patient was scheduled for definitive vaginal reconstructive surgery. In this case, it is worth noting the association of infrequent malformations and also the signs and symptoms very common in pediatric practice due to a pathology rarely considered in the differential diagnosis, and the importance of an early and complete assessment of this type of malformations for a timely treatment.

Vesicovaginal reflux presenting as transient urocolpos-A diagnostic dilemma.

Urocolpos refers to urinary distension of the vagina; it commonly results from vesicovaginal fistula or reflux. In this case report, we present the clinical and radiological aspects of an 18-year-old female presenting with no significant urinary complaints, but with imaging findings of hydrocolpos. This would disappear after voiding. Vesicovaginal reflux resulting in urocolpos is a rarely diagnosed condition, and the radiologist may be mystified by the intermittent nature of the findings. We emphasize the importance of recognizing the entity before proposing surgical treatment.

Pancake kidney with a single ureter, hydrometrocolpos, and imperforate anus: a set of uncommon malformations in a female adolescent. / Riñón pélvico fusionado con uréter único, hidrometrocolpos y ano imperforado: conjunción de malformaciones infrecuentes en una adolescente.

An 11-year-old girl with a history of imperforate anus, urinary tract infection, and intermittent episodes of constipation presented with abdominal pain and nausea for 1 week. The x-rays revealed hydrometrocolpos and fused pelvic kidney with a single hydronephrotic ureter. The vaginal examination revealed a nonpermeable transverse vaginal septum. The collection was temporarily drained and symptoms resolved. The patient was scheduled for definitive vaginal reconstructive surgery. In this case, it is worth noting the association of infrequent malformations and also the signs and symptoms very common in pediatric practice due to a pathology rarely considered in the differential diagnosis, and the importance of an early and complete assessment of this type of malformations for a timely treatment.

Una niña de 11 años de edad con antecedentes de ano imperforado, infección urinaria y episodios de constipación intermitentes se presentó a la consulta con cólicos abdominales y náuseas de una semana de evolución. Estudios radiológicos revelaron hidrometrocolpos y fusión renal pélvica con uréter único hidronefrótico. El examen vaginal evidenció un tabique transverso no permeable. Se evacuó temporalmente la colección con resolución de los síntomas. La paciente fue programada para cirugía vaginal reconstructiva definitiva. Se destaca en este caso no solo la asociación de malformaciones infrecuentes, sino una sintomatología muy común en la práctica pediátrica a causa de una patología rara vez considerada en el diagnóstico diferencial, y la importancia de una evaluación precoz y completa de este tipo de malformaciones para un tratamiento oportuno.

Challenges in prenatal diagnosis of foetal anorectal malformation and hydrocolpos - Case report.

Introduction: and importance: Foetal hydrocolpos and anorectal malformation are difficult to diagnose prenatally due to abundance of differential diagnoses. This case report presents the challenges of diagnosing such disorders. Case presentation: A G3P2A0 woman came at 32 weeks of pregnancy with a referral for foetal ovarian cyst. Ultrasound revealed a singleton breech pregnancy, estimated foetal weight 3528 g. A septate abdominal cyst measuring 11.31 × 7.17 cm and polyhydramnios were present. Elective caesarean section delivered a female baby weighing 2820 g and measuring 43 cm. Neonatal examination revealed a right lateral suprapubic mass and a rectovestibular fistula. A sinoscopy revealed a suspected hydrocolpos. An abdominal hydrocolpos drainage was performed; a patent urachus and normal bilateral adnexa were present. Clinical discussion: Hydrocolpos is a rare congenital disorder due to distal obstruction of various etiologies. It may be mistaken with other pathologies, including fetal ovarian cysts. A genitourinary congenital abnormality may occur in conjunction with other abnormalities, including gastrointestinal tract anomalies. The presence of imperforate anus and/or fistula should alert the clinician of a possible association with VACTERL syndrome. Conclusion: Hydrocolpos is a rare congenital genitourinary disorder with various differential diagnoses. Simultaneous presence of other abnormalities is likely, with possible association to other syndromes.

Fetal and Newborn Management of Cloacal Malformations.

Cloaca is a rare, complex malformation encompassing the genitourinary and anorectal tract of the female in which these tracts fail to separate in utero, resulting in a single perineal orifice. Prenatal sonography detects a few cases with findings such as renal and urinary tract malformations, intraluminal calcifications, dilated bowel, ambiguous genitalia, a cystic pelvic mass, or identification of other associated anomalies prompting further imaging. Multi-disciplinary collaboration between neonatology, pediatric surgery, urology, and gynecology is paramount to achieving safe outcomes. Perinatal evaluation and management may include treatment of cardiopulmonary and renal anomalies, administration of prophylactic antibiotics, ensuring egress of urine and evaluation of hydronephrosis, drainage of a hydrocolpos, and creation of a colostomy for stool diversion. Additional imaging of the spinal cord and sacrum are obtained to plan possible neurosurgical intervention as well as prognostication of future bladder and bowel control. Endoscopic evaluation and cloacagram, followed by primary reconstruction, are performed by a multidisciplinary team outside of the neonatal period. Long-term multidisciplinary follow-up is essential given the increased rates of renal disease, neuropathic bladder, tethered cord syndrome, and stooling issues. Patients and families will also require support through the functional and psychosocial changes in puberty, adolescence, and young adulthood.

Pyocolpos: A rare cause of recurrent abdominal pain - Always insist on a thorough physical examination.

Thorough physical examination is a must for proper diagnosis of medical diseases and to elucidate all physical findings. Abdominal pain is a very common presentation to Emergency Department, compromising 5%-10% of all visits, and around a quarter are discharged with no particular diagnosis. Pyocolpos is one of those presentations that requires a thorough physical examination, including that of the external genitalia. A 3-year-old girl presented with recurrent history of abdominal pain and a recent history of fever with acute abdomen picture-like presentation, with difficulty voiding over the past few days. She was previously assessed for recurrent abdominal pain, without any identifiable etiology. Upon inspection for indwelling catheter insertion, absence of the vaginal introitus was noted with a bulging mass and an imperforate hymen. Pyocolpos was diagnosed and drained following hymenotomy. Pyocolpos is a rare complication of hydrocolpos. Enlarged vagina causes extrinsic compression to nearby structures, such as the bladder trigone and ureters, causing hydronephrosis, hydroureter, and other sequel including acute kidney injury and urosepsis. An imperforate hymen must be considered in the differential for chronic and recurrent abdominal pain, and can be easily identified by a thorough physical examination, including that of the external genitalia, which is a must in today's every medical practice.

An approach to giant neonatal hydrocolpos with normally sited anus - Diagnosis and management including a novel one stage operation.

INTRODUCTION: Giant non cloacal neonatal hydrocolpos is a rare entity where early recognition of the underlying cause and drainage of the distended vagina is paramount. Conventionally, immediate management has been by abdominal tube vaginostomy or, in urogenital sinus by vaginal clean intermittent catheterisation (CIC). Though effective in providing temporary relief, the abdominal tube vaginostomy is replete with complications, making it imperative to search for alternatives. Here we describe our method of emergent and definitive management of these children, where definitive repair, depending on the aetiology, has been made possible in a single stage. MATERIAL AND METHODS: Seven girls who presented primarily or with history of giant non cloacal neonatal hydrocolpos at two tertiary care centres from January 2013 to March 2021 were studied retrospectively. None had imperforate anus or a bulging hymenal membrane. Four had high vaginal atresia (VA) and three had Urogenital sinus (UGS), one with high confluence. Four girls (2 VA, 2 UGS) presented to us as neonates and were managed by emergency drainage procedures other than tube vaginostomy: CIC in one (low UGS) and a novel abdominoperineal U flap vaginoplasty in three (2 VA, 1 high confluence UGS) which was also the definitive procedure. This procedure involved fashioning an inferiorly based inverted U flap from the posterior wall of distended vagina which is tubularised and flipped down by an abdomino-perineal procedure. The girl with a low UGS later underwent a perineal procedure. Three presented elsewhere as neonates (2 VA, 1 UGS) and had abdominal tube vaginostomy as the emergency drainage procedure. This was complicated by tube dislodgement and pyocolpos till successful reconstruction was performed by us. Successful reconstructions included the abdominoperineal U flap vaginoplasty in one (VA) and a perineal procedure in another (low UGS). One girl with high VA had an inflamed vagina after tube vaginostomy, precluding the U flap vaginoplasty, and underwent abdominal vaginal mobilisation and pull through complicated by partial vaginal necrosis with septic complications. RESULTS: Reconstruction resulted in a patent and a good calibre neo-vagina in all at a mean follow up of 48 months. CONCLUSION: Immediate management of giant non cloacal neonatal hydrocolpos may either be vaginal CIC or a surgical procedure. The abdomino-perineal U flap vaginoplasty is a one stage neonatal procedure that provides effective drainage and forms a definitive neo-vagina. An abdominal tube vaginostomy can be avoided except where neonatal surgical expertise is unavailable.

Gynecological Problems in Newborns and Infants.

Pediatric-adolescent or developmental gynecology has been separated from general gynecology because of the unique issues that affect the development and anatomy of growing girls and young women. It deals with patients from the neonatal period until maturity. There are not many gynecological problems that can be diagnosed in newborns; however, some are typical of the neonatal period. This paper aims to discuss the most frequent gynecological issues in the neonatal period.

Symptomatic Imperforate Hymen in Early Infancy: A Case Report.

Imperforate hymen, though a congenital anomaly, usually presents late in puberty as lower abdominal pain, primary amenorrhea, and cyclical pain. Blood collects in vagina and uterus, proximal to imperforate hymen leading to their distention. Its presentation at infancy is a rare entity. We report such a rare case of symptomatic imperforate hymen in infancy, who presented with acute retention of urine, chills and rigor. Abdominal examination revealed an intra-abdominal mass in the lower abdomen and pelvis with the absence of vaginal opening on perineal examination. Contrast enhanced computed tomography abdomen showed large abdominopelvic cystic lesion posterior to the urinary bladder and anterior to the rectum consistent with a highly distended vagina. She was managed by the incision of the imperforate hymen and drainage of the pus. A high index of suspicion is necessary whenever a female infant presents with abdomino-pelvic mass with symptoms of fever or urinary retention.

Diagnóstico y manejo de Himen Imperforado en una lactante menor / Diagnosis and management of Imperforated Hymen in an infant

INTRODUCCIÓN: El himen imperforado es la malformación congénita más frecuente del tracto genital femenino. La mayoría de los casos no se pesquisan en la infancia, debido a un examen genital insuficiente. Su diagnóstico y resolución deben ser precoces y definitivas para evitar complicaciones posteriores. OBJETIVO: Presentar el caso de una lactante portadora de himen imperforado, y actualizar la información sobre las técnicas de examen genital para detectar esta patología en forma precoz y su tratamiento. CASO CLÍNICO: Lactante de 3 meses, consultó por aumento de volumen protruyente en la zona del introito. En el examen físico se realizó maniobra de valsalva que dio salida a una masa homogénea, nacarada de superficie lisa, entre los labios mayores, sugerente himen imperforado. Se complementó el estudio con ultrasonido ginecológico, el que demostró la presencia de hidrocolpos, descartando otras anomalías. Se realizó una himenotomía, que dio salida a abundante material seroso, sin mal olor, y luego se completó la himenectomía mediante la resección de la membrana himeneal. En control al 4to mes posterior a la intervención se constató un himen ampliamente permeable. CONCLUSIÓN: Los equipos de atención neonatal, requieren capacitación acerca de la técnica correcta del examen genital externo de la recién nacida y lactante. La himenectomía es la técnica que permite resolver definitiva mente el cuadro evitando complicaciones.

INTRODUCTION: Imperforated hymen is the most frequent congenital malformation of the female genital tract. Most cases are not investigated in childhood, due to an insufficient genital examination. Its diagnosis and resolution must be early and definitive to avoid subsequent complications. OBJECTIVE: A clinical case of an infant with imperforated himen is presented. To update on genital examination technique necessary to detect this pathology in the newborn and infants, and the proper treatment. CLINICAL CASE: 3-months-old infant that consulted due to an increase in bulging volume in the introitus area. In the physical examination, the Valsalva's maneuver was performed allowed the ex pulsion of a homogeneous pearly mass with a smooth surface, between the labia majora, suggesting imperforate hymen. The study was complemented with gynecological ultrasound, which demons trated the presence of hydrocolpos, ruling out other anomalies. A hymenotomy was performed, which allowed for the evacuation of abundant serous material, with no bad smell, and then the hymenectomy was completed by resection of the hymenal membrane. In follow-up monitoring 4 months after the intervention, a widely permeable hymen was found. CONCLUSION: Neonatal care teams require training on the correct technique of external genital examination of the newborn and infant. Hymenectomy is the technique that allows definitely resolving the condition, avoiding complications.

Cloacal Malformation in Female Children: Outcome of Initial Management.

OBJECTIVE: To document our experience of initial management of Cloacal malformation in female patients. METHODS: A descriptive retrospective study was conducted in the Department of Pediatric Surgery of the National Institute of Child Health Karachi from January 2010 to September 2018. Female patients with diagnosis of Cloacal malformation were included in this study. Data regarding the age at presentation, mode of presentation, clinical features, presence of hydrocolpos, and associated anomalies were noted. Surgical procedures performed in these patients and the outcomes were also documented. Data was analyzed on SPSS Version 20. RESULTS: Sixty females were included in the study. Age ranged from birth to three years with a median of four days. Patients admitted through emergency were 44 (73.33%) while 16 (26.66%) were admitted through outpatient clinic. Hydrocolpos was found in 15 (25.00%) patients. Five (8.33%) patients had massive abdominal distention and were presented with severe respiratory distress. Among them three had massive hydrocolpos, one patient had pneumoperitoneum secondary to Meckel's perforation and one patient was having massive colonic dilatation. Hydronephrosis and hydroureter were found in 14 (23.33%) patients, while dilated bladder was found in three (5.00%) patients. After optimization of patients, bowel diversion was done as transverse colostomy in 39 (65.00%) patients, high sigmoid colostomy in 17(28.33%) patients while ileostomy was done in three (5.00%) patients. All patients with hydrocolpos had tube vaginostomy. None of the patients required bladder diversion and urinary tract dilatations were gradually subsided in nine patients in the post-operative period. Associated anomalies were found in 22(36.66%) of cases predominantly of sacral origin. Operative complications were found in 18(30.00%) patients, including stoma site in infection in 12(20.00%) patients, vaginal tube dislodgement in two patients, wrong placement of tube in one patient and vaginostomy stenosis in one case. While bowel stoma retraction occurred in four patients, prolapsed was found in three patients. Renal failure occurred in one patient. In total, 37(61.00%) patients had smooth recovery, 18(30.00%) patients had some complications, while Five (8.33%) patients were expired due to sepsis. CONCLUSIONS: Majority of cloacal malformations present in neonatal life. Initial management is an important step in dealing with these patients. Care must be taken during the abdominal exploration regarding drainage of hydrocolpos and appropriate placement of bowel stoma.

[Early correction of hypospadias in girl with a disorder of sex development. clinical case].

The presented clinical case of a girl with a salt-wasting form of congenital adrenal hyperplasia in combination with chronic recurrent infection and lower urinary tract dysfunction demonstrates the need to change conventional two-staged approach to surgical feminization in favor of a one-stage intervention in order to prevent a progression of genitourinary complications. After controlling for the underlying condition, the one-stage feminization was performed, including modified tightening introitoplasty using a Passerini-Glazel flap and a correction of hypertrophic clitoris and labia minora. Good short- and long-term results were achieved.

Vaginal reconstruction for distal vaginal atresia without anorectal malformation: is the approach different?

INTRODUCTION: Distal vaginal atresia is a rare condition and treatment approaches are varied, usually driven by symptoms. METHODS: A retrospective review was performed to identify patients with distal vaginal atresia without anorectal malformation. Data collected included age and symptoms at presentation, type and number of operations, and associated anomalies. RESULTS: Eight patients were identified. Four presented at birth with a hydrocolpos and four presented with hematometrocolpos after 12 years of age. Number of operations per patient ranged from one to seven with an average of three. The vaginal reconstruction was achieved by perineal vaginal mobilization in four patients and abdomino-perineal approach in four patients. One patient, with a proximal vagina approximately 7 cm from the perineum, required partial vaginal replacement with colon. In addition, she had hematometrocolpos with an acute inflammation at the time of reconstruction despite menstrual suppression and drainage which may have contributed to the difficulty in mobilizing the vagina. In five patients, distal vaginal atresia was an isolated anomaly. In the other three cases, associated anomalies included: mild hydronephrosis that improved after hydrocolpos decompression (2), cardiac anomaly (2), and vertebral anomaly (1). CONCLUSION: In this series, a distended upper vagina/uterus was a common presentation and the time of reconstruction was driven by the presence of symptoms. Drainage of the hydrocolpos/hydrometrocolpos with menstrual suppression in post-pubertal patients, followed by further work-up, and planned reconstruction is a good surgical strategy.

Persistent cloaca and caudal duplication in a monovular twin, a rare case report.

INTRODUCTION: A cloaca occurs when genitourinary tract and bowel converge into a common channel. We report a case of partial caudal duplication, persistent cloaca and vestigial appendage in a monovular female twin infant. PRESENTATION OF CASE: This is a monochorinonic-diamniotic twin born at 36 weeks with apgars of 9/9. She had a duplicated labia with two clitorises, and a partially formed accessory foot with 2 toes protruding from the right gluteal region. There was anal atresia and a punctate urethral opening in the right genitalia through which she voided spontaneously. X-ray of the accessory foot had rudimentary metatarsals and phalanges. There was left hydroureteronephrosis and a hydrocolpos causing severe mass effect. On the first day of life, she had exploratory laparotomy with a diverting colostomy and mucus fistula and drainage of hydrocolpos. At 6 months of age, she had removal of the accessory foot with flap closure of the perineal defect and vesicostomy. At 15 months of age she had laparotomy for repair of cloaca, excision of presacral pelvic mass and the duplicated vulva. DISCUSSION: Theories of etiology include failure of regression of Kovalevsky's canal (a communication that connects the amniotic and yolk sac), an incomplete form of twinning through iatrogenic damage to the zona pellucida or a failed triplet formation from a single embryo. CONCLUSION: Caudal duplication with persistent cloaca and vestigial appendage is a rare and complex malformation. Having a unified surgical and medical team to preserve quality of life and to treat complications is of key importance.

The Dilemma of Sex of Rearing: A Case of a 45,X/46,XY Neonate with Hydrocolpos.

BACKGROUND: A rare disorder of sex development is 45,X/46,XY mosaicism, which is phenotypically very heterogenous, ranging from normal male (or female) to that of genital ambiguity of varying degrees. CASE: We report a case of a neonate with 45,X/46,XY mosaicism and hydrocolpos, and we point out the dilemma and the difficulty in gender assignment. SUMMARY AND CONCLUSION: Gender assignment of cases with frank genital ambiguity is often difficult to be determined, because several factors have to be taken into consideration, such as genital appearance, anticipated urological and sexual function, capacity for future fertility, gonadal malignancy risk, and psychosocial factors. A multidisciplinary approach is definitely needed in the management of such cases.

Short-term "double natural orifice catheterization": Nonoperative management of hydrocolpos in persistent cloaca patients - case series.

PURPOSE: Management of hydrocolpos in cloaca patients is of clinical importance. We report a nonoperative method for the management of hydrocolpos in the form of initial catheter decompression, followed by an endoscopy with catheters placement into both the bladder and vagina, and leaving these catheters for 9 and 14days respectively. METHODS: The medical records of six cloaca patients with hydrocolpos in the last 12years were reviewed. The outcomes measured were the renal function, bladder emptying, the presence/resolution of hydronephrosis, and the recurrence of hydrocolpos. RESULTS: Complete drainage of hydrocolpos was achieved in four out of six cases and partial drainage in two. On common channel endoscopy, in four patients the structures were identified and balloon catheters inserted. After catheter removal, the vagina and urinary tract remained adequately drained through the natural cloacal opening with no post-micturition residual urine, resolution of hydroneprosis within 60days, preserved renal function, and no hydrocolpos reaccumulation. CONCLUSION: Initial decompression and short time catheterization can be the definite solution for some cloaca patients with hydrocolpos. Our case-series showed a success rate in two-thirds of patients by achieving the three main goals; permanent hydrocolpos derivation, undisturbed voiding, and preservation of renal function. LEVEL OF EVIDENCE: Study can be classified as a Treatment Study, LEVEL IV Case series with no comparison group.