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Japanese Journal of Radiology Aug 2021Hemato/hydrocolpos due to congenital urogenital anomalies are rare conditions discovered in neonatal, infant, and adolescent girls. Diagnosis is often missed or delayed... (Review)
Review
Hemato/hydrocolpos due to congenital urogenital anomalies are rare conditions discovered in neonatal, infant, and adolescent girls. Diagnosis is often missed or delayed owing to its rare incidence and nonspecific symptoms. If early correct diagnosis and treatment cannot be performed, late complications such as tubal adhesion, pelvic endometriosis, and infertility may develop. Congenital urogenital anomalies causing hemato/hydrocolpos are mainly of four types: imperforate hymen, distal vaginal agenesis, transverse vaginal septum, and obstructed hemivagina and ipsilateral renal anomaly, and clinicians should have adequate knowledge about these anomalies. This article aimed to review the diagnosis and treatment of these urogenital anomalies by describing embryology, clinical presentation, imaging findings, surgical management, and postoperative outcomes.
Topics: Female; Humans; Hydrocolpos; Kidney; Kidney Diseases; Urogenital Abnormalities; Vagina
PubMed: 33840015
DOI: 10.1007/s11604-021-01115-7 -
Children (Basel, Switzerland) Jun 2022Cloaca 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... (Review)
Review
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.
PubMed: 35740825
DOI: 10.3390/children9060888 -
Journal of Clinical Medicine Mar 2021Pediatric-adolescent or developmental gynecology has been separated from general gynecology because of the unique issues that affect the development and anatomy of... (Review)
Review
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.
PubMed: 33806632
DOI: 10.3390/jcm10051071 -
Journal of Obstetrics and Gynaecology... Oct 2020
PubMed: 33041562
DOI: 10.1007/s13224-019-01279-3 -
Annals of Medicine and Surgery (2012) Dec 2022and importance: Foetal hydrocolpos and anorectal malformation are difficult to diagnose prenatally due to abundance of differential diagnoses. This case report presents...
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.
PubMed: 36582906
DOI: 10.1016/j.amsu.2022.104949 -
Children (Basel, Switzerland) Feb 2022Hydrometrocolpos (HMC) is a rare condition where fluids or secretions accumulate in the vagina (hydrocolpos) or up to the uterus (hydrometrocolpos). This case series...
Hydrometrocolpos (HMC) is a rare condition where fluids or secretions accumulate in the vagina (hydrocolpos) or up to the uterus (hydrometrocolpos). This case series study reports three infants with different etiologies and presentations of HMC and aims to review literature for proper workup upon initial diagnosis. The first neonate antenatally presented with a huge cystic mass. HMC secondary to imperforate hymen was proved, and hymenotomy was performed at 2 days of age. The second participant presented with persistent urogenital sinus and hematopoietic chimerism, possibly due to transfusion from her twin brother via placenta anastomoses. At 2 months of corrected age, she had difficult defecating, and sonogram revealed HMC with normal appearance of uterus and ovaries. Regular follow-ups and surgical reconstruction will be conducted before puberty. The third patient had cloacal malformation and multiple congenital anomalies at birth. Vesicovaginal fistula-related HMC was detected and managed with surgical drainage in the neonate stage. The girl began menstruation with dysmenorrhea at 12 years. The image studies demonstrated hematometrocolpos secondary to left-side hemivaginal septum, uterine didelphy, and ipsilateral renal agenesis, indicating Herlyn-Werner-Wunderlich syndrome. HMC can be diagnosed easily via sonogram. Careful external genitalia examinations help to identify persistent urogenital sinus or cloacal malformation. Occasionally, the HMC may be part of syndrome manifestations or associated with sex chromosome anomalies. Clinicians may conduct surveillance of renal, cardiac, and skeletal systems as well as chromosome study for early diagnosis and management.
PubMed: 35204939
DOI: 10.3390/children9020219 -
Pakistan Journal of Medical Sciences 2020To document our experience of initial management of Cloacal malformation in female patients.
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.
PubMed: 32063957
DOI: 10.12669/pjms.36.2.1095 -
Surgical Case Reports Oct 2023Sacrococcygeal 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...
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.
PubMed: 37903968
DOI: 10.1186/s40792-023-01772-y -
Journal of Pediatric and Adolescent... Apr 2018Microperforate hymen is a rare congenital anomaly characterized by a small pinpoint opening in the hymen; girls with this anomaly are prone to develop ascending pelvic... (Review)
Review
BACKGROUND
Microperforate hymen is a rare congenital anomaly characterized by a small pinpoint opening in the hymen; girls with this anomaly are prone to develop ascending pelvic infections and recurrent urinary tract infections.
CASE
We report the case of a 3-year-old girl who presented with fevers, abdominal pain, recurrent vaginitis, and dysuria. She was found to have a microperforate hymen and pyocolpos. She was treated with intravenous antibiotics and underwent hymenotomy and drainage of 150 cc of purulent fluid.
SUMMARY AND CONCLUSION
This case highlights the need to perform thorough genital inspection and to consider hymenal anomalies in the differential diagnosis of girls who present with recurrent dysuria, vaginitis, fevers, and abdominal pain. Early intervention might prevent the development of pyocolpos and other sequelae associated with this anomaly.
Topics: Anti-Bacterial Agents; Child, Preschool; Colpotomy; Drainage; Female; Humans; Hydrocolpos; Hymen; Plastic Surgery Procedures; Suppuration; Urinary Tract Infections; Vaginitis
PubMed: 28838865
DOI: 10.1016/j.jpag.2017.08.006 -
Ultrasound (Leeds, England) Feb 2023The obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is characterized by the triad uterus didelphys, obstructed hemivagina, and ipsilateral renal...
INTRODUCTION
The obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is characterized by the triad uterus didelphys, obstructed hemivagina, and ipsilateral renal dysplasia. To make a radiological diagnosis, knowledge of this syndrome is of paramount importance. Early recognition may prevent complications such as unnecessary surgical procedures, endometriosis, and infections, which could adversely affect fertility.
CASE REPORT
A 1-day-old female newborn in whom a right-sided cystic kidney abnormality was seen on antenatal ultrasound was admitted with anuria and intralabial mass. Besides the multicystic dysplastic right kidney, ultrasound revealed a uterus didelphys with right-sided uterus dysplasia, an obstructed right hemivagina, and an ectopic ureteric insertion. The diagnosis of obstructed hemivagina and ipsilateral renal anomaly syndrome with hydrocolpos was made and the hymen was incised. Later, ultrasound helped in diagnosing a pyelonephritis in the afunctional right kidney that was not draining into the bladder (hence no culture could be obtained), requiring intravenous antibiotics and a nephrectomy.
DISCUSSION
Obstructed hemivagina and ipsilateral renal anomaly syndrome is an anomaly of the Müllerian and Wolffian ducts of unknown cause. Patients typically present after menarche with (progressive) abdominal pain, dysmenorrhea, or urogenital malformations. In contrast, prepubertal patients can present with urinary incontinence or an (external) vaginal mass. The diagnosis is confirmed by an ultrasound or magnetic resonance imaging. Follow-up includes repeated ultrasounds and monitoring of kidney function. Treatment consists of drainage of the hydrocolpos/hematocolpos; in some cases, further surgery is indicated.
CONCLUSION
Consider obstructed hemivagina and ipsilateral renal anomaly syndrome in girls with genitourinary abnormalities: early recognition prevents complications later in life.
PubMed: 36794112
DOI: 10.1177/1742271X221102576