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.

Hydrometrocolpos etiology and management: past beckons the present.

Hydrometrocolpos is a rare condition in which the uterus and the vagina are grossly distended with a retained fluid other than pus or blood. It may present during the neonatal period or later at puberty. Most cases reported earlier were stillbirths and were diagnosed only on autopsy. Antenatal diagnosis is now possible with the advent of ultrasound. An early diagnosis and speedy management is the key to survival. Many previous case reports have focused on the varied clinical presentations, multiple causes, associated syndromes and/or the radiological diagnosis of this condition. However, management options for different types of hydrometrocolpos have not yet been concisely discussed. We have reviewed the literature and tried to summarize the management options applicable to most case scenarios of hydrometrocolpos.

Uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome) suspected on the presence of hydrocolpos on prenatal sonography.

We report the case of a female neonate with ipsilateral renal agenesis and uterus didelphys with blind hemivagina, also known as Herlyn-Werner-Wunderlich (HWW) syndrome. Prenatal sonography revealed the absence of the left kidney and a retrovesical cystic lesion suspected as hydrometrocolpos. Postnatal evaluation confirmed that the cystic lesion was a hydrocolpos associated with double uterus and blind hemivagina (HWW syndrome). HWW syndrome can be suspected prenatally if a retrovesical cystic lesion is detected in a female fetus with unilateral absence of kidney.

Prenatal diagnosis of hydrometrocolpos in a Down syndrome fetus.

We report the prenatal sonographic diagnosis of hydrometrocolpos caused by an imperforate hymen in a Down syndrome fetus, with spontaneous evacuation on the third day of life. In this case, sonographic evaluation in the 37th week revealed a large retrovesical, sharply marginated, heterogeneous, unilocular cystic structure within the fetal abdomen extending to the left side of the umbilicus.

A case of McKusick-Kaufman Syndrome.

We are reporting a rare case of McKusick- Kaufman Syndrome in a ten-month old female infant from Addis Ababa with difficulty of urination often days duration. Clinical exam revealed supra-pubic mass with tenderness and had left hand postaxial polydactily. Ultrasound and CT scans showed the mass to be hydrometrocolpos posterior to the bladder. Intravenous urography revealed a lower abdomen-pelvic mass displacing and compressing the ureters with bilateral hydronephrosis. Further pelvic exam under anaesthesia revealed hydrometrocolpos with vaginal agenesis. This, to the best knowledge of the authors, is the first ever reported case of the syndrome in Ethiopia.

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.

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.

Microperforate Hymen and Pyocolpos: A Case Report and Review of the Literature.

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.

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.

A rare case of hydrometrocolpos from persistent urogenital sinus in patient affected by adrenogenital syndrome.

Persistent urogenital sinus (PUGS) is a congenital pathological condition characterized by an abnormal communication between the urethra and vagina, which has an estimated incidence of 0.6/10,000 female births. It could be the only known malformation or part of a syndrome. PUGS is commonly shown by a pelvic mass, related to a distended bladder, hydrometrocolpos which is due to an obstruction leading to the dilation of the vagina and uterus (i.e., imperforate hymen, transverse vaginal septum or atresia, and PUGS) or both. We present a case of female patient with classical congenital adrenal hyperplasia, diagnosed on the 7th day of life, with ambiguous genitalia, untreated surgically only with hormone therapy by parental decision. The patient, at the age of 5 years and 5 months, came to our observation for a pelvic ultrasound, which revealed retrovesical neoformation with anechoic content and regular walls. We performed the ultrasound examination that showed the dilation of the cervix and the vaginal canal with anechoic finely corpuscolated content in the declining portion, compatible with hydrometrocolpos from probable persistence of the urogenital sinus. The voiding cystourethrography (VCUG) confirmed the ultrasound diagnosis, with evidence of urogenital sinus. In conclusion, ultrasound is the first diagnostic tool, but need to be completed by other technical procedures, which VCUG or magnetic resonance imaging to observe the site of fusion of the urinary and genital tract.

An unusual case of urinary retention in a competitive gymnast.

Hydrocolpos is the accumulation of blood behind an imperforate hymen. Patients often present in early adolescence with cyclic abdominal pain. We present the case of an elite gymnast who, after 6 months of symptoms and a series of incorrect diagnoses, was diagnosed with hydrocolpos. It is likely that the patient's extraordinary training regimen contributed to the difficulty in diagnosis.

Nonobstructive hydrocolpos due to vesicovaginal reflux: expanding the differential diagnosis.

We report the clinical details and imaging findings for a case of vesicovaginal reflux presenting as gross urocolpos in a 15-year-old female. Findings included a large fluid-filled vagina on full-bladder scan in the absence of any anatomical abnormality, which disappeared completely after micturition. It is important for radiologists to be aware of this entity as it is rarely encountered and leads to very confusing findings, which could result in an erroneous diagnosis.

Tumoración genital interlabial en recién nacida / Genital interlabial mass in newborn girl

El himen imperforado es una rara malformación congénita que ocurre en el 0,1% de los recién nacidos; sin embargo, es la anomalía obstructiva más frecuente del aparato genital femenino. Suele presentarse de forma esporádica y aislada, pero en ocasiones se puede presentar asociado a otras malformaciones, principalmente genitourinarias. Hay dos picos de máxima incidencia: en el periodo neonatal y en la adolescencia. Debido a las múltiples complicaciones que puede presentar, es importante un diagnóstico precoz, preferentemente en la etapa neonatal. El tratamiento es siempre quirúrgico. Presentamos el caso de una recién nacida que en la exploración en las primeras horas de vida presenta una tumoración genital interlabial

The imperforate hymen is a rare congenital malformation that occurs in 0.1% of newborns; however, is the most frequent obstructive anomaly of the female genital tract. It usually occurs sporadically and isolated, but sometimes it can occur associated with other malformations, mainly genitourinary. There are two peaks of maximum incidence: neonatal and in adolescence. Due to the multiple complications that can present, an early diagnosis is important, preferably in the neonatal stage. The treatment is always surgical. We present the case of a newborn girl with a genital interlabial mass detected in the first hours of life

Acquired labial sinechiae and hydrocolpos secondary to Stevens-Johnson syndrome.

Stevens-Johnson syndrome frequently affects the genitalia. Vaginal problems have been recognized in female patients; however, reports about the problem and its treatment are rare. Labial sinechiae have not yet been reported as sequelae of Stevens-Johnson syndrome. Amenorrhea, cyclical abdominal pain, and a hypogastric mass in girls affected by Stevens-Johnson syndrome could indicate acquired vaginal obstruction. Extensive labial sinechiae in such patients can cause dysuria, urinary tract infection, and sexual dysfunction. After a diagnosis of Stevens-Johnson syndrome in girls, it is prudent to schedule a prepubertal genital examination to diagnose genital disease preemptively and avoid obstructed menstruation and future sexual problems.

Congenital vaginal obstruction in neonates and infants: recognition and management.

BACKGROUND: Congenital vaginal obstruction commonly presents after puberty and presentation in infancy is uncommon. Due to lack of awareness, diagnosis may be missed in infants, sometimes with disastrous consequences. METHOD: A retrospective review of 9 neonates and infants treated for congenital vaginal obstruction in 16 years. RESULTS: The patients were aged 2 days to 4 months (median 28 days) at presentation. The main presenting feature was a lower abdominal mass, producing intestinal and urinary obstruction in 7 patients. In 2 patients, other conditions were suspected and diagnosis made only intraoperatively and in one patient, diagnosis was incidental during treatment for another condition. The cause of vaginal obstruction were low transverse vaginal septum 6, imperforate hymen 2, high transverse vaginal septum one. Associated anomalies were present in 5 patients (persistent cloaca 3, Hirschsprung disease 1, polydactyly 1). Low vaginal septum was treated by incision and drainage of hydrometrocolpos, imperforate hymen by hymenotomy and drainage of hydrometrocolpos and high vaginal septum by excision. There was recurrence of hydrometrocolpos in one patient who had low vaginal septum, requiring repeat surgery. One patient, in whom diagnosis was missed, died from overwhelming infection. Short term follow-up was good. CONCLUSION: Congenital vaginal obstruction is an uncommon presentation in this setting. Missing the diagnosis can lead to disastrous consequences. A high index of suspicion is required for prompt recognition and treatment.