Article

Congenital hydrocolpos

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Abstract

1. 1. Congenital hydrocolpos is caused by a combination of vaginal obstruction and fetal cervical gland secretion caused by stimulus of the maternal hormones. 2. 2. The vaginal obstruction may be caused by atresia, imperforate hymen, or possibly by a congenital fold of tissue which causes a valvelike obstruction in a partially stenotic vagina. 3. 3. The most important factor in treatment of this anomaly is the establishment of a correct preoperative diagnosis. 4. 4. When the correct diagnosis is made, most of these patients will be cured by a relatively simple transperineal drainage procedure. 5. 5. Long-term follow-up is essential since the distended, hypertrophic vagina and the associated hydronephrosis tend to develop intercurrent infection during their slow return to normal.

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... Hydrocolpos has been associated with urinary obstruction causing bilateral hydroureter and hydronephrosis. [3][4][5][6][7][8][9] When this is severe, there may be in utero oligo/anuria resulting in oligohydramnios. Some cases may be less severe and may present later in infancy as an abdominal mass with urinary retention and frequency. ...
... 3,6,7,9,24 -26 Nonlethal respiratory difficulties have also been reported and may be attributable to an abdominal mass effect with subsequent elevation of the diaphragm resulting in pulmonary compression. 4,[27][28][29][30][31] Antenatal Diagnosis of Hydrocolpos Shimada et al. 32 reviewed 45 fetuses with antenatal diagnosis of genitourinary abnormalities and oligohydramnios. The average gestational age at detection of the oligohydramnios was 30 weeks, as was our case. ...
... In some cases, the abdomen may be so distended as to cause dystocia at delivery. 4 In cases caused by imperforate hymen, there is generally a bulging seen at the vaginal introitus. On occasion, this may also occur with a low transverse vaginal septum, with these cases being misdiagnosed as an imperforate hymen. ...
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This is a case report of an unusual cause of Potter sequence. Autopsy showed lethal pulmonary hypoplasia in association with a transverse vaginal septum, hydrocolpos, and a secondary obstructive uropathy.
... Fetal hydrocolpos is detected by ultrasonographic examination as a retrovesical cystic mass, and most cases are detected in the third trimester of pregnancy or after birth [7], although they are in some cases diagnosed as early as the second trimester [8]. Hydrocolpos results from obstruction at different levels of the vagina, and the fluid accumulation consists of cervical and vaginal secretions that develop secondary to circulating maternal estrogens [9,10]. Hydrocolpos occurs as a result of transverse vaginal septum, vaginal atresia, imperforate hymen, or cloacal anomaly. ...
Article
Introduction Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly [1-3]. There are three reported cases of OHVIRA diagnosed in the neonatal period [4-6]. In all three cases, renal agenesis and/or retrovesical cyst were detected by prenatal ultrasound and MRI was performed after birth. Here the authors report the first case of OHVIRA diagnosed prenatally by fetal MRI. Case Report A 33-year-old woman, gravida 0, para 0, was referred to the present institution at 28 weeks' gestation because of a fetal pelvic cystic mass. Detailed ultrasonographic examination revealed a female fetus with a retrovesical cyst and right renal agenesis. At 35+4 weeks' gestation, MRI was performed for detailed evaluation of the fetal pelvis. MRI confirmed uterus didelphys, retro-vesical cyst measuring 23×17×33 mm, and right renal agenesis (Figure 1). Left renal length was 36×20 mm. The cyst was located between the bladder and rectum on T1-weighted image (T1WI), and was connected to the cervix of the uterus didelphys on steady-state free precession (SSFP) image, of which contrast reflected the T2/T1 ratio. Based on these findings, the cyst was thought to originate from the genital tract. In addition, the cyst was low intensity on T1WI and high intensity on SSFP, similar to amniotic fluid, suggesting the cyst contained serous fluid. Therefore the cyst was diagnosed as hydrocolpos: an expanded, fluid filled vagina. No anorectal anomalies were visualised. Thus the prena-tal diagnosis by fetal ultrasonographic examination and MRI was OHVIRA syndrome. The female infant weighing 2,645 grams with Apgar scores of 8 and 9 after one and five minutes, respectively, and umbilical ar-Summary The authors report the first case of prenatal fetal MRI diagnosis of unilateral hydrocolpos, uterus didelphys, and ipsilateral renal age-nesis in a case of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. A 33-year-old woman was referred to the present institution because of a fetal pelvic cyst. Detailed ultrasound revealed a female fetus with a retrovesical cyst and right renal age-nesis. Fetal MRI confirmed unilateral hydrocolpos, uterus didelphys, and normal intestinal tract of the fetus. Fetal MRI is a useful modality for prenatal diagnosis of OHVIRA as a method of evaluating the pelvic structures and excluding other cloacal anomalies.
... Congenital hydrocolpos is thought to be caused by a combination of vaginal obstruction and fetal cervical gland secretion caused by stimulus of maternal hormones [2]. Vaginal obstruction is caused by either imperforate hymen, occluding membrane at lower third of vagina, transverse vaginal septum or vaginal atresia. ...
... The accumulation of secretions causes distension of the obstructed uterus in puberty or in infancy due to the effect of maternal hormones. The resulting mass increases abdominal pressure and can cause respiratory compromise, edema from venous compression or hydronephrosis from ureteral compression (4). Emergency surgery may be necessary, and the complications can be life threatening. ...
Article
McKusick-Kaufman syndrome is a human developmental anomaly syndrome comprising mesoaxial or postaxial polydactyly, congenital heart disease and hydrometrocolpos. This syndrome is diagnosed most frequently in the Old Order Amish population and is inherited in an autosomal recessive pattern with reduced penetrance and variable expressivity. Homozygosity mapping and linkage analyses were conducted using two pedigrees derived from a larger pedigree published in 1978. The PedHunter software query system was used on the Amish Genealogy Database to correct the previous pedigree, derive a minimal pedigree connecting those affected sibships that are in the database and determine the most recent common ancestors of the affected persons. Whole genome short tandem repeat polymorphism (STRP) screening showed homozygosity in 20p12, between D20S162 and D20S894, an area that includes the Alagille syndrome critical region. The peak two-point LOD score was 3.33, and the peak three-point LOD score was 5.21. The physical map of this region has been defined, and additional polymorphic markers have been isolated. The region includes several genes and expressed sequence tags (ESTs), including the jagged1 gene that recently has been shown to be haploinsufficient in the Alagille syndrome. Sequencing of jagged1 in two unrelated individuals affected with McKusick-Kaufman syndrome has not revealed any disease-causing mutations.
... Hydrocolpos is vaginal distension with fluid accumulation due to a combination of stimulated secretory glands and vaginal obstruction [1]. The fluid accumulation can also be due to the presence of urogenital sinus with collection of urine. ...
Article
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Introduction. Neonatal hydrocolpos is a rare condition. Hydrocolpos is cystic dilatation of the vagina with fluid accumulation due to a combination of stimulation of secretary glands of the reproductive tract and vaginal obstruction. The differential for a neonatal presentation of lower abdominal mass includes urogenital anomalies, Hirschsprung's, disease or sacrococcygeal teratoma. Prenatal diagnosis and early newborn imaging studies leads to early detection and treatment of these cases. Case. We report here two cases of neonatal hydrocolpos with prenatal diagnosis of lower abdominal mass. Postnatally, ultrasound, MRI imaging, and cystoscopy confirmed large cystic mass as hydrocolpos with distal vaginal obstruction. Both patients had enlarged renal system secondary to mass effect. Conclusion. High index of suspicion for hydrocolpos in a newborn presenting with fetal diagnosis of infraumbilical abdominal mass will facilitate timely intervention and prevention of complications.
... The accumulation of secretions causes distension of the obstructed uterus in puberty or in infancy due to the effect of maternal hormones. The resulting mass increases abdominal pressure and can cause respiratory compromise, edema from venous compression or hydronephrosis from ureteral compression (4). Emergency surgery may be necessary, and the complications can be life threatening. ...
Article
Full-text available
McKusick-Kaufman syndrome is a human developmental anomaly syndrome comprising mesoaxial or postaxial polydactyly, congenital heart disease and hydrometrocolpos. This syndrome is diagnosed most frequently in the Old Order Amish population and is inherited in an autosomal recessive pattern with reduced penetrance and variable expressivity. Homozygosity mapping and linkage analyses were conducted using two pedigrees derived from a larger pedigree published in 1978. The PedHunter software query system was used on the Amish Genealogy Database to correct the previous pedigree, derive a minimal pedigree connecting those affected sibships that are in the database and determine the most recent common ancestors of the affected persons. Whole genome short tandem repeat polymorphism (STRP) screening showed homozygosity in 20p12, between D20S162 and D20S894 , an area that includes the Alagille syndrome critical region. The peak two-point LOD score was 3.33, and the peak three-point LOD score was 5.21. The physical map of this region has been defined, and additional polymorphic markers have been isolated. The region includes several genes and expressed sequence tags (ESTs), including the jagged1 gene that recently has been shown to be haploinsufficient in the Alagille syndrome. Sequencing of jagged1 in two unrelated individuals affected with McKusick-Kaufman syndrome has not revealed any disease-causing mutations.
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Congenital imperforate hymen is an external urogenital anomaly that has a small differential diagnosis. With careful antenatal sonographic evaluation of the female perineum, identification of a thin protruding membrane consistent with imperforate hymen can be made. We report a case in which an imperforate hymen was diagnosed in a fetus at 25 weeks' gestation. It appeared as a thin membrane that distended the vagina and spread the labia majora because of retained secretions. An associated renal abnormality was present, representing either a right multicystic dysplastic kidney or cystic dysplasia. Imperforate hymen and hydrocolpos can be diagnosed as early as the second trimester. Because of the additional renal anomaly in this case, it is suggested that the presence of prenatally diagnosed imperforate hymen warrants a careful survey of the remaining fetal anatomy to rule out associated abnormalities. The possibility of a familial occurrence should be considered, and the appropriate history should be obtained.
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Hydrocolpos and the hydrometrocolpos are anomalies resulting from vaginal and uterine retention and accumulation of cervicovaginal secretions caused by congenital malformation. It is a rare pathology affecting the female newborn and infant and much less often young girls. It appears clinically as an abdominal mass associated with absence or abnormality of the vaginal opening. The diagnosis is confirmed by abdominal echography and CT scan. Treatment varies from the simple X-shaped hymenotomy for the isolated imperforate hymen to major surgery for substantial retentions and complex urogenital abnormalities. The authors report two cases of hydrocolpos and hydrometrocolpos discovered in the neonatal period. These were two newborn babies resulting from poorly followed pregnancies at which the clinical examination noted in both cases a large abdominal mass. The physical examination, imagery and surgical exploration demonstrated the existence of enormous hydrocolpos with imperforate hymen in 1 case and hydrometrocolpos with distal vaginal atresia in the second case. The treatment consisted of draining the secretions for both patients, after an X-shaped hymenotomy for the first and after section anastomosis of the vaginal atresia for the second. Both cases progressed favorably. Copyright © 2012 Elsevier Masson SAS. All rights reserved.
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Hydrometrocolpos is the result of an obstruction of the genital tract by an intact hymen, a midplane transverse septum, cloacal anomalies or vaginal atresia. This obstruction allows an accumulation of secretions from the infant cervical mucous glands in the vagina and uterus stimulated by maternal oestrogen (Wilson et al, 1978; Nyberg, 1990). The incidence is reported to be between one in 16 000 births (Westerhout et al, 1964) and one in 30 000 births (Hill & Hirsch, 1985). Although several cases of hydrometrocolpos have been reported in the world literature (Mahoney & Chamberlain, 1940; Spencer & Levy, 1962; Reed & Griscom, 1973), there are very few reports of the ultrasonographic appearances of this condition (Sailer, 1979; Sawhney et al, 1990). We present two cases of hydrometrocolpos which showed classical ultrasound features and review the literature on the subject. A 1-day-old white girl, the product of a normal pregnancy and delivery, presented with a fluctuating mass protruding from the vagina. Clinical examination revealed a palpable abdominal mass and a swollen labia. An ultrasound of the abdomen and pelvis was performed using a 5 MHz probe. A 6 cm × 4 cm cystic left adnexal mass was demonstrated with no posterior acoustic enhancement. The mass contained several internal echoes (Fig. 1). The bladder was not distended and therefore not seen, but both kidneys were normal. The vaginal membrane was incised and 150 ml of milky white fluid was expelled.
Article
Hydrocolpos should be considered systematically when an abdominopelvic cystic mass is diagnosed in a female fetus. Because the prognosis and neonatal management of isolated hydrocolpos with spontaneous resolution differs greatly from that of hydrocolpos associated with a cloacal malformation, it is important to ascertain prenatally whether there are associated anomalies. We report the prenatal characteristics of three fetuses with hydrocolpos; in two cases there was spontaneous resolution and one infant was born with digestive tract atresia. The principal ultrasound findings were an oblong anechoic pelvic mass with or without a sagittal septum, located behind a normal bladder. On magnetic resonance imaging (MRI), the cervical imprint on the vagina confirmed the diagnosis of hydrocolpos and helped to diagnose cloacal malformation by demonstrating the absence of meconium beside the bladder on T1 sequences. Our cases show that MRI is useful for differentiating isolated hydrocolpos from hydrocolpos associated with cloacal malformation.
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