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The role of MRI in young adolescent girls with hematocolpos
Abstract
Imperforate hymen is a relatively rare congenital anomaly which may lead to the development of hematocolpos during puberty. Clinical examination reveals imperforate hymen but abdominal ultrasound and tumor markers findings may be misleading. The presented case shows how MRI may contribute to confirm this diagnosis, define the extent of the collection exclude coexisting abnormalities and avoid an unnecessary surgical intervention.A case of an 11 years old girl is presented who was referred to our clinic with the symptoms of pelvic pain and urine retention. Transabdominal ultrasound findings of pelvic mass and an elevated tumor marker Ca 125 contributed to a referral for exploratory laparotomy with possible diagnosis of ovarian malignancy. After admission in our tertiary center a careful history raised the diagnosis of hematocolpos and the clinical examination showed an imperforate hymen. An MRI of the pelvis was performed to confirm the diagnosis of hematocolpos and exclude other coexisting congenital abnormalities. She was managed by crucial hymenectomy and drainage.The diagnosis of hematocolpos should always be considered in the differential of lower pelvic pain in a young adolescent girl. Detailed family history and clinical examination are mandatory to establish a diagnosis. MRI of the pelvis should be performed to confirm the clinical diagnosis, not only to evaluate the extent of the collection and the presence of possible coexisting anatomic variations of the urogenital tract, but also to avoid an unnecessary surgical intervention by laparoscopy or laparotomy.
... Tumor marker CA-125 has been described in association with imperforated hymen presentation [3]. CA-125 is normally expressed in any tissue derived from coelomic epithelium such that of serosal surfaces, peritoneum, pericardium and pleura, as well as in Mullerian epithelium. ...
... MRI is non-invasive readily available technique than can be used safely in young patients. It can also exclude pelvic, renal, ovarian and uterine causes that carry similar clinical presentation as hematometrocolpos [3]. ...
Here we present a case of the imperforated hymen and transverse vaginal septum with associated hematometrocolpos, blood accumulation in the vaginal and uterine cavities, in a 15-year-old female patient. Virginity sparing technique was conducted given the cultural values of the patient and her family. The rarity of this condition along with special surgical technique in limited health care facility setting signifies this case report. Keywords: obstructive reproductive tract; hymen; vaginal septum. How to cite: Al-Lami RA et al., Imperforated hymen with transverse vaginal septum in a 15-year-old patient.
... The vulvar patency allowing the onset of menstruation in girls of puberty is a sign of healing. Hymenal ring sclerosis has been reported in a study by Castaigne and Haddad on 9 patients operated on for hymenal imperforation [37][38][39]. ...
... The vulvar patency allowing the onset of menstruation in girls of puberty is a sign of healing. Hymenal ring sclerosis has been reported in a study by Castaigne and Haddad on 9 patients operated on for hymenalimperforation[37][38][39]. ...
... In addition, a transabdominal ultrasonography may reveal a cystic mass in the pelvis corresponding to the dilated vagina-uterus [2]. These cases require monitoring with magnetic resonance imaging in order to determine the thickness of the imperforate hymen and exclude concomitant congenital Müllerian duct anomalies [7,8]. Delay in the diagnosis and treatment of imperforate hymen may cause complications including infection, endometriosis and infertility consequently [9]. ...
Introduction: Imperforate hymen is a rare local fusion anomaly of the female reproductive tract and diagnosis can be easily missed. Cyclic lower abdominal pain and amenorrhea are common symptoms in cases left untreated after menarche. Perineal pain may be an unusual presenting symptom of imperforate hymen. In order to avert complications related to delayed treatment, hymenotomy is considered effective management. Case Report: We herein describe a case of a 13-year-old adolescent girl with an imperforate hymen presenting with lower abdominal and perineal pain. The girl underwent and hymenotomy and postoperative period was uneventful. Perineal and pelvic pain relieved instantly after surgical treatment and during follow up her menstrual cycles were regular.
... A rectal ultrasonographic approach is excellent method to aid in diagnosis of imperforate hymen when transvaginal approach is not feasible.It is useful because of close proximity to the pelvic organs [17] . However, MRI of the pelvis should be performed for the confirmation of diagnosis, to know the extent of collection and to rule out possible coexisting anatomical variations of urogenital tract [18] . ...
... Elle serait la meilleure technique d'exploration complémentaire donnant sur les séquences pondérées en T2 une très bonne analyse anatomique morphologique de la malformation. Les séquences pondérées en T1 confirmeront le contenu hématique dans le vagin et dans la corne utérine sus-jacente [4,5]. Le traitement de l'hématocolpos dépend de son étiologie. ...
L'hématocolpos est l'accumulation progressive du sang menstruel dans la cavité vaginale à la puberté. Il est souvent la conséquence d'une imperforation de l'hymen. Il se traduit sur le plan clinique par des douleurs pelviennes cycliques et une aménorrhée primaire. Plus rarement, il peut se révéler par une masse pelvienne. L'échographie est l'examen de choix pour le diagnostic de l'hématocolpos sur imperforation de l'hymen. L'imagerie par résonance magnétique (IRM) est l'examen d'imagerie de référence pour confirmer l'hématolcolpos et exclure d'autres malformations du canal de Muller ou des malformations urologiques associées. Nous rapportons un cas d'hématocolpos secondaire à une imperforation hyménéale diagnostiqué chez une jeune fille présentant une aménorrhée primaire et une masse pelvienne. Le diagnostic était posé par l'échographie et l'IRM et confirmé par l'intervention chirurgicale.
Hematocolpos is a rare condition, where menstrual blood fills the vagina, instead of being expelled, due to a series of uterovaginal pathologies, the most frequent of which is the imperforate hymen. To date, few cases of hematocolpos have been reported in the literature. We report a case of hematometrocolpos due to imperforate hymen initially misdiagnosed as constipation and subsequently as ovarian mass; moreover, the present study undertakes a systematic review of studies on hematometrocolpos due to imperforate hymen to synthesize available knowledge on epidemiology, diagnosis, and management about this rare condition. A total of 35 studies, describing 61 patients, were identified. The presence of hematocolpos should be suspected in premenarchal patients complaining of low abdominal pain, abdominal swelling, and urinary retention. Genital examination disclosing a tender, pale hymen and ultrasound represent a useful tool for diagnosis. The goal of the management is to timely perform hymenotomy to drain the hematocolpos, followed by hymenectomy to prevent recurrence. Follow-up is needed to diagnose possible recurrences. In the case of an adolescent girl complaining of genital pain associated with primary amenorrhea, hematocolpos due to imperforate hymen should be suspected.
Journal of african clinical cases and reviews / Journal africain des cas cliniques et revues www.jaccrafrica.com ISSN 1859-5138 Open access Cas clinique Hématocolpos révélé par une rétention urinaire : à propos d'un cas Hematocolpos revealed by urine retention: about a case Résumé L'hématocolpos correspond à l'accumulation progressive du sang menstruel dans la cavité vaginale à la puberté résultant souvent d'une imperforation de l'hymen. Sur le plan clinique, l'aménorrhée primaire est le signe le plus constant. Il peut se révéler par des douleurs pelviennes cycliques, une masse pelvienne et exceptionnellement par une rétention urinaire. L'échographie est l'examen de choix pour le diagnostic de l'hématocolpos sur imperforation de l'hymen. Nous rapportons un cas d'hématocolpos secondaire à une imperforation hyménéale diagnostiqué chez une jeune fille présentant une aménorrhée primaire et une rétention urinaire. Le diagnostic était posé par l'examen clinique et l'échographie. Il est confirmé par l'intervention chirurgicale. Mots-clés : Hématocolpos, aménorrhée et rétention urinaire. Abstract Hematocolpos is the gradual accumulation of menstrual blood in the vaginal cavity at puberty often resulting from imperforation of the hymen. Clinically, primary amenorrhea is the most consistent sign. It can be revealed by cyclic pelvic pain, a pelvic mass and exceptionally by urinary retention. Ultrasound is the examination of choice for the diagnosis of hematocolpos on imperforate hymen. We report a case of hematocolpos secondary to hymenal imperforation diagnosed in a young girl presenting with primary amenorrhea and urinary retention. The diagnosis was made by physical examination and ultrasound. It is confirmed by surgical intervention.
The girl, 15 years old, was sent to renal ultrasound for abdominal pain, back pain, pollakisuria and proteinuria. Difficulties lasted for four days. ATB treatment was without effect. Sonographic findings in the kidney were completely physiological. During examination of the urinary bladder there were displayed 2 spherical hypoechoic structures with communication between them. After finding out that the girl has amenorrhea, there was suspicion of hematocolpos, which was confirmed during a gynecological examination.
In the present case, ultrasound examination ruled out the presence of hematosalpinx or other gynecological tumors. This was of great importance to the surgical intervention, especially for avoiding laparotomy. Dilatation of the vagina and uterus, due to imperforate hymen with retrograde menstruation should be considered in the differential diagnosis of abdominal pain in premenarchal girls (Fig. 2, Ref. 15). Full Text (Free, PDF) www.bmj.sk.
Imperforate hymen is a urogenital tract anomaly that represents the most frequent congenital malformation of the female genital tract. CA 19-9 and CA 125 are widely used as tumor markers, however several benign conditions are also known to increase serum CA 19-9 and CA 125 levels. According to classical textbook knowledge, imperforate hymen is not listed under the benign conditions that increase serum CA 19-9 and CA 125 levels. In this case report we describe a relation between imperforate hymen and elevated serum CA 19-9 and CA 125 levels.
Acute urinary retention is unusual in children and is usually a candidate for visiting the emergency department upon initial discovery. We report a 12-year-old girl who complained of acute urinary retention. Ultrasonography demonstrated a large echogenic mass over the vagina and mild dilation of the uterus. Imperforate hymen associated with hematocolpos and hematometrium was diagnosed. Cruciate hymenotomy was performed. The symptoms resolved after treatment. Adolescent girls who complain of urinary symptoms with no previous menstruation should have their external genitalia examined in order to rule out the possibility of imperforate hymen as the cause of acute urinary retention.
The most significant corporal change observed in adolescent girls is the onset of menstruation, which occurs between 12 and 13 years of age. In several cases, described with the term 'precocious puberty', pubertal development can begin at a significantly younger age. The term 'delayed puberty' refers to absence of pubertal development in a girl over the age of 14. Amenorrhoea can occur due to a broad spectrum of causes, such as anatomic deficiencies of the reproductive tract and hormonal disorders. Hypogonadotrophic hypogonadism, which implies a permanent malfunction in gonadotrophin secretion; hypergonadotrophic hypogonadism, which involves poor ovarian response in gonadotrophin stimulation; and hyperprolactinaemia can also lead to amenorrhoea. Significant amount of stress on the adolescent girl can cause hypothalamic dysfunction, leading to a situation called 'hypothalamic amenorrhoea'. Abnormal uterine bleeding (AUB), and especially the subtype of dysfunctional uterine bleeding (DUB), is the most urgent gynaecological problem during adolescence, while dysmenorrhoea (also referred to as painful menstruation) is the most frequent problem for which adolescents and their parents refer to a physician.
Pediatric pelvic MRI has had dramatic advances in the past few years. This review documents studies demonstrating the accuracy of MRI for the evaluation of uterine and vaginal anomalies and discusses the salient changes to MRI methods that are particularly applicable to evaluating the pediatric patient with these developmental anomalies.
MRI has high accuracy for evaluation of uterine and vaginal anomalies. Significant advances, such as volumetric imaging, increased resolution, decreased motion artifacts, and shorter examination time, have increased the access and utility of MRI for pediatric patients.
MRI techniques have evolved markedly in the past several years, providing a robust method of evaluating uterine and vaginal anomalies in the pediatric patient.
The fundamental limit for NMR imaging is set by an intrinsic signal-to-noise ratio (SNR) for a particular combination of rf antenna and imaging subjects. The intrinsic SNR is the signal from a small volume of material in the sample competing with electrical noise from thermally generated, random noise currents in the sample. The intrinsic SNR has been measured for a number of antenna-body section combinations at several different values of the static magnetic field and is proportional to B0. We have applied the intrinsic and system SNR to predict image SNR and have found satisfactory agreement with measurements on images. The relationship between SNR and pixel size is quite different in NMR than it is with imaging modalities using ionizing radiation, and indicates that the initial choice of pixel size is crucial in NMR. The analog of "contrast-detail-dose" plots for ionizing radiation imaging modalities is the "contrast-detail-time" plot in NMR, which should prove useful in choosing a suitable pixel array to visualize a particular anatomical detail for a given NMR receiving antenna.
Although imperforate hymen occurs in approximately 0.1% of female newborns, familial occurrence of imperforate hymen has been reported only once.
We report two families in which imperforate hymen was diagnosed in three siblings of each family. One family is described in detail; the patients were two postmenarchal young women and one premenarchal girl.
Imperforate hymen usually occurs sporadically but can be familial. We advise screening all female newborns and children for vaginal patency, especially family members of an affected child. Identification of other families with a similar problem might point to a specific mode of inheritance.
Imperforate hymen is a rare occurrence in younger women resulting in hydrocolpos and hydrometrocolpos. Though imperforate hymen may be diagnosed in infancy, females ages 9-15 years most often present with the problem. Imperforate hymen usually is a congenital anomaly, but has been reported as a result of sexual abuse. This case report reviews the presentation and treatment of a 13-year-old female with imperforate hymen.
High-resolution magnetic resonance (MR) imaging with pelvic phased-array and endoluminal coils provides information on vaginal abnormalities heretofore not available with other imaging modalities. Congenital anomalies of the vagina can result from disorders of lateral fusion of the descending müllerian ducts and ascending urogenital sinus, developmental absence of the müllerian ducts, or disorders of vertical fusion. In these conditions, MR imaging can depict the presence or absence of the vagina, cervix, and uterus and help determine whether these structures contain a septum or are duplicated or distended with blood. Gartner duct cysts and Bartholin gland cysts have the same signal intensity characteristics, but the former are located in the anterolateral aspect of the proximal third of the vagina and the latter are in the posterolateral portion of the lower vagina. Entero-, vesico-, and ureterovaginal fistulas result from obstetric and surgical trauma, radiation therapy, or inflammatory bowel disease. MR imaging has great potential for detection and characterization of these fistulas. MR imaging can be used to detect and stage primary and secondary vaginal tumors. However, neither signal intensity nor patterns of enhancement enable confident prediction of the histologic subtype of vaginal malignancy.
McKusick-Kaufman syndrome (MKKS) is a rare, recessively inherited syndrome reported mainly in young children and is characterised by vaginal atresia with hydrometrocolpos, postaxial polydactyly, and congenital heart defect. Bardet-Biedl syndrome (BBS) is the generic name for a genetically heterogeneous group of autosomal recessive disorders characterised by retinal dystrophy or retinitis pigmentosa (appearing usually between 10 and 20 years of age), postaxial polydactyly, obesity, nephropathy, and mental disturbances, or, occasionally, mental retardation. Typically, MKKS is diagnosed (and reported) in very young children, whereas the diagnosis of BBS often is delayed to the teenage years. We report here a series of nine patients diagnosed in infancy with MKKS because of the presence of vaginal atresia and postaxial polydactyly, who later developed obesity and retinal dystrophy, thus turning out to be instances of BBS. The overlap of BBS and MKKS is a real diagnostic pitfall and its importance has to be stressed, for genetic counselling, for clinical management and follow up, and for molecular approaches. The diagnosis of MKKS should be considered with caution in all published cases described exclusively in the neonatal period and in those with mental retardation. We strongly recommend all children seen in infancy with a diagnosis of MKKS to be re-evaluated for RP and other signs of BBS.
Imperforate hymen is an uncommon anomaly of the reproductive tract, occurring in approximately 0.1% of newborn females. The familial occurrence of imperforate hymen in a child, her mother, and her mother's monozygotic twin is reported.
Case report.
Academic medical center.
Three affected family members.
Karyotype and pedigree analysis.
The proband, presenting with peritonitis, was evaluated at age 12 for imperforate hymen because this condition was diagnosed in her mother at age 14. At age 14, the mother's monozygotic twin was asymptomatic except for primary amenorrhea and was also demonstrated to have imperforate hymen. No other reproductive system abnormalities were known to be present in the remaining family members. Chromosomal structural analysis confirmed that the mother of the proband had no chromosomal abnormalities.
The occurrence of imperforate hymen in two consecutive generations of a family is consistent with a dominant mode of transmission, either sex-linked or autosomal. Previously reported examples of siblings with imperforate hymen suggested a recessive mode of inheritance. Taken together, these cases suggest that imperforate hymen can be caused by mutations in several genes. This case highlights the importance of evaluating all family members of affected patients. Familial examples of other developmental anomalies of the female reproductive tract also suggest a multifactorial genetic etiology.
We report on a female fetus with prenatally suspected hydrometrocolpos. Postnatal evaluation additionally revealed ambiguous genitalia, anorectal atresia, vertebral segmentation anomalies and congenital intestinal aganglionosis. Colostomy was performed, but postoperative recovery was complicated by pulmonary hypertension and renal failure, resulting in death at day 18. Postmortem examination furthermore revealed a small ventricular septal defect, as well as rectovaginal and urethrovaginal fistulae, causing massive dilatation of the septated vagina (hydrocolpos). The possibility of an overlapping VACTERL and MURCS association is discussed.
When investigating pelvic pathologic conditions in female pediatric patients, one needs to be aware of the developmental changes that take place around puberty. The prepubertal uterus is thin, with a fundus equal in size to the cervix. Owing to the hormonal stimulation of puberty, the uterus enlarges and the fundus becomes prominent. The ovaries are demonstrated with ultrasonography (US) at all ages. Ovarian volume increases after 6 years of age. Microcystic follicles are normally seen throughout childhood. US is the modality of choice for imaging the pediatric female pelvis. The main indications for pelvic US in the pediatric age group are pubertal precocity or pubertal delay, pelvic pain or pelvic masses, and ambiguous genitalia. Vaginal bleeding in the prepubertal child can be due to a vaginal foreign body, vaginal rhabdomyosarcoma, or precocious puberty. Common causes of primary amenorrhea in teenagers include gonadal dysgenesis (Turner syndrome) and müllerian (uterovaginal) anomalies. Pelvic pain or pelvic masses in pediatric patients can be due to ovarian torsion, hemorrhagic ovarian cyst, pelvic inflammatory disease, or ectopic pregnancy.
Two cases of haematocolpos in adolescent girls due to imperforate hymen are reported. Both of them presented with lower abdominal pain and urinary retention. Hymenotomy was performed in both the cases. The condition is discussed with brief review of literature.
Imperforate hymen is an uncommon obstructive anomaly of the female reproductive tract, which usually presents with intermittent and cyclical abdominal pain. Although it is usually sporadic, four cases of familial occurrence of imperforate hymen have been reported so far. Imperforate hymen is seldom associated with complications if it is detected early. In view of the possible familial occurrence, it is important to have a high index of suspicion in women who present with imperforate hymen. A detailed menstrual history of other female family members is necessary to enable early detection.
A 14-year-old premenarcheal adolescent girl presented with lower abdominal discomfort and urine retention. After clinical and imaging examination, an imperforate hymen and a large hematocolpos along the upper part of the vagina was diagnosed. Incision of the imperforate hymen did not lead to drainage of blood or fluid. A complete transverse vaginal septum in the middle third of the vagina was identified and when incised drained approximately 200 mL of a dense brownish fluid. Laparoscopy showed a small unicornuate uterus, confirmed by hysteroscopy. This is the first case in the literature of concurrent imperforate hymen, transverse complete vaginal septum, and unicornuate uterus, and it highlights the potential of a multifactorial embryologic genetic etiology.
The detection of vaginal lesions has increased with the expanding use of cross-sectional imaging. Magnetic resonance imaging (MRI) - with its high-contrast resolution and multiplanar capabilities - is often useful for characterizing vaginal masses. Vaginal masses can be classified as congenital, inflammatory, cystic (benign), and neoplastic (benign or malignant) in etiology. Recognition of the typical MR imaging features of such lesions is important because it often determines the treatment approach and may obviate surgery. Finally, vaginal MR imaging can be used to evaluate post-treatment changes related to previous surgery and radiation therapy. In this article, we will review pertinent vaginal anatomy, vaginal and pelvic MRI technique, and the MRI features of a variety of vaginal lesions with pathological correlation.
To assess accuracy of magnetic resonance imaging (MRI) for the delineation of morphological abnormalities of the vagina in patients with congenital anomalies of the genito-urinary tract.
Fifty-one patients (median age 19 years; range 12-40 years) were studied. All were consecutively referred for MRI to assess genital tract anatomy, between 1996 and 2004, from a clinic specializing in congenital abnormalities of the urogenital tract. All patients were assessed clinically and underwent MRI. Images were reviewed retrospectively by an experienced radiologist. Where there was discordance between clinical and radiological findings a consensus diagnosis was achieved by the gynaecologists and radiologists reviewing all of the clinical and radiological evidence together, including assessment of vaginal length.
The clinical data were incomplete for five women and the images non-diagnostic in two cases; consequently, 44 of 51 women had complete datasets and could be evaluated. Vaginas were abnormal in 30 of the 44 patients. There was discordance between the clinical and imaging findings at the initial review in three of the 44 cases (6.8%). After consensus review, and with the inclusion of measurement of the vaginal length on MRI, the MRI and clinical findings were concordant in all cases. The initial discordance was due to two vaginal dimples not being appreciated on MRI and one case in which presence of vaginal tissue proximal to a mid-segment obstruction was not appreciated clinically.
MRI is an accurate method of imaging vaginal anomalies. However, to achieve reliable results the radiologist requires details of previous surgery and the vaginal length must be measured.
This article describes the considerable technical achievements that have been made in MR imaging in the evaluation of pediatric patients. The latest techniques in improving signal intensity, resolution, and speed are discussed. The multitude of new options for pediatric MR imaging are illustrated, including higher field strength imaging, multi-channel coil technology coupled with parallel imaging, and new pulse sequence designs. Several future directions in the field of pediatric body and musculoskeletal imaging also are highlighted.
Hydrometrocolpos and poly-dactyly: a common neonatal presentation of Bardet–Biedl and
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Congenital malformation of the female genital tract: diagnosis and management Obstructive Mullerian anomalies. Philadelphia: Lippincott Williams and Wilkins
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Attaran M, Gidwani FTG. Congenital malformation of the female genital tract: diagnosis and management. In: Falcone G, editor. Obstructive Mullerian anomalies. Philadelphia: Lippincott Williams and Wilkins; 1999. p. 145–68.