Spectrum of Hydrometrocolpos in Pediatric Patients: A Pictorial Essay and Authors’ Experience

ABSTRACT

Context:

Hydrometrocolpos (HMC) is an uncommon congenital condition affecting females with a range of symptoms that can arise due to various structural abnormalities, including an imperforate hymen, vaginal atresia, transverse vaginal septum, urogenital sinus, cloacal malformations, and rarely syndromic disorders.

Aims:

This study aims to deepen the understanding of the diverse manifestations, emphasize the importance of early diagnosis, and explore surgical management strategies to improve outcomes of HMC in pediatric patients.

Settings and Design:

An ambispective study was performed in a tertiary care teaching institute from 2016 to 2024.

Materials and Methods:

All pediatric patients with HMC managed by the authors were evaluated for their symptoms, radiological imaging results, associated anomalies, surgical procedures, and treatment outcomes.

Results:

There were 17 pediatric patients; six showed a vestibular bulge, another eight had an abdominal mass, and three were diagnosed with anorectal malformations. One case was detected before birth, presenting with bilateral hydroureteronephrosis and oligohydramnios. Two patients had complex syndromic associations, including Herlyn–Werner–Wunderlich syndrome and McKusick–Kaufman syndrome. Treatment strategies varied depending on the underlying cause, with simpler cases, including an imperforate hymen and transverse vaginal septum, being managed with incision and drainage, and more complex conditions requiring abdominal vaginostomy. All patients exhibited varying degrees of hydroureteronephrosis caused by lower urinary tract obstruction.

Conclusion:

HMC presents with diverse etiologies, necessitating individualized management approaches. Early diagnosis and comprehensive evaluation are critical in preventing complications and optimizing surgical outcomes. A multidisciplinary approach, integrating pediatric surgery, radiology, and gynecology, is essential for effective diagnosis and management.

INTRODUCTION

Hydrometrocolpos (HMC) is an uncommon congenital disease in females with an incidence of 1:16000 female births.[1] HMC is the dilatation of the female genital tract, which occurs due to blockage in the vaginal outflow, preventing normal drainage, causing fluid-like mucus/pus/blood to build up in the uterus and vagina. There is a coexistence of estrogenic stimulation and vaginal obstruction. This obstruction may result from relatively common structural abnormalities, such as an imperforate hymen (0.05%–0.1%), or more rarely from conditions including vaginal atresia, a transverse vaginal septum, a urogenital sinus, or cloacal malformations. The severity of HMC depends on the extent of the blockage, which could be a complete obstruction or an incomplete/partial obstruction. Some cases are detected early in infancy due to noticeable abdominal swelling, while others may go unnoticed until complications such as urinary retention, kidney issues, or infections develop.[2,3,4,5]

HMC is not merely an isolated structural defect but may also be associated with rare complex syndromic and nonsyndromic conditions. Associations include anorectal malformations, genitourinary anomalies, and the VACTERL association.[6] The complex syndromic conditions include cloacal dysgenesis sequence, McKusick–Kaufman syndrome, Herlyn–Werner–Wunderlich syndrome, Ellis–van Creveld syndrome, and Bardet–Biedl syndrome, among others.[7,8,9,10,11,12]

HMC and postaxial polydactyly during the perinatal period may confuse the differential diagnosis of McKusick–Kaufman syndrome, Bardet–Biedl syndrome, and Ellis–van Creveld syndrome. This may result in dilemmas in the management of the patient. Early detection through prenatal imaging or comprehensive postnatal evaluation with ultrasound and magnetic resonance imaging (MRI) can help mitigate complications. Surgical intervention strategies for HMC depend on the primary etiology and the presence of associated anomalies.[7,8,9,10,11,12] By analyzing our cases, we aim to deepen understanding of the heterogeneous spectrum of HMC, underscore the critical role of early diagnosis, and delineate the diverse etiologies and clinical presentations. We further highlight individualized surgical strategies based on underlying anatomical and syndromic associations while evaluating postoperative outcomes and complications to inform future clinical decision-making and improve patient outcomes.

MATERIALS AND METHODS

An ambispective study was performed between 2016 and 2024 in a tertiary care teaching institute. The details were extracted from medical records retrospectively from 2016 to 2023 and prospectively thereafter. All pediatric patients with HMC managed by the senior author (RG) were evaluated on the basis of their symptoms, radiological imaging results, associated anomalies, surgical procedures, and treatment outcomes. This work is performed, according to the Code of Ethics of the World Medical Association for experiments involving humans (Declaration of Helsinki); the ethical clearance of the study (institutional ethical clearance: IEC NO. 230/MC/EC/2025, dated: 04/03/2025).

Exclusion criteria

Patients aged ≥18 years were excluded from the study.

Admission records of patients diagnosed with HMC were meticulously evaluated based on their symptoms, imaging results, underlying etiologies, associated anomalies, surgical procedures, and treatment outcomes.

The patients were classified into five types based on the type and extent of obstruction [Figure 1].[5] Type I is low (hymenal) vaginal obstruction (imperforate hymen), Type II is mid-plane transverse vaginal membrane or septum, Type IIIa is high obstruction with distal vaginal atresia, Type IIIb is high obstruction with distal vaginal atresia and gluteal swelling, and Type IV and Type V are vaginal atresia with persistence of the urogenital sinus and vaginal atresia with cloacal anomaly, respectively [Figure 1].

Figure 1.

Types of hydrometrocolpos according to level of obstruction as per the classification of Gupta and Sharma.[5] B: Urinary bladder, U: Uterus, V: Vagina, R: Rectum. Type I : Imperforate hymen, Type II: Transverse vaginal septum, Type IIIa: Distal vaginal atresia, Type IIIb: Distal vaginal atresia and gluteal swelling, Type IV: Vaginal atresia with persistence of the urogenital sinus and Type V: Vaginal atresia with cloacal anomaly

RESULTS

A total of 17 patients with HMC presented at varying ages [Table 1]. Of the 17 patients included, 12 were part of a retrospective review, while 5 were prospectively enrolled. The clinical presentation varied based on the underlying etiology, with Type I – 6, Type II – 2, Type IIIa – 4, Type IV – 2, and Type V – 3. Six patients exhibited a bulge in the vestibule, which prompted further evaluation [Figure 2]. Furthermore, another eight patients presented with an abdominal lump (pelvic mass), leading to imaging studies that confirmed HMC [Figure 3]. Three patients were initially diagnosed with anorectal malformations, and HMC was identified during their evaluation.

Table 1.

Details of hydrometrocolpos in pediatric patients in our study

Diagnosis	Type of HMC	Clinical presentation	Frequency with association	Surgical Management	Postoperative complications	Follow-up and definitive procedure
Imperforate hymen	Type I	Bulge in the vaginal vestibule	6 (one antenatally diagnosed)	Incision and drainage with hymenectomy/hymenoplasty (definitive procedure)	Sepsis (1) managed with neonatal intensive care and antibiotics	1–9 years
Transverse vaginal septum	Type II	Lump abdomen (suprapubic)	2	Incision and drainage with excision of septum (definitive procedure)	None	5–9 years
Vaginal atresia	Type III	Abdominal lump, lower urinary tract symptoms Neonatal intestinal obstruction (ileal atresia) with abdominal lump	2	Abdominal vaginostomy (1) End-to-back ileo-ileal anastomosis and abdominal vaginostomy (1)	Recurrent UTI (1)	3–5 years Vaginal pull-through (1)
Herlyn–Werner–Wunderlich syndrome	Type III	Abdominal lump, retention of urine with severe UTI	1	Abdominal Tube vaginostomy (excision of septum)	None	2 years
McKusic–Kaufman syndrome	Type III	Abdominal lump, postaxial polydactyly	1	Abdominal vaginostomy (1)	None	3 years
Urogenital sinus	Type IV	Abdominal lump	2	Abdominal vaginostomy (1) feeding tube decompression with vesicostomy (1)	Recurrent UTI (1)	5 years Lost to follow-up (1)
Persistent cloaca	Type V	Single perineal opening with abdominal lump	3 (one with EA with TEF)	Transverse colostomy with abdominal vaginostomy (2), feeding tube decompression (1)	Recurrent UTI (2)	3–5 years Posterior sagittal anorectal vaginourethroplasty (2)

HMC: Hydrometrocolpos, UTI: Urinary tract infection, EA with TEF: Esophageal atresia with tracheoesophageal fistula

Figure 2.

Clinical photographs of (a) a 1-week-old neonate showing bulging in the vestibule (red arrow), (b) aspiration, (c) intraoperative incision and drainage, and (d) hymenoplasty. Clinical photographs of (e) a 2-day-old neonate showing bulging in the vestibule (red arrow), (f) drainage, and hymenectomy. Clinical photographs of (g) a 3-day-old neonate showing bulging in the vestibule (red arrow), (h) hymenoplasty and drainage

Figure 3.

Clinical photographs of (a-c) a 5-month-old child showing a lump in the abdomen arising from the pelvis and reaching above the umbilicus (red arrow) with bulging in the vestibule (green arrow) managed by incision and drainage and with excision of the transverse vaginal septum. Computed tomography images with coronal (d and e), sagittal (f), and axial sections (g) depicting fluid-containing dilated, thick-walled, fluid-filled vagina (white arrow) and uterus with bladder compression and left-sided hydronephrosis due to ureteric compression

All the patients with imperforate hymen (Type I) presented to us between the ages of 1 day and 4 weeks of birth, with a complaint of bulging in the vestibule [Figure 2]. Ultrasound shows a collection in the vagina up to the uterus in some cases. All the patients were managed by incision and drainage and hymenectomy/hymenoplasty. Among the cases, one patient had a prenatal diagnosis of HMC, which was also associated with bilateral hydroureteronephrosis and oligohydramnios. Further evaluation revealed an imperforate hymen (Type I) as the underlying cause.

One patient presented at the age of 5 months with an abdominal lump. Another female at 14 years presented with primary amenorrhea, cyclical lower abdominal pain, and an abdominal lump. Ultrasound and computed tomography (CT) were suggestive of a thick-walled cystic mass reaching the left hypochondrium with urinary bladder compression in both patients. On evaluation, these patients were diagnosed with transverse vaginal septum (Type II) and managed with incision and drainage through the perineal approach, along with excision of the septum [Figures 3 and 4].

Figure 4.

Clinical photographs of (a and b) a 14-year-old girl showing aspiration of hematometrocolpos and opening (red arrow) after division and excision of the transverse vaginal septum

One patient presented at 11 months of age with an abdominal lump and retention of urine with severe urinary tract infection, oliguria, and deranged renal function [Figure 5]. On imaging (abdominal abdominal ultrasound followed by contrast-enhanced CT [CECT]) the findings were suggestive of a dilated, fluid filled vagina with retroflexed uterus; the right kidney was not visualised, moderate dilatation of left pelvicalyceal system and compressed urinary bladder was present [Figure 5]. Further evaluation with MRI revealed a bifid uterus and a vaginal septum (Type II), leading to the diagnosis of Herlyn–Werner–Wunderlich syndrome (OHVIRA syndrome) [Figure 5]. The child was preoptimized for surgery. A Pfannenstiel incision was chosen; the dilated hemivagina was opened and decompressed. The dilated uterus was also decompressed internally. A tube vaginostomy was performed as the initial procedure [Figure 5]. The patient is planned for colovaginoplasty (definitive procedure). The hydroureteronephrosis had resolved in follow-up.

Figure 5.

Clinical photograph of (a) an 11-month-old girl showing a lump in the abdomen arising from the pelvis and reaching the umbilicus (red arrow) with absence of vaginal opening (atresia) (black arrow). Ultrasound images of this patient with OHVIRA syndrome showing (b) a dilated, thick-walled, fluid-filled vagina (green arrow) causing compression on the bladder (red arrow) and (c) moderate dilation of the pelvicalyceal system (blue arrow). Magnetic resonance images (d) of the sagittal sections showing a dilated, thick-walled, fluid-filled vagina (white arrow) with a dilated retroflexed uterus. Computed tomography images (e) of the coronal sections showing a dilated and tortuous ureter (green arrow) and urinary bladder (blue arrow) with an absent right kidney suggestive of OHVIRA syndrome. Intraoperative photographs (f and g) showing dilated hemivagina (white arrow) being aspirated (pus) and drainage through vaginotomy; dilated retroflexed uterus (blue arrow) is also seen decompressed internally. The inset image (h) showing postoperative photographs with tube vaginostomy at 1-year follow-up. (i) Diagrammatic representation of OHVIRA syndrome

A 3-year-old girl presented with a lump in the abdomen and features of urinary tract obstruction. The CT scan suggested HMC. Abdominal vaginostomy was performed for vaginal atresia (Type III) [Figure 6].

Figure 6.

Intraoperative photographs of a 3-year-old girl patient with vaginal atresia showing (a and b) a dilated, thick-walled vagina (white arrow) with drainage through vaginotomy (black arrow). Computed tomography images with coronal sections showing (c and d) a dilated, thick-walled, fluid-filled vagina (red arrow); sagittal sections showing (e and f) a dilated, thick-walled, fluid-filled vagina (red arrow) reaching the upper abdomen and compression of adjacent organs

A neonate presented at the 2^nd day of life with an abdominal lump and had postaxial polydactyly. Further evaluation identified cardiac defects, moderate hydroureteronephrosis, and vaginal atresia (Type III), confirming McKusick–Kaufman syndrome [Figure 7]. This case was treated with an abdominal vaginostomy.

Figure 7.

Clinical photographs of (a-d) a neonate with McKusick–Kaufman syndrome showing an abdominal lump arising from the pelvis and reaching above the umbilicus (red arrow) with absence of vaginal opening (atresia), (b) suprapubic aspiration of pyocolpos (black arrow), treated with an abdominal vaginostomy (white arrow) and (d) postaxial polydactyly (blue arrow). Clinical photographs of (e and f) a 2-week-old neonate with urogenital sinus presenting with an abdominal lump (red arrow) and managed with an abdominal vaginostomy (white arrow)

A neonate presented with features of neonatal intestinal obstruction, along with a palpable abdominal lump, mandating surgical intervention. Exploration showed classical ileal atresia (Type 3a) with vaginal atresia (Type III). An end-to-end ileo-ileal anastomosis was performed for ileal atresia; an abdominal tube vaginostomy was completed for HMC [Figure 8]. Vaginal pull-through (definitive procedure) was performed at the age of 8 years [Figure 8].

Figure 8.

Clinical photographs of (a and b) a patient with vaginal atresia (white arrow), (c) a picture of tube vaginostomy (black arrow). (d) A photograph of a vaginal pull-through operation which was performed at the age of 8 years; vaginal opening (blue arrow) is appreciated. Clinical photograph of (e) a single perineal opening (white arrow) diagnostic of cloaca, (e) a single perineal opening (cloaca); (f) intraoperative picture showing dilated vagina and uterus (green arrow) and transverse colostomy being performed. (g) Intraoperative photograph showing dilated vagina (back arrow) and uterus (blue arrow) being decompressed through vaginostomy

Two patients presented at 8 days and 2 weeks of life with an abdominal lump. On evaluation, diagnosed as urogenital sinus (Type IV), and the ultrasound was suggestive of a fluid-filled cystic lesion posterior to the bladder wall, compressing the bladder with hydroureteronephrosis (HUN) [Figure 8]. Abdominal vaginostomy was done in one patient; the other patient required feeding tube decompression with vesicostomy for retention of urine owing to failure of catheterization and bladder decompression.

Cloacal anomaly with associated malformations: three patients diagnosed with a cloacal anomaly (Type V) presented to us at 1 day of life; ultrasound revealed HMC. One of the patients had an associated esophageal atresia with tracheoesophageal fistula; thoracotomy with primary repair, left transverse colostomy, and vaginostomy with catheterization was performed as the initial procedure [Figure 8].

Patients presenting with an abdominal lump (pelvic mass) due to HMC underwent abdominal vaginostomy as the initial surgical intervention. Patients with associated anomalies were managed according to the abnormality [Table 1]. In all of the cases, hydroureteronephrosis due to lower urinary tract obstruction was noted, emphasizing the need for early detection and prompt management to prevent renal complications. Postoperative antibiotic chemoprophylaxis was contemplated for the prevention of ascending infection in patients with hydroureteronephrosis and the presence of vesicouretric reflux (VUR), if any. Appropriate parental counseling was done regarding the disease profile and definitive treatment. Associated malformations were managed in consultation with respective specialties. All the patients were in regular follow-up for 6 months to 5 years, except one patient with urogenital sinus (lost to follow-up). There was resolution of hydroureteronephrosis postsurgery in 12 out of 17 patients on follow-up. Among the remaining five, one is lost to follow-up, and the rest four still have mild hydroureteronephrosis, but all of them are currently asymptomatic. Among the patients with vaginostomy as the initial procedure, vaginal pull-through was performed in one; posterior sagittal anorectal vaginourethroplasty was done in two patients with cloaca, and in the remaining, we are planning to do a vaginal replacement procedure [Table 1]. There was a lack of long-term follow-up (limitation).

DISCUSSION

HMC originates from Greek words: hydro, which means water, metro, which means uterus, and colpos, which means vagina. The distension of the uterus and the vagina with a fluid, usually in the presence of a distal obstruction; the main brunt is borne by the colpos. HMC is either secretory or urinary, as per the contents.[2,3,4,5] The fluid accumulation in HMC is usually mucus due to complete vaginal mechanical obstruction and, less often, urine due to incomplete distal obstruction. In the urinary HMC (common cloaca or urogenital sinus), urine accumulates in the vagina as a result of backwash during micturition in the absence of any distal vaginal mechanical blockage.[3,4,5] Meconium HMC may be seen in neonates with a common cloaca with rectum decompressing in the vagina [Figure 8]. Secondary infection of the retained fluid with coliform bacteria is not uncommon.[1]

The embryological basis of HMC depends upon its type. An imperforate hymen (Type I HMC) results due to failure of degeneration of the epithelial cord in the Müllerian tubercle. A transverse vaginal septum (Type II HMC) resulted due to incomplete coalescence of vacuoles that develop as the epithelial cord begins to degenerate. Vaginal atresia (Type III HMC) is the outcome of persistence of a portion of the solid cord of cells in the fused Müllerian ducts above the hymen.[13,14,15,16] In Type IV or vaginal atresia with persistence of the urogenital sinus, there is abnormal accumulation of either mucus or urine in the uterus and vagina due to a distal vaginal obstruction. There is a failure of the normal separation of the urogenital sinus and Müllerian ducts during the 6^th to 10^th weeks of gestation, causing the vagina and urinary tract to empty into a common channel. Persistent cloaca results due to the failure of differential growth and division of the common channel (cloacal chamber) in the developing embryo. The type V HMC develops due to vaginal outflow obstruction within the common channel having a single perineal opening. There is fluid, mucus, or urine accumulation in the uterus and vagina.[17] Failure of Müllerian duct fusion results in duplication anomalies and consequent HMC in some of the patients.

A review of literature suggested different types of vaginal malformations; a few important ones are enumerated. Monie and Sigurdson reviewed all of the previous classifications and proposed a simple classification based on the anatomy.[18] It was followed by Buttram’s (1979, 1982) classification,[19,20] American fertility society classification (1988),[21] Tolete-Velcek et al. classification,[22] Oppelt et al., or vagina, cervix, uterus, adnex-associated malformations classification.[17] These classifications were based on the anatomical forms.[17,18,19,20,21,22] Ruggeri et al. proposed a classification of vaginal malformations based on embryological, anatomical, and clinical criteria and their surgical management.[23] Gupta and Sharma have simplified the classification of HMC into five types based on the level of obstruction and the severity of malformation.[5] We have also systematized our cases of HMC based on this classification. Types I, II, and III are simple obstructions, typically isolated, and are usually secretory. Types IV and V are complex types associated with urinary or fecal (cloaca) contamination.[5]

HMC occurs at the extremes of childhood, i.e. neonatal period, when there is increased production and consequent accumulation of cervical and vaginal secretions under the influence of maternal estrogen stimulation. Antenatal ultrasound helps in the early diagnosis of HMC and associated malformations. HMC is a pointer toward several syndromic and nonsyndromic malformations.[5,24,25,26] HMC appears as a hypoechogenic retrovesical pelvic cystic mass in the fetal abdomen, often associated with hydronephrosis. It may be associated with oligohydramnios.

Postnatal evaluation constitutes a thorough clinical examination, including examination of the perineum and vaginal vestibule. HMC usually presents with a lower midline pelvic mass associated with or without a hymenal protuberance. The clinical presentation of HMC varies depending on the extent of compression exerted by the enlarging uterovaginal mass on surrounding structures.[27,28,29] An ultrasound of the abdomen with perineum is sought; abdominal scan shows a retrovesical dilated echogenic cystic structure with or without upper urinary tract anomalies. A transperineal scan could measure the distance of the obstructive vaginal septum from the perineum. The differential diagnosis includes enteric duplication cyst, meconium pseudocyst, choledochal cyst, ovarian cyst, mesenteric cyst, rectal duplication, bladder duplication, urachal cyst, sacrococcygeal teratoma, and cystic neuroblastoma.[2,3,4] MRI has a supplemental diagnostic value because of its excellent anatomical localization and detailing. In addition, it helps in defining the exact site of communication between the vagina and the urinary tract.

Micturating cystourethrogram, dye studies, and endoscopy are performed to evaluate the anatomical details, length of the common channel or urogenital sinus, and fistulous communication. CECT or CT urography helps in urological evaluation and assesses associated congenital anomalies and syndromes.

The most common cause of HMC is Type I/imperforate hymen (60%–70%).[5] It is a relatively common condition in pediatric surgical practice. The presentation is early in the neonatal period with a thin translucent membrane bulging in the vaginal vestibule. The neonate (under the effect of maternal hormones) additionally has enlarged breasts and a swollen vulva. Other, less common causes are Type II and Type III. HMC is accountable for almost 15% of lower abdominal lumps in female infants.[24,25,26] Urinary tract compression is a frequent finding, often resulting in different degrees of hydroureteronephrosis, whereas bowel obstruction due to mass effect is relatively uncommon.[28,29,30,31] In older girls, diagnosis is often delayed as extensive investigations focus on excluding more prevalent causes of urinary and bowel obstruction.[28,29,30] HMC should be contemplated early in the variance diagnosis in female infants having an abdominal lump, with or without urinary or bowel obstruction.[32]

HMC is commonly linked to urinary and intestinal tract malformations, ranging from persistent urogenital sinus to complex cloacal anomalies.[33,34,35] Despite originating from the same embryological structures, these malformations can lead to distinct anatomical defects.[36,37]

Congenital anomalies of the Müllerian tract are estimated to occur in approximately 2%–3% of women.[38] The OHVIRA syndrome is characterized by an obstructed hemivagina and an ipsilateral renal anomaly, which was first described in 1922. It is traditionally known as Herlyn–Werner–Wunderlich syndrome and is more recently referred to by the acronym OHVIRA.[11,12,39,40,41,42] OHVIRA syndrome typically occurs in association with uterine didelphys, though it may occasionally be seen with a septate uterus.[38,39] Ipsilateral renal agenesis is the most commonly reported urologic anomaly in OHVIRA syndrome, although there are other abnormalities such as multicystic dysplastic kidney and renal duplication.[39] The pathogenesis of OHVIRA syndrome is understood to be multifactorial. Early and severe obstruction at the pelvico-ureteric junction can lead to agenesis of the ipsilateral kidney and may concurrently disrupt the development of the Müllerian duct at the same level. Given the close embryological relationship between the urinary and reproductive systems, females with renal anomalies should be assessed for potential structural abnormalities of the reproductive tract later in life, and vice versa.[43]

McKusick–Kaufman syndrome comprises vaginal atresia, HMC (95% of females), postaxial polydactyly (98%), hexadactyly, hydrops fetalis, and congenital heart disease (14%).[7]

Typical constituents of Bardet–Biedl syndrome are genital anomalies (59%–98%), HMC (81% of females), rod-cone dystrophy or retinitis pigmentosa (93%), obesity (72%–92%), postaxial polydactyly (63%–81%), renal abnormalities (53%), learning difficulties (61%), anosmia/hyposmia, congenital heart diseases, brachydactyly/syndactyly, ataxia/poor coordination, and secondary features: developmental delay, speech delay, dental anomalies, and diabetes mellitus.[7,8]

Ellis–van Creveld syndrome comprises chondrodystrophy, polydactyly, and HMC.[44] Langer–Giedion syndrome constitutes multiple exostoses, short stature, unique facial features, a small head, skeletal abnormalities, and learning difficulties.[45]

Early detection through prenatal imaging or postnatal ultrasound can help mitigate complications and guide appropriate surgical intervention. Prenatal suspicion of HMC should prompt ultrasound evaluation to identify associated anomalies and assess pelvic anatomy. However, due to limitations in late-gestation imaging, MRI plays a crucial role when ultrasound (USG) fails to provide sufficient detail.[2,3,4,26,34,35] Characteristic imaging of HMC includes: (a) a midline abdominal mass located posterior to the urinary bladder, (b) a fluid-filled mass with a mid-plane septum, (c) direct continuity of the lump with the distended uterus and (d) a “four-chamber” appearance suggesting a septate vagina with two uterine cavities.[2,3,4,25,34,35]

While HMC can be identified as early as 25 weeks of gestation, most of the cases are diagnosed in the third trimester or even after birth.[2,3,4,25,34,35] Determining the origin of the mass using ultrasound can be challenging, as it needs to be differentiated from the bladder, which is encircled by the two umbilical arteries. In such cases, MRI proves especially valuable, with T1-weighted imaging helping differentiate the uterus and rectum while clarifying the mass’s connection to surrounding structures.[2,4,25,34]

Treatment strategies for HMC depend on the underlying etiologies and the presence of associated anomalies. An algorithm for the management of HMC based on the classification of the underlying obstruction is presented in Figure 9. While simple cases, such as Type 1 and Type II, may be resolved through drainage procedures (definitive procedure), complex anomalies like vaginal atresia or cloacal malformations require an initial safe temporary drainage procedure through the abdominal route by vaginostomy, followed by more intricate reconstructive multistage surgeries. The goal of intervention is not only to relieve obstruction by drainage of the collection but also to preserve reproductive and urological function, minimizing the chances of long-term complications.

Figure 9.

Algorithm for the management of hydrometrocolpos

Surgical procedure for isolated HMC caused by lower genital tract obstruction generally yields excellent outcomes. However, treatment success is less predictable in cases involving persistent urogenital sinus and is even more uncertain in cloacal malformations.[32] Given the complexity of these anomalies, a comprehensive, multidisciplinary care approach involving pediatric surgeons, radiologists, gynecologists, and urologists is essential to optimize surgical outcomes, address long-term challenges, and improve the overall quality of life for affected individuals.[46] Beyond surgical correction, long-term management must address potential complications, including infertility, chronic pelvic pain, and urinary dysfunction. Mental health concerns such as anxiety and depression are also significant, particularly in patients having pain associated with menstruation, urination, and sexual activity.

CONCLUSION

HMC presents with a range of etiological factors, posing challenges in both diagnosis and surgical management. The causes vary from simple conditions like imperforate hymen to complex anomalies such as cloacal malformations, each requiring a tailored treatment approach. A comprehensive evaluation is essential to identify associated anomalies and determine the most appropriate intervention tailored to the anomaly. Early diagnosis prevents urinary tract infection, further renal damage, and obstructive uropathy. A multidisciplinary approach involving pediatric surgeons, radiologists, and gynecologists is often required for accurate diagnosis and optimal management. The important considerations are the type of HMC (I–V), severity of the blockage, and the presence of associated abnormalities, which guide surgical management. In addition, parental counseling is crucial to ensure understanding of treatment options, expected outcomes, and long-term prognosis, allowing for informed decision-making and better patient care.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.