UNASSIGNED: Several schoolgirls attain reproductive age with undiagnosed gynaecological problems which pose challenges in their livelihood. These conditions include precocious puberty, congenital reproductive tract abnormalities, and delayed sexual development. Many children with these conditions face additional challenges including physical pain, psychological trauma and delayed diagnosis.
UNASSIGNED: A 14-year-old girl presented with acute on chronic pelvic pain and haematocolpometra due to imperforate hymen during COVID-19 pandemic. She has not undergone cultural virginity test in her community. The hymenal membrane was unusually non-bulging despite the haematocolpometra. A partial hymenotomy with a narrow margin of excision was performed.
UNASSIGNED: The hymenal orifice later obliterated and resulted in a repeat partial hymenectomy where a wide surgical margin of the hymen was excised.
UNASSIGNED: A wide rather than narrow partial hymenectomy prevents obliteration of the hymenal orifice after surgery for imperforate hymen. There is a need for timely interventions such as counselling and community awareness that prevent undue consequences of an imperforate hymen and its treatment including pain and possible inability to pass cultural virginity test in some African communities.

OBJECTIVE: 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.
RESULTS: 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.

OBJECTIVE: Hydrometrocolpos is distension of the vagina and uterus with fluid other than pus or blood. It is due to distal vaginal obstruction, and one of its possible causes is agenesis of the distal vagina. Patients with distal vaginal atresia may present in the neonatal period due to stimulation of uterine and cervical glands by maternal estrogen. In this study, we presented our results in managing neonates with complicated hydrometrocolpos due to distal vaginal atresia through a single-stage abdomino-perineal pull-through vaginoplasty.
METHODS: During the period from July 2003 to June 2018, neonates presented to the Department of Pediatric Surgery, Faculty of Medicine, Ain Shams University with complicated hydrometrocolpos (complicated with obstructive uropathy, intestinal obstruction, urinary tract infection, or systemic sepsis) due to distal vaginal atresia were included in the study. All the patients underwent single-stage abdomino-perineal pull-through vaginoplasty with drainage of the accumulated fluid. The patients were followed up for re-accumulation of fluid or vaginal stenosis for at least 4 years after the procedure.
RESULTS: During the specified period, 14 neonates with complicated hydrometrocolpos due to distal vaginal atresia (complicated with obstructive uropathy, intestinal obstruction, urinary tract infection, or systemic sepsis) presented to the Department of Pediatric Surgery, Faculty of Medicine, Ain Shams University and underwent single-stage abdomino-perineal pull-through vaginoplasty with drainage of the accumulated fluid. Their age ranged between 4 and 18 days (mean 10, median 8). Two patients (14%) developed vaginal stenosis, which responded well to dilatation, and none of the patients had re-accumulation of the fluid during the follow-up period.
CONCLUSIONS: Single-stage abdomino-perineal pull-through vaginoplasty in neonates with complicated distal vaginal atresia (complicated with obstructive uropathy, intestinal obstruction, urinary tract infection, or systemic sepsis) is an appropriate and feasible technique with a satisfactory outcome.
METHODS: Treatment study.
UNASSIGNED: IV.

McKusick-Kaufman syndrome is a rare genetic disorder that affects limb development, genital formation, and heart function. It is caused by mutations in the MKKS gene on chromosome 20. Individuals with this condition may have extra fingers or toes, fused labia or undescended testes, and, less commonly, severe heart defects. Diagnosis involves a physical examination and genetic testing, while treatment focuses on symptom management, including surgical intervention if necessary. The prognosis varies depending on the severity of associated complications. In a recent case, a 27-year-old woman with fetal hydrometrocolpos gave birth to a female neonate with extra digits on both hands and feet, fused labia, and a small vaginal opening. The neonate also had a large abdominal cystic mass, and echocardiography revealed a patent foramen ovale. Genetic testing confirmed an MKKS gene mutation, and the hydrometrocolpos required surgical management. Early diagnosis and intervention can improve outcomes for individuals with this syndrome.

Neonatal hydrometrocolpos (HMC) is a cystic dilatation of a neonate\'s vagina and uterus occurring secondary to congenital vaginal obstruction, with or without maternal estrogenic stimulation of uterine and cervical glands causing increased secretions during the prenatal and postnatal period. Diagnosis is made using ultrasonography and further confirmed by MRI. HMC in a neonate can rarely present with congenital anomalies such as polydactyly, which may indicate a variety of underlying genetic syndromes. There is a deficit in the literature as to whether the development of HMC in a neonate of consanguineous parents is an isolated finding or solely related to an underlying syndrome. We hope to help bridge this gap by reporting a case of a 12-day-old neonate presenting with hydrometrocolpos and polydactyly, born to consanguineous parents.

We present a case of a 12.5-year-old girl who has suffered from recurrent urinary tract infections for many years but has never undergone a detailed diagnostic process. Only as a teenager did she complain of acute pain in her lower abdomen and it turned out that her genital organs had not properly developed. She had an obstructive defect in the reproductive tract. When there was a significant amount of discharge collected in the lumen of the genital tract and the organs had distended, acute pain appeared, which allowed us to make the diagnosis. In the diagnostic process, transperineal ultrasonography turned out to be extremely helpful, allowing us to establish the type and thickness of the obstruction. The patient underwent excision of transverse vaginal septum, and postoperative silicon dilators were used to prevent the recurrence of the obstruction. There was no recurrence of urinary infection or complications during the 11 months of follow-up.

Hydrometrocolpos is a pelvic cystic mass representing the distension of the vagina and uterus due to a lower genital tract obstruction causing accumulation of utero-cervical-vaginal secretions or urine in the vagina and endometrial cavity. Prenatal diagnosis is uncommon and differential diagnosis of the underlying etiologies is quite challenging in the prenatal period. We present three cases of female fetuses with hydrometrocolpos and discuss the prenatal differential diagnoses in the light of ultrasound findings along with postnatal diagnoses and outcomes.

UNASSIGNED: and importance: McKusick-Kaufman syndrome (MKS) is a rarely reported autosomal recessive syndrome characterized by hydrometrocolpos (HMC), polydactyly and various gastrointestinal and renal manifestations.
UNASSIGNED: We present a case of suspected MKS in a prenatal ultrasound with dilated lateral ventricles of the brain and HMC.
UNASSIGNED: Main differential diagnosis includes Bardet-Beidel syndrome (BBS) which can present with HMC and polydactyly but retinal manifestations are a differentiating feature from MKS.
UNASSIGNED: Both of the disease syndromes are diagnosed clinically after birth.Keywords: McKusick Syndrome, Bardet-beidel syndrome, hydrometrocolpos, case report.

BACKGROUND: The prenatal detection rate of fetal uterine effusion is very low, and current case reports mainly focus on pathological hydrometrocolpos. We presented two cases of fetal physiological uterine effusion with different ultrasonic characteristics and compared them with one case of hydrometrocolpos with the hope of identifying strategies to reduce misdiagnosis of fetal uterine effusion.
METHODS: This paper reports the cases of two female fetuses with abnormal pelvic echoes in the third trimester, referred to a tertiary center to be screened for suspected pelvic teratoma and cystic mass, respectively. Ultrasound consultation revealed fetal uterine effusion. The two fetuses were delivered at our hospital after a full term. Re-examining the uterus and adnexa of the neonates revealed that the uterine effusion had subsided naturally. Another female fetus had a large cystic mass in the pelvic cavity in the third trimester, and prenatal examination indicated fetal hydrometrocolpos. The fetus was delivered at our hospital after a full term. The hydrometrocolpos existed even after birth. After consultation with a neonatal surgeon and gynecologist, the newborn was diagnosed with congenital imperforate hymen with hydrometrocolpos. Hymen puncture and open drainage led to a good prognosis.
CONCLUSIONS: Prenatal ultrasonography plays an important role in diagnosing and differentiating between physiological and pathological fetal uterine effusion. It can help reduce misdiagnoses that can lead to incorrect clinical decisions.

This case report is of a 35-week female neonate with a cystic abdominal mass. Physical examination was notable for post-axial polydactyly, distended abdomen, and abnormal urethral opening. Differential diagnosis includes Bardet-Biedl Syndrome (BBS), an autosomal recessive ciliopathy. Genetic panel revealed she was a carrier for a BBS mutation.