Exploring the intersection of tuberous sclerosis and precocious puberty unveiled by hematocolpos.

We present the case of a 6-year-old girl who initially presented with acute pelvic pain, ultimately diagnosed with imperforate hymen leading to hematocolpos. Further investigation revealed additional clinical features including academic struggles, mood swings, and cutaneous findings, prompting consideration of a neurocutaneous syndrome. Magnetic Resonance Imaging (MRI) revealed features consistent with tuberous sclerosis complex (TSC), including radial migration lines in the subcortical white matter and an incidental arachnoid cyst. Notably, this case exhibited a unique presentation with absence of typical TSC findings such as subependymal nodules or cortical tubers. Additionally, precocious puberty, rarely associated with TSC, was observed, suggesting a potential link between hypothalamic lesions and hormonal imbalance. This case underscores the importance of comprehensive evaluation in pediatric patients presenting with seemingly unrelated symptoms, as it may unveil underlying conditions necessitating tailored management strategies.

Management of Hematometra and Hematocolpos in Obstructive Mullerian Anomalies by Image-Guided Drainage with Interventional Radiology: A Multi-Institutional Case Series.

STUDY OBJECTIVE: To describe cases of image-guided drainage of symptomatic hematometrocolpos from obstructive Müllerian anomalies as a temporizing measure to manage acute pain symptoms and delay definitive management of the obstructive Müllerian anomalies that require complex reconstruction METHODS: Institutional Review Board exemption from all included institutions was obtained. A retrospective case series from 3 academic children's hospitals of 8 females under the age of 21 with symptomatic hematometrocolpos due to obstructive Müllerian anomalies drained by image-guided percutaneous transabdominal vaginal or uterine drainage with interventional radiology was reviewed and described. RESULTS: Eight pubertal patients with obstructive Müllerian anomalies (6 patients with distal vaginal agenesis, 1 patient with an obstructed uterine horn, and 1 patient with a high obstructed hemi-vagina) and symptomatic hematometrocolpos are reported. All patients with distal vaginal agenesis had greater than 3 cm lower vaginal agenesis, which would usually require complex vaginoplasty and use of postoperative stents. Given their immaturity and inability to use stents or dilators postoperatively or medical complexity, they subsequently underwent ultrasound-guided drainage of hematometrocolpos with interventional radiology to relieve pain symptoms, followed by menstrual suppression. The patients with obstructed uterine horns had complex medical and surgical histories requiring perioperative planning; they also underwent ultrasound-guided drainage of hematometra as a temporizing measure to manage acute symptoms. CONCLUSION: Patients presenting with symptomatic hematometrocolpos due to obstructive Müllerian anomalies might not be psychologically mature enough to undergo definitive complex reconstruction, which requires vaginal stent or dilator use postoperatively to prevent stenosis and other complications. Image-guided percutaneous drainage of symptomatic hematometrocolpos serves as a temporizing measure by offering pain relief until patients are ready to undergo surgical management and/or to allow time for complex surgical planning.

Recurrent formation of haematocolpos in a young girl with multiple congenital anomalies of the urogenital tract.

An 11-year-old girl, a known case of left crossed fused renal ectopia and sacral hypoplasia presented to the gynaecological OPD in Karachi, Pakistan, in February 2019 with complaints of abdominal pain. On examination, she was found to have a septum covering her vaginal orifice. She was subsequently diagnosed with haematocolpos secondary to imperforate hymen. Incision and drainage was done. However, despite surgical management, she continued to have recurrent formation of haematocolpos for the next two months secondary to multiple complete and partial transverse vaginal septa and post-operative formation of adhesions. Definitive management was done with ultrasound guided needle puncture and drainage, followed by post-operative tampon use to maintain patency.

Mimics of malignancy caused by concurrent imperforate hymen and transverse vaginal septum: an instructive case and review of the literature.

The coexistence of imperforate hymen and vaginal septum is rare and their ability to mimic malignant manifestations have not been frequently reported. This current case report describes a 13-year-old girl that presented with cyclic abdominal pain for 6 months. She was found to have a huge mass via abdominal plain film X-ray and sonography, with inexplicably high levels of serum carcinoembryonic antigen, cancer antigen (CA)-19-9 and CA-125. Pelvic computed tomography imaging disclosed two huge cystic lesions in the uterine and upper vaginal cavities. Surgical intervention conformed the diagnosis of a concurrent imperforate hymen and transverse vaginal septum, echoing the imaging findings of haematocolpometra. Her tumour marker levels gradually returned to normal after surgery. This rare case of concomitant imperforate hymen and transverse vaginal septum highlights that haematocolpometra, a benign disease that might mimic malignancy, should be taken into consideration in any adolescent females with an abdominal mass and amenorrhoea to ensure an early diagnosis and timely appropriate management.

Diagnosis and Management of Hymenal Variants: ACOG Committee Opinion, Number 780.

At puberty, a patient with an imperforate hymen typically presents with a vaginal bulge of thin hymenal tissue with a dark or bluish hue caused by the hematocolpos behind it. Other findings that may be present include an abdominal mass, urinary retention, dysuria, constipation, and dyschezia. On evaluation, the goal is to differentiate an imperforate hymen from other obstructing anatomic etiologies, such as labial adhesions, urogenital sinus, transverse vaginal septum, or distal vaginal atresia. Surgical intervention is necessary only in symptomatic prepubertal patients. After confirmation of the diagnosis, surgical intervention usually is deferred until pubertal estrogenization has occurred because the imperforate hymen may open spontaneously at puberty. It is important to complete an abdominal and a perineal examination. If the physical examination reveals a bulging hymen and ultrasonography reveals hematocolpos, further imaging is not required. However, if the diagnosis is not certain or there is a concern for a distal vaginal atresia, cervical atresia, an obstructed uterine horn, or transverse or longitudinal vaginal septum, magnetic resonance imaging is recommended. The ideal time for surgical intervention on hymenal tissue is before the onset of pain and after onset of pubertal development, when the vaginal tissue is estrogenized. Surgical management of clinically significant hymenal variations involves excision of the hymenal tissue and rarely is associated with long-term sequelae. If there is concern that the patient has a distal vaginal atresia or a transverse vaginal septum, the patient should be referred to a center with expertise in the management of these conditions.

[Primary amenorrhea by transverse vaginal septum: a case report and review of the literature]. / Amenorrea primaria por tabique transverso vaginal inferior: reporte de un caso y revisión de la bibliografía.

The transverse vaginal septum is one of the rarest anomalies of the reproductive tract classified depending on location. It is manifested by primary amenorrhea, cyclic pain and progressive mass growth at abdominopelvic level, ultrasound and magnetic resonance imaging provides the diagnosis, location and thickness of a transverse vaginal septum, treatment is surgical. We present a case of a teenage patient with primary amenorrhea due to lower transverse vaginal septum with surgical resection and satisfactory follow-up with successful vaginal patency.

Use of Bakri balloon for treatment of persistent hematocolpos after a LeFort Colpocleisis: a case report.

LeFort colpocleisis is a simple and effective procedure for pelvic organ prolapse in women who no longer wish to preserve coital function. If the vaginal channels created during this procedure are not large enough, blood or pus may collect within the uterus or proximal vagina. Hematocolpos, accumulation of blood in apical vagina, may be difficult to manage especially when a patient is frail and has medical comorbidities. Here, we present a case of LeFort colpocleisis in which excessive anticoagulation led to an infected hematocolpos and persistent bleeding. This was successfully managed with a Bakri balloon via a vaginal channel without recurrence of her prolapse.

Acute urinary retention in an adolescent girl and important learning points.

We presented a case of a 13-year-old girl who attended the emergency department with acute urinary retention and 1400 mL residual urine after catheterisation. She had no significant medical history, neurological examination was normal and she had not reached menarche. She was found to have a haematocolpos on ultrasound scan which was compressing the urinary bladder. Examination under anaesthesia confirmed an imperforate hymen and therefore an incision was performed and the haematocolpos drained. She managed to pass urine normally the day following her procedure. In this article, we emphasise on the differential diagnosis in this case and the learning points derived from it.