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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., (Copyright © 2023. Published by Elsevier Inc.)

Background: Müllerian aplasia or Mayer-Rokitansky-Küster-Hauser syndrome is described as congenital absence of the proximal vagina with or without absence of the cervix and uterus, most often recognized after the onset of primary amenorrhea., Case: An 18-year-old woman presented to a free medical clinic in Arcahaie, Haiti with primary amenorrhea, abdominal distention, and cyclic monthly abdominal pain. Physical exam was significant for uterus palpable superior to the umbilicus, absence of vagina, and rectal exam without palpable vagina or cervix. Transabdominal and transperineal ultrasound examinations did not reveal hematocolpos. Exploratory laparotomy revealed severe endometriosis with bilateral hematosalpinx, markedly distended uterus, no proximal vagina, and normal ovaries. Uterine specimen was filled with blood and no clear cervix was present., Summary and Conclusion: Diagnosis of vaginal and cervical agenesis is complicated in low-resource settings and treatment must be modified when subspecialty care and consistent follow-up are not available., (Copyright © 2018 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Background: Abdominal pain, secondary amenorrhea, and abnormal uterine bleeding are common gynecologic presentations in adolescence. Rarely this can be associated with an acquired hematometra. Hematometra is a condition of retained blood or clot within the uterus. High-dose progestogenic agents in this age group have been implicated in the accumulation of a hematometra without other explanation., Cases: We present 4 cases of hematometra after depomedroxyprogesterone acetate (DMPA) therapy in previously menstruating adolescents. All 4 presented with abdominal pelvic pain and/or persistent abnormal uterine bleeding, with the diagnosis confirmed via ultrasound. Suction dilation and curettage was required in each case., Summary and Conclusion: DMPA is a possible cause of hematometra and should be considered in anatomically normal young women experiencing pain or abnormal bleeding out of character for typical long-term DMPA use., (Crown Copyright © 2018. Published by Elsevier Inc. All rights reserved.)

Study Objective: The objective of this study was to assess short-term benefits and risks of utero-vaginal anastomosis done for cases of cryptomenorrhea due to cervical atresia with vaginal aplasia., Design: Prospective study., Setting: Surgical procedures were done between December 2013 and September 2015 at the department of Obstetrics and Gynecology, Cairo University Hospital., Participants: Five patients who had cryptomenorrhea due to cervical atresia associated with vaginal aplasia were included., Interventions: Utero-vaginal anastomoses were performed in 2 stages; a stage of McIndoe vaginoplasty and a stage of excision of the atretic cervical tissue and anastomosing the uterus to the neovagina. Follow-up was done by gynecological and ultrasound examination in a duration ranged from 12 to 36 months., Main Outcome Measures: Occurrence of regular menstrual flow and relief of the severe cyclic pain., Results: All patients had relief of the severe cyclic pain. Four patients had regular menstrual flow. One patient developed occlusion of the track after 1 year and needed dilatation once. Three patients developed low vaginal stenosis without occlusion of the track. One patient had rectal injury repaired without causing postoperative morbidity., Conclusion: Uterovaginal anastomosis is a promising conservative management option for cervical atresia with vaginal aplasia, which has benefits but is not free of risks. Long-term follow-up is still needed to judge its feasibility. We recommend performing McIndoe vaginoplasty as a starting stage before the anastomosis preferably in a separate setting., (Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Background: McKusick Kaufman Syndrome (MKS), a rare genetic condition, presents in the neonatal period with a classic triad of postaxial polydactyly, congenital heart disease, and hydrometrocolpos. The diagnosis is typically clinical, based on the presence of polydactyly and hydrometrocolpos., Case: We report the case of a 13-year-old female, who was diagnosed with MKS in infancy and underwent vaginal reconstructive surgery for a urogenital sinus. She was lost to follow-up thereafter. She presented to our institution at age 13 with pyometra, pyosalpinx, and tubo-ovarian abscess due to a stenotic cervix obstructing menstrual outflow., Summary and Conclusion: Gynecologic follow-up is imperative in patients with history of vaginal reconstruction to monitor for hematometra from outflow obstruction to prevent life threatening secondary bacterial infections., (Published by Elsevier Inc.)

A 16-year-old adolescent girl presented with chronic pelvic pain. Pelvic ultrasound and MRI showing a uterus didelphys, normal left uterus and cervix, right pelvic fluid collection and right unilateral renal agenesis. After two unsuccessful vaginal surgeries for drainage of hematotrachelos and creation of an outflow tract, patient underwent unilateral total abdominal hysterectomy with final pathology confirming hematotrachelos and non-communicating cervical atresia on the right. Patient on postoperative follow-up doing well., (Published by Elsevier Inc.)

Background: Robert's uterus is an asymmetric septate uterus. This malformation is very rare, particularly in the pediatric age group. Radiologic findings could be surprising, but are very specific. We report here a representative case., Case: A 15-year-old patient complaining of recurrent abdominal cramps consulted the emergency department for evaluation of acute pain. Radiologic studies showed a circular lesion that had developed within the posterior wall of the uterus. The patient underwent surgery for a suspected Robert's uterus and an endometriectomy of the blind cavity was performed. There was no communication between the blind cavity and the ipsilateral fallopian tube., Summary and Conclusion: Pediatric surgeons and gynecologists should be aware of this atypical obstructive Müllerian malformation and its management in order to avoid inappropriate management of these young patients.

Background: Cryptomenorrhea at menarche is commonly due to mullerian duct anomalies. Uterine avulsion due to blunt pelvic trauma is a very rare cause of cryptomenorrhea. But so far no case of uterine avulsion has been reported due to a childhood trauma without pelvic fracture., Case: A 15-year-old young girl was evaluated for delayed menarche and the diagnosis of hematometra with obstruction at the level of internal cervical os was made on radiological examination. Detailed history revealed significant childhood blunt pelvic trauma when at the age of 3 years she was run over by a tractor. But the presence or absence of pelvic fracture was never documented as no radiographs were obtained. On abdominopelvic exploration cervix was well formed. Uterine body was found to be separated from the supravaginal cervix and there was a peritoneal window in between. During surgery hematometra was drained and the continuity of the uterine outflow tract was restored. Post surgery patient started having normal periods., Summary and Conclusions: Though very rare, uterine avulsion during pelvic trauma may cause cryptomenorrhea. Therefore such history should be sought in all cases of obstruction of the cervical canal because cryptomenorrhea has a much better prognosis than cervical congenital dysgenesis.

Background: Transverse vaginal septum is a rare cause of primary amenorrhea. It has a reported incidence of 1:2,100-1:72,000 and a variety of clinical presentations., Case: A 16-year-old patient presented with primary amenorrhea and a large abdominal mass that was palpated on clinical exam and confirmed on MRI imaging. A diagnosis of transverse vaginal septum was ultimately made and the patient underwent a successful surgical excision of the septum and vaginal reconstruction., Conclusion: The presentation, etiology, diagnosis, and surgical treatment of a transverse vaginal septum are discussed.

Five cases of uterus didelphys, unilateral imperforate vagina and ipsilateral renal agenesis are reported with a follow-up from 1 to 5 years. Diagnosis included typical revelation at puberty (2 cases) but also in the first months of life (2 cases). One case was only diagnosed at the age of 6 years, and the presence of an ectopic ureter was noted in the imperforate vagina. The authors point out the necessity of precise evaluation and careful management in order to maintain an intact reproductory tract. Simple excision of the septum is recommended which can most of the time be done by the vaginal approach.