CONGENITAL IMPERFORATE HYMEN WITH BILATERAL HYDRONEPHROSIS, POLYDACTYLY AND LARYNGOCELE: A Rare Neonatal Presentation

Öztürk, Hülya; Yazıcı, Burhan; Küçük, Adem; Senses, Dursun Alí

doi:10.3109/15513811003620609

Cited by 11 publications

(9 citation statements)

References 12 publications

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“…As the condition progress, a chemical reaction in the uterine tube led to obstruction; progression of the hydrometrocolpos and decease of the ascites occurred. Although IH does not usually cause symptoms until puberty, Ozturk et al [9] reported a newborn of IH associated with hydrometrocolpos, bilateral hydronephrosis, polydactyly, and laryngocele. In our patient, the left cystic lesion was hydrometrocolpos.…”

Section: Discussionmentioning

confidence: 97%

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Congenital urethrovaginal fistula associated with imperforate hymen causing fetal urinary ascites and abdominal cystic lesions: A case report and literature review

Ohno

Nakaoka

Takama

et al. 2015

Journal of Pediatric Surgery Case Reports

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a b s t r a c tA primipara presented to our hospital due to fetal ascites, bilateral hydronephrosis and hydrometrocolpos. The female baby had two giant abdominal cysts, a single atrium and foot polydactyly. Absence of the vaginal orifice was noticed, and the hymen was broken through; the left cyst collapsed after fluid drainage. The right cyst was punctured and drained, which improved the hydronephrosis. Contrast imaging showed medium pouring into the abdominal cavity via the uterine tube; the right cyst was a 6 Â 5 cm closed cavity. Vaginal uroplania persisted even after hospital discharge. At the age of 4 years, cystoscopy showed a fistulous opening on the urethra 5 mm distal from the internal urethral orifice. A catheter inserted via the opening was found in the vagina. In our case, urine poured into the abdominal cavity through the fistula and uterine tube; following obstruction of the uterine tube, giant hydrometrocolpos and hydrosalpinx appeared. Three cases of congenital urethrovaginal fistula (CUVF) with imperforate hymen have been reported. A remnant of communication between the uterovaginal primordium and urogenital sinus may be responsible for CUVF.Ó 2015 The Authors. Published by Elsevier Inc. All rights reserved.Congenital urethrovaginal fistula (CUVF) is an extremely rare genitourinary anomaly, and etiology of CUVF remains unknown [1]. Only three CUVF cases associated with imperforate hymen (IH) have been reported [2,3]. Here, we present a newborn of CUVF with IH causing massive urinary ascites and large abdominal cystic lesions in fetal period. Furthermore, the pathology of the patient and etiology of CUVF are discussed. CaseA 28-years-old primipara was referred to our hospital with fetal ascites and bilateral hydronephrosis at 28 weeks of gestation. Amniotic fluid examination showed a normal female karyotype.Fetal magnetic resonance imaging (MRI) demonstrated bilateral hydronephrosis and massive ascites that significantly pushed up the thoracic cavity. In the lateral view, hydrometrocolpos, dilated urinary bladder, and a luminal structure between them were visible ( Fig. 1). At 30 weeks of gestation, the ascites gradually decreased; however, cystic lesions appeared in the fetal abdominal cavity at 32 weeks of gestation.A newborn baby, weighing 3,107 g, was born at 34 weeks of gestation by emergency Caesarean section due to fetal distress. Tracheal intubation, artificial ventilation, and nitric oxide inhalation were performed immediately in response to the patient's severe abdominal distension and respiratory failure. Abdominal ultrasonography revealed two giant cystic lesions. She had a single atrium and left-foot polydactyly. One day after birth, bulging of the vaginal vestibule and absence of the vaginal orifice were both observed. The external urethral orifice and anus were at normal position. Perineal ultrasonography showed a part of the cyst across a thin membrane. She was subsequently diagnosed with IH; the hymen was then punctured with forceps. The left cystic lesion collapsed after drainag...

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Section: Discussionmentioning

confidence: 97%

“…The solid vaginal plate then canalizes and the hymen usually dissolves during the late prenatal period. Failure of the hymen to perforate can result in IH [9].…”

Section: Discussionmentioning

confidence: 98%

Congenital urethrovaginal fistula associated with imperforate hymen causing fetal urinary ascites and abdominal cystic lesions: A case report and literature review

Ohno

Nakaoka

Takama

et al. 2015

Journal of Pediatric Surgery Case Reports

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“…Imperforate hymen most usually passes unrecognized in neonatal life, being identified later due to associated problems or their complications. [5] The aetiological basis for development of HMC in the neonate is therefore yet unclear. There had not been reported cases of HMC in the Niger Delta region of Nigeria; but we have observed a sudden increase in hospital incidence in the past 2 years.…”

Section: Introductionmentioning

confidence: 99%

Experience with neonatal hydrometrocolpos in the Niger Delta area of Nigeria: Upsurge or increased recognition?

Okoro¹,

Obiorah²,

Enyindah³

2016

Afr J Paediatr Surg

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Background:Hydrometrocolpos (HMC) is a clinical condition in which there is a cystic distension of the vagina (hydrocolpos), uterus and sometimes, fallopian tubes (hydrosalpinx) with fluid. This study described our experience with cases of HMC seen in our practice, and highlighted the flare in our practice.Patients and Methods:A retrospective study of cases of HMC managed at the University of Port Harcourt Teaching Hospital between September 2010 and August 2012.Results:There were seven cases; their ages ranged from 2 to 27 days (median 13 days). All the patients had abdominal distension but abdominal mass was obvious only in four. Other features varied depending on the presence of sepsis or other associated anomalies. The diagnosis of HMC was missed in all cases by the referring clinicians. One patient was referred with the diagnosis of tracheoesophageal fistula and esophageal atresia, 2 with bladder outlet obstruction, 1 with intestinal obstruction, 2 with anorectal malformation, and 1 with neonatal sepsis. Ultrasound identified bulky uterus in two cases and upper urinary tract dilatation in 3 patients. Six patients had laparotomy, 1 had hymenotomy only. Postoperative complications were basically wound sepsis and rectovaginal fistula resulted.Conclusion:Diagnosis of HMC should be considered as a differential in newborn girls presenting with lower abdominal mass. Attention to clinical detail is necessary to avoid a misdiagnosis.

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“…Vaginal atresia (VA) is one of them. Missing portion of the vagina is replaced with fibrous tissue (5). According to the American Society for Reproductive Medicine 1998 Classification, vaginal atresia is categorized as Type I (6).…”

Section: Introductionmentioning

confidence: 99%

“…According to the American Society for Reproductive Medicine 1998 Classification, vaginal atresia is categorized as Type I (6). Some authors believe that transverse vaginal septum is partial vaginal aplasia (5).…”

Section: Introductionmentioning

confidence: 99%

Distal vaginal atresia misdiagnosed as imperforate hymen: A case managed by transperineal vaginal pull through (distal colpoplasty)

Awad

El-Agwany

2015

The Egyptian Journal of Radiology and Nuclear Medicine

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Three different types of vaginal outlet obstruction are described in the literature: imperforate, transversal vaginal septum and vaginal atresia. Diagnoses were made in different ages of life, from neonatal to the teenage years. Clinical and ultrasound examinations and MRI dominated in establishing the diagnosis. Complex malformations of female genital tract are not so common.Their correct identification is of paramount importance for appropriate management. A thorough knowledge of embryology, pre-operative imaging with MRI and examination under anaesthesia is essential to identify accurately the constellation of anomalies and to plan appropriate management. This case reports distal vaginal agenesis in an 13 year old girl which was managed by dissecting the lower half of vagina and pull-through vaginoplasty. Rarity and variable presentation of congenital genital tract anomalies can lead to delayed diagnosis and erroneous management. A high index of suspicion and cross-sectional imaging can help in early diagnosis. A comprehensive management is imperative to preserve the reproductive potentials, as significant proportion of patients may experience sexual difficulties, menstrual irregularity, and infertility.

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CONGENITAL IMPERFORATE HYMEN WITH BILATERAL HYDRONEPHROSIS, POLYDACTYLY AND LARYNGOCELE: A Rare Neonatal Presentation

Cited by 11 publications

References 12 publications

Congenital urethrovaginal fistula associated with imperforate hymen causing fetal urinary ascites and abdominal cystic lesions: A case report and literature review

Congenital urethrovaginal fistula associated with imperforate hymen causing fetal urinary ascites and abdominal cystic lesions: A case report and literature review

Experience with neonatal hydrometrocolpos in the Niger Delta area of Nigeria: Upsurge or increased recognition?

Distal vaginal atresia misdiagnosed as imperforate hymen: A case managed by transperineal vaginal pull through (distal colpoplasty)

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