The Experts below are selected from a list of 306 Experts worldwide ranked by ideXlab platform
Aziz Baydada - One of the best experts on this subject based on the ideXlab platform.
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Congenital Vaginal Atresia: about an uncommon case.
The Pan African medical journal, 2020Co-Authors: Aziz Slaoui, Intissar Benzina, Sarah Talib, Amina Etber, Najia Zeraidi, Amina Lakhdar, Aicha Kharbach, Aziz BaydadaAbstract:Congenital Vaginal Atresia is a rare congenital abnormality of the female reproductive tract due to a failure of canalisation in the urogenital sinus. We report the uncommon case of a 14-year-old girl with a primary amenorrhea associated to a cyclical pelvic pain, in whom examination objectified a Vaginal cup that replaced the introitus. Ultrasound examination and magnetic resonance imaging (MRI) revealed Atresia of the lower third of the vagina. The diagnosis of partial Vaginal aplasia on functional uterus was retained, the patient had a perineal vaginoplasty. The evolution was satisfactory with regular cycles and improvement of pelvic pain. The decline is three years. Congenital Vaginal Atresia is a rare malformation classically and clinically pictured as a primary amenorrhea with chronic cyclic pelvic pain. Diagnosis is based on clinical examination and imaging. The MRI is designed to assess the importance of Atresia and guide surgical management while the surgical technique aims to restore the integrity of the utero-Vaginal tract and to increase the possibility of pregnancy for these patients.
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Winter syndrome: about an uncommon case report
BMC women's health, 2020Co-Authors: Aziz Slaoui, Sarah Talib, Abdelali Kallali, Mariem Rouijel, Aziz BaydadaAbstract:Background Congenital genital tract outflow obstruction may occur at different levels and with different clinical presentations. Winter syndrome was first described in 1968 as an association of renal, genital and middle ear anomalies. This syndrome is characterized by autosomal recessive transmission, unilateral or bilateral renal hypoplasia, distal Vaginal Atresia, and moderate to severe conductive hearing loss with malformation of the ossicles. The diagnosis is usually made when symptoms of obstruction are obvious. It presents most commonly with primary amenorrhea in a girl with a normal XX genotype, ovarian and hormone function; and cyclical abdominal pain. Ultrasound confirm the physical examination, revealing the presence of a normal uterus and cervix, normal ovaries and fallopian tubes, and a large hematocolpos. Case presentation This case reports Winter syndrome in a 14-year-old girl which Vaginal Atresia was managed by a trans perineal Vaginal pull through. Conclusions Winter syndrome is a rare congenital condition whose clinical picture is that of an adolescent girl with primary amenorrhea and cyclic pelvic pain due to Vaginal Atresia, varying degrees of renal dysgenesis and deafness due to malformation of the ossicles of the middle ear. Diagnosis is based on clinical examination and imaging. Magnetic resonance imaging allows assessing the importance of Atresia and thus guiding surgical management. The goals of surgical intervention are to provide relief from pain, ensure normal sexual intercourse and to preserve fertility. A thorough knowledge of embryology, pre-operative imaging with MRI and clinical examination is essential to plan an appropriate surgical management.
Bhupender Yadav - One of the best experts on this subject based on the ideXlab platform.
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Ultrasound-guided introital drainage of pyometrocolpos
Journal of Pediatric Surgery Case Reports, 2018Co-Authors: Il Kyoon Kim, Ranjith Vellody, Hans G. Pohl, Karun Sharma, Bhupender YadavAbstract:Abstract Pyometrocolpos can be caused by congenital malformations such as distal Vaginal Atresia and imperforate hymen. Patients usually present with obstructive urinary tract infections, acute kidney injury, or sepsis. Percutaneous drainage of the infected fluid can help treat the patient; however, recurrence is of concern. In this case report, we present a case of a child with recurrent pyometrocolpos due to distal Vaginal Atresia despite initial percutaneous drainage. To our knowledge, this is the first report of ultrasound-guided introital drainage of pyometrocolpos with relief of symptoms obviating the need for repeat drainage or immediate surgery.
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Ultrasound-guided introital drainage of pyometrocolpos
Elsevier, 2018Co-Authors: Il Kyoon Kim, Ranjith Vellody, Hans G. Pohl, Karun Sharma, Bhupender YadavAbstract:Pyometrocolpos can be caused by congenital malformations such as distal Vaginal Atresia and imperforate hymen. Patients usually present with obstructive urinary tract infections, acute kidney injury, or sepsis. Percutaneous drainage of the infected fluid can help treat the patient; however, recurrence is of concern. In this case report, we present a case of a child with recurrent pyometrocolpos due to distal Vaginal Atresia despite initial percutaneous drainage. To our knowledge, this is the first report of ultrasound-guided introital drainage of pyometrocolpos with relief of symptoms obviating the need for repeat drainage or immediate surgery. Keywords: Pyometrocolopos, Interventional radiology, Ultrasound guided, Introital drainag
Kimiyo Takagi - One of the best experts on this subject based on the ideXlab platform.
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Genital abnormalities in Pallister–Hall syndrome: Report of two patients and review of the literature†
American Journal of Medical Genetics Part A, 2010Co-Authors: Yoko Narumi, Tomoki Kosho, Goro Tsuruta, Masaaki Shiohara, Ei Shimazaki, Tetsuo Mori, Ayako Shimizu, Yasuhiko Igawa, Shuji Nishizawa, Kimiyo TakagiAbstract:We describe two patients with Pallister-Hall syndrome (PHS) with genital abnormalities: a female with hydrometrocolpos secondary to Vaginal Atresia and a male with micropenis, hypoplastic scrotum, and bilateral cryptorchidism. Nonsense mutations in GLI3 were identified in both patients. Clinical and molecular findings of 12 previously reported patients who had GLI3 mutations and genital abnormalities were reviewed. Genital features in the male patients included hypospadias, micropenis, and bifid or hypoplastic scrotum, whereas all the females had hydrometrocolpos and/or Vaginal Atresia. No hotspot for GLI3 mutations has been found. The urogenital and anorectal abnormalities associated with PHS might be related to dysregulation of SHH signaling caused by GLI3 mutations rather than hormonal aberrations. We recommend that clinical investigations of genital abnormalities are considered in patients with PHS, even those without hypopituitarism.
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Genital abnormalities in Pallister-Hall syndrome: Report of two patients and review of the literature.
American journal of medical genetics. Part A, 2010Co-Authors: Yoko Narumi, Tomoki Kosho, Goro Tsuruta, Masaaki Shiohara, Ei Shimazaki, Tetsuo Mori, Ayako Shimizu, Yasuhiko Igawa, Shuji Nishizawa, Kimiyo TakagiAbstract:We describe two patients with Pallister-Hall syndrome (PHS) with genital abnormalities: a female with hydrometrocolpos secondary to Vaginal Atresia and a male with micropenis, hypoplastic scrotum, and bilateral cryptorchidism. Nonsense mutations in GLI3 were identified in both patients. Clinical and molecular findings of 12 previously reported patients who had GLI3 mutations and genital abnormalities were reviewed. Genital features in the male patients included hypospadias, micropenis, and bifid or hypoplastic scrotum, whereas all the females had hydrometrocolpos and/or Vaginal Atresia. No hotspot for GLI3 mutations has been found. The urogenital and anorectal abnormalities associated with PHS might be related to dysregulation of SHH signaling caused by GLI3 mutations rather than hormonal aberrations. We recommend that clinical investigations of genital abnormalities are considered in patients with PHS, even those without hypopituitarism.
N. Alchihabi - One of the best experts on this subject based on the ideXlab platform.
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Surgical management of congenital high Vaginal Atresia
Orvosi hetilap, 1993Co-Authors: N. Alchihabi, A. PinterAbstract:In the past 15 years five neonates and children have been treated for high Vaginal Atresia at the Surgical Unit of Department of Pediatrics, University Medical School, Pecs, Hungary. In three of the six patients distal Atresia of the vagina was found (Type III). Two of the three were neonates and had a large hydrometrocolpos and the third, a 13-year-old girl, hematometrocolpos. In the fourth patient the Vaginal Atresia was associated with cloacal and anorectal malformation (Type V). In the fifth there was a duplication of uterus and vagina; however, only one of the two vaginas was atretic. In three patients a pull-through of the vagina was carried out, in one of them according to Pena, in another a transvesical approach, as suggested by Monfort, was used. Following repeated surgeries in the patient with cloacal malformation the vagina was replaced with large intestine. In the case of duplication of vagina and uterus one half of the duplication was removed.
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High Vaginal Atresia: surgical considerations
Pediatric Surgery International, 1992Co-Authors: A. Pinter, N. AlchihabiAbstract:During the past 15 years, six patients have been treated for high Vaginal Atresia in the Surgical Unit of the Department of Pediatrics, University Medical School of Pécs, Hungary. In three of the patients distal Atresia of the vagina was found (type III); two of the three were neonates and had a large hydrometrocolpos and the third, a 13-year-old girl, had a hematometrocolpos. In the fourth patient the Vaginal Atresia was associated with a cloacal and anorectal malformation (type V). In the fifth there was a duplication of uterus and vagina; however, only one of the two vaginas was atretic. In the sixth patient the Vaginal occlusion was not congenital but acquired. In three patients a pull-through of the vagina was carried out; in one the procedure according to Pena and in another a transvesical approach according to Monfort was used. In the patient with a cloacal malformation, the vagina was replaced by large intestine in several stages. In the patient with duplication of the vagina and uterus one-half of the duplication was removed. The acquired occlusion was treated by the abdominoperineal approach.
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High Vaginal Atresia: surgical considerations
Pediatric Surgery International, 1992Co-Authors: A. Pinter, N. AlchihabiAbstract:During the past 15 years, six patients have been treated for high Vaginal Atresia in the Surgical Unit of the Department of Pediatrics, University Medical School of Pecs, Hungary. In three of the patients distal Atresia of the vagina was found (type III); two of the three were neonates and had a large hydrometrocolpos and the third, a 13-year-old girl, had a hematometrocolpos. In the fourth patient the Vaginal Atresia was associated with a cloacal and anorectal malformation (type V). In the fifth there was a duplication of uterus and vagina; however, only one of the two vaginas was atretic. In the sixth patient the Vaginal occlusion was not congenital but acquired. In three patients a pull-through of the vagina was carried out; in one the procedure according to Pena and in another a transvesical approach according to Monfort was used. In the patient with a cloacal malformation, the vagina was replaced by large intestine in several stages. In the patient with duplication of the vagina and uterus one-half of the duplication was removed. The acquired occlusion was treated by the abdominoperineal approach.
Aziz Slaoui - One of the best experts on this subject based on the ideXlab platform.
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Congenital Vaginal Atresia: about an uncommon case.
The Pan African medical journal, 2020Co-Authors: Aziz Slaoui, Intissar Benzina, Sarah Talib, Amina Etber, Najia Zeraidi, Amina Lakhdar, Aicha Kharbach, Aziz BaydadaAbstract:Congenital Vaginal Atresia is a rare congenital abnormality of the female reproductive tract due to a failure of canalisation in the urogenital sinus. We report the uncommon case of a 14-year-old girl with a primary amenorrhea associated to a cyclical pelvic pain, in whom examination objectified a Vaginal cup that replaced the introitus. Ultrasound examination and magnetic resonance imaging (MRI) revealed Atresia of the lower third of the vagina. The diagnosis of partial Vaginal aplasia on functional uterus was retained, the patient had a perineal vaginoplasty. The evolution was satisfactory with regular cycles and improvement of pelvic pain. The decline is three years. Congenital Vaginal Atresia is a rare malformation classically and clinically pictured as a primary amenorrhea with chronic cyclic pelvic pain. Diagnosis is based on clinical examination and imaging. The MRI is designed to assess the importance of Atresia and guide surgical management while the surgical technique aims to restore the integrity of the utero-Vaginal tract and to increase the possibility of pregnancy for these patients.
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Winter syndrome: about an uncommon case report
BMC women's health, 2020Co-Authors: Aziz Slaoui, Sarah Talib, Abdelali Kallali, Mariem Rouijel, Aziz BaydadaAbstract:Background Congenital genital tract outflow obstruction may occur at different levels and with different clinical presentations. Winter syndrome was first described in 1968 as an association of renal, genital and middle ear anomalies. This syndrome is characterized by autosomal recessive transmission, unilateral or bilateral renal hypoplasia, distal Vaginal Atresia, and moderate to severe conductive hearing loss with malformation of the ossicles. The diagnosis is usually made when symptoms of obstruction are obvious. It presents most commonly with primary amenorrhea in a girl with a normal XX genotype, ovarian and hormone function; and cyclical abdominal pain. Ultrasound confirm the physical examination, revealing the presence of a normal uterus and cervix, normal ovaries and fallopian tubes, and a large hematocolpos. Case presentation This case reports Winter syndrome in a 14-year-old girl which Vaginal Atresia was managed by a trans perineal Vaginal pull through. Conclusions Winter syndrome is a rare congenital condition whose clinical picture is that of an adolescent girl with primary amenorrhea and cyclic pelvic pain due to Vaginal Atresia, varying degrees of renal dysgenesis and deafness due to malformation of the ossicles of the middle ear. Diagnosis is based on clinical examination and imaging. Magnetic resonance imaging allows assessing the importance of Atresia and thus guiding surgical management. The goals of surgical intervention are to provide relief from pain, ensure normal sexual intercourse and to preserve fertility. A thorough knowledge of embryology, pre-operative imaging with MRI and clinical examination is essential to plan an appropriate surgical management.
