- Email: [email protected]
Sclerotherapy for Treatment of a Labial Lymphatic Malformation
e46
Poster Abstracts / J Pediatr Adolesc Gynecol 25 (2012) e27ee48
irregular menstruation. The results of pelvic MRI performed at local hospital was uterine didelphys and obstructed left hemivagina with hematocolpos. But, our reading of the MRI was septate uterus with left hemivaginal obstruction and hematocolpos. And her kidney were normal findings in ultrasonography. She underwent resection of vaginal septum and diagnostic pelviscopy. Comments: Uterine didelphys is confused with sepatate uterus in patients with hemivaginal obstruction. The point of discrimination is that the shape of uterine fundus is convex in septate uterus. Unilateral renal agenesis is well known finding but not an obligatory in these anomalies. Because the management of these two uterine anomaly is different in the point of future fertility, correct diagnosis is important.
44. OHVIRA With a Twist: Obstructed Hemivagina Ipsilateral Renal Anomaly With Urogenital Sinus in Two Patients Irene Ma MD, Amy Williamson MD, Dorothy Rowe MD, Michael Ritchey MD, Kathleen Graziano MD Phoenix Children's Hospital, Phoenix, AZ, USA
Background: The syndrome obstructed hemivagina ipsilateral renal anomaly (OHVIRA) is a rare diagnosis in females that involves a combination of the triad of obstructed hemivagina, uterine anomaly (usually uterus didelphys), and ipsilateral renal anomaly. Presentation with non-specific pelvic symptoms can delay diagnosis. The OHVIRA syndrome can be associated with other anomalies due to abnormal embryologic development of the urogenital system. We therefore present two patients who had OHVIRA syndrome complicated by a urogenital sinus. Case: Case #1: A 14-y-o female with known absence of the left kidney and long-standing urinary incontinence presented with abdominal distention. Menarche was at age 12. An MRI revealed an obstructed left hemivagina and hemiuterus. Exam under anesthesia revealed a single orifice at the introitus. Cystoscopy revealed absence of the bladder neck; a separate vaginal opening could not be visualized. At exploration, the patient was found to have obstruction of a left hemiuterus and hemivagina and a small confluence of the right hemiuterus and hemivagina with the single opening on the perineum. She underwent reconstruction with enterocystoplasty, creation of a continent catheterizable channel and bladder neck closure. The obstructed left hemiuterus and hemivagina were removed and a vaginoplasty was performed to create patency for the right hemivagina. Case #2: A 4-y-o female with known absence of the right kidney and urinary incontinence was found on cystoscopy to have a single common channel at the introitus that communicated with the bladder and a hemivagina on the left. She underwent bladder augmentation with catheterizable channel and bladder neck closure, removal of a rudimentary hemiuterus on the right and vaginoplasty to establish patency of the left hemivagina with the common channel. Comments: OHVIRA is a rare congenital anomaly that can be associated with other developmental malformations. Our cases support the importance of identifying the presence of a urogenital sinus in the setting of the OHVIRA syndrome, as the surgical management for these patients may be affected. In both cases the urogenital sinus was preserved as the vaginal opening. Neither patient has required an introitoplasty.
paramesonephric and metanephric duct development during early € llerian structures may be gestation. The insult to development of the Mu a result of polygenetic and multifactorial inheritance. Kinship analysis studies have suggested that there is a 12 fold increase in first degree € llerian anomalies. Phenotypic relatives of individuals affected with Mu € llerian anomalies and familial clustering of cases heterogeneity of Mu renders differentiation between genetic and environmental contributions difficult. This case report describes monozygotic twins who presented with equal and opposite OHVIRA syndrome. Case: Monozygotic twins were born at full term following an uncomplicated pregnancy. Twin A presented at age 13 with cyclic left sided abdominal pain with menses. On examination, she was found to have leftsided hematocolpos. Ultrasound and MRI identified a uterine didelphys and left renal agenesis. Conversely, Twin B experienced cyclic right sided abdominal pain and on imaging of the pelvis was found to have a rightsided hematometra, uterine didelphys and right renal agenesis. € llerian anomalies have been shown to have a familial Comments: Mu component to their inheritance. OVHIRA syndrome presenting in monozygotic twins with mirror imaged anomalies supports familial aggregation and might suggest a monogenetic phenotype of OHVIRA syndrome.
46. Sclerotherapy for Treatment of a Labial Lymphatic Malformation Heather Appelbaum MD 1, David Siegel MD 2, Bradley Escaravage MD 2 1 The Steven and Alexandra Cohen Children's Medical Center of New York, New Hyde Park, New York 2 Long Island Jewish Medical Center, New Hyde Park, New York
Background: Lymphatic malformations arise as a result of inappropriate development of lymphatic channels. These lesions are typically multicompartmental, cross fascial planes, infiltrate viscera, and extend along neurovascular bundles. The most common indications for treatment of lymphatic malformations include mass effect, pain, bleeding, recurrent infection, or cosmetic concern. Historically, lymphatic malformations have been treated with surgical excision. Surgical morbidity includes frequent localized recurrence, peripheral nerve injury, infection, and delayed wound healing. More recent literature suggests that lymphatic malformations of the head, neck, trunk, abdomen, and extremities respond well to percutaneous sclerotherapy with more rapid recovery, improved cosmetic outcome, and less risk of nerve damage or infection. This is the first case to describe successful management of a large labial lymphatic malformation with percutaneous sclerotherapy. Case: 17 year old presents with enlargement of the right labia which was first recognized at the time of menarche. The mass was non tender and there were no urinary or bowel complaints. On examination, the right labia majora measured 10cm X 5cm (see figure 1). The labial mass was fluctuant, non tender, and without erythema or induration. The
45. Obstructed Hemivagina and Ipsilateral Renal Anomaly in Monozygotic Twins Nicole Smizer MD, Heather Appelbaum MD The Steven and Alexandra Cohen Children's Medical Center of New York, New Hyde Park, New York
Background: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome has been widely reported in the literature. This anatomical defect results from simultaneous embryologic alteration in
Fig. 1. Labial lymphatic malformation.Ă
Poster Abstracts / J Pediatr Adolesc Gynecol 25 (2012) e27ee48
e47
Fig. 4. Post labioplasty.Ă
may provide a less invasive approach to treatment of large lymphatic malformations of the labia. This case suggests that doxycycline percutaneous sclerotherapy is a safe and effective treatment for labial lymphatic malformations.
47. A Unique Support System for Teens Diagnosed With MRKH-Mayer Rokitansky Kuster Hauser Syndrome and Their Families
Fig. 2. Sclerotherapy of labial lymphatic malformation.Ă
Fig. 3. Eights weeks post sclerotherapy.Ă
mass did not change with Valsalva maneuvers nor was it reducible. There was no clitoromegaly and the remaining genital structures were normal. Translabial ultrasound identified an anechoic labial cyst with thin septations measuring 8.7 X 3.5 X 4.8cm. MRI confirmed these sonographic findings. Percutaneous drainage was performed with a 4 French centesis needle using ultrasound guidance (see figure 2). Following a fluoroscopic contrast study and aspiration of the contrast, doxycycline sclerotherapy was performed using 30cc of concentrated doxycycline (20 mg/cc). The sclerosant was aspirated after 20 minutes of dwell time. Subsequent delayed labioplasty was performed with excellent cosmetic results (see figure 3,4). Comment: Doxycycline functions as an inhibitor of angiogenesis by interfering with cell proliferation and migration via inhibition of matrix metalloproteinase and suppression of vascular endothelial growth factor induced angiogensis and lymphangiogenisis. It causes a fibrocytic cellular reaction with deposition of fibrin and collagen resulting in cystic regression. While surgical treatment remains the standard of care, sclerotherapy
Phaedra Thomas RN, BSN, Amanda Kohn BFA, Francie Mandel LICSW, Vicki Burke RN, BSN, Shaunna King RN, Marc R. Laufer MD Center for Young Women's Health (CYWH) and the Division of Gynecology, Children's Hospital Boston, Boston, MA
Background: Vaginal agenesis occurs in 1 out of every 4,000-10,000 females. The most common cause is congenital absence of the uterus and vagina (also referred to as mullerian aplasia, mullerian agenesis, or MRKH syndrome). Most adolescents select vaginal dilation using dilators or surgery to create a neovagina; however, some teens elect not to create a vagina or to delay treatment. The psychological implications of MRKH can be profound, thus education and support are essential as part of a comprehensive MRKH program. Our team developed the idea of offering a conference based on requests from teen participants in our moderated online MRKH chat. Methods: We polled patients and their parents regarding their interest in attending a conference that would provide both up-to-date information and support, and their response was overwhelmingly positive. Our goals were to create a conference designed specifically for teen girls diagnosed with MRKH and their families, develop and distribute engaging, understandable health guides, and provide participants with an opportunity to meet other teens and their families in a safe and supportive environment. We developed an evaluation tool to collect feedback from conference participants in order to improve future conferences. This abstract focuses on data from a series of 6 MRKH conferences held annually, starting in 2006. The MRKH conference is open to the public and held at a large pediatric institution. An average of 40 participants attend each year, from 6 countries and 23 US states to date. The program includes a medical presentation on MRKH and support groups for teens, parents, and family members led by clinical nurse specialists and a clinical social worker. The day concludes with a health team panel that summarizes the presentation and provides an opportunity for participants to ask questions. We also host a live webcast of the presentation for those unable to attend. Each participant receives a packet including our MRKH health guides for teens and parents, which are also accessible via our website. The teen MRKH guide has received 24,082 unique page views on the site since 2008, and the parent MRKH guide has received 12,003. Participants are asked to complete an evaluation to assess their overall satisfaction with the conference. Findings are entered into a database and reviewed by the conference planning committee, with the goal of improving future conferences. Over the past 6
Poster Abstracts / J Pediatr Adolesc Gynecol 25 (2012) e27ee48
irregular menstruation. The results of pelvic MRI performed at local hospital was uterine didelphys and obstructed left hemivagina with hematocolpos. But, our reading of the MRI was septate uterus with left hemivaginal obstruction and hematocolpos. And her kidney were normal findings in ultrasonography. She underwent resection of vaginal septum and diagnostic pelviscopy. Comments: Uterine didelphys is confused with sepatate uterus in patients with hemivaginal obstruction. The point of discrimination is that the shape of uterine fundus is convex in septate uterus. Unilateral renal agenesis is well known finding but not an obligatory in these anomalies. Because the management of these two uterine anomaly is different in the point of future fertility, correct diagnosis is important.
44. OHVIRA With a Twist: Obstructed Hemivagina Ipsilateral Renal Anomaly With Urogenital Sinus in Two Patients Irene Ma MD, Amy Williamson MD, Dorothy Rowe MD, Michael Ritchey MD, Kathleen Graziano MD Phoenix Children's Hospital, Phoenix, AZ, USA
Background: The syndrome obstructed hemivagina ipsilateral renal anomaly (OHVIRA) is a rare diagnosis in females that involves a combination of the triad of obstructed hemivagina, uterine anomaly (usually uterus didelphys), and ipsilateral renal anomaly. Presentation with non-specific pelvic symptoms can delay diagnosis. The OHVIRA syndrome can be associated with other anomalies due to abnormal embryologic development of the urogenital system. We therefore present two patients who had OHVIRA syndrome complicated by a urogenital sinus. Case: Case #1: A 14-y-o female with known absence of the left kidney and long-standing urinary incontinence presented with abdominal distention. Menarche was at age 12. An MRI revealed an obstructed left hemivagina and hemiuterus. Exam under anesthesia revealed a single orifice at the introitus. Cystoscopy revealed absence of the bladder neck; a separate vaginal opening could not be visualized. At exploration, the patient was found to have obstruction of a left hemiuterus and hemivagina and a small confluence of the right hemiuterus and hemivagina with the single opening on the perineum. She underwent reconstruction with enterocystoplasty, creation of a continent catheterizable channel and bladder neck closure. The obstructed left hemiuterus and hemivagina were removed and a vaginoplasty was performed to create patency for the right hemivagina. Case #2: A 4-y-o female with known absence of the right kidney and urinary incontinence was found on cystoscopy to have a single common channel at the introitus that communicated with the bladder and a hemivagina on the left. She underwent bladder augmentation with catheterizable channel and bladder neck closure, removal of a rudimentary hemiuterus on the right and vaginoplasty to establish patency of the left hemivagina with the common channel. Comments: OHVIRA is a rare congenital anomaly that can be associated with other developmental malformations. Our cases support the importance of identifying the presence of a urogenital sinus in the setting of the OHVIRA syndrome, as the surgical management for these patients may be affected. In both cases the urogenital sinus was preserved as the vaginal opening. Neither patient has required an introitoplasty.
paramesonephric and metanephric duct development during early € llerian structures may be gestation. The insult to development of the Mu a result of polygenetic and multifactorial inheritance. Kinship analysis studies have suggested that there is a 12 fold increase in first degree € llerian anomalies. Phenotypic relatives of individuals affected with Mu € llerian anomalies and familial clustering of cases heterogeneity of Mu renders differentiation between genetic and environmental contributions difficult. This case report describes monozygotic twins who presented with equal and opposite OHVIRA syndrome. Case: Monozygotic twins were born at full term following an uncomplicated pregnancy. Twin A presented at age 13 with cyclic left sided abdominal pain with menses. On examination, she was found to have leftsided hematocolpos. Ultrasound and MRI identified a uterine didelphys and left renal agenesis. Conversely, Twin B experienced cyclic right sided abdominal pain and on imaging of the pelvis was found to have a rightsided hematometra, uterine didelphys and right renal agenesis. € llerian anomalies have been shown to have a familial Comments: Mu component to their inheritance. OVHIRA syndrome presenting in monozygotic twins with mirror imaged anomalies supports familial aggregation and might suggest a monogenetic phenotype of OHVIRA syndrome.
46. Sclerotherapy for Treatment of a Labial Lymphatic Malformation Heather Appelbaum MD 1, David Siegel MD 2, Bradley Escaravage MD 2 1 The Steven and Alexandra Cohen Children's Medical Center of New York, New Hyde Park, New York 2 Long Island Jewish Medical Center, New Hyde Park, New York
Background: Lymphatic malformations arise as a result of inappropriate development of lymphatic channels. These lesions are typically multicompartmental, cross fascial planes, infiltrate viscera, and extend along neurovascular bundles. The most common indications for treatment of lymphatic malformations include mass effect, pain, bleeding, recurrent infection, or cosmetic concern. Historically, lymphatic malformations have been treated with surgical excision. Surgical morbidity includes frequent localized recurrence, peripheral nerve injury, infection, and delayed wound healing. More recent literature suggests that lymphatic malformations of the head, neck, trunk, abdomen, and extremities respond well to percutaneous sclerotherapy with more rapid recovery, improved cosmetic outcome, and less risk of nerve damage or infection. This is the first case to describe successful management of a large labial lymphatic malformation with percutaneous sclerotherapy. Case: 17 year old presents with enlargement of the right labia which was first recognized at the time of menarche. The mass was non tender and there were no urinary or bowel complaints. On examination, the right labia majora measured 10cm X 5cm (see figure 1). The labial mass was fluctuant, non tender, and without erythema or induration. The
45. Obstructed Hemivagina and Ipsilateral Renal Anomaly in Monozygotic Twins Nicole Smizer MD, Heather Appelbaum MD The Steven and Alexandra Cohen Children's Medical Center of New York, New Hyde Park, New York
Background: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome has been widely reported in the literature. This anatomical defect results from simultaneous embryologic alteration in
Fig. 1. Labial lymphatic malformation.Ă
Poster Abstracts / J Pediatr Adolesc Gynecol 25 (2012) e27ee48
e47
Fig. 4. Post labioplasty.Ă
may provide a less invasive approach to treatment of large lymphatic malformations of the labia. This case suggests that doxycycline percutaneous sclerotherapy is a safe and effective treatment for labial lymphatic malformations.
47. A Unique Support System for Teens Diagnosed With MRKH-Mayer Rokitansky Kuster Hauser Syndrome and Their Families
Fig. 2. Sclerotherapy of labial lymphatic malformation.Ă
Fig. 3. Eights weeks post sclerotherapy.Ă
mass did not change with Valsalva maneuvers nor was it reducible. There was no clitoromegaly and the remaining genital structures were normal. Translabial ultrasound identified an anechoic labial cyst with thin septations measuring 8.7 X 3.5 X 4.8cm. MRI confirmed these sonographic findings. Percutaneous drainage was performed with a 4 French centesis needle using ultrasound guidance (see figure 2). Following a fluoroscopic contrast study and aspiration of the contrast, doxycycline sclerotherapy was performed using 30cc of concentrated doxycycline (20 mg/cc). The sclerosant was aspirated after 20 minutes of dwell time. Subsequent delayed labioplasty was performed with excellent cosmetic results (see figure 3,4). Comment: Doxycycline functions as an inhibitor of angiogenesis by interfering with cell proliferation and migration via inhibition of matrix metalloproteinase and suppression of vascular endothelial growth factor induced angiogensis and lymphangiogenisis. It causes a fibrocytic cellular reaction with deposition of fibrin and collagen resulting in cystic regression. While surgical treatment remains the standard of care, sclerotherapy
Phaedra Thomas RN, BSN, Amanda Kohn BFA, Francie Mandel LICSW, Vicki Burke RN, BSN, Shaunna King RN, Marc R. Laufer MD Center for Young Women's Health (CYWH) and the Division of Gynecology, Children's Hospital Boston, Boston, MA
Background: Vaginal agenesis occurs in 1 out of every 4,000-10,000 females. The most common cause is congenital absence of the uterus and vagina (also referred to as mullerian aplasia, mullerian agenesis, or MRKH syndrome). Most adolescents select vaginal dilation using dilators or surgery to create a neovagina; however, some teens elect not to create a vagina or to delay treatment. The psychological implications of MRKH can be profound, thus education and support are essential as part of a comprehensive MRKH program. Our team developed the idea of offering a conference based on requests from teen participants in our moderated online MRKH chat. Methods: We polled patients and their parents regarding their interest in attending a conference that would provide both up-to-date information and support, and their response was overwhelmingly positive. Our goals were to create a conference designed specifically for teen girls diagnosed with MRKH and their families, develop and distribute engaging, understandable health guides, and provide participants with an opportunity to meet other teens and their families in a safe and supportive environment. We developed an evaluation tool to collect feedback from conference participants in order to improve future conferences. This abstract focuses on data from a series of 6 MRKH conferences held annually, starting in 2006. The MRKH conference is open to the public and held at a large pediatric institution. An average of 40 participants attend each year, from 6 countries and 23 US states to date. The program includes a medical presentation on MRKH and support groups for teens, parents, and family members led by clinical nurse specialists and a clinical social worker. The day concludes with a health team panel that summarizes the presentation and provides an opportunity for participants to ask questions. We also host a live webcast of the presentation for those unable to attend. Each participant receives a packet including our MRKH health guides for teens and parents, which are also accessible via our website. The teen MRKH guide has received 24,082 unique page views on the site since 2008, and the parent MRKH guide has received 12,003. Participants are asked to complete an evaluation to assess their overall satisfaction with the conference. Findings are entered into a database and reviewed by the conference planning committee, with the goal of improving future conferences. Over the past 6