Mini-Review Obstructed Hemivagina with Ipsilateral Renal Anomaly Xiomara M. Santos MD *, Jennifer E. Dietrich MD, MSc Division of Pediatric and Adolescent Gynecology, Department of Obstetrics and Gynecology and Pediatrics, Baylor College of Medicine, Houston, TX
a b s t r a c t The association of obstructed hemivagina with ipisilateral renal anomaly (OHVIRA) is a well-described entity. While there has been an increased familiarity with this disorder, the exact incidence of OHVIRA is unknown. Our aim was to review the available literature on this topic, look at common presentations, and uncommon presentations. This condition is a well-described entity but requires careful evaluation, because unique presentations do occur. Diagnostic challenges include time of presentation and symptoms associated with presentation. Surgical challenges include microperforation of the hemivaginal septum, pelvic inflammatory disease, thick septum, or high septum. Each of these must be managed carefully. Although a solitary kidney is frequently thought to be associated with OHVIRA, dysplastic kidneys, pelvic kidneys, or ectopic ureters can occur. Prompt and accurate diagnosis is essential for relief of symptoms and prevention of complications. To minimize problems associated with delayed diagnosis, magnetic resonance imaging evaluation is recommended along with referral to a center with expertise in these conditions. Key Words: Obstructed hemivagina with ipsilateral renal anomaly, Solitary kidney, Dysplastic kidneys, Pelvic kidneys, Ectopic ureters
Introduction
€ llerian anomalies occur in approximately 7% of Mu reproductive age women.1 Approximately 40% of these cases are associated with renal anomalies.2 The combination of an obstructed hemivagina and uterus didelphys was first described in 1922, and the triad of obstructed hemivagina, uterus didelphys, and ipisilateral renal anomaly was initially reported in 19503,4 (Fig. 1). Since the 1980s, the triad has also been called Herlyn-Werner-Wunderlich syndrome, although not consistently.5,6 The acronym OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) was initially suggested by Laufer and colleagues in 2007 to provide a common basis for describing the syndrome.7 This acronym also allows inclusion of other uterine anomalies, such as septate uterus, which was present in 22% of patients with OHVIRA in 1 series.8 The exact incidence of OHVIRA is not known; however, it is anticipated that among female patients presenting with a uterine duplication, 6% have an obstructed hemivagina, of which 65% occur on the right side.7,9 Renal agenesis is found in 63%-81% of cases with uterine duplication and in 92%100% of those with obstructed hemivagina.2 Uterine dupli€ llerian cation occurs as a result of failed fusion of the Mu ducts by 9 weeks' gestational age.10 During development, fusion may occur at the level of the vagina, and 75% of cases present with a complete (nonobstructed) or partial (obstructed) vaginal septum.11 OHVIRA occurs with the partially formed vaginal septum, resulting in 1 unobstructed vagina and 1 obstructed vagina.7 Some have postulated that genetics play a role; however, to date this
* Address correspondence to: Xiomara M. Santos, MD, Department of Obstetrics and Gynecology, Baylor College of Medicine, 6651 Main St, Suite F1050, Houston, TX 77030; Phone: (832) 826-7464; fax: (832) 825-9349 E-mail address: [email protected] (X.M. Santos).
has not been confirmed and developmental differences have been cited as multifactorial thus far. The majority of patients present following menarche due to pain-related symptoms.7 Patients may present earlier if concerns related to the spine or kidneys arise in the neonatal period, prompting imaging studies that reveal an anomaly.6 Historically, the clinical presentation has been considered to be similar among patients; however, it has become increasingly clear that there is significant heterogeneity. This may be due partly to better recognition of the condition by providers, as well as through changes in search terminology within the medical literature given the previously coined nomenclature: OHVIRA.7
Clinical Presentation and Diagnosis
In the classic presentation, adolescents present with progressive pelvic pain or dysmenorrhea.7 Cycles are often irregular for up to 3 years after onset of the first period, and most females with this condition do not present immediately after menarche. Over time, due to entrapment of menstrual contents within the obstructed hemivagina, this smaller vagina distends, resulting in pain, especially during and just after menstrual cycles.7 The vaginal septum on the affected side may be thin or thick. A thin vaginal septum typically distends from the upper vaginal fornix region of the patent vagina downward, and it can present as a vaginal sidewall bulge on examination.7 In this classic presentation, diagnosis of OHVIRA is made after the initial examination and/or imaging reveals the presence of a vaginal or pelvic mass, leading to further investigation.12 Because the presence of a dilated hemivagina (hematocolpos) frequently distorts the anatomy, diagnosis on ultrasound can be challenging and therefore pelvic magnetic resonance imaging (MRI) is recommended as the gold standard imaging
1083-3188/$ - see front matter Ó 2014 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpag.2014.09.008
2
X.M. Santos, J.E. Dietrich / J Pediatr Adolesc Gynecol xxx (2014) 1e4
Fig. 1. Obstructed right hemivagina and ipsilateral renal agenesis.Ă
modality (Fig. 2).6,13 In the past, laparoscopy or laparotomy was advocated for the clarification of anatomy; however, given the reported high accuracy of pelvic MRI in these cases, it is recommended to reserve laparoscopy for those cases where diagnosis is not clear after imaging or when MRI is not available.7 In cases where the vaginal septum is very thin, the pressure from retained menstrual products over time can result in a small perforation, leading to communication
between the 2 vaginas.14 This may result in prolonged menses or chronic vaginal discharge due to menses occurring normally on one side of the patent vagina and menses occurring slowly on the partially obstructed side, because menses are attempting to pass through a small microperforation.6,15 Small communications at the low isthmic or cervical level have also been described.6 The presence of a microperforation can make the diagnosis more challenging because initial imaging may not reveal the hemivagina fully in the absence of distention of the hemivaginal space. In addition, the presence of small opening in the otherwise obstructed vaginal septum may allow ascending migration of pathogenic bacteria, leading to infection of the hematocolpos and clinical findings consistent with pelvic inflammatory disease and abscess formation.14,15 Some patients may present with a dilated uterine cavity (hematometra) and/or a dilated fallopian tube (hematosalpinx), as well as bleeding into the peritoneal space due to blood stasis and retrograde menstruation from the obstructed side.6,16 These patients can present with acute-onset abdominal pain, fever, and vomiting.6 A thick vaginal septum limits the ability of the hemivagina to distend distally from within the vagina and makes retrograde menstruation more likely. In addition, this may lead to endometriosis, which has been reported in approximately 23% of patients with OHVIRA.7,14,16,17 In the nonclassic presentation, patients may initially present with a solitary kidney or a multicystic dysplastic
Fig. 2. Imaging findings of patient with left obstructed hemivagina. (A) Ultrasound findings of left obstructed hemivagina showing hematocolpos and hematometra. (B) MRI findings of same patient showing normal right uterus and right hemivagina and (C) MRI findings for left obstructed hememivagina showing left hematocolpos and hematometra.
X.M. Santos, J.E. Dietrich / J Pediatr Adolesc Gynecol xxx (2014) 1e4
kidney on prenatal ultrasound and the, during the course of neonatal follow-up, additional pelvic abnormalities or “cystic” structures may be noted, prompting further evaluation.6,15 Additionally, sometimes the condition is suspected when a child presents with a known history of a renal anomaly prior to menarchal onset. This may prompt a workup early in puberty given the high degree of associa€ llerian anomalies.2 tion between renal anomalies and Mu Although ipsilateral renal agenesis on the obstructed hemivaginal side is the most common renal anomaly associated with OHVIRA, other renal anomalies, including dysplastic kidney, pelvic kidney, and ectopic ureter, have also been reported.2,7 There are reports of an ectopic ureter with insertion into the obstructed hemivagina.15,18 This anomaly is often overlooked, and in some cases diagnosis is made when the patient reports persistent urine leakage through the vagina after the resection of vaginal septum.18 The presence of an ectopic ureter inserting into obstructed hemivagina can also lead to ascending infection and abscess formation. It is important to evaluate the renal system before excision of the vaginal septum to rule out other anomalies that might need to be addressed concomitantly. The presence of urogenital sinus in 2 patients with obstructed hemivagina and renal agenesis was recently described, emphasizing the wide variety of clinical presentations and associated anomalies.19 Consultation with urology is highly recommended for any renal anomaly. Management
Prompt and accurate diagnosis of OHVIRA is essential for the rapid relief of symptoms and for the prevention of complications such as endometriosis, pelvic adhesions, and infection. The definitive management of OHVIRA is excision of the vaginal septum and drainage of hematometrocolpos. In the past, a 2-stage procedure was commonly performed.7 The first surgery involved draining the hematocolpos, and during the second surgery, the vaginal septum was resected after a period of wound healing. Throughout the years, experience with single-stage procedure has increased and is currently advocated as the recommended approach, with a second procedure performed only if needed for optimization.7,16 The procedure is performed by excising the vaginal septum and closing the vaginal defect by suturing together the mucosal surfaces.10 The use of vaginoscopy before resection of the septum facilitates identifying the presence or absence of a microperforation. If a microperforation is identified, then the opening can be canalized and extended. For cases where no microperforation is seen, the use of a spinal needle for aspiration can facilitate identification of the correct hemivaginal space, even in the setting of oblique lying hemivagina (Fig. 3). Old dark blood withdrawn through the spinal needle identifies the space-containing hematocolpos.15 Once identified, the incision can be easily extended to identify the margins of the septum and hemivagina. For cases with a high thin septum, the use of a Foley catheter can help bring the septum distally to better facilitate excision.15 In this case, a spinal needle may be used to aspirate and identify the hematocolpos, and then a Foley catheter may be introduced through the same incision,
3
Fig. 3. Identification of obstructed hemivaginal space. Anatomic illustration of obstructed right hemivagina with arrow indicating proposed area for spinal needle insertion.
inflated, and drawn distally for improved exposure. The incision can then be extended beyond the margins of the catheter. In some instances, an extremely high septum and narrow vagina can preclude safe excision of vaginal septum, requiring hemihysterectomy for definitive management.7,20 A thick vaginal septum can also be challenging because the ability for this hemivagina to distend is limited. Nonetheless, it is important to plan in advance because inadequate excision of the septum can lead to stenosis with reobstruction.7,20,21 Suggested surgical techniques for excision of thicker septa are similar to those described for thick transverse vaginal septum. A circumferential “Z”-plasty technique allows for healing along the suture line to contract the incision longitudinally rather than transversally.22 Some authors suggest using a vaginal mold or stent postoperatively to decrease the risk of stenosis.23 The use of a biologically inert tracheobrochial stent to maintain patency and prevent stenosis in a case with high thick septum has also been described as a successful option.21 While prompt diagnosis and management of OHVIRA are essential, definitive management is not a surgical emergency, except for rare presentations with fever and peritonitis. In general, pain control can be achieved with the use of analgesic medications and hormonal contraceptives for menstrual suppression while awaiting surgical intervention.15 Because the presence of hematocolpos facilitates the excision of vaginal septum, some authors recommend discontinuation of hormonal suppression at least 3 months before surgery.15 Given the lack of long-term data regarding excision of vaginal septum in the prepubertal period and the potential surgical challenges of these cases at any age, it is recommended to delay resection of vaginal septum until the pubertal period. While most patients are adequately treated with the excision of vaginal septum alone and the risk of complications is low, specific surgical expertise in the management of these conditions is critical to avoid significant complications, including the presence of widespread infection, abscess formation, risk of laparotomy, hemihysterectomy, and loss of adnexal structures.14,17,20 While the reported
4
X.M. Santos, J.E. Dietrich / J Pediatr Adolesc Gynecol xxx (2014) 1e4
experience with OHVIRA occurring with an infectious process is limited to small numbers, cases treated conservatively also appear to have good outcomes. Management in the presence of infection should take into consideration extent of the infection, the presence or absence of abscess, and clinical stability of the patient. Conclusions
Prompt and accurate diagnosis of OHVIRA is essential for the relief of symptoms and prevention of complications. Although renal agenesis is the most common renal anomaly present, other anomalies have also been described and preoperative evaluation of renal system should be undertaken on every patient. To minimize problems associated with delayed diagnosis, MRI evaluation is recommended along with referral to a center with expertise in these conditions. References 1. Grimbizis GF, Campo M: Congenital malformations of the female genital tract: the need for a new classification system. Fertil Steril 2010; 94:401 2. Hall-Craggs MA, Kirkham A, Creighton SM: Renal and urological abnormalities occurring with Mullerian anomalies. J Pediatr Urol 2013; 9:27 3. Purslow CE: A case of unilateral haematocolpos, hematometra and haematosalpinx. J Obstet Gynaecol Br Emp 1922; 29:643 4. Embrey MP: A case of uterus didelphys with unilateral gynatresia. Br Med J 1950; 1:820 5. Karag'ozov I: Herlyn-Werner-Wunderlich syndrome. Akush Ginekol 1983; 22: 70 6. Orazi C, Lucchetti MC, Schingo PM, et al: Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol 2007; 37:657
7. Smith NA, Laufer MR: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril 2007; 87:918 8. Haddad B, Barranger E, Paniel BJ: Blind hemivagina: long-term follow up and reproductive performance in 42 cases. Hum Reprod 1999; 14:1962 9. Heinonen PK: Complete septate uterus with longitudinal vaginal septum. Fertil Steril 2006; 85:700 10. Breech LL, Laufer MR: Mullerian anomalies. Obstet Gynecol Clin North Am 2009; 36:47 11. Gholoum S, Puligandia PS, Hui T, et al: Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome). J Pediatr Surg 2006; 41:987 € llerian anomalies: diagnosis, 12. Vallerie AM, Breech LL: Update in Mu management, and outcomes. Curr Opin Obstet Gynecol 2010; 22:381 13. Troiano RN, McCarthy SM: Mullerian duct anomalies: imaging and clinical issues. Radiology 2004; 233:19 14. Zurawin RK, Dietrich JE, Heard MJ, et al: Didelphic uterus and obstructed hemivagina with renal agenesis: case report and review of literature. J Pediatr Adolesc Gynecol 2004; 17:137 15. Capito C, Echaieb A, Lortat-Jacob S, et al: Pitfalls in the diagnosis and management of obstructive uterovaginal duplication: a series of 32 cases. Pediatrics 2008; 122:e891 € du € cu € N, Go € nenc¸ G, Is¸c¸i H, et al: Herlyn-Werner-Wunderlich syndrome: 16. Gu timely diagnosis is important to preserve fertility. J Pediatr Adolesc Gynecol 2012; 25:e111 17. Candiani GB, Fedele L, Candiani M: Double uterus, blind hemivagina, and ipsilateral renal agensis: 36 cases and long-term follow up. Obstet Gynecol 1997; 90:26 18. Wang ZJ, Daldrup-Link H, Coakley FV, et al: Ectopic ureter associated with uterine didephys and obstructed hemivagina: preoperative diagnosis by MRI. Pediatr Radiol 2010; 40:358 19. Ma I, Williamson A, Rowe D, et al: OHVIRA with a twist: obstructed hemivagina ipsilateral renal anomaly with urogenital sinus in 2 patients. J Pediatr Adolesc Gynecol 2014; 27:104 20. Berger-Chen S, Ritch JM, Kim JH, et al: An unusual presentation of uterine didelphys and obstructed hemivagina. J Pediatr Adolesc Gynecol 2012; 25:e129 21. Cooper AR, Merritt DF: Novel use of a tracheobronchial stent in a patient with uterine didelphys and obstructed hemivagina. Fertil Steril 2010; 93:900 22. Wierrani F, Bodner K, Spangler B, et al: “Z”-plasty of the transverse vaginal septum using Garcia's procedure and the Grunberger modification. Fertil Steril 2003; 79:608 23. Lacy J, Cornell G, Walmer D, et al: Simple vaginal mold for use in the postoperative care of patients with a transverse vaginal septum. Fertil Steril 2007; 87:1225
a b s t r a c t The association of obstructed hemivagina with ipisilateral renal anomaly (OHVIRA) is a well-described entity. While there has been an increased familiarity with this disorder, the exact incidence of OHVIRA is unknown. Our aim was to review the available literature on this topic, look at common presentations, and uncommon presentations. This condition is a well-described entity but requires careful evaluation, because unique presentations do occur. Diagnostic challenges include time of presentation and symptoms associated with presentation. Surgical challenges include microperforation of the hemivaginal septum, pelvic inflammatory disease, thick septum, or high septum. Each of these must be managed carefully. Although a solitary kidney is frequently thought to be associated with OHVIRA, dysplastic kidneys, pelvic kidneys, or ectopic ureters can occur. Prompt and accurate diagnosis is essential for relief of symptoms and prevention of complications. To minimize problems associated with delayed diagnosis, magnetic resonance imaging evaluation is recommended along with referral to a center with expertise in these conditions. Key Words: Obstructed hemivagina with ipsilateral renal anomaly, Solitary kidney, Dysplastic kidneys, Pelvic kidneys, Ectopic ureters
Introduction
€ llerian anomalies occur in approximately 7% of Mu reproductive age women.1 Approximately 40% of these cases are associated with renal anomalies.2 The combination of an obstructed hemivagina and uterus didelphys was first described in 1922, and the triad of obstructed hemivagina, uterus didelphys, and ipisilateral renal anomaly was initially reported in 19503,4 (Fig. 1). Since the 1980s, the triad has also been called Herlyn-Werner-Wunderlich syndrome, although not consistently.5,6 The acronym OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) was initially suggested by Laufer and colleagues in 2007 to provide a common basis for describing the syndrome.7 This acronym also allows inclusion of other uterine anomalies, such as septate uterus, which was present in 22% of patients with OHVIRA in 1 series.8 The exact incidence of OHVIRA is not known; however, it is anticipated that among female patients presenting with a uterine duplication, 6% have an obstructed hemivagina, of which 65% occur on the right side.7,9 Renal agenesis is found in 63%-81% of cases with uterine duplication and in 92%100% of those with obstructed hemivagina.2 Uterine dupli€ llerian cation occurs as a result of failed fusion of the Mu ducts by 9 weeks' gestational age.10 During development, fusion may occur at the level of the vagina, and 75% of cases present with a complete (nonobstructed) or partial (obstructed) vaginal septum.11 OHVIRA occurs with the partially formed vaginal septum, resulting in 1 unobstructed vagina and 1 obstructed vagina.7 Some have postulated that genetics play a role; however, to date this
* Address correspondence to: Xiomara M. Santos, MD, Department of Obstetrics and Gynecology, Baylor College of Medicine, 6651 Main St, Suite F1050, Houston, TX 77030; Phone: (832) 826-7464; fax: (832) 825-9349 E-mail address: [email protected] (X.M. Santos).
has not been confirmed and developmental differences have been cited as multifactorial thus far. The majority of patients present following menarche due to pain-related symptoms.7 Patients may present earlier if concerns related to the spine or kidneys arise in the neonatal period, prompting imaging studies that reveal an anomaly.6 Historically, the clinical presentation has been considered to be similar among patients; however, it has become increasingly clear that there is significant heterogeneity. This may be due partly to better recognition of the condition by providers, as well as through changes in search terminology within the medical literature given the previously coined nomenclature: OHVIRA.7
Clinical Presentation and Diagnosis
In the classic presentation, adolescents present with progressive pelvic pain or dysmenorrhea.7 Cycles are often irregular for up to 3 years after onset of the first period, and most females with this condition do not present immediately after menarche. Over time, due to entrapment of menstrual contents within the obstructed hemivagina, this smaller vagina distends, resulting in pain, especially during and just after menstrual cycles.7 The vaginal septum on the affected side may be thin or thick. A thin vaginal septum typically distends from the upper vaginal fornix region of the patent vagina downward, and it can present as a vaginal sidewall bulge on examination.7 In this classic presentation, diagnosis of OHVIRA is made after the initial examination and/or imaging reveals the presence of a vaginal or pelvic mass, leading to further investigation.12 Because the presence of a dilated hemivagina (hematocolpos) frequently distorts the anatomy, diagnosis on ultrasound can be challenging and therefore pelvic magnetic resonance imaging (MRI) is recommended as the gold standard imaging
1083-3188/$ - see front matter Ó 2014 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpag.2014.09.008
2
X.M. Santos, J.E. Dietrich / J Pediatr Adolesc Gynecol xxx (2014) 1e4
Fig. 1. Obstructed right hemivagina and ipsilateral renal agenesis.Ă
modality (Fig. 2).6,13 In the past, laparoscopy or laparotomy was advocated for the clarification of anatomy; however, given the reported high accuracy of pelvic MRI in these cases, it is recommended to reserve laparoscopy for those cases where diagnosis is not clear after imaging or when MRI is not available.7 In cases where the vaginal septum is very thin, the pressure from retained menstrual products over time can result in a small perforation, leading to communication
between the 2 vaginas.14 This may result in prolonged menses or chronic vaginal discharge due to menses occurring normally on one side of the patent vagina and menses occurring slowly on the partially obstructed side, because menses are attempting to pass through a small microperforation.6,15 Small communications at the low isthmic or cervical level have also been described.6 The presence of a microperforation can make the diagnosis more challenging because initial imaging may not reveal the hemivagina fully in the absence of distention of the hemivaginal space. In addition, the presence of small opening in the otherwise obstructed vaginal septum may allow ascending migration of pathogenic bacteria, leading to infection of the hematocolpos and clinical findings consistent with pelvic inflammatory disease and abscess formation.14,15 Some patients may present with a dilated uterine cavity (hematometra) and/or a dilated fallopian tube (hematosalpinx), as well as bleeding into the peritoneal space due to blood stasis and retrograde menstruation from the obstructed side.6,16 These patients can present with acute-onset abdominal pain, fever, and vomiting.6 A thick vaginal septum limits the ability of the hemivagina to distend distally from within the vagina and makes retrograde menstruation more likely. In addition, this may lead to endometriosis, which has been reported in approximately 23% of patients with OHVIRA.7,14,16,17 In the nonclassic presentation, patients may initially present with a solitary kidney or a multicystic dysplastic
Fig. 2. Imaging findings of patient with left obstructed hemivagina. (A) Ultrasound findings of left obstructed hemivagina showing hematocolpos and hematometra. (B) MRI findings of same patient showing normal right uterus and right hemivagina and (C) MRI findings for left obstructed hememivagina showing left hematocolpos and hematometra.
X.M. Santos, J.E. Dietrich / J Pediatr Adolesc Gynecol xxx (2014) 1e4
kidney on prenatal ultrasound and the, during the course of neonatal follow-up, additional pelvic abnormalities or “cystic” structures may be noted, prompting further evaluation.6,15 Additionally, sometimes the condition is suspected when a child presents with a known history of a renal anomaly prior to menarchal onset. This may prompt a workup early in puberty given the high degree of associa€ llerian anomalies.2 tion between renal anomalies and Mu Although ipsilateral renal agenesis on the obstructed hemivaginal side is the most common renal anomaly associated with OHVIRA, other renal anomalies, including dysplastic kidney, pelvic kidney, and ectopic ureter, have also been reported.2,7 There are reports of an ectopic ureter with insertion into the obstructed hemivagina.15,18 This anomaly is often overlooked, and in some cases diagnosis is made when the patient reports persistent urine leakage through the vagina after the resection of vaginal septum.18 The presence of an ectopic ureter inserting into obstructed hemivagina can also lead to ascending infection and abscess formation. It is important to evaluate the renal system before excision of the vaginal septum to rule out other anomalies that might need to be addressed concomitantly. The presence of urogenital sinus in 2 patients with obstructed hemivagina and renal agenesis was recently described, emphasizing the wide variety of clinical presentations and associated anomalies.19 Consultation with urology is highly recommended for any renal anomaly. Management
Prompt and accurate diagnosis of OHVIRA is essential for the rapid relief of symptoms and for the prevention of complications such as endometriosis, pelvic adhesions, and infection. The definitive management of OHVIRA is excision of the vaginal septum and drainage of hematometrocolpos. In the past, a 2-stage procedure was commonly performed.7 The first surgery involved draining the hematocolpos, and during the second surgery, the vaginal septum was resected after a period of wound healing. Throughout the years, experience with single-stage procedure has increased and is currently advocated as the recommended approach, with a second procedure performed only if needed for optimization.7,16 The procedure is performed by excising the vaginal septum and closing the vaginal defect by suturing together the mucosal surfaces.10 The use of vaginoscopy before resection of the septum facilitates identifying the presence or absence of a microperforation. If a microperforation is identified, then the opening can be canalized and extended. For cases where no microperforation is seen, the use of a spinal needle for aspiration can facilitate identification of the correct hemivaginal space, even in the setting of oblique lying hemivagina (Fig. 3). Old dark blood withdrawn through the spinal needle identifies the space-containing hematocolpos.15 Once identified, the incision can be easily extended to identify the margins of the septum and hemivagina. For cases with a high thin septum, the use of a Foley catheter can help bring the septum distally to better facilitate excision.15 In this case, a spinal needle may be used to aspirate and identify the hematocolpos, and then a Foley catheter may be introduced through the same incision,
3
Fig. 3. Identification of obstructed hemivaginal space. Anatomic illustration of obstructed right hemivagina with arrow indicating proposed area for spinal needle insertion.
inflated, and drawn distally for improved exposure. The incision can then be extended beyond the margins of the catheter. In some instances, an extremely high septum and narrow vagina can preclude safe excision of vaginal septum, requiring hemihysterectomy for definitive management.7,20 A thick vaginal septum can also be challenging because the ability for this hemivagina to distend is limited. Nonetheless, it is important to plan in advance because inadequate excision of the septum can lead to stenosis with reobstruction.7,20,21 Suggested surgical techniques for excision of thicker septa are similar to those described for thick transverse vaginal septum. A circumferential “Z”-plasty technique allows for healing along the suture line to contract the incision longitudinally rather than transversally.22 Some authors suggest using a vaginal mold or stent postoperatively to decrease the risk of stenosis.23 The use of a biologically inert tracheobrochial stent to maintain patency and prevent stenosis in a case with high thick septum has also been described as a successful option.21 While prompt diagnosis and management of OHVIRA are essential, definitive management is not a surgical emergency, except for rare presentations with fever and peritonitis. In general, pain control can be achieved with the use of analgesic medications and hormonal contraceptives for menstrual suppression while awaiting surgical intervention.15 Because the presence of hematocolpos facilitates the excision of vaginal septum, some authors recommend discontinuation of hormonal suppression at least 3 months before surgery.15 Given the lack of long-term data regarding excision of vaginal septum in the prepubertal period and the potential surgical challenges of these cases at any age, it is recommended to delay resection of vaginal septum until the pubertal period. While most patients are adequately treated with the excision of vaginal septum alone and the risk of complications is low, specific surgical expertise in the management of these conditions is critical to avoid significant complications, including the presence of widespread infection, abscess formation, risk of laparotomy, hemihysterectomy, and loss of adnexal structures.14,17,20 While the reported
4
X.M. Santos, J.E. Dietrich / J Pediatr Adolesc Gynecol xxx (2014) 1e4
experience with OHVIRA occurring with an infectious process is limited to small numbers, cases treated conservatively also appear to have good outcomes. Management in the presence of infection should take into consideration extent of the infection, the presence or absence of abscess, and clinical stability of the patient. Conclusions
Prompt and accurate diagnosis of OHVIRA is essential for the relief of symptoms and prevention of complications. Although renal agenesis is the most common renal anomaly present, other anomalies have also been described and preoperative evaluation of renal system should be undertaken on every patient. To minimize problems associated with delayed diagnosis, MRI evaluation is recommended along with referral to a center with expertise in these conditions. References 1. Grimbizis GF, Campo M: Congenital malformations of the female genital tract: the need for a new classification system. Fertil Steril 2010; 94:401 2. Hall-Craggs MA, Kirkham A, Creighton SM: Renal and urological abnormalities occurring with Mullerian anomalies. J Pediatr Urol 2013; 9:27 3. Purslow CE: A case of unilateral haematocolpos, hematometra and haematosalpinx. J Obstet Gynaecol Br Emp 1922; 29:643 4. Embrey MP: A case of uterus didelphys with unilateral gynatresia. Br Med J 1950; 1:820 5. Karag'ozov I: Herlyn-Werner-Wunderlich syndrome. Akush Ginekol 1983; 22: 70 6. Orazi C, Lucchetti MC, Schingo PM, et al: Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol 2007; 37:657
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