CASE SERIES
Longitudinal Vaginal Septa With Associated Uterine Anomalies: A Case Series Snigdha Alur, MD, Katrina Nicandri, MD, and Bala Bhagavath, MD Objectives: Vaginal septa and associated mullerian anomalies can be difficult to diagnose and manage. This case series intends to educate the readers about the benefits and limitations of radiologic techniques both for the investigation as well as for the surgical management of these cases. It also seeks to inform them about a unique combination of vaginal and uterine anomalies known as Herlyn-Werner-Wunderlich syndrome. Cases: Three cases were discussed, each with a different presentation. All of the cases included obstructed right hemivagina and right renal agenesis. One case was associated with uterine septum and the other 2 cases were associated with uterus didelphys. Conclusions: The correct diagnosis of associated uterine anomalies in patients presenting with vaginal septa can be challenging. Diagnosis solely based on imaging should be cautioned because of its limitations. Use of ultrasound in the operating room can be a valuable tool. Key Words: vaginal septum, HWWS, mullerian anomalies, MRI (Female Pelvic Med Reconstr Surg 2015;21: e23–e26)
L
ongitudinal vaginal septa represent failure of the complete canalization of the vagina.1 In contrast, transverse vaginal septa form from vertical fusion defects. Transverse vaginal septa vary in location in the vagina but are generally in the middle or upper third of the vagina, are less than or equal to 1-cm thick, and have a reported incidence of 1:80,000.1 Congenital uterine anomalies are frequently associated with vaginal septa but more often with longitudinal vaginal septa than transverse vaginal septa.1 Congenital uterine anomalies have an incidence in the general population ranging from 0.001% to 10%.2 The types of anomalies in order of decreasing incidence are septate uterus, bicornuate uterus, arcuate uterus, unicornuate uterus, and uterus didelphys.1 Characterized by the presence of 2 uterine bodies and 2 cervices, uterine didelphys comprises 5% of all mullerian anomalies and results from a complete failure of fusion of the mullerian ducts.1 Uterus didelphys is interesting in that it is accompanied by longitudinal vaginal septa in 75% of cases. The combination of obstructed hemivagina, uterus didelphys, and unilateral renal agenesis is known as Herlyn-Werner-Wunderlich syndrome (HWWS).1 The obstructed hemivagina results from a longitudinal vaginal septum attaching to the vaginal side wall. Most patients with obstructed vaginal septa present with cyclical abdominal pain, secondary to the presence of hematocolpos or hematometra. Although HWWS was initially thought to be rare, with only 115 cases reported in the first 60 years since the syndrome was first reported, more than 170 cases have since been reported in the last 30 years alone.2 This apparent increase could at least be partly due to increased reporting more than a true increase in incidence. It has been hypothesized that as the use of From the Department of Obstetrics and Gynecology, Strong Memorial Hospital, University of Rochester, Rochester, NY. Reprints: Snigdha Alur, MD, University of Rochester Medical Center, Obstetrics and Gynecology Residency, 601 Elmwood Ave, Box 668 Rochester, NY 14642. E-mail: [email protected]. The authors have declared they have no conflicts of interest. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. DOI: 10.1097/SPV.0000000000000140
magnetic resonance imaging (MRI) and ultrasound increases, the identification and subsequent reporting of this syndrome will also increase.3 Magnetic resonance imaging and ultrasonography are often cited as the first-line imaging tools to aid in diagnosis.1,3,4 However, as explained later in this article, the key is to use them as a supplement rather than rely on them as the sole means of diagnosis. Recognition and prompt treatment of cases of obstructed vaginal septum can be very important because retrograde menstruation and prolonged hematocolpos or hematometra can have a negative effect on current and future reproductive health. The following 3 cases all presented to the same institution within a span of 8 months and represent various manifestations of longitudinal vaginal septa and their management, including 2 cases of HWWS. These cases were chosen because of the short time frame in which they presented at this institution and because they highlighted the following: diagnostic challenges that can occur, unique causes for delay in ultimate management, and importance of intraoperative sonogram in management.
CASE 1 The patient is an 18-year-old woman (gravida 0) who presented to the office with painful heavy vaginal bleeding. One year before presentation, she had undergone a laparoscopy and hysteroscopy (through the left cervix) at another institution that revealed a complete uterine septum and distention of the right side of the uterus. This distention was thought to be due to a hematometra formed from the uterine septum as well as a fallopian tube blocked by scar tissue from past surgery. This led to inability of blood to egress from the right side of the uterus. She had a hysteroscopic resection of this septum and drainage of the hematometra. She presented to our institution several months later with recurrence of her symptoms. Hematological evaluation was unremarkable. An MRI was performed, which showed a septate uterus communicating at the fundus with marked dilatation of the right uterine horn measuring up to 7.5 cm in the transverse dimension. Retrograde menstruation with distension of the fallopian tubes was present, which was greater on the right side than the left side. In addition, absence of the right kidney was also noted. Although not formally reported in the MRI, the imaging was suggestive to the gynecologist of an obstructed right hemivagina. Given the symptoms and the imaging, the decision was made to proceed with further evaluation in the operating room (OR). Examination in the OR revealed a bulging septum on the right side of the vagina and a normal-appearing left cervix. The right cervix could not be visualized. An 18-gauge spinal needle was placed through the obstructed hemivagina under ultrasound guidance to identify the correct plane. The septum was then incised, and a 30-cc Foley catheter was placed through this and inflated. The Foley catheter was used to guide scoring of the septum with Bovie cautery followed by complete excision of the vaginal septum. The mucosal edges were reapproximated using a running locking stitch to create a patent orifice. Hysteroscopic identification of the uterine body and both cornua was performed through the left cervix. Laparoscopy was then performed, which showed a blood-filled right fallopian tube with adhesions to the
Female Pelvic Medicine & Reconstructive Surgery • Volume 21, Number 3, May/June 2015
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Female Pelvic Medicine & Reconstructive Surgery • Volume 21, Number 3, May/June 2015
Alur et al
pelvic side wall (Figs. 1, 2). The right ovary appeared normal. The pelvic adhesions were resected and the right fallopian tube was removed because of presence of severe distention and adhesions. A normal left fallopian tube and left ovary were documented before completing the procedure. The patient was discharged home the same day in stable condition. She was seen in the office approximately 3 weeks after her procedure. Her menses had just started with no problems or pain, and her examination revealed a patent vagina and healing stitches from the surgery.
CASE 2 The patient is a 20-year-old woman (gravida 0) who presented with heavy menses in the setting of uterus didelphys and congenital absence of the right kidney diagnosed several years ago at an outside institution. The patient was prescribed oral contraceptive pills with minimal response. A pelvic ultrasound was obtained, which revealed the already known uterus didelphys as well as a fluid-filled structure in the inferior pelvis possibly indicating a right hematocolpos. Options were discussed and a decision was made to proceed with surgical intervention. An MRI was obtained for preoperative planning that confirmed the suspicion of a vaginal septum and demonstrated a right-sided fluid-filled structure distending the vagina measuring 4.0 3.0 8.0 cm as well as endocervical and endometrial canal suggestive of hematometrocolpos. The patient decided to postpone the surgery because of time constraints with schooling and presented several months later with foul-smelling vaginal discharge. Pelvic examination at that time was significant for a hole measuring less than 1 mm distal and to the right of the visible cervix with copious purulent drainage. There was a concern for microperforation in the obstructed right hemivagina with development of a pyocolpos and a possible pyometra. She was transferred to the hospital for treatment with intravenous antibiotics and was then taken to the OR. Examination under anesthesia revealed a normal-appearing cervix at the apex of the vagina on the left side. A bulge extending almost to the introitus was noted on the right with a small pinpoint hole at the apex draining purulent fluid. Because the septum was bulging and very obvious with the presence of a natural opening through which purulent material was draining, ultrasound guidance was not necessary for resection. An incision was made
FIGURE 1. From Case 1: Bicornuate uterus with marked dilatation of the right uterine horn measuring up to 7.5cm in the transverse dimension.
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FIGURE 2. From Case 1: Pelvic adhesions from the right fallopian tube to the pelvic sidewall.
sharply in the vaginal mucosa, and a large amount of purulent fluid was evacuated. A digital examination through this incision revealed a cervix on the right side of the vagina. The incision in the vaginal mucosa was extended using a knife until it spanned from the level of the left-sided cervix to the hymenal ring. The vaginal septum was removed in 1-cm-long sequential pieces, first from the inferior edge of the septum followed by the superior edge. Figure-of-8 stitches were placed after removal of each piece to aid with hemostasis. After complete resection of the septum, bimanual examination of both cervices could be performed easily. The patient was discharged home with oral antibiotics after she had received 24 hours of postoperative antibiotics. She followed up in the office 1 month after her procedure. Examination at that time revealed healing sutures and 2 normalappearing cervices with no evidence of infection or adhesions.
CASE 3 The patient is a 14-year-old girl (gravida 0) who presented to the emergency department with complaints of abdominal pain and worsening dysmenorrhea. She reported regular menses since menarche 9 months ago but reported progressively more painful and heavy periods. A pelvic ultrasound revealed a 16.6 10.3 15.6-cm fluid and debris-filled right adnexal mass indicative of pathology in the right fallopian tube. Of note, the patient also reported a history of right nephrectomy in infancy for a dysplastic right kidney. Because of this history and the presence of a dilated right fallopian tube, there was a concern for hematosalpinx and retrograde menstruation from a possible mullerian anomaly such as in HWWS. A pelvic examination was not performed secondary to patient refusal. Because of inadequate pain control, the patient was admitted to the hospital and an MRI was obtained, which revealed 2 separate uterine horns with a markedly dilated right uterine horn and vagina suggesting a hematometrocolpos. The findings were consistent with a diagnosis of uterus didelphys with obstructed right hemivagina. Because of poor pain control and the MRI findings previously, the decision was made to proceed with evaluation in the OR. Examination under anesthesia revealed an obstructed hemivagina on the right and a patent left vagina. Bimanual examination was significant for palpation of the left cervix. Under ultrasound guidance, a spinal needle was threaded through the vaginal septum to identify the fluid collection. A small incision was then made in the septum and a 30-cc Foley catheter was placed through this and inflated. The Foley catheter was used to guide complete excision of the vaginal septum, and the © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Copyright © 2015 Wolters Kluwer Health | Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Female Pelvic Medicine & Reconstructive Surgery • Volume 21, Number 3, May/June 2015
mucosal edges were reapproximated to create a patent orifice. The catheter was then removed, and the hematocolpos was drained. Hysteroscopic examination was then performed through each cervical os to identify each uterine horn and to ensure patency. A diagnostic laparoscopy was then performed, which revealed old blood from retrograde menstruation, 2 normal-appearing fallopian tubes, and 2 normal ovaries. It also revealed 2 uterine bodies consistent with uterus didelphys. The patient had an uncomplicated postoperative course significant for immediate improvement in abdominal pain. The postoperative visit 3 weeks later in the clinic revealed an asymptomatic patient with patent vagina on both sides.
SUMMARY AND CONCLUSIONS Although mullerian anomalies have many variations, unique combinations including vaginal septum accompanying a uterine septum might be more prevalent than initially thought.2 It is interesting to note that all of the 3 patients reviewed here presented within a span of 8 months to a single institution. This could be explained by the fact that this institution is a large academic center and referrals are more common, as in the first case. It could, however, also be due in part to increased use of imaging modalities such as the MRI. The diagnosis of mullerian anomalies such as vaginal septa can often be delayed or missed because of variable presentations including patients who remain asymptomatic. For example, although patients with transverse vaginal septa often present with hematocolpos soon after menarche, patients with longitudinal vaginal septa may remain asymptomatic. In our first case, the diagnosis of longitudinal vaginal septa was missed at the outside institution and hysteroscopy was performed only through the left cervix before presentation at our institution. In our second case, diagnosis of uterus didelphys was known previously; however, the presence of vaginal septa was not established until presentation to our institution as well. Diagnosis can be delayed in the case of uterine anomalies if only 1 horn is obstructed because the patient can menstruate regularly through the other horn. In addition, if a patient is treated with agents such as Depo-Provera, menses can be diminished or eliminated, thereby delaying the diagnosis as well.4 Increased accessibility, accuracy, and use of imaging modalities such as the MRI can contribute to accurate diagnosis and may account for the perceived rise in incidence of mullerian anomalies.2,3 A study done in the journal Radiology found a 100% accuracy of MRI in diagnosing mullerian duct anomalies.5 Magnetic resonance imaging has been found to be very useful because of its increased sensitivity when imaging soft tissue pelvic anatomy.4 Despite this increased accuracy, it is very important to correlate findings on imaging studies with clinical presentation as exemplified by the cases presented here. In our first case, the MRI suggested a septate uterus with no mention of an obstructed hemivagina. A pelvic ultrasound failed to reveal the complete diagnosis in our third case when compared with the MRI. Because of limitations in imaging, misdiagnosis with consequent delay in intervention can still occur. It has even been suggested that correct identification of the specific type of mullerian anomalies is increased if the radiologist is previously alerted to the suspicion rather than having to make a new diagnosis.3 In addition, imaging has a lower likelihood of picking up certain sequelae. A case report and literature review conducted by Zurawin et al,4 for example,discussed how MRI was unable to accurately assess for the presence of associated endometriosis, adhesions, or pelvic infection. Therefore, although the availability of imaging is an asset to assist with diagnosis, it should not be used as the sole decision maker. Ideally, imaging © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Longitudinal Vaginal Septa Case Series
ought to be interpreted in conjunction with clinical presentation, evaluation, and suspicion. The use of imaging intraoperatively can be very beneficial in many surgical scenarios. This is especially true in surgery for mullerian anomaly. Unless the vaginal septum is much distended with blood, the extent of the collection may not be clear to identify. This could have led to the missed diagnosis at the previous institution for our first case, where the patient had hysteroscopic resection of a uterine septum without recognition of the concomitant obstructed vaginal septum. The value of intraoperative use of ultrasound is characterized in cases 1 and 3. In both cases, the vaginal bulges were not obvious and ultrasound guidance was helpful in gaining entry into the obstructed vagina Both patients with HWWS (uterus didelphys, obstructed hemivagina, and concomitant renal anomalies) presented with the anomaly on the right side. It is thought that, in these cases, the lesion occurs from embryologic arrest that simultaneously affects the mullerian and metanephric ducts.6 The true incidence of this malady is unknown but has been reported to be between 0.1% and 3.8%.4 Presentation is variable from the classic abdominal pain as in our first case to pyometra or pyocolpos as demonstrated by our second case. A literature review by Vercellini et al7 suggests that, in women with uterus didelphys, the incidence of right-sided renal agenesis and obstructed hemivagina was as high as 65%. Both of our cases presented with right-sided obstruction, supporting this finding. Management of obstructed hemivagina was performed through a single-stage vaginoplasty in all cases. Although some advocate for a 2-stage procedure because of the anatomic distortion from hematocolpos and chronic inflammation, recent experience with a single-stage procedure has increased and results in fewer procedures and associated complications.3 Although longterm follow-up has not occurred for the presented patients, prior studies have shown a favorable outcome, with low rates of stricture formation or need for additional surgery. In 1 large case review, dysmenorrhea and abdominal pain improved in 87% and 100% of cases, respectively.8 Prompt treatment is essential to avoid compromising fertility as demonstrated by our first patient where prolonged hematosalpinx led to adhesion formation and required excision of the tube and also by our second patient who developed pyometra after delaying definitive treatment. In addition, endometriosis, which can also increase the risk for adhesion formation, has been hypothesized to be of frequent prevalence in patients with HWWS syndrome, cited to be of 23% prevalence compared with a baseline incidence of 10% to 50%.3 For this reason, we used laparoscopy in addition to hysteroscopy to evaluate for such sequelae such as adhesions, which the MRI is not sensitive to pick up. In summary, although the use of imaging studies results in increased detection of mullerian anomalies, the exact nature of the anomaly can only be accurately diagnosed by careful correlation with clinical presentation and findings. Relying solely on imaging can result in misdiagnosis and delay in appropriate management. Use of ultrasound examination intraoperatively is a valuable tool in obstructed vaginal septum cases, and management can often be performed as a single-stage vaginoplasty. REFERENCES 1. Cooper A, Merritt D. Vulvovaginal and Müllerian anomalies. In: Kliegman R, ed. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier; 2011:1874e1–1874e6. 2. Chang A, Siegel C, Moley K, et al. Septate uterus with cervical duplication and longitudinal vaginal septum: a report of five new cases. Fertil Steril 2004;81(4):1133–1136.
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3. Smith N, Laufer M. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril 2007; 87(4):918–922.
6. Fedele L, Motta F, Frontino G, et al. Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases. Hum Reprod 2013;28(6):1580–1583.
4. Zurawin RK, Dietrich JE, Heard MJ, et al. Didelphic uterus and obstructed hemivagina with renal agenesis: case report and review of the literature. J Pediatr Adolesc Gynecol 2004;17(2):137–141.
7. Vercellini P, Daguati R, Somigliana E, et al. Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review. Fertil Steril 2007;87(4):719–724.
5. Pellerito JS, McCarthy SM, Doyle MB, et al. Diagnosis of uterine anomalies: relative accuracy of MR imaging, endovaginal sonography, and hysterosalpingography. Radiology 1992;183(3):795–800.
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8. Haddad B, Barranger E, Paniel BJ. Blind hemivagina: long-term follow-up and reproductive performance in 42 cases. Hum Reprod 1999;14(8):1962–1964.
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Copyright © 2015 Wolters Kluwer Health | Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Longitudinal Vaginal Septa With Associated Uterine Anomalies: A Case Series Snigdha Alur, MD, Katrina Nicandri, MD, and Bala Bhagavath, MD Objectives: Vaginal septa and associated mullerian anomalies can be difficult to diagnose and manage. This case series intends to educate the readers about the benefits and limitations of radiologic techniques both for the investigation as well as for the surgical management of these cases. It also seeks to inform them about a unique combination of vaginal and uterine anomalies known as Herlyn-Werner-Wunderlich syndrome. Cases: Three cases were discussed, each with a different presentation. All of the cases included obstructed right hemivagina and right renal agenesis. One case was associated with uterine septum and the other 2 cases were associated with uterus didelphys. Conclusions: The correct diagnosis of associated uterine anomalies in patients presenting with vaginal septa can be challenging. Diagnosis solely based on imaging should be cautioned because of its limitations. Use of ultrasound in the operating room can be a valuable tool. Key Words: vaginal septum, HWWS, mullerian anomalies, MRI (Female Pelvic Med Reconstr Surg 2015;21: e23–e26)
L
ongitudinal vaginal septa represent failure of the complete canalization of the vagina.1 In contrast, transverse vaginal septa form from vertical fusion defects. Transverse vaginal septa vary in location in the vagina but are generally in the middle or upper third of the vagina, are less than or equal to 1-cm thick, and have a reported incidence of 1:80,000.1 Congenital uterine anomalies are frequently associated with vaginal septa but more often with longitudinal vaginal septa than transverse vaginal septa.1 Congenital uterine anomalies have an incidence in the general population ranging from 0.001% to 10%.2 The types of anomalies in order of decreasing incidence are septate uterus, bicornuate uterus, arcuate uterus, unicornuate uterus, and uterus didelphys.1 Characterized by the presence of 2 uterine bodies and 2 cervices, uterine didelphys comprises 5% of all mullerian anomalies and results from a complete failure of fusion of the mullerian ducts.1 Uterus didelphys is interesting in that it is accompanied by longitudinal vaginal septa in 75% of cases. The combination of obstructed hemivagina, uterus didelphys, and unilateral renal agenesis is known as Herlyn-Werner-Wunderlich syndrome (HWWS).1 The obstructed hemivagina results from a longitudinal vaginal septum attaching to the vaginal side wall. Most patients with obstructed vaginal septa present with cyclical abdominal pain, secondary to the presence of hematocolpos or hematometra. Although HWWS was initially thought to be rare, with only 115 cases reported in the first 60 years since the syndrome was first reported, more than 170 cases have since been reported in the last 30 years alone.2 This apparent increase could at least be partly due to increased reporting more than a true increase in incidence. It has been hypothesized that as the use of From the Department of Obstetrics and Gynecology, Strong Memorial Hospital, University of Rochester, Rochester, NY. Reprints: Snigdha Alur, MD, University of Rochester Medical Center, Obstetrics and Gynecology Residency, 601 Elmwood Ave, Box 668 Rochester, NY 14642. E-mail: [email protected]. The authors have declared they have no conflicts of interest. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. DOI: 10.1097/SPV.0000000000000140
magnetic resonance imaging (MRI) and ultrasound increases, the identification and subsequent reporting of this syndrome will also increase.3 Magnetic resonance imaging and ultrasonography are often cited as the first-line imaging tools to aid in diagnosis.1,3,4 However, as explained later in this article, the key is to use them as a supplement rather than rely on them as the sole means of diagnosis. Recognition and prompt treatment of cases of obstructed vaginal septum can be very important because retrograde menstruation and prolonged hematocolpos or hematometra can have a negative effect on current and future reproductive health. The following 3 cases all presented to the same institution within a span of 8 months and represent various manifestations of longitudinal vaginal septa and their management, including 2 cases of HWWS. These cases were chosen because of the short time frame in which they presented at this institution and because they highlighted the following: diagnostic challenges that can occur, unique causes for delay in ultimate management, and importance of intraoperative sonogram in management.
CASE 1 The patient is an 18-year-old woman (gravida 0) who presented to the office with painful heavy vaginal bleeding. One year before presentation, she had undergone a laparoscopy and hysteroscopy (through the left cervix) at another institution that revealed a complete uterine septum and distention of the right side of the uterus. This distention was thought to be due to a hematometra formed from the uterine septum as well as a fallopian tube blocked by scar tissue from past surgery. This led to inability of blood to egress from the right side of the uterus. She had a hysteroscopic resection of this septum and drainage of the hematometra. She presented to our institution several months later with recurrence of her symptoms. Hematological evaluation was unremarkable. An MRI was performed, which showed a septate uterus communicating at the fundus with marked dilatation of the right uterine horn measuring up to 7.5 cm in the transverse dimension. Retrograde menstruation with distension of the fallopian tubes was present, which was greater on the right side than the left side. In addition, absence of the right kidney was also noted. Although not formally reported in the MRI, the imaging was suggestive to the gynecologist of an obstructed right hemivagina. Given the symptoms and the imaging, the decision was made to proceed with further evaluation in the operating room (OR). Examination in the OR revealed a bulging septum on the right side of the vagina and a normal-appearing left cervix. The right cervix could not be visualized. An 18-gauge spinal needle was placed through the obstructed hemivagina under ultrasound guidance to identify the correct plane. The septum was then incised, and a 30-cc Foley catheter was placed through this and inflated. The Foley catheter was used to guide scoring of the septum with Bovie cautery followed by complete excision of the vaginal septum. The mucosal edges were reapproximated using a running locking stitch to create a patent orifice. Hysteroscopic identification of the uterine body and both cornua was performed through the left cervix. Laparoscopy was then performed, which showed a blood-filled right fallopian tube with adhesions to the
Female Pelvic Medicine & Reconstructive Surgery • Volume 21, Number 3, May/June 2015
www.fpmrs.net
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Copyright © 2015 Wolters Kluwer Health | Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Female Pelvic Medicine & Reconstructive Surgery • Volume 21, Number 3, May/June 2015
Alur et al
pelvic side wall (Figs. 1, 2). The right ovary appeared normal. The pelvic adhesions were resected and the right fallopian tube was removed because of presence of severe distention and adhesions. A normal left fallopian tube and left ovary were documented before completing the procedure. The patient was discharged home the same day in stable condition. She was seen in the office approximately 3 weeks after her procedure. Her menses had just started with no problems or pain, and her examination revealed a patent vagina and healing stitches from the surgery.
CASE 2 The patient is a 20-year-old woman (gravida 0) who presented with heavy menses in the setting of uterus didelphys and congenital absence of the right kidney diagnosed several years ago at an outside institution. The patient was prescribed oral contraceptive pills with minimal response. A pelvic ultrasound was obtained, which revealed the already known uterus didelphys as well as a fluid-filled structure in the inferior pelvis possibly indicating a right hematocolpos. Options were discussed and a decision was made to proceed with surgical intervention. An MRI was obtained for preoperative planning that confirmed the suspicion of a vaginal septum and demonstrated a right-sided fluid-filled structure distending the vagina measuring 4.0 3.0 8.0 cm as well as endocervical and endometrial canal suggestive of hematometrocolpos. The patient decided to postpone the surgery because of time constraints with schooling and presented several months later with foul-smelling vaginal discharge. Pelvic examination at that time was significant for a hole measuring less than 1 mm distal and to the right of the visible cervix with copious purulent drainage. There was a concern for microperforation in the obstructed right hemivagina with development of a pyocolpos and a possible pyometra. She was transferred to the hospital for treatment with intravenous antibiotics and was then taken to the OR. Examination under anesthesia revealed a normal-appearing cervix at the apex of the vagina on the left side. A bulge extending almost to the introitus was noted on the right with a small pinpoint hole at the apex draining purulent fluid. Because the septum was bulging and very obvious with the presence of a natural opening through which purulent material was draining, ultrasound guidance was not necessary for resection. An incision was made
FIGURE 1. From Case 1: Bicornuate uterus with marked dilatation of the right uterine horn measuring up to 7.5cm in the transverse dimension.
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FIGURE 2. From Case 1: Pelvic adhesions from the right fallopian tube to the pelvic sidewall.
sharply in the vaginal mucosa, and a large amount of purulent fluid was evacuated. A digital examination through this incision revealed a cervix on the right side of the vagina. The incision in the vaginal mucosa was extended using a knife until it spanned from the level of the left-sided cervix to the hymenal ring. The vaginal septum was removed in 1-cm-long sequential pieces, first from the inferior edge of the septum followed by the superior edge. Figure-of-8 stitches were placed after removal of each piece to aid with hemostasis. After complete resection of the septum, bimanual examination of both cervices could be performed easily. The patient was discharged home with oral antibiotics after she had received 24 hours of postoperative antibiotics. She followed up in the office 1 month after her procedure. Examination at that time revealed healing sutures and 2 normalappearing cervices with no evidence of infection or adhesions.
CASE 3 The patient is a 14-year-old girl (gravida 0) who presented to the emergency department with complaints of abdominal pain and worsening dysmenorrhea. She reported regular menses since menarche 9 months ago but reported progressively more painful and heavy periods. A pelvic ultrasound revealed a 16.6 10.3 15.6-cm fluid and debris-filled right adnexal mass indicative of pathology in the right fallopian tube. Of note, the patient also reported a history of right nephrectomy in infancy for a dysplastic right kidney. Because of this history and the presence of a dilated right fallopian tube, there was a concern for hematosalpinx and retrograde menstruation from a possible mullerian anomaly such as in HWWS. A pelvic examination was not performed secondary to patient refusal. Because of inadequate pain control, the patient was admitted to the hospital and an MRI was obtained, which revealed 2 separate uterine horns with a markedly dilated right uterine horn and vagina suggesting a hematometrocolpos. The findings were consistent with a diagnosis of uterus didelphys with obstructed right hemivagina. Because of poor pain control and the MRI findings previously, the decision was made to proceed with evaluation in the OR. Examination under anesthesia revealed an obstructed hemivagina on the right and a patent left vagina. Bimanual examination was significant for palpation of the left cervix. Under ultrasound guidance, a spinal needle was threaded through the vaginal septum to identify the fluid collection. A small incision was then made in the septum and a 30-cc Foley catheter was placed through this and inflated. The Foley catheter was used to guide complete excision of the vaginal septum, and the © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Copyright © 2015 Wolters Kluwer Health | Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Female Pelvic Medicine & Reconstructive Surgery • Volume 21, Number 3, May/June 2015
mucosal edges were reapproximated to create a patent orifice. The catheter was then removed, and the hematocolpos was drained. Hysteroscopic examination was then performed through each cervical os to identify each uterine horn and to ensure patency. A diagnostic laparoscopy was then performed, which revealed old blood from retrograde menstruation, 2 normal-appearing fallopian tubes, and 2 normal ovaries. It also revealed 2 uterine bodies consistent with uterus didelphys. The patient had an uncomplicated postoperative course significant for immediate improvement in abdominal pain. The postoperative visit 3 weeks later in the clinic revealed an asymptomatic patient with patent vagina on both sides.
SUMMARY AND CONCLUSIONS Although mullerian anomalies have many variations, unique combinations including vaginal septum accompanying a uterine septum might be more prevalent than initially thought.2 It is interesting to note that all of the 3 patients reviewed here presented within a span of 8 months to a single institution. This could be explained by the fact that this institution is a large academic center and referrals are more common, as in the first case. It could, however, also be due in part to increased use of imaging modalities such as the MRI. The diagnosis of mullerian anomalies such as vaginal septa can often be delayed or missed because of variable presentations including patients who remain asymptomatic. For example, although patients with transverse vaginal septa often present with hematocolpos soon after menarche, patients with longitudinal vaginal septa may remain asymptomatic. In our first case, the diagnosis of longitudinal vaginal septa was missed at the outside institution and hysteroscopy was performed only through the left cervix before presentation at our institution. In our second case, diagnosis of uterus didelphys was known previously; however, the presence of vaginal septa was not established until presentation to our institution as well. Diagnosis can be delayed in the case of uterine anomalies if only 1 horn is obstructed because the patient can menstruate regularly through the other horn. In addition, if a patient is treated with agents such as Depo-Provera, menses can be diminished or eliminated, thereby delaying the diagnosis as well.4 Increased accessibility, accuracy, and use of imaging modalities such as the MRI can contribute to accurate diagnosis and may account for the perceived rise in incidence of mullerian anomalies.2,3 A study done in the journal Radiology found a 100% accuracy of MRI in diagnosing mullerian duct anomalies.5 Magnetic resonance imaging has been found to be very useful because of its increased sensitivity when imaging soft tissue pelvic anatomy.4 Despite this increased accuracy, it is very important to correlate findings on imaging studies with clinical presentation as exemplified by the cases presented here. In our first case, the MRI suggested a septate uterus with no mention of an obstructed hemivagina. A pelvic ultrasound failed to reveal the complete diagnosis in our third case when compared with the MRI. Because of limitations in imaging, misdiagnosis with consequent delay in intervention can still occur. It has even been suggested that correct identification of the specific type of mullerian anomalies is increased if the radiologist is previously alerted to the suspicion rather than having to make a new diagnosis.3 In addition, imaging has a lower likelihood of picking up certain sequelae. A case report and literature review conducted by Zurawin et al,4 for example,discussed how MRI was unable to accurately assess for the presence of associated endometriosis, adhesions, or pelvic infection. Therefore, although the availability of imaging is an asset to assist with diagnosis, it should not be used as the sole decision maker. Ideally, imaging © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Longitudinal Vaginal Septa Case Series
ought to be interpreted in conjunction with clinical presentation, evaluation, and suspicion. The use of imaging intraoperatively can be very beneficial in many surgical scenarios. This is especially true in surgery for mullerian anomaly. Unless the vaginal septum is much distended with blood, the extent of the collection may not be clear to identify. This could have led to the missed diagnosis at the previous institution for our first case, where the patient had hysteroscopic resection of a uterine septum without recognition of the concomitant obstructed vaginal septum. The value of intraoperative use of ultrasound is characterized in cases 1 and 3. In both cases, the vaginal bulges were not obvious and ultrasound guidance was helpful in gaining entry into the obstructed vagina Both patients with HWWS (uterus didelphys, obstructed hemivagina, and concomitant renal anomalies) presented with the anomaly on the right side. It is thought that, in these cases, the lesion occurs from embryologic arrest that simultaneously affects the mullerian and metanephric ducts.6 The true incidence of this malady is unknown but has been reported to be between 0.1% and 3.8%.4 Presentation is variable from the classic abdominal pain as in our first case to pyometra or pyocolpos as demonstrated by our second case. A literature review by Vercellini et al7 suggests that, in women with uterus didelphys, the incidence of right-sided renal agenesis and obstructed hemivagina was as high as 65%. Both of our cases presented with right-sided obstruction, supporting this finding. Management of obstructed hemivagina was performed through a single-stage vaginoplasty in all cases. Although some advocate for a 2-stage procedure because of the anatomic distortion from hematocolpos and chronic inflammation, recent experience with a single-stage procedure has increased and results in fewer procedures and associated complications.3 Although longterm follow-up has not occurred for the presented patients, prior studies have shown a favorable outcome, with low rates of stricture formation or need for additional surgery. In 1 large case review, dysmenorrhea and abdominal pain improved in 87% and 100% of cases, respectively.8 Prompt treatment is essential to avoid compromising fertility as demonstrated by our first patient where prolonged hematosalpinx led to adhesion formation and required excision of the tube and also by our second patient who developed pyometra after delaying definitive treatment. In addition, endometriosis, which can also increase the risk for adhesion formation, has been hypothesized to be of frequent prevalence in patients with HWWS syndrome, cited to be of 23% prevalence compared with a baseline incidence of 10% to 50%.3 For this reason, we used laparoscopy in addition to hysteroscopy to evaluate for such sequelae such as adhesions, which the MRI is not sensitive to pick up. In summary, although the use of imaging studies results in increased detection of mullerian anomalies, the exact nature of the anomaly can only be accurately diagnosed by careful correlation with clinical presentation and findings. Relying solely on imaging can result in misdiagnosis and delay in appropriate management. Use of ultrasound examination intraoperatively is a valuable tool in obstructed vaginal septum cases, and management can often be performed as a single-stage vaginoplasty. REFERENCES 1. Cooper A, Merritt D. Vulvovaginal and Müllerian anomalies. In: Kliegman R, ed. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier; 2011:1874e1–1874e6. 2. Chang A, Siegel C, Moley K, et al. Septate uterus with cervical duplication and longitudinal vaginal septum: a report of five new cases. Fertil Steril 2004;81(4):1133–1136.
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Female Pelvic Medicine & Reconstructive Surgery • Volume 21, Number 3, May/June 2015
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3. Smith N, Laufer M. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril 2007; 87(4):918–922.
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4. Zurawin RK, Dietrich JE, Heard MJ, et al. Didelphic uterus and obstructed hemivagina with renal agenesis: case report and review of the literature. J Pediatr Adolesc Gynecol 2004;17(2):137–141.
7. Vercellini P, Daguati R, Somigliana E, et al. Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review. Fertil Steril 2007;87(4):719–724.
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Copyright © 2015 Wolters Kluwer Health | Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
