Endometriosis
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A Rare Case of a Secretory and Obstructed Uterine Horn Fistulized to an Ipsilateral Rudimentary Ureter

A Rare Case of a Secretory and Obstructed Uterine Horn Fistulized to an Ipsilateral Rudimentary Ureter
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Video Description

We would like to present a rare case of secretory and obstructed uterine horn fistulized to a rudimentary ureter managed by laparoscopic cornuectomy with concomitant resection of hemato-ureter and endometriosis.

Presented By

 

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Dr. Azelie Paulus
Dr Sarah Lacroix Maheux
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Dr. Kristina Arendas
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Dr. Madeleine Lemyre
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Dr. Phillippe Laberge

Affiliations

University Laval

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What is A Rare Case of a Secretory and Obstructed Uterine Horn Fistulized to an Ipsilateral Rudimentary Ureter?

This rare case involves a unique anatomical abnormality where a secretory and obstructed uterine horn develops a fistula, or abnormal connection, to an ipsilateral (same side) rudimentary ureter. Here’s a breakdown of this condition:

  • Secretory and Obstructed Uterine Horn: A uterine horn is a part of the uterus that, in certain congenital anomalies, can develop independently from the main uterus. When secretory, this horn produces menstrual-like fluid, but if it’s obstructed, the fluid has no natural outlet, causing pain, pressure, and risk of infection.

  • Fistula Formation: Due to increased pressure from fluid buildup, the obstructed uterine horn can form a fistula, an abnormal passageway, which in this case connects to a rudimentary ureter (an underdeveloped ureter that doesn’t fully connect to the kidney or bladder).

  • Ipsilateral Rudimentary Ureter: Since this rudimentary ureter is on the same side as the uterine horn, it provides a pathway for the trapped fluid to drain into the urinary system, potentially causing further complications like urinary tract infections or retrograde flow into the kidney.

  • Clinical Presentation and Diagnosis: Patients often present with cyclical pain, recurrent urinary infections, and sometimes abnormal urinary discharge. Diagnosis requires detailed imaging, such as MRI or ultrasound, to identify the uterine and urinary tract structures involved.

  • Surgical Management: Treatment usually involves surgical removal of the obstructed uterine horn and repair of the fistula, ensuring normal anatomy and function are preserved as much as possible.

This condition is exceptionally rare and presents significant diagnostic and surgical challenges, requiring a multidisciplinary approach to manage both the gynecologic and urologic aspects.

What are the Risks of A Rare Case of a Secretory and Obstructed Uterine Horn Fistulized to an Ipsilateral Rudimentary Ureter?

A rare case of a secretory and obstructed uterine horn fistulized to an ipsilateral rudimentary ureter presents several risks, given its complex anatomy and potential for serious complications:

  • Infection Risk: The fistula between the uterine horn and ureter creates a direct path for bacteria, increasing the risk of recurrent urinary tract infections (UTIs) and potentially severe pelvic or kidney infections.

  • Pain and Discomfort: Obstruction in the uterine horn leads to fluid buildup, causing significant cyclic pain and pressure, especially during menstruation. Over time, this can lead to chronic pelvic pain and discomfort.

  • Renal Complications: Retrograde flow of secretions from the uterine horn into the urinary tract can cause hydronephrosis (kidney swelling) or other kidney damage, especially if untreated. A rudimentary ureter also poses a risk for impaired kidney function on the affected side.

  • Diagnosis and Surgical Challenges: Identifying this condition requires highly detailed imaging, and surgery is complex due to the need for precision in removing the obstructed horn and repairing the fistula. The proximity of the abnormal structures to other organs increases the risk of injury to nearby tissues, like the bladder or main ureter.

  • Recurrence or Adhesion Formation: Surgery in this area may lead to scar tissue (adhesions), which can cause further pain, bowel obstruction, or, in rare cases, recurrence of symptoms if any remnants of the obstructed uterine horn are left behind.

Proper diagnosis and carefully planned surgical intervention are essential to minimize these risks and achieve a successful outcome.

Video Transcript: A Rare Case of a Secretory and Obstructed Uterine Horn Fistulized to an Ipsilateral Rudimentary Ureter

The minimally invasive gynaecology team of Laval University in Quebec presents a rare case of secretory and obstructed uterine horn, fistulized to a rudimentary ureter, managed by laparoscopic cornuectomy with concomitant resection of hemato-ureter and endometriosis. This video demonstrates the laparoscopic management of a severe malformation, with deep endometriosis.

This video describes the case of an 18-year-old who presented with severe pelvic pain, progressively worsening since menarche, associated with anaemia. Pre-operative findings on MRI were a secretory and obstructed rudimentary uterine horn, an ipsilateral fistula to a distending hemato-ureter, with absence of ipsilateral kidney and deep infiltrative endometriosis lesions.

A rudimentary horn is a rare Mullerian anomaly, representing about 5% of these anomalies. Renal and ureteral agenesis or hypoplasia, as well as endometriosis lesions, are commonly diagnosed with these conditions.

The patient underwent a laparoscopic cornuectomy, with concomitant resection of hemato-ureter, and endometriosis likely contributing to the pain symptoms. Before surgery, we use a GnRH agonist and add-back therapy in order to block menstruations.

The step-by-step surgical approach is presented. Hysteroscopy was performed to confirm the obstruction. Laparoscopy was performed to resect the obstructed structures and endometriosis.

During the hysteroscopy, there was one cervix leading to one cavity, with a single tubal ostium corresponding to the left tube. Initial exploration of the pelvis revealed a bicornuate uterus, with two horns and two fallopian tubes. The non-obstructed cavity is the one on the left. We decided on a dual approach, with hysteroscopy followed by laparoscopy to confirm the Mullerian anomaly and the obstruction.

We can see a voluminous right ureter, corresponding to the rudimentary hemato-ureter on imaging. Our communication with the left horn could be easily missed by the imaging. However, the hematometry let us suspect the obstruction was real. We decided to perform a cornuectomy for the right non-communicative horn. Here, we opened the entire leaf of the broad ligament revealing the hemato-ureter.

The next step is vasopressin injection into the fibromuscular band. A dilution of 30 units in 100 ccs of NaCl is used. A flexible laparoscopic syringe needle allows circumferential injection of vasopressin in order to decrease the risk of bleeding. A combined ultrasonic and bipolar energy device is used for the myometrial dissection. The obstructed cavity is reached, allowing us to visualise the endometrial tissue that needs to be fully resected, without compromising the myometrium from the remaining uterus.

Ureterolysis is meticulous up to the pelvic brim. After careful dissection, it appears to be connected to the rudimentary horn. The rudimentary ureter also needs to be removed because it is fistulized with the right horn and contains blood. After the ureterolysis is completed, a surgical clip is placed on the proximal rudimentary ureter, where it is no longer dilated. Here, the leak of old blood confirms the hemato-ureter.

After carefully removing the rudimentary ureter, the cornuectomy is continued. Again, we take care to remove all endometrial tissue to avoid entrapping secretory endometrium in the sutured myometrium. Later in the surgery, morcellation is performed to extract the surgical parts.

Careful exploration of the pelvis for endometriosis lesions was performed at the beginning of surgery. Complete excision of the identified lesions is important for pain management and future fertility. One lesion of the left uterosacral ligament was resected after carefully dissecting away the left ureter, being connected to her only kidney.

Hysteroscopy, concomitant to a laparoscopy, was performed in order to confirm an appropriate thickness of the myometrium at the site of cornuectomy and, again, absence of communication with the uterine cavity.

Given the absence of myometrial defects, the appropriate thickness of remaining myometrium and absence of communication with the uterine cavity, a strengthening X suture is applied at the site of cornuectomy, using an 0 monofilament. A cystoscopy was performed and confirmed there was no ureteral connection with the bladder on the right side. The left ureteral stream was well viewed.

In conclusion, this is a rare case of Mullerian anomaly that is fistulized into a rudimentary ureter. It presents a diagnostic and surgical challenge. Pre-operative imaging is crucial for Mullerian malformations. Renal and ureteral anomalies, as well as endometriosis lesions, are commonly diagnosed with these conditions. Careful pre-operative planning with a skilled surgical team is necessary for a successful complete excision of lesions and obstructed structures.

I would like to thank our entire team for their cooperation on this video. Thank you for watching.