Total Laparoscopic Hysterectomy in a Patient with Cervicovaginal Agenesis

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Video Description

In this video, we present a case of total laparoscopic hysterectomy in a patient with cervical agenesis. Our objectives are to review the presentation and management of cervical agenesis and to highlight the keys to hysterectomy in this population. Although cervical agenesis is rare, with an incidence of 1 in 80,000 to 100,000, the surgical techniques shown in this video are helpful in all cases where the placement of a uterine manipulator is not possible. The case is of a transmale with a unicornuate uterus, cervicovaginal agenesis, and a large hematosalpinx and endometrioma. The keys to hysterectomy in these patients are obtaining detailed preoperative imaging to determine the presence of associated anomalies, consistent upward traction on the uterus, and careful delineation of the caudal border of the uterus. With these keys in mind, the viewer will be better prepared to approach hysterectomy in patients with uterine anomalies.

Presented By

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Dr. Peter Thiel

Affiliations

Mount Sinai & Women’s College Hospital

University of Toronto

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What is Total Laparoscopic Hysterectomy in a Patient with Cervicovaginal Agenesis?

A total laparoscopic hysterectomy (TLH) in a patient with cervicovaginal agenesis is a specialized surgical procedure performed to remove the uterus in individuals who are born without a fully developed cervix and vagina (cervicovaginal agenesis). Here’s an overview of this unique approach:

  • Condition Overview: Cervicovaginal agenesis is a congenital condition where the lower reproductive tract (cervix and vagina) is either absent or underdeveloped. Patients may experience menstrual difficulties, pain, or reproductive challenges.

  • Laparoscopic Technique: TLH uses minimally invasive laparoscopic techniques, where small incisions allow for the insertion of a camera and surgical instruments. This approach offers the benefits of reduced scarring, shorter recovery times, and lower infection risks compared to open surgery.

  • Procedure Adjustments for Agenesis: Due to the anatomical differences in patients with cervicovaginal agenesis, surgeons make adjustments to access and remove the uterus effectively. The procedure may require careful navigation of altered anatomy and specialized tools to ensure safety and accuracy.

This approach allows for effective removal of the uterus while minimizing recovery time and post-operative complications.

What are the Risks of Total Laparoscopic Hysterectomy in a Patient with Cervicovaginal Agenesis?

Performing a total laparoscopic hysterectomy (TLH) in a patient with cervicovaginal agenesis involves unique risks due to the anatomical complexities associated with this condition. Here are the primary risks:

  • Injury to Surrounding Organs: The altered anatomy in cervicovaginal agenesis can make it challenging to identify and protect nearby structures, such as the bladder, ureters, and intestines, increasing the risk of accidental injury.

  • Anesthesia Risks: As with any surgery, anesthesia carries potential complications, including respiratory issues, allergic reactions, or adverse responses to medications.

  • Increased Surgical Complexity: With cervicovaginal agenesis, typical surgical landmarks may be missing or repositioned, which can complicate the procedure, requiring advanced laparoscopic skills and potentially longer operative times.

  • Infection and Bleeding: Although laparoscopic surgery generally has a lower infection rate, the risk still exists, especially given the challenges of working in a complex anatomical field. Blood loss can also be more difficult to manage in these cases.

  • Postoperative Adhesions and Pain: Scar tissue, or adhesions, may form around the surgical site, which can cause chronic pain or discomfort, potentially affecting other abdominal organs.

  • Conversion to Open Surgery: Due to the challenges of cervicovaginal agenesis, there may be a need to switch to an open abdominal approach if complications arise, which would extend recovery time and increase the risk of further complications.

Given these unique risks, performing TLH in patients with cervicovaginal agenesis requires careful preoperative planning, experienced surgical teams, and a clear understanding of the patient’s altered anatomy to minimize potential complications.

Video Transcript: Total Laparoscopic Hysterectomy in a Patient with Cervicovaginal Agenesis

Total laparoscopic hysterectomy in a patient with cervicovaginal agenesis. We have no disclosures to report.

The objective of this video is to review the presentation and management of cervical agenesis and to highlight the keys to laparoscopic hysterectomy in these patients.

Cervical agenesis is rare, comprising about 3% of all uterine malformations. Around 50% of cases are associated with complete or partial vaginal agenesis, and neurological anomalies are present in 10 to 20% of patients.

Agenesis occurs through multiple mechanisms, including failures of uterine elongation, local atrophy, and failed canalization. There exists a wide range of phenotypes, from complete agenesis to milder forms as depicted here. It is termed dysgenesis, if any cervical tissue is present.

Patients present with primary amenorrhoea and cyclical abdominal pain due to outflow tract obstruction. This obstruction also increases the risk of endometriosis.

The initial management is with menstrual suppression to prevent ongoing accumulation of blood products. This gives time for imaging and informed decision making. Accurate imaging is imperative to determine the presence of a vagina and the exact type of cervical anomaly.

Surgical options include dilation with or without creation of a neovagina, a range of uterine conserving procedures aimed at creating a functional outflow tract, or hysterectomy. Conservative options are associated with a 90% menstruation rate and 10% live birth rate.

Our case is of a trans male patient with cervicovaginal agenesis, and progressively worsening abdominal pain and distension. He desired definitive management with a hysterectomy.

Preoperative MRI revealed a left-sided unicornuate uterus, and hematometra with a large left adnexal lesion consistent with a hematosalpinx. This sagittal section allows for appreciation of the hematometra and absent cervix. Here we see the bladder and rectum and can note the absence of a vagina between these structures. The absence of a hematocolpos further supports the diagnosis of vaginal agenesis.

The keys to hysterectomy in patients with cervical agenesis are detailed preoperative imaging to assess for associated anomalies and the presence of a vagina, strong upward traction from your assistant, and clear delineation of the caudal margin of resection.

Initial views were consistent with imaging, showing a left-sided endometrioma and hematosalpinx. Not seen on preoperative imaging is a small right non-communicating horn identified here.

Adhesiolysis and drainage of the endometrioma allows for better visualisation of the anatomy, where we can now clearly see the unicornuate uterus to the left and the right-sided horn.

We begin on the left, where the round ligament is grasped and divided to gain access into the retroperitoneal space. Dissection here allows for a clear identification of the left ureter. A window is made in the posterior leaf of the broad ligament to allow for safe ligation of the left IP ligament.

With the uterus on strong rightward traction, we began to lateralise the left ureter. The anterior peritoneum is then divided before turning attention back to the left lateral parametrium.

The left uterine artery and lateral parametrium are divided, and we can begin to appreciate the caudal limit of our dissection. Upward traction on the uterus is essential during these steps to both aid in dissection and to reduce the risk of ureteric injury.

After complete transection of the left lateral parametrium, the prominence of the caudal uterus can be appreciated.

We then turn our attention to the right where the round ligament is first transected, before freeing the rudimentary horn from the mesosalpinx and posterior broad ligament.

With access to the right lateral parametrium, we can now divide the right uterine artery and continue our dissection in a similar fashion to the left until the distal aspect of the uterus is seen.

We then began the amputation of the uterus on the left. With no manipulator in the uterus, strong upward traction is, again, imperative to avoid injury and to help delineate the plane between the uterus and the fibrotic tissue below.

We repeatedly re-evaluate this caudal limit to ensure our dissection remains on track. As demonstrated here, blunt dissection, is used in combination with advanced bipolar energy to develop the correct plane.

Millimetre by millimetre, the uterus is slowly freed from the underlying connective tissue. Here, we can see the benefit of traction as the caudal margin is well seen, along with the uterosacral ligaments.

The blind endo uterus is clearly seen after the hysterectomy is complete. Hemostasis was confirmed, and no suturing was required. The specimen was morcellated at the umbilicus.

To review. The keys to successful hysterectomy in patients with cervical agenesis include detailed imaging, upward uterine traction, and clear delineation of the caudal margin.