Laparoscopic reconstructive management of cervical agenesis

Summary

Objective:
To asses the viability of laparoscopic management of cervical agenesis.

Design:
Case report.

Setting:
II. Gynaecology and Obstetrics Dpt. at the University Hospital of F.D. Roosevelt, Banská Bystrica, Slovakia.

Methodology:
Diagnostic management and laparoscopic technique.

Results:
Congenital agenesis of the cervix is a rare anomaly of the inner genital. It may be associated with vaginal aplasia and less frequently with other anomalies like an incomplete fusion of müllerian ducts. Hysterectomy was frequently used to resolve main symptoms with respect to frequent complications associated with reconstructive surgery. Recently we can see a comeback to reconstructive surgery as a result of new medical technologies (new antibiotics and antibacterial sutures). Laparoscopic surgery is considered to be the main approach. The treatment strategy is related to a detailed individual pre-surgery investigation. Success of the surgical treatment is measured by normal menstruation and restored fertility. In this article we report the laparoscopic approach in creating the uterovaginal anastomosis in 20-year old women with cervical agenesis and duplex uterine anomaly and review the treatment options.

Conclusion:
Laparoscopic treatment of cervical agenesis is eligible and might be considered as a first-line treatment option.

Key words:
cervical agenesis, uterine malformations, uterovaginal anastomosis, laparoscopic surgery, müllerian anomaly.

INTRODUCTION

Cervical agenesis is a rare müllerian anomaly combined with possible partial or complete vaginal aplasia and with renal anomalies. The absence of the cervix incidence is low, by expert estimate 1 : 1 in 80 000 to 100 000 births [11]. As per classification of congenital anomalies by The American Fertility Society (AFS 1988), the cervical agenesis is classified as a type Ib müllerian anomaly [2]. Clinical picture usually includes primary amenorrhea, cyclic pelvic pain and a presence of pelvic resistance related to hematometra, hematosalpinx or endometriosis. Chronic hematometra and retrograde menstruation result in endometriosis and inflammatory complications. The pain is related to cryptomenorrhea and endometriosis [6]. Uterus unicornis cum cornu rudimentario is classified as a failure in the fusion of the müllerian ducts. AFS type IIc is manifested by the contralateral rudimentary uterine horn without the endometrial cavity. Unlike other congenital defects the treatment of cervical agenesis is still controversial. The objective of the treatment is to ensure cyclic menstruation, to remove the pain and the management of infertility [6].

We describe the case of a 20-year old female with the rare combined congenital anomaly of the inner genital classified as a syndrome of incomplete duplication with the agenesis of the cervix. We discuss the possibilities of reconstructive surgery and its clinical and reproductive results. We assess the benefits of laparoscopy to the diagnostics and treatment of the findings induced by the cervical agenesis.

CASE REPORT

The patient was referred to our unit in 2009 with primary amenorrhea. Based on the initial clinical, sonographic and laparoscopic examinations we made a diagnosis of uterus unicornis cum cornu rudimentario, hypoplasio vaginae non comunicans.

On examination the patient had normal secondary sexual characteristics, blind-ending vagina of proportionate size and a palpable mass in the lesser pelvis. It was not possible to palpate and differentiate the adnexa in detail. Subsequent transvaginal and transabdominal sonographic examinations confirmed the original diagnosis of uterus unicornis cum cornu rudimentario l. sin. non comunicans, agenesis cervicis uteri. Bilaterally differentiated ovaries were of adequate size and texture. Ultrasonography revealed no anomalies of the uropoetic system. The patient was approached with the proposal to perform reconstructive laparoscopic-vaginal surgery in order to create the uterovaginal canal. An additional magnetic resonance imaging scan was required before performing the surgery. The patient did not respond to the proposal.

Some 21 months later in 2011 the patient was again referred to our unit by a private gynaecologist with the acute pelvic pain and cystic tumor finding in the area of the right adnexa. Ultrasonography confirmed the growth of tumor from 40 mm to 57 mm during one month. The finding was demonstrated by magnetic resonance scans (fig. 1, 2, 3, 4). Clinical and sonographic examinations confirmed a fixed cystic tumor of the size 65x60 mm in the area of the right adnexa, with a screened content. The uterine cavity was dilated to 16 mm and filled with a fluid, identically screened content. As a part of a differential diagnosis we considered hematosalpinx or endometriotic cyst in view of previously diagnosed congenital defect of inner genital.

Persistent pelvic pain and clinical finding indicated a laparoscopic treatment. Laparoscopy confirmed the congenital anomaly of inner genital and endometriotic cyst at the right ovary. We also found a hypoplastic right uterine horn to which endometriotic cyst of given size adhered, with the induced adhesive periooforitis (fig. 5). From the area of the absent cervix a rudimentary horn came out to the left, communicating with the adnexa of normal appearance (fig. 6).

Laparoscopic surgery involved the complete salpingoovariolysis and enterolysis in order to restore the altered anatomic conditions in the area of the right adnexa. This was followed by adhesiolysis, a complete stripping of endometrioma, with hemostasis and ovary transfer (fig. 7, 8). At this point we decided to abandon the procedure because the situation was not convenient for the reconstructive surgery correcting the congenital defect. The reconstructive surgery was postponed until a period of a medication therapy. The application of analogs was aimed to inactivate the endometriosis and to reduce adhesions. Histology confirmed the endometriotic cyst without atypicalities.

The reconstructive surgery was planned for the period after the application of analogs when the endometrium was detectable by sonography. Considering our experience with the radical laparoscopic trachelectomy we opted for the laparoscopic--vaginal approach. The second laparoscopic intervention after the 6 months of medication assessed the therapeutic effect of analogs as satisfactory. Endometriotic score according to r-AFS dropped from 38 to 11 points. Minimal residuum of the peritoneal endometriosis and membranous adhesions were treated by lysis, exstirpation and bipolar vaporisation. Thereafter vesicovaginal septum was dissected and bladder partially liberated (fig. 9, 10). This procedure made the bottom part of the uterus accessible in the safe distance from the ureters.

Uterine corpus was then dissected with hooked scissors in the area of the anticipated lowermost portion of the endometrial cavity. The dissection was performed cranially until the uterine cavity was opened and the endometrium differentiated (fig. 11).

A probe was inserted vaginally to outline the apex of vagina before it was laparoscopically dissected. The opening thus created was further adjusted by fingers to the required lumen (fig. 12, 13).

The 2-0 vicryl sutures (polydioxanon Ethicon) were placed on the lowermost portion of the uterus and drawn into vagina and with these sutures we perform the uterovaginal anastomosis vaginally (fig. 14). The anastomosis was then finished laparoscopically using individual sutures (fig. 15). Thus created uterovaginal communication was not supported by any endoprosthesis. Operation was finalized by antiadhesive gel (Hyalobarrier gel, Nordic) applied laparoscopically and vaginally onto the anastomosis site. Final result is documented on fig. 16. The Foley catheter was placed in bladder deriving the clear urine.

Total operation time was 3 hours 10 minutes, with the measured blood loss 500 ml. The loss was not substituted by blood derivatives. Antibiotic and thromboembolic profylaxy and analgetics were applied. The patient made an uncomplicated recovery and was discharged on the 7th day following a sonographic examination. Antibacterials were applied vaginally and antianemics orally.

Post-operative examination was performed 4 weeks later. Patient reported first normal menstruation with minor dysmenorrhoea after the effect of analogs subsided. Vaginal examination established functional uterovaginal anastomosis with sutures partially absorbed. Ultrasonography revealed the uterine cavity undilated, ovaries finding was negative and cavum Douglasi contained minimum of exudate. Kidneys showed no swelling. Clinical outcome was subjectively and objectively assessed to be satisfactory.

DISCUSSION

Congenital cervical anomalies are rare defects of inner genital that are manifested in two basic modifications: 1) aplasia of cervix and 2) dysgenesis of cervix, with 4 subtypes described [10]. The associated anomalies of the urinary tract are rare, with few occurrences of a vaginal atresia of different scope. This anomaly is very rarely combined with congenital defect of the uterus. This situation is represented by our case

Management of congenital cervical agenesis was changing in the past years depending on the advances of medicine. Many authors recommend hysterectomy as the first-line treatment option for patients with functional endometrium and absenting cervix and vagina. Hysterectomy is a preferred option in cases with a fragmented cervix. It is obvious that hysterectomy eliminates main symptoms like cryptomenorrhoea, endometriosis and pelvic pain, however it brings about the irreversible loss of fertility. Hysterectomy also eliminates the risks associated with the recurring reconstructive surgical interventions and the possibility of sepsis as a result of the ascending infection. The decision between reconstructive surgery and hysterectomy with subsequent surrogate motherhood involves careful consideration of risks and benefits of both approaches [6].

Reproductive technologies enable fertility be treated with conservative, non-surgical techniques, however without the possibility to resolve the causal agent. In 2001 Lai et al. reported a successful pregnancy in 32 year old patient following the transmyometrial (Tawako technique) and transtubal embryotransfer (TET) when the reconstructive surgery failed. After the cesarean section they restored the uterovaginal communication and functionality was secured by an amniotic graft [8].

The probability of a spontaneous pregnancy after the reconstructive treatment is lower because of the presence of endometriosis and cervical factor of infertility [8]. Many re-canalization techniques aim to create a communication between the uterine cavity and vagina using various types of stents. Re-canalization enables cyclic menstruation, however the fibrous tissue with no cervical glands limits the chances of fertilization. Re-canalization techniques like drilling are simple to perform, however restenosis occurs in 40 to 60% [7]. The advanced laparoscopic techniques are currently replacing the laparotomic approach. Reported complications accompanying the re-canalization include endometritis, PID, persistent pelvic pain, lesions of bladder and bowel, re-blocking, recurrent surgical treatments, as well as deaths resulting from sepsis. The result of re-canalization depends on the size of the created canal, frequency of stenting, the presence of rudimentary cervical glands and other factors [5]. In the era of modern broad-spectrum antibiotics and antibacterial sutures we pursue a comeback to reconstructive treatment [9]. Epithelial grafts will secure the persistence of the newly created cervical canal [8, 9].

Forming the uterovaginal anastomosis after exstirpation of the obliterated ligament in place of the cervix is another alternative of a reconstructive procedure. The procedure basically imitates a trachelectomy in oncological patients. Some authors prefer hysterectomy for the purpose of a retrograde probe in the lowermost portion of the uterine cavity. The uterovaginal anastomosis assumes a circular suture of the vagina to the uterus at the level of the endometrium line. Both laparotomic and laparoscopic approaches are reported in the literature [1, 3, 5, 7, 9].

In 2006 Creighton et al. reported a case of laparoscopically performed uterovaginal anastomosis by means of hysterotomy and achieving normal menstruation [3]. In our case, considering our experience with the radical laparoscopic trachelectomy, we opted for the laparoscopic approach. Having dissected vesicovaginal septum and fixing the edges of the lower portion of uterus we opened the endometrial cavity with a progressive cranially directed blunt dissection. The anastomosis was performed vaginally and laparoscopically. Deffarges et al. reported outcomes of 18 cases of laparotomic uterovaginal anastomosis and observed that only 22 % of the patients required further surgery. Vaginal aplasia was found in one third of the patients. Author reported 6 spontaneous pregnancies in 4 of the patients [4]. Roberts et al. reviewed available surgical methods of management of the congenital cervical anomalies. In the cases of cervical agenesis with present vagina they specify reconstructive uterovaginal anastomosis [10]. The need of cerclage during gestation is controversial and gestations end with an elective cesarean [4].

CONCLUSION

A detailed preoperative investigation and a selection of an adequate strategy are the necessary conditions affecting the outcome of the surgical management of the congenital cervical agenesis. With proper decision the uterovaginal anastomosis should be considered as the first line treatment option. Reconstructive surgery should be performed in specialized units with a proper expertise in correcting the congenital defects of inner genital. Laparoscopic approach necessitates the techniques of the advanced laparoscopic suture and dissection. When above conditions are satisfied then laparoscopic approach becomes the first line treatment option with all the advantages of the mini invasive surgery [6, 7]. Bringing on the menstruation has a significant psychological effect in the development of a young woman. The reported case demonstrates the expertise level of our unit for the given type of the gynaecological surgery.

MUDr. Tibor Bielik, PhD., h. doc.

II. gynekologicko-pôrodnícka klinika SZU

FNsP F. D. Roosevelta

nám. L. Svobodu 1

974 01 Banská Bystrica

Slovenská republika

e-mail: tbielik@nspbb.sk