Hum. Reprod. Advance Access originally published online on April 7, 2004
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Human Reproduction, Vol. 19, No. 5, 1204-1210,
May 2004
© 2004 European Society of Human Reproduction and Embryology
Successful end-to-end cervico-cervical anastomosis in a patient with congenital cervical fragmentation: Case report
1st Department of Obstetrics and Gynecology, Aristotle University of Thessaloniki, Greece
1 To whom correspondence should be addressed at: Tsimiski 51 Street, 546 23 Thessaloniki, Greece. e-mail: grimbi{at}med.auth.gr
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Abstract |
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Cervical agenesis or dysgenesis (fragmentation, fibrous cord and obstruction) is an extremely rare congenital anomaly. Conservative surgical approach to these patients involves uterovaginal anastomosis, cervical canalization, and cervical reconstruction. In failed conservative surgery, total hysterectomy is the treatment of choice. We report what we believe to be the first successful end-to-end cervico-cervical anastomosis of an unusual case of congenital cervical fragmentation. A 15 year old Caucasian female presented complaining of primary amenorrhoea and cyclic, monthly abdominal pain. At laparotomy, a symmetrical transverse disruption of the cervix in a central and distal part was found and an end-to-end cervico-cervical anastomosis of the two cervical fragments was performed with the use of a 16F Foley catheter as a stent into the endocervical canal. One month after surgery the patient had normal menses. We conclude that a thorough investigation of the patient with suspected cervical anomaly is necessary and conservative surgical treatment should be applied as a first-line treatment option. In the presence of functional and intact cervical segments, the reconstruction of the cervical canal with an end-to-end cervico-cervical anastomosis is feasible and effective.
Key words: cervical agenesis/cervical aplasia/cervical dysgenesis/cervical fragmentation/uterine malformations
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Introduction |
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Müllerian anomalies are not uncommon conditions, having a mean prevalence in the general population of
4% (Grimbizis et al., 2001
). The American Fertility Society (AFS) has classified these conditions, according to availability of treatment and fertility prognosis, into six major anatomic types. According to the AFS classification, type I includes all cases of hypoplasia/agenesis; congenital agenesis or dysgenesis of uterine cervix are further subclassified as type IB (American Fertility Society, 1988). Moreover, Rock et al. (1995) have organized cervical agenesis (or aplasia) and dysgenesis (or dysplasia) into four categories: cervical agenesis, cervical fragmentation, cervical fibrous cord and cervical obstruction.
The prevalence of Müllerian hypoplasia/agenesis (type I anomalies) seems to be low, representing 3% of uterine malformations or 0.1% of the general population (Grimbizis et al., 2001). The incidence of congenital agenesis or dysgenesis of the uterine cervix is even lower and mostly unknown and in half of the cases it is associated with vaginal aplasia (Fujimoto et al., 1997). Furthermore, the conservative surgical management of women with these malformations remains controversial (Deffarges et al., 2001) and total hysterectomy is still proposed as a treatment option (Fliegner and Pepperell, 1994; Rock et al., 1995).
A case of a young female with congenital cervical fragmentation is reported. The diagnosis and the subsequent conservative surgical management are discussed. To our knowledge, this is the first case report of successful surgical cervico-cervical anastomosis in a patient with congenital cervical fragmentation.
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Case report |
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A 15 year old female presented to the outpatient gynaecology clinic complaining of cyclic, monthly, lower abdominal pain and primary amenorrhoea. Development of secondary sex characteristics was normal (Tanner staging 4 and 5 for breast and pubis). The patient had a road accident 3 years ago when she underwent emergency exploratory laparotomy for rupture of mesenteric arteries. The medical and surgical history was otherwise normal. There was no history of in utero exposure to diethylstilbestrol. As the patient was virgin, a bimanual rectal examination was performed with normal findings. An ultrasound examination showed that uteri corpus and both ovaries were normal. A computed tomography scan sustained the presence of a uterus. Serum hormonal levels were within normal limits.
A combined laparoscopic and hysteroscopic approach was decided upon. In hysteroscopy, an endometrial cavity view was unable to be obtained. Although vagina, external cervical os and cervical canal appeared normal, the cervical canal ended abruptly and a transverse cervical diaphragm occluded the route. A hysteroscopic attempt to pass through the occluded cervix resulted in a false route and the intrusion of the hysteroscope into the uterovesical space. Using laparoscopy, the uterus had a normal appearance, both ovaries were normal, but there were moderate haematosalpinges bilaterally and extended endometriosis in the right pelvic fossa. Cauterization of pelvic endometriosis, adhesion lysis and salpingostomy was performed. Any further attempt to treat was abandoned at that time. The patient was discharged on GnRH analogues (leuprolide acetate, 3.75 mg, monthly injections) for 3 months to avoid retrograde menstruation.
At readmission, initial laparoscopy confirmed the above findings. Laparotomy was then performed under general anaesthesia in a semilithotomy position allowing both abdominal and vaginal approaches. At dissection of the anterior space (between bladder and the uterus) cervix was found, totally, disrupted into a central and distal part. Distal cervical part was in place attached to the vagina with Mackenrot and uterosacral ligaments. There was a transverse complete disruption of the cervix medially. Corpus uteri, isthmus uteri and the central part of the cervix were detached from the distal part of the cervix within the broad ligament in a medial plane. A surgical end-to-end anastomosis was decided upon and performed in the following steps: (i) surgical anatomical preparation of both cervical edges, (ii) sharp opening, patency check and dilation of the cervical canal distally and medially to the point of disruption, (iii) insertion of a 16F Foley catheter transvaginally through the distal cervical part and forward of the catheter into the endometrial cavity where it was anchored with 5 ml of fluid pumped into the catheter balloon, (iv) suturing of cervical edges to each other with the use of Vicryl absorbable suture No. 3.0, (v) suturing of anterior and posterior uterovaginal/rectovaginal vault (Figures 1 and 2). The length of the remaining cervix was 3 cm. The procedure was uneventful. Antibiotics were administered postoperatively. The Foley catheter remained in uterine cavity for 9 days. Initial ultrasonographic transabdominal imaging of the uterus showed adequate fixation of the two cervical parts to each other (Figure 3). There were no postoperative complications.
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The patient was discharged on the pill. A month later she had normal menses.
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Discussion |
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To the best of our knowledge, this is the first case of successful end-to-end cervico-cervical anastomosis of a congenital cervical fragmentation reported in the literature. According to the American Fertility Society (1988
) this malformation should be classified as Class IB, together with the rest of congenital agenesis or dysgenesis of the uterine cervix. The presence of an intact upper female genital tract (corpus uteri, tubes and ovaries) in such a case imposes an attempt to restore normality. The potential reconstruction of the integrity of the uterovaginal route and the possibility of conception after such an operation pose a unique therapeutic challenge. In the presented case, the cervix was disrupted in a symmetrical way in a transverse level into a central and a lower cervical segment. Any further abnormality of the urogenital tract was not found. Initially it was thought that cervical disruption was the permanent result of the major pelvic trauma reported from the history of the patient. However, the operative findings did not confirm this notion. First, both cervical edges were smooth and clear of scarred tissue. Any traumatic dissection of uterus would result in asymmetrical fragments attached densely to the surrounding tissues. Second, no adhesions were found in the pouch of Douglas at the level of disruption. Common surgical sense suggests that major pelvic trauma would result in extensive intraperitoneal adhesions at the traumatic level.
Transverse cervical defects (AFS class IB) could be sub-classified into cervical agenesis (or aplasia) and dysgenesis (or dysplasia). Since 1900, 116 cases of transverse cervical defects have been reported in the literature (Tables I and II). Cervical agenesis is often associated with upper or complete vaginal aplasia (in 60 out of 83 reported cases). On the other hand, vaginal aplasia is an unusual finding in cases of cervical dysgenesis (in 1 out of 33 cases). According to Rock et al. (1995), the group of cervical dysgenesis could be further divided into: (i) cervical fragmentation (six cases including this case report), (ii) fibrous cervical cord where the cervical stroma has been substituted by a fibrous cord (12 cases), and (iii) in cervical obstruction (15 cases) (Rock et al., 1995) (Tables I and II).
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Patients with cervical agenesis or dysgenesis associated with or without vaginal aplasia have serious medical problems, whereas their fertility potential is absent. Obstructive Müllerian anomalies have a detrimental effect on the condition of pelvic organs associated with pelvic pain (Jacob and Griffin, 1989
; Deffarges et al., 2001). Pelvic endometriosis often complicates obstructive Müllerian anomalies (Tables I and II) and it has a further detrimental effect on the anatomical integrity and the fertility potential of the woman. Hydrosalpinges, firm adhesions, ovarian endometriotic cysts, and frozen pelvis due to endometriosis in neglected cases, combined in many of these patients with the coexistence of further Müllerian abnormalities, do not offer a favourable prognosis in these cases (Jacob and Griffin, 1989; Deffarges et al., 2001). Finally, these patients already have compromised fertility, if any.
Surgical treatment is therefore necessary. Conservative surgical approach, aiming at the restoration of the anatomical integrity of the female genital tract, presents a permanent challenge. Even in the presence of severely damaged tubes, the restoration of the uterovaginal route and preservation of the uterus offers the possibility of applying IVF for achieving a pregnancy (Anttila et al., 1999).
In cases of cervical dysgenesis, where a part or the whole cervix with mucus is present, the operation consists of a straightforward anastomosis or canalization as, theoretically, the preservation of a functional cervical canal with cervical mucus production will protect from ascending infections. Anastomosis is the restoration of the genital tract with direct suturing of distal and proximal cervical segments. This can be done only if intact and functional cervical segments are present as in this case. Canalization is the direct drilling and sounding of the cervix with insertion of a stent. Canalization has been proposed as a main treatment option in cases of cervical obstruction, but it is also applied in cases of cervical fibrous cord or cervical agenesis (Geary and Weed, 1973; Farber and Marchant, 1975; Niver et al., 1980; Helbing, 1983; Welker et al., 1988; Rock et al., 1995; Fujimoto et al., 1997; Letterie, 1998; Hovsepian et al., 1999; Lee et al., 2001; Bugmann et al., 2002; Wu et al., 2002). However, this technique has been complicated with deadly peritonitis, high failure rates and poor functional results (Geary and Weed, 1973; Farber and Marchant, 1976; Niver et al., 1980; Fujimoto et al., 1997; Casey and Laufer, 1997).
In cases of cervical agenesis or cervical dysgenesis, where the cervix is absent or there is no functional cervical part (cervical fragmentation/fibrous cord), uterovaginal anastomosis and cervical reconstruction (creation of neocervix) have been proposed as treatment options. In patients with coexistent vaginal aplasia, the creation of a neovagina is necessary. Uterovaginal anastomosis is the restoration of the genital tract by direct suturing of the isthmus uteri to the vagina. Cervical reconstruction is, practically, an attempt to restore the functional anatomy of the genital tract with the creation of a neocervix. Cervical reconstruction involves the use of grafts for the repair of the cervix such as slim thickness skin grafts (Potter, 1958, quoted from Jacob and Giffin, 1989; Cukier, 1986), full thickness skin grafts (Rock et al. 1995; Lee et al., 2001), bladder mucosa graft (Bugmann et al., 2002), or saphenous vein graft (Helbing, 1983, quoted from Jacob and Giffin, 1989).
The overall success rate of these techniques is 60% (69 out of 116 cases) (Tables I and II) and there have been reported six successful pregnancies after restoration of the uterovaginal route (Rock et al., 1995). However, surgical correction in these cases is associated with a high incidence of complications and failure and an ideal technique is not yet found. It is not, thus, surprising that total hysterectomy still offers numerous benefits and is supported as a treatment option by several authors (Jacob and Griffin, 1989; Fliegner and Pepperell, 1994; Rock et al., 1995), especially in cases of cervical agenesis and fragmentation (32% overall hysterectomy rate, 37 out of 116 cases) (Rock et al., 1995). Hysterectomised patients will not experience further retrograde menstruation, adhesion formation or abdominal pain (Bugmann et al., 2002). Finally, the application of uterine surrogacy offers an alternative to form their own family.
The case presented in this report is unique regarding the type of malformation. The presence of a fragmented but intact cervix favored a therapeutic decision over a canalization procedure by end-to-end anastomosis of the cervical ends, instead of an uterovaginal anastomosis or cervical reconstruction. The procedure was performed with the use of a Foley catheter as a stent. The use of the Foley catheter was decided upon in order to avoid obstruction or stenosis of the anastomosis during the healing process and a minimum of 8 days is thought to be necessary for that purpose. The preoperative use of GnRH analogues seems to minimize the chronic pelvic infection related to endometriosis, offering improved surgical circumstances. On the other hand, in the other five cases of cervical fragmentation reported in the literature, the cervical fragments were not functional and an end-to end anastomosis was not feasible. Rock et al. (1995) failed to correct surgically their four cases of cervical fragmentation and they applied total hysterectomy as an initial therapeutic procedure in two cases and as a second operation in the other two cases after failed cervical reconstruction. The fifth case of uterine fragmentation was treated successfully with cervical reconstruction (Maliphant, 1948, quoted from Jacob and Griffin, 1989)
In conclusion, the successful conservative surgical management of an uncommon case of congenital cervical fragmentation is presented. The reconstruction of the cervical canal with an end-to-end cervico-cervical anastomosis and the subsequent restoration of menstrual cycle are described. Thorough investigation and conservative intervention seem to be the first line approach in such cases.
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Submitted on October 20, 2003; accepted on January 13, 2004.
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