Study Type – Therapy (case series)
Level of Evidence 4
Study Type – Therapy (case series)
Level of Evidence 4
• To apply the technique of pelvic osteotomy to a select group of adult female patients with exstrophy, in view of the low complication rate and acceptable success rate of the technique.
• We applied this technique as an adjunct to uterovaginal suspension and abdominal wall repair after our prior success in treating patients with extreme pelvic diastasis.
• Bilateral innominate (transverse) and vertical iliac osteotomies were done from an anterior approach and an external fixator was placed. The fixator and pelvic bones were gradually cranked together over a period of 2–3 weeks until the diastasis was less than 4 cm.
• Then sacrocolpopexy was performed with revision of the abdominal wall and revision genitoplasty along with the placement of an intrasymphyseal titanium plate.
• Six patients presented with uterine/vaginal prolapse and concerns about the appearance of their abdominal wall and genitalia. Three patients had undergone uterine suspension before and one patient had had five prior attempts at suspension, which failed. The mean (range) age was 22.3 (18–26) years.
• All the patients underwent staged reduction of pubic diastasis and sacrocolpopexy along with revision of the abdominal wall and in four cases revision genitoplasty was also performed. The mean (range) diastasis was 12.8 (8–18) cm and 2.8 (3–4) cm before and after staged reduction respectively.
• Complications included transient femoral nerve palsy (two) and foot drop secondary to sciatic nerve stretch (one), which resolved with time. The symphyseal plate needed to be removed in three patients: secondary to persistent pain at the site (one), persistent discharge from the lower abdominal wound (one) and erosion into the anterior wall of the vagina (one).
• At a mean (range) follow-up of 60.7 (2–137) months the cosmetic and functional outcome of the abdominal wall reconstruction and genitoplasty was good with all the patients being satisfied. Five patients are currently sexually active and none has had a recurrence of their prolapse. None has become pregnant yet.
• Although the morbidity of this procedure in the adult is not insignificant, it is a valuable adjunct to pelvic floor reconstruction in young women of childbearing age even in those who have had prior suspension procedures.
• In addition, it allows the movement of lateral previously unoperated skin into the midline, allowing scar and skin replacement with healthier more vascularized tissue.
The management of classical bladder exstrophy in adolescent and adult patients is complex. Most of these patients come with complications and a significant diastasis of the pubic bones. Most have an extreme diastasis (>6 cm) and the approximation may not be possible in a single step [1–6]. These patients often have a history of multiple failed closures in early childhood, carry an ugly abdominal wall scar, and have herniation, unattractive external genitalia and recurrent pelvic organ prolapse [5,7,8]. The prolapse is secondary to bony changes in exstrophy that, along with the abnormality in pelvic floor musculature, predisposes them to prolapse [9–11]. Osteotomy and pubic approximation takes the tension of the closure and allows better restoration of pelvic floor anatomy, thus decreasing the risk of bladder and uterine/vaginal prolapse [1–6,12–15]. With extreme bony diastasis combined transverse anterior innominate and posterior iliac osteotomy with external fixation and gradual pubic approximation can be of benefit [1–4]. We present our experience with the technique for the treatment of adult female patients with complex exstrophy.
These patients came to our unit in early adulthood with uterine/vaginal prolapse and/or concerns about the unattractive nature of their abdominal wall and external genitalia (Table 1). With prior success with our technique of staged pelvic closure in treating patients with extreme pelvic diastasis, we decided to try the technique as an adjunct to uterovaginal suspension and abdominal wall repair . The technique has been described previously [1–4]. Anterior innominate and vertical iliac osteotomies were performed through anterior inguinal incisions. Two interfragmentary (5 mm) pins were placed in the superior segment and two in the inferior segment and the patient was placed in an external fixator with modified Buck’s traction (Fig. 1a). The pelvic bones were gradually cranked together at the bedside under appropriate sedation and analgesia over a period of 2–3 weeks until the diastasis was less than 4 cm (Fig. 1b). At this time sacrocolpopexy was performed along with revision of the abdominal wall and revision genitoplasty. The patients remained in traction for 6–8 weeks until good callous formation had occurred as identified by sequential radiographs.
A total of six patients presented with uterine/vaginal prolapse and/or concerns about the appearance of their abdominal wall and genitalia (Table 1). Of these, three patients had undergone uterine suspension before and one patient had had five prior attempts at suspension, which failed. The mean (range) age was 22.3 (18–26) years. The mean (range) diastasis before staged reduction was 12.8 (8–18) cm. All the patients underwent staged reduction of pubic diastasis and sacrocolpopexy along with revision of the abdominal wall, and in four cases revision genitoplasty was also performed. The revision genitoplasty included re-adaptation of bifid clitoris (clitoroplasty), labial reconstruction, monsplasty, introitoplasty and vaginoplasty.
The mean (range) diastasis after staged reduction was 2.8 (3–4) cm. Complications included transient femoral nerve palsy (two) and foot drop secondary to sciatic nerve stretch (one), which needed physiotherapy and resolved with time. In three patients the symphyseal plate that was placed at the time of repair needed to be removed because of persistent pain at the site (one) and persistent discharge from the lower abdominal wound (one), and in one patient the symphyseal plate had eroded into the anterior wall of the vagina. At a mean (range) follow-up of 60.7 (2–137) months the cosmetic and functional outcome of abdominal wall reconstruction and genitoplasty was good with all the patients being satisfied with the repair. Five patients are currently sexually active and none has had a recurrence of their prolapse. None has become pregnant yet.
Pelvic osteotomy is an integral part of the modern repair of complex classical and cloacal exstrophy cases [12,13,16]. The objectives of any such repair in the adult female are adequate pubic bone approximation, closure of the pelvic ring, realignment of the pelvic floor muscles to prevent internal genital organ prolapse, placement of the urethrovesical unit deep in the pelvis and a tension-free cosmetically appealing abdominal wall closure. To achieve these ideals various types of repairs and osteotomies have been described [17–20]. Despite these developments, many young female patients still continue to suffer with the pain of an ugly abdominal wall scar, huge wound hernias, unattractive external genitalia and prolapse of the uterus and vagina [5–8,12,15]. Multiple repairs for bladder reconstruction and attempts to achieve continence result in unattractive abdominal wall scars, which tend to weaken at each such attempt and further predispose to large hernias or even bladder prolapse. These multiple failed repairs often leave unattractive divergent mons with a midline bald escutcheon and a bifid clitoris. All six of our patients had large unattractive scars, large abdominal wall hernias and some degree of bladder prolapse, and four patients required revision genitoplasty to make them aesthetically acceptable.
Prolapse of the internal genital organs is a serious sequelae in post-pubertal girls with exstrophy, and the incidence has been reported as between 30% and 60%[5,7,8,15]. A wide diastasis, deficiency of bony segments and abnormal pelvic bony rotations result in abnormal distribution of pelvic floor muscles with a wide levator ani hiatus and abnormal positioning of the puborectalis sling. The anomaly produces a non-ellipsoidal, shallow and weak pelvis [9,10]. A lax round ligament and short anterior vaginal wall that brings the cervix close to the introitus further predisposes the uterus to prolapse [5,7,8,15]. The degree of prolapse is directly proportional to the extent of diastasis, the divergence of the pelvic floor muscles and the diameter of the pelvic diaphragm [9,10]. Two such patients had 14 and 18 cm of diastasis in our series. Prolapse is common after puberty, is commonly reported after correction of a narrow introitus and tends to occur during pregnancy [7,15]. This is why many authors advise preemptive suspension at the time of initial closure [15,21]. Unfortunately, most of the suspension procedures fail due to inadequate bony pelvic ring, the lack of adequate muscular support and a widened levator hiatus [7,8,15,21]. It is noteworthy that in one of our patients (patient number 4) five attempts at uterine suspension had failed. This may be one reason why occasionally even ablative procedures have been advised .
Aadalen et al.  used osteotomy for the first time in patients with exstrophy. The primary goal of osteotomy is to close the incomplete pelvic ring and in the past this has mostly been achieved through posterior iliac osteotomy [16,22]. As reported earlier the length of the anterior bony segment is nearly 30% shorter in these patients and may be more than that in cases of severe diastasis [9,10]. So, if the correction is to be done the entire pelvis needs to be over-rotated internally to correct the shortening of the anterior segment as well as for malrotation, which may not be possible with posterior iliac osteotomy alone in every patient . Thus posterior iliac osteotomy alone has been reported to fail in such situations [6,23]. It could not prevent uterovaginal prolapse in these patients and around 11% of the patients are reported to develop prolapse even after posterior iliac osteotomy . Today bilateral anterior osteotomies are the most common procedures [16,19,23]. In adults and adolescents one type of osteotomy alone may not be sufficient to take care of extreme diastasis. The bony pelvis is rigid with a wider diastasis [8–16 cm], and pelvic floor defect is much more complex with a shorter perimeter [4,6,9]. We have earlier reported our results of staged pelvic closure with combined bilateral transverse and vertical osteotomies in extreme diastasis in children [1–4]. We found that this approach restores the pelvis anatomy more completely with minimal postoperative migration of the pubic bone segments. The same procedure was applied in these adult female patients. The staged procedure allows easy approximation of the pubic bones, hence narrowing of the true pelvis to the smallest anteroposterior diameter. It realigns the pelvic floor muscles more efficiently; it recruits the puborectalis sling and the pubococcygeous and ischiococcygeous muscles and gets them back close to the midline where they should ideally be located so they can support the midline structures [1–4,7,20]. Three of our six patients had recurrent uterine/vaginal prolapse and all were successfully taken care of with this approach.
Some of the patients had problems in the postoperative phase and some of them needed interventions. The neurological complications – transient femoral nerve palsy and foot drop – resolved without any residual deformity. Complications related to the symphyseal plate were managed successfully without any further morbidity. Although complications can occur the benefits and overall satisfactory outcome definitely outweigh these known complications .
Staged reduction of pubic diastasis is beneficial for revising the closure in adolescents and adult patients with exstrophy with huge abdominal wall defects, unattractive genitalia and recurrent uterine/vaginal prolapse. While the morbidity of this procedure in the adult is not insignificant, it is a valuable adjunct to pelvic reconstruction in young women of childbearing age even in those who have had prior suspension procedures. In addition, it allows the movement of lateral previously unoperated skin into the midline allowing scar and skin replacement with healthier more vascularized tissue. However, this should only be performed in a centre where expertise in advanced osteotomy exists and where surgeons are familiar with the pelvic floor anatomy in exstrophy.