British Journal of Urology (1992), 7 0 , 6 7 8 4 8 2
01992 British Journal of Urology
Bladder Exstrophy and Anterior Pelvic Osteotomy S. PEROVIC, R. BRDAR and D. SCEPANOVlC Department of Paediatric Surgery and Urology, University Children's Hospital, Belgrade, Yugoslavia
Summary-Between August 1988 and December 1991,36 children with bladder exstrophy underwent surgery for primary bladder reconstruction. Each child was either untreated or had already been treated unsuccessfully. The operative technique involved bilateral osteotomy of the superior ramus of the pubic bone. In infants the cartilaginous ischiopubic junction, acting as an articulation, allowed symphyseal approximation, while in older children this was achieved by fracture of the inferior ramus of the pubic bone. The bladder was either closed or, in most cases, the exstrophic bladder plate was inserted deep into the pelvis, allowing subsequent epithelialisation of the bladder and further formation and growth. Follow-up up for 3.5 years showed bladder capacities of 40 to 150 ml. Some patients underwent an additional augmentation enterocystoplasty. Primary bladder reconstruction remained uncompromised in 7 patients who developed moderate (and 1 complete) rediastasis of the pubic bones. All exstrophic bladders are reconstructible, particularly in older children.
Primary reconstruction of bladder exstrophy with or without posterior iliac osteotomy has not given satisfactory results in our patients, and in older children the results were particularly poor. Thus we found the resports by Frey and Cohen (1988,1989) of great interest as they indicated a completely new approach to primary reconstruction of bladder exstrophy with the promise of more successful results. We began to use the technique shortly after publication of the first article and it is now our method of choice for primary reconstruction of bladder exstrophy. Patients and Methods
Thirty-six patients, aged from 3 months to 12 years (27 male and 9 female), with bladder exstrophy were treated by anterior pelvic osteotomy between August 1988 and December 1991. The indications for treatment were (1) previously failed bladder reconstruction (one or more operations) (n =24), (2) untreated exstrophy (n =9), (3) ~~
Presented at the Third Annual Meeting of the European Society of Paediatric Urology in Cambridge, 2&22 March 1992
bladder neck reconstruction in conjunction with epispadias repair (n =2) and (4) cloaca1 exstrophy (n = 1). The only contraindication was hypoplasia, i.e. pubic bone aplasia. However, in a patient with unilateral hypoplasia of the superior ramus of the pubic bone anterior osteotomy was nevertheless successful. Operative procedure
Mobilisation of the exstrophic bladder with wide mobilisation of the abdominal skin with subcutaneous tissue, and formation of the para-exstrophy skin flaps, formed the first stage of the procedure. Closure of the bladder, followed by reconstruction of the bladder neck and proximal urethra, comprised the next step but this was feasible in only 4 cases. It was not possible in the remaining 30 patients because of a small bladder plate and hypertrophied or polypoid bladder mucosa. In such circumstances the bladder plate was inserted deep into the pelvis, while only the bladder neck and proximal urethra were reconstructed by using the para-exstrophy skin flaps. The initial lengthening of the penis during primary reconstruction of the bladder facilitates several procedures : mobilisation
678
BLADDER EXSTROPHY AND ANTERIOR PELVIC OSTEOTOMY
of the corpora cavernosa up to their attachment to the pubic bones ; separation of the neurovascular bundle from the corpora cavernosa; cutting of the urethral plate and its partially proximal mobilisation from the corpora cavernosa; elongation of the epispadiac urethra by para-exstrophy skin flaps; approximation of the corpora cavernosa due to anterior pelvic osteotomy and symphyseal approximation. Osteotomy of the superior ramus of the pubic bone is the key to success in primary reconstruction of the exstrophic bladder. It is performed in the space between the adductor and pectineal muscles. Osseous retractors are inserted into the obturator foramen to protect the obturator nerve and blood vessels. Osteotomy is most often performed with an osseous chisel or a wire saw. Symphyseal approximation is possible in infants because the cartilaginous ischiopubic junction acts as an articulation. In older children the inferior ramus of the pubic bones is fractured during approximation of the superior ramus, thus enabling almost tension-free symphyseal approximation. Unless the inferior ramus of the pubic bones is fractured, symphyseal approximation is under considerable tension and there is a permanent tendency to diastasis. Symphyseal approximation causes a defect between the osteotomised superior ramus of the pubic bones. It may remain as such or may be bridged by a bone transplant, which was performed in 2 cases. A combination of wires, bolts and plates is used for
679
osteosynthesis, which is easilyperformed and highly stable in older children owing to the strength and structure of their bones. In neonates and infants osteosynthesis is more difficult to perform and is less stable. After symphyseal osteosynthesis, adaptation and suture of the rectal muscles and abdominal wall soft tissue are performed mostly without tension. When bladder exstrophy is associated with cryptorchidism, orchiopexy is performed at the same time (Figs 1 and 2).
Fig. 2 Aspect after bilateral osteotomy of superior ramus of the pubic bone and osteosynthesis following complete symphyseal adaptation.
Fig. 1 Schematic representation of anterior pelvic osteotomy by Frey and Cohen (1989). Prior to osteotomy the inguinal ligament, pectineal, adductor and rectal muscles must be identified. The arrow indicates the site of osteotomy between the adductor and pectineal muscles.
Fig. 3 Aspect in 5-year-old boy following several failed attempts at primary bladder reconstruction; small bladder plate with hypertrophic mucosa. Symphysealdiastasis is 10 cm.
680
BRITISH JOURNAL OF UROLOGY
Fig. 4 (A) Plain X-ray showing symphysealdiastasis. (B) Plain X-ray after anterior pelvic osteotomy.
Urinary diversion is maintained by urethral splints or directly from the bladder for the next 2 to 3 weeks, and wound drainage for 1 week. Immobilisation is maintained for 4 weeks and walking is allowed after 6 weeks. Antibiotic therapy is given for 3 weeks.
with augmentation enterocystoplasty had been performed in another hospital) (Figs 3-6). The following complications were noted : bleeding from the obturator veins developed during surgery in 2 patients and was successfully treated; 3 patients developed partial wound dehiscence with formation of a vesico-cutaneousfistula which closed
Results The patients have been followed up for 3 years and 5 months. In all cases a new bladder with a capacity of 40 to 150 ml was formed. When the bladder plate was placed in the pelvis, the new bladder was formed by secondary epithelialisation. Histologically, newly formed bladder was seen on the anterior wall, with hypotrophic muscle fibre surrounded by well developed connective tissue on the posterior wall and a marked muscular layer with some connective tissue. The small bladder capacity necessitated augmentation enterocystoplasty in 12 patients (Sigma or Mainz pouch) and the remaining patients await a similar procedure. Epispadias correction, as the final step in the management of the epispadias-exstrophy complex, was performed in 12 patients and continence was achieved in 8 without additional bladder neck reconstruction. A successful anti-incontinence procedure was achieved by combining the Young-Dees method with anterior pelvic osteotomy in caseS (Primary reconstruction of the bladder neck in conjunction
Fig. 5 Result following surgery. Bilateral orchiopexy was also performed because of cryptorchidism.
BLADDER EXSTROPHY AND ANTERIOR PELVIC OSTEOTOMY
68 1
have been substantially better than those obtained from other techniques (Jeffs et al., 1982; Mollard et al., 1986; Ransley et al., 1989; Snyder, 1990). A 3year follow-up period has proved adequate to assess the efficacy of the method. Anterior pelvic osteotomy allows symphyseal approximation even in most severe cases (up to 12 cm diastasis), with tension-free rectus muscle approximation and reconstruction of the anterior abdominal wall. The method is particularly successful when previous surgery has failed, with or without polypoid bladder mucosa. Burying the bladder in the pelvis allows the formation of a new bladder by secondary Discussion epithelialisation developing from the bladder plate. The results achieved by primary reconstruction of In older children the procedure is easy because of bladder exstrophy using anterior pelvic osteotomy the strength and structure of their bones. Fracture
spontaneously; complete symphyseal rediastasis was noted in only 1 patient, while moderate rediastasis occurred in 7. Symphyseal rediastasis did not affect the final result of reconstruction of the bladder and anterior abdominal wall. A stable pelvic ring without compromised gait was achieved in all patients. Four children developed a hernia; in 1 child this was bilateral, resulting from a defect in the reconstruction of the inguinal part of the anterior abdominal wall. There were no cases of public bone osteitis.
Fig. 6 (A) Micturating cystourethrogram (side view) 1 year after primary bladder reconstructionshowing newly formed bladder created by secondary epithelialisation of bladder plate buried deep in the pelvis. New bladder capacity is 80 ml. Bilateral vesicouretericreflux. Angulationof the neck and proximalurethrapositionedbelow the symphysis. (B) Micturatingcystourethrogram shows good bladder capacity following bladder augmentation (Sigma pouch).
682
of the inferior ramus of the pubic bones, following symphyseal approximation, is the key to tensionfree approximation. In infants this leads to the formation of a cartilaginous ischiopubic junction, although this is continually under high tension. Rediastasis of the pubic bones often occurs in infants but does not compromise the method, and approximation of the anterior abdominal wall and formation of a new bladder were achieved in all cases. In our experience, the results of posterior pelvic osteotomy are unsatisfactory because most patients developed rediastasis, wound dehiscence and prolapse of the exstrophic bladder. Pelvic ring reconstruction permits angulation of the bladder neck and proximal part of the urethra, which represents one of the stages in the antiincontinence procedure. In addition to other procedures (mobilisation of the corpora cavernosa and neurovascular bundles, elongation of the urethral plate by para-exstrophy skin flaps, erc), approximation of the pubic bones permits elongation of the penis. The stability of the pelvic ring is good, gait remains uncompromised and the cosmetic appearance is satisfactory. Our experience shows that the defect between the osteotomised parts of the superior ramus of the pubic bones does not affect pelvic stability, thus making it unnecessary to bridge the defect with a bone transplant. The main disadvantage of the method is hernia, which may develop as the result of a large defect between the osteotomised parts of the superior ramus in the inguinal part of the anterior abdominal wall. Anterior pelvic osteotomy allows primary bladder reconstruction to be postponed until the child is between the first and the second year of life, when the best results can be achieved, and no gradation
BRITISH JOURNAL OF UROLOGY
in the degree of reconstructibility of the exstrophic bladder is necessary. Each exstrophic bladder is reconstructible, regardless of grade, if anterior pelvic osteotomy is used. Despite these satisfactory results, however, the search must continue for better solutions.
References Frey, P. and Cohen, S. J. (1988). Blasenextrophie-anteriore Osteotomie des Beckens-ine neue operative Technik zur Erleichterung der Stabilisierung des Beckens und Abdominalverschlusses. Z. Kinderchir., 43, 171-173. Frey, P. and Cohen, S. J. (1989). Anterior pelvic osteotomy. A new operative technique facilitating primary exstrophy closure. Br. J. Urol., 64,641443. Jeffs, R. D., Guice, S. L. and Oesch, I. (1982). The factors in successful exstrophy closure. J. Urol., 127,974-976. Mollard, P., Basset, T., Deseubis, M. et al. (1986). RBsultats de la reconstruction vksicale et urBtrale pour exstrophie. Chir. Pidiatr., 27,27-32. Ransley, P. G., Duffy, P. G . and Wollin, M. (1989). Bladder exstrophy closure and epispadias repair. In Operative Surgery-Pediatric Surgery, ed. Spitz, L. Pp. 627432. London: Butterworth. Snyder, H. M. (1990). The surgery of bladder exstrophy and epispadias. In Operative Paediatric Surgery, ed. Frank, D. J. and Johnston, J. H. Pp. 152-185. Edinburgh, London, Melbourne, New York: Churchill Livingstone.
The Authors S. PeroviC, MD, PhD, Professor of Paediatric Surgery and Urology; Head, University Children’s Surgical Hospital. R. Brdar, MD, Assistant Professor of Paediatric Surgery. D. SEepanoviC, MD, PhD, Assistant Professor of Paediatric Surgery.
Requests for reprints to: Professor S. PeroviC, Department of Paediatric Surgery and Urology, University Childrens’ Surgical Hospital, Tirsova 10, 11000 Belgrade, Yugoslavia.
01992 British Journal of Urology
Bladder Exstrophy and Anterior Pelvic Osteotomy S. PEROVIC, R. BRDAR and D. SCEPANOVlC Department of Paediatric Surgery and Urology, University Children's Hospital, Belgrade, Yugoslavia
Summary-Between August 1988 and December 1991,36 children with bladder exstrophy underwent surgery for primary bladder reconstruction. Each child was either untreated or had already been treated unsuccessfully. The operative technique involved bilateral osteotomy of the superior ramus of the pubic bone. In infants the cartilaginous ischiopubic junction, acting as an articulation, allowed symphyseal approximation, while in older children this was achieved by fracture of the inferior ramus of the pubic bone. The bladder was either closed or, in most cases, the exstrophic bladder plate was inserted deep into the pelvis, allowing subsequent epithelialisation of the bladder and further formation and growth. Follow-up up for 3.5 years showed bladder capacities of 40 to 150 ml. Some patients underwent an additional augmentation enterocystoplasty. Primary bladder reconstruction remained uncompromised in 7 patients who developed moderate (and 1 complete) rediastasis of the pubic bones. All exstrophic bladders are reconstructible, particularly in older children.
Primary reconstruction of bladder exstrophy with or without posterior iliac osteotomy has not given satisfactory results in our patients, and in older children the results were particularly poor. Thus we found the resports by Frey and Cohen (1988,1989) of great interest as they indicated a completely new approach to primary reconstruction of bladder exstrophy with the promise of more successful results. We began to use the technique shortly after publication of the first article and it is now our method of choice for primary reconstruction of bladder exstrophy. Patients and Methods
Thirty-six patients, aged from 3 months to 12 years (27 male and 9 female), with bladder exstrophy were treated by anterior pelvic osteotomy between August 1988 and December 1991. The indications for treatment were (1) previously failed bladder reconstruction (one or more operations) (n =24), (2) untreated exstrophy (n =9), (3) ~~
Presented at the Third Annual Meeting of the European Society of Paediatric Urology in Cambridge, 2&22 March 1992
bladder neck reconstruction in conjunction with epispadias repair (n =2) and (4) cloaca1 exstrophy (n = 1). The only contraindication was hypoplasia, i.e. pubic bone aplasia. However, in a patient with unilateral hypoplasia of the superior ramus of the pubic bone anterior osteotomy was nevertheless successful. Operative procedure
Mobilisation of the exstrophic bladder with wide mobilisation of the abdominal skin with subcutaneous tissue, and formation of the para-exstrophy skin flaps, formed the first stage of the procedure. Closure of the bladder, followed by reconstruction of the bladder neck and proximal urethra, comprised the next step but this was feasible in only 4 cases. It was not possible in the remaining 30 patients because of a small bladder plate and hypertrophied or polypoid bladder mucosa. In such circumstances the bladder plate was inserted deep into the pelvis, while only the bladder neck and proximal urethra were reconstructed by using the para-exstrophy skin flaps. The initial lengthening of the penis during primary reconstruction of the bladder facilitates several procedures : mobilisation
678
BLADDER EXSTROPHY AND ANTERIOR PELVIC OSTEOTOMY
of the corpora cavernosa up to their attachment to the pubic bones ; separation of the neurovascular bundle from the corpora cavernosa; cutting of the urethral plate and its partially proximal mobilisation from the corpora cavernosa; elongation of the epispadiac urethra by para-exstrophy skin flaps; approximation of the corpora cavernosa due to anterior pelvic osteotomy and symphyseal approximation. Osteotomy of the superior ramus of the pubic bone is the key to success in primary reconstruction of the exstrophic bladder. It is performed in the space between the adductor and pectineal muscles. Osseous retractors are inserted into the obturator foramen to protect the obturator nerve and blood vessels. Osteotomy is most often performed with an osseous chisel or a wire saw. Symphyseal approximation is possible in infants because the cartilaginous ischiopubic junction acts as an articulation. In older children the inferior ramus of the pubic bones is fractured during approximation of the superior ramus, thus enabling almost tension-free symphyseal approximation. Unless the inferior ramus of the pubic bones is fractured, symphyseal approximation is under considerable tension and there is a permanent tendency to diastasis. Symphyseal approximation causes a defect between the osteotomised superior ramus of the pubic bones. It may remain as such or may be bridged by a bone transplant, which was performed in 2 cases. A combination of wires, bolts and plates is used for
679
osteosynthesis, which is easilyperformed and highly stable in older children owing to the strength and structure of their bones. In neonates and infants osteosynthesis is more difficult to perform and is less stable. After symphyseal osteosynthesis, adaptation and suture of the rectal muscles and abdominal wall soft tissue are performed mostly without tension. When bladder exstrophy is associated with cryptorchidism, orchiopexy is performed at the same time (Figs 1 and 2).
Fig. 2 Aspect after bilateral osteotomy of superior ramus of the pubic bone and osteosynthesis following complete symphyseal adaptation.
Fig. 1 Schematic representation of anterior pelvic osteotomy by Frey and Cohen (1989). Prior to osteotomy the inguinal ligament, pectineal, adductor and rectal muscles must be identified. The arrow indicates the site of osteotomy between the adductor and pectineal muscles.
Fig. 3 Aspect in 5-year-old boy following several failed attempts at primary bladder reconstruction; small bladder plate with hypertrophic mucosa. Symphysealdiastasis is 10 cm.
680
BRITISH JOURNAL OF UROLOGY
Fig. 4 (A) Plain X-ray showing symphysealdiastasis. (B) Plain X-ray after anterior pelvic osteotomy.
Urinary diversion is maintained by urethral splints or directly from the bladder for the next 2 to 3 weeks, and wound drainage for 1 week. Immobilisation is maintained for 4 weeks and walking is allowed after 6 weeks. Antibiotic therapy is given for 3 weeks.
with augmentation enterocystoplasty had been performed in another hospital) (Figs 3-6). The following complications were noted : bleeding from the obturator veins developed during surgery in 2 patients and was successfully treated; 3 patients developed partial wound dehiscence with formation of a vesico-cutaneousfistula which closed
Results The patients have been followed up for 3 years and 5 months. In all cases a new bladder with a capacity of 40 to 150 ml was formed. When the bladder plate was placed in the pelvis, the new bladder was formed by secondary epithelialisation. Histologically, newly formed bladder was seen on the anterior wall, with hypotrophic muscle fibre surrounded by well developed connective tissue on the posterior wall and a marked muscular layer with some connective tissue. The small bladder capacity necessitated augmentation enterocystoplasty in 12 patients (Sigma or Mainz pouch) and the remaining patients await a similar procedure. Epispadias correction, as the final step in the management of the epispadias-exstrophy complex, was performed in 12 patients and continence was achieved in 8 without additional bladder neck reconstruction. A successful anti-incontinence procedure was achieved by combining the Young-Dees method with anterior pelvic osteotomy in caseS (Primary reconstruction of the bladder neck in conjunction
Fig. 5 Result following surgery. Bilateral orchiopexy was also performed because of cryptorchidism.
BLADDER EXSTROPHY AND ANTERIOR PELVIC OSTEOTOMY
68 1
have been substantially better than those obtained from other techniques (Jeffs et al., 1982; Mollard et al., 1986; Ransley et al., 1989; Snyder, 1990). A 3year follow-up period has proved adequate to assess the efficacy of the method. Anterior pelvic osteotomy allows symphyseal approximation even in most severe cases (up to 12 cm diastasis), with tension-free rectus muscle approximation and reconstruction of the anterior abdominal wall. The method is particularly successful when previous surgery has failed, with or without polypoid bladder mucosa. Burying the bladder in the pelvis allows the formation of a new bladder by secondary Discussion epithelialisation developing from the bladder plate. The results achieved by primary reconstruction of In older children the procedure is easy because of bladder exstrophy using anterior pelvic osteotomy the strength and structure of their bones. Fracture
spontaneously; complete symphyseal rediastasis was noted in only 1 patient, while moderate rediastasis occurred in 7. Symphyseal rediastasis did not affect the final result of reconstruction of the bladder and anterior abdominal wall. A stable pelvic ring without compromised gait was achieved in all patients. Four children developed a hernia; in 1 child this was bilateral, resulting from a defect in the reconstruction of the inguinal part of the anterior abdominal wall. There were no cases of public bone osteitis.
Fig. 6 (A) Micturating cystourethrogram (side view) 1 year after primary bladder reconstructionshowing newly formed bladder created by secondary epithelialisation of bladder plate buried deep in the pelvis. New bladder capacity is 80 ml. Bilateral vesicouretericreflux. Angulationof the neck and proximalurethrapositionedbelow the symphysis. (B) Micturatingcystourethrogram shows good bladder capacity following bladder augmentation (Sigma pouch).
682
of the inferior ramus of the pubic bones, following symphyseal approximation, is the key to tensionfree approximation. In infants this leads to the formation of a cartilaginous ischiopubic junction, although this is continually under high tension. Rediastasis of the pubic bones often occurs in infants but does not compromise the method, and approximation of the anterior abdominal wall and formation of a new bladder were achieved in all cases. In our experience, the results of posterior pelvic osteotomy are unsatisfactory because most patients developed rediastasis, wound dehiscence and prolapse of the exstrophic bladder. Pelvic ring reconstruction permits angulation of the bladder neck and proximal part of the urethra, which represents one of the stages in the antiincontinence procedure. In addition to other procedures (mobilisation of the corpora cavernosa and neurovascular bundles, elongation of the urethral plate by para-exstrophy skin flaps, erc), approximation of the pubic bones permits elongation of the penis. The stability of the pelvic ring is good, gait remains uncompromised and the cosmetic appearance is satisfactory. Our experience shows that the defect between the osteotomised parts of the superior ramus of the pubic bones does not affect pelvic stability, thus making it unnecessary to bridge the defect with a bone transplant. The main disadvantage of the method is hernia, which may develop as the result of a large defect between the osteotomised parts of the superior ramus in the inguinal part of the anterior abdominal wall. Anterior pelvic osteotomy allows primary bladder reconstruction to be postponed until the child is between the first and the second year of life, when the best results can be achieved, and no gradation
BRITISH JOURNAL OF UROLOGY
in the degree of reconstructibility of the exstrophic bladder is necessary. Each exstrophic bladder is reconstructible, regardless of grade, if anterior pelvic osteotomy is used. Despite these satisfactory results, however, the search must continue for better solutions.
References Frey, P. and Cohen, S. J. (1988). Blasenextrophie-anteriore Osteotomie des Beckens-ine neue operative Technik zur Erleichterung der Stabilisierung des Beckens und Abdominalverschlusses. Z. Kinderchir., 43, 171-173. Frey, P. and Cohen, S. J. (1989). Anterior pelvic osteotomy. A new operative technique facilitating primary exstrophy closure. Br. J. Urol., 64,641443. Jeffs, R. D., Guice, S. L. and Oesch, I. (1982). The factors in successful exstrophy closure. J. Urol., 127,974-976. Mollard, P., Basset, T., Deseubis, M. et al. (1986). RBsultats de la reconstruction vksicale et urBtrale pour exstrophie. Chir. Pidiatr., 27,27-32. Ransley, P. G., Duffy, P. G . and Wollin, M. (1989). Bladder exstrophy closure and epispadias repair. In Operative Surgery-Pediatric Surgery, ed. Spitz, L. Pp. 627432. London: Butterworth. Snyder, H. M. (1990). The surgery of bladder exstrophy and epispadias. In Operative Paediatric Surgery, ed. Frank, D. J. and Johnston, J. H. Pp. 152-185. Edinburgh, London, Melbourne, New York: Churchill Livingstone.
The Authors S. PeroviC, MD, PhD, Professor of Paediatric Surgery and Urology; Head, University Children’s Surgical Hospital. R. Brdar, MD, Assistant Professor of Paediatric Surgery. D. SEepanoviC, MD, PhD, Assistant Professor of Paediatric Surgery.
Requests for reprints to: Professor S. PeroviC, Department of Paediatric Surgery and Urology, University Childrens’ Surgical Hospital, Tirsova 10, 11000 Belgrade, Yugoslavia.
