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ORIGINAL ARTICLE
Year : 2013  |  Volume : 12  |  Issue : 4  |  Page : 212-216  

Exstrophy anomalies: Preliminary review of 18 cases in Maiduguri, North-eastern Nigeria


1 Department of Surgery, Paediatric Surgical Unit, University of Maiduguri Teaching Hospital, Maiduguri, Borno State, Nigeria
2 Department of Orthopaedic and Traumatology, University of Maiduguri Teaching Hospital, Maiduguri, Borno State, Nigeria
3 Department of Anaesthesia, University of Maiduguri Teaching Hospital, Maiduguri, Borno State, Nigeria
4 Department of Paediatrics, University of Maiduguri Teaching Hospital, Maiduguri, Borno State, Nigeria

Date of Web Publication4-Dec-2013

Correspondence Address:
J Y Chinda
Deeper Life Bible Church, PMB 516, Maiduguri, Borno State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1596-3519.122688

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   Abstract 

Background: Exstrophy anomalies, although rare, pose a tremendous challenge to both surgeons and surrogates. Management is intricate and experience with large members is restricted to a few centres worldwide. This report is a review of 18 cases of these anomalies managed in University of Maiduguri Teaching Hospital (UMTH) over 8 years, highlighting the intricacies of management, and contributes to the existing data bank on this subject matter in our environment.
Materials and Methods: We reviewed the records of all cases of anomalies that were managed in the index hospital from December 2002 to December 2010.
Results: Eighteen cases were studied. The youngest was a 2-day-old neonate and the oldest was 6 years at repair, with a median age of 4 months. There were 7 boys and 9 girls with bladder exstrophy, and the remaining two were girls with cloacal exstrophy, giving a male-female ratio of 7:11 for the exstrophy anomalies. Eight patients (33.5%) had associated congenital anomalies; 16.7% had undescended testes (UDT), anorectal malformation (ARM) and patent processus vaginalis (PPV); and duplicate bladder occurred in 5.6% of the patients. All but one patient had bilateral posterior iliac osteotomy at bladder closure. Out of the 17 patients that had osteotomy, 76.5% had satisfactory bladder closure (no complication), 5.9% had superficial wound dehiscence, 11.8% had bladder neck dehiscence and 5.9% had complete disruption. The lone patient that did not undergo osteotomy had complete bladder dehiscence. Postoperative immobilization was done for 3 weeks for 16 cases; one was discontinued after 1 week because of complication. The longest follow-up was for 2 years and the shortest for 2 weeks. Four patients (22.2%) did not turn up for follow-up.
Conclusion: Exstrophy anomalies although rare remain a major challenge in paediatric surgery. Iliac osteotomy, among other technical considerations, remains a cornerstone for successful bladder closure.

   Abstract in Spenish 

Résumé
Fond:
Exstrophie anomalies, bien que rare, posent un défi énorme pour les chirurgiens et les substituts. Gestion est complexe et expérience avec les gros membres est limitée à quelques centres dans le monde entier. Ce rapport est une étude de 18 cas de ces anomalies de gérée dans Université de Maiduguri Teaching Hospital (UMTH) plus de 8 ans, en soulignant la complexité de la gestion, et contribue à la Banque de données existante sur ce sujet dans notre environnement.
Matériel et Méthodes : Nous avons examiné les dossiers de tous les cas d'anomalies qui ont été gérés dans l'hôpital de l'indice de décembre 2002 à décembre 2010.
Résultats: Dix-huit cas ont été étudiés. Le plus jeune était un nouveau-né âgé de 2 jours et la plus ancienne était de 6 ans à la réparation, avec un âge médian de 4 mois. Il y avait sept garçons et 9 filles avec exstrophie vésicale, et les deux autres étaient des filles avec exstrophie cloacale, donnant un ratio mâle - femelle de 07:11 pour les anomalies de l'exstrophie. Huit patients (33,5 %) avaient associé à des anomalies congénitales ; 16,7 % cryptorchidie (UDT), malformation anorectale (ARM) et brevet de processus vaginalis (PPV); et en double vessie survenu dans 5,6 % des patients. Tous, sauf un patient avait bilatéraux ostéotomie iliaque postérieure à la fermeture de la vessie. Sur les 17 patients ayant une ostéotomie, 76,5 % eu fermeture vésicale satisfaisante (sans complication), 5,9 % avaient la déhiscence de la plaie superficielle, 11,8 % avaient de déhiscence de col de la vessie et 5,9 % avaient une désorganisation complète. Le seul patient qui n'a pas subi d'ostéotomie eu déhiscence complète de la vessie. Immobilisation postopératoire a été faite pendant 3 semaines pour les 16 cas ; l'un a été interrompu après 1 semaine à cause de la complication. Le suivi plus longs a été pendant 2 ans et le plus court pour 2 semaines. Quatre patients (22,2 %) n'a pas tourné vers le haut pour suivi.
Conclusion: Exstrophie anomalies rares cas restent un défi majeur en chirurgie pédiatrique. Ostéotomie iliaque, parmi d'autres considérations techniques, demeure une pierre angulaire pour la fermeture de la vessie réussie.
Mots-clés: Anomalies de l'exstrophie, nord-est du Nigéria

Keywords: Epispadias-Exstrophy complex, exstrophy anomalies, anterior abdominal wall defects


How to cite this article:
Chinda J Y, Abubakar A M, Dabkana T M, Kwari D, Mava Y, Bwala K J, Wabada S. Exstrophy anomalies: Preliminary review of 18 cases in Maiduguri, North-eastern Nigeria. Ann Afr Med 2013;12:212-6

How to cite this URL:
Chinda J Y, Abubakar A M, Dabkana T M, Kwari D, Mava Y, Bwala K J, Wabada S. Exstrophy anomalies: Preliminary review of 18 cases in Maiduguri, North-eastern Nigeria. Ann Afr Med [serial online] 2013 [cited 2020 Aug 5];12:212-6. Available from: http://www.annalsafrmed.org/text.asp?2013/12/4/212/122688


   Introduction Top


Exstrosphy anomalies are rare but potentially devastating and technically challenging. Bladder exstrophy, although the commonest sub-group of the epispadias-exstrophy complex, has considerable variability. [1] It is said to occur in one in every 40,000-50,000 live births with a male-female ratio of 2:1. [2]

Management of these anomalies requires intricate multi-stage reconstructive procedures, and considerable effort and patience on the part of the surrogates.

In our country, Nigeria, there is a paucity of literature on this intriguing anomaly. Hence it is imperative to review our experience from January 2002 to December 2010 to add to the existing literature.


   Materials and Methods Top


This is a retrospective review of exstrophy anomalies managed in University of Maiduguri Teaching Hospital (UMTH) among children 15 years and younger.

Data extracted from patient case notes, operation, and admission and discharge registers included name, sex, age, hospital number, associated anomalies, type of operation performed, and outcome of bladder closure and duration of follow-up. These were collected and analysed. Six cases whose case files could not be found were excluded from the study. We observed that the following steps were very critical and crucial to the success of exstrophy surgeries:

  1. Extra-peritoneal dissection of the bladder plate while taking care to protect the ureters
  2. Excision of the rim of skin from the plate to prevent the emergence of carcinoma later
  3. Placement of a suprapubic catheter, and bilateral ureteric and urethral stents
  4. Two-layered tubularization of the bladder plate
  5. Bilateral posterior iliac osteotomy and approximation of the pubic symphysis with nylon I
  6. Immobilization by gallow traction.



   Results Top


There were 16 cases of bladder exstrophy and two cases of cloacal exstrophy that were managed over an 8-year period. Seven of the bladder exstrophy were boys and nine were girls. Both cases of cloacal exstrophy were girls.

The youngest and the oldest patient at the time of repair were a 2-day-old neonate and a 6-year-old child, respectively, with a median of 4 months [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6].
Table 1: Sex distribution of exstrophy anomalies


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Figure 2: Eyebrow incisions for bilateral iliac osteotomy

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Figure 3: Tubularization of the bladder plate

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Figure 4: Complete first-stage repair

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Figure 5: Improvised gallows traction

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Figure 6: One year postoperative

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There were seven boys with bladder exstrophy, whereas nine girls had bladder exstrophy and two others with cloacal exstrophy, giving a male-female ratio of 7:11 for exstrophy anomalies [Table 1]. Eight patients (33.5%) had associated congenital anomalies, which included undescended testes (UDT) in three patients (16.7%), and anorectal malformation (ARM), patent processus vaginalis (PPV) and duplicate bladder in one patient each (5.6%), as shown in [Table 2].
Table 1: Sex distribution of exstrophy anomalies


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Table 2: Associated anomalies in the exstrophy complex


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All cases except one had bilateral posterior iliac osteotomy at bladder closure, which was done at the time of varied presentation.

Out of the 17 patients who had exstrophy anomaly and osteotomy, 13 (76.5%) had satisfactory bladder closure (no complication), 1 (5.9%) had superficial wound dehiscence, 2 (11.8%) had bladder neck dehiscence and 1 (5.9%) had complete dehiscence. But the only patient who did not have bilateral iliac osteotomy had complete bladder dehiscence [Table 3]. Postoperatively 16 patients were fully immobilized on improvised gallow traction [Figure 3] for a period of 3 weeks; the remaining one developed wounds on the sites of traction application, which was discontinued after one week of application. There was no mortality recorded in the study. The longest follow-up was for a period of 2 years and the shortest was for 2 weeks; four patients (22.2%) did not turn up for follow-up.
Table 3: Complications of exstrophy surgeries


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   Discussion Top


Bladder exstrophy although rare poses social and psychological embracement to parents and management challenges to both parents and surgeons. In most reports [1],[2],[3],[6] it has a slight male preponderance, but in this study we observed a slight female predominance as shown in [Table 1].

Prenatal diagnosis is possible with well-established facilities and diligence. Failure to localize the fluid-filled bladder on multiple examinations is usually highly suggestive.

All our patients were diagnosed postnatally through spot diagnosis for a dearth of the aforementioned factors.

In such anomalies, other associated anomalies such as the components of the VACTERL association are possible, but in this series, the following congenital anomalies were observed: UDT was most frequent, in 16.7% of the patients, whereas PPV, ARM and duplicate bladder occurred in 5.6% of the patients as shown in [Table 2]. This reinforces the truism to always search for other associated congenital anomalies while detecting one. In contrast to the predominance of the associated UDT in this series and others, [13] retractile testes were found to be more common in Shittu et al.'s. [3] These anomalies were corrected at the time of bladder closure.

At diagnosis we try to assess bladder capacity by observing the degree of bladder plate protrusion and by depressing the bladder mucosa inward using the index finger. When bladder capacity is considered adequate, the decision is taken to close the bladder.

Sometimes bladder capacity is very low at birth, so the surgery is delayed to allow bladder plate growth and the patient is discharged home on antibiotics with the instruction to keep the bladder moist with saline-soaked gauze.

The goals of surgical treatment are to obtain a continent child with a normal upper urinary tract and abdominogenital reconstruction, which makes possible a normal sexual and social life. [3]

Early surgery is imperative to prevent and mitigate epithelial metaplasia and inflammatory changes, which can compromise bladder capacity.

Due to improvements in surgical techniques, reconstruction of all forms of exstrophy has now become possible. The best functional results have been obtained with the Modern Staged Surgical Reconstruction (MSSRE) developed at Johns Hopkins. [4],[5]

Previously, reconstruction for bladder exstrophy was usually associated with a high failure rate, [7] especially in our environment, because of daunting constraints, late presentation, lack of availability of bladder neck, other incontinence procedures, etc., As a result, in the past, treatment of most of these cases was limited to urinary diversions, [8],[9],[10] which were fraught with complications such as hyper-chloremic metabolic acidosis, recurrent nephritis and anastomotic stenosis. [3],[11],[12]

Success has been reported [3],[13] with bladder closure without osteotomy, with nylon danning of the overlying fascia. But we found that the combination of nylon danning and symphysial approximation gives a better outcome. There were two cases that had complete bladder dehiscence in this series: One that did not undergo osteotomy and another that had traction immobilization discontinued after 1 week because of development of complications at the site of traction application [Table 3]. These underscores the importance of achieving and maintaining symphysial approximation through either anterior or posterior iliac osteotomy, and the use of traction or body cast for maintenance. We employed the posterior approach because of our familiarity with the technique, although the anterior approach proved to be the gold standard.

Follow-up of these patients is rather too short for meaningful detection of evolving complications. Patients with exstrophy anomalies should be followed up for life so that emergent long-term challenges of bladder exstrophy management vis-à-vis urinary incontinence, back pressure changes, metabolic complications and even epithelial dysplastic changes can be detected early and managed accordingly.

Incontinence of urine remains a major challenge among children with exstrophy anomalies, even after bladder closure. Mainz pouch operation [14] in experienced hands offers great relief in this direction. It is the procedure of choice in late-presenting cases with compromised bladder capacity, failed first-stage bladder closure and failed bladder neck reconstruction repair.


   Conclusion Top


Exstrophy anomalies, although rare, remain a major challenge in paediatric surgical practice. Iliac osteotomy, among other technical considerations, remains a cornerstone for successful bladder closure.


   Acknowledgments Top


The authors are gratefu to Dr. A Katagum for his valuable contributions in computer work and our family members for their unflinching moral and psychological supports.

 
   References Top

1.Mitrofanoff P. Trans-appendicular continent cystostomy in the management of the neurogenic bladder. Chir Pediatr 1980;21:297-305.  Back to cited text no. 1
    
2.Gargollo PC, Borer JG. Contemporary outcomes in bladder exstrophy. Curr Opin Urol 2007;4:272:80.  Back to cited text no. 2
    
3.Shittu OB. Management of ectopia vasica in Ibadan: An 8 year review. West Afr J Med 2005;3:196-9.  Back to cited text no. 3
    
4.Gearhart JP. The bladder exstrophy-epispadias complex. In: Es Gearhart JP, Rink RR, Mouriquand P, editors. Pediatric Urology. Ch. 32. Philadelphia: Saunders; 1994. p. 511-46.  Back to cited text no. 4
    
5.Gearhart JP, Jeffs RD. Exstrophy of the bladder, epispadias and other bladder anomalies. In: Campbell's Urology, 6 th ed. Walsh PC, Retik AB, Stamey TA, Darracot Vaughan E Jr, editors. vol. 2, WB Saunders Co. p. 1772-821.  Back to cited text no. 5
    
6.Brack JW, O'Neill JA Jr. Bladder Exstrophy in Pediatric Surgery. 5 th ed. O'Neill JA Jr, Marc I, Rowe Grosfeld JL, Fonkalsnid EW, Coran AG, editors, vol. 2. Mosby. p. 1709-25.  Back to cited text no. 6
    
7.Husmann DA, McLorie GA, Churchill BM. Closure of the exstrophic bladder: An evaluation of the factors leading to its success and its importance on urinary continence. J Urol 1989;142:522-4.  Back to cited text no. 7
    
8.Leadbetter GW Jr. Surgical correction of total urinary incontinence. J Urol 1964;91:261-6.  Back to cited text no. 8
    
9.Coffey RC. Transplantation of the ureter into the large intestine in the absence of a functioning bladder. Surg Gynecol Obstet 1921;32:383-91.  Back to cited text no. 9
    
10.Goodwin WE, Harris AP, Kauffman JJ, Beal JM. Open, transcolonic ureterointestinal anastomosis. Surg Gynecol Obstet 1953;97:295-300.  Back to cited text no. 10
    
11.Megalli M, Lattimer JK. Review of the management of 140 cases of exstrophy of the bladder. J Urol 1973;109:246-8.  Back to cited text no. 11
    
12.Segura JW, Kelalis PP. Long term results of ureterosigmoidostomy in children with bladder exstrophy. J Urol 1975;114:138-40.  Back to cited text no. 12
    
13.Arap S, Martins Giron A, Menezes de Góes G. Initial results of the complete reconstruction of bladder exstrophy. Urol Clin North Am 1980;7:477-91.  Back to cited text no. 13
    
14.Thuroff JW, Alken P, Riedmiller H, Jacobi GH, Hohenfellner R. 100 cases of Mainz pouch: Continuing experience and evolution. J Urol 1988;140:283-8.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]


This article has been cited by
1 Perioperative Challenges in Repeat Bladder Exstrophy Repair - Case Report
Otu Enenyi Etta,Monday Ituen
Open Access Macedonian Journal of Medical Sciences. 2015; 3(3): 432
[Pubmed] | [DOI]



 

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