Transcript
Pediatric Urology Primary Congenital Bladder Diverticulain Children Jose Maria Garat, Oriol Angerri, Jorge Caffaratti, Pietro Moscatiello,and Humberto Villavicencio OBJECTIVES To present the symptoms, treatment, evolution, and pathologic findings of primary (nonobstruc-tive) diverticula in childhood. Primary (nonobstructive) diverticula are not frequent in child-hood. Bladder diverticula have classically been defined as “hernias of the mucosa throughmuscular fibers of the bladder wall.” METHODS From September 1985 through September 2005, we treated 11 children categorized hashaving primary congenital bladder diverticula. At diagnosis, their ages ranged between 7months and 9 years (mean 4.6 years). Ten were boys and one was a girl. The main reasonfor consultation was febrile urinary infection (n 5), recurrent urinary infections (n 3),enuretic syndrome (n 1), gross hematuria (n 1), and pain in the right iliac fossa (n 1). One child had Ehlers-Danlos syndrome. All the diverticula were larger than 2 cm indiameter. Of the 11 children, 10 underwent surgery. Clinical observation was chosen for the9-month-old infant. RESULTS All 10 operated children had developed favorably at a follow-up of 2 to 20 years (mean 9.3). The9-month-old child continued under observation for 5 years. We observed no diverticulumrecurrence and no postoperative vesicoureteral reflux in the reimplanted children. Pathologicexamination was performed of the excised diverticula in 7 cases, and muscular fibers were foundin all of them; however, most of the fibers were very fine, particularly at the dome of thediverticulum. CONCLUSIONS The results of our study have shown that urinary tract infection is the most common symptomof bladder diverticula. Surgical treatment provided good results, without any recurrence ormorbidity. The bladder diverticula were lined with fine muscular fibers. UROLOGY 70: 984–988,2007. © 2007 Elsevier Inc. B ladder diverticula have been classically defined as“hernias of the bladder mucosa through muscularfibers of the bladder wall.” 1 As we shall prove byanalyzing our case-by-case experience, this construct doesnot have absolute validity, because in most cases, thediverticulum was lined with fine muscular fibers. In thevast majority of cases, bladder diverticula are secondaryto bladder outlet obstruction or neurogenic bladder. Thatis, they are either acquired or secondary diverticula (eventhough they can also develop prenatally); these are notthe subject of our report.Primary (nonobstructive) diverticula are not frequentin childhood. They are single in most cases. The secondmost frequent cases are double diverticula, which are veryrarely found in the nonobstructive polydiverticular blad-der. This entity shows a somewhat different behavior(that we examine) compared with single diverticula. 2,3 Some collagen-affecting syndromes such as the Wil-liams-Beuren syndrome, Ehlers-Danlos syndrome, andMenkes syndrome, that, among other manifestations,have multiple bladder diverticula also present with ele-ments different from those of the rest of diverticula.In this study, we report on large (single or multiple)primary congenital diverticula (ie, larger than 2 cm indiameter), because we believe that small diverticula aredifferent clinicopathologic entities and most need notreatment.In all cases, the frequency of diverticula is greateramong males. Ever since urinary infections have beensystematically studied, the frequency of a bladder diver-ticula diagnosis has increased from 0.7% to 1.7%. 1 MATERIAL AND METHODS From September 1985 through September 2005, we treated 11children with what can be categorized as primary congenital From the Paediatric Urology Division, Urology Service, Fundació Puigvert, Barcelona,SpainReprint requests: Oriol Angerri, M.D., Paediatric Urology Division, Urology Ser-vice, Fundació Puigvert, C/Cartagena 340-350, Barcelona 08025 Spain. E-mail:
[email protected]: February 8, 2007, accepted (with revisions): June 26, 2007 984 © 2007 Elsevier Inc. 0090-4295/07/$32.00All Rights Reserved doi:10.1016/j.urology.2007.06.1108 bladder diverticula (PCBD). At diagnosis, their ages rangedfrom 7 months to 9 years (mean 4.6 years). Ten were boys andone was a girl.The main reason for consultation was febrile urinary infec-tion (n 5), recurrent urinary infection (n 3), enureticsyndrome (n 1), gross hematuria (n 1), and pain in theright iliac fossa (n 1). One of the children had Ehlers-Danlossyndrome.The diagnostic procedures included retrograde and voidingcystography in all cases; however, in 4 children, it was theultrasound findings that raised suspicion. All the diverticulawere larger than 2 cm in diameter. In most cases, the bladdersize was not stated, so it was not possible to discern the diver-ticulum/bladder size relationship.Of the 11 patients, 7 had a single diverticulum, 2 had adouble diverticulum, and 2 had a multidiverticular bladderwith no obstructive elements. The diverticula were accom-panied by unilateral vesicoureteral reflux (grade 1 and grade2) in 3 patients and bilateral reflux (grade 3) in 1 patient.The diverticula were mostly posterolateral (n 7); one wasbilaterally paraureteral, and two bladders were multidiver-ticular.Urodynamic studies were performed in 4 cases to excludedetrusor overactivity or obstruction due to dyssynergia—two of the studies were normal. In the case of the multidiverticularbladder, a bladder outlet obstruction was ruled out, but a hy-pocontractile detrusor was reported. One patient had a “flat-tened” uroflow. Nine patients underwent preoperative urethrocystoscopy,which, in all cases, enabled us to observe the diverticularmouths and also to confirm the normality of the urethralchannel and bladder neck.Ten children underwent surgery, one before consultationat our center (the patient with Ehlers-Danlos syndrome).Clinical observation was chosen for the 9-month-old infant.The surgical approaches were intravesical and extravesical,with diverticulum/diverticula resection (Fig. 1). The patientwith the multidiverticular bladder did not undergo excisionof all the diverticula, but only the five largest ones from atotal of nine.Ureterovesical reimplantation of the Cohen type was per-formed in 5 patients. Of these 5 patients, 4 (five ureters) hadreflux and 1 underwent dissection of a ureter that was quiteattached to the diverticulum wall and was inserted in thebladder in conditions that could quite probably favor postoper-ative reflux. No ureteral reimplantation was performed in themultidiverticular bladder. Neither was it done in the patientwith Ehlers-Danlos syndrome. RESULTS All 10 operated children developed favorably, withouturinary tract infections during 2 to 20 years of follow-up(mean 9.3). The 9-month-old child continued underobservation for 5 years. We observed no diverticulum/diverticula recurrence, and no postoperative vesico-ureteral reflux in the reimplanted children.The boy with Ehlers-Danlos syndrome (who had un-dergone surgery at another hospital) underwent resectionof some diverticula; however, the latest cystogram wasquite similar to the preoperative one (recurrence?).The case of the multidiverticular bladder was quiteparticular. The child, who had severe preoperative void-ing disorders, normalized his voiding function, and uro-dynamic studies showed “recovery” of the detrusor con-tractility. However, bilateral grade 2 reflux appeared.Initially, the child underwent chemoprophylaxis. Theexcised diverticula did not recur. At 9 years of follow-up,he continued to be asymptomatic.The child with a preoperative “flattened” flow and aweak stream had normal flowmetry and voiding aftersurgery.A pathologic examination was performed on the ex-cised diverticula in 7 cases, and muscular fibers werefound in all; however, most of the fibers were very fine,particularly at the dome of the diverticulum (Fig. 2). COMMENT We selected a group of patients with PCBD who hadthe common characteristic of having undergone sur-gery. Diverticula resection enabled us to observe thatin all those cases in which a pathologic examinationhad been performed (7 cases), distended muscular fi-bers were present forming a thin layer. This fact some-what belies the classic diverticulum definition of “a Figure 1. Open surgery of bladder diverticula. Figure 2. Pathologic examination of bladder diverticulashowing fine muscular fibers. UROLOGY 70 (5), 2007 985 mucosal hernia through the muscular wall. 1 In anycase, this fine layer lacks the contractility that allowsthe diverticulum to appropriately void into the blad-der. Also, particularly when large, an intradiverticularresidue always remains. 1 Forsythe and Smyte 2 in 1959, and Johnston 3 in 1960were the first to describe primary diverticula in nonob-structed children. From then on, it has been necessary toestablish a clear differentiation between primary diver-ticula and diverticula secondary to posterior urethralvalves, neurogenic bladder, and so forth, which should betreated before treating the diverticula.Zia-Ul-Miraj 4 emphasized that secondary diverticulaare genuine bladder mucosa hernias. Both secondary andprimary diverticula have been observed in the fetus andnewborn babies, 1 and, therefore, both could be deemedcongenital.PCBDs are predominantly seen in males, and ourcases were no exception to this rule with 10 boys and1 girl. PCBDs are mainly diagnosed when patients are3 to 7 years old, 1,5 and they are generally found in thecourse of ultrasonography or radiology for urinary tractinfection. 6 Two theories attempt to explain their occurrence.Some diverticula occur since hernia appearance is due tohigh vesical pressure. 1,5 This would explain their greatermale predominance, because males void at greater pres- sures, even in utero. Stephens 5 and Johnston 3 believethat the muscle failure is primitive. Linke and Mongiat-Artus 7 stated that PCBDs develop at points at whichmuscle fusion is incomplete, such as the joint betweenthe trigone and the dome of the bladder, two entitieswith differing embryologic srcin.PCBDs can be categorized into three types 1,5 :1. Posterolateral: present in approximately 10% of cases,not related to ureteral openings, and usually not asso-ciated with vesicoureteral reflux; however, they cangrow considerably and compress the urethra.2. Paraureteral: most diverticula (90%) belong to thistype and are related to the ureteral hiatus that formsthe diverticulum neck. Within this type are two sub-groups stratified by the topography of the ureteralmeatus: ureter in the bladder (associated with reflux),and ureter in the diverticulum (associated with high-degree reflux and renal dysplasia). 8 3. Multidiverticular bladders (no obstruction): isolated(more frequent in girls) and associated with syn-dromes such as prune belly, Ehlers-Danlos, Williams-Beuren, Menkes syndrome, and so forth.PCBD manifestations can be quite varied and some-times are wholly asymptomatic. They are frequentlyassociated with recurrent urinary tract infections.They can cause bladder neck obstruction in males. 6,9–13 According to Sheldon and Essig, 12 this isseen in 80% of cases during the first year of age (50%during the first 3 months).Their association with vesicoureteral reflux has variedgreatly from one series to another. In our 11 cases, 4 wereassociated with vesicoureteral reflux. Generally, these areparaureteral diverticula, and sometimes this situation de-termines the treatment. They can also develop internallithiasis. 6 Patients with these diverticula can present withlow-flow (“flattened”) voiding disorders, as occurred with2 of our patients. This can result from non-neurogenicvesicosphincteral dyssynergia, in which case it would beconsidered a secondary diverticulum. 14 However, it canoccur in other situations, as clearly observed by us in 2 of our patients: polydiverticular bladder and a 5-cm-diame-ter diverticulum. Males presented with dysuria and a veryweak stream. The flowmetry will be “flattened.” No uri-nary obstruction will be found on urodynamic study orurethroscopy. The explanation is as follows: the detrusorcontracts but the urine enters the diverticulum/divertic-ula more easily than into the bladder neck, thus simulat-ing detrusor undercontractility. When the diverticula arerepaired, the patient immediately recovers their normalvoiding rhythm, normal flow, and absence of postvoidresidual urine volume. The problem is a pressure leak thatwe have termed “vesicodiverticular reflux” (Fig. 3). Other clinical manifestations can include hematuria, which oc-curred in 1 of our patients, or pain in the iliac fossa(rarer).Multidiverticular bladder in patients with Ehlers-Danlos, Menkes, or Williams-Beuren syndrome is quiteanother entity. 1,14–17 The vast majority of cases of thisdisorder have been described in males, as was our case. Jorion and Michel 18 described the case of a girl withEhlers-Danlos syndrome. Its mechanism of occurrenceprobably results from congenital weakness of the de-trusor muscular fibers, associated with the congenitalcollagen disease. Surgical abstention is favored inthese cases, except in a very few specific instances,because diverticular recurrence is almost always therule. Generally, they are diverticula that appear at any Figure 3. Vesicodiverticular reflux. 986 UROLOGY 70 (5), 2007 bladder location, but usually far from the ureteralopenings.PCBD can be diagnosed through ultrasonography. 19 When the diverticulum is very large, it is not easy todiscern it from the bladder. In 4 of our patients, we wereable to suspect a diverticulum when we performed ultra-sound scanning. The most widely used method is voidingcystourethography. 1,6,8,20,21 It is important to performoblique projections for a better topographic survey of thediverticulum. Sometimes, it can only be observed onvoiding or postvoiding plates. 11,21 Magnetic resonanceimaging of the urinary tract has been used in the past fewyears, as has echo-Doppler ultrasonography. 19 A bladderdiverticulum is considered large if it measures more thanone third of the bladder diameter. 9,22 It is important to perform urodynamic studies (cystom-etry and flowmetry) both preoperatively and postopera-tively. In most of our cases we did so, and they wereextremely useful, particularly for ruling out any associatedobstructive or functional pathologic findings.The management of large PCBDs is surgical to pre-vent or to treat complications, and in some cases toimprove the child’s voiding. Resection will resolve thecompressive bladder outlet obstruction (if any) andprevent postvoid residual urine, thus preventing uri-nary tract infection recurrence. Diverticulum removalalso eliminates “pressure leaks” and improves bladdervoiding.Afshar et al . 22 studied the progress of PCBD-associatedvesicoureteral reflux in 84 patients and compared themwith a similar series of 95 patients without diverticula.They concluded that no developmental differences ex-isted between the two groups and that the spontaneousresolution rate was similar. They were studying divertic-ula that did not require surgery because of their size. Intheir large series of 47 PCBDs, Piró et al. 21 established thetreatment according to the diverticular size and the pres-ence of reflux (45%). However, the current managementof vesicoureteral reflux is a more conservative one, andmany of those would not require surgical management atpresent.Diverticular removal can be done using an open orlaparoscopic approach. 7 Kok et al. 23 reported the firstlaparoscopic diverticulectomy in a 6-year-old child.Yu 24 described an extravesical open technique to treatsimultaneous diverticulum removal and ureteral reim-plantation. Such a technique can be extrapolated tothe laparoscopic approach. All of our cases weretreated using an intravescial and extravesical openapproach, as favored by most investigators. Ureteralreimplantation is not mandatory and would only beindicated if reflux were present or for technical rea-sons. Special care should be taken not to injure the vasdeferens. 1,7 According to Sariham and Aber, 25 small,asymptomatic diverticula and those associated withEhler-Danlos syndrome should not undergo resection.Once the diverticulum has been removed, recurrencesare rare. 26 Development was very good during thelong-term follow-up in our patients. CONCLUSIONS Urinary tract infection is the most common symptomof bladder diverticula. Surgical treatment providesgood results without any recurrence or morbidity. Ourpathologic examinations revealed that primary nonob-structive bladder diverticula are lined with fine mus-cular fibers. References 1. Garat JM: Malformaciones vesicales, en Garat R, Gosálbez R (Eds): Urología Pediátrica . Salvat, Barcelona, 1987, pp 287–313.2. Forsythe I, and Smyte B: Diverticulum of the bladder in children:a study of 13 cases. Pediatrics 24: 322–325, 1959.3. Johnston J: Vesical diverticula without urinary obstruction inchildhood. J Urol 84: 535–538, 1960.4. Zia-Ul-Miraj M: Congenital bladder diverticulum: a rare cause of bladder outlet obstruction in children. J Urol 162: 2112–2113,1999.5. Stephens D: The vesicoureteral hiatus and paraureteral diverticula. J Urol 121: 786–790, 1979.6. Canning D, Koo H, and Duckett J: Anomalies of the bladder andcloaca, in Gillenwater J, Grayhack J, Howards S, et al (Eds): Adult and Pediatric Urology. Mosby, St. Louis, 1996, vol 3, pp2445–2488.7. Linke C, and Mongiat-Artus P: Traitement des diverticules vési-caux. Ann Urol 38: 103–111, 2004.8. Barrett D, Malek R, and Kelalis P: Observation on vesical diver-ticulum in childhood. J Urol 116: 234–236, 1976.9. Bogdanos J, Paleodimos I, Korakianitis G, et al: The large bladderdiverticulum in children. J Pediatr Urol 1: 267–272, 2005.10. Shukla A, Bellah R, Canning D, et al: Giant bladder diverticulacausing bladder outlet obstruction in children. J Urol 172: 1977–1979, 2004.11. Verghese M, and Belman B: Urinary retention secondary to con-genital bladder diverticula in infants. J Urol 132: 1186–1188,1984.12. Sheldon C, and Essig K: Congenital bladder diverticulum causingbladder outlet obstruction: case report and review of the literature.Pediatr Surg Int 9: 141–143, 1994.13. Oge O, Gemalmaz H, and Ozeren B: Acute urinary retention in achild caused by a congenital bladder diverticulum. J Pediatr Surg 37: 926–927, 2002.14. Martín-Crespo R, Luque R, Navascues J, et al: Alteraciones funci-onales de la vejiga en los niños portadores de divertículo vesicalprimario. Arch Esp Urol 50: 661–667, 1997.15. Levard G, Agrain Y, Ferkadji L, et al: Urinary bladder diverticulaand the Ehlers-Danlos syndrome in children. J Pediatr Surg 24: 1184–1186, 1989.16. Bade J, Ypma A, Vanelk P, et al: A pelvic mass: bladder divertic-ulum with haemorrhage in Ehlers-Danlos patient. Scand J Urol Nephrol 28: 319–321, 1994.17. Galifer R, Veyrac C, López C, et al: Malformations vésicalesrares. Encyclopédie médico-chirurgicale 18-208C10. Elsevier,Paris, 2003.18. Jorion J, Michel M: Spontaneous rupture of bladder diverticula ina girl with Ehlers-Danlos syndrome. J Pediatr Surg 34: 483–484,1999.19. Maynor C, Kiewer M, Hertzberg B, et al: Urinary bladder divertic-ula: sonographic diagnosis and interpretative pitfalls. J UltrasoundMed 16: 189–194, 1996.20. Pieretti R, and Pieretti-Van Marcke R: Congenital bladder diver-ticula in children. J Pediatr Surg 34: 468–473, 1999. UROLOGY 70 (5), 2007 987 21. Piró C, Martín J, Campos J, et al: Divertículos vesicales primarios.Estudio de 47 casos. Cir Pediatr 2: 191–195, 1989.22. Afshar K, Malek R, Bakhshi M, et al: Should the presence of congenital para-ureteral diverticulum affect the management of vesicouretereral reflux? J Urol 174: 1590–1593, 2005.23. KokK,SeneviratneH,ChuaH, et al: Laparoscopicexcisionofcongenitalbladder diverticulum in a child. Surg Endosc 14: 501–502, 2000.24. Yu T: Extravesical diverticuloplasty for the repair of a paraureteraldiverticulum and the associated refluxing ureter. J Urol 168: 1135–1137, 2002.25. Sariham H, and Aber M: Congenital bladder diverticula in infants.Eur Urol 33: 101–103, 1998.26. González J, Rodó J, Arza H, et al: Divertículos vesicales en el niño.Rev Esp Pediatr 45: 54–57, 1989. 988 UROLOGY 70 (5), 2007
