Case Report Exstrophy-Epispadias Complex Variants: A Hybrid Case Alba Ganarin 1,2,*, Michele Corroppolo 2, Giosuè Mazzero 2, Clara Revetria 2, Fabio Beretta 2 and Enrico Ciardini 2 1 Pediatric Surgery Unit, Department of Women’s and Children’s Health, University Hospital of Padova, 35128 Padova, Italy 2 Pediatric Surgery Unit, Santa Chiara Hospital, 38122 Trento, Italy; [email protected] (M.C.); [email protected] (G.M.); [email protected] (C.R.); [email protected] (F.B.); [email protected] (E.C.) * Correspondence: [email protected] Abstract: The term exstrophy-epispadias complex refers to a group of midline defects ranging from epispadias to cloacal exstrophy. Bladder exstrophy is the most frequent malformation of this spectrum and it can present as a classical or a variant form. We report a case of a hybrid bladder exstrophy variant having some characteristics of both a duplicate bladder exstrophy and a superior vesical fistula. Keywords: exstrophy-epispadias complex; duplicate bladder exstrophy; exstrophy variants 1. Introduction Bladder exstrophy incidence is about 2.1–4.0 to 100,000, with its variant ten times Citation: Ganarin, A.; Corroppolo, more infrequent [1]. The main variants are: pseudoexstrophy, duplicate bladder exstrophy, M.; Mazzero, G.; Revetria, C.; Beretta, superior vesical fistula/fissure and covered exstrophy. Duplicate bladder exstrophy is F.; Ciardini, E. Exstrophy-Epispadias defined as the presence of a normal complete bladder and an exstrophic plate on the ante- Complex Variants: A Hybrid Case. rior abdominal wall [2]. Furthermore, hybrid form existence makes their recognition and Pediatr. Rep. 2021, 13, 177–180. classification even more challenging. We here describe a case of a patient having character- https://doi.org/10.3390/pediatric istics of two variants. Knowing how to recognize the differences between classical bladder 13020024 exstrophy and its variants can help the clinician make the correct diagnosis and treatment. Academic Editor: Maurizio Aricò 2. Case Report A full-term male newborn (birth weight: 3410 g) with a prenatal diagnosis of single Received: 20 November 2020 umbilical artery presented with an abdominal wall lesion consistent with bladder exstrophy. Accepted: 23 March 2021 Published: 7 April 2021 On physical examination, there was a normally inserted umbilical cord and below it, an abdominal midline defect with a mucosal plate, with no urine leakage (Figure1). Publisher’s Note: MDPI stays neutral Urine output was normally per-urethral. As no fistulous tract was found after trying with regard to jurisdictional claims in cannulation and no methylene blue spillage was noted after retrograde bladder filling, published maps and institutional affil- communication between mucosal plate and bladder or ureters was initially excluded. An iations. initial diagnosis of true duplicate bladder exstrophy was therefore made. The patient had normal male external genitalia, normally positioned and conformed anus and no other macroscopic malformations. Abdominal ultrasound (US) showed normal kidneys, a left ureter with a 3 mm distal tract dilatation and a normal bladder. A symphysis pubis diastasis was described on plain Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. X-ray. To confirm urinary tract anatomy, also a voiding cystourethrogram was performed This article is an open access article (Figure2): no communication between the normal bladder and the abdominal lesion was distributed under the terms and documented; a first grade left vesicoureteral reflux was described. During the next days, conditions of the Creative Commons urine leakage was noted but, even if the everted mucosa was wet, still no fistula was found. Attribution (CC BY) license (https:// In order to clarify the diagnosis (true bladder exstrophy duplication versus superior vesical creativecommons.org/licenses/by/ fistula), we decided to perform a computed tomography urography (CTU): no fistulous 4.0/). tract was documented. Pediatr. Rep. 2021, 13, 177–180. https://doi.org/10.3390/pediatric13020024 https://www.mdpi.com/journal/pediatrrep Pediatr. Rep. 2021, 13, FOR PEER REVIEW 2 Pediatr. Rep. 2021, 13, FOR PEER REVIEW 2 Pediatr. Rep. 2021, 13 178 raphy (CTU): no fistulous tract was documented. raphy (CTU): no fistulous tract was documented. Figure 1. Lower abdomen with exstrophic mucosal plate and normal external genitalia. FigureFigure 1.1.Lower Lower abdomenabdomen withwith exstrophicexstrophic mucosalmucosal plateplate andand normal normal external external genitalia. genitalia. Figure 2. Voiding cystourethrogram (lateral projection): no leakage of contrast medium between FigurebladderFigure 2.2. ( AVoidingVoiding) and exstrophic cystourethrogramcystourethrogram mucosal plate (lateral (B )projection projection): marked with): no no aleakage leakageradiopaque of of contrast contrast object. medium medium between between bladder (A) and exstrophic mucosal plate (B) marked with a radiopaque object. bladder (A) and exstrophic mucosal plate (B) marked with a radiopaque object. At the age of 14 days, the baby was taken to the operating room. An initial cystos- At the age of 14 days, the baby was taken to the operating room. An initial cystos- copyAt showed the age normal of 14 urethra days, the and baby bladder was and taken a le toft theectopic operating ureteral room. meatus An located initial at cys- the copy showed normal urethra and bladder and a left ectopic ureteral meatus located at the toscopybladder showedneck. A normaldot-like urethrasolution and of continuity bladder and was a leftobserved ectopic at ureteral the bladder meatus dome, located con- bladder neck. A dot-like solution of continuity was observed at the bladder dome, con- atsistent the bladder with a neck.possible A dot-like urinary solution fistula. Th of continuitye exstrophic was bladder observed plate at was the bladderisolated dome, and a sistent with a possible urinary fistula. The exstrophic bladder plate was isolated and a consistenttubular connection with a possible between urinary this and fistula. the Thenormal exstrophic bladder bladder was found, plate but was no isolated communica- and a tubular connection between this and the normal bladder was found, but no communica- tubulartion between connection the structures between this was and confirmed. the normal Both bladder the wasbladder found, plate but noand communication its connection tion between the structures was confirmed. Both the bladder plate and its connection betweenwere excised the structures and bladder was wall confirmed. reconstructed Both the(Figure bladder 3). A plate tension-free and its connection abdominoplasty were were excised and bladder wall reconstructed (Figure 3). A tension-free abdominoplasty excisedwas performed and bladder without wall necessity reconstructed of an (Figureosteotomy;3). A symphysis tension-free pubis abdominoplasty was approximated was was performed without necessity of an osteotomy; symphysis pubis was approximated performedwith non-absorbable without necessity stitches. of Post-operati an osteotomy;ve symphysiscourse was pubis uneventful was approximated and patient with was with non-absorbable stitches. Post-operative course was uneventful and patient was non-absorbabledischarged on stitches.the sixth Post-operative post-operative course day. wasThe uneventfulhistopathologic and patient examination was discharged was con- discharged on the sixth post-operative day. The histopathologic examination was con- onsistent the sixthwith bladder post-operative mucosa day. and Thedid not histopathologic describe any fistulous examination tract. was consistent with bladdersistent with mucosa bladder and didmucosa not describeand did anynot describe fistulous any tract. fistulous tract. There were no complications during the outpatient follow-up and urinary tract US, There were no complications during the outpatient follow-up and urinary tract US, performed after one month from surgical correction, was normal. performed after one month from surgical correction, was normal. Pediatr. Rep. 2021, 13 179 Pediatr. Rep. 2021, 13, FOR PEER REVIEW 3 FigureFigure 3. 3.Bladder Bladder (black(black star)star) exstrophicexstrophic plateplate (white(white star)star) andand their connection (arrow) before ( A) and after repair (B). and after repair (B). 3. DiscussionThere were no complications during the outpatient follow-up and urinary tract US, performedThe bladder after one exstrophy-epispadias month from surgical correction,complex represents was normal. an anterior midline defect and includes a wide spectrum of anomalies; its described incidence could be approxi- 3.mated Discussion in 1 to 10,000 live births. Bladder exstrophy, one of the possible anomalies, has an incidenceThe bladder of 2.1–4.0 exstrophy-epispadias to 100,000 live births complex [1]. Furthermore, represents an variations anterior midline of bladder defect exstro- and includesphy are a described, wide spectrum representing of anomalies; an even its described rarer entity, incidence 10 couldtimes bemore approximated infrequent in [3]. 1 toPseudoexstrophy 10,000 live births. is Bladderconsidered exstrophy, the rarest one and of mo thest possible benign anomalies,variant: it is has defined an incidence as an in- oftact 2.1–4.0 bladder to 100,000covered livewith births skin and [1]. a Furthermore, diastasis of the variations pubis and of rectus bladder muscles. exstrophy Superior are described,vesical fissure representing and fistula an even consist