1514 | Isolated Congenital Megacystis with Sponta- neous Resolution: An Exceedingly Rare Entity Farshid Alizadeh Keywords: urinary‎bladder;‎abnormalities;‎intestinal‎diseases;‎colon;‎infant;‎newborn. INTRODUCTION Megacystis‎is‎a‎rare‎condition‎in‎infants,‎which‎is‎usually‎associated‎with‎refluxing‎megaureters,‎prune-belly‎syndrome‎(PBS),‎infravesical‎obstruction‎or‎presents‎as‎ the‎megacystis‎microcolon‎intestinal‎hypoperistalsis‎syndrome‎(MMIHS).‎ Rarer‎even,‎is‎isolated‎congenital‎megacystis‎(ICM).‎The‎etiology‎of‎this‎entity‎is‎unknown.‎ Myenteric‎plexus‎pathology(1)‎and‎a‎mild‎form‎of‎MMIHS(2)‎have‎been‎proposed‎as‎plausible‎ pathologies.‎‎Here‎we‎add‎another‎case‎that‎resolved‎spontaneously. CASE REPORT A‎full-term‎4-day-old‎neonate‎boy‎was‎referred‎to‎our‎center‎because‎of‎delayed‎and‎infre- quent‎voiding.‎His‎fetal‎ultrasound‎had‎shown‎a‎distended,‎thin‎walled‎bladder‎without‎hyd- roureteronephrosis‎(HUN)‎and‎oligohydramnios‎at‎week‎32.‎He‎passed‎meconium‎soon‎after‎ birth,‎however‎did‎not‎void‎during‎the‎first‎24‎hours.‎After‎this‎period,‎voiding‎started‎but‎ infrequently‎(three‎to‎four‎times‎a‎day).‎Ultrasound‎study‎of‎abdomen‎showed‎a‎huge‎bladder‎ with‎160‎mL‎of‎volume.‎No‎HUN‎or‎increased‎bladder‎wall‎thickness‎was‎observed.‎Serum‎ creatinine‎was‎in‎the‎normal‎range‎throughout‎the‎follow-up.‎Urine‎culture‎was‎negative‎and‎ Corresponding Author: Farshid Alizadeh, MD Unit 10, No.22, 16th Alley, Shams Abadi Ave., Isfahan, Iran. Tele: +98 311 2350532 E-mail: f_alizadeh@med.mui.ac.ir Received November 2012 Accepted March 2013 Isfahan Urology and Kidney Transplantation Research Center, Isfahan University of Medical Sciences, Isfahan, Iran. CASE REPORT Case Report 1515Vol. 11 | No. 02 | March- April 2014 |U R O LO G Y J O U R N A L Isolated Congenital Megacystis | Alizadeh the‎patient‎did‎not‎develop‎urinary‎tract‎infection‎(UTI)‎dur- ing‎the‎follow-up‎period.‎Physical‎examination‎showed‎no‎ abnormality‎in‎the‎genital,‎rectal‎and‎sacral‎areas.‎Neuro- logical‎examination‎was‎also‎normal.‎ Because‎of‎the‎lack‎of‎any‎signs‎of‎bowel‎obstruction,‎the‎ pediatric‎gastroenterologist‎did‎not‎request‎barium‎enema‎or‎ rectal‎biopsy.‎During‎performing‎the‎voiding‎cystourethrog- raphy‎(VCUG),‎the‎baby‎did‎not‎void‎up‎to‎the‎volume‎of‎ 150‎mL‎and‎the‎cystogram‎showed‎a‎huge‎bladder‎without‎ trabeculation‎or‎reflux‎(Figure).‎The‎radiologist‎stopped‎the‎ test‎at‎this‎volume‎because‎of‎concern‎regarding‎iatrogenic‎ bladder‎rupture.‎Therefore,‎there‎was‎no‎urethrogram‎for‎the‎ evaluation‎of‎the‎urethra.‎ Since‎the‎bladder‎wall‎was‎smooth‎and‎no‎HUN‎was‎present,‎ we‎decided‎not‎to‎perform‎cystoscopy.‎Instead,‎we‎advised‎ the‎parent‎to‎start‎clean‎intermittent‎catheterization‎(CIC).‎ They‎were‎reluctant‎to‎accept‎this‎suggestion.‎Therefore,‎we‎ advised‎them‎to‎come‎for‎frequent‎follow-up‎while‎on‎an- tibiotic‎prophylaxis.‎Serial‎ultrasound‎showed‎progressive‎ shrinkage‎of‎the‎bladder,‎reducing‎to‎80‎mL‎at‎the‎age‎of‎six‎ months.‎Bowel‎habit‎was‎still‎normal. DISCUSSION Reported‎cases‎of‎ICM‎are‎very‎few‎with‎different‎clinical‎ course‎and‎management‎strategies. Inamdar‎and‎colleagues‎reported‎a‎case‎of‎“vesical‎gigan- tism”‎that‎was‎initially‎managed‎by‎bladder‎catheterization,‎ then‎ cutaneous‎ vesicostomy‎ because‎ of‎ recurrent‎ bladder‎ distention, and later, by reduction cystoplasty due to high post-voiding‎residual‎urine.(3)‎They‎believed‎that‎this‎entity‎ may‎“results‎from‎an‎expansion‎of‎the‎portion‎of‎the‎urinary‎ bladder‎that‎develops‎from‎the‎allantois”. Shsimizu‎ and‎ colleagues‎ reported‎ another‎ case‎ that‎ was‎ managed‎expectantly‎up‎to‎the‎age‎of‎four‎years,‎however,‎ the‎child‎developed‎UTI‎at‎that‎age.‎A‎hypocontractile‎detru- sor‎and‎increased‎compliance‎were‎the‎urodynamic‎findings.‎ Rectal‎biopsy‎revealed‎hypoganglionosis‎of‎the‎submucous‎ and‎myenteric‎plexuses‎without‎thinning‎of‎the‎longitudinal‎ muscle‎and‎connective‎tissue‎proliferation.‎They‎managed‎ the‎patient‎by‎CIC‎and‎proposed‎that‎she‎might‎have‎a‎mild‎ form‎of‎chronic‎intestinal‎pseudo‎obstruction‎syndrome. Recently another case report appeared in the literature, pre- senting‎a‎case‎of‎ICM‎that‎resolved‎by‎the‎age‎of‎one‎year‎ and‎remained‎asymptomatic‎afterwards‎without‎any‎thera- peutic‎intervention.(4)‎ Since‎ICM‎is‎a‎very‎rare‎entity,‎when‎a‎baby‎is‎born‎with‎ megacystis,‎ other‎ more‎ prevalent‎ pathologies‎ should‎ be‎ ruled‎out‎first.‎PBS‎is‎diagnosed‎by‎the‎classic‎presentation‎ of‎abdominal‎musculature‎defect‎and‎undescended‎testes.‎ Infravesical‎obstruction‎could‎be‎diagnosed‎by‎a‎VCUG.‎A‎ complete‎neurological‎and‎gastrointestinal‎evaluation‎is‎also‎ mandatory‎to‎look‎for‎any‎signs‎of‎intestinal‎obstruction.‎ Urodynamic‎study‎could‎demonstrate‎detrusor‎hypocontrac- tility,‎if‎present.‎If‎there‎is‎no‎other‎anomaly,‎the‎diagnosis‎ of‎ICM‎is‎suggested.‎Management‎of‎these‎patients‎should‎ be‎individualized‎based‎on‎the‎presence‎of‎urinary‎retention,‎ high‎post-voiding‎residual‎urine‎and‎UTI.‎ CIC,‎vesicostomy‎and‎observation‎with‎administration‎of‎ antibiotic‎prophylaxis‎are‎all‎viable‎options.‎Regardless‎of‎ the‎chosen‎therapeutic‎option,‎long-term‎follow-up‎is‎man- datory,‎because‎the‎clinical‎course‎of‎this‎entity‎is‎not‎fully‎ understood. CONCLUSION The‎clinical‎course‎in‎this‎case‎highlight‎the‎fact‎that‎not‎all‎ cases‎of‎ICM‎need‎extensive‎work-up‎and‎when‎the‎clini- cal‎setting‎justifies,‎cystoscopy,‎urodynamics‎and‎repeated‎ VCUGs‎could‎be‎avoided. Figure .Voiding cystourethrography demonstrates a huge blad- der without trabeculation or reflux. 1516 | REFERENCES 1. Perk H, Serel TA, Anafarta K, Kosar A, Uluoglu O, Sari A. Megacys- tis secondary to myenteric plexus pathology. Presentation of two cases. Urol Int. 2001;67:313-5. 2. Shimizu M, Nishio S, Ueno K, et al. Isolated congenital megacystis without intestinal obstruction: a mild variant of chronic intestinal pseudoobstruction syndrome? J Pediatr Surg. 2011;46:e29-32. 3. Inamdar S, Mallouh C, Ganguly R. Vesical gigantism or congenital megacystis. Urology. 1984;24:601-3. 4. Johnson EK, Nelson CP. Spontaneous resolution of isolated con- genital megacystis: The incredible shrinking bladder. J Pediatr Urol. 2013;9:e46-50. Case Report