We report a pair of dizygotic twins with type 1 posterior urethral valves. Concomitant and discordant presentation of posterior urethral valves in twins is rare, with a handful of cases in the literature. The occurrence of identical pathology in dizygotic twins probably suggests the possibility of an inherited trait as it does exist in monozygotic twins.Keywords: fraternal twins, identical pathology, inherited trait, posterior urethral valve

Adejuyigbe, Olusanya

Igwe, Arua O.

Owa, Joshua A.

Sowande, Oludayo A.

Talabi, Ademola O.

English

AJOL - African Journals Online

Downloadedfromhttps://journals.lww.com/apsbyBhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3l7ttZ9b/VuKxIwH3Dy/2pqEl0VxTbhh37J87j9nSKYU=on08/07/2018Downloadedfromhttps://journals.lww.com/apsbyBhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3l7ttZ9b/VuKxIwH3Dy/2pqEl0VxTbhh37J87j9nSKYU=on08/07/2018Posterior urethral valves in fraternal twins: case reportand review of the literatureAdemola O. Talabia, Oludayo A. Sowandea, Olusanya Adejuyigbea,Joshua A. Owab and Arua O. IgweaWe report a pair of dizygotic twins with type 1 posteriorurethral valves. Concomitant and discordant presentation ofposterior urethral valves in twins is rare, with a handful ofcases in the literature. The occurrence of identicalpathology in dizygotic twins probably suggests thepossibility of an inherited trait as it does exist inmonozygotic twins. Ann Pediatr Surg 14:190–191 © 2018Annals of Pediatric Surgery.Annals of Pediatric Surgery 2018, 14:190–191Keywords: fraternal twins, identical pathology, inherited trait,posterior urethral valvesDepartments of aSurgery and bPaediatric and Child Health, Obafemi AwolowoUniversity Teaching Hospitals Complex, Ile-Ife, Osun State, NigeriaCorrespondence to Ademola O. Talabi, MBBCh, FWACS, Department ofSurgery, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife,Osun State 2005, NigeriaTel: + 234 803 346 9206; fax: + 234 802 243 1901;e-mail: ademolatalabi1608@gmail.comReceived 15 September 2017 accepted 6 January 2018IntroductionPosterior urethral valves (PUV) are the commonest causeof bladder outlet obstruction in male neonates. The backpressure effect of bladder obstruction may lead toprogressive deterioration of upper urinary tract. It hasbeen postulated that ∼ 50% of children with PUV developend-stage renal disease in 10 years [1,2]. The etiology ofPUV is unknown. Some authors suggest that these valvesresult from abnormal insertion of the mesonephric ductinto the urogenital sinus [3,4]. The association betweenmonozygotic twins, nontwin siblings, and PUV has beendescribed in literature. Although there is no establishedgenetic link to this anomaly, it has been postulated that anautosomal recessive pattern of inheritance possibly existsfrom genetic study of families with affected siblings [1].There is paucity of reports on the diagnosis of PUV innonidentical twins. Here, we describe a case of PUV in aset of dizygotic twins who presented with varying degreeof bladder outlet obstruction and renal impairment.Case reportA set of twin boys was born via repeat cesarean deliveryto a 28-year-old gravida 2 para 1 woman. They weredichorionic and diamniotic twins delivered at 37 weeksof gestation. The first twin (twin 1) weighed 3.1 kg atbirth whereas the second twin (twin 2) weighed 2.8 kg.Twin 1 initially appeared to be normal and said to havepassed urine with a good urinary stream until 7 days afterbirth when he developed high-grade fever, refused totolerate feed, and passed bloody urine. There was nobleeding in any other part of the body. Prenatal ultrasoundscreening did not detect PUV. Baby was not pale, wasanicteric, was well hydrated, but febrile (temperature was38.8°C). There was a palpable suprapubic mass, and bothkidneys were ballotable. No Potter’s facie was seen. Hewas placed on continuous bladder drainage with urethralcatheter which led to decrease in suprapubic distension.Abdominal ultrasound showed bilateral hydronephrosis,dilated upper urinary system, and significant bladder wallthickening. An intravenous urogram with a micturatingphase confirmed PUV with bilateral vesicoureteric refluxworse on the right. Preoperative packed cell volume andserum chemistry findings were normal. Urinalysis showedmacroscopic hematuria and reduced specific gravity. Urineculture yielded no growth. At 16th day of life, he hadvalvotomy using a Mohan’s valvotome done at 3, 12, 9,and 6 o’clock positions. Urethral catheter was removed atpostoperative day 4, and the patient later passed urinewith normal stream. One month after surgery, the patientpresented with features of urinary sepsis necessitatingadmission and use of parenteral antibiotics. Patient wasmonitored with serial abdominal ultrasound scans, whichshowed sustained improvement of left upper urinary tractdilatation with, however, worsening dilatation and hydro-nephrosis on the right. A micturating cystourethrogramshowed a right grade V vesicoureteric reflux. Hesubsequently had a right loop ureterostomy with adequateright renal decompression. Patient has had frequent clinicvisits, and serial abdominal ultrasound scans showsustained improvement of both kidneys and goodcorticomedulary differentiation. Intravenous urogram andserum chemistry showed prompt excretion in bothkidneys and good renal function, respectively.Twin 2 was suspected to have bladder outlet obstructionsecondary to PUV following a prenatal ultrasound doneat 35 weeks of gestation. This necessitated urethralcatheterization at birth. A repeat abdominal ultrasound atdelivery showed a distended urinary bladder withthickened (0.3 cm) and trabeculated bladder wall. Therewere bilateral ureteric (0.11 cm diameter), pelvis, andcalyceal dilatations with bilateral hydronephrosis. Pre-operative packed cell volume, serum chemistry, andurinalysis findings were normal. Urine culture yieldedEscherichia coli sensitive to cefuroxime, and he was placeon parenteral antibiotic. He subsequently had valvotomydone as described for twin 1. Urethral catheter wasremoved at postoperative day 4, and patient had goodurinary stream. Micturating cystourethrogram done3 months later showed normal smooth bladder wall, withno evidence of reflux. Patient has since had several clinicvisits. Serial serum chemistry and intravenous urogramfindings were normal with good somatic growth.190 Case report1687-4137 © 2018 Annals of Pediatric Surgery DOI: 10.1097/01.XPS.0000530174.60069.b4Copyright r 2018 Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited.DiscussionPUV is increasingly being detected by routine prenatalultrasound in most developed nations. The characteristicfindings are bilateral hydronephrosis, a distended bladder,and a dilated posterior urethra (keyhole sign) [1]. Prenatalultrasound diagnosis and fetal screening in general are notroutinely employed in our clime in spite of its availabilitybecause of misconception of our patients and lack ofthorough fetal scanning on the part of the sinologist [5,6].In this set of twins, prenatal ultrasound was done to assessgestational age, fetal development and sex. The detection ofPUV in one of the twins was an incidental finding, and assuch, it was not surprising that the diagnosis was missed inthe other twin. Unfortunately, the possibility of identicalpathology in the first twin was not considered at birth until hebecame symptomatic and investigated. The relative delay inthe diagnosis of this anomaly in the first twin has underscoredthe importance of high index of suspicion among twins andsiblings of individual with PUV, and routine prenatalscreening of all fetuses for possible congenital malformation.Early diagnosis of PUV is necessary for reducingassociated anatomical and functional (compliance) defectof the bladder and upper urinary tracts because renalimpairment and other complications begin to develop asearly as the antenatal period [1]. In the index case report,this set of twins was detected and treated early in theperinatal period and has been doing well for almost3 years since birth, though the first twin had severehydroureter which necessitated loop ureterostomy.There are few reports in English literature on familialPUV, and in particular on dizygotic twins. Burttet et al. [1]in an extensive review of literature found 12 cases offamilial nontwin sibling with PUV. Christman et al. [7]reported two sets of discordant twin siblings (the first setbeing dizygotic and the second being monozygotic) inwhich only one of each pair presented late with PUV.Other researchers [3,4] documented the occurrence ofPUV in monozygotic twins. The association of PUV withtwins and nontwin siblings has not been elucidated. Someauthors opined that the high concordance rate of PUV inmonozygotic twins has been taken as evidence for agenetic predisposition to this anomaly [3,4,8]. We alsohypothesized that the occurrence of PUV in dizygotictwins may probably suggest the possibility of a genetic traitas does exist with monozygotic and nontwin siblings.We recommend urologic evaluation of the male siblingand family member of a male child with PUV.Conflicts of interestThere are no conflicts of interest.References1 Burttet LM, Rosito TE, Nabinger GB, Berger M, Neto BS. Posterior urethralvalves disorder in non-twin siblings: case report and literature review. MedSurg Urol 2014; 3:133.2 Sudarsanan B, Nasir AA, Puzhankara R, Kedari PM, Uunithan GR, DamisettiKRP. Posterior urethral valves: a single center experience over 7 years. PeadtrSurg Int 2009; 25:283–287.3 Thomalla JV, Mitchell ME, Garett RE. Posterior urethral valves in sibling. Urol1989; 33:291–294.4 Livne PM, Delaune J, Gonzales ET Jr. Genetic etiology of posteriorurethral valves. J Urol 1983; 130:781–784.5 Ohagwu CC, Abu PO, Odo MC, Chiegwu H. Maternal perception of barriersto utilization of prenatal ultrasound in prenatal care in the northern part ofNigeria. Clin Mother Child Health 2010; 7:1195–1199.6 Talabi AO, Sowande OA, Etonyeaku CA, Salako AA, adejuyigbe O. Posteriorurethral valves in children: pattern of presentation and outcome of initialtreatment in Ile-Ife, Nigeria. Nig J Surg 2015; 21:151–156.7 Christman MS, Zderic SA, Kolon TF. Delayed presentation of posteriorurethral valves in discordant twins. Urology 2012; 79:1365–1367.8 Morini F, Ilari M, Casati A, Pisera A, Oriolo L, Cozzi DA. Posterior urethralvalves and mirror image anomalies in monozygotic twins. Am J Med Gen Part A2002; 112:99–102.Posterior urethral valve in dizygotic twins Talabi et al. 191Copyright r 2018 Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited.

Posterior urethral valves in fraternal twins: case report and review of the literature

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Posterior urethral valves in fraternal twins: case report and review of the literature

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