We here report a rare association of megalourethra with pouch colon with prune belly syndrome. We also provide a newer embryological and prognostic perspective to this association.Keywords: megalourethra, prune belly syndrome pouch colon, scaphoi

Bawa, Monika

Garge, Saurabh

Rao, Katragadda Lakshmi Narasimha

English

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Prune belly syndrome with pouch colon with scaphoidmegalourethra: a newer embryological and prognosticperspectiveSaurabh Garge, Monika Bawa and Katragadda Lakshmi Narasimha RaoWe here report a rare association of megalourethra withpouch colon with prune belly syndrome. We also provide anewer embryological and prognostic perspective to thisassociation. Ann Pediatr Surg 11:42–45 c 2015 Annals ofPediatric Surgery.Annals of Pediatric Surgery 2015, 11:42–45Keywords: megalourethra, prune belly syndrome pouch colon, scaphoidDepartment of Pediatric Surgery, Advanced Pediatric Center, PGIMER,Chandigarh, IndiaCorrespondence to Saurabh Garge, MCh, Department of Pediatric Surgery,Advanced Pediatric Center, PGIMER, Chandigarh 160012, IndiaTel: + 91 172 274 7585 x5320; fax: + 91 172 274 4401/274 5078;e-mail: saurabhgarge8@gmail.comReceived 15 November 2012 accepted 26 May 2014IntroductionPrune belly syndrome (PBS), also known as Eagle–Barrettsyndrome, comprises a triad of anomalies that includeabdominal wall flaccidity, urologic abnormalities, andbilateral cryptorchidism. The incidence of PBS isbetween 1 in 29 000 and 1 in 40 000 live male births,with incidence four times higher in twins [1–6]. Maleindividuals are affected 20 times more often than femaleindividuals. The underlying etiology of the urinary tractobstruction, severity of associated abnormalities, and thedegree of pulmonary hypoplasia influence the survivaland outcome of infants with PBS. Congenital pouch colonis characterized by replacement of varying length of colonby a dilated pouch that almost invariably has a wide highfistulous communication with the genitourinary tract.There have been four instances where pouch colon hasbeen found associated with prune belly. We here describethe fifth case of such occurrence and also review theprevious cases (Table 1) [1–3,7]. Our case also hadscaphoid megalourethra with urethral stenosis, which isthe first instance of this rare occurrence. The previousreport of megalourethra was truly speaking an anteriorurethral diverticulum, without any proximal or distalobstruction [1].Case reportA term male neonate, born through normal vaginaldelivery of an uneventful supervised pregnancy, wasbrought to our hospital at 28 h of life because of animperforate anus and progressively increasing abdominaldistension. The baby was dehydrated and hypothermic,with a birth weight of 2.05 kg. His abdomen was flabby,with visible loops of intestine. Two lumps were seen, oneover the right hypochondrium and another over thehypogastrium. The lump over the right hypochondriumwas 7 cm 5 cm in its maximum horizontal and verticaldimensions, respectively. It was cystic in consistency,transilluminant, and ballotable. The lump over thehypogastrium was because of a distended bladderpalpable 6 cm from the pubic symphysis. The bladderwas expressible, with urine getting collected in thedilated urethra. A scaphoid megalourethra with pinpointmeatus with absent corporal tissue was found. Onpressing the urethra, patient voided turbid purulenturine. The scrotum lacked rugosities and bilateral testiswere undescended. They were not palpable even in theinguinal region (Fig. 1). The anal opening was absent,with poorly developed buttocks and median raphe. Across-table prone lateral radiograph and erect anteropos-terior abdominal radiograph were suggestive of a highrectal anomaly with a pouch colon (Fig. 2). Ultrasono-graphy of the abdomen showed bilateral hydronephrosiswith right-sided anteroposterior diameter of 5.5 cm. Theureters were not visualized due to excessive bowel gas.Echocardiography revealed presence of coarctation ofaorta with patent ductus arteriosus and atrial septaldefect of 5 mm.Exploratory laparotomy revealed deficient musculartissue in the abdominal wall. Intraoperatively, type IVpouch colon with a wide colovesical fistula was present. Itlacked haustrations, appendices epiploicae, and taeniacoli. There was an abrupt transition from the normalcaliber proximal bowel to the distal dilated pouch. Thebladder was grossly distended and contained purulenturine (Fig. 3). The colovesical fistula was ligated, thepouch colon was excised, and an end descendingcolostomy was fashioned. The bladder opening wasclosed and a suprapubic cystostomy was placed as theurethra was not catheterizable. Bilateral intra-abdominaltestis was also found.A dye study at the bed side showed scaphoid megalour-ethra with large bladder (Fig. 4). The intraoperative urineculture grew Escherichia coli. Blood cultures grew Klebsiellapneumonia. The patient developed sepsis and died on thefifth postoperative day, despite all possible measures.DiscussionPBS, a rare congenital anomaly, exists almost exclusivelyin male individuals and consists of genital and urinaryabnormalities with partial or complete absence ofabdominal wall musculature [1,2].The syndrome has a broad spectrum of affected anatomywith different levels of severity. Additional associatedanomalies exist involving the respiratory tract, gastro-intestinal tract, cardiac system, and musculoskeletal42 Case report1687-4137 c 2015 Annals of Pediatric Surgery DOI: 10.1097/01.XPS.0000452064.76439.47Copyright © Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited.system. Gastrointestinal anomalies are seen in over 30%of these patients [3]. Malrotation, intestinal atresia,volvulus, embryonic mesentery, and wandering spleen arecommon gastrointestinal associations [3–6]. Exomphalos,gastroschisis, tracheoesophageal fistula, and anorectalmalformations are rare gastrointestinal associations [4–6].Pouch colon, as a rare gastrointestinal association, hasbeen reported in only four instances before [1–3,7].Congenital pouch colon, also known as congenital shortcolon, is a high anorectal malformation in which a varyinglength of colon is replaced by a dilated pouch that almostinvariably has a wide high fistulous communication withthe genitourinary tract [8]. Pouch colon has beenclassified into four types depending on the segment ofnormal colon present [8].The anterior urethra in cases of PBS is usually normal;however, many urethral anomalies such as atresia,stenosis, and megalourethra have been reported [1,6].Congenital megalourethra is characterized by severedilatation of the anterior urethra and lack of penileerectile tissue development [1,6]. Absence of proximaland distal obstruction, absence of erectile tissue, anddilation of entire anterior urethra differentiate it fromurethral diverticulum [1,6]. Our patient had a scaphoidmegalourethra with a urethral stenosis that could not becatheterized. This is probably the first report of anassociation of PBS, pouch colon, and scaphoid megalour-ethra. Our patient lacked discernible erectile tissue;however, the previous report of anterior urethral diverti-culum does not mention anything about the condition oferectile tissue [1].The exact etiology of PBS is unknown, although severalembryologic theories attempt to explain the anom-aly [1–3]. Overdistension of the abdomen due to bladderdistension, mesodermal arrest, and defects in yolk sacare among the most common etiologies [1–3,7]. Theassociation of examphalos, gastroschisis, absent derma-tome, and now pouch colon with PBS lends credence tothe mesodermal arrest theory, early during embryogen-esis [4–7]. A vascular compromise in the region of theTable 1 Prune belly syndrome associated anomaliesSerial numbers References Type of pouch colon Urethral anatomy Associated abnormalities1 Bangroo et al. [1] Type I Anterior urethral diverticulum None2 Baba et al. [7] Type IV Microurethra Absent dermatome3 Raghavan et al. [3] Type I Penoscrotal hypospadias None4 This study Type IV Scaphoid megalourethra Coarctation of aorta, patent ductus arteriosus, atrial septal defectFig. 1Megalourethra, empty scrotum, flaccid abdomen.Prune belly syndrome with pouch colon Garge et al. 43Copyright © Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited.hindgut during early intrauterine development couldaccount for pouch colon malformation [1]. We feel thatthe presence of abnormally long mesentery and nonfixa-tion of colon to posterior abdominal wall may lead toantenatal vascular events that may have led to pouchcolon formation. However, it seems more plausible thatboth mesodermal arrest and vascular events early inembryogenesis may be responsible.Pouch colon association with PBS can be associatedwith poorer prognosis than an isolated PBS. Thisassociation can have both antenatal and postnatalFig. 2Erect abdominal radiograph and CTPL showing pouch colon and high ARM. ARM, anorectal malformation; CTPL, cross table pelvic lateral (view).Fig. 3Intraoperative images.44 Annals of Pediatric Surgery 2015, Vol 11 No 1Copyright © Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited.concerns. Antenatally, the pouch colon adds to addeddistension of abdomen, both physically and also due toits contribution to urethral obstruction. The thickmeconium in the bladder coming through the colovesicalfistula or the angulations of the bladder neck due to thefistula may hinder micturition. This may add toassociated oligohydramnios and lead to more severepulmonary hypoplasia. Furthermore, pouch colon hasbeen found to be associated always with vesicouretericreflux, which is a cardinal part of PBS too [1]. Postnatally,after excision of pouch, these patients have high chancesof developing neurogenic bladder. There can be threecauses for this: first, the overdistended bladder due tooutlet obstruction; second, surgical dissection posterior tothe bladder to reach and ligate wide colovesical fistula;and third, the suture line of the wide colovesical fistula.The patients of PBS may need a supravesical diversion(ureterostomies), cutaneous vesicostomy, anterior ure-thral reconstruction, reduction cystoplasty, ureteral re-construction, and reconstruction of anterior abdominalwall [1,2,6,7]. In patients with pouch colon and mega-lourethra, another issue during surgery is regardingurinary diversion [6]. Presence of a cutaneous vesicost-omy and colostomy nearby may be a cause of urinary tractinfections. Thus, in this case, we did a suprapubiccystostomy, which was placed away from the stoma.ConclusionThus, the association of pouch colon and megalourethrawith PBS has an overall poorer prognosis. The anomalymay be a result of unique combination of vascular eventsand mesodermal defects early in embryogenesis. Properpreoperative assessment and explanation of poor prog-nosis, intraoperative surgical acumen for posterior bladderdissection, placement of vesicostomies and colostomies,and postoperative expert nursing care are major pre-requisites for proper management of this rare association.AcknowledgementsConflicts of interestThere are no conflicts of interest.References1 Bangroo AK, Tiwari S, Khetri R, Sahni M. Congenital pouch colon with prunebelly syndrome and megalourethra. Pediatr Surg Int 2005; 21:474–477.2 Yadav K. Short colon associated with the prune belly syndrome. Int Surg1979; 64 (No. 4):83–85.3 Raghavan M, Haripriya U, Pradeep PV. Rare association of prune bellysyndrome with pouch colon. Pediatr Health Med Ther 2011; 2011:9–12.4 Short KL, Groff DB, Cook L. The concomitant presence of gastroschisis andprune belly syndrome in a twin. J Pediatr Surg 1985; 20:186–187.5 Walker J, Prokurat AI, Irving IM. Prune belly syndrome associated withexomphalos and anorectal agenesis. J Pediatr Surg 1987; 22:215–217.6 Mahajan JK Ojha S, Rao KL. Prune-belly syndrome with anorectalmalformation. Eur J Pediatr Surg 2004; 14:351–354.7 Baba AA, Hussain SA, Shera AH, Patnaik R. Prune belly syndrome withpouch colon and absent dermatome. Afr J Paediatr Surg 2010; 7:25–27.8 Rao KLN, Menon P. Congenital pouch colon associated with anorectalagenesis (pouch colon syndrome). Pediatr Surg Int 2005; 21:125–126.Fig. 4Dye study showing large bladder and scaphoid megalourethra.Prune belly syndrome with pouch colon Garge et al. 45Copyright © Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited.

Prune belly syndrome with pouch colon with scaphoid megalourethra: A newer embryological and prognostic perspective

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Prune belly syndrome with pouch colon with scaphoid megalourethra: A newer embryological and prognostic perspective

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