Cloacal dysgenesis sequence with bilateral renal agenesis, bladder agenesis, pulmonary hypoplasia and left choanal atresia: A case report

Esra Ozcakir, Sefer Tolga Okay, Ipek Guney Varal, Mete Kaya

Abstract


A case of posterior urethral valve is described with multiple urethroperineal fistulas. This association is extremely rare. The crucial point in the diagnosis is to distinguish these fistulas from the urethral duplications that actually guide the treatment. The presence of these fistulas in patients with posterior urethral valve may have a beneficial effect on renal function as it reduces the pressure on the bladder. On the other hand, incomplete valve ablation may contribute to the recurrence of the fistula.


Keywords


Cloacal dysgenesis sequence, bilateral renal agenesis, bladder agenesis, pulmonary hypoplasia, left choanal atresia.

Full Text:

PDF

References


Bargaje A, Yerger JF, Khouzami A, et al. Cloacal dysgenesis sequence. Ann Diagn Pathol. 2008; 12(1):62–66.

Escobar LF, Weaver DD, Bixler D, et al. Urorectal septum malformation sequence: report of six cases and embryological analysis. Am J Dis Child. 1987; 141(9):1021 - 24.

Sahinoglu Z, Mulayim B, Ozden S, et al. The prenatal diagnosis of cloacal dysgenesis sequence in six cases: can the termination of pregnancy always be the first choice? Prenat Diagn. 2004; 24(1):10–16.

Jegadeesh S, Mahajan JK. Cloacal dysgenesis sequence with bilateral renal agenesis and normal pulmonary development in twin pregnancy. BMJ Case Rep. 2016; 2016:bcr2015214130.

Pauli RM. Lower mesodermal defects: a common cause of fetal and early neonatal death. Am J Med Genet. 1994; 50(2):154–72.

Phelan JP, Smith CV, Broussard P, et al. Amniotic fluid volume assessment with the four-quadrant technique at 36–42 weeks’ gestation. J Reprod Med. 1987; 32(7):540–42.

McFadden DE, Pantzar JT. Genital system. In Developmental Pathology of The Embryo and Fetus, Dimmick JE, Kalousek DK (eds). J.B. Lippincott: Philadelphia; 1992. pp. 605–624.

Qureshi F, Jacques SM, Yaron Y et al. Prenatal diagnosis of cloacal dysgenesis sequence: differential diagnosis from other forms of fetal obstructive uropathy. Fetal Diagn Ther. 1998; 13(2):69–74.

Yanai T, Urita Y, Amagai T, et al. Long-term survival with cloacal dysgenesis sequence. Pediatr Surg Int. 2012; 28(1):107–10.

Kanamori Y, Iwanaka T, Nakahara S et al. Survival in a neonate with complete urorectal septum malformation sequence after fetal vesico-amniotic shunting for a prominently dilated cloaca. Fetal Diagn Ther. 2008; 24(4):458–61.

Kramer RL, Johnson MP, Qureshi F. Concordance for cloacal dysgenesis. Fetal Diagn Ther. 1997; 12(5):279 - 82.

Kaya M, Sancar S, Ozcakir E, et al. Omphalocele, exstrophy of cloaca, imperforate anus and spinal defect (OEIS Complex): A case report. Pediatr Urol Case Rep. 2015; 2(4):17-24.

Chaubal N, Dighe M, Shah M, et al. Calcified meconium: an important sign in the prenatal sonographic diagnosis of cloacal malformation. J Ultrasound Med. 2003; 22(7):727-30.

Dannull K, Sung J. Cloacal dysgenesis diagnosis by prenatal ultrasound and MRI. Pediatr Radiol. 2014; 44(2):230–33.

Patil SJ, Phadke SR. Urorectal septum malformation sequence: ultrasound correlation with fetal examination. Indian J Pediatr. 2006; 73(4):287–93.

Mukhtar RA, Baskin LS, Stock PG et al. Long-term survival and renal transplantation in a monozygotic twin with cloacal dysgenesis sequence. J Pediatr Surg. 2009; 44(12):e31–e33.




DOI: http://dx.doi.org/10.14534/j-pucr.2020258451

Refbacks

  • There are currently no refbacks.


Indexing/Abstracting

DOAJ, Tübitak/Ulakbim Turkish Medical Database, Academic OneFile, Health Reference Center Academic, US National Library of Medicine (NLM) Catalog, ICI Journals Master List (Index Copernicus), OCLC-WorldCat, Genamics-JournalSeek, J-Gate, Google Scholar, Ulrichsweb, CNKI Scholar, NAVER Academic, Research Bible, Cross Ref, EZB (Electronic Journals Library), JournalTOCs, HINARI, Norwegian Social Science Data Services (NSD), Turkiye Citation Index, SCIARY (Worldwide Science Educational Library), BASE (Bielefeld Academic Search Engine), NAIST, ICMJE, PubsHub, AcademicKeys, OAJI, OpenAIRE, Turk Medline, Publons, ROAD, SHERPA-ROMEO, SJFinder, Pubget, SciLit, Journal Finder, NYU Health Sciences Library, BIU Santé, Science Library Index, Ingenta Connect

Index Copernicus Value (ICV) 2018: 100.00

Pediatric Urology Case Reports journal does not charge a fee for submission, processing of manuscripts through peer-reviews, editing or publication of the articles.

Creative Commons License
Pediatric Urology Case Reports is licensed under a Creative Commons Attribution 4.0 International License.


eztekno