Jump to ContentJump to Main Navigation
Show Summary Details
More options …

Open Medicine

formerly Central European Journal of Medicine

Editor-in-Chief: Darzynkiewicz, Zbigniew

IMPACT FACTOR 2018: 1.221

CiteScore 2018: 1.01

SCImago Journal Rank (SJR) 2018: 0.329
Source Normalized Impact per Paper (SNIP) 2018: 0.479

ICV 2018: 156.09

Open Access
See all formats and pricing
More options …
Volume 8, Issue 3


Volume 10 (2015)

Unilateral renal agenesis in a neonate with congenital diaphragmatic hernia

Kyoung Han
  • Department of Pediatrics, Jeju National University Hospital, Aran 13gil 15, Jeju city, Jeju Special Self-Governing Province, 690-767, Korea
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Kwang Kim
  • Department of Surgery, Jeju National University Hospital, Aran 13gil 15, Jeju city, Jeju Special Self-Governing Province, 690-767, Korea
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Jee Chang
  • Department of Thoracic Surgery, Jeju National University Hospital, Aran 13gil 15, Jeju city, Jeju Special Self-Governing Province, 690-767, Korea
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Young Kim
  • Department of Pediatrics, Jeju National University Hospital, Aran 13gil 15, Jeju city, Jeju Special Self-Governing Province, 690-767, Korea
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
Published Online: 2013-04-17 | DOI: https://doi.org/10.2478/s11536-013-0152-y


Congenital diaphragmatic hernia (CDH) is a rare and severe disorder with a high mortality rate among infants. Unilateral renal agenesis (URA) is a relatively common congenital urinary malformation. Here, we present the case of a newborn infant with left CDH associated with ipsilateral renal agenesis. The male patient was born weighing 3.850 g through normal spontaneous vaginal delivery at 38 weeks and 6 days of gestational age at a maternity hospital. He was transferred to our neonatal intensive care unit due to respiratory distress with tachypnea, grunting and cyanosis after birth. A chest radiography indicated parts of the bowel in the thoracic cavity, consistent with CDH. Renal ultrasonography indicated no kidney structure on the left side and a 5.6 cm right kidney with normal echogenicity. Repair of the diaphragmatic hernia was performed three days after birth. Most of the colon, small bowel, stomach and spleen were located in the left pleural cavity, but the left kidney was not seen. Subsequent dimercaptosuccinic acid scintigraphy indicated non-visualized functional cortical uptake in the left kidney on day 13 after birth. Thus, we report the successful treatment of a patient with CDH accompanied by URA.

Keywords: Congenital diaphragmatic hernia; Bochdalek hernia; Unilateral renal agenesis

  • [1] Maheshwari A., Carlo W.A., Diaphragmatic hernia, In: Kliegman R.M., Stanton B.F., St.Geme J.W., Schor N.F., Behrman R.E. (Eds.), Nelson textbook of pediatrics, 19th ed., Elsevier, 2011 Google Scholar

  • [2] Zaiss I., Kehl S., Link K., Neff W., Schaible T., Sütterlin M., et al., Associated malformations in congenital diaphragmatic hernia. Am. J. Perinatol., 2011, 28, 211–218 http://dx.doi.org/10.1055/s-0030-1268235CrossrefGoogle Scholar

  • [3] Skari H., Bjornland K., Haugen G., Egeland T., Emblem R., Congenital diaphragmatic hernia: a meta-analysis of mortality factors. J. Pediatr. Surg., 2000, 35, 1187–1197 http://dx.doi.org/10.1053/jpsu.2000.8725CrossrefGoogle Scholar

  • [4] Siebert J.R., Benjamin D.R., Juul S., Glick P.L., Urinary tract anomalies associated with congenital diaphragmatic defects. Am. J. Med. Genet., 1990, 37, 1–5 http://dx.doi.org/10.1002/ajmg.1320370102CrossrefGoogle Scholar

  • [5] Doroshow L.W., Abeshouse B.S., Congenital unilateral solitary kidney: report of 37 cases and a review of the literature. Urol. Surv., 1961, 11, 219–229 Google Scholar

  • [6] Stoll C., Alembik Y., Roth M.P., Dott B., Sauvage P., Risk factors in internal urinary system malformations. Pediatr. Nephrol., 1990, 4, 319–323 http://dx.doi.org/10.1007/BF00862508CrossrefGoogle Scholar

  • [7] Barakat A.J., Drougas J.G., Occurrence of congenital abnormalities of kidney and urinary tract in 13,775 autopsies. Urology, 1991, 38, 347–350 http://dx.doi.org/10.1016/0090-4295(91)80150-6CrossrefGoogle Scholar

  • [8] Parikh C.R., McCall D., Engelman C., Schrier R.W., Congenital renal agenesis: case-control analysis of birth characteristics. Am. J. Kidney Dis., 2002, 39, 689–694 http://dx.doi.org/10.1053/ajkd.2002.31982CrossrefGoogle Scholar

  • [9] Kim J.W., Lee S.E., Jung Y.H., Han K.H., Lee H.K., Kang H.G., et al., Clinical characteristics and associated anomalies in children with solitary kidney. J. Korean Soc. Pediatr. Nephrol., 2010, 14, 42–50 http://dx.doi.org/10.3339/jkspn.2010.14.1.42CrossrefGoogle Scholar

  • [10] Klaassens M., van Dooren M., Eussen H.J., Douben H., den Dekker A.T., Lee C., et al., Congenital diaphragmatic hernia and chromosome 15q26: determination of a candidate region by use of fluorescent in situ hybridization and array-based comparative genomic hybridization. Am. J. Hum. Genet., 2005, 76, 877–882 http://dx.doi.org/10.1086/429842CrossrefGoogle Scholar

  • [11] Skarsgard E.D., Harrison M.R., Congenital diaphragmatic hernia: the surgeon’s perspective. Pediatr. Rev., 1999, 20, e71–78 http://dx.doi.org/10.1542/pir.20-10-e71CrossrefGoogle Scholar

  • [12] Bollmann R., Kalache K., Mau H., Chaoui R., Tennstedt C., Associated malformations and chromosomal defects in congenital diaphragmatic hernia. Fetal. Diagn. Ther., 1995, 10, 52–59 http://dx.doi.org/10.1159/000264193CrossrefGoogle Scholar

  • [13] Losty P.D., Vanamo K., Rintala R.J., Donahoe P.K., Schnitzer J.J., Lloyd D.A., Congenital diaphragmatic hernia—Does the side of the defect influence the incidence of associated malformations? J. Pediatr. Surg., 1998, 33, 507–510 http://dx.doi.org/10.1016/S0022-3468(98)90099-9CrossrefGoogle Scholar

  • [14] Campbell M.F., Congenital absence of one kidney: Unilateral renal agenesis. Ann. Surg., 1928, 88, 1039–1044 http://dx.doi.org/10.1097/00000658-192812000-00009CrossrefGoogle Scholar

  • [15] Cascio S., Paran S., Puri P., Associated urological anomalies in children with unilateral renal agenesis. J. Urol., 1999, 162, 1081–1083 http://dx.doi.org/10.1016/S0022-5347(01)68074-1CrossrefGoogle Scholar

  • [16] Schreuder M.F., Unilateral anomalies of kidney development: why is left not right? Kidney Int., 2011, 80, 740–745 http://dx.doi.org/10.1038/ki.2011.204CrossrefWeb of ScienceGoogle Scholar

  • [17] Robson W., Leung A., Rogers R., Unilateral renal agenesis. Adv. Pediatr., 1995, 42, 575–592 Google Scholar

  • [18] Antonius T., van Bon B., Eggink A., van der Burgt I., Noordam K., van Heijst A., Denys-Drash syndrome and congenital diaphragmatic hernia: another case with the 1097G > A(Arg366His) mutation. Am. J. Med. Genet. A., 2008, 146A, 496–499 http://dx.doi.org/10.1002/ajmg.a.32168CrossrefGoogle Scholar

  • [19] Scott D.A., Cooper M.L., Stankiewicz P., Patel A., Potocki L., Cheung S.W., Congenital diaphragmatic hernia in WAGR syndrome. Am. J. Med. Genet. A., 2005, 134, 430–433 http://dx.doi.org/10.1002/ajmg.a.30654CrossrefGoogle Scholar

  • [20] Slavotinek A.M., Single gene disorders associated with congenital diaphragmatic hernia. Am. J. Med. Genet. C. Semin. Med. Genet., 2007, 145C, 172–183 http://dx.doi.org/10.1002/ajmg.c.30125CrossrefGoogle Scholar

  • [21] Pritchard-Jones K., Fleming S., Davidson D., Bickmore W., Porteous D., Gosden C., et al., The candidate Wilms’ tumour gene is involved in genitourinary development. Nature, 1990, 346, 194–197 http://dx.doi.org/10.1038/346194a0CrossrefGoogle Scholar

  • [22] Nordenskjöld A., Tapper-Persson M., Anvret M., No evidence of WT1 gene mutations in children with congenital diaphragmatic hernia. J. Pediatr. Surg., 1996, 31, 925–927 http://dx.doi.org/10.1016/S0022-3468(96)90412-1Google Scholar

  • [23] Itoh A., Miyabayashi T., Ohno M., Sakano S., Cloning and expressions of three mammalian homologues of Drosophila slit suggest possible roles for Slit in the formation and maintenance of the nervous system. Brain Res. Mol. Brain Res., 1998, 62, 175–186 http://dx.doi.org/10.1016/S0169-328X(98)00224-1CrossrefGoogle Scholar

  • [24] Piper M., Georgas K., Yamada T., Little M., Expression of the vertebrate Slit gene family and their putative receptors, the Robo genes, in the developing murine kidney. Mech. Dev., 2000, 94, 213–217 http://dx.doi.org/10.1016/S0925-4773(00)00313-0CrossrefGoogle Scholar

  • [25] Liu J., Zhang L., Wang D., Shen H., Jiang M., Mei P., et al., Congenital diaphragmatic hernia, kidney agenesis and cardiac defects associated with Slit3-deficiency in mice. Mech. Dev., 2003, 120, 1059–1070 http://dx.doi.org/10.1016/S0925-4773(03)00161-8CrossrefGoogle Scholar

About the article

Published Online: 2013-04-17

Published in Print: 2013-06-01

Citation Information: Open Medicine, Volume 8, Issue 3, Pages 358–361, ISSN (Online) 2391-5463, DOI: https://doi.org/10.2478/s11536-013-0152-y.

Export Citation

© 2013 Versita Warsaw. This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License. BY-NC-ND 3.0

Comments (0)

Please log in or register to comment.
Log in