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Chromosome 16 Mutations and Congenital
Genitourinary Malformations
Daniel Faustin, Morgan Barlow*, Anuja Trivedi and Naila Ferris
Wyckoff Heights Medical Center, USA
Introduction
Posterior urethral valve (PUV) disorder is an obstructive
anomaly of the genitourinary tract. Found only in males, this
condition is caused by an obstructing membrane in the posterior
valve of the urethra. During the early stages of embryogenesis, the
most caudal end of the wolffian duct is absorbed into the primitive
cloaca, forming the posterior urethral folds [1]. In fetuses with
PUV, the development of these structures is arrested resulting in
fusion of these primitive folds, leading to an obstruction of the
outflow of urine. This pathologic finding presents on a spectrum
of varying degrees, wherein the obstruction may cause only mild
symptoms and present as late as early infancy or cause severe and
irreversible damage during early fetal development. In this case,
we investigate the possible association between chromosome 16
and the development of the renal system, and the potential for
congenital malformation in the setting of genetic alteration.
Case
We describe a 30-year-old G1P0 with a fetus that has multiple
congenital anomalies and a chromosome 16 duplication at 16q24.2.
This patient initially presented to our institution at 12 weeks
gestation without any obstetrical complaints. She had no past
medical or surgical history and no family history of congenital or
chromosomal abnormalities. Initial sonogram revealed a viable
fetus. Prenatal labs were found to be within normal limits, aside
from a positive quantiferon gold.
Fetal anatomy survey performed at 20 weeks gestation revealed
multiple fetal anomalies, including bilateral cystic dilation of the
pelvic and calyceal spaces, bilateral hydronephrosis, an enlarged
urinary bladder, and oligohydramnios. Several sonographic
images suggested a dilated posterior urethra, demonstrated by
the pathognomonic “keyhole appearance”. PUV was suspected
at this time and close follow-up was recommended. The patient
was evaluated again at 22 weeks gestation, and a targeted fetal
ultrasound revealed worsening oligohydramnios with persistent
distention of the urinary system. These findings confirmed the
suspicion of posterior urethral valve disorder.
Figure 1: Fetal bladder with “keyhole” appearance.
Figure 2: Fetal bladder with “keyhole” appearance, alternate view.
The patient was counseled on the findings and expectations
of this anomaly during the anatomy scan and genetic testing was
offered. Amniocentesis showed elevated AFP (23.1 mcg/mL)
*Corresponding author: Morgan Barlow, Wyckoff Heights Medical Center,
Department of OB/Gyn, USA
Received Date: November 13, 2018
Published Date: November 20, 2018
ISSN: 2641-6247 DOI: 10.33552/WJGWH.2018.01.000511
World Journal of
Gynecology & Women’s Health
Case Report Copyright © All rights are reserved by Morgan Barlow
This work is licensed under Creative Commons Attribution 4.0 License WJGWH.MS.ID.000511.