PEDIATRICS Vol. 105 No. 5
May 2000,
p. e61
ELECTRONIC ARTICLE:
A Prospective Search for Urinary Tract Abnormalities in
Infants With Isolated Preauricular Tags
David Kohelet, MD and
Eliana Arbel, MD
From the Department of Neonatology, Edith Wolfson Medical
Center and Sackler School of Medicine, Tel Aviv University, Tel Aviv,
Israel.
 |
ABSTRACT |
Objective. To determine whether
isolated preauricular tags are associated with urinary tract
abnormalities.
Methodology. Seventy consecutive infants with isolated
preauricular tags were examined by ultrasonography for urinary tract
abnormalities on day 3 or 4 of life between January 1993 and August
1999, after parental consent and ethics approval. Karyotype analysis
was conducted in all infants with urinary tract abnormalities. The
study group was compared with a control group of 69 infants without
preauricular tags hospitalized during the same period. The control
group consisted of infants who underwent urinary tract ultrasonography
as part of an investigation for persistent regurgitation and/or
vomiting associated with cyanotic spells.
Results. Urinary tract abnormalities were detected in 6 infants with isolated preauricular tags (6/70; 8.6%). Types of
anomalies were as follows: hydronephrosis in 5 cases and horseshoe
kidney in 1 case. The causes of hydronephrosis were ureteropelvic
junction obstruction in 3 cases and vescicoureteric reflux in 2 cases. None of the infants in the control group had such abnormalities. All infants with urinary tract abnormalities had normal chromosomes. No
statistically significant differences existed between groups concerning
birth weight, gestational age, intrauterine growth, and male-to-female
ratio.
Conclusions. This study suggests a significant prevalence
of urinary tract abnormalities in infants with preauricular tags. We
recommend, therefore, that urinary tract ultrasonography be conducted
in the routine assessment of infants with isolated preauricular
tags.
Key words:
infant,
preauricular tags,
urinary tract
anomalies,
ultrasonography.
The association between ear malformations and renal
anomalies was recognized as early as 1957.1
Preauricular tags, protuberant fleshy papules located anterior to the
tragus or just anterior to the crus of the helix, are commonly found in
the neonatal period and are considered to be of minor clinical
importance.2 However, several studies have demonstrated
that preauricular tags and pits may be associated with a higher
incidence of conductive and/or sensorineural hearing impairment3,4 and urinary tract
abnormalities.3,5,6 The cause of such associations,
whether hereditary or not, has not been determined. The association
between isolated preauricular tags and urinary tract abnormalities has
not been thoroughly investigated.
We conducted a cross-sectional study designed to assess whether infants
with isolated preauricular tags may be at risk for such abnormalities.
| |
METHODS |
All infants with preauricular tags born between January 1993 and
August 1999 at Edith Wolfson Medical Center in Holon, Israel, were
screened. An attending neonatologist examined all screened infants for
additional congenital anomalies. Infants were excluded from the study
if 1 or more of the following were present: 1) associated ear anomalies
or other congenital anomalies; 2) a family history of renal anomalies
in parents or siblings; and 3) parental refusal of informed consent.
The investigational review board at our institution approved the study,
and signed parental consent was obtained.
Ultrasonography of the urinary tract was performed on all eligible
infants on day 3 or 4 of life. The same operator, who was unaware of
the clinical course of each infant, examined all infants. When
required, other imaging modalities (radionuclide scintigraphy and
voiding cystography) were used subsequently at the pediatric follow-up
clinic to provide accurate diagnosis and functional information.
Voiding cystography was conducted in all infants to rule out
vescicoureteric reflux, and radionuclide scintigraphy was performed to
diagnose ureteropelvic junction obstruction and assess renal
function.
Karyotype analysis was conducted in all infants with urinary tract
abnormalities. The study group of 70 infants with isolated preauricular
tags was compared with a control group of infants without preauricular
tags. The control group consisted of 69 infants who underwent
ultrasonography of the urinary tract after day 5 of life as part of an
investigation for persistent regurgitation and/or vomiting associated
with cyanotic spells. Infants from both groups were hospitalized at the
same time period. Demographic and clinical data were collected for each
infant in both groups (Table 1 and Table
2). Hydronephrosis was defined as
pyelocaliectasis on renal ultrasonography. The degree of
pyelocaliectasis was graded as 0 to 4 according to the classification
of the Society for Fetal Urology.7
Comparisons between variables were conducted using the t
test, for independent samples. A level of significance was set at P < .05. The 
2 test was used
to compare proportions between groups. Data are presented as the
mean ± the standard deviation.
| |
RESULTS |
During the study, 17 632 infants were born at our
institution. Based on clinical indications and prenatally suspected
findings, urinary tract abnormalities were detected by ultrasonography
in 37 infants (37/17 632; .2%; 2/1000 births).
Preauricular tags were detected in 83 infants. In 12 infants,
preauricular tags were associated with ear anomalies as follows:
atretic external canal (n = 1); accessory auricle
(n = 1); dysplastic auricle (n = 2);
and preauricular pits (n = 12). In 1 infant with an
isolated preauricular tag, there was a family history of polycystic
kidney disease. The study group thus totaled 70 infants with isolated preauricular tags. None of the infants with isolated preauricular tags
had family members with such malformations. The overall incidence of
isolated preauricular tags in our population was 4.0/1000 (71/17 632). Thirty-six isolated preauricular tags were left-sided (51%), 23 were
right-sided (33%), and 11 were bilateral (16%). Characteristics of
infants with urinary tract abnormalities detected by
ultrasonography are demonstrated in Table 1. Urinary tract
ultrasonography and other imaging modalities disclosed that 6 of 70 infants had abnormalities, 1 infant had bilateral grade 2 hydronephrosis secondary to grade 2 bilateral vescicoureteric reflux, 2 infants had grade 2 right-sided hydronephrosis secondary to
ureteropelvic junction obstruction, 1 infant had grade 3 right-sided
hydroureternephrosis secondary to grade 4 bilateral vesicoureteric
reflux, 1 infant had a horseshoe kidney, and 1 infant had grade 2 left-sided hydronephrosis secondary to ureteropelvic junction
obstruction. No relationship was found between urinary tract
abnormality and the side of preauricular tags. At recent evaluations,
none of the infants had reached a grade of obstruction that warranted
surgical intervention. Our study documented abnormalities in the
urinary tract of 6 (8.6%) of the 70 infants with isolated preauricular
tags. None of the infants in the control group had urinary tract
abnormalities. All infants with urinary tract abnormalities had normal
chromosomes. None of the infants in both groups evidenced other
associated congenital anomalies (on clinical examination).
Characteristics of infants with isolated preauricular tags and infants
of the control group are shown in Table 2. The mean birth weight of the
study group was 3383 ± 394 g, and the mean gestational age
was 39.1 ± 1.7 weeks. The intrauterine growth of all infants in
the study group was appropriate and the female-to-male ratio was 1:1.2.
No sta-tistically significant differences between the 2 groups
concerning birth weight, gestational age, and male-to-female ratio were
detected. However, the difference between the incidence of urinary
tract abnormalities was statistically significant (P < .02).
| |
DISCUSSION |
The major finding of this study was that preauricular tags are
associated with urinary tract abnormalities. The incidence of isolated
preauricular tags detected in the study population (4/1000) is
consistent with other reports.3,4 This study documents a
prevalence of 8.6% of urinary tract abnormalities in infants with
isolated preauricular tags. The prevalence of such abnormalities in
this group is higher compared with the prevalence in a control group of
infants (P < .02). Using ultrasonography as an initial
diagnostic tool, we defined the prevalence of hydronephrosis in infants
with preauricular tag. However, because ultrasonography is not a
reliable tool for diagnosing vescicoureteric reflux in the neonatal
period, we may assume that even more infants with preauricular tags
might have had reflux if voiding cystography had been performed, given
that 2 infants had vescicoureteric reflux.
The ratio between the number of infants with urinary tract
abnormalities born at our institution and the total number of births during the study is similar to the incidence of .28% detected by
routine prenatal ultrasonographic screening of 11 986 pregnant women
in Sweden.8
Melnick et al9 described an association between ear
malformations (gross ear deformities), branchial anomalies, and renal
dysplasia and coined the term "branchio-oto-renal syndrome." It has
been recently demonstrated that this syndrome is caused by mutations in
the EYA1 gene.10
Although several investigators have reported an association between ear
and urinary tract abnormalities, the ear abnormalities consisted of
gross ear deformities.1,11 The association between
isolated preauricular tags and urinary tract abnormalities has not been
supported by other studies. Kugelman et al,4 who conducted
ultrasonography in 23 infants with preauricular tags and pits, did not
find associated abnormalities of the urinary tract. Conceivably, the
discrepancies with our findings could be attributed to the small sample
size of their population. A family history of urinary tract
abnormalities could not account for such differences, because none of
the infants in our study had a positive family history for urinary
tract abnormalities.
The association between isolated preauricular tags and urinary tract
abnormalities is difficult to explain based on localized concomitant
disorders of blood flow and/or cell migration. Gorlin et
al12 proposed to lump together several conditions including facioauriculo-vertebral syndrome, hemifacial micrognatia, oto-mandibular dysostosis, Goldenhar syndrome, the first branchial arch
anomalies, and the first and second branchial arches anomalies, proposing the term "oculo-auriculo-vertebral spectrum." The
pathogenesis of the above spectrum is unknown. However, the wide
variety of malformations in the above spectrum, including pulmonary and
renal abnormalities would suggest a disorder of
blastogenesis.13 Findings of this study support the
hypothesis that the association between minor ear abnormalities (namely
preauricular tags) and urinary tract abnormalities could be an
additional condition of the oculo-auriculo-vertebral spectrum.
Therefore, we recommend that urinary tract ultrasonography be conducted
on all infants with isolated preauricular tags.
| |
AKNOWLEDGMENTS |
We are grateful for the assistance of Dr Michaela Wizlling from
the Pediatric Radiology Unit at our institution, and for the helpful
suggestions of Dr Mona Boaz.
| |
FOOTNOTES |
Received for publication Oct 18, 1999; accepted Dec 20, 1999.
Reprint requests to (D.K.) Department of Neonatology, Edith
Wolfson Medical Center, 58100 Holon, Israel. E-mail:
kdavid{at}post.tau.ac.il
| |
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Pediatrics (ISSN 0031 4005). Copyright ©2000 by the American Academy of Pediatrics
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Abstract
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