AM ER IC AN JOURNAL OF OT OLARYNGOLOGY–H E A D A N D NE CK M E D IC IN E A ND S U RGE RY 3 7 (2 0 1 6) 2 63–2 6 4

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“Collodion baby”: A unique challenge for newborn

hearing screening☆,☆☆,★,★★,☆☆☆,★★★

Kayla M. Jasper, BS a , Philip Gaudreau, MD b , Todd V. Cartee, MD c ,

Brian K. Reilly, MD, FACS, FAAP b,⁎
a
Bachelor of Science in Biology, The George Washington University, School of Medicine and Health Sciences, 2300 I St NW, Washington DC
b
Division of Otolaryngology, Children’s National Medical Center, 111 Michigan Ave NW, Washington, DC
c
Department of Dermatology, Penn State Hershey Dermatology, 500 University Drive, UPC 1, Suite 100, Hershey, PA

ARTI CLE I NFO A BS TRACT

Article history: We present an infant with collodion membrane who had an obstructed external auditory
Received 3 November 2015 canal, causing the infant to fail her newborn hearing screen (otoacoustic emissions)
bilaterally. An auditory brainstem response (ABR) test was deferred due to the reported
increased risk of infections in these babies. Meticulous but gentle debridement of the
membranes on the external auditory canal, using a combination of otic drops (ofloxacin),
emollients (baby oil/mineral oil), and suctioning, permitted the infant to ultimately pass
otoacoustic emissions bilaterally and subsequent serial audiograms.
© 2016 Elsevier Inc. All rights reserved.

1. Case presentation
A female was born at 39 weeks gestation covered in a shiny,
pink, taut, translucent membrane with edematous feet and
hands. These findings are consistent with a collodion baby
(Fig. 1) [1]. The infant was delivered by an uncomplicated
cesarean section with APGARS of 9 and 9, at 1 and 5 min
respectively. The parents had no family history of ichthyosis.
The taut skin did not cause any peripheral vascular insufficien-
cy or restrictive respiratory compromise.
Ophthalmology consult noted slight ectropion of the
eyelids causing mild lagophthalmos. We observed external
auditory canal stenosis with a membranous layer blocking
the meatus. The patient’s tympanic membranes were unable
to be evaluated due to external auditory canal stenosis, and
the infant failed otoacoustic emissions bilaterally (Fig. 2). An
auditory brainstem response test (ABR) was not performed
because the risk of infection contraindicated the procedure
[2]. The increased risk of infection is secondary to the
augmented likelihood of the membrane adhering to the
electrode and denuding the epidermis upon removal.
A layer of Aquaphor® applied to the patient shortly after
birth relieved some of the tautness. Initial supportive treat-
ment also consisted of 60% humidified isolet, continued
application of petroleum-based emollients over her skin,
and erythromycin and lacrilube ointment to her eyes. Caloric
and electrolyte supplementation and close monitoring for
body temperature and signs of sepsis were continued.
At twenty days of life, the infant still presented with a
thickened hyperkeratotic shiny membrane covering her face,

☆
Disclosures:
☆☆
Competing interests: None.
★
Sponsorship: None.
★★
Funding Source: None.
☆☆☆
Each of the authors has contributed to, read and approved the manuscript.
★★★
This manuscript has not been previously published, nor is it under consideration for publication elsewhere.
⁎ Corresponding author. Division of Otolaryngology, Children’s National Medical Center, 111 Michigan Ave NW, Washington, DC.
E-mail addresses: kmjasper@gwu.edu (K.M. Jasper), pgaudrea@childrensnational.org (P. Gaudreau), todd.cartee@gmail.com
(T.V. Cartee), breilly@childrensnational.org (B.K. Reilly).

http://dx.doi.org/10.1016/j.amjoto.2015.12.009
0196-0709/© 2016 Elsevier Inc. All rights reserved.
264 AM ER IC AN JOURNAL OF OT OLA RYNGOLOGY–H E A D A N D NE CK M E D ICI N E AN D S U RGE RY 3 7 (2 0 1 6) 2 63 –2 6 4
Fig. 1 – Collodion membrane seen covering the patient’s body.
bilateral ears, neck, trunk, and extremities while fragments of
the membrane elements were beginning to slough off of her
trunk secondary to desquamation that occurs from underly-
ing infant movement. As the membrane shed, she was
diagnosed with underlying ichthyosis. She was treated with
a prolonged regimen of mineral oil and liquid paraffin drops
for over a month along with intermittent suctioning of the
external auditory canal in clinic as the shedding continued.
Thereafter, the infant was able to pass otoacoustic emissions
bilaterally and subsequent serial audiograms. Currently, she
is progressing normally along her growth curve and has
resolved auditory canal stenosis with no hearing difficulty or
predisposition for otitis media/otitis externa.
2. Discussion
The phenomenon of “collodion baby” is uncommon. Collodi-
on baby is not a diagnosis but a description of a clinical
presentation common to many forms of ichthyosis. Ultimate-
ly, phenotype development and genetic testing are required
to confirm the specific diagnosis. Genetic testing revealed the
baby to have two known pathogenic mutations in
transglutaminase 1 that have been associated with autosomal
recessive congenital ichthyosis. The ichthyoses are a dispa-
rate group of genetic disorders of epidermal cornification,
which all share impairment of normal skin desquamation.
This typically leads to epidermal thickening, scaling (reten-
tion hyperkeratosis), and cutaneous inflammation. Pheno-
types range in severity with some being quite mild. However,
the collodion baby presentation is usually associated with a
severe autosomal recessive congenital ichythyosis, such as
lamellar ichthyosis, harlequin ichthyosis or congenital
ichthyosiform erythroderma. In addition, collodion babies
are at risk for respiratory difficulties, electrolyte abnormali-
ties, dehydration, and infection.
Only about 10% of collodion babies spontaneously resolve
within a few weeks with little, if any, long-term sequelae —
the so called “self-healing” collodion baby [3]. In most cases,
collodion babies will have lifelong cutaneous dysfunction
Fig. 2 – External auditory ear canal obstructed due to
collodion membrane.
with different manifestations dependent on the specific
underlying ichthyosis. Collodion babies commonly suffer
from external auditory canal stenosis due to the hyperkera-
totic membrane, which covers the ear canal [4].
Collodion babies are treated with aggressive supportive care.
A regimen of emollients is initiated immediately, ideally within
a humidified incubator. In particular, initial treatment should
rely on desquamation through emollients such as liquid
paraffin, Aquaphor® or mineral oil as opposed to manual
debridement. Keratolytic agents are generally avoided due to
the poor cutaneous barrier and potential toxicities [5]. After the
membrane sheds, the ear canal can often be debrided revealing
a normal caliber canal with normal tympanic anatomy as was
the case with our patient. This is best achieved with gentle
suctioning in the clinic setting, under microscopy with the baby
carefully restrained, so as to not injure delicate ear canal skin or
the tympanic membrane. Hearing loss is typically conductive
and transient in nature, and newborn screening should be
postponed until after the desquamation of the entire ear canal.
Subsequent management of the ichthyosis and any associated
hearing loss should include periodic otoscopy and bi-annual
audiograms the first years of life.
REFERENCES
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ichthyosis. [Accessed May 8, 2015].
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