European Annals of Otorhinolaryngology, Head and Neck diseases 135 (2018) 217–219

Available online at

ScienceDirect
www.sciencedirect.com

What is your diagnosis?

Anosmia and nasal obstruction

J.J. Braun a,∗ , S. Riehm b
a
Service ORL-CCF, hôpital de Hautepierre, avenue Molière, 67098 Strasbourg cedex, France
b
Service de radiologie 1, hôpital de Hautepierre, avenue Molière, 67098 Strasbourg cedex, France

1. Clinical history
A 61-year-old man consulted for longstanding anosmia. He also
reported a history of known birch pollen allergy and progres-
sively deteriorating right nasal obstruction. Otorhinolaryngological
examination revealed simple inflammatory rhinitis with very diffi-
cult access to the right nasal cavity due to marked septal deviation.
The imaging assessment (CT and MRI) demonstrated partial opaci-
fication of the nasal cavities and paranasal sinuses by a tissue
proliferation, which was much more marked on the right side
(Fig. 1a to d).

∗ Corresponding author at: 8, Quai Kellermann, 67000 Strasbourg, France.

E-mail address: braun.jean-jacques@wanadoo.fr (J.J. Braun).

https://doi.org/10.1016/j.anorl.2018.03.002
1879-7296/© 2018 Elsevier Masson SAS. All rights reserved.
218 J.J. Braun, S. Riehm / European Annals of Otorhinolaryngology, Head and Neck diseases 135 (2018) 217–219

Fig. 1. a: CT scan, sagittal view; b: MRI coronal T2-weighted sequence; c: MRI coronal T2-weighted sequence; d: HR MRI coronal T2-weighted sequence (FIESTA sequence).

What is your diagnosis?

 J.J. Braun, S. Riehm / European Annals of Otorhinolaryngology, Head and Neck diseases 135 (2018) 217–219
2. Responses
Three diagnoses can be proposed to explain this patient’s clinical
features:
• nasal polyposis with a polyp in the right nasal cavity with pos-
teroinferior extension implanted on the anteroinferior wall of the
right sphenoid sinus (Fig. 1a and b);
• respiratory epithelial adenomatoid hamartoma of the olfactory
clefts with marked right predominance (Fig. 1c) confirmed by
histological examination;
• bilateral agenesis of the olfactory bulbs and hypoplasia of the
olfactory sulci (Fig. 1d).
3. Comments
Nasal polyposis would be somewhat atypical in view of the
very asymmetrical signs and the essentially posteroinferior exten-
sion (Fig. 1a and b). Histological examination demonstrated simple
eosinophil-rich nasal polyposis.
Respiratory epithelial adenomatoid hamartoma has been more
recently described (Wenig and Heffner 1995) and is not well
known by otorhinolaryngologists, radiologists and pathologists. It
predominantly arises in the olfactory clefts. On endoscopic exami-
nation, respiratory epithelial adenomatoid hamartoma often has a
yellowish appearance with a firmer consistency on palpation com-
pared to nasal polyposis. On CT imaging, the olfactory clefts are
homogeneously opacified with essentially transverse extension [1].
219
On MRI (T2-weighted sequence), the clearly circumscribed opacifi-
cation has a suggestive cerebriform appearance in this context [2]
(Fig. 1c).
Congenital anosmia is rare and may be either isolated [3] or part
of a more complex syndrome, as in some forms of ciliopathies such
as Bardet-Biedl syndrome [4].
This patient had no olfactory reference since childhood. This
very longstanding anosmia was confirmed by olfactometry accord-
ing to the UPSIT technique.
The MRI assessment demonstrated agenesis of the olfactory
bulbs and hypoplasia of the olfactory sulci (Fig. 1d) explaining the
anosmia, while the clinical history confirmed its congenital nature,
unrelated to nasal polyposis or hamartoma.
Disclosure of interests
The authors declare that they have no competing interest.
References
[1] Nguyen DT, Gauchotte G, Arous F, Vignaud JM, Jankowski R. Respiratory epithe-
lial adenomatoid hamartoma of the nose: an updated review. Am J Rhinol Allergy
2014;28(5):187–92.
[2] Braun JJ, Riehm S, Averous G, Billing A, Veillon F. MRI in respiratory epithelial
adenomatoid hamartoma of nasal cavities. J Neuroradiol 2013;40(3):216–9.
[3] Hummel T, Whitcroft KL, Andrews P, et al. Position paper on olfactory dysfunc-
tion. Rhinology 2017;54(Suppl 25):1–30.
[4] Braun JJ, Noblet V, Kremer S, et al. Value of MRI olfactory bulb evaluation in
the assessment of olfactory dysfunction in Bardet-Biedl syndrome. Clin Genet
2016;90(1):79–83.