Professional Documents
Culture Documents
DOI 10.1007/s00266-012-9912-3
Received: 28 October 2011 / Accepted: 8 April 2012 / Published online: 31 May 2012
Ó Springer Science+Business Media, LLC and International Society of Aesthetic Plastic Surgery 2012
Abstract With the exception of septal deviation, con- suggesting that the cartilages had never been there and that
genital anomalies of the nasal cartilages constitute a small the problem was congenital. There was no family history of
fraction of all congenital nasal anomalies. This report the condition, no evidence of similar abnormalities in the
presents a patient with isolated agenesis of all the nasal parents, and no history of maternal drug use or infection
cartilages, which has never been recorded previously. during gestation. The blood test results were negative for
Level of Evidence IV This journal requires that authors syphilis (PRP and TPHA), and the patient had no history of
assign a level of evidence to each article. For a full inhalation of irritants such as cocaine or acidic fumes.
description of these Evidence-Based Medicine ratings, Physical examination demonstrated a collapsed nose, a
please refer to the Table of Contents or the online retracted columella, and a short bony dorsum with low
Instructions to Authors www.springer.com/00266. projection. The quadrangular septal cartilage and the lower
and upper lateral cartilages (LLC/ULC) were absent. The
Keywords Congenital agenesis Congenital nasal nose was composed of fleshy alar lobules with deep creases
anomalies Nasal cartilage between each lobule and the alar portion of the nose
bilaterally, perhaps due to the lack of LLCs. The nasal tip
was completely collapsed and lacked definition and pro-
Case Report jection, with creases in the region where the soft triangle
normally is located. The vertical dimension of the upper lip
A 6-year-old girl was referred to our clinic with a report of was increased relative to the projection of the nose.
a flattened nose (Fig. 1). Her medical history showed no The use of ultrasound and computed tomography (CT)
maxillofacial trauma, nasal or midface surgery, or previous scanning confirmed the absence of the quadrangular septal
treatment for any nasal problems. Her parents indicated cartilage, ULC, and LLC, but there were no other anom-
that they noticed this deformity a few months after her birth alies in the craniomaxillofacial region (Fig. 2). A system-
and that the problem persisted as she grew up. atic examination of the patient’s organs showed only a
Palpitation of her nose indicated no cartilaginous small ventricular septal defect, with no other abnormalities
structures in the external portion of the nose or the septum, in the lungs, liver, kidneys, extremities, or genitals.
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Aesth Plast Surg (2012) 36:946–948 947
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948 Aesth Plast Surg (2012) 36:946–948
midface with a flat, small nose and results from maxillary References
hypoplasia. In Robinson and Hilger’s patient, the ULC
remained, and anomalies in other cartilages including the 1. Millard DR Jr (1994) Embryonic rationale for the primary
correction of classical congenital clefts of the lip and palate.
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In the reported case, the only anomaly observed is the 2. Patten BM (1961) The normal development of the facial region. In:
complete absence of the nasal cartilages. The patient has a Pluzansky S (ed) Congenital anomalies of the face and associated
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impossible to define the exact embryologic mechanism, the Otorhinolaryngol 12:311–326
deformity observed in our patient may have been caused by 4. Scott JH (1953) The cartilage of the nasal septum (a contribution
arrest in formation, inadequate ossification, or reabsorption to the study of facial growth). Br Dent J 95:37–43
5. Robinson B, Hilger P (1989) Hereditary agenesis of nasal
of the anteroinferior part of the primitive nasal septum [6]. cartilage: surgical implications. Arch Otolaryngol Head Neck
At the time of consultation, we did not obtain samples Surg 115:985–988
for genetic analysis. However, it would be interesting to 6. Mohri M, Amatsu M (2000) Congenital defects of the vomer. Ann
observe whether this patient shows evidence of genetic Otol Rhinol Laryngol 109:497–499
traits linked to other congenital nasal anomalies or whether
this is definitely a new syndrome. Our major question at
this writing is how to reconstruct the deformity, and we
invite surgeons with experience in this type of facial sur-
gery to contact us with advice on how to proceed.
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