Volume 3, Issue 4 , Pages 155-157, December 2008
Proboscis lateralis: A case report
Article Outline
- Summary
- 1. Introduction
- 2. Clinical case presentation
- 3. Discussion and comments
- Acknowledgment
- References
- Copyright
Summary
We present a case of proboscis lateralis, a rare congenital deformity, in a female gender patient, which describes the lesion, the physical examination, the tomographic study, the transnasal flexible laryngoscopy, and lesion histology.
A literature review was performed regarding the associated malformations described, the embryological pathogenesis, as well as considerations on the surgical treatment.
Keywords: Congenital, Nose, Abnormalities
1. Introduction
Proboscis lateralis is a rare congenital anomaly which occurs in approximately 1 each 100,000 births [1]. There are just a few cases related in literature. It is typically described as a tubular, pendulum-like structure, with a consistency similar to the lateral nasal wall, which usually originates in the inner corner of the orbit, and, atypically, outside of it. It has a small longitudinal central channel, with a stratified epithelium, which discharges mucus, draining it to an orifice in the distal. The proximal end is closed, and it has no communication with adjacent structures. It is usually associated to nasal anomalies on the same side, with malformations in the nose area in most cases, as well as with heminasal aplasia or hypoplasia. Nasal cavity is partially developed, and the nose is rarely normal [2], [3], [4].
Such malformation is followed by other anomalies, and the ophthalmic ones being more frequent, present in 44% of the patients (anophthalmia, microphthalmia and colobomas), facial anomalies in 38% (maxillary hypoplasia or facial clefts) and craniocerebral anomalies in 18% encephalocele and holoprosencephaly [5], [6].
Iris coloboma and cleft palate and labial clefts are more frequently associated. Histopathological examination shows the skin with sebaceous glands, conjunctive tissue with sebaceous glands and striated muscular fibers of the same type found in nasal muscles, and some cartilaginous element, corresponding to the normal sidewall nose cartilage [5].
The treatment adopted is surgical, with the proboscis as the ideal donor for the reconstruction of the nasal deformities associated, due to its texture, color and neighboring location characteristics. Surgery is recommended before 4 y.o., before school age, due to the psychological consequences both for the patient and the family [6], [7], [8], [9].
Proboscis is apparently derived from the lateral nasal process, when several ipsilateral nasal abnormalities are observed. In the case of proboscis formation with a normal nose development, the possibility of an hypertrophy of a small part of the lateral nasal process is, remaining from the normal tissue, unleashed by an exogenous stimulation, during the 3rd or 4th week of fetal life [10], [11].
Congenital malformations of the nose are rare and result from an aberrant embryological development. Differential diagnoses include duplications, polyrrhinia, supernumerary nostril, and proboscis lateralis.
2. Clinical case presentation
Patient MGM, 4 days of life, female gender, pheodermic, attended in the pediatric otorhinolaryngology ambulatory of Pam Saudade-SUS from Belo Horizonte, in February, 1999. She had normal body measurements at birth.
She presented a tubular, superficial, soft lesion, originated in the upper part of the left side nasal wall, covered by normal-colored skin. The lesion had approximately 1.5
cm length×1.0cm wide (Fig. 1). The terminal end was soft, flexible, and presented an outer orifice which drained serous secretion and a longitudinal inner channel, without any apparent communication with the left nasal fossa. The nose was apparently well formed with unobstructed fossae and a moderate left septum deviation. The oral cavity examination, otoscopy, and the general physical examination did not show any other abnormalities. The child had a good general state, without any impairment on normal activities such as feeding and breathing. The lesion was an isolated finding, unrelated to any underlying systemic disorder or specific genetic syndrome.
Fig. 1.
Probocis lateralis in the left side nasal wall.
Gestational history without any serious events. Primiparous mother, without any reports on using medications and episodes of infection during the gestational period. There is no family history on malformations or consanguinity. Normal hospital delivery, term pregnancy without any events.
CT scan (Fig. 2) has shown a vegetant tubular-like lesion, in the left nasal region, with density in the soft parts, without infiltrative characteristics over the adjacent structures and without evidences of other facial and cranial malformations associated.
Fig. 2.
CT scan: vegetant tubular like lesion in the left nasal region.
The transnasal flexible laryngoscopy has shown unobstructed nasal fossae, with left nasal fossa stricture by a septum deviation and the presence of seromucous secretion, unobstructed adenoids. Nasopharynx and larynx without any changes. When observing the orifice of the proboscis channel, we notice a channel of approximately 0.4cm diameter by 0.7cm length, with blind end, without communication with the left nasal fossa, draining serous secretion.
The patient was forwarded for surgical treatment in the plastic surgery service of Júlia Kubitschek Hospital-FHEMIG from Belo Horizonte.
The histopathological examination of the lesion has shown: proboscis with 1.5cm×1.2cm×1.1cm. Material comprised by skin flap, showing the section, cystic central area, coated with a squamous epithelium with an abundant fibrous conjunctive tissue, cartilaginous ring, and a lot of sebaceous glands.
3. Discussion and comments
Approximately 50 cases of proboscis lateralis were described in literature since it was first mentioned by Foster, in 1861, in his monography: Congenital Malformation of Human body [3]. The common characteristic of that anomaly is the presence of a tubular appendix, originated mostly in the inner corner of the orbit, and associated to some kind of nasal malformation [3], [4], [8], [12], [13], [14].
Initially, only cases involving Caucasian patients were described, but currently there are cases described in other racial groups, although they are less frequent. There is a predominance of the male gender, and there is no significant difference as for the location of the lesion to the left or to the right [3].
In 1985, Boo-Chai [3], described the 34 cases published until then dividing them into 4 groups: Group 1, proboscis with normal nose; Group 2, proboscis with malformation in the nose; Group 3, proboscis with nose, eyes, and annexes anomalies; Group 4, proboscis with nose, eyes, and annexes anomalies, and labial and palatine cleft.
Most cases described until then are found in Group 3. The case we describe presents a moderate deformity in the left nostril, thus belonging to the Group 2 of the classification herein.
The treatment is surgical, aiming at removing the proboscis, reconstructing the areas with malformations, and restoring their functions. There are several surgical techniques, depending on the location of the proboscis and the malformations associated. Most surgeries described used the very proboscis tissue for nasal reconstructions and grafts. Cases with a good nasal development, as the one described herein, must be surgically corrected during the first weeks of life, since there are no functional or anesthetic side-effects. Changes in nasal growth due to the early surgery are less severe and, if necessary, corrected further. There is a consensus about performing the surgical intervention as soon as possible, due to the social and psychological difficulties generated both to the family and especially to the child. In the case described herein, the surgery was performed when the child was 45 days of life, and because it was a practically normal nose, of a patient in good general state, without any other anomalies, and especially, because of the social repercussion in the family [7], [8], [9]. The proboscis was amputated, and its skin was used in order to repair the area where it was taken from.
Histopathological examination was compatible to the ones described in literature.
Acknowledgment
We are grateful to the Support Center for Scientific Publications of Santa Casa de São Paulo, Faculty of Medical Sciences for the editorial assistance.
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PII: S1871-4048(08)00020-8
doi:10.1016/j.pedex.2008.03.001
© 2008 Elsevier Ireland Ltd. All rights reserved.
Volume 3, Issue 4 , Pages 155-157, December 2008
