L A T E R A L NASAL PROBOSCIS

By B. W. FRANKLINB1SHOP, F.R.C.S.E.
Durban, South Africa
EMBRYOLOGICALLY this condition presents a very interesting problem, and to
understand it, one must recapitulate briefly the development of the nose.
The nasal placodes invaginate to form the nasal pits, which are forerunners of
the nasal cavities. The boundaries of the pits are the medial and lateral processes,
which form the external nose. It is likely that lateral proboscis results from
malformation of these nasal processes. The two lateral nasal processes develop
into the lateral nasal wall and aim nasi. At the outer extremities these lateral
nasal processes meet the maxillary processes, which do not form any part of the
lateral nasal wall, and are at that stage of development divided by a cleft, the
lachrymal nasal cleft, running obliquely down from the eye.
The long medial nasal processes are fused on both sides through their lower
parts (the process globulares) with the short lateral ones forming simultaneously
the lower circumference of the anterior nares and vestibule. In those cases where
the nose on the same side showed normal development, the proboscis probably
developed from a variable proportion of the lateral nasal process. Where the nose
on the same side is entirely absent, one can assume that the proboscis was derived
from both lateral and medial nasal processes.
The dimple at the free end of each proboscis probably represents the original
olfactory pit which failed to form the nasal cavity and this would account for the
absence of nasal cavities.
In my case there was a central canal lined with epithelium running the entire
length of the proboscis, while there was, in the posterior half on the left side, an
attempt at formation of a nasal cavity.
Some writers are of the opinion that the medial and nasal processes may be
said to have taken part in the formation of a lateral nasal proboscis, only if the parts
normally formed by the medial process are missing. According to them, the
structures so formed include the pre-maxillary bone, and one would therefore have
expected that in the case described by Rosen and Gitlin all the incisor teeth would
have been missing. In my case all the incisor teeth were present.
The above theory is capable of two alternative interpretations. (I) The
pre-maxilla (globular process) is usually regarded as the lower part of the medial
nasal process, but according to Streeter (I948), the pre-maxilla growth centre is
distinct from the medial nasal process. It would thus seem possible for distinctive
development of the medial nasal processes to occur with the pre-maxillary centre
being unaffected. The latter would then go on to form the central part of the
upper jaw, including incisor teeth and their alveolar processes. (2) Alternatively
it may be argued that the pre-maxillary centre also enters completely into the
formation of the proboscis, and that in its absence the maxillary process reaches the
midline. The incisor teeth would then be formed from the maxillary processes.
The relationship of lateral proboscis to the condition known as split nose is
also of interest. It has been maintained that the lateral nasal proboscis could be a
variant of the median split nose. In some cases of the latter the two halves of the
18
 LATERAL NASAL PROBOSCIS 19
external nose are almost tube-like in formation. No explanation can be offeredfor
the occurrence of this conditionin my case. It was associated with hydramniosin
the mother.
The existenceof other congenitalanomaliesof the gastro-intestinaland cerebral
nervous systemhas been noted.
In presenting this most unusual and relatively rare condition, first described
by Selenkoffin I884, I hope that it will stimulate discussion and also add to the
list of reported cases.
In a perusal of available medical literature, I could find only three references
to the condition : Blair and Brown (I931) ; Biber (I949) ; and Rosen and Gitlin
(i959).
Case of Rosen and Gitlin.--These writers described a case of bilateral nasal
proboscis which showed in addition pigmentation of the conjunctiva of the right
eye, the left eye showing anophthalmiaand coloboma of the upper lid. The child
did not survive.
It is apparent from the literature that the condition is associated with ocular
abnormalities. Rosenand Gitlin quote Seefelderas findingassociatediris coloboma
while the olfactorius and cribriform plate were missing. They also quote Landow,
Tarufl~, and Buyardiwho found lower lid clefts at the inner angle ; KJrchmayrwho
found iris and choroidal clefts ; Zacherli who found a high arched palate ; Peter
who found a rudimentary eye and an upper lid cleft; and Tend]an who found
malformation of the upper lid, iris and retina.

Case of Biber.--Biber considers the conditionis relativelyrare. In reviewing

the literature he notes that the followingsuggestionshave been made : (I) germinal
anomaly ; (2) healed encephalococle; (B) exogen, or external factors such as
amniotic cords. For this to play a part, it must occur early in f~etallife in what he
calls the " earliest malformationperiod of four to five weeks." After reviewing
the embryologyof the nose and face generally,Biber considers that the lateral nasal
process provides the material for the formationof the lateral nasal proboscis. The
under-development or absence of the lateral nasal wall must be regarded as a
detached and dislocatedtransplant. He describes his case as a tube-like structure,
2 cm. long, arising from the region of the inner canthus of the orbit. It had a
central canal which was exuding clear fluid and which extended for about 3 cm.,
ending blindly and having no connectionwith the nasal cavity. Bothnasal bones
were palpable. On the right side the nasal wall was soft and the a]~c nasi were
absent. The tube was found to have no connection whatsoever with the nasa]
cavity.
The interesting thing to note is that the cases mentioned also showed various
degrees of malformationof the nasal part formedby this process, and differentcleft
formations in its neighbourhood, by failure of union of these processes, and it is
suggested that there is a linkage between these abnormal clefts and the condition
under discussion.
Biber states that his case did not have a coloboma of the lower lid as has been
described by others. This, he considers, may be explained by the onset of the
exogen at a somewhat later phase in f~etal development. He also mentioned that
in his case the tube was of elastic consistency, and was lifted up in a horizontal
position and shortened while the child cried, and at the same time a drop of clear
20 BRITISH JOURNAL OF PLASTIC S U R G ER Y

fluid appeared in the orifice. The tip of the nose was twisted slightly to the right,
as was the columella. There was no disturbance of the upper lip. There was
apparently no nasal cavity on the affected side, and the X-ray showed normal
development of the nasal skeleton on both sides. There were no eye abnormalities.
Biber refers to Peter's diagram, showing the part which various facial processes
play in the formation of the nose, and he says that judging from this we may presume
a selective failure of development of the lower part of the lateral nasal process,
suggesting that it is the lower part of the lateral nasal process which fails to fuse
through some noxious injury and so produces a dislocated appendage. In quoting
the various cases in the literature, he found that in all the previous cases the proboscis
was removed, and he quotes Professor Keith as stating that the eye should be
removed at the same time in order to avoid the formation of a fistula and subsequent
lachrymation, and that a fistula is particularly to be avoided since it may lead to
subsequent meningeal infections.
Biber states that as a result of considering all the cases published, it was
obvious that there was no connection with the inside of the skull, and my findings
on following the tube coincide with this. In his case he decided to utilise the
proboscis for the creation of the left side of the nose. He split the tube vertically
after establishing a communication with the right nasal cavity, by partial removal
of the covering layers behind which a normally developed nasal cavity was found.
After the proboscis had been split vertically, it was opened out and its lining was
used as fining for the new nasal cavity ; the stretched-out skin was sutured to the
skin of the nose in the midline and to the side of the face, and in that way the alto
nasi and the lateral wall were formed. Two weeks later the proboscis was cut off
its base, and the wound closed by a suture which healed rapidly. The operation
was done when the patient was six months old, but he considers that it should be
done after maturity, so that the growth problems will not be involved.
Case of Blair and Brown.--These writers called their patient a case of
half-nose. The right half of the nose was absent, and they described a tubular
fleshy mass on the right side growing from the region of the inner canthus, and
open below with a patulous tube of mucosa, which they thought might represent
either the lining of the missing half of the external nose or the naso-lachrymal duct.
The tears appeared to drain out through this tube. They also described cases of
half-nose where there were tubercles above the inner canthus, which might be
related to the condition under discussion. They attempted to correct their case by
using the tube with lining and covering to reconstruct the right half of the nose,
but this proved inadequate.
Personal Case.mI was presented with this condition in an African boy of
about 7 years of age. There was an absent left side of the nose, including the ala
nasi, and there was no external nasal orifice. The nasal bones on the left side were
present. Arising from the region of the left inner canthus was a tube-like structure
about I in. in circumference and about 2 in. long with the patent distal end. There
was a lined cavity, which appeared to go the whole length of the structure itself,
and there seemed to be a slight discharge from it. I did not find any elasticity in
the structure, and it did not appear to be capable of any movement. There
appeared to be some definite communication through the central canal with the
region of the lachrymal sac, but this could never be definitely established.
In every other respect the child was normal. There was no agenesis and the
 LATERAL NASAL PROBOSCIS 21
eyes were normal. No history was obtainable other than that he had been born
with this condition.
X-ray of his skull revealed nothing abnormal. I felt that the tube might
represent the naso-lachrymal duct, and at one stage in the treatment it was followed
up and found to end somewhere in the region of the lachrymal sac. There was no
apparent epiphora on that side. I planned an operation which unknown to me had
been performed by Biber in 1949.
T h e skin of the tube-like structure was dissected free from the lining tissue,
which was followed up as mentioned before, to the region of the lachrymal sac.
T h e nasal bone was split, and the tube-like structure was brought down to the
direction of the nose under the nasal bone. T h e skin over the absent left side of
the nose was elevated, and a dissection made to try to establish a nasal cavity.
After removal of bone and a good deal of soft tissue, a channel was established
with the posterior part of the left nasal cavity. The lining of the tube-like structure
was then brought down in an attempt to recreate lining of the nasal cavity. The
skin covering of the proboscis was then opened out fully and sutured to a cut edge
on the dorsal bridge line of the nose in the mid-line, and laterally to a prepared
wound edge in the naso-labial line on the left cheek. An attempt was made to
utilise the circumference of the free end of the skin of this tube-like structure, to
reproduce the left alar base. It took several adjustments to make anything look
really presentable, and I felt that up to the time of last seeing the patient a reasonable
result was obtained that would pass muster.
At the time the patient was last seem, there did appear to be some sort of nasal
passage on the left side, but unfortunately the child was returned to his kraal and
we have since lost all trace of him and the final result was not entirely satisfactory.
Histological F i n d i n g s . - - T h e following histological findings have been
reported. The skin covering shows sebaceous glands. The connective tissue
shows striated muscle fibres of the same kind as are normally found in the facio-
nasal mimic muscles, and some cartilaginous element corresponding to the normal
cartilage of the lateral nasal wall. T h e mucous lining contains a stratified columnar
epithelium. Some writers reported lobules of the typical structure of a lachrymal
gland, whilst others found only mucous glands. Nerve fibres have been found in
the subcutaneous tissues in the distal part corresponding to the nasal vestibule.
Small hairs, sebaceous and sweat glands and striated squamous epithelium have
also been found. In all the known cases described, there are the same morphological
and histological struetures--a soft and elastic trunk, club-shaped at its distal end,
with a small opening being an outlet of a canal running along the whole axis of the
trunk. Proximally it has a blind ending, and shows no connection with a more or
less developed nasal cavity.
I consider that I must agree with the suggested theory of origin of this condition
that it is a failure of the development of the lateral nasal process, but I also feel
that some abnormality in the construction of the nasal lachrymal cleft plays a part.
REFERENCES
BIBER, J. J. (1949). ft. Laryngol. Otolaryngol., 63, 734.
BLAIR, V. P. and BROWN,J. B. (1931). Surg. Gynec. Obstet., 53, 797.
ROSEN, Z. and GITLIN, G. (1959). Arch. Otolaryngol., 70, 545.
STREETER,G. L. (1948). Contr. Embryol. Carneg. Instn., 32, 133.
Submitted for publication, March I963.